• Similar to neuroendocrine carcinomas at other sites
• Aggressive neoplasms capable of local recurrence and distant metastasis (Ann Pathol 2005;25:205)

• Atypical carcinoids are considered moderately differentiated neuroendocrine carcinomas
• Undifferentiated or poorly differentiated neuroendocrine carcinomas may be small cell carcinoma

• Rarely causes Cushing's syndrome (Indian J Pathol Microbiol 2010;53:148)
• Primary thymic neuroendocrine carcinomas need to be distinguished from ectopic parathyroid tumors and paragangliomas (Semin Diagn Pathol 2005;22:223)

• 47 year old man neuroendocrine carcinoma of posterior mediastinum (Arch Pathol Lab Med 1999;123:933)
• 63 year old woman with atypical carcinoid and neurofibromatosis type 1 (Arch Pathol Lab Med 2000;124:319)

• Larger cells with more cytoplasm than small cell carcinoma, organoid growth pattern, spindle cell foci
• May have prominent angiomatoid features (Hum Pathol 1999;30:635)

Nests of cells with uniform nuclei and finely granular chromatin

Nests, ribbons and festoons

Various stains

• Chromogranin A, cytokeratin, CEA, EMA, NSE, synaptophysin

• CD99

• Desmosomes, neurosecretory granules

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