Neurofibroma originated from the intrathoracic vagal nerve in a patient with von Recklinghausen's disease--a case report

A neurofibroma originated from the intrathoracic vagal nerve was presented. A 26-year-old male was found to have an abnormal shadow in the right chest filed on a routine examination. He was complicated with Von Recklinghausen's disease and showed multiple "cafe au lait" spots over his extremities and trunk but no subcutaneous mass. A block resection of the mass was carried for the mediastinal tumor. The tumor was arising from the right intrathoracic vagal nerve which located just below the origin of recurrent nerve and was measured 3 X 3 X 6 cm. Histological examination of the specimen revealed neurofibroma. His postoperative course was uneventful. A brief review in Japan was made on 22 cases collected from the literature.     

A case of mediastinal vagal nerve neurinoma: a familial neurinomatosis]

We experienced an operation of a 16-year-old female of mediastinal vagal neurinoma. Abnormal shadow was pointed out on chest X-ray at physical examination of high school. Right thoracotomy was performed under the diagnosis of mediastinal tumor. There was a walnut sized mass on the right vagal nerve at just peripheral side of the recurrent nerve branch. The vagal nerve was cut at both sides of the mass not to injure the recurrent nerve. The tumor was removed en block. Two little finger sized masses were recognized in serratus anterior muscle and 5 th. intercostal nerve. These removed masses were diagnosed as neurinoma pathologically. While the mother of this case had been admitted on another hospital because of multiple neurinomatosis with bilateral acoustic neurinoma. Thus this case is of familial neurinomatosis, suggesting it's heredity like as neurofibromatosis (von Recklinghausen's disease).     

Forme fruste of von Recklinghausen's disease: unilateral association of an orbital neurofibroma, a trigeminal neurinoma, and an acoustic neurinoma. Case Report

A patient who presented with a tumor of the left ambient cistern, a left cerebellopontine angle tumor, and a left orbital tumor causing left hearing loss and left exophthalmos without café au lait spots or cutaneous neurofibromas is described. There was no family history of von Recklinghausen's disease. A cerebellopontine angle tumor removed by a suboccipital craniectomy was an acoustic neurinoma. An ambient cistern tumor was approached through a subtemporal route. A tumor arising from the trigeminal nerve was also a neurinoma. An orbital neurofibroma was excised by a frontal craniotomy with removal of the orbital roof. This rare unilateral association of neurinomas and a neurofibroma on the left side was thought to be a forme fruste of von Recklinghausen's disease, and it could be considered a presentation of a mosaic of von Recklinghausen's disease.     

Malignant schwannoma of the intrathoracic vagus nerve: Report of a case

We report herein the case of a 48-year-old man with malignant schwannoma of the intrathoracic vagus nerve associated with von Recklinghausen's disease. Malignant intrathoracic vagal tumors are extremely rare and to our knowledge, only four other cases have been documented in the literature, none of which were associated with von Recklinghausen's disease.     

Intrathoracic vagal nerve schwannoma preoperative diagnosis is correct because of clinical and characteristic ct findings —A case report and review of the literature—

A 60-year-old man was operated with the clinical diagnosis of intrathoracic vagal nerve schwannoma because of characteristic CT findings and no symptom of neurofibromatosis. Thoracoscopic surgery was performed and it was confirmed pathologically. Intrathoracic vagal nerve tumor is rare, so preoperative diagnosis seems to be difficult. We review the intrathoracic vagal nerve tumors reported in Japan (52 cases of schwannoma and 9 cases of neurofibroma), and analyzed the tumor location, furthermore, the relationship of neurofibromatosis and schwannoma and neurofibroma on vagal nerve tumor.     

Solitary retroperitoneal neurofibroma: a case report

A solitary neurofibroma arising in the retroperitoneal space without any other stagma of von Recklinghausen's disease is reported. Confusion with another nerve sheath tumor, a schwannoma is a diagnostic pitfall. Histochemical and immunohistochemical stainings of the tumor are useful for the diagnosis of solitary neurofibroma.     

A rapidly growing benign intrathoracic neurofibroma after lung lobectomy.

A 67-year-old male underwent a right upper lung lobectomy for lung cancer in January 1993. Follow-up chest X-rays revealed a progressive and rapidly growing intrathoracic mass in the right thorax. The mass, however, did not resemble a tumor recurrence, and the patient complained only of shortness of breath. Computerized tomography and magnetic resonance imaging confirmed the presence of the intrathoracic mass and its associated compression of the residual lung. A right thoracotomy was performed in January 1998, and a mass found arising from the sympathetic nerve trunk was resected. Microscopic examination revealed stellate or spindle-shaped cells in myxoid stroma with sparsely distributed collagen fibers. Immunohistochemically, the cells were positive for neuron-specific enolase, and the tumor was identified as neurofibroma. The patient did not suffer from von Recklinghausen's disease, and there was no family history of the disease. After resection of the neurofibroma, the compressed lung was able to re-expand, and the patient's shortness of breath disappeared. At one year postoperative, the patient remains well, and there is no evidence of recurrence.     

