Perivascular Epithelioid Cell Tumor (PEComa) in the Ascending Colon: A Case Report

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor composed of perivascular epithelioid cell (PEC).1 The PEC is characterized by epithelioid appearance, clear to eosinophilic granular cytoplasm, perivascular location, and coexpression of smooth muscle and melanocytic markers.^(1-5) The PEC-related tumors include angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations. Many PEComas have been associated with the tuberous sclerosis complex, an autosomal dominant disorder characterized by seizures, mental retardation, and the development of tumors in multiple organs including the brain and skin.1,6,7 We report a case of colonic PEComa with personal and family history of tuberous sclerosis complex (TSC).

關鍵字

Perivascular epithelioid cell tumor ； PEComa ； TSC

並列摘要

血管旁類上皮細胞瘤 (perivascular epithelioid cell tumor) 是一群由類上皮細胞所形成的罕見腫瘤。這些類上皮細胞的特徵是會圍繞著血管分布，並可以同時表現出具黑色素細胞分泌功能的標記，以及肌肉組織的標記。此類腫瘤多發生於軟組織，有一些病例被報告發生於腎臟、肝臟、子宮等臟器中，但發生於結腸的病例很少。由於病例稀少，目前尚無標準治療方針與準確地預測病人臨床表現。我們報告一位升結腸血管旁類上皮細胞瘤的病例，表現症狀是右側腹痛及便血。經大腸鏡和電腦斷層掃描發現升結腸腫瘤併腸套疊。病患接受右側結腸切除手術，經過病理切片及免疫染色多種抗體檢查後確診是血管旁類上皮細胞瘤。部分血管旁類上皮細胞瘤和結節硬化症(Tuberous Sclerosis Complex; TSC)有關，部分血管旁類上皮細胞瘤和結節硬化症的基因改變相似。

並列關鍵字

血管旁類上皮細胞瘤；結節硬化症；升結腸

參考文獻

1. A.L. Folpe, Z.D. Goodman, K.G. Ishak, A.F.G. Paulino, E.M. Taboada, S.A. Meehan, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres Am J Surg Pathol, 24 (9) (2000), pp. 1239–1246

2. D. Govender, R.M. Sabaratnam, A.S. Essa Clear cell "sugar" tumor of the breast. Another extrapulmonary site and review of the literature Am J Surg Pathol, 26 (5) (2002), pp. 670–675

3. H.D. Tazelaar, K.P. Batts, J.R. Srigley Primary extrapulmonary sugar tumor (PEST): a report of four cases Mod Pathol, 14 (6) (2001), pp. 615–622

4. R. Vang, R.L. Kempson Perivascular epithelioid cell tumor (″PEComa') 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) (2002), pp. 1–13

5. Chang, Karen L., Folpe, Andrew L. Diagnostic Utility of Microphthalmia Transcription Factor in Malignant Melanoma and Other Tumors Adv Anat Pathol, 8 (5) (2001), pp. 273–275

國際替代計量

Perivascular Epithelioid Cell Tumor (PEComa) in the Ascending Colon: A Case Report

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