Fundamental Otology: Pediatric & Adult Practice Quinton Gopen
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Auricle1

Quinton Gopen,
Sunita Bhuta
Chapter Outline
  • ❖ Anatomy
    • • Embryology
    • • General Anatomy
  • ❖ Disorders
    • • Congenital Malformations
      • – Microtia and Anotia
      • – Prominauris
      • – Preauricular Pits, Sinuses, and Cysts
      • – Skin Tags
    • • Infections—Bacterial
      • – Impetigo
      • – Erysipelas
      • – Auricular Abscess
    • • Infections—Viral
      • – Herpes-Zoster (Ramsay Hunt Syndrome, Herpes Zoster Oticus)
    • • Traumatic
      • – Lacerations
      • – Hematomas
      • – Avulsion
    • • Inflammatory—Skin Conditions
      • – Seborrheic Dermatitis
      • – Psoriasis
      • – Contact Dermatitis
      • – Excessive Scarring
    • • Inflammatory—Cartilaginous Conditions
      • – Chondritis
      • – Relapsing Polychondritis
      • – Auricular Pseudocysts
      • – Chondrodermatitis Nodularis Chronica Helicis (Winkler's Nodule)
    • • Neoplasms—Benign
      • – Solar lentigo (sun spots, freckles)
      • – Cylindroma
      • – Epidermal cysts (sebaceous cysts)
      • – Seborrheic keratosis
      • – Pilomatrixoma
      • – Vascular lesions
      • – Lymphatic malformations
      • – Nevi
    • • Neoplasms—Malignant
      • – Actinic keratosis
      • – Lentigomaligna
      • – Keratoacanthoma
      • – Basal cell carcinoma
      • – Squamous cell carcinoma
      • – Melanoma
      • – Rhabdomyosarcoma
      • – Merkel cell carcinoma
 
ANATOMY
 
Embryology
The auricle is derived from mesenchymal cells termed the Hillocks of His. These mesenchymal proliferations are located at the dorsal ends of the first and second pharyngeal arches clustered around the first pharyngeal cleft. The Hillocks of His surround what is to become the external auditory canal (from the first pharyngeal cleft). The Hillocks fuse together in a complex fashion to create the different subunits of the auricle. The first pharyngeal arch contains Hillocks one through three. The first Hillock makes up much of the tragus, with the second and third Hillocks creating the helical crus anteriorly (second 2Hillock) and the helix superiorly (third Hillock). The second pharyngeal arch then accounts for Hillocks four through six. The fourth and the fifth Hillocks fuse to become the antihelix and the sixth Hillock creates much of the anti-tragus and lobule.
 
General Anatomy
The external ear can also be referred to as the pinna or auricle. The skin overlying the cartilage of the external ear adheres tightly to the underlying cartilage anteriorly, but has a comparatively loose attachment to the cartilage posteriorly. The auricular cartilage has a complex configuration giving the ear its general shape and appearance (Fig. 1.1 and Table 1.1).
The helix of the auricle is the outermost rim of cartilage. It has a curved course that rolls anteriorly creating a small overhanging shelf. The antihelix is a raised portion of cartilage which runs just beneath the helix and follows its general course. As the antihelix courses superiorly and anteriorly, the antihelix divides into two crura, the superior antihelical crus and the inferior antihelical crus. These two crura define a depression in the cartilage anteriorly, termed the triangular fossa (also called the navicular fossa). The fossa between the helix and antihelix posterior to the triangular fossa is termed the scaphoid fossa or scapha. The conchal bowl is the inferior most sulcus, which starts just posterior to the external auditory meatus. Its posterior edge is created by the antihelical fold. The conchal bowl can be divided into two subunits based on the attachment of the helical crus, which is the anterior and inferior extension of the helical rim. Within the conchal bowl are the concha cavum, which lies below the helical crus, and the concha cymba, which lies above the helical crus. The tragus is a portion of cartilage just anterior to the external auditory canal and lies laterally on the bone of the anterior ear canal. The anti-tragus is a small raised portion of cartilage just opposite the tragus, located inferior and posterior to the tragal cartilage. The inferior sulcus between the tragus and anti-tragus is referred to as the intertragic notch. The lobule is the lowest portion of the auricle and is the only part of the auricle which does not contain any cartilage. It is comprised of fat and subcutaneous fibrous tissue.
Small postauricular muscles, controlled by the motor portion of the facial nerve, attach and support the posterior and medial portions of the pinna. The sensory nerves innervating the auricle come from the auriculotemporal nerve (anterior), the lesser occipital nerve (superior, backside of auricle) and the greater auricular nerve (posterior and inferior). A small component of the sensory innervation arises from the facial nerve and is carried via the nervous intermedius to innervate a small portion of the conchal bowl (Fig. 1.2).
The blood supply to the auricle is quite redundant and comes from branches of the external carotid artery, including the postauricular and superficial temporal branches (Fig. 1.3). The venous drainage is highly variable but often occurs in part through the postauricular vein into the external jugular system. The lymphatic drainage of the auricle drains into the superficial and deep lobes of the parotid anteriorly, and posteriorly into the postauricular and occipital nodal basins. The entire auricle drains into the cervical lymph node chains, particularly levels II–V (Fig. 1.4).
 
DISORDERS
 
Congenital Malformations
 
Microtia and Anotia
Microtia and anotia are developmental abnormalities of the normal auricle, which result in either a small, misshapen auricle (microtia) (Fig. 1.5) or a complete absence of the auricle (anotia).
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Figure 1.1: Auricular cartilage: The cartilaginous landmarks of the ear are labeled.
Table 1.1   Auricular cartilage and fossa
Helix
Outermost rim of cartilage
Antihelix
Raised prominence running just below helix
Superior antihelical crus
Superior and anterior extension of antihelix
Inferior antihelical crus
Inferior and anterior extension of antihelix
Triangular fossa (navicular fossa)
Fossa between antihelical crura
Scaphoid fossa (scapha)
Fossa between helix and antihelix posterosuperiorly
Conchal bowl
Sulcus residing posterior to the external auditory canal
Concha cavum
Subdivision of the conchal bowl lying inferior to the helical crus
Concha cymbum
Subdivision of the conchal bowl lying superior to the helical crus
Tragus
The anterior and lateral boundary of the external auditory meatus
Anti-tragus
Inferior and posterior to the tragus
Intertragic notch
Sulcus between the tragus and anti-tragus
Lobule
Just below the anti-tragus, contains no cartilage
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Figure 1.2: Auricle innervation.
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Figure 1.3: Auricle blood supply.
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Figure 1.4: Auricle nodal drainage.
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Figure 1.5: Microtia: A lateral view demonstrating microtia.
The auricle develops from the six axonal Hillocks of His which are located around the first pharyngeal cleft. They arise during the sixth week of gestation. Developmental abnormalities of the auricle can be divided into four subtypes or grades of severity as shown in Table 1.2. Grades I–III are increasingly severe forms of microtia, with grade IV representing anotia.
Microtia can be addressed surgically. Most clinicians recommend waiting until the age of six before undergoing any surgical corrections. This allows for the patient to be mature enough to cooperate in the postoperative care as well as allowing the rib cartilage to be of suitable size for use in grafting. The surgery is a complex sculpting of the rib cartilage with creation of a thin skin pocket to house the sculpted cartilage. The surgery involves several stages in many cases. Artificial tissues, such as medpore, can be used but carry a higher risk of infection and extrusion.
 