Cervicothoracic vagal neurilemoma: Report of a case

We report herein the rare case of a 45-year-old man with a cervicomediastinal neurilemoma of the vagus nerve. The tumor was 160×40×35 mm in size and extended from the angle of the right mandible to the aortic arch. Despite this being the largest such tumor ever reported, the patient presented without any symptoms. Thus, although vagal neurilemoma is uncommon, it should nevertheless be included in the differential diagnosis of any asymptomatic mass along the vagus nerve. In the evaluation of such masses, magnetic resonance imaging can provide useful information regarding not only the location, but also the nature of the lesion.     

Presacral solitary giant neurofibroma without neurofibromatosis type 1 presenting as pelvic mass--case report.

A 35-year-old woman presented with a solitary neurofibroma in an unusual presacral location without neurofibromatosis manifesting as bilateral chronic sciatica for 2 years. She was initially considered as having a giant right ovarian mass, but was referred with a prediagnosis of solitary giant sacral nerve sheath tumor. The initial differential diagnosis was based on neuroimaging. A right-sided J incision with the extraperitoneal approach provided good exposure and handling of the tumor bed. Almost total excision without neurological deficit was possible. The histological diagnosis was neurofibroma. Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease are quite rare. Intrapelvic tumors are often diagnosed at a later stage. Neuroimaging is very helpful to delineate this unusual site and the extent of tumor development, and to determine the appropriate surgical intervention. A clear understanding of retroperitoneal anatomy is essential for safe removal of such tumors. Complete resection is preferred to prevent local recurrence and malignant transformation. Although root section is inevitable, neurological deficit is unlikely.     

Giant neurofibroma arising from the intercostal nerve: a rare case report

Neurofibroma is a benign nerve sheath tumor of the peripheral nervous system. A young male presented with complaints of gradually increasing breathlessness and heaviness on the right side of the chest, and percussion notes over the chest were impaired all over the right lung fields except the basal zone. Contrast-enhanced computed tomography scan of the thorax revealed a large heterogeneous mass occupying most of the right hemithorax. Intraoperatively, the huge mass measuring about 18 cm?×?13 cm?×?12 cm was seemed to arise from the right fourth intercostal space. Histopathology revealed a neurofibroma. Giant intrathoracic neurofibroma is very uncommon and its malignant potential should be evaluated.     

Isolated neurofibroma of the stomach. Case report and review of the literature]

An uncommon case of gastric neurofibroma is described: it was an incidental finding during assessment for abdominal pain, possibly due to pancreatitis, in a 58 year old man, with no sign of von Recklinghausen's disease. The generic diagnosis of gastric wall neoplasia was made by CT scanning; the neoplasm was resected with wedge resection of gastric wall. Histological and ultrastructural examination revealed a neurofibroma. Gastrointestinal stromal tumors are rare occurrence and usually are of smooth muscle derivation: a small percentage arises from nerve sheet, but such a distinction is never sharp. Neurogenic gastric tumors are usually benign and only 10% of von Recklinghausen associated neurofibromas can undergo malignant transformation. Wide excision of the tumor appears therefore the treatment of choice.     

A case of mediastinal schwannoma originating from the intrathoracic vagal nerve

A 46-years-old man was admitted because of an abnormal shadow on X-ray. The operation underwent under the diagnosis of benign tumor in the antero-superior mediastinum. At the operation, the tumor arise from the right intrathoracic vagal nerve and it was resected with the transection of vagal nerve. The histological diagnosis was schwannoma. Postoperative course was uneventful except the slight hoarseness. Intrathoracic vagal tumor is rare and we have found 19 reported cases among Japanese literatures. This case is considered to be the 20th case originated from the intrathoracic vagal nerve in Japan.     

Symmetric dumbbell ganglioneuromas of bilateral C2 and C3 roots with intradural extension associated with von Recklinghausen's disease: case report

BACKGROUND: Ganglioneuromas are rare benign tumors arising most commonly from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in association with von Recklinghausen's disease are rare. CASE DESCRIPTION: A 35-year-old man with von Recklinghausen's disease presented with tetraparesis and respiratory dysfunction. Preoperative neuroimaging revealed an intradural mass extending from the foramen magnum to the C4 vertebral level, as well as bilateral extravertebral extension connecting it with bilateral paraspinal lesions in a dumbbell fashion. Four intradural tumors associated with the bilateral C2 and C3 nerves and located ventrally were removed, leaving the intraforaminal and extradural portion intact. The procedure resulted in postoperative symptomatic improvement. Second, extravertebral tumors of the left neck, which were not related to the cervical sympathetic nerve, were removed. The pathologic diagnosis of the tumors of both the intradural space and cervical neck was ganglioneuroma. CONCLUSION: We present an extremely rare case in an adult with von Recklinghausen's disease who had bilateral, symmetric and multiple dumbbell ganglioneuromas with intradural extension, and also multiple bilateral ganglioneuromas at the neck. The intradural ganglioneuromas were suspected to have originated from the posterior root ganglions of the bilateral C2 and C3 nerves and to have extended ventrally to the spinal cord involving not only sensory but also motor rootlets; the ganglioneuroma of the neck was suspected to have originated from the cervical nerve itself.     

