Case of jugular foramen schwannoma associated with tuberous sclerosis

We report a case of jugular foramen schwannoma associated with tuberous sclerosis. A 28-year-old female with tuberous sclerosis presented to Hikone Municipal Hospital with subacute onset of somnolence, ataxic gait and frequent vomiting. Her tuberous sclerosis was diagnosed at the age of 9, when she developed hydrocephalus caused by subependymal giant cell astrocytoma near the foramen of Monro, which was treated by surgical resection and VP shunt placement followed by radiation and chemotherapy. Brain MR images revealed a 3 cm enhancing mass extending from the left jugular foramen to the cerebellopontine cistern, accompanied by perifocal edema of the brain stem and cerebellar hemisphere. The tumor was partially removed via suboccipital craniotomy and histologically diagnosed as a schwannoma. After surgery her symptoms promptly improved and 4 months later she underwent additional stereotactic radiosurgery. No tumor regrowth has been observed at 94 months. Intracranial schwannoma complicating tuberous sclerosis is very rare. Only one other case has been reported in the literature.     

Retro-tympanic pulsatile mass originating from dumb-bell jugular foramen schwannoma

Summary Background. Jugular foramen (JF) tumours are uncommon with paraganglioma, schwannoma and meningioma occurring most commonly in this location. JF schwannoma with extension to the retro-tympanic area has been described only once. Methods. 20-year-old man presented with headache, blurred vision, vomiting and diplopia. Findings. A left pulsatile retro-tympanic mass was seen at otoscopy. A jugular foramen tumour was found on CT and MR images. The intracranial portion of the tumour later diagnosed as schwannoma was removed. Control ENT examination confirmed that the residual retro-tympanic mass was no-longer pulsatile. Conclusions. Jugular foramen schwannomas may also extend into the retro-tympanic area. Correspondence: Bulent Bakar, M.D., Department of Neurosurgery, MESA Hospital, Yasam Caddesi No: 5, 06510 Sogutozu, Ankara, Turkey     

Glioma of the jugular foramen. Case report.

A case is presented of a jugular foramen syndrome caused by an ectopic glioma. Treatment was by intracapsular removal through a suboccipital craniectomy.     

Twelve cases of jugular foramen neurinoma

Twelve patients with jugular foramen neurinoma were operated on at our clinic between 1974 and 1990. The initial signs and symptoms were variable; dysfunctions of the 7th, 8th, 9th, 10th, and 11th nerves were frequently observed. Among these, involvement of the eighth nerve was most frequent, and three patients were given a misdiagnosis of acoustic neurinoma. Computed tomography scan and magnetic resonance imaging were useful not only for the correct diagnosis, but also for planning the surgical treatment and postoperative followup. Surgical resection was accomplished with four different approaches: (1) suboccipital approach without opening the jugular foramen, (2) suboccipital approach with opening the jugular foramen, (3) suboccipital approach with opening the jugular foramen combined with infralabyrinthine approach, (4) infralabyrinthine approach. The surgical approach depended on the presence of intracranial tumor and on