Clear cell myomelanocytic tumor of ligamentum teres
DOI:
https://doi.org/10.4322/acr.2024.503Keywords:
Immunohistochemistry, Liver Neoplasms, Perivascular Epithelioid Cell NeoplasmsAbstract
Clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres is a rare hepatic tumor, a variant of the perivascular epithelioid cell tumor (PEComa) family. CCMMT is the rarest variant of hepatic PEComas. Only a few cases of CCMMT have been reported in the English literature. Because of its rarity, less is known about its biological behavior. We present a case of a 31-year-old female who complained of abdominal pain, bilious vomiting, and abdominal fullness over two months. The radiological impression was of focal nodular hyperplasia. The histological examination of the resection specimen revealed a well-circumscribed tumor arranged in fascicles, sheets, and a whorling pattern. The tumor cells were spindle to epithelioid shaped with abundant clear to pale eosinophilic cytoplasm. The tumor cells expressed both myoid (smooth muscle actin) and melanocytic (MelanA and HMB45) markers, while they were negative for hepatocytic and vascular markers. Thus, based on histology and immunohistochemistry, a diagnosis of CCMMT was made. This case presents the diagnostic challenges of CCMMT and discusses the differential diagnosis with a literature review.
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Folpe AL, Goodman ZD, Ishak KG, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol. 2000;24(9):1239-46. http://doi.org/10.1097/00000478-200009000-00007. PMid:10976698.
Tanaka Y, Ijiri R, Kato K, et al. HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cellsugar’tumors. Am J Surg Pathol. 2000;24(9):1295-9. http://doi.org/10.1097/00000478-200009000-00015. PMid:10976706.
Wang Z-S, Xu L, Ma L, Song M-Q, Wu L-Q, Zhou X. Hepatic falciform ligament clear cell myomelanocytic tumor: a case report and a comprehensive review of the literature on perivascular epithelioid cell tumors. BMC Cancer. 2015;15:1004. http://doi.org/10.1186/s12885-015-1992-4. PMid:26698563.
Nie P, Wu J, Wang H, et al. Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation. Cancer Imaging. 2019;19(1):32. http://doi.org/10.1186/s40644-019-0212-x. PMid:31171030.
Tan Y, Zhang H, Wang X. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Indian J Pathol Microbiol. 2014;57(3):453-5. http://doi.org/10.4103/0377-4929.138766. PMid:25118744.
Tan Y, Zhang H, Xiao E-H. Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics—analysis of 32 cases and review of the literature. Clin Radiol. 2013;68(6):555-61. http://doi.org/10.1016/j.crad.2012.10.021. PMid:23245276.
Akitake R, Kimura H, Sekoguchi S, et al. Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. Intern Med. 2009;48(24):2083-6. http://doi.org/10.2169/internalmedicine.48.2133. PMid:20009396.
Tan Y, Xiao E. Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features—analysis of 7 cases and review of the literature. Abdom Imaging. 2012;37(5):781-7. http://doi.org/10.1007/s00261-012-9850-1. PMid:22278345.
Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F. PEComas: the past, the present and the future. Virchows Arch. 2008;452(2):119-32. http://doi.org/10.1007/s00428-007-0509-1. PMid:18080139.
Priola AM, Priola SM, Cataldi A, Marci V, Fava C. Acute abdomen as an unusual presentation of hepatic PEComa. A case report. Tumori. 2009;95(1):123-8. http://doi.org/10.1177/030089160909500124. PMid:19366072.
Larbcharoensub N, Karnsombut P, Jatchavala J, Wasutit Y, Nitiyanant P. Primary hepatic clear cell myomelanocytic tumor: case report and review of the literature. Acta Pathol Microbiol Scand Suppl. 2007;115(12):1454-9. http://doi.org/10.1111/j.1600-0463.2007.00733.x. PMid:18184420.
Ergün S, Akıncı O, Çomunoğlu N, Kocael A. Clear-cell myomelanocytic tumor of ligamentum teres hepatis. Cerrahpaşa Medical Journal. 2021;45(1):57-9. http://doi.org/10.5152/cjm.2021.21007.
Agaram NP, Sung YS, Zhang L, et al. Dichotomy of genetic abnormalities in PEComas with therapeutic implications. Am J Surg Pathol. 2015;39(6):813-25. http://doi.org/10.1097/PAS.0000000000000389. PMid:25651471.
Schoolmeester JK, Dao LN, Sukov WR, et al. TFE3 translocation–associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. Am J Surg Pathol. 2015;39(3):394-404. http://doi.org/10.1097/PAS.0000000000000349. PMid:25517951.
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