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Presented by Justin Bishop, MD and prepared by Sarah Karram, MD
This case talks about a 60 year old man with hoarseness and a laryngeal mass.
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Question 1 of 1
1. Question
Week (625): Case 2
60 year old man with hoarseness and a laryngeal mass.
Correct
Answer: Moderately-differentiated neuroendocrine carcinoma
The tumor consists of a polypod proliferation of cells growing as nests and sheets in the laryngeal submucosa. The tumor cells have abundant amphophilic cytoplasm and a vaguely plasmacytoid appearance. The tumor cells demonstrate nuclear pleomorphism, chromatin that varies from stippled to vesicular in quality, and variably prominent nucleoli. A few mitotic figures are identified, as are foci of single-cell necrosis. By immunohistochemistry the cells are positive for cytokeratin, synaptophysin, and calcitonin. There was no history of a thyroid tumor.
Moderately differentiated neuroendocrine carcinoma (NEC) (also known as atypical carcnioid tumor) is an epithelial neoplasm demonstrating neuroendocrine differentiation and histologic and prognostic features intermediate between well-differentiated and poorly-differentiated NEC. It is the most common NEC of the larynx, much more common than the rare well-differentiated NEC (carcnioid tumor). They occur most frequently in men in their 6th and 7th decades (mean, 63 years). Most affected patients are heavy cigarette smokers. More than 90% occur in the supgraglottic region. Patients present with hoarseness, dysphagia, sore throat, and occasionally hemoptysis. Patients rarely have a paraneoplastic syndrome due to aberrant hormone production by the tumor.
The histopathology of moderately differentiated neuroendocrine carcinoma is similar to that of well differentiated neuroendocrine carcinoma, with tumor cells growing as nests, cords, sheets, and trabeculae of round to slightly spindled cells with abundant amphophilic to eosinophilic granular cytoplasm. Gland-like structures or rosettes may be seen. The tumor nuclei may exhibit stippled, evenly dispersed chromatin or may show more nuclear atypia with prominent nucleoli. The defining features are necrosis and/or a mitotic rate of 2-10 mitoses per 2 mm2 of 10 high-power fields. Some examples demonstrate oncocytic cellular change or stromal amyloid deposition.
Moderately-differentiated NEC is positivite for cytokeratins and at least one neuroendocrine marker (i.e., synaptophysin, chromogranin, CD56). Ki-67 immunostaining is not used in the grading of ENC of the head and neck. These tumors are often positive for calcitonin, a potential pitfall particularly in a lymph node metastasis where it can be mistaken for medullary thyroid carcinoma. (serum calcitonin is the best way to distinguish these tumors – it is elevated in medullary carcinoma but not in laryngeal NEC).
Moderately-differentiated NEC is much more aggressive than its well-differentiated counterpart, with nearly 30% of patients presenting with advanced disease, a recurrence rate of approximately 60% and a 5-year survival of approximately 50%. Patients have been treated with surgery with adjuvant radiotherapy in some cases. Older studies reporting even more aggressive behavior included tumors now recognized as poorly differentiated NEC (especially large cell neuroendocrine carcinoma).
Incorrect
Answer: Moderately-differentiated neuroendocrine carcinoma
The tumor consists of a polypod proliferation of cells growing as nests and sheets in the laryngeal submucosa. The tumor cells have abundant amphophilic cytoplasm and a vaguely plasmacytoid appearance. The tumor cells demonstrate nuclear pleomorphism, chromatin that varies from stippled to vesicular in quality, and variably prominent nucleoli. A few mitotic figures are identified, as are foci of single-cell necrosis. By immunohistochemistry the cells are positive for cytokeratin, synaptophysin, and calcitonin. There was no history of a thyroid tumor.
Moderately differentiated neuroendocrine carcinoma (NEC) (also known as atypical carcnioid tumor) is an epithelial neoplasm demonstrating neuroendocrine differentiation and histologic and prognostic features intermediate between well-differentiated and poorly-differentiated NEC. It is the most common NEC of the larynx, much more common than the rare well-differentiated NEC (carcnioid tumor). They occur most frequently in men in their 6th and 7th decades (mean, 63 years). Most affected patients are heavy cigarette smokers. More than 90% occur in the supgraglottic region. Patients present with hoarseness, dysphagia, sore throat, and occasionally hemoptysis. Patients rarely have a paraneoplastic syndrome due to aberrant hormone production by the tumor.
The histopathology of moderately differentiated neuroendocrine carcinoma is similar to that of well differentiated neuroendocrine carcinoma, with tumor cells growing as nests, cords, sheets, and trabeculae of round to slightly spindled cells with abundant amphophilic to eosinophilic granular cytoplasm. Gland-like structures or rosettes may be seen. The tumor nuclei may exhibit stippled, evenly dispersed chromatin or may show more nuclear atypia with prominent nucleoli. The defining features are necrosis and/or a mitotic rate of 2-10 mitoses per 2 mm2 of 10 high-power fields. Some examples demonstrate oncocytic cellular change or stromal amyloid deposition.
Moderately-differentiated NEC is positivite for cytokeratins and at least one neuroendocrine marker (i.e., synaptophysin, chromogranin, CD56). Ki-67 immunostaining is not used in the grading of ENC of the head and neck. These tumors are often positive for calcitonin, a potential pitfall particularly in a lymph node metastasis where it can be mistaken for medullary thyroid carcinoma. (serum calcitonin is the best way to distinguish these tumors – it is elevated in medullary carcinoma but not in laryngeal NEC).
Moderately-differentiated NEC is much more aggressive than its well-differentiated counterpart, with nearly 30% of patients presenting with advanced disease, a recurrence rate of approximately 60% and a 5-year survival of approximately 50%. Patients have been treated with surgery with adjuvant radiotherapy in some cases. Older studies reporting even more aggressive behavior included tumors now recognized as poorly differentiated NEC (especially large cell neuroendocrine carcinoma).
