Two cases of debulking surgery for lower limb diffuse plexiform neurofibroma with transcatheter arterial embolisation

IntroductionDiffuse plexiform neurofibroma (DPN) in patients with neurofibromatosis type 1 (NF1) causes motility dysfunction in severe cases. Transcatheter arterial embolisation (TAE) is an effective haemorrhage control method in vascular tumour surgery.Presentation of caseWe performed debulking surgery for DPN in the buttock and posterior thigh of two NF1 patients. Preoperative TAE with gelatine particles to tumour feeder vessels was conducted in both cases. Operative bleeding volumes were 500 and 4970 mL, respectively. In the latter case, the resection area extended to the upper poles of the buttocks, and the tumour invaded deeply into the surrounding tissues. Massive haemorrhage occurred, and internal iliac arterial balloon was inflated temporarily to further suppress the bleeding. Delayed wound healing due to TAE occurred; debridement and wound closure were required. Motor function improvement was confirmed in both patients.DiscussionBleeding volumes varied because of highly developed collateral pathways and tumour invasiveness. As the upper pole of the buttock was perfused by the superior gluteal artery and its numerous collateral vessels, complete haemostasis was difficult despite adequate TAE. Because delineating the tumour border from the normal tissue was impossible due to the high tumour invasiveness, cutting into the hypervascular tumour was inevitable. As gelatine particles were absorbed but remained within the vessels, prolonged wound ischaemia and delayed healing occurred.ConclusionAlthough TAE with gelatine particles and balloon occlusion were reliable haemorrhage control methods in debulking surgery for lower limb DPN, optimal haemorrhage control technique, compatible with haemostasis and wound healing, was desired.     

Syndrome‐associated soft tissue tumours

Soft tissue neoplasms may be associated with a variety of genetic disorders and malformation syndromes, especially when they arise in children, adolescents and early adulthood. This review summarizes the principal histopathological types of soft tissue tumours which occur in various syndromes, with an emphasis on pathological features, genetic aspects and considerations for the diagnostic pathologist.     

A rare case report of an solitary neurofibroma in postcricoid region of hypopharynx

Rationale:Postcricoid neurofibroma is an extremely rare hypopharynx tumor that can be challenging in both diagnosis and treatment. This case sheds light on the possibility of treatment with transoral microsurgery before pursuing open cervical incisions.Patient concerns: A 43-year-old man presented with a four months history of a persistent foreign body sensation and mild dysphagia. Indirect and direct laryngoscopy at admission revealed a round and smooth submucosal mass in the postcricoid region.Diagnosis:A laryngeal enhanced computed tomography and laryngoscopy suggested that the tumor located in hypopharynx, with clear boundary and slightly strengthened edge. A supporting laryngoscopy surgery was performed under general anesthesia and a biopsy confirmed solitary neurofibroma of the postcricoid region.Interventions:The tumor was successfully resected en bloc transorally through supporting laryngoscope, and obviated the need for open cervical surgery and tracheostomy.Outcomes:The patient recovered well without any intraoperative or postoperative complication and was discharged from hospital 2 days after surgery. There was no recurrence after 6 months follow-up.Lessons:Postcricoid neurofibroma is an extremely rare hypopharynx tumor that can be diagnostically challenging. To the best of our knowledge, this is the first case reported of solitary neurofibroma originating from the postcricoid region of the hypopharynx and was surgically removed with transoral surgery through supporting laryngoscope.     

Small ileal neurofibroma causing intussusception in a non-neurofibromatosis patient

Neurofibromas in the small intestine are usually accompanied by von Recklinghausen's disease (neurofibromatosis), and usually originate in the intramuscular plexus of Auerbach. We present here a solitary neurofibroma, which caused an ileocolic intussusception, originating in the submucosal plexus of Meissner in a non-neurofibromatosis patient. To our knowledge, there is no previous report of a neurofibroma originating in the plexus of Meissner. This condition was clearly confirmed by macroscopic and microscopic evaluation.     

A case of neurofibroma located in the retroperitoneum involving the uncinate process of the pancreas

A case of solitary neurofibroma in the retroperitoneum involving the uncinate process of the pancreas, which has been reported in only one case in Japan and less than 20 cases in the world literature, is described. A 57-year-old male complained of back pain and microhematuria. Plain and enhanced abdominal computed tomography (CT) indicated an 8 × 6 cm hypovasular tumor located in the uncinate process. We initially assumed it to be a cancer of the pancreas. His physical examination and blood chemistry result were normal. Various diagnostic imaging methods however, disclosed a rare type of tumor. The tumor was resected by pancreaticoduodenectomy and histological examination revealed it to be neurofibroma.     

Localized Neurofibromas in the Bilateral Orbits

Localized neurofibromas are rare in the orbit and, unlike the more common plexiform neurofibromas, are not typically associated with von Recklinghausen neurofibromatosis. We present a rare case of localized neurofibromas in the bilateral orbits.