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Presented by Peter Illei, M.D. and prepared by Mark Samols, M.D., Ph.D.
Case 3: 57 y.o. male patient with a lung mass an enlarged supraclavicular lymph node that was biopsied.
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1. Question
Week 544: Case 3
57 y.o. male patient with a lung mass an enlarged supraclavicular lymph node that was biopsied.images/samols/0121133a.jpg
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images/samols/0121133e.jpgCorrect
Answer: Metastatic combined large cell neuroendocrine carcinoma
Histology:
Discussion: Sections show solid sheets and large nests of tumor cells with areas of necrosis. The cells have large, round to oval nuclei with fine chromatin and prominent nucleoli. The cytoplasm is eosinophilic with fine vacuoles. Focal acinar structures and intracytoplasmic vacuoles are present (best seen on mucin stain). Immunostains demonstrate strong positivity for CD56, and synaptophysin, as well as focal staining for chromogranin, TTF and CK7. The carcinoma does not express CK20, PANK, p63, CK5/6, and Napsin- A. A mucicarmine stain is focally positive. In addition, the tumor has a high Ki67 labeling index (~80%), and is negative for the prostate markers PSA, PSAP and NKX3. Mutation analyses were performed and show no alterations in the EGFR, ALK and K-ras genes.
In the 2004 WHO classification the category of combined large cell neuroendocrine carcinoma (LCNEC) is recognized for tumors that also have another non-small cell carcinoma component (like adenocarcinoma, squamous cell carcinoma, giant cell carcinoma or spindle carcinoma. Like small cell carcinoma LCNEC-s are histologically heterogeneous and may have a non-neuroendocrine component. In the 2004 WHO classification the term combined LCNEC was chosen arbitrarily to classify these tumors until “future studies better define their biologic behavior”. Combined LCNEC with small cell carcinoma also occurs but those are classified under combined small cell carcinoma. At the present, these tumors should also be subjected to molecular studies in order to provide optimal therapy for those tumors that may have an actionable genetic alteration.
Reference(s):
– Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart pp. 47-48, WHO 2004.Incorrect
Answer: Metastatic combined large cell neuroendocrine carcinoma
Histology:
Discussion: Sections show solid sheets and large nests of tumor cells with areas of necrosis. The cells have large, round to oval nuclei with fine chromatin and prominent nucleoli. The cytoplasm is eosinophilic with fine vacuoles. Focal acinar structures and intracytoplasmic vacuoles are present (best seen on mucin stain). Immunostains demonstrate strong positivity for CD56, and synaptophysin, as well as focal staining for chromogranin, TTF and CK7. The carcinoma does not express CK20, PANK, p63, CK5/6, and Napsin- A. A mucicarmine stain is focally positive. In addition, the tumor has a high Ki67 labeling index (~80%), and is negative for the prostate markers PSA, PSAP and NKX3. Mutation analyses were performed and show no alterations in the EGFR, ALK and K-ras genes.
In the 2004 WHO classification the category of combined large cell neuroendocrine carcinoma (LCNEC) is recognized for tumors that also have another non-small cell carcinoma component (like adenocarcinoma, squamous cell carcinoma, giant cell carcinoma or spindle carcinoma. Like small cell carcinoma LCNEC-s are histologically heterogeneous and may have a non-neuroendocrine component. In the 2004 WHO classification the term combined LCNEC was chosen arbitrarily to classify these tumors until “future studies better define their biologic behavior”. Combined LCNEC with small cell carcinoma also occurs but those are classified under combined small cell carcinoma. At the present, these tumors should also be subjected to molecular studies in order to provide optimal therapy for those tumors that may have an actionable genetic alteration.
Reference(s):
– Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart pp. 47-48, WHO 2004.
