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Presented by Pedram Argani, M.D. and prepared by Orin Buetens, M.D.
Case 1: 18-year-old female with an ovarian mass.
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Question 1 of 1
1. Question
Week 40: Case 1
18-year-old female with an ovarian mass./images/1985a.jpg
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/images/1985d.jpgCorrect
Answer: Clear cell sarcoma of tendon sheath involving ovary (melanoma of soft parts)
Histology: The tumor consists of nests and short fascicles of spindled to epithelioid cells with clear to finely stippled eosinophilic cytoplasm. On higher power, the cells have distinctive prominent nucleoli, and are not pleomorphic. The fibrovascular septae separating the tumor have a hyaline appearance in areas. This tumor labeled for S-100 protein, HMB45 and microphthalmia transcription factor (MiTF).
Discussion: Malignant melanoma is immunophenotypically indistinguishable from clear cell sarcoma of tendon sheath, as both label for S-100, HMB45, and MiTF. Both contain melanin pigment and both demonstrate melanosomes ultrastructurally. Melanoma is typically more pleomorphic than clear cell sarcoma of tendon sheath, and lacks the typical fascicular architecture. Definitive distinction can be made with cytogenetics; clear cell sarcoma is characterized by a t(12;22) (q13;q12) translocation that uses the EWS to the ATF-1 genes. This translocation is not identified in malignant melanoma. Ovarian fibrothecoma usually presents in patients over 40 years of age. Like the above lesion, these tumors are spindled and may be associated with hyaline extracellular material that forms plaques. However, the thecomatous areas should demonstrate intracytoplasmic lipid, and neither fibromas nor thecomas label for melanocytic markers. Ovarian clear cell carcinomas may also be associated with hyaline material, but this is typically seen in the fibrovascular cores that support papillary formations. Clear cell carcinoma classically demonstrates hobnail epithelial cells and a tubulocystic growth pattern. These tumors label for cytokeratins but not for melanocytic markers.
Clear cell sarcoma of tendon sheath typically affects young adults, and is classically located in the region of the foot and ankle. The tumor is typically treated by radical excision, and has a 50% mortality. Large tumor size and necrosis are poor prognostic factors. In the current case, no history of a previous surgery was provided. It is possible that this is an example of a rare lesion presenting in an extremely unusual location; i.e., in the ovary.
Incorrect
Answer: Clear cell sarcoma of tendon sheath involving ovary (melanoma of soft parts)
Histology: The tumor consists of nests and short fascicles of spindled to epithelioid cells with clear to finely stippled eosinophilic cytoplasm. On higher power, the cells have distinctive prominent nucleoli, and are not pleomorphic. The fibrovascular septae separating the tumor have a hyaline appearance in areas. This tumor labeled for S-100 protein, HMB45 and microphthalmia transcription factor (MiTF).
Discussion: Malignant melanoma is immunophenotypically indistinguishable from clear cell sarcoma of tendon sheath, as both label for S-100, HMB45, and MiTF. Both contain melanin pigment and both demonstrate melanosomes ultrastructurally. Melanoma is typically more pleomorphic than clear cell sarcoma of tendon sheath, and lacks the typical fascicular architecture. Definitive distinction can be made with cytogenetics; clear cell sarcoma is characterized by a t(12;22) (q13;q12) translocation that uses the EWS to the ATF-1 genes. This translocation is not identified in malignant melanoma. Ovarian fibrothecoma usually presents in patients over 40 years of age. Like the above lesion, these tumors are spindled and may be associated with hyaline extracellular material that forms plaques. However, the thecomatous areas should demonstrate intracytoplasmic lipid, and neither fibromas nor thecomas label for melanocytic markers. Ovarian clear cell carcinomas may also be associated with hyaline material, but this is typically seen in the fibrovascular cores that support papillary formations. Clear cell carcinoma classically demonstrates hobnail epithelial cells and a tubulocystic growth pattern. These tumors label for cytokeratins but not for melanocytic markers.
Clear cell sarcoma of tendon sheath typically affects young adults, and is classically located in the region of the foot and ankle. The tumor is typically treated by radical excision, and has a 50% mortality. Large tumor size and necrosis are poor prognostic factors. In the current case, no history of a previous surgery was provided. It is possible that this is an example of a rare lesion presenting in an extremely unusual location; i.e., in the ovary.
