Chapter-065 Pheochromocytoma—Molecular Genetics, Diagnosis, and Treatment Strategies

BOOK TITLE: ESI Manual of Clinical Endocrinology

1. Rajaratnam Simon
2. Pai Rekha
Publishing Year
Author Affiliations
1. Christian Medical College, Ida Scudder Road, Vellore 632 004, Tamil Nadu, India, Christian Medical College, Vellore, Tamil Nadu, India, National Diabetes and Endocrine Centre, Royal Hospital, Muscat, Oman
2. Christian Medical College, Vellore, Tamil Nadu, India; Christian Medical College, Vellore, Tamil Nadu, India
Chapter keywords
Chromaffin cells, positron emission tomography scan, calcium channel blockers, metyrosine, prazosin, terazosin, systemic chemotherapy, radiation therapy


Pheochromocytomas (PHEOs) are catecholamine-secreting tumors occur from chromaffin cells of the adrenal medulla. It is also called a paragangliomas (PGLs). The three hereditary syndromes associated with adrenal PHEOs namely, Neurofibromatosis 1 (NF1), Multiple endocrine neoplasia type 2 (MEN2) and Von Hippel–Lindau (VHL) syndrome. Biochemical tests, CT scan, MRI, functional imaging (positron emission tomography scan (PET) are used for diagnostic the disease. Mutations in the RET, VHL gene and succinate dehydrogenase family (SDHB, SDHC, and SDHD) also give rise to PGLs and PHEOs. Alpha-adrenergic blockers, calcium channel blockers, metyrosine and some drugs such as prazosin, terazosin, and doxazosin are useful for the treatment purposes. Pheochromocytoma crisis causes hemodynamic instability leading to end-organ damage and dysfunction. The laparoscopic surgical technique is used to treat this type of tumor. Malignant tumor occurs more frequently in patients with metastatic PHEO. There is no curative treatment for malignant tumors but the mainstay of treatment includes surgical debulking, 131I- MIBG therapy, systemic chemotherapy and radiation therapy.

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