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Presented by Hind Nassar, M.D.. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 2: The patient is a 16 year old male with a nasal mass.
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Question 1 of 1
1. Question
Week 387: Case 2
The patient is a 16 year old male with a nasal mass.images/nassar033009-2a.jpg
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images/nassar033009-2e.jpgCorrect
Answer: Rhabdomyosarcoma (alveolar type)
Histology: The tumor is composed of malignant small blue cells with areas of necrosis. The tumor cells are hyperchromatic with high N/C ratio focally with an eosinophilic cytoplasm. Rare multinucleated tumor cells are seen. They are focally arranged in a nested pattern separated by fibrous stroma.
Discussion: The differential diagnosis includes a wide range of neoplastic lesions, most commonly at this age lymphoma, leukemia, Ewing’s sarcoma/PNET and rhabdomyosarcoma. By immunostains, the tumor cells in this case were positive for desmin, myogenin and CD56 and negative for keratins, S100, CD45, and CD99. Therefore the tumor represents an alveolar rhabdomyosarcoma. This tumor is characterized by a specific genetic translocation t (2, 13) involving the genes PAX3 and FKHR and more rarely t (1, 13) involving the genes PAX7 and FKHR. Lymph node metastases can be observed in this tumor. Alveolar rhabdomyosarcoma is a tumor with poor prognosis.
Incorrect
Answer: Rhabdomyosarcoma (alveolar type)
Histology: The tumor is composed of malignant small blue cells with areas of necrosis. The tumor cells are hyperchromatic with high N/C ratio focally with an eosinophilic cytoplasm. Rare multinucleated tumor cells are seen. They are focally arranged in a nested pattern separated by fibrous stroma.
Discussion: The differential diagnosis includes a wide range of neoplastic lesions, most commonly at this age lymphoma, leukemia, Ewing’s sarcoma/PNET and rhabdomyosarcoma. By immunostains, the tumor cells in this case were positive for desmin, myogenin and CD56 and negative for keratins, S100, CD45, and CD99. Therefore the tumor represents an alveolar rhabdomyosarcoma. This tumor is characterized by a specific genetic translocation t (2, 13) involving the genes PAX3 and FKHR and more rarely t (1, 13) involving the genes PAX7 and FKHR. Lymph node metastases can be observed in this tumor. Alveolar rhabdomyosarcoma is a tumor with poor prognosis.
