Valderrama S Javier, Schnettler R David, Zamorano H Marcelo, Lahsen H Juan Pablo
Licenciado en Medicina, Universidad Católica del Maule, Talca, Chile.
Cirujano Pediátrico, Magíster en Bioética, Facultad de Medicina Universidad Católica del Maule, Servicio de Cirugía Infantil, Hospital Regional de Talca, Talca, Chile.
Rev Chil Pediatr. 2016 May-Jun;87(3):199-203. doi: 10.1016/j.rchipe.2015.10.009. Epub 2015 Nov 22.
Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%.
To present an unusual location of this uncommon condition in an infant, and review the related literature.
A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease.
Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood.
脂肪母细胞瘤是一种脂肪组织的良性肿瘤。它是一种罕见疾病,几乎仅见于3岁以下儿童。它通常发生于四肢,表现为进行性生长的无痛性体积增大,最终诊断需通过病理和细胞遗传学分析来确定。首选治疗方法是完整切除,由于复发率高达25%,建议随访期长达五年。
介绍这种罕见疾病在一名婴儿中的不寻常发病部位,并回顾相关文献。
一名16个月大的儿童,腹部增大6个月且持续进展,伴有食欲减退,无其他症状。影像学检查显示一个几乎累及整个腹腔的类脂质样影像,高度提示脂肪母细胞瘤。对一个直径18厘米的腹膜后肿瘤进行了切除,该肿瘤与相邻器官粘连。组织学分析足以确诊,无需进行细胞遗传学分析。随访显示疾病无复发。
鉴于这种疾病的罕见性及其不寻常的表现,我们报告此临床病例,以便在儿童腹部肿块的鉴别诊断中予以考虑。