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Presented by Justin A. Bishop, MD and prepared by Sarah Karram, MD
This case talks about:
Case 3: 40 year old man with a tumor in his “pharynx.”
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Question 1 of 1
1. Question
Week (610): Case 3
40 year old man with a tumor in his “pharynx.”
Correct
Answer: Sclerosing rhabdomyosarcoma
Histology: This is a spindle cell tumor that has prominent hyaline matrix-like material. The tumor is mitotically active, but relatively uniform at the cellular level. Cytokeratins, p40, S100, and calponin were negative. Desmin is focally positive (in a dot-like manner), and myogenin was also focal.
Discussion: The most common forms of rhabdomyosarcoma (RMS) are alveolar and embryonal. Newer variants include pleomorphic, spindle cell, and sclerosing RMS. Sclerosing RMS is a newly-described variant that commonly affects the head and neck of adults. Unlike the more classic forms of RMS, sclerosing RMS has abundant hyaline stromal material that brings to mind other tumors such as myoepithelial carcinoma, chrondrosarcoma, and osteosarcoma. In addition, rhabdomyoblasts are only rarely identified in sclerosing RMS. The diagnosis must be confirmed by immunohistochemistry. Desmin is usually positive, but it is often focal and may show a peculiar dot-like staining pattern. Myogenin is typically focal or may even be negative; in contrast, MyoD1 is diffuse. While previously regarded as a variant of embryonal RMS, spindle cell and sclerosing variants of RMS were combined into a single, distinct entity in the latest WHO classification. Spindle cell/sclerosing RMS is a high-grade malignancy with a generally poor prognosis.
Incorrect
Answer: Sclerosing rhabdomyosarcoma
Histology: This is a spindle cell tumor that has prominent hyaline matrix-like material. The tumor is mitotically active, but relatively uniform at the cellular level. Cytokeratins, p40, S100, and calponin were negative. Desmin is focally positive (in a dot-like manner), and myogenin was also focal.
Discussion: The most common forms of rhabdomyosarcoma (RMS) are alveolar and embryonal. Newer variants include pleomorphic, spindle cell, and sclerosing RMS. Sclerosing RMS is a newly-described variant that commonly affects the head and neck of adults. Unlike the more classic forms of RMS, sclerosing RMS has abundant hyaline stromal material that brings to mind other tumors such as myoepithelial carcinoma, chrondrosarcoma, and osteosarcoma. In addition, rhabdomyoblasts are only rarely identified in sclerosing RMS. The diagnosis must be confirmed by immunohistochemistry. Desmin is usually positive, but it is often focal and may show a peculiar dot-like staining pattern. Myogenin is typically focal or may even be negative; in contrast, MyoD1 is diffuse. While previously regarded as a variant of embryonal RMS, spindle cell and sclerosing variants of RMS were combined into a single, distinct entity in the latest WHO classification. Spindle cell/sclerosing RMS is a high-grade malignancy with a generally poor prognosis.
