Zezoff David, Lowas Stefanie, Cleary Muriel, Akalin Ali, Riaz Farhana, Gauguet Jean-Marc
Department of Radiology, Saint Vincent Hospital, Worcester, MA 01608, USA.
Department of Pediatrics, Division of Oncology, UMass Memorial Health, Worcester, MA 01655, USA.
Radiol Case Rep. 2024 Sep 23;19(12):6199-6204. doi: 10.1016/j.radcr.2024.08.143. eCollection 2024 Dec.
Epithelioid inflammatory myofibroblastic sarcoma, a variant of the inflammatory myofibroblastic tumor, is a rare tumor that is not well described in the radiologic literature. We present a case of a 14-year-old male adolescent who presented with fever, fatigue, and weight loss symptoms and was found to have an abdominal mass on contrast enhanced CT. Initial differentials included lymphoma, pheochromocytoma, desmoid, and sarcoma, and pathological evaluation revealed an epithelioid inflammatory myofibroblastic sarcoma. The mass was separate from the surrounding structures of the left upper abdomen with unique radiologic features not previously described in the literature. Prior literature examples of epithelioid inflammatory myofibroblastic sarcoma described a heterogenous, enhancing lobulated mass, and our case was a lobulated, avidly enhancing homogenous mass on CT with surrounding inflammation and avid uptake on PET/CT. In addition to the imaging features, we describe the surgical findings, the pathologic features of the tumor, and the oncologic treatment of this patient. This case highlights the importance of including rare tumors such as epithelioid inflammatory myofibroblastic sarcoma as a potential differential consideration of an avidly enhancing homogenous abdominal mass in an adolescent.
上皮样炎性肌纤维母细胞肉瘤是炎性肌纤维母细胞瘤的一种变体，是一种罕见肿瘤，放射学文献中对其描述不多。我们报告一例14岁男性青少年病例，该患者出现发热、乏力和体重减轻症状，在增强CT检查时发现腹部有肿块。初步鉴别诊断包括淋巴瘤、嗜铬细胞瘤、硬纤维瘤和肉瘤，病理评估显示为上皮样炎性肌纤维母细胞肉瘤。该肿块与左上腹周围结构分离，具有文献中未曾描述过的独特放射学特征。既往上皮样炎性肌纤维母细胞肉瘤的文献病例描述为不均匀强化的分叶状肿块，而我们的病例在CT上是一个分叶状、明显强化的均匀肿块，周围有炎症，PET/CT上有明显摄取。除了影像学特征外，我们还描述了手术所见、肿瘤的病理特征以及该患者的肿瘤治疗情况。该病例强调了将上皮样炎性肌纤维母细胞肉瘤等罕见肿瘤作为青少年中明显强化的均匀腹部肿块的潜在鉴别诊断考虑因素的重要性。
