Khatri Arti, Mahajan Nidhi, Sengar Mamta, Agarwal Anil
Departments of Pathology, Chacha Nehru Bal Chikitsalaya, Geeta Colony, DELHI, INDIA.
Turk Patoloji Derg. 2020;1(1):268-274. doi: 10.5146/tjpath.2020.01479.
Lipofibromatosis is a recently recognized slow growing rare pediatric tumor. Paucity of its cytological description in the literature leads to its pre operative misdiagnosis and further incomplete management. A twelve-month-old female presented with a rapidly progressive mass in the right thigh and buttock region. On examination, the mass was huge and involved the medial, posterior and lateral aspects of the thigh. The cytological smears showed mature adipocytes with few spindled out cells. FNA was reported as a lipoma, corroborating with the radiological presumptive diagnosis. However, histopathological and immunohistochemical features favoured a diagnosis of Lipofibromatosis. The cytological smears were reviewed and a cyto-histo correlation was established. The diagnosis of Lipofibromatosis rests upon classical cytological features in a clinically and radiologically suggestive picture. An early and accurate diagnosis if established can help the surgeon plan excision with wider margins as incomplete excision is associated with a high rate of recurrence.
脂肪纤维瘤病是一种最近才被认识到的生长缓慢的罕见儿科肿瘤。文献中对其细胞学描述较少,导致术前误诊及后续治疗不完整。一名12个月大的女性患儿,右大腿和臀部区域出现迅速进展的肿块。检查发现,肿块巨大,累及大腿内侧、后侧和外侧。细胞学涂片显示有成熟的脂肪细胞,伴有少量梭形细胞。细针穿刺抽吸活检(FNA)报告为脂肪瘤,与放射学初步诊断一致。然而,组织病理学和免疫组化特征支持脂肪纤维瘤病的诊断。复查细胞学涂片并建立了细胞-组织学相关性。脂肪纤维瘤病的诊断基于临床和放射学提示性表现中的典型细胞学特征。如果能早期准确诊断,有助于外科医生计划更广泛切缘的切除,因为切除不完整与高复发率相关。