Batool Sajida, Ahuja Arvind, Chauhan Devender Singh, Bhardwaj Minakshi, Meena Atul Kumar
Atal Bihari Vajpayee Institute of Medical Sciences, Dr Ram Manohar Lohia Hospital, Department of Pathology, New Delhi, India.
Atal Bihari Vajpayee Institute of Medical Sciences, Dr Ram Manohar Lohia Hospital, Department of Pediatric Surgery, New Delhi, India.
Autops Case Rep. 2021 May 25;11:e2021288. doi: 10.4322/acr.2021.288. eCollection 2021.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised . Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.
上皮样炎性肌纤维母细胞肉瘤（EIMS）是炎性肌纤维母细胞瘤的一种罕见变体。它具有侵袭性的临床病程和高复发率。EIMS主要影响儿童和年轻人。在此，我们报告一名4个月大的婴儿患有该疾病，其表现为腹部肿块。影像学检查显示一个大的低密度肠系膜病变，累及腹部右半部分和中部区域。连同附着的一小段小肠一并切除了肿块。大体上，一个大的包膜完整的肿瘤从小肠系膜长出。组织学检查显示肿瘤由上皮样细胞至梭形细胞组成，松散地排列在黏液样背景中，有大量血管和淋巴浆细胞性炎性浸润。免疫组化显示肿瘤细胞ALK1（细胞核）、结蛋白、平滑肌肌动蛋白、CD68呈阳性，CD30呈局灶性阳性。最终诊断为小肠EIMS。据我们所知，该病例是文献报道中最年轻的病例。
