Abstract

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.

Keywords

Publication details

DOI
10.5001/omj.2023.08
Journal
Oman Medical Journal
Publisher
Oman Medical Specialty Board
Open access
Gold open access

Cite this article

APA 7

Sandrasecra, S., Vasan, S., Henry, F., Abdullah, S., Hashim, M. N. M., Merican, S. R. H. I., & Wong, M. P. K. (2023). Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1. Oman Medical Journal. https://doi.org/10.5001/omj.2023.08

MLA 9

Sandrasecra, Sanjeev, et al. "Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1." Oman Medical Journal, 2023. https://doi.org/10.5001/omj.2023.08.

Chicago (author–date)

Sandrasecra, Sanjeev, Senthil Vasan, Fitjerald Henry, Salmi Abdullah, Mohd Nizam Md Hashim, Siti Rahmah Hashim Isa Merican, and Michael Pak-Kai Wong. 2023. "Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1." Oman Medical Journal. https://doi.org/10.5001/omj.2023.08.

Harvard

Sandrasecra, S., Vasan, S., Henry, F., Abdullah, S., Hashim, M. N. M., Merican, S. R. H. I. and Wong, M. P. K. (2023) 'Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1', Oman Medical Journal. doi:10.5001/omj.2023.08.

Vancouver

Sandrasecra S, Vasan S, Henry F, Abdullah S, Hashim MNM, Merican SRHI, et al. Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1. Oman Medical Journal. 2023. doi:10.5001/omj.2023.08

IEEE

S. Sandrasecra, S. Vasan, F. Henry, S. Abdullah, M. N. M. Hashim, S. R. H. I. Merican, and M. P. K. Wong, "Perineum Spindle Cell Sarcoma in Neurofibromatosis Type 1," Oman Medical Journal, 2023, doi: 10.5001/omj.2023.08.