脂肪母细胞瘤44例临床病理分析

Lipoblastoma:a clinicopathologic analysis of 44 cases

目的 探讨脂肪母细胞瘤的临床、病理及预后。方法 对 4 4例脂肪母细胞瘤临床资料、病理切片和随访结果进行分析。结果 脂肪母细胞瘤 ,手术切除时的平均年龄为 (2 0 8± 14 6 )个月 ,患儿最大 5岁 ,男性多于女性。病变多发生于四肢、头颈部、腰背部及腋部。复发率 18 2 % ,弥漫型明显多于局限型。肿瘤最大径多为 3～ 7cm ,切面比脂肪瘤灰白。镜下见未成熟和成熟的脂肪组织小叶由丰富毛细血管、小静脉的狭窄纤维间隔分割 ,伴不同程度的黏液样基质。结论 脂肪母细胞瘤需与黏液脂肪肉瘤、纤维脂肪肉瘤和婴儿纤维性错构瘤相鉴别。脂肪母细胞瘤生物学行为完全良性 ,虽有局部复发 ,但从无转移及恶变 。

Purpose To investigate the clinical, pathological features and prognosis of lipoblastoma. Methods The clinical data, pathological sections and prognosis in 44 cases of lipoblastoma were analysed. Results There was a male preponderance. The median age of patients was (20 8±14 6) months, in which the oldest was 5 years old. The majority arose in the extremities, head and neck, waist and back, and axilla. The recurrance was 18 2%, in which the diffuse type was more common than circumscribed type. The median size was mostly between 3～7 cm in diameter. The cut surface was paler than lipoma. Microscopically, lobules of immature and mature adipose tissues were separated by narrow fibrous septa with numerous capillaries and venules, and accompanied with varying degree of myxoid matrix. Conclusions The differential diagnoses include myxoid lipoblastoma, fibrolipoma and fibrous hamartoma of infancy. Lipoblastoma is completely benign biological behavior, although some tumors present local recurrance. There is no metastasis or malignant transformation. The treatment of lipoblastoma is local excision.