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Scalp Plexiform Neurofibrosarcoma With Intrathoracic Fibrosarcoma: A Case Report
Titus O Chukwuanukwu1, Alvan-Emeka K Ukachukwu2, Kenneth C Etukokwu3
1Plastic and Reconstructive Surgery Unit, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, NGA.
Cureus
|September 16, 2022
Summary
Neurofibromatosis type 1 (NF1) can manifest as rare, aggressive fibrosarcomas in multiple locations. This case highlights a scalp neurofibrosarcoma and a fatal intrathoracic fibrosarcoma in an NF1 patient.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- Neurofibromatosis type 1 (NF1) is a common genetic disorder.
- NF1 predisposes individuals to various tumors, including neurofibromas and malignant peripheral nerve sheath tumors.
Observation:
- An 18-year-old female with NF1 presented with a long-standing scalp plexiform neurofibromatosis.
- She also had café-au-lait spots and a family history of NF1.
- Histology confirmed a malignant peripheral nerve sheath tumor (neurofibrosarcoma) of the scalp.
Findings:
- The patient subsequently developed an aggressive, infiltrative intrathoracic fibrosarcoma.
- Despite subtotal surgical excision via thoracotomy, the tumor was highly hemorrhagic and recurred.
- The patient unfortunately died following the second surgical intervention.
Implications:
- This case underscores the aggressive nature and potential for multiple, diverse tumors in NF1.
- Early and comprehensive evaluation of all body systems is crucial in NF1 patients.
- Aggressive surgical management for intrathoracic fibrosarcoma has a poor prognosis, necessitating further research into adjuvant therapies.
Keywords:
intra-thoracic metastasesmalignant peripheral nerve sheath tumor (mpnst)neurofibromatosis type 1 (nf1)neurofibrosarcomascalp plexiform neurofibroma
