Department of Pediatric Surgery, Hadassah-Hebrew University Medical Center, Jerusalem 91120, Israel.
J Pediatr Surg. 2010 Apr;45(4):830-3. doi: 10.1016/j.jpedsurg.2010.01.015.
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor. Perivascular epithelioid cell tumors of the gastrointestinal tract are very rare, with only about 20 previous reported cases. We present a 5.5-year-old boy with PEComa of the right colon. Treatment consisted of tumor resection only, without additional adjuvant therapy. Two years after surgery, he remains free of tumor. To the best of our knowledge, this is the youngest reported child with PEComa of the colon. We review the literature concerning PEComas in children, especially those of the gastrointestinal tract. We emphasize the importance of correct immunohistochemistry diagnosis, recommended treatment, and surveillance of this unique family of tumors.
血管周上皮样细胞瘤（PEComa）是一种罕见的间叶性肿瘤。胃肠道的血管周上皮样细胞瘤非常罕见，此前仅有约 20 例报道。我们报告了 1 例 5.5 岁的右侧结肠 PEComa 患儿。治疗仅为肿瘤切除术，未行其他辅助治疗。术后 2 年，患儿肿瘤无复发。据我们所知，这是报道的年龄最小的结肠 PEComa 患儿。我们复习了关于儿童 PEComa 的文献，尤其是胃肠道的病例。我们强调了正确的免疫组化诊断、推荐的治疗方案以及对这类独特肿瘤家族进行监测的重要性。
