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Presented by Dr. Andres Matoso and prepared by Dr. Maryam Shabihkhani
19 year old male with retroperitoneal mass
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1. Question
19 year old male with retroperitoneal mass
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Correct: A
Histology: This tumor is composed of malignant teratoma and adjacent prominent blood vessel neoplasm with endothelial cells with marked nuclear atypia.
Discussion: This is an example of somatic malignancy (angiosarcoma) arising from a teratoma. The definition of somatic malignancy arising in teratoma is a teratoma with a secondary component that resembles a somatic-type malignant neoplasm as seen in other organs and tissues (e.g. sarcomas and carcinomas). The somatic type malignancy should have an infiltrative pattern and occupy at least a 4x field (5 mm). Sarcomas are the most common histologic type, and more than half of these are rhabdomyosarcomas. PNET/Ewing sarcomas are also relatively frequent but they do not harbor the typical ESWR1 translocation. Nephroblastomas (Wilms tumors) are rare but well documented.
References:
1. Comiter CV, Kibel AS, Richie JP, et al. Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases. J Urol. 1998;159:859-863.
2. Guo CC, Punar M, Contreras AL, et al. Testicular germ cell tumors with sarcomatous components: an analysis of 33 cases. Am J Surg Pathol. 2009;33:1173-1178.
3. Colecchia M, Necchi A, Paolini B, et al. Teratoma with somatic-type malignant components in germ cell tumors of the testis: a clinicopathologic analysis of 40 cases with outcome correlation. Int J Surg Pathol. 2011;19:321-327.
4. Ahmed T, Bosl GJ, Hajdu SI. Teratoma with malignant transformation in germ cell tumors in men. Cancer. 1985;56:860-863Incorrect
Correct: A
Histology: This tumor is composed of malignant teratoma and adjacent prominent blood vessel neoplasm with endothelial cells with marked nuclear atypia.
Discussion: This is an example of somatic malignancy (angiosarcoma) arising from a teratoma. The definition of somatic malignancy arising in teratoma is a teratoma with a secondary component that resembles a somatic-type malignant neoplasm as seen in other organs and tissues (e.g. sarcomas and carcinomas). The somatic type malignancy should have an infiltrative pattern and occupy at least a 4x field (5 mm). Sarcomas are the most common histologic type, and more than half of these are rhabdomyosarcomas. PNET/Ewing sarcomas are also relatively frequent but they do not harbor the typical ESWR1 translocation. Nephroblastomas (Wilms tumors) are rare but well documented.
References:
1. Comiter CV, Kibel AS, Richie JP, et al. Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases. J Urol. 1998;159:859-863.
2. Guo CC, Punar M, Contreras AL, et al. Testicular germ cell tumors with sarcomatous components: an analysis of 33 cases. Am J Surg Pathol. 2009;33:1173-1178.
3. Colecchia M, Necchi A, Paolini B, et al. Teratoma with somatic-type malignant components in germ cell tumors of the testis: a clinicopathologic analysis of 40 cases with outcome correlation. Int J Surg Pathol. 2011;19:321-327.
4. Ahmed T, Bosl GJ, Hajdu SI. Teratoma with malignant transformation in germ cell tumors in men. Cancer. 1985;56:860-863
