| Title: |
Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1 |
| Authors: |
Sanjeev Sandrasecra; Senthil Vasan; Fitjerald Henry; Salmi Abdullah; Mohd Nizam Md Hashim; Siti Rahmah Hashim Isa Merican; Michael Pak-Kai Wong |
| Source: |
Oman Medical Journal, Vol 38, Iss 1, Pp e471-e471 (2023) |
| Publisher Information: |
Oman Medical Specialty Board |
| Publication Year: |
2023 |
| Collection: |
Directory of Open Access Journals: DOAJ Articles |
| Subject Terms: |
neurofibromatosis 1; sarcoma; soft tissue neoplasms; perineum; Medicine |
| Description: |
Soft tissue neoplasm is common among patients with neurofibromatosis type 1 (NF-1). We present a case of a middle-aged woman with NF-1 who presented with a painless, insidiously increasing perineal mass for the past eight months. She underwent colonoscopy, computed tomography staging, magnetic resonance imaging of the pelvis, core-needle biopsy, and later wide local excision of left perineum swelling. Histopathological examination showed a high-grade spindle cell sarcoma, which is a rare association among NF-1. Spindle cell sarcoma is a group of malignant soft tissue tumors with locally destructive growth with spindle-shaped neoplastic cells. The solitary spindle cell sarcoma of the perineum is rare, and wide local resection with a clear margin is the only treatment at present—the scarcely available evidence limiting the use of adjuvant chemotherapy, immunotherapy, and radiotherapy. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
https://omjournal.org/articleDetails.aspx?coType=1&aId=3325; https://doaj.org/toc/1999-768X; https://doaj.org/toc/2070-5204; https://doaj.org/article/f9c93173f43646038100a5586c943d6a |
| DOI: |
10.5001/omj.2023.08 |
| Availability: |
https://doi.org/10.5001/omj.2023.08; https://doaj.org/article/f9c93173f43646038100a5586c943d6a |
| Accession Number: |
edsbas.CCF2EC89 |
| Database: |
BASE |