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A rare case of myxoid pleomorphic liposarcoma in an infant: A report
Sumit Gami1, Sansar Babu Tiwari2, Kamal Gautam1
1Oxford University Clinical Research Unit, Patan Academy of Health Sciences, Lagankhel, Kathmandu, Nepal.
Insights
Myxoid pleomorphic liposarcoma (MPL) is a rare pediatric chest tumor. Complete surgical excision is crucial for managing this aggressive cancer, with molecular studies aiding diagnosis.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Medical Imaging
Background:
- Myxoid pleomorphic liposarcoma (MPL) is an uncommon, aggressive liposarcoma primarily affecting children and infants.
- MPL commonly presents in the chest, posing surgical challenges due to its location and potential for recurrence.
Purpose of the Study:
- To highlight the diagnostic and management considerations for pediatric MPL presenting as a large intrathoracic mass.
- To emphasize the importance of prompt diagnosis and complete surgical resection in improving outcomes for pediatric MPL.
Main Methods:
- Case presentation of a 12-month-old infant with respiratory distress due to a large left-sided chest mass.
- Diagnostic imaging included Computed Tomographic (CT) scan.
- Histopathological examination and molecular studies (Immunohistochemistry - IHC, Fluorescent in-situ Hybridization - FISH) for confirmation.
Main Results:
- CT scan revealed a large solid mass in the left hemithorax, displacing the mediastinum.
- Histopathology confirmed myxoid pleomorphic liposarcoma, negative for MDM2.
- MPL demonstrated aggressive behavior with a high propensity for metastasis and recurrence.
Conclusions:
- Intrathoracic tumors in infants warrant consideration of soft tissue malignancies like MPL.
- Complete surgical excision, supplemented by chemotherapy and irradiation, is the cornerstone of MPL treatment.
- FISH and IHC are essential for accurate diagnosis and confirmation of MPL.
Introduction And Importance:
MPL (myxoid pleomorphic liposarcoma) is an uncommon type of liposarcoma that affects mostly children and infants. Its aggressive behavior and tendency to recur warrant complete excision despite the challenges of troublesome locations.
Case Presentation:
A 12-month-old infant presented with an insidious onset of noisy breathing and respiratory distress not relieved by supplemental oxygen via face mask. Examination revealed dullness and decreased air entry on the left chest. Computed Tomographic (CT) scan showed a large solid mass occupying the left hemithorax and displacing the mediastinum to the right. Intraoperatively, a large solid mass arising from the left chest wall and attached to the fifth rib was seen. Histopathology of the resected mass showed myxoid pleomorphic liposarcoma which is non-reactive for MDM2 immunostain.
Clinical Discussion:
Unlike other liposarcomas, myxoid pleomorphic liposarcoma occurs in children, commonly in the chest. CT scan is the preferred imaging modality. Treatment is by complete excision where possible. Molecular studies like Fluorescent in-situ Hybridization (FISH) and Immunohistochemistry (IHC) is used for confirmation. It has a high propensity to metastasize and recurrence is expected. Chemotherapy and irradiation following complete resection decrease the disease recurrence.
Conclusion:
Soft tissue malignancy must be considered in the differential diagnosis of a large intrathoracic tumor in an infant. FISH and IHC are essential for confirmation.
