Section of Pediatric and Adolescent Gynecology, Gynecology Clinic, The Hospital for Sick Children and University of Toronto, 555 University Avenue, Toronto, Ontario, Canada.
J Pediatr Urol. 2013 Feb;9(1):e64-7. doi: 10.1016/j.jpurol.2012.08.007. Epub 2012 Oct 25.
Lipoblastoma is a rare, rapidly growing, benign mesenchymal tumor composed of various stages of maturing adipocytes that most often occurs in children under the age of 3. The common locations are the extremities and the trunk. Presentation in the genitoinguinal area is rare. We report a case of a 13-month-old female infant with a 4-month history of a progressively enlarging left labial mass that encompassed her left labium majora and inguinal region. Pelvic MRI confirmed growth from previous ultrasound size of 3 × 2 × 1 cm to 7 × 2 × 2 cm. Composition was suggestive of adipose tissue. The mass was excised through a left inguinal incision. The final pathology results described a lipoblastoma. Six year follow-up has not revealed any signs or symptoms of recurrence. Circumscribed lipoblastomas should be distinguished from their infiltrative counterpart, diffuse lipoblastoma or lipoblastomatosis, which can be more difficult to excise and thus, more likely to recur. Lipoblastoma should also be distinguished from myxoid liposarcoma, which has malignant features, carries a high risk of recurrence, and requires a more aggressive management protocol. Although rare, lipoblastoma should be considered as part of the differential diagnosis of a rapidly growing vulvar mass in prepubertal children.
脂肪母细胞瘤是一种罕见的、快速生长的良性间叶肿瘤,由不同阶段成熟的脂肪细胞组成,多发生于 3 岁以下儿童。常见部位为四肢和躯干。生殖-腹股沟区的表现较为罕见。我们报告了一例 13 个月大的女性婴儿,其左侧大阴唇和腹股沟区有一个 4 个月逐渐增大的左唇肿块。骨盆 MRI 证实肿块从之前超声检查的 3×2×1cm 大小增长至 7×2×2cm。其成分提示为脂肪组织。肿块通过左侧腹股沟切口切除。最终的病理结果描述为脂肪母细胞瘤。6 年随访未发现任何复发迹象或症状。局限性脂肪母细胞瘤应与浸润性的弥漫性脂肪母细胞瘤或脂肪母细胞瘤病相区别,后者更难以切除,因此更有可能复发。脂肪母细胞瘤还应与黏液样脂肪肉瘤相区别,后者具有恶性特征,复发风险高,需要更积极的治疗方案。虽然罕见,但脂肪母细胞瘤应作为快速生长的青春期前儿童外阴肿块的鉴别诊断之一。