A large retroperitoneal lipoblastoma

Abstract

Introduction:Lipoblastoma is a rare benign soft tissue tumor that occurs most commonly in infants and children. However, retroperitoneal lipoblastomas are rare, occurring in <5% of cases. We report a case of large retroperitoneal lipoblastoma and the largest collection of known retroperitoneal lipoblastomas in children in the literature.Case presentation:A 3-year-old girl presented with left abdominal mass. Magnetic resonance imaging (MRI) revealed a soft tissue mass measuring 12 × 8 × 6 cm in the retroperitoneal region. The mass had a clearly defined margin and a reticular pattern with an interposing fat component. Based on these findings, the mass was suspected to be a soft-tissue tumor, most likely lipoblastoma.Laparotomy with resection of the retroperitoneal mass was performed. The tumor was easily dissected from the retroperitoneal space without injury to surrounding structure.A histopathological examination demonstrated the mature proliferation of adipocytes and spindle-shaped cells separated by fibrovascular septa accompanied by myxoid changes. The cells were separated into lobules by septa, and areas of immature adipocytes showing a signet-ring or multivacuolar appearance were present at the periphery. Histopathological diagnosis was lipoblastoma. Follow-up at 6 months revealed no evidence of recurrence.Conclusion:Retroperitoneal lipoblastoma is rare and tends to be large in size when diagnosed at presentation. Complete resection should not be delayed, as impingement on the surrounding structures is imminent.

Fetal Lipoblastoma Presenting as a Retroperitoneal Tumor in Adults: A Case Report

“…Lipoblastoma (LB) is a rare benign mesenchymal soft tissue neoplasm that occurs most frequently in childhood and rarely in adults [1,2]. It has a prevalence of 0.6% of benign soft tissue tumors [3].…”

“…It has a prevalence of 0.6% of benign soft tissue tumors [3]. The vast majority is detected in children under three years old, with more than 80% of cases before three years old and 40% before one year old [1,3]. It is more common in males than in females [1,2,4].…”

“…The vast majority is detected in children under three years old, with more than 80% of cases before three years old and 40% before one year old [1,3]. It is more common in males than in females [1,2,4]. It is a neoplasm of embryonic white blood cells, which usually presents as a well-defined tumor or presents as a diffuse process known as localized lipoblastomatosis (LBS) [2].…”

“…It is a neoplasm of embryonic white blood cells, which usually presents as a well-defined tumor or presents as a diffuse process known as localized lipoblastomatosis (LBS) [2]. Approximately 70% of these tumors occur in the extremities, trunk, head, and neck; however, retroperitoneal lipoblastomas are less common (<5% of cases) [1][2][3]. The differential diagnosis of the tumor is broad and includes sarcomas, neuroblastomas, and teratomas [1].…”

“…Approximately 70% of these tumors occur in the extremities, trunk, head, and neck; however, retroperitoneal lipoblastomas are less common (<5% of cases) [1][2][3]. The differential diagnosis of the tumor is broad and includes sarcomas, neuroblastomas, and teratomas [1]. Although histologically it is benign, it has a locally invasive behavior with a risk of recurrence if it is not completely removed [5].…”