Prominauris
Prominauris can be defined as ears that protrude excessively from the side of the head. Normal ears protrude from the side of the head by less than 2 cm and an angle of less than 30 degrees (Becker 1949, Elliott 1990).
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Table 1.2   Developmental abnormalities of the auricle
Grade
Malformations
I
External auditory canal small but present
II
No external auditory canal or stenotic external auditory canal Partial external ear formation with helical remnant, antihelical remnant, lobule
III
Small external ear remnant and absent ear canal (most common) Vertical “peanut” of disorganized cartilage with a malformed lobule
IV
Anotia—complete absence of the external ear
The most common reason for prominauris is an underdeveloped antihelical fold. One common subtype of prominauris is the so-called lop ear, or cup ear. This occurs when the underdeveloped antihelical fold is combined with a central depression around the ear canal. Prominauris can be corrected with various types of otoplasty.
 
Preauricular Pits, Sinuses, and Cysts
Preauricular pits, sinuses, and cysts are all small openings just anterior to the helical root that course under the skin with variable terminations. Preauricular sinuses and cysts are similar to pits except they go deeper into the surrounding tissues and have a cyst at their termination. Pits, sinuses, and cysts all have an epithelial lining to the tract and are prone to infection as they build up trapped desquamated debris from their skin lined tracts. This desquamated debris can often be expressed with gentle pressure from the orifice and appears as a cheesy whitish substance.
The treatment of choice is surgical excision of the tract and any associated cystic components. It is crucial to differentiate these lesions from branchial cleft cysts, which can have much deeper tracts and can be intimately associated with the facial nerve, ear canal, or other structures. Excision involves an elliptical incision encompassing the opening in the skin. The excision of the tract may be facilitated with lacrimal probes and can be safely taken down to the underlying temporalis fascia if needed (Fig. 1.6).
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Figure 1.6: Preauricular pit: Marked by the arrow.
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Skin Tags
These are commonly found in the preauricular area and can be single or multiple. Some skin tags can contain cartilage and have a variable attachment. There is an increased risk for hearing loss as well and routine hearing screening is recommended. The skin tags can be removed with layered closure.
 
Infections—Bacterial
 
Impetigo
This is a superficial infection of the epidermis with Staphylococcus aureus. It appears as small, infected vesicles or pustules that quickly rupture and dry to produce a honey-colored crust. It is highly contagious. It is usually not painful but causes significant itching. Treatment is with topical ointment, such as bactroban, to the affected area.
 
Erysipelas
A superficial cellulitis caused by group Aβ-hemolytic Streptococcus. The auricle appears erythematous and is tender and swollen. It can quickly spread across anatomical boundaries and may be associated with systemic symptoms such as fever, chills, anorexia, and malaise. The infection tends to be well demarcated from surrounding uninfected skin. Appropriate antibiotics are indicated.
 
Auricular Abscess
Auricular abscesses represent a loculation of purulent infection. They present with pain, erythema, and fluctuance over the area of the abscess. They can arise from a hematoma that becomes infected, insect bites to the ear, or any progression of an infection in this area. The treatment is urgent surgical incision and drainage. The incision should be packed open to facilitate continued drainage. The appropriate antibiotics should be administered and adjusted based on culture results.
 
Infections—Viral
 
Herpes-Zoster (Ramsay Hunt Syndrome, Herpes Zoster Oticus)
Herpes-Zoster is a viral infection with the Varicella zoster virus which often leads to the presentation of Ramsay Hunt syndrome. This syndrome is manifested by facial paralysis along with vesicles within the ear, including on the auricle. The vesicles are usually in the distribution of a dermatome, most typically the mandibular distribution of the trigeminal nerve in the case of Ramsay Hunt syndrome. The syndrome is covered more in Chapter 6 on facial paralysis (Fig. 1.7).
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Figure 1.7: Herpes-Zoster Oticus: This is an example of herpes zoster oticus, with numerous vesicles seen in and around the ear along with hemorrhagic areas and focal areas of bacterial suprainfection. Many of the vesicles have started to crust over.
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Traumatic
 
Lacerations
Lacerations of the auricle can be superficial or deep involving the cartilage. All carry a risk of ensuing chondritis which must be identified and treated expeditiously. Most lacerations can be closed primarily with a small risk of infection. Every effort should be made to avoid leaving any exposed cartilage pieces, as this greatly increases the risk of subsequent chondritis. For wounds that are particularly dirty, such as human bites and grossly contaminated areas, primary closure may be contraindicated. In such cases, wound debridement with healing by secondary intention may be more prudent to keep the risk of infection at a minimum. This is particularly true if the injury occurred for a prolonged period of time before assessment. Antibiotic coverage of skin pathogens is generally recommended for most auricular lacerations to help minimize the risk of chondritis and necrosis of the auricle. As a general principle, the blood supply to the ear is robust and even questionable segments of the auricle usually survive after wound closure. To this point, wound debridement should be kept to a minimum with only non-viable pieces of tissue removed. The ear should be appropriately cleaned before closure along with tetanus prophylaxis. Postoperative antibiotic coverage is warranted, with high-risk injuries treated using intravenous antibiotics (Lavasani et al. 2010).
 