Rupture of the flexor pollicis longus in von Recklinghausen''s disease

An unusual cause of inability to flex the interphalangeal joint of the thumb in a patient with von Recklinghausen's disease is reported. A tumor in the cubital fossa and the basic characteristics of this disease led to a preoperative misdiagnosis of anterior interosseous nerve paralysis. However, surgical exploration and histological examination showed that the correct diagnosis was rupture of the flexor pollicis longus tendon caused by intratendinous proliferation of a neurofibroma. This is the first report in the English-language literature of a flexor tendon rupture caused by intratendinous tumor proliferation.     

Multiple schwannoma of the intrathoracic vagal nerve; report of a case

A 67-year-old male referred to our hospital for the close examination of abnormal chest X-ray findings. Chest X-ray films showed abnormal shadows in the right lung field. Chest computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed the 3 tumors which located in superior, middle and posterior mediastinum. The operation underwent under the diagnosis of neurogenic tumors originating from the right intrathoracic vagal nerve. At the operation, the tumors existed the vagal nerve distal to the recurrent laryngeal nerve and they were resected with the transection of vagal nerve. The masses were histologically diagnosed as schwannoma. We reported a rare case of multiple schwannoma arising from the intrathoracic vagal nerve. He had no postoperative complications, and he has been free from recurrence for 4 years postoperatively.     

Lung metastasis of malignant peripheral nerve sheath tumor: report of a case

A 37-year-old man with von Recklinghausen's disease admitted to our hospital because of chest abnormal shadow. He had underwent extended radical tumorectomy for malignant peripheral nerve sheath tumor (MPNST) in left lower limb 33 months before. Chest X-ray and computed tomography (CT) scan revealed solitary tumor on right S10. Tumor was resected under thoracoscopic surgery. Histological diagnosis was metastasis of MPNST. MPNST with lung metastasis showing very poor prognosis. The patient is doing well 2 years after pulmonary resection without recurrence. Careful follow up is important for MPNST.     

Neurofibroma of the bladder wall in von Recklinghausen''s disease

Neurofibromatosis, or von Recklinghausen's disease, is an autosomal dominant disease with multiple neurofibroma and café-au-lait spots. However, neurofibroma in the bladder wall is a rare condition in von Recklinghausen's disease. A 31-year-old man with neurogenic voiding dysfunction due to sacral meningocele and acute urinary retention with neurofibroma of the bladder wall is presented with detailed radiologic evaluation. Patients with von Recklinghausen's disease should be carefully evaluated if urological symptoms exist.     

Solitary neurofibroma of the spermatic cord

Neurofibromas, which arise from perineural and Schwann cells, commonly occur throughout the body, but they rarely have been reported to originate from the spermatic cord. The solitary neurofibroma is a localized tumour that, by definition, occurs in patients who do not have von Recklinghausen's disease. Its exact incidence is unknown because of the difficulty in excluding von Recklinghausen's disease in some cases. Leiomyomas, lipomas, fibromas, haemangiomas, and epidermoid cysts have been described as benign tumours of the scrotum, but rarely has an extratesticular solitary neurofibroma of the spermatic cord been reported. We report a case of a solitary neurofibroma arising from the left spermatic cord. No signs of von Recklinghausen's disease were identified.     

Pacinian neurofibroma of the hand: a case report and literature review

Pacinian neurofibroma is a rare, benign tumor not associated with von Recklinghausen's disease (neurofibromatosis). Histologically, it is composed of a proliferation of structures resembling normal pressure receptors. A case of pacinian neurofibroma of the hand in a 4-year-old child is presented, along with a review of the literature.     

Neurogenic tumors of the brachial plexus: report of two cases.

Two cases of solitary neurogenic tumors of the brachial plexus unassociated with von Recklinghausen's disease are presented. One patient had a malignant schwannoma. The lesion of the other patient was benign and was diagnosed pathologically as a plexiform neurofibroma. These uncommon neurogenic tumors of the brachial plexus unassociated with von Recklinghausen's disease pose diagnostic and surgical problems. The initial clinical presentation is usually that of a painless supraclavicular mass. At the time of surgical exploration, the exact site or nerve of origin cannot always be identified. If motor loss is caused by such a tumor of the brachial plexus, it usually indicates a malignant lesion and a poor prognosis. Although wide radical excision of a malignant neurogenic tumor is indicated surgically, one of our patients had an early malignant recurrence that necessitated immediate amputation.