Rawal Gireesha, Zaheer Sufian, Ahluwalia Charanjeet, Dhawan Indrani
Department of Pathology, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India.
J Med Case Rep. 2019 Jan 18;13(1):15. doi: 10.1186/s13256-018-1896-4.
Malignant peripheral nerve sheath tumors are malignant tumors arising from a peripheral nerve or displaying nerve sheath differentiation. Gastrointestinal malignant peripheral nerve sheath tumors are rare and malignant peripheral nerve sheath tumor of the colon is even rarer. To date, only five cases have been reported as malignant peripheral nerve sheath tumor arising in the colon. This is probably the first case report of malignant peripheral nerve sheath tumor of the transverse colon associated with peritoneal metastasis.
A 25-year-old Indian man presented with a large abdominal mass. A computed tomography scan revealed a large 18 cm-sized mass in his transverse colon, suggestive of gastrointestinal stromal tumor. A wide local excision was performed. Histopathology showed sheets and fascicles of elongated to spindle-shaped tumor cells showing a moderate degree of pleomorphism and atypia. Based on morphology and immunohistochemistry, a final diagnosis of malignant peripheral nerve sheath tumor of the transverse colon was given. A peritoneal metastatic tumor deposit was identified grossly and confirmed on histopathology.
This is a rare case report discussing the detailed diagnostic approach along with an extensive review of the literature for malignant peripheral nerve sheath tumor arising in the colon.
恶性外周神经鞘瘤是起源于外周神经或显示神经鞘分化的恶性肿瘤。胃肠道恶性外周神经鞘瘤罕见，结肠恶性外周神经鞘瘤更为罕见。迄今为止，仅有5例结肠原发性恶性外周神经鞘瘤的报道。本文可能是首例横结肠恶性外周神经鞘瘤伴腹膜转移的病例报告。
一名25岁印度男性因腹部巨大肿块就诊。计算机断层扫描显示其横结肠有一个18厘米大小的巨大肿块，提示胃肠道间质瘤。遂行广泛局部切除。组织病理学显示，肿瘤细胞呈片状和束状排列，由细长形至梭形细胞组成，具有中度多形性和异型性。基于形态学和免疫组化结果，最终诊断为横结肠恶性外周神经鞘瘤。术中肉眼发现腹膜转移瘤结节，组织病理学检查予以证实。
本文是一篇罕见病例报告，详细讨论了诊断方法，并对结肠恶性外周神经鞘瘤的相关文献进行了广泛综述。
