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Presented by Dr. Cimino-Mathews and prepared by Dr. Armen Khararjian.
This case talks about: A 25 year-old female with a lung mass.
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Question 1 of 1
1. Question
Week 626: Case 2
25 year-old female with a lung mass.
Correct
Answer: B. Granular Cell Tumor
Histology:
The lung lobectomy shows a peri-bronchial lesion centered in the submucosa, with polypoid projections into the bronchial lumen. The lesion consists of sheets and nests of round to polygonal cells with abundant eosinophilic, granular cytoplasm, round nuclei and prominent nucleoli. There is no necrosis, pleomorphism, or increased mitotic activity. Immunostains show the lesional cells to be positive for CD68 and S100, and to be negative for cytokeratin AE1/AE3.Discussion:
Granular cell tumors are neoplasms arising from peripheral nerve Schwann cells and can occur throughout the body, although they characteristically involve the head and neck region. Granular cell tumors may be grossly well-circumscribed or infiltrative, and clinically can mimic a carcinoma. When occurring in submucosal regions, granular cell tumors can incite a prominent pseudoepitheliomatous hyperplasia in the overlying squamous epithelium, mimicking an in situ or invasive squamous cell carcinoma.Histologically, granular cell tumors resemble histiocytes and the differential diagnosis includes xanthrogranulomatous inflammation or fat necrosis. In addition, when granular cell tumors infiltrate as cords of cells, they can resemble histiocytoid carcinoma (such as histiocytoid lobular carcinoma of the breast). Immunophenotypically, granular cell tumors are positive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin.
The majority of granular cell tumors are benign and are treated with complete excision, however malignant granular cell tumors that can metastasize do occur. The presence of three or more of the following features suggests malignancy in a granular cell tumor: the presence of necrosis, increased mitotic activity (>2 mitoses per 10 high power fields), vesicular nuclei with markedly prominent nucleoli, and pleomorphism.
References:
1. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779-94.
2. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Archives Pathol Lab Med. 2004;128(7):771-5.Incorrect
Answer: B. Granular Cell Tumor
Histology:
The lung lobectomy shows a peri-bronchial lesion centered in the submucosa, with polypoid projections into the bronchial lumen. The lesion consists of sheets and nests of round to polygonal cells with abundant eosinophilic, granular cytoplasm, round nuclei and prominent nucleoli. There is no necrosis, pleomorphism, or increased mitotic activity. Immunostains show the lesional cells to be positive for CD68 and S100, and to be negative for cytokeratin AE1/AE3.Discussion:
Granular cell tumors are neoplasms arising from peripheral nerve Schwann cells and can occur throughout the body, although they characteristically involve the head and neck region. Granular cell tumors may be grossly well-circumscribed or infiltrative, and clinically can mimic a carcinoma. When occurring in submucosal regions, granular cell tumors can incite a prominent pseudoepitheliomatous hyperplasia in the overlying squamous epithelium, mimicking an in situ or invasive squamous cell carcinoma.Histologically, granular cell tumors resemble histiocytes and the differential diagnosis includes xanthrogranulomatous inflammation or fat necrosis. In addition, when granular cell tumors infiltrate as cords of cells, they can resemble histiocytoid carcinoma (such as histiocytoid lobular carcinoma of the breast). Immunophenotypically, granular cell tumors are positive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin.
The majority of granular cell tumors are benign and are treated with complete excision, however malignant granular cell tumors that can metastasize do occur. The presence of three or more of the following features suggests malignancy in a granular cell tumor: the presence of necrosis, increased mitotic activity (>2 mitoses per 10 high power fields), vesicular nuclei with markedly prominent nucleoli, and pleomorphism.
References:
1. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779-94.
2. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Archives Pathol Lab Med. 2004;128(7):771-5.
