Rastogi Kanika, Singh Lavleen
Department of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi, India.
Chacha Nehru Bal Chikitsalaya, Delhi, India.
Fetal Pediatr Pathol. 2019 Dec;38(6):490-495. doi: 10.1080/15513815.2019.1619206. Epub 2019 Jun 2.
Lipofibromatosis is a benign pediatric soft tissue tumor arising preferentially in the distal extremities. Histologically, the tumor shows abundant adipose tissue admixed with a spindle cell component, often concentrated in septal and perimysial locations. The index case is being presented to discuss the histopathological and immunohistochemical clues to differentiate it from other fibrofatty tumors of childhood. An 11-month-old male child presented with a slowly growing mass on the upper back. MRI findings were suggestive of an adipocytic tumor. Microscopy revealed a lesion composed of mature adipocytes and intervening fibrous bands with infiltration into the adjacent skeletal muscle, features of lipofibromatosis. Lipofibromatosis should be considered in the differential diagnosis of a pediatric fibrofatty tumor. Accurate diagnosis is essential for proper patient management as incomplete removal of the tumor may result in recurrence.
脂肪纤维瘤病是一种主要发生于儿童四肢远端的良性软组织肿瘤。在组织学上，该肿瘤表现为大量脂肪组织与梭形细胞成分混合存在，常集中于间隔和肌束膜部位。本文报告1例病例，旨在探讨其组织病理学和免疫组化特征，以与其他儿童纤维脂肪性肿瘤相鉴别。一名11个月大男童，上背部出现一缓慢生长的肿块。磁共振成像（MRI）结果提示为脂肪细胞性肿瘤。显微镜检查显示病变由成熟脂肪细胞和其间的纤维带组成，并浸润至邻近骨骼肌，符合脂肪纤维瘤病的特征。在儿童纤维脂肪性肿瘤的鉴别诊断中应考虑脂肪纤维瘤病。准确诊断对于正确的患者管理至关重要，因为肿瘤切除不完全可能导致复发。
