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Lipofibromatosis: Central Nervous System Involvement by a Benign Neoplasm
Alejandra Rebolledo1, Laura J Guerra2, Ander Eo Dubón1
1Department of Pediatrics, Dr. Belisario Domínguez ISSSTE General Hospital, Tuxtla Gutiérrez, Chiapas, Mexico.
Fetal and Pediatric Pathology
|May 10, 2022
Summary
A rare pediatric tumor, lipofibromatosis, can aggressively infiltrate the central nervous system. This case highlights the challenges in diagnosing and surgically treating this infiltrative soft tissue tumor in infants.
Area of Science:
- Pediatric Oncology
- Pediatric Neurosurgery
- Pathology
Background:
- Lipofibromatosis is a rare soft tissue tumor primarily affecting pediatric extremities.
- Atypical lipofibromatosis can exhibit aggressive infiltrative growth patterns.
- Early diagnosis and complete surgical resection are crucial for favorable outcomes.
Observation:
- An infant presented with a rapidly growing cervicothoracic tumor extending from the brainstem to T6.
- Initial imaging suggested myelomeningocele, but subsequent MRI revealed an infiltrative mass.
- The tumor caused significant neurological deficits, including respiratory failure and quadriparesis.
Findings:
- Pathological confirmation of lipofibromatosis in a critical cervicothoracic location.
- Multiple surgical attempts were unsuccessful in achieving complete tumor removal.
- The tumor's infiltrative nature posed significant surgical challenges.
Implications:
- Lipofibromatosis can involve the central nervous system, leading to severe morbidity.
- Surgical management of CNS-infiltrating lipofibromatosis is complex and may require multidisciplinary approaches.
- This case underscores the need for high vigilance in diagnosing and managing atypical pediatric soft tissue tumors.
