Abstract
BackgroundLipoblastoma is a rare benign mesenchymal tumor of embryonal fat affecting mainly children below 3 years of age. It presents usually as a painless rapidly growing soft lobular mass in the extremities, trunk, and less frequently in the head-neck region. Preoperative imaging is used to assess the extent of disease and aid surgical planning. Complete surgical excision without injury to surrounding vital structures is the treatment of choice.Case presentationWe report three interesting lipoblastoma cases: mediastinal lipoblastoma with airway compression and ipsilateral diaphragmatic eventration, neck lipoblastoma with intrathoracic extension, and huge thoracic inlet lipoblastoma with compression of common carotid and Lt subclavian arteries, brachial plexus, and ipsilateral diaphragmatic eventration. Complete excision of lipoblastoma mass was done via neck incision in two cases, and the third case required thoracoscopic excision of intrathoracic remnant 6 months later. All three patients had an excellent outcome.ConclusionsManagement of cervicothoracic lipoblastoma is a surgical challenge due to the potential for rapid growth and extension to different fascial planes; however, successful excision can be achieved via a neck approach. Complete surgical excision is essential to prevent local recurrence and improve the outcome.
Highlights
Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat affecting mainly children below 3 years of age
Lipoblastoma was first described by Kirshnan et al in 1926 [1, 2] is a benign tumor of embryonal fat affecting infancy and early childhood with 90% occurring before 3 years of age and 40% in the first year of life [1, 3]
Mediastinal, pleural, pulmonary, and lower neck lipoblastomas can present with dyspnea, wheeze, cough, stridor, and life-threatening asphyxia due to airway compression, vessel compression, superior vena cava syndrome, chest infection, neck swelling, chest wall protrusion, and neurological symptoms resulting from spinal cord compression [2, 5]
Summary
Lipoblastoma is a rare benign tumor of embryonal fat that occurs more commonly in male infants and children less than 3 years of age. We present rare cases of cervicothoracic lipoblastomas with ipsilateral diaphragmatic eventration and upper airway compression. Preoperative imaging is not diagnostic but is beneficial to assess the extent of disease and aid surgical planning. Mediastinal lipoblastoma is a surgical challenge due to the potential for rapid growth and extension to different fascial planes; successful excision can be achieved via a neck approach. Complete surgical excision without injury to surrounding vital structures is the treatment of choice as it yields an excellent prognosis. There is a tendency for these lesions to recur despite presumed complete excision. Follow-up for a minimum of 5 years is recommended
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