Malignant Peripheral Nerve Sheath Tumour Associated With Von Recklinghausen\'s Disease: Case Report

Legbo, JN; Shehu, BB; Malami, SA

doi:10.4314/eamj.v82i1.9294

Cited by 5 publications

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“…[ 6 9 10 ] Among the scanty reports of peripheral nerve sheath tumors from Sub-Saharan Africa, none was specified as MTTs. [ 11 12 13 ] Retroperitoneal triton tumors are even rarer and <10 have been reported in literature so far out of which only one was in a child. [ 6 7 ]…”

Section: Discussionmentioning

confidence: 99%

A case of retroperitoneal malignant triton tumor in a Nigerian boy

et al. 2017

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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.

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Section: Discussionmentioning

confidence: 99%

A case of retroperitoneal malignant triton tumor in a Nigerian boy

et al. 2017

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“…The rarity of MPNSTs in the sub-Saharan population is attested to by the few reports and low numbers from the region [12–15]. Odebode et al in a clinicopathological review covering 22 years, found 98 patients with nerve sheath tumors, of whom 3.1% had PMNSTs [13].…”

Section: Discussionmentioning

confidence: 99%

“…Limb-sparing excision with chemoradiotherapy in combination or separately is increasingly favored. The role of chemotherapy for MPNSTs is not yet defined, although a favorable response in both children and adults has been reported [1, 7, 12, 22]. Local recurrence of MPNST is high, even with multimodal therapy, reported to be between 30% to 65% [27, 28].…”

Section: Discussionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

Nthumba

Juma

2011

ISRN Surgery

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Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 revealed 333 nerve sheath tumors, of which 31 were MPNSTs. Four representative case reports are presented. Discussion. MNPSTs have rarely been reported from sub-Saharan Africa; in this study, they constituted 9.3% of all nerve sheath tumors. The trunk (42%) and limbs (45%) were the most frequently affected anatomical sites. Late presentation of malignant lesions in this environment is exemplified by the four case presentations patients. Conclusions. This report confirms observations from studies on MPNSTs from other environments. Anatomically centrally located MPNSTs may have a higher incidence in sub-Saharan Africa than in the West. Because NF1-associated MPNSTs are difficult to diagnose clinically, and because surgery is the only mode of therapy that offers a complete cure, a lifetime follow-up is important, as this would enable diagnosis of early lesions amenable to surgical extirpation.

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“…About one in ten cases are associated with irradiation [6]. Tumor is usually found in the lower extremities, but one-ninth of all lesions occur in the head and neck region, usually associated with the large cranial nerves.…”

Section: Discussionmentioning

confidence: 99%

10.4021/jocmr2009.09.1264

et al. 2009

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Malignant schwannoma of the anterior abdominal wall nerves is extremely rare. Malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas and it is found in 4% of patients with neurofibromatosis 1. We present a case of malignant schwannoma in a 28-year-old female patient with neurofibromatosis 1. She presented with a painful mass in the right upper quadrant of her abdomen. The tumor location was in the abdominal wall in explorative laparatomy and malignant schwannoma was diagnosed in pathologic assessment. The tumor recurred in 3 months and computed tomography showed two masses in the right side of abdominopelvic cavity. Thereafter, second complete surgical resection was performed and pathologic finding was the same. In spite of administering chemotherapy after second surgery,the tumor recurred and magnetic resonance imaging finding showed a huge heterogeneously enhancing mass with adhesion to the inner side of the abdominal wall. The patient died because of acute respiratory failure due to multiple bilateral pulmonary metastases. Tumor location and rapid recurrence was unique in our patient.KeywordsMalignant peripheral nerve sheath tumor; Malignant schwannoma; Abdominal wall

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Malignant Peripheral Nerve Sheath Tumour Associated With Von Recklinghausen\'s Disease: Case Report

Cited by 5 publications

References 0 publications

A case of retroperitoneal malignant triton tumor in a Nigerian boy

A case of retroperitoneal malignant triton tumor in a Nigerian boy

Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

10.4021/jocmr2009.09.1264

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