Hematomas
Trauma to the auricle, particularly from wrestling, is a common cause of auricular hematomas. As the head is pinned against the ground, rotational shearing forces result as the wrestler tries to maneuver. This is a common cause of auricular hematomas. These hematomas cause significant damage to the cartilage as the auricular cartilage obtains its nourishment through the perichondrial attachments. When a hematoma develops between the perichondrium and cartilage, the cartilage undergoes necrosis and often deformity. This deformity is commonly referred to as a cauliflower ear.
Consequently, it is important to drain auricular hematomas in a timely fashion and attempt to reapproximate the perichondrial layer with a compressive dressing. Simple aspiration with a needle has a high rate of recurrence. Therefore, a formal incision with evacuation of the hematoma is recommended in most cases. After drainage of the hematoma, a number of different techniques have been described for obtaining compression of the perichondrial layers. These include absorbable mattress sutures, cotton bolsters, dental roll bolsters with mattress sutures, thermoplastic splinting, and button compression. Some surgeons also advocate placing a drain within the evacuated site or even more aggressive excision of neocartilage and fibrinous healing tissue from the hematoma site. Antibiotics should also be administered, with fluoroquinolones a good choice as they offer exceptional cartilage penetration.
 
Avulsion
Avulsion of the auricle can be partial or complete (Fig. 1.8). Partial avulsion of the auricle may be amenable to direct re-attachment depending on the size of the segment involved. Total auricular avulsion remains a difficult problem requiring urgent treatment. In some cases, the detached auricle can be reattached with the blood supply repaired using a microvascular arterial anastomosis. The venous drainage to the ear, however, is difficult if not impossible to repair. Some clinicians advocate creating a postauricular pocket in the subcutaneous tissues where a portion or all of the detached auricle is placed to augment its blood supply and survivability. In some cases, salvaging the cartilage framework and placing it into a postauricular pocket is recommended for delayed repair with skin graft coverage (Fig. 1.9).
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Figure 1.8: Partial auricular avulsion: The superior portion of the helical rim has been avulsed along with the overlying skin and subcutaneous tissues. The cartilage of the inferior antihelical crus, the superior antihelical crus, the helical crus, the triangular fossa, the scaphoid fossa, and the antihelix superiorly is exposed (arrow). The patient remains in a cervical collar. The blue arrow marks the tragus for orientation.
 
Inflammatory—Skin Conditions
 
Seborrheic Dermatitis
This is a chronic inflammatory disorder presenting with flat, scaly, flaky, itchy skin with diffuse borders and usually occurs where sebaceous glands are present in the greatest number. This includes around the ear, nose, and scalp. The flaking or scaling of the skin is usually quite persistent. Patches of white or yellow scales form commonly within the ear, particularly in the postauricular crease but also within the external auditory meatus. The yeast Pityrosporum ovale has been implicated in this condition. When this condition occurs in neonates, it is termed cradle cap. This cap is hypothesized to be caused from excess skin oils combined with a fungal infection from yeast called Malassezia. It has a higher incidence in patients with HIV or Parkinson's disease but it is not contagious. It may be difficult to distinguish seborrheic dermatitis and psoriasis in many cases as they present in a similar location and with similar lesions. Treatment consists of topical creams containing ketoconazole, selenium, or corticosteroids.
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Figure 1.9: Postauricular pocket for avulsion: The exposed cartilage has been cleaned and buried in a subcutaneous pocket created above the ear. The arrow denotes the site of the skin incision with buried cartilage underneath.
 
Psoriasis
Psoriasis affects as many as 2% of the population and is an autoimmune inflammatory disorder of the skin that results in thick, red, flaky skin in patches, or silvery scales that are often heaped up (Christophers 2003). There may be irritants that cause the condition, which typically has episodes where it flares up. Common irritants include trauma, stress, infections, and certain medications such as β-blockers, lithium, and angiotensin-converting-enzyme (ACE) inhibitors. The condition tends to 10improve with sun exposure (Garvey et al. 2008). Although it is commonly found on the elbows, knees, and scalp, psoriasis can also be found on the external ear. When it involves the ear, it may not demonstrate all of the classic findings but instead may present with nonspecific red, dry skin that itches (Garvey et al. 2008). Common locations include the postauricular crease, the conchal bowl, and the entrance to the external auditory meatus. Treatment includes topical corticosteroids as well as vitamin D ointments applied to the affected areas. Creams may be more effective for the outer ear whereas drops may be better for the external auditory canal (Fig. 1.10).
 
Contact Dermatitis
This is a skin reaction that occurs after direct contact with an offending agent. It can be due to contact with an allergen or a caustic exposure.
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Figure 1.10: Auricular psoriasis: Psoriasis of the conchal bowl and preauricular crease is marked by the separate yellow arrows.
Contact dermatitis typically lasts for several days and results in an erythematous patch, which is quite itchy and may burn. Common offending agents include various plants, such as poison ivy or poison oak, or various chemicals. A common cause of contact dermatitis on the external ear is the use of jewelry containing nickel. Other metals can cause contact dermatitis, but nickel is notorious for causing contact dermatitis and is often present in jewelry, such as 14-carat gold. Treatment is to remove the offending agent. Antihistamines may be helpful in controlling the itching and hydrocortisone cream can be applied directly to the area to downregulate the immune response. Severe cases can be treated with oral corticosteroids.
 
Excessive Scarring
Keloids and hypertrophic scars: Hypertrophic scars and keloids are considered abnormal responses to scar formation. Keloid scars are firm, raised, fibrous scars that extend beyond the boundaries of the original wound and typically do not improve over time. They also commonly grow back after excision. Hypertrophic scars typically stay within the boundaries of the original wound and tend to improve or regress over time. Although their histopathologic appearance is similar with both containing excess amounts of type III collagen, keloid scars have randomly organized collagen fibers in excess, whereas hypertrophic scars have a more normal parallel alignment to the collagen fibers. Keloids have an increased incidence in darker pigmented skin such as that of Blacks, Hispanics, and Asians. Hypertrophic scars are less associated with darker pigmented skin. Keloids are also more common in younger ages, with a peak risk between 10 years and 20 years of age. Sites most commonly affected include the sternum, shoulders, upper arms, earlobes, and cheek areas. Trauma and burns carry a high risk of keloid formation as well (Fig. 1.11).
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Figure 1.11: Auricular Keloid: Two large keloid scars can be seen attached to the lobule.
Many treatments have been developed with variable success. Keys to minimizing keloid scar formation are closure of the wound in a tension-free manner, avoidance of excessive manipulation of the tissue with trauma, and minimization of any nidus for infection, all of which decrease the risk of keloid formation and hypertrophic scars. A mainstay of treatment is excision with appropriate closure and injection of corticosteroids into the resection bed. Silicone sheeting, pressure bandages, pulsed-dye laser, and cryotherapy have also been used with some success. A combination of the above therapies also increases the chance of resolution after excision. If conservative measures such as these fail, low-dose radiation has a high rate of cure for these lesions (Juckett and Hartman 2009).
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Figure 1.12: Auricular chondritis: Demonstrated in this picture as erythema involving the cartilaginous portions of the auricle. Note the relative sparing of the lobule, a structure that does not contain cartilage.
 
Inflammatory—Cartilaginous Conditions
 
Chondritis
Chondritis of the auricle is, by definition, any inflammatory process involving a portion or all of the cartilage of the ear. Common causes include mechanical trauma, infections, and rheumatologic diseases. Chondritis of the auricle presents as a painful, erythematous, edematous pinna. The lobule is not involved as it does not contain cartilage (Fig. 1.12).
Mechanical trauma results from sporting activities, in particular wrestling, as well as surgical manipulations and other trauma to the ear. Hematomas, as discussed earlier, can result in chondritis and cartilage destruction. Chondritis can also occur from surgical 12manipulations, particularly placing suture through cartilage or exposing cartilage as part of the surgical procedure. A recent review found auricular chondritis occurred in 5.6–12.2% of patients undergoing Moh's surgical resection for auricular lesions (Kaplan and Cook 2004; Mailler-Savage et al. 2008). Every attempt at covering exposed cartilage at the end of the procedure will minimize the risk of chondritis. Ear piercings and acupuncture are additional forms of trauma that can also lead to chondritis. A recent outbreak of chondritis after ear piercings was linked to pseudomonal infections from contaminated aftercare solution (Fisher et al. 2005).
Infections are another common cause of auricular chondritis. Infections are more commonly found in the ear after surgical manipulations when compared to other anatomic areas (Futoryan and Grande 1995). The skin is quite adherent to the cartilage, particularly anteriorly, and all but the most superficial infections of the skin involve some degree of the underlying cartilage. The infectious process may start as an otitis externa and progress to involve the auricle. In some cases, bites to the ear from small insects can also lead to subsequent infection. Antibiotic coverage of bacterial pathogens is important, with pseudomonal and staphylococcal infections being the most prevalent.
Burn injuries can also lead to chondritis through direct thermal damage. The tissue proteins are denatured by heat with ensuing inflammation and scar formation. Burns often cause significant contracture of the skin over the cartilage during healing with resulting deformity. Silvadene cream can be applied topically to help the healing process and prevent infection, with aggressive wound care and debridement critical for an optimal result. Any necrotic cartilage should be removed. Depending on the depth and scope of the burn, the auricle may not be viable.
Chondritis can occur as an inflammatory response resulting from an autoimmune condition. Any patient with recurrent auricular chondritis in the absence of any trauma or inciting factors should be thoroughly worked up for potential rheumatologic diseases. Relapsing polychondritis in particular should be excluded. A sedimentation rate may be helpful in this regard as it is almost always elevated in relapsing polychondritis. Biopsies are occasionally useful in the diagnosis but often show only non-specific changes. Other autoimmune conditions that can present with auricular chondritis include Wegener's granulomatosis and systemic lupus erythematosus.
 
Relapsing Polychondritis
This is an autoimmune condition whereby autoantibodies are generated towards cartilage, usually type II collagen. The disease was first described by Jaksh-Watenhorst in 1923 who termed it “polychondropathia”; then, in 1960, Pearson introduced the name “relapsing polychondritis”. Type IV collagen is involved less frequently. Over half of patients carry the allele HLA-DR4 (Lang et al. 1993). Up to one third of patients have a concomitant rheumatologic disease, such as systemic lupus erythematosus or rheumatoid arthritis among others (Gergely and Poor 2004). Patients can also have involvement of the inner ear, including sensorineural hearing loss and vestibular dysfunction. Inner ear involvement in relapsing polychondritis is covered more thoroughly in Chapter 10. Unlike the cartilage inflammation which typically improves dramatically with corticosteroid therapy, the hearing loss due to this condition does not tend to improve with therapy (Bachor et al. 2006). Although the pinna is one of the more common sites of involvement in this disorder, other sites of cartilage can be involved, including the nose, 13eyes, larynx, trachea, ribs, cardiac valves, and joints. The condition tends to involve the elastic cartilaginous tissue. Biopsies of the tissue tend to be nonspecific but include perichondrial inflammation with fibrous tissue ingrowth and inflammatory mediators.
A set of criteria for the diagnosis of relapsing polychondritis has been proposed and modified throughout the years (McAdam et al. 1976). Three of the following six criteria must be met to confirm the diagnosis of relapsing polychondritis:
  1. Recurrent auricular chondritis
  2. Inflammatory arthritis
  3. Nasal chondritis
  4. Ocular inflammation
  5. Chondritis of the respiratory tract
  6. Cochlear or vestibular damage (sensorineural hearing loss, tinnitus, vertigo, ataxia).
Others have come up with less strict diagnostic criteria. Damiani considers relapsing polychondritis confirmed if only one of the major McAdam's criteria are met with confirmatory histology, or if two separate lesions are identified with a commensurate response to medical treatment (Damiani and Levine 1979). Michet gives confirmation of relapsing polychondritis if any two of the following three sites are involved: auricle, nasal, and laryngotracheal. Alternatively, if only one of these three sites are involved but the patient has ocular inflammation, hearing loss, vestibular dysfunction, or seronegative arthritis, the diagnosis is confirmed (Michet et al. 1986).
Treatment is with high dose corticosteroids slowly tapered over time. A typical dose of prednisone is 0.5–1.0 mg/kg/day. Dapsone can also be used at a dose of 100–200 mg/day. Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes helpful as are other immunosuppressive medications, including azathioprine, methotrexate, hydroxychloroquine, colchicine, cyclophosphamide, cyclosporine, and infliximab (Rapini and Warner 2006). Surgical therapy to reconstruct any devitalized cartilage is important particularly in the respiratory tract where a loss of cartilage can result in respiratory distress. Current mortality rates for the disease are around 6% (Michet et al. 1986).
 
