A case of retroperitoneal malignant triton tumor in a Nigerian boy

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.

A Case Report of a Malignant Triton Tumor of the Left Retroperitoneum in a Young Female Without Neurofi bromatosis

“…However, sites such as the viscera, mediastinum, intracranium, and retroperitoneum are quite rare. Currently, there are less than 10 reported cases of retroperitoneal MTT in relevant literature [3,5]. The rarity of this condition and its clinical signifi cance has prompted the reporting of this case.…”

“…While MPNSTs account for only 5%-10% of all soft tissue sarcomas, there exist MPNST variations that occur with greater rarity [2]. Specifi cally, Malignant Triton Tumors (MTT) are a subset of MPNST with rhabdomyoblastic differentiation and account for only 5% of all MPNST cases [3]. MTTs were fi rst described by Mason in 1932 and found to occur in patients with Neurofi bromatosis type 1 (NF-1) in slightly more than 50% of cases.…”

“…MTTs were fi rst described by Mason in 1932 and found to occur in patients with Neurofi bromatosis type 1 (NF-1) in slightly more than 50% of cases. This is because sMTTs are believed to originate from Schwann cells of peripheral nerves or within existing neurofi bromas [3,4]. Diagnosis of MTT is confi rmed by immunohistochemical screening for desmin, vimentin, actin, myoglobulin, and S-100 protein.…”

“…Like MPNST, Malignant Triton Tumors appear most often in sites including the head, neck, extremities, and trunk [3].…”

Scalp Plexiform Neurofibrosarcoma With Intrathoracic Fibrosarcoma: A Case Report

“…It is a benign tumor of peripheral nerves with proliferating neural crest-derived Schwann cells, perineural cells, and endoneural fibroblasts [1,3,4]. Genetically, it arises from the defective NF1 gene on chromosome 17q11.2 whose protein product neurofibromin is a tumor suppressor and mutates spontaneously in 50% of cases [1,[5][6][7][8][9]. NF1 is inherited as autosomal dominant with complete penetrance and is seen in all races and genders [3,[6][7][8].…”