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Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
Clinical history: 50 y/o Female with a breast mass
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Question 1 of 1
1. Question
Clinical history: 50 y/o Female with a breast mass
Correct
Diagnosis: A. Granular cell tumor
Histology: The resection specimen reveals an infiltrative process comprised of epithelioid cells with round nuclei, centrally located and prominent nucleoli, and abundant eosinophilic granular cytoplasm. The cells are infiltrating around and through benign ducts and lobules. There is no evidence of an atypical intraductal proliferation. There is no appreciable mitotic activity within the cells. Immunostains show the cells to be diffusely immunoreactive for S100 and inhibin, and to be negative for AE1/AE3 and Cam5.2.
Discussion: The morphologic features are classic for a granular cell tumor, which a type of peripheral nerve sheath tumor that can rarely involve the breast. Granular cell tumors characteristically involve the head and neck, where they can incite a prominent pseudoepitheliomatous hyperplasia in overlying squamous epithelium. In the breast, granular cell tumors clinical, radiographically and grossly mimic infiltrating carcinoma because they can appear as a fixed, stellate and infiltrative solid mass. Histologically, granular cell tumors display abundant eosinophilic, granular cytoplasm and can be either epithelioid or spindled, and occur either as nests of cells or as single file rows of cells. The differential diagnosis includes histiocytic proliferations such as xanthogranulomatous inflammation and fat necrosis, as well as other pink cell tumors—melanoma, apocrine carcinomas, and histiocytoid lobular carcinomas. Immunohistochemistry can resolve any diagnostic dilemma, as granular cell tumors are immunoreactive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin and more specific melanocytic markers (HMB45, Melan A). The majority of granular cell tumors are benign and are adequately treated with complete excision.
References:
1. Adeniran A, Al-Ahmadie H, Mahoney MC, Robinson-Smith TM. Granular cell tumor of the breast: a series of 17 cases and review of the literature. The breast journal. 2004;10(6):528-31Brown AC, Audisio RA, Regitnig P. Granular cell tumour of the breast. Surgical oncology. 2011;20(2):97-105.
2. De Simone N, Aggon A, Christy C. Granular cell tumor of the breast: clinical and pathologic characteristics of a rare case in a 14-year-old girl. J Clin Oncol. 2011;29(22):e656-7.
3. 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.
4. Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110 patients. Journal of surgical oncology. 1980;13(4):301-16.
5. 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
Diagnosis: A. Granular cell tumor
Histology: The resection specimen reveals an infiltrative process comprised of epithelioid cells with round nuclei, centrally located and prominent nucleoli, and abundant eosinophilic granular cytoplasm. The cells are infiltrating around and through benign ducts and lobules. There is no evidence of an atypical intraductal proliferation. There is no appreciable mitotic activity within the cells. Immunostains show the cells to be diffusely immunoreactive for S100 and inhibin, and to be negative for AE1/AE3 and Cam5.2.
Discussion: The morphologic features are classic for a granular cell tumor, which a type of peripheral nerve sheath tumor that can rarely involve the breast. Granular cell tumors characteristically involve the head and neck, where they can incite a prominent pseudoepitheliomatous hyperplasia in overlying squamous epithelium. In the breast, granular cell tumors clinical, radiographically and grossly mimic infiltrating carcinoma because they can appear as a fixed, stellate and infiltrative solid mass. Histologically, granular cell tumors display abundant eosinophilic, granular cytoplasm and can be either epithelioid or spindled, and occur either as nests of cells or as single file rows of cells. The differential diagnosis includes histiocytic proliferations such as xanthogranulomatous inflammation and fat necrosis, as well as other pink cell tumors—melanoma, apocrine carcinomas, and histiocytoid lobular carcinomas. Immunohistochemistry can resolve any diagnostic dilemma, as granular cell tumors are immunoreactive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin and more specific melanocytic markers (HMB45, Melan A). The majority of granular cell tumors are benign and are adequately treated with complete excision.
References:
1. Adeniran A, Al-Ahmadie H, Mahoney MC, Robinson-Smith TM. Granular cell tumor of the breast: a series of 17 cases and review of the literature. The breast journal. 2004;10(6):528-31Brown AC, Audisio RA, Regitnig P. Granular cell tumour of the breast. Surgical oncology. 2011;20(2):97-105.
2. De Simone N, Aggon A, Christy C. Granular cell tumor of the breast: clinical and pathologic characteristics of a rare case in a 14-year-old girl. J Clin Oncol. 2011;29(22):e656-7.
3. 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.
4. Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110 patients. Journal of surgical oncology. 1980;13(4):301-16.
5. 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.
