Retroperitoneal lipoblastoma: A discussion of current management

Texas Children's Hospital, Department of Pediatric Surgery, Baylor College of Medicine, Houston, TX.
Journal of Pediatric Surgery (Impact Factor: 1.39). 10/2012; 47(10):e51-4. DOI: 10.1016/j.jpedsurg.2012.07.052
Source: PubMed


Retroperitoneal lipoblastomas are rare tumors found in young pediatric patients. Despite their large size at presentation, complete resection is usually achieved with minimal complications. Diagnosis is typically made after pathologic examination of the operative specimen, and cytogenetic analysis may be a key component in differentiating lipoblastoma from other lipomatous tumors. We present one such case of a large, retroperitoneal lipoblastoma and discuss the management of this uncommon entity.

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    • "Hibernoma is a peculiar tumor of brown fat that occurs most commonly in the thigh of adults and is cured with complete excision [17]. Lipoblastoma is a benign tumor that develops from immature adipocytes in young children [18]. "
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    ABSTRACT: While inguinal hernia is common in the primary care office, the differential diagnosis is extensive and includes infectious, inflammatory and neoplastic processes. Varicocele is another frequent, generally benign condition which occasionally reflects serious disease entities. Left-sided or bilateral varicoceles account for the overwhelming majority of varicoceles because the left gonadal vein drains into the left renal vein in contrast to the right gonadal vein, which drains directly into the inferior vena cava, thus making left-sided or bilateral venous congestion more likely. Presence of an uncommon unilateral right-sided varicocele thus warrants further radiological workup, in particular CT abdomen and pelvis, to evaluate for retroperitoneal pathology. We describe a case in which appropriate use of a variety of imaging modalities including testicular ultrasound and CT led to an important diagnosis of a large, well-differentiated liposarcoma in the right retroperitoneum of a patient with a right-sided groin mass.
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    ABSTRACT: Background: Lipoblastomas are rare, benign, soft tissue tumors that occur primarily in young children. Treatment includes complete excision and surveillance for recurrence. Lipoblastomas can be indistinguishable from other benign lipomatous tumors and liposarcomas. Cytogenetic analysis can provide the definitive diagnosis in questionable cases, because benign and malignant lipomatous tumors exhibit specific nonrandom cytogenetic abnormalities. The purpose of the present study was to discuss the disease management and outcomes in a large contemporary group of patients with lipoblastoma. Materials and methods: A retrospective chart review of patients diagnosed with lipoblastoma presenting from 2000-2011 was conducted. The data from these patients were compared with data from a previously published historical group of patients (1985-1999) from the same children's hospital. Results: We identified 37 patients in the contemporary cohort group and compared them with 25 patients from the historical group. The tumor involvement sites were similar. The current cohort group had a lower recurrence rate, although this might have been underestimated owing to a shorter follow-up period (median 1.4 y, range 2 wk to 11.0 y). Preoperative imaging findings led to an incorrect diagnosis in 62% of the patients. Cytogenetic analysis was used to help determine the final diagnosis in 50% of the cases. In 39% of cases, translocations involved the long arm of chromosome 8, the most common anomaly in lipoblastoma. Conclusions: Lipoblastomas are rare tumors in young children that can be misclassified as other malignant or benign lipomatous tumors with markedly different outcomes and treatments. We recommend that cytogenetic analysis be routinely used for all pediatric lipomatous tumors to provide an accurate diagnosis and guide appropriate therapy and follow-up.
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    ABSTRACT: Palpable inguinal mass in children should be differentiated from inguinal hernia, hydrocele, lymph node, and tumor. Though using ultrasonography, fatty tumor would be misdiagnosed as incarcerated inguinal hernia containing fatty component. We experienced the huge inguinal lipoblastoma in 5-year-old girl mimicking recurrent incarcerated hernia. Laparoscopic exploration revealed it was not incarcerated hernia but well demarcated bulging mass from abdominal wall. Mass was about 10×4×3 cm and extended from internal inguinal ring to saphenous opening. It was near total excised because of right external iliac vein injury. Pathologically, it was proven as lipoblastoma containing mature adipocyte with lipoblast and fibrous septa. Postoperatively, we noticed a segmental thrombotic occlusion of external iliac vein. After 1 year, she has no symptom related to occluded vessel. The remained lipoblastoma showed no interval change. Even lipoblastoma has a good prognosis with low recurrence rate, we need careful follow-up.
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