Clinicopathological study of neurofibromatosis type 1: an experience in Nigeria

Odebode, TO; Afolayan, Eao; Adigun, IA; Daramola, O O M

doi:10.1111/j.1365-4632.2005.02386.x

Cited by 18 publications

(17 citation statements)

References 9 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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“…Odebode et al in a clinicopathological review covering 22 years, found 98 patients with nerve sheath tumors, of whom 3.1% had PMNSTs [13]. In Samaila and Adewuyi's review of 382 cutaneous malignancies, only three were MPNSTs, representing 0.8% of the tumors [14].…”

Section: Discussionmentioning

confidence: 99%

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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“…The estimated population prevalence is between 1/2,000 and 1/8,000 worldwide although that from certain parts of the developing countries is less well documented [6]. Reports on the pattern of NF1 from Nigeria for instance are limited only to occasional hospital-based case series usually only documenting respective systemic complications of cases in late clinical presentations [18,19].…”

Section: Introductionmentioning

confidence: 99%

Cerebral pleomorphic xanthoastrocytoma associated with NF1: an updated review with a rare atypical case from Africa

et al. 2011

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The occurrence of cerebral pleomorphic xanthoastrocytoma (PXA) in individuals with neurofibromatosis type 1 (NF1) is very rare. We present a 10-year-old Nigerian boy with NF1 who was found to harbor a thalamic-lateral ventricular solid mass lesion whose histologic and immunohistochemical findings were in keeping with PXA. We also carried out an updated review of the PXA-NF1 literature and found only eight previous reports of this clinical disease association. These reports have been limited to only certain regions of the world, with none yet reported from Africa, South America, Australia, and Eastern Europe. As far as we know, this might be the first such report from Africa. The case we present, in addition, demonstrated some other unique clinical, radiological, and histopathologic characteristics which have been highlighted in this review.

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Clinicopathological study of neurofibromatosis type 1: an experience in Nigeria

Abstract: In our setting, NF1 is more common among males, with the highest density of neurofibromas covering the extremities.

Cited by 18 publications

References 9 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

Cerebral pleomorphic xanthoastrocytoma associated with NF1: an updated review with a rare atypical case from Africa

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