Persistent hyperparathyroidism (HPT) after successful kidney transplantation (RTx) (tertiary HPT; THPT) is a common complication in patients with RTx and may affect bone disease, deterioration of graft function and cardiovascular events. Parathyroidectomy (PTx) is the most successful treatment for resolving advanced HPT in patients with THPT. However, the surgical indications for THPT and timing of the operation are problematic because hypercalcemia can be resolved spontaneously. Subtotal and total PTx with autotransplantaion are widely accepted for THPT. The evidence to know which procedure is more appropriated could not be found. Recently the deterioration of kidney graft function after PTx for THPT has been reported and hypoparathyroidism after PTx may be avoided. Recently cinacalcet has been applied for patients with THPT and the medicine can dramaticaly control HPT and hypercalcemia. Possible risks of cinacalcet are hypocalcemia and increased calciuria and the approval for THPT remains highly controversial. A large number of prospective controlled clinical trial should be required.

The anesthetic management of pheochromocytoma is complicated and challenging. However, preoperative pharmacologic preparation has greatly improved perioperative outcome, and modern anesthetic drugs combined with advanced monitoring contribute to intraoperative stability. There is great regional and international variation in which intraoperative anesthetic technique is employed and there are little data to suggest one approach over another. Nevertheless, most management principles are universal. Progress lies in improved data collection that is increasingly available with electronic anesthesia information systems.

A systematic review of the existing literature was carried out, identifying studies through a search of the Medline literature via PubMed and through scanning of references of these papers. These papers were then subject to an assessment of quality of reporting using a scoring system modified from previously published criteria.

50 studies published between 1997 and 2010 were included. These studies included 916 polymorphisms across 62 genes involved in a diverse range of cellular processes using 10,704 cases and 27,707 controls. The median quality score was 70.4% (range 52-93%). Areas of strength and weaknesses in study reporting were identified.

Research on the genetic susceptibility to nonmedullary thyroid cancer is sparse. Published studies are of suboptimal quality, have analyzed few variants and positive findings have not been replicated. Adherence to recently published guidelines on methodology should be encouraged. A consortium led approach involving multiple centers, including large numbers of patients in well-defined study protocols is required to investigate this subject comprehensively.

A 32-year-old male patient, a known case of chronic myeloid leukemia (CML) with 11 × 9 cm GIANT functioning insulinoma in the uncinate region of pancreas, with no local invasion or metastatic disease, is presented. In view of CML, enucleation of the tumor done. Histopathology was s/o ‘insulinoma of uncertain biological behavior’. Postoperatively, the patient was followed up for one year and found normal.

Severe hypercalcemia due to primary hyperparathyroidism (PHPT) is rare in the setting of MEN 2A.

Two patients with MEN 2A and severe hypercalcemia were identified recently. Their clinical presentation, evaluation, surgical management and outcomes are reviewed.

Two patients with MEN 2A were identified with severe hypercalcemia secondary to a parathyroid adenoma. Calcium levels were elevated to 12.7 mg/dL and 15.1 mg/dL, respectively (normal range = 8.9-10.1 mg/dL). In each case, a single parathyroid adenoma was identified and surgically excised with normalization of parathyroid and calcium levels postoperatively. Clinical manifestations at the time of diagnosis included constipation, polyuria, hypercalciuria, and decreased bone mineral density.

Severe elevation of serum calcium is a rare presentation of PHPT in MEN2A. The differential diagnosis should include parathyroid adenoma, hyperplasia and parathyroid carcinoma. Early surgical management is essential in the treatment of hyperparathyroidism with severe hypercalcemia to prevent further complications.

Up to 25% of pheochromocytomas and paragangliomas (PGL) are associated with germline mutations in RET, VHL, NF1, and subunits A, B, C, or D of succinate dehydrogenase (SDH). SDHB mutations are associated with malignant extra-adrenal PGL. Codon specific genotype-phenotype relationships have not been identified. Herein is described a case of malignant bladder paraganglioma from a novel W200R SDHB mutation. The literature is reviewed and clinical management is discussed.

Literature review was performed to analyze the relationship between SDHB and PGL, yielding 45 unique articles, which were reviewed and cross-referenced.

SDHB mutations have a 50% penetrance by age 35, 1/3 of paragangliomas are multifocal, 31 to 48% are malignant, and 50 to 70% of these malignant PGL develop metastases.

Based on the aggressive nature of the SDHB mutation, we recommend preoperative staging, an aggressive treatment regimen, and intensive screening for recurrence.

Pheochromocytoma is a tumor arising from adrenal medulla in > 90% of cases. It is a great mimic, featuring in differential diagnosis of disorders of multiple organ systems. Here we present a prototypical case of right adrenal pheochromocytoma, highlighting this unique feature of the tumor. It presented with repeated hypertensive crises with predominant involvement of one major organ system in each episode. All of them were reversible with timely intervention and successful removal of the tumor, highlighting the functional basis rather than organic pathology.

In this article, we review the management of giant adrenal myelolipoma and review the literature in regard to incidence and management. Patient presented with abdominal fullness and discomfort with CT revealing a large, well-circumscribed right-sided suprarenal mass. A thoracoabdominal incision allowed exploration and removal of the mass together with the right adrenal gland. Pathology revealed the mass to be a giant adrenal myelolipoma, a benign but rare tumor.

Hemiagenesis is the least frequent thyroid congenital anomaly. We report the case of a primary hypothyroidism due to thyroid hemiagenesis in a patient for whom, later on, a papillary carcinoma of the contralateral lobe was diagnosed. A child with known left lobe thyroid agenesis was found to have a 12 mm nodule in the right thyroid lobe during routine follow-up. Needle biopsy demonstrated the nodule to represent a papillary thyroid cancer and total thyroidectomy and lymphadenectomy of the central compartment was undertaken. Such event represents an extremely rare occurrence since there are only five other reports in literature that deal with a papillary carcinoma in a patient with thyroid hemiagenesis.