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Paraganglioma

A paraganglioma is a rare neoplasm that can be found in the head and neck region and other less common areas. They are usually considered benign and complete surgical removal results in cure. However, in about 3% of cases they are malignant and have the ability to metastasize. Paragangliomas are still sometimes called glomus tumors (not to be confused with glomus tumors of the skin) and chemodectomas, but paraganglioma is the currently accepted and preferred term. more...

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Medicines

Paragangliomas arise from the glomus cells, which are special chemoreceptors located along blood vessels that have a role in regulating blood pressure and blood flow. The main concentration of glomus cells are found are in the carotid body, located in the upper neck at the branching of the common carotid artery, and the aortic bodies, located near the aortic arch. The glomus cells are a part of the paraganglion system composed of the extra-adrenal paraganglia of the autonomic nervous system, derived from the embryonic neural crest. Thus, paragangliomas are a type of neuroendocrine tumor, and are closely related to pheochromocytomas. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.

Paragangliomas are found predominantly in the abdomen (85%) and the thorax (12%), and only 3% are found in the head and neck region. Most occur as single tumors. When they occur in multiple sites they are usually found as a part of a heritable syndrome such as multiple endocrine neoplasia types II-A and II-B and Carney's complex.

According to the World Health Organization classification of neuroendocrine tumors, paragangliomas are classified as having a neural cell line of origin. In the categorization proposed by Wick, the paragangliomas belong to Group II.

Inheritance

Familial paragangliomas account for approx. 25% of cases, are often multiple and bilateral, and occur at an earlier age. Mutations of the genes SDHD (previously known as PGL1), PGL2, and SDHC (previously PGL3) have been identified as causing familial head and neck paragangliomas. Mutations of SDHB play an important role in familial adrenal pheochromocytoma and extra-adrenal paraganglioma (of abdomen and thorax), although there is considerable overlap in the types of tumors associated with SDHB and SDHD gene mutations.

Pathology

The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly vascular tumors and may have a deep red color.

On microscopic inspection, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen. These cell balls are separated by fibrovascular stroma and surrounded by sustenacular cells.

By light microscopy, the differential diagnosis includes related neuroendocrine tumors, such as carcinoid tumor, neuroendocrine carcinoma, and medullary carcinoma of the thyroid; middle ear adenoma; and meningioma.

With immunohistochemistry, the chief cells located in the cell balls are positive for chromogranin, synaptophysin, neuron specific enolase, serotonin and neurofilament; they are S-100 protein negative. The sustenacular cells are S-100 positive and focally positive for glial fibrillary acid protein. By histochemistry, the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.

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Paraganglioma: a case of pulmonary nodules
From CHEST, 10/1/05 by Jose A. Delgado

INTRODUCTION: We report a case of an asymptomatic male presenting with bilateral, disseminated and well-defined pulmonary nodules. The patient had undergone resection of a retroperitoneal paraganglioma 6 years earlier; close follow-up had revealed no evidence of recurrence. After our evaluation, a VATS with wegde resection of the lingula was performed and histologic diagnosis of metastatic paraganglioma was established.

CASE PRESENTATION: This is a 51-year-old African-American male seen in consultation for bilateral long nodules. Six years ago he was diagnosed with an abdominal mass during a routine clinical evaluation. Subsequent imaging studies confirmed the presence of a 10x10x10cm retroperitoneal tumor between me aorta and left ureter, and resection of a 700g mass was performed. The histologic examination revealed an extra-adrenal paraganglioma with evidence of capsular and extracapsular infiltration and angiolymphatic invasion. Immunohistochemical stains for chromogranin were positive. The patient underwent radiation therapy, and follow-up octrectide scan and CT abdomen revealed no evidence of tumor activity or recurrence. Hormonal assays were negative for elevated 24-h metanephrine and for serum catecholamines on two separate occasions. The patient had undergone a follow-up MRI of the abdomen 1 month before our evaluation; disseminated nodules were visualized in the liver and lung bases. His past medical history is also remarkable for chronic active hepatitis C, for which he had received treatment with interferon alfa-2b and ribavirin as well as pegylated interferon in the past 2 years; the patient has remained well compensated. He is a former smoker and has a remote history of IV drug use. At the time of our evaluation the patient was asymptomatic, and the review of systems was negative. He was normotensive, hemodynamically stable and there were no stigmata o chronic liver disease. His liver function studies and coagulation parameters were normal except for an elevated alfa-fetoprotein level of 20.8. The patient subsequently underwent VATS with wedge resection of the lingula, which confirmed the suspected diagnosis of metastatic paraganglioma. The immunohistochemical stains were positive for chromogranin and synaptophysin. As of this writing, no further intervention has been considered.

DISCUSSIONS: Extra-adrenal pheochromocytomas, or paragangliomas, are rare neuroendocrine tumors with infrequent pulmonary involvement. This case reveals one of the possible intrathoracic manifestations: metastatic pulmonary disease. Although most cases present with the classical paroxysmal attacks or symptoms related to catecholamine excess, our patient interestingly never exhibited any of those symptoms. We based the diagnosis on the MRI and CT scan findings, as well as on the histopathologic exam--with positive immunohistochemical markers such as synaptophysin and chromogranin. Paragangliomas are potentially malignant 40% of the time, with a mean survival of 1 to 2 years in metastatic disease. Surgical resection has been advocated in wen-localized tumors. The role of radiation and combination chemotherapy has been reported to be effective in disseminated disease; however, this role is still under investigation.

CONCLUSION: Paragangliomas are tumors with infrequent intrathoracic manifestations. The related classical symptoms may be absent, and the hormonal assays may be negative as well. In such cases, we recommend basing the diagnosis on MRI and CT imaging findings, as well as on histopathologic exaan with immunohistochemical markers. In addition we believe surgery should be considered in cases involving well-demarcated lesions.

REFERENCES:

(1) Sandur S, Dasgupta A et al. Thoracic involvement with pheochromocytoma. Chest 1999;115:511-521

(2) Edstrom E et al. The management of benign and malignant pheochromocytoma and abdominal paragangliomas.Eur J Surg Onc 2003;29:278-83

(3) Shibahara Jet al. Primary pulmonary paraganglioma. Am J Surg Pathol 2004;28:825-29

DISCLOSURE: Jose Delgado, None.

Jose A. Delgado MD * Ganesan Murali MD Albert Einstein Medical Center, Philadelphia, PA

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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