Abstract
Clear-cell myomelanocytic tumors (CCMT) of the perivascular epithelioid cell tumor (PEComa) family have been recently reported. We report a case involving a 12-year-old girl. The tumor (9 × 7.5 × 7 cm) was a firm, tan–gray mass with heavily dark pigmentation, massive hemorrhage, and necrosis, and was located in the right broad ligament attached to the right ovary. Histologically, the tumor was composed of polygonal cells exhibiting diffuse hemorrhage, multifocal necroses, and vascular invasion. Most of the tumor cells contained melanin pigments with Fontana–Masson positivity and ultrastructurally suspicious, membrane-bound premelanosomes. Immunohistochemical staining was positive against HMB-45 and focally positive for smooth muscle actin. The tumor recurred in the form of multiple conglomerated masses of the right iliac fossa, with the greatest measuring up to 3.8 cm in dimension, within 1 year. Most CCMT are believed to originate from falciform ligament/ligamentum teres. To the best of our knowledge, this is the second report of a CCMT arising in the broad ligament with typical morphology and contributory ancillary results. Further study for proper subclassification of the PEComa family should be validated, not by anatomic site but by clinical behavior.
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References
Adachi S, Hanada M, Kobayashi Y, Tsutahara K, Fukuhara S, Mori N, Hara T, Mukai H, Shimasaku E, Kawai M, Kishikawa N, Yamaguchi S (2004) Heavily melanotic perivascular epithelioid celar cell tumor of the kidney. Pathol Int 54:261–265
Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, Bacchi C, Sin VC, Wong NL, Coady M, Cahn JK (2001) Abdominopelvic sarcoma of perivascular epithelioid cells: report of four cases in young women, one with tuberous sclerosis. Mod Pathol 14:563–568
Bonetti F, Pea M, Martignoni G, Zamboni G (1992) PEC and sugar [Letter]. Am J Surg Pathol 16:307–308
Cibas ES, Goss GA, Kulke MH et al (2001) Malignant epithelioid angiomyolipoma (‘sarcoma ex angiolipoma’) of the kidney: a case report and review of the literature. Am J Surg Pathol 25:121–126
Fadare O, Parkash V, Yilmaz Y, Mariappan MR, Ma L, Hileeto D, Qumsiyeh MB, Hui P (2004) Perivascular epitelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal “PEComatosis”: a clinicopathological study with comparative genomic hybridization analysis. World J Surg Onc 2:35–47
Fink D, Marsden DE, Edwards L, Camaris C, Hacker NF (2004) Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. Int J Gynecol Cancer 14:1036–1039
Folpe AL, Goodman ZD, Ishak KG et al (2000) Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol 24:1239–1246
Ruco LP, Pilozzi E, Weddard BM et al (1998) Epithelioid lymphagioleiomyomatosis-like tumor of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology 33:91–93
Pea M, Martignoni G, Zamboni G et al (1996) Peivascular epithelioid call [Letter]. Am J Surg Pathol 20:185–188
Vang R, Kempson R (2002) Perivascular epithelioid cell tumor (‘PECom’) of the uterus: a subset of HMB-45 positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol 26:1–13
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Kim, HJ., Lim, SJ., Choi, H. et al. Malignant clear-cell myomelanocytic tumor of broad ligament—a case report. Virchows Arch 448, 867–870 (2006). https://doi.org/10.1007/s00428-006-0161-1
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DOI: https://doi.org/10.1007/s00428-006-0161-1