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Presented by Pedram Argani, M.D. and prepared by Priya Banerjee, M.D.
Case 2: 7 year old male with an orbital mass.
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Question 1 of 1
1. Question
Week 360: Case 2
7 year old male with an orbital massimages/8_4_08 2a.jpg
images/8_4_08 2b.jpg
images/8_4_08 2c.jpgCorrect
Answer: Embryonal rhabdomyosarcoma with Anaplasia
Histology: This is a primitive spindle cell lesion characterized by high cellularity, high mitotic rate including atypical mitosis, coarse nuclear chromatin, and foci of marked nuclear pleomorphism. By immunohistochemistry, the neoplastic cells label for myogenin and for desmin. These are the typical features of Embryonal Rhabdomyosarcoma. The marked nuclear pleomorphism and atypical mitotic figures are sufficient to allow the diagnosis of Anaplasia.
Discussion: Alveolar Rhabdomyosarcoma (ARMS) is typically a small round blue cell tumor. ARMS also typically demonstrates more diffuse immunoactivity for myogenin than embryonal rhabdomyosarcoma. Anaplasia may be superimposed upon embryonal or alveolar rhabdomyosarcoma. The presence of Anaplasia often correlates with inactivating p53 gene mutations, which correlates with diffuse immunoreactivity for mutant p53 protein. Rhabdomyomas lack cytologic atypia or significant mitotic activity.
Incorrect
Answer: Embryonal rhabdomyosarcoma with Anaplasia
Histology: This is a primitive spindle cell lesion characterized by high cellularity, high mitotic rate including atypical mitosis, coarse nuclear chromatin, and foci of marked nuclear pleomorphism. By immunohistochemistry, the neoplastic cells label for myogenin and for desmin. These are the typical features of Embryonal Rhabdomyosarcoma. The marked nuclear pleomorphism and atypical mitotic figures are sufficient to allow the diagnosis of Anaplasia.
Discussion: Alveolar Rhabdomyosarcoma (ARMS) is typically a small round blue cell tumor. ARMS also typically demonstrates more diffuse immunoactivity for myogenin than embryonal rhabdomyosarcoma. Anaplasia may be superimposed upon embryonal or alveolar rhabdomyosarcoma. The presence of Anaplasia often correlates with inactivating p53 gene mutations, which correlates with diffuse immunoreactivity for mutant p53 protein. Rhabdomyomas lack cytologic atypia or significant mitotic activity.