Auricular Pseudocysts
These are rare intracartilaginous fluid collections that present on the anterior surface of the auricle. They are also termed idiopathic pseudocystic chondromalacia. Although there may be a history of minor trauma, it often arises without a cause. It is termed a pseudocyst because the cyst cavity lacks a true epithelial lining and presents within the cartilage. It is a painless swelling with intact skin that may show minimal signs of inflammation (Fig. 1.13). Simple aspiration results in a high rate of recurrence. Treatment options include aspiration followed by intralesional steroid injection, systemic corticosteroid treatment, and formal pseudocyst excision.
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Figure 1.13: Auricular pseudocyst: A pseudocyst is shown involving the posterior aspects of the concha cavum and concha cymbum as demarcated by the arrow.
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Many surgeons employ a bolster or other type of pressure dressing after the pseudocyst has been evacuated. The difficulties arising from pseudocyst formation are its high rate of recurrence and its propensity to damage the delicate cartilage leading to auricular deformities.
 
Chondrodermatitis Nodularis Chronica Helicis (Winkler's Nodule)
This is a very painful erythematous nodule that appears on the helix or antihelix of the auricle. There is often an adherent scab on the surface. These nodules are not considered neoplastic but rather arise from chronic inflammation. They tend to occur in males over the age of fifty (Wade 1979), more commonly in men with fair complexion. Although the exact mechanism of formation remains unknown, many think it is due to trauma; either from chronic pressure, sun exposure, or excessive cold, or other causes of chronic inflammation and low grade perichondritis. This sets up a cascade of chronic dermal inflammation with edema, necrosis, and ultimately nodule formation as seen in the condition. There is also an association with autoimmune diseases.
The nodule enlarges initially and reaches a maximum size where it remains. The nodule is quite firm and raised from the surrounding skin. Histopathologically, these lesions appear as chronic ulcers with surrounding granulation tissue. There is ulceration of the skin surrounding necrotic cartilage often infiltrated by inflammatory cells and bacteria. Although avoidance of the inciting trauma can improve the lesions, many patients require surgical removal of the lesions which is definitive. Intralesional steroids and cryotherapy have also been used but with limited success.
 
Neoplasms—Benign
 
Solar Lentigo (Sun Spots, Freckles)
These are hyperpigmented light brown or black flat lesions that form from excess sun exposure. They are formed by melanocytes that produce excess melanin in response to the sun exposure. They can be single or multiple. They are considered benign but do denote excess sun exposure, putting the patient at risk for various other neoplasms. If cosmetically bothersome, the lesions can be removed with liquid nitrogen, chemical peeling, or lasers. Hydroquinone is somewhat less effective and reliable in treating these lesions.
 
Cylindroma
This tumor arises from the epidermal adnexal structures, and is also known as a turban tumor. It occurs on the external ear and histopathologically contains rounded masses of small dark cells that appear as a puzzle-like configuration with surrounding eosinophilic material.
 
Epidermal Cysts (Sebaceous Cysts)
Epidermal cysts, also called sebaceous cysts, occur when a collection of cells become trapped beneath the skin. This can occur from hair follicles or skin trauma. The trapped cells secrete keratin which collects within the cyst. The cyst contains foul smelling debris and is prone to infections. If infected, the cyst may drain spontaneously (Fig. 1.14). Excision is the treatment of choice and if acutely infected, an incision and drainage may be required first, with planned excision once the infection has been treated.
 
Seborrheic Keratosis
Seborrheic keratosis are very common benign epithelial tumors that are related to excessive sun exposure.
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Figure 1.14: Auricular epidermal inclusion cyst: A small epithelial cyst can be seen emanating from the inferior aspect of the helical rim posteriorly.
They typically begin to appear in middle age and increase in number with time. They appear to be “stuck onto” the skin and have a pigmented appearance (Fig. 1.15). They are usually asymptomatic but can become inflamed with ensuing tenderness or itching. They can be removed with chemical application of trichloroacetic acid, tazarotene cream, cryotherapy, or surgical excision.
 
Pilomatrixoma
A pilomatrixoma is a benign growth that arises from cells responsible for hair growth. It is a subcutaneous firm nodule that occurs more commonly in children than adults. A pilomatrixoma often contains calcifications. It tends to enlarge slowly over time and is usually otherwise asymptomatic. The treatment of choice is surgical excision.
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Figure 1.15: Auricular seborrheic keratosis: A small postauricular seborrheic keratosis is marked by the arrow.
 
Vascular Lesions
Vascular lesions are the most common congenital abnormality (Tucci et al. 2009). Mulliken has divided vascular lesions into two major categories: hemangiomas and vascular malformations (Mulliken and Glowacki 1982).
 
Hemangiomas
Hemangiomas are benign vascular tumors of the endothelial cells that appear as a red discolored area on the skin. They are usually not present at birth, appear in the first weeks of life, and grow in early infancy followed by spontaneous resolution later in childhood. Hemangiomas can be divided into rapidly involuting and non-involuting. They can also be categorized into capillary hemangiomas and cavernous hemangiomas. Cavernous hemangiomas have much larger blood vessels and contain thicker vessel walls 16than capillary hemangiomas. Hemangiomas are asymptomatic. They present quite frequently at birth and often grow rapidly for several months. Many hemangiomas go on to involute over the first decade of life and do not require specific treatment. Treatment options include oral corticosteroid therapy, intralesional corticosteroids, β-blockers, interferon, vincristine, and surgical removal.
 
Vascular malformations
These can be divided into low flow and high flow malformations. Low flow malformations are subcategorized into capillary, venous, and lymphatic malformations. High flow malformations are artero-venous and mixed malformation. Vascular malformations are structural abnormalities present at birth. They grow in proportion to the body and do not involute spontaneously.
 
Lymphatic Malformations
Lymphatic malformations are benign abnormalities of the lymph system. They are currently considered a subclassification of vascular anomalies under the category of vascular malformations. Lymphatic malformations can be categorized as macrocystic, microcystic, and mixed. In macrocystic lesions, the cysts are equal to or greater than 2 cm3 in size and can be single or multiple. Microcystic lesions contain cysts smaller than 2 cm3 and mixed lesions contain an element of both macrocystic and microcystic lesions. Lymphatic malformations involving the auricle are uncommon (Fig. 1.16).
Lymphatic malformations are notoriously infiltrative and tend to occur in close proximity to vital structures in the head and neck. Options for management include surgical removal, but their infiltrative nature makes this difficult in many cases. Subtotal resection often results in expansion and proliferation of the residual malformation. Sclerotherapy is used often in these lesions. Bleomycin, ethanol, doxycycline, and most recently OK-423 have all been used with success according to the literature (Renton and Smith 2011).
zoom view
Figure 1.16: Auricular lymphatic malformation: An extensive postauricular and auricular lymphatic malformation is shown here which is displacing the ear anteriorly.
 
