Paraganglioma

Eisenhofer G et al Current progress and future challenges in the biochemical diagnosis and treatment of pheochromocytomas and paragangliomas. Florm Metab Res 2008 40 329. [PMID 18491252]... 

Increases in plasma or urinary concentrations of L-dopa, dopamine, and methoxytyramine are not sensitive or specific markers of pheochromocytomas. However, when such increases accompany large increases in norepinephrine and normetanephrine, the pattern suggests metastatic disease. Pheochromocytomas that produce exclusively dopamine are extremely rare, but can be found in patients with paragangliomas, particularly where these have metastasized. 

McNicol AM. Differential diagnosis of pheochromo-cytomas and paragangliomas. Endocr Path 2001 12 407-15. 

Moran CA, Albores-Saavedra J, Wenig BM, et al. Pigmented extraadrenal paragangliomas a clinicopathologic and immunohistochemical study of five cases. Cancer. 1997 79 398-402. 

Tumors that may be confused with CUPS within the mediastinum include thymic neoplasm (thymoma, thymic carcinoid, or thymic carcinoma), thyroid tumors, lymphomas, paragangliomas, and germ cell tumors. Thymomas are generally easy to recognize owing to their characteristic admixture of neoplastic thymic epithelial cells with non-neoplastic lymphocytes. The neoplastic thymic epithelial cells are positive for keratins,... 

Rosai J. Cytokeratin expression in cauda equina paragangliomas. Author s response to letter. Am JSurgPathol. 1999 23 491. 

The differential diagnosis for neuroendocrine tumors in the sinonasal tract will include olfactory neuroblastoma, small cell neuroendocrine carcinoma, carcinoid, atypical carcinoid, paraganglioma, and possibly even sinonasal undifferentiated carcinoma. Once these more specific neuroendocrine entities have been excluded, there remains a small group of tumors that cannot be further classified. These should be referred to as neuroendocrine carcinoma, not otherwise specified. These tumors can be graded using standard morphologic features. 

The differential diagnosis might include olfactory neuroblastoma, paraganglioma, and carcinoid. 

Feature Pituitary Adenoma Olfactory Neuroblastoma Paraganglioma Carcinoid... 

TABLE 9.11 Immunohlstochemical Staining Pattern for Tumors in the Differential Diagnosis for Paragangliomas ... 

Stain Carcinoid Tumor Paraganglioma Medullary Melanoma Renal Cell Metastatic (Thyroid CA, Carcinoma, or Lung Carcinoma)... 

The classification of neuroendocrine tumors of the head and neck has been controversial.The WHO classification has divided the tumors into four categories typical carcinoid, atypical carcinoid, small cell neuroendocrine carcinoma, and paraganglioma. Each of these tumor types is discussed separately. Immunohisto-chemical stains will not easily separate the four types of... 

The differential diagnosis of carcinoid and atypical carcinoid tumors of the larynx includes paraganglioma, melanoma, and medullary thyroid carcinoma. Paragangliomas are rarely always negative for cyto-keratins and also exhibit the characteristic susten-tacular cell pattern with SlOO protein. Thyroid transcription factor-1 (TTEl) is positive in medullary thyroid carcinoma, as are calcitonin and CEA. Melanomas will typically stain with HMB45 and tyrosinase, both of which are negative in neuroendocrine carcinomas. 

Paragangliomas of the head and neck can occur in many locations. The most common is the neck, usually related to the carotid or vagal bodies, or the middle ear (jugu-lotympanic paragangliomaParagangliomas can rarely occur in other locations as well such as the larynx, nasal cavity and paranasal sinuses, and oral cavity. 197,277 symptoms of these tumors depend on the anatomic location, but patients may present with a pulsatile mass lesion. Paragangliomas in all of these locations have similar histologic and immunophenotypic appearance. 

FIGURE 9.35 S-100 in paraganglioma highlighting the sustentac-ular cells. (S-100, x200 original magnification.)... 

Paragangliomas located in the usual or unusual locations in the neck must be differentiated from other epithelial neuroendocrine tumors such as carcinoids, atypical carcinoids, small cell neuroendocrine carcinoma, and medullary thyroid carcinoma.The staining panel that will differentiate these tumors is... 

Paragangliomas of the head and neck can occur in rather specific locations. 

Erickson D, Kudva YG, Ebersold MJ, et al. Benign paragangliomas clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001 86(11) 5210-5216. 

Sennaroglu L, Sungur A. Histopathology of paragangliomas. Otol Neurotol. 2002 23(1) 104-105. 

Martinez-Madrigal F, Bosq J, Micheau C, et al. Paragangliomas of the head and neck. Immunohistochemical analysis of 16 cases in comparison with netrro-endocrine carcinomas. Pathol Res Pract. 1991 187(7) 814-823. 

Barnes EL, Taylor S. Carotid body paragangliomas a clinicopathologic and DNA analysis of 13 tumors. Arch Otolaryngol. 1991 116 447-453. 

Min KW. Diagnostic usefulness of sustentacular cells in paragangliomas immunocytochemical and ultrastructural investigation. Ultrastruct Pathol. 1998 22(5) 369-376. 

Kliewer KE, Wen DR, Cancilla PA. Paragangliomas assessment ofprognosisby histologic,immunohistochemical,andtrltrastmcttiral techniques. Hum Pathol. 1989 20 29-39. 

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