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A Case of Retroperitoneal Malignant Triton Tumor in a Nigerian Boy.

Authors :
Ugwu JO
Onwukamuche ME
Ekwunife HO
C Emejulu JK
Modekwe V
Osuigwe OA
Source :
Nigerian journal of surgery : official publication of the Nigerian Surgical Research Society [Niger J Surg] 2017 Jul-Dec; Vol. 23 (2), pp. 141-144.
Publication Year :
2017

Abstract

Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%-10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting in a 12-year-old Nigerian child who was brought with bilateral lower limb weaknesses, weight loss, and a right lumbar mass. There were multiple café au lait spots on the body. Abdominal computerized tomographic scan revealed a huge right retroperitoneal mass crossing the midline, compressing adjacent structures with multilevel intraspinal extensions. Core needle biopsy performed and both histology and immunohistochemical studies confirmed the diagnosis, but patient demised in the course of care. The aim is to heighten suspicion of this extremely very rare malignant tumor in children with NF-1.<br />Competing Interests: There are no conflicts of interest.

Details

Language :
English
ISSN :
1117-6806
Volume :
23
Issue :
2
Database :
MEDLINE
Journal :
Nigerian journal of surgery : official publication of the Nigerian Surgical Research Society
Publication Type :
Academic Journal
Accession number :
29089742
Full Text :
https://doi.org/10.4103/njs.NJS_57_16