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.
BackgroundTo our knowledge, there is no prior randomized study on the utility of Syferol-IHP (blend of virgin coconut oil and Ocimum sanctum oil) when coadministered with a triple therapy schedule.AimThis study determined the efficacy and safety of Syferol-IHP as adjunct to conventional triple therapy for the treatment of peptic ulcer disease (PUD).MethodsA pilot double-blind randomized trial was conducted in patients with confirmed diagnosis (endoscopy-guided biopsy) of PUD. Eligible patients were randomized to Pylorest (a three-in-one tablet containing rabeprazole 20 mg, amoxicillin 1 g, and clarithromycin 500 mg) and Syferol-IHP for 2 weeks, followed by rabeprazole and Syferol-IHP for 2 weeks or Pylorest and placebo for 2 weeks, followed by rabeprazole and placebo for 2 weeks. Repeat endoscopy-guided biopsy and histology were done 4 weeks posttherapy. Primary outcome measures were the healing of ulcer and eradication of Helicobacter pylori. Secondary outcome measures were the disappearance of epigastric pain, gastritis, and duodenitis. Analysis was by intention-to-treat.ResultsOf the 63 patients enrolled, 60 patients had complete evaluation, with 37 patients receiving Pylorest and Syferol-IHP and 23 patients receiving Pylorest and Placebo. Healing of the PUD in favor of Pylorest and Syferol-IHP was significantly higher for gastric ulcer (RR=0.000, 95% CI=undefined, P=0.048) but not for duodenal ulcer (RR=0.400, 95% CI=0.07–2.37, P=0.241). H. pylori eradication was 100% with Syferol-IHP vs 50% with placebo (P=0.066). Epigastric pain (reduction to 16.2% vs 43.5%; P=0.021), gastritis (reduction to 13.5% vs 39.1%; P = 0.024), and duodenitis (reduction to 0% vs 8.7%; P=0.327) were observed in the Syferol-IHP and Pylorest vs placebo and Pylorest groups, respectively. Adverse events (RR=0.971, 95% CI=0.46–2.04, P=0.937) and laboratory parameters were not significantly different pre- and posttherapies (P>0.05, for both groups).ConclusionAlthough both treatment arms were equally safe, co-administration of Syferol-IHP and triple therapy is more efficacious than triple therapy alone for treating PUD. Pan African Clinical trial registry identifier number is PACTR201606001665364.
Neurofibromatosis type 1 (NF1) is the most common form of neurofibromatosis. It is associated with neurofibromas, gliomas, neurofibrosarcomas, and neuroendocrine and hematopoietic tumors. We present a case of scalp plexiform neurofibromatosis associated with intrathoracic fibrosarcoma.An 18-year-old female presented with a 15-year history of plexiform scalp mass. She had multiple café-aulait patches on her trunk and extremities and a first-degree relative with a plexiform right shoulder mass. She was managed by a multidisciplinary team of plastic and reconstructive surgeons, neurosurgeons, cardiothoracic surgeons, otorhinolaryngologists, ophthalmologists, pulmonologists, and pathologists. The histology of the excised scalp mass was that of a malignant peripheral nerve sheath tumor (neurofibrosarcoma). She subsequently developed upper chest and back pain with associated breathlessness and was found to have an intra-thoracic tumor. She had two sessions of exploratory right thoracotomy with subtotal excision of an aggressive, highly hemorrhagic, infiltrative mucinous tumor. The histology was a fibrosarcoma. The patient died a few hours following the second thoracotomy.NF1 is associated with several tumors, among which are neurofibrosarcomas. Intra-thoracic fibrosarcoma requires aggressive surgical resection; recurrence may be delayed with radiotherapy and chemotherapy. The prognosis is however poor, and survival beyond one year is unusual. Once one tumor is found, other body systems should be evaluated for the possibility of other tumors.
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