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Presented by Justin Bishop, MD and prepared by Sarah Karram, MD
This case talks about a 70 year old man with hoarseness and a laryngeal mass.
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Question 1 of 1
1. Question
Week (625): Case 3
70 year old man with hoarseness and a laryngeal mass.
Correct
Answer: Large cell neuroendocrine carcinoma
The tumor grows as basaloid nests and trabeculae with peripheral palisading of tumor nuclei. There are numerous mitotic figures as well as necrosis. Focal rosette formation is seen. The tumor cells are large with abundant cytoplasm and tumor nuclei with open chromatin and prominent nucleoli. The tumor was positive for synapotphysin, cytokeratin, and CD56 and negative for CK5/6 and p63.
Laryngeal neuroendocrine carcinomas (NECs) are graded well-differentiated (i.e., carcinoid tumors), moderately differentiated (i.e., atypical carcinoid tumors), and poorly-differentiated. In the past, high-grade NECs that were not small cell carcinoma were classified as moderately differentiated, but now the poorly differentiated NECs are now subdivided into small cell carcinoma and large cell neuroendocrine carcinoma (LCNEC).
Laryngeal LCNECs tend to arise in older men (mean, 60 years). More than 90% of affected patients are cigarette smokers. LCNEC can arise virtually anywhere within the head and neck, but there is a predilection for the supraglottic larynx. Patients present with hoarseness and/or dysphagia. Many patients have regional or distant metastases at presentation.
LCNEC has a “neuroendocrine” architecture with organoid nesting, palisading, rosettes, and/or trabeculae; it is composed of medium-to-large sized cells with abundant cytoplasm. The nuclei of LCNEC have coarse chromatin, sometimes with a speckled, “salt and pepper” quality, and usually a single prominent nucleolus. LCNEC exhibits necrosis and >10 mitotic figures per 2 mm2 or 10 high-power fields. Rare examples of LCNEC harbor a component of squamous cell carcinoma either within the invasive tumor or within the overlying mucosa (i.e., squamous cell carcinoma-in-situ).
By immunohistochemistry, LCNEC is positive for cytokeratins. The neuroendocrine differentiation of these tumors is confirmed by staining with at least one neuroendocrine marker (i.e., synaptophysin, chromogranin, CD56). TTF-1 immunoexpression is variable. LCNEC is weakly positive or negative for p63, and are consistently negative for CK5/6. Ki67 immunolabeling is typically high (typically >50%) but is not used in the grading of neuroendocrine carcinomas.
Poorly differentiated NEC of the larynx (both small cell carcinoma and LCNEC) are aggressive malignancies with high rates of regional and distant metastasis. Almost 70% of patients present with advanced disease, and 5-year-survivals range from 5-20%. Patients are typically treated with a combined chemotherapy and external beam radiation.
Incorrect
Answer: Large cell neuroendocrine carcinoma
The tumor grows as basaloid nests and trabeculae with peripheral palisading of tumor nuclei. There are numerous mitotic figures as well as necrosis. Focal rosette formation is seen. The tumor cells are large with abundant cytoplasm and tumor nuclei with open chromatin and prominent nucleoli. The tumor was positive for synapotphysin, cytokeratin, and CD56 and negative for CK5/6 and p63.
Laryngeal neuroendocrine carcinomas (NECs) are graded well-differentiated (i.e., carcinoid tumors), moderately differentiated (i.e., atypical carcinoid tumors), and poorly-differentiated. In the past, high-grade NECs that were not small cell carcinoma were classified as moderately differentiated, but now the poorly differentiated NECs are now subdivided into small cell carcinoma and large cell neuroendocrine carcinoma (LCNEC).
Laryngeal LCNECs tend to arise in older men (mean, 60 years). More than 90% of affected patients are cigarette smokers. LCNEC can arise virtually anywhere within the head and neck, but there is a predilection for the supraglottic larynx. Patients present with hoarseness and/or dysphagia. Many patients have regional or distant metastases at presentation.
LCNEC has a “neuroendocrine” architecture with organoid nesting, palisading, rosettes, and/or trabeculae; it is composed of medium-to-large sized cells with abundant cytoplasm. The nuclei of LCNEC have coarse chromatin, sometimes with a speckled, “salt and pepper” quality, and usually a single prominent nucleolus. LCNEC exhibits necrosis and >10 mitotic figures per 2 mm2 or 10 high-power fields. Rare examples of LCNEC harbor a component of squamous cell carcinoma either within the invasive tumor or within the overlying mucosa (i.e., squamous cell carcinoma-in-situ).
By immunohistochemistry, LCNEC is positive for cytokeratins. The neuroendocrine differentiation of these tumors is confirmed by staining with at least one neuroendocrine marker (i.e., synaptophysin, chromogranin, CD56). TTF-1 immunoexpression is variable. LCNEC is weakly positive or negative for p63, and are consistently negative for CK5/6. Ki67 immunolabeling is typically high (typically >50%) but is not used in the grading of neuroendocrine carcinomas.
Poorly differentiated NEC of the larynx (both small cell carcinoma and LCNEC) are aggressive malignancies with high rates of regional and distant metastasis. Almost 70% of patients present with advanced disease, and 5-year-survivals range from 5-20%. Patients are typically treated with a combined chemotherapy and external beam radiation.
