Patra Susama, Ayyanar Pavithra, Padhi Somanath, Purkait Suvendu, Muduly Dillip K, Samal Subash Chandra
Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Department of Surgical Oncology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Am J Case Rep. 2019 Aug 10;20:1175-1181. doi: 10.12659/AJCR.916251.
BACKGROUND Malignant peripheral nerve sheath tumor (MPNST) with pure epithelioid histology MPNST (epithelioid-MPNST) is a distinctly rare variant of MPNST described by World Health Organization. Occurrence of MPNST in the tubular gut is extremely rare with nearly 20 cases reported in the last 40 years. CASE REPORT In this case report, we described 2 such cases of epithelioid-MPNST occurring in a 65-year male and 47-year-old female, who both presented with bleeding rectal polyps. We also report on our comprehensive systematic literature review. CONCLUSIONS To the best of our knowledge, these are possibly the first two cases of pure epithelioid-MPNST at an unusual site. This needs to be differentiated from lesions more commonly encountered at the related anatomical site, such as malignant melanoma, poorly differentiated carcinoma, lymphoma, or even a gastrointestinal stromal tumor, by appropriate panel of antibodies.
背景 具有纯上皮样组织学特征的恶性外周神经鞘瘤（MPNST）（上皮样MPNST）是世界卫生组织描述的一种极为罕见的MPNST变体。MPNST发生于管状肠道极为罕见，过去40年中仅有近20例报道。病例报告 在本病例报告中，我们描述了2例上皮样MPNST病例，分别发生在一名65岁男性和一名47岁女性身上，他们均表现为直肠息肉出血。我们还报告了全面系统的文献综述。结论 据我们所知，这可能是首例发生在不寻常部位的两例纯上皮样MPNST。需要通过适当的抗体组合将其与在相关解剖部位更常见的病变，如恶性黑色素瘤、低分化癌、淋巴瘤，甚至胃肠道间质瘤相鉴别。