Nevi
Nevi can vary in size and shape. They can be present at birth or appear later in life. Histologically, they appear as collections of melanocytes. Dysplastic nevi are bigger than ordinary nevi and have a more irregular contour and color pattern. These dysplastic nevi have a greatly increased risk of degeneration into melanoma. The larger the nevi, the greater the incidence of malignant degeneration into melanoma. Surgical excision is performed if the mole is concerning or bothersome.
 
Neoplasms—Malignant
In general, malignant neoplasms of the auricle are fairly common. This can be attributed to frequent sun exposure, particularly in men. Several independent investigators have estimated auricular malignancies to comprise around 5% of all cutaneous malignancies (Pless 1976; Bailin et al. 1980; Bumsted and Ceilley 1982).17
 
Actinic Keratosis
Actinic keratosis, also termed solar keratosis or senile keratosis, is a premalignant lesion that occurs from sun exposure. It appears as an erythematous patch of skin that is often raised from the surrounding healthy skin. It tends to have a flat top with a sandpaper texture. Actinic keratosis is more common in fair skinned individuals along with patients that are on chronic immunosuppressive therapies. It also increases in incidence with increasing age. The risk of malignant transition into squamous cell carcinoma is around 5% (Aasi and Leffell 2003). It can be treated with curettage, cryotherapy, topical tretinoin, 5-fluorouracil, laser, dermabrasion, or excision.
 
Lentigomaligna
Also termed a Hutchinson's freckle, lentigomaligna is a melanoma-in-situ lesion. It has a rate of malignant transformation into an invasive melanoma of around 5%. It appears as a brown to black macular spot with somewhat irregular boundaries that typically slowly enlarges over time. Although cryosurgery, radiotherapy, curettage, laser surgery, and various topical treatments have been tried, lentigomaligna has a significant recurrence rate. Surgical excision is the treatment of choice and may be assisted by Moh's technique for lesions in anatomically important areas.
 
Keratoacanthoma
Keratoacanthoma is considered a low-grade skin malignancy that appears as a volcano-like bump in the affected area of the pinna. It appears quickly and grows to its maximum size in one to two months. It is due to excessive sun exposure and typically affects males with fair complexions in the middle aged to elderly. The incidence of local or regional metastasis is quite low, but can occur. Treatment is most often excision of the lesion, but cryotherapy and radiation can also be effective.
 
Basal Cell Carcinoma
This is the least aggressive skin carcinoma (Fig. 1.17A). It arises from the basal cell layer, the deepest cell layer found within the epidermis. Eighty-five percent of basal cell carcinomas, the most common skin cancer, are found in the head and neck area. Risk factors for developing basal cell carcinoma include excessive sunlight exposure, typically with a history of sunburns in the past. Radiation therapy has also been associated with basal cell carcinoma formation. Other risk factors include arsenic exposure and immunosuppression.
The condition xeroderma pigmentosum is an autosomal recessive genetic disease leading to the inability of DNA repair after ultraviolet damage. Multiple cutaneous malignancies including basal cell carcinomas occur along with corneal opacification and neurologic deficits. Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder that causes multiple basal cell carcinomas and odontogenic keratocyst formation along with palmar pitting, intracranial calcifications, and rib abnormalities.
Basal cell carcinoma has an extremely low incidence of metastasis, below 0.1%, and can be managed with much smaller surgical margins than squamous cell carcinomas and melanomas, both of which require more aggressive surgical resection. For basal cell carcinoma, the surgical margin around the lesion need only be 2−4 mm in size. Although basal cell carcinomas are usually treated with surgical resection, they are quite sensitive to radiation therapy as well. Basal cell carcinomas are characterized by a pearly appearance to the skin lesion with telangiectasias and rolled borders (Fig. 1.17B). Subtypes based on histology include superficial, nodular, pigmented, infiltrative, morpheaform (sclerosing), and basal squamous.
18
zoom view
Figure 1.17A: Basal cell carcinoma of the auricle (BCCA): A basal cell carcinoma is shown involving the central portion of the auricle.
The infiltrative, morpheaform, and basal squamous subtypes are considered significantly more aggressive than the superficial, nodular, and pigmented subtypes. The more aggressive variant of basal cell carcinoma, basal squamous cell carcinoma, should be treated and considered as a subtype of squamous cell carcinoma due to its aggressive clinical features. Moh's micrographic mapping is a common way of obtaining negative margins by minimizing resection to the surrounding delicate structures.
The staging for basal cell carcinomas is the same system used for cutaneous squamous cell carcinomas and is presented in Table 1.3.
 
Squamous Cell Carcinoma
Squamous cell carcinoma is a cutaneous malignancy with the capacity for local destruction along with metastatic spread (Fig. 1.18A). Risk factors for squamous cell carcinoma include sun exposure, fair skin, preexisting lesions, previous radiation, immunosuppressions, scars, and burns. Squamous cell carcinoma appears as an erythematous indurated patch of skin with indistinct borders. The lesion often has ulcers or excoriated areas and is prone to bleeding (Fig. 1.18B). It enlarges over time. Squamous cell carcinomas tend to spread initially into the local lymphatic drainage system and present with enlarged lymph nodes. For auricular lesions, the first echelon drainage sites are the preauricular lymph nodes within the parotid gland and the postauricular lymph nodes. The lymph nodes draining the auricle include cervical lymph nodes levels II−V. Beyond local regional spread to the lymphatic system, squamous cell carcinoma tends to metastasize to the lung, liver, or bones. The staging system for squamous cell carcinoma is presented in Table 1.3.
zoom view
Figure 1.17B: Pathology slide of BCCA: This slide shows island of basaloid tumor cells with peripheral palasading of nuclei, characteristic of basal cell carcinoma.
Squamous cell carcinoma, somewhat surprisingly, is the most common malignancy of the auricle accounting for 55% of all malignancies in this site, a greater frequency than basal cell carcinoma (Pless 1976; Bumsted and Ceilley 1981). The most common sites of involvement on the auricle are the helix followed by the posterior pinna, the antihelix, and triangular fossa (Freedlander and Chung 1983; Shockley and Stucker 1987).
19
Table 1.3   Staging system for cutaneous squamous cell carcinoma
Primary site:
T0
No evidence of primary tumor
T1
Less than 2 cm (with less than 2 high risk factors)
T2
Greater than 2 cm (or any size with 2 or more high risk factors)
T3
Invasion into maxilla, mandible, orbit, or temporal bone
T4
Invasion of the skeleton (appendicular or axial) or perineural involvement of skull base
High-risk factors include depth of invasion greater than 2 mm, perineural invasion, anatomic locations of either the ear or nonhair-bearing lip, and either poorly differentiated or undifferentiated histology.
Nodal status:
N0
None
N1
Single ipsi node 3 cm or less
N2a
Single ipsi node between 3−6 cm
N2b
Multiple ipsi nodes < 6 cm
N2c
Bilateral or contralateral nodes < 6 cm
N3
Any node greater than 6 cm
Metastasis
M0
None
M1
Distant metastasis
Staging
0
Tis
N0
M0
I
T1
N0
M0
II
T2
N0
M0
III
T3
N0
M0
T1
N1
M0
T2
N1
M0
T3
N1
M0
IV
T4
N any
M0
T any
N2
M0
T any
N3
M0
T any
N any
M1
20
zoom view
Figure 1.18A: Squamous cell carcinoma of the auricle (SCCA): Shows an extensive squamous cell carcinoma involving the auricle and occluding the external auditory canal.
Some investigators believe that squamous cell carcinomas of the auricle have a higher incidence of recurrence and metastasis than squamous cell carcinomas in other sites of the body (Blake and Wilson 1974; Shiffman 1975; Bailin and Levine 1980; Bumsted and Ceilley 1981; Yoon et al. 1992). Others feel this notion arises from inadequate treatments in this area. Yoon in 1992 presented metastatic data from 10 studies with a combined 1,546 patients and found 173 had metastasis, or 11%. The range was 6−27% for each study. This compared with a review of 3 studies totaling 7,788 cutaneous squamous cell carcinomas of the skin from other sites and found 152 had metastasis, or 2% (range 2−6%) (Yoon et al. 1992).
zoom view
Figure 1.18B: Pathology slide of SCCA: This slide shows invasive keratinizing squamous carcinoma arising from overlying squamous epithelium. Keratin pearls are readily seen within the tumor.
Several theories have been put forth to explain the increased risk of recurrence and metastasis. One theory is that the close approximation to the underlying cartilage and the numerous complex folds and turns within the auricle makes resection more difficult. The depth of invasion of the carcinoma does portend to higher rates of metastasis as do incomplete resections (Clark and Soutar 2008). Another theory suggests that the increased incidence of metastasis is due to the close proximity and high likelihood of involvement of the external auditory canal.
The treatment of squamous cell carcinoma of the auricle is wide local excision of the lesion with at least 1 cm margins circumferentially. This excision includes a 1 cm deep or medial margin. In some cases, this necessitates a total auriculectomy. Depending on the extent of the carcinoma, postoperative radiation therapy may be indicated. Careful surveillance is indicated not only to monitor the resected lesion but also to detect new carcinomas that may form within other sun exposed areas.21
 
Melanoma
Malignant melanoma is a cutaneous carcinoma of the melanin producing melanocyte cells. Excess sun exposure predisposes individuals to develop melanoma, but melanoma can also occur from a preexisting nevus or lentigomaligna lesion. Roughly 20−30% of melanomas are located within the head and neck and of these 10−15% are found on the auricle (Pack et al. 1970; Byers et al. 1980; Gussack et al. 1983; Urist et al. 1984; O'Brien et al. 1995). Melanoma tends to present in elderly males with a left-sided predominance due to sun exposure while driving. The most common location for melanoma to arise on the auricle is the helix, accounting for roughly half of all auricular lesions, with the lobule being the second most common area (Byers et al. 1980; Bono et al. 1997; Narayan and Ariyan 2001; Cole et al. 2003).
Melanoma can be classified histologically into four distinct subtypes: superficial spreading, nodular, lentigo maligna, and acrallentiginous. The most common histologic subtype involving the auricle is the superficial spreading subtype, with the other subtypes being less common in this area (Jahn et al. 2006; Ravin et al. 2006).
Although somewhat controversial, many investigators consider 1 cm surgical margins a minimum for melanoma of the external ear. Some advocate margins of 1 cm for lesser than 1 mm depth of invasion and 2 cm for greater than 1 mm depth of invasion (Jahn et al. 2006). Others have advocated up to 4 cm margins, but have not proven statistical benefits to the additional margin. Several investigators advocate a total auriculectomy with parotidectomy and neck dissection for all cases of auricular melanoma (Pack et al. 1970). Most recent evidence supports standard 2 cm margins as used in excising melanoma in other parts of the body (Cole et al. 1992; Bono et al. 1997; Narayan and Ariyan 2001) (Fig. 1.19).
The incidence of nodal metastasis ranges from 12% to 36% depending on the study and the level of invasion (Jahn et al. 2006; Ravin et al. 2006; Shpitzer et al. 2007). The lymphatic drainage of the auricle is known to be highly variable. Consequently, many investigators have advocated for sentinel node sampling. Nodal drainage sites for auricular melanoma are most often into the neck and the tail of the parotid (Cole et al. 2003). Elective neck dissection remains controversial. A study of 82 patients showed elective neck dissection on patients reduced the incidence of recurrence for head and neck melanoma (Fisher 1989). However, other investigators could not show any benefit to elective neck dissection (Veronesi et al. 1977; Balch and Buzaid 1996; Cascinelli et al. 1998). Sentinel node biopsy remains controversial with numbers too small to quantify benefit.
zoom view
Figure 1.19: Melanoma: In this slide, malignant melanocytes with intracytoplasmic light brown pigment and atypical enlarged nuclei are seen on the left of the slide. On the right of the slide, heavily pigmented coarse dark brown melanophages are identifiable.
22
In a recent review of auricular melanoma, patients had a five-year survival rate of 65% with systemic metastasis developing in 22% of patients. Melanoma thickness, local recurrence, and lymph node spread all adversely affected outcome. Ear melanoma has a worse prognosis when compared to melanoma in other parts of the body (Ravin et al. 2006). The depth of the melanoma invasion is a critical issue and determines much of the stage of the disease. The Breslow thickness represents the level of the deepest penetration of the melanoma. The tumor's vertical height is measured from the most superficial point of the granular layer to the area of deepest penetration. The Breslow and Clark staging systems, as well as the current tetranitromethane (TNM) staging system, are presented in Tables 1.4 and 1.5.
The Clark levels were also developed to portend prognostic information. The area of deepest invasion is again used for staging purposes. The current TNM staging system is considered more accurate than the Clark levels. The Clark levels are listed below.
 
Rhabdomyosarcoma
Rhabdomyosarcoma is certainly a disease of the pediatric population. Although rhabdomyosarcoma involving the middle ear and temporal bone account for 7% of all pediatric rhabdomyosarcomas of the head and neck, rhabdomyosarcoma of the auricle is an extremely rare entity (Raney et al. 1983; Burrows et al. 1994; Ragsdale et al. 2009). Cutaneous rhabdomyosarcoma accounts for only 0.7% of all cases of rhabdomyosarcoma (Leuschner et al. 1993). Auricular rhabdomyosarcoma usually presents as a polypoid mass that bleeds intermittently, particularly with trauma to the area. Rhabdomyosarcoma is discussed more thoroughly in Chapter 4.
 
Merkel Cell Carcinoma
Merkel cells are proprioceptors within the skin. These cells are considered neuroendocrine cells. Merkel cell carcinoma, also called neuroendocrine carcinoma of the skin, is a malignancy comprised of these cells. Merkel cell carcinoma occurs in roughly 3 per 1,000,000 individuals and is an aggressive malignancy that occurs most often in the elderly, usually over the age of 65.
Table 1.4   Breslow and Clark staging systems for melanoma
Breslow Grading System
Clark Levels
Level
Depth
5 yr survival
I
< 1 mm
95−100%
I
Epidermis only
II
1–2 mm
80−95%
II
Papillary dermis
III
2.1− 4mm
60−75%
III
Junction of papillary and reticular dermis
IV
> 4 mm
50%
IV
Into reticular dermis
V
Into subcutaneous fat
23
Table 1.5   TNM staging for melanoma
T staging
Tis
in situ confined to epidermis
T1a
1.0 mm or less (T1b if ulcerated or mitotic rate 1/mm 2 or more)
T2a
1.01 to 2.0 mm (T2b if ulcerated or mitotic rate 1/mm2 or more)
T3a
2.01 to 4.0 mm (T3b if ulcerated or mitotic rate 1/mm2 or more)
T4a
>4.0 mm (T4b if ulcerated or mitotic rate 1/mm2 or more)
N x—nodes not assessed
N0—no regional nodes
N1—a single regional node
N2—spread to 2 or 3 nodes
N3—spread to 4 or more nodes
a designation is microscopic disease in node only
b designation is visible on imaging or palpable on exam
c designation denotes small satellite lesions within skin and lymphatic channels around tumor
M0 no metastasis
M1a metastasis to skin, subcutaneous tissue, distant lymph nodes with normal blood HDL level
M1b metastasis to lung with normal blood HDL level
M1c metastasis to other organs or distant spread to any site with elevated HDL level
Stage
Stage
0
Tis, No, Mo
IIIa
T1a to 4a, N1a or N2a, Mo
Ia
T1aNoMo
IIIb
T1b to 4b, N1a or N2a, Mo
Ib
T1b or T2a, NoMo
T1a to 4a, N1b, N2b, N2c, Mo
IIa
T2b or T3a, NoMo
IIIc
T1b to 4b, N1b, N2b, N2c, Mo
IIb
T3b or T4a, NoMo
Any T, N3, Mo
IIc
T4bNoMo
IV
Any T, any N, M1 (a, b, or c)
Men are affected 2.5 times as often as women. The incidence is increased in fair skinned individuals with a history of sun exposure and in immunosuppressed individuals. Estimates approximate that half of all cases of Merkel cell carcinoma occur within the head and neck area, but the auricle is generally considered an unusual site of occurrence.24
The gross appearance of the tumor is reddish blue or pink, nodular, subcutaneous, lesions which rarely ulcerate. The tumor may also have telangiectasias on the surface (Karapantzos et al. 2003). Merkel cell carcinoma histologically appears as a small, blue, round cell tumor (Fig. 1.20) that stains positive for neuron specific enolase, cytokeratin (CK), and chromograninon immunostaining, an important distinguishing factor from other cutaneous malignancies (Akhtar et al. 2000) (Fig. 1.21).
Merkel cell carcinoma has a propensity for local recurrence (30−50%) and lymph node spread (50−80%) (Akhtar et al. 2000). It also has a relatively high rate of metastatic spread, estimated at 20−40% (Hitchcock et al. 1988). The prognosis is generally worse for younger age patients and men. The three-year survival has been estimated at 55% (Shaw and Rumball 1991). Complete surgical excision is the most appropriate treatment of Merkel cell carcinoma. This includes wide surgical margins, up to 3 cm. Due to the high rate of local lymph node spread at 50% on elective neck dissections, treatment of the regional lymphatic drainage with surgical excision is critical. Postoperative radiation therapy is usually indicated for this aggressive malignancy. Merkel cell carcinoma is believed to be radiosensitive as well as chemosensitive. Chemotherapeutic agents used for metastasis and palliation include cyclophosphamide and adriamycin as well as vincristine, dacarbazine, cisplatinum, octreotide, and etoposide (Karapantzos et al. 2003). A staging system for Merkel cell carcinoma has been developed and is presented in Table 1.6.
zoom view
Figure 1.20: Merkel cell carcinoma: Sheets of closely packed tumor cells with high nuclear cytoplasmic ratios and numerous mitoses are seen characteristic of Merkel cell carcinoma.
zoom view
Figure 1.21: Merkel cell carcinoma CK20 immunostaining: Perinuclear dot-like staining of neurofilaments by cytokeratin 20 is helpful in confirming the diagnosis of Merkel cell carcinoma and demonstrated here.
25
Table 1.6   Merkel cell carcinoma staging system
T0
no evidence primary tumor
Tis
in situ
T1
< 2 cm
T2
2−5 cm
T3
> 5 cm
T4
invades bone, muscle fascia, or cartilage
N0
no adenopathy
N1a
micrometastasis
N1b
macrometastasis
N2
in transit metastasis
M0
no metastasis
M1a
metastasis to skin, subcutaneous tissues, distant lymph nodes
M1b
metastasis to lung
M1c
metastasis to visceral sites
Staging
0
Tis
N0
M0
I
T1
N0
M0
II
T2-4
N0
M0
III
any T
N1 or N2
M0
IV
any T
any N
M1
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