Surgical and Anesthetic Management of a Mediastinal Fatty Tumor: Lipoblastoma

Abstract

Lipoblastoma is a rare fatty tumor that is diagnosed almost exclusively in children. Presentation often consists of respiratory symptoms; chest computed tomography shows a hypodense, low, attenuated mediastinal mass. Surgical approach and anesthetic management are dependent on the location of the tumor and the degree of airway compression; in most cases, a thoracotomy is performed, although a sternotomy is used in selected cases. Final diagnosis can be confirmed using molecular genetic analysis; a genetic hallmark of lipoblastoma is the rearrangement of chromosomal region 8q12 and the PLAG1 gene. Tumor recurrence is rare when a complete resection is performed.

Lipoblastoma: a clinicopathologic review of 23 cases from a major tertiary care center plus detailed review of literature

“…The oncogene PLAG1 (pleomorphic adenoma gene 1) is located on band 8q12. PLAG1 is activated by a breakpoint in the 8q11–13 region or polysomy of chromosome 8 [ 10 , 11 , 23 , 25 , 28 – 32 ]. Molecular analysis is important in the accurate diagnosis and optimum management.…”

“…Close follow up for minimum of 5 years is recommended. Various studies have not reported any recurrences in completely excised tumors with follow ups of 2 months–10 years and median follow up times ranging from 38 to 42 months [ 11 , 14 , 15 , 19 , 22 , 24 , 25 , 27 , 30 ].…”

Lipoblastoma mediastínico en pediatría

“…El cuadro clínico va a depender del tamaño y ubicación del tumor, pudiendo presentarse con síntomas respiratorios como también de manera asintomática 7 . El diagnóstico definitivo será dado por el estudio anátomo-patológico, pero se puede sospechar inicialmente con una adecuada anamnesis, examen físico y estudios imagenológicos.…”

Robotic resection of recurrent pediatric lipoblastoma

“…Although these tumors are benign, they have a tendency to grow rapidly and impinge on and envelop surrounding structures, leading to symptoms. These tumors are most commonly associated with regions of immature adipose tissue in the soft tissue of the trunk and extremities, but they have also been shown to arise in the mediastinum, anterior chest, abdomen, neck, face, rectal area, and presacral region . There have been only case reports and case series in literature; however, the mainstay of treatment relies on surgical excision unless extreme circumstances are present .…”