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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Malignant peripheral nerve sheath tumor of the kidney
Stephen B Williams1, Gregory R Szlyk, Michael J Manyak
1Department of Urology, The George Washington University Medical Center, Washington, DC 20037, USA.
Abstract:
Malignant peripheral nerve sheath tumors are rare in the genitourinary organs, with few reports of occurrence in the kidney. We describe a patient with a renal malignant peripheral nerve sheath tumor, discovered after excision of a malignant peripheral nerve sheath scalp lesion, with additional masses in the lung and shoulder on metastatic evaluation. This patient underwent neoadjuvant intravenous doxorubicin therapy, followed by surgical resection of the scalp, lung and shoulder lesions in addition to a radical nephrectomy.
Insights
Malignant peripheral nerve sheath tumors are rare in the kidney. This case highlights a patient with a renal tumor alongside scalp, lung, and shoulder masses, treated with chemotherapy and surgery.
Area of Science:
- Oncology
- Nephrology
- Surgical Pathology
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon neoplasms.
- Genitourinary involvement, particularly in the kidney, is exceptionally rare for MPNSTs.
Observation:
- A patient presented with a renal MPNST discovered after excision of a scalp MPNST.
- Metastatic evaluation revealed additional MPNSTs in the lung and shoulder.
Findings:
- The patient received neoadjuvant intravenous doxorubicin chemotherapy.
- Surgical resection was performed for scalp, lung, shoulder lesions, and a radical nephrectomy for the renal tumor.
Implications:
- This case expands the understanding of rare renal MPNST occurrences.
- It underscores the importance of thorough metastatic evaluation for MPNSTs.
- Multimodal treatment including chemotherapy and surgery can be effective.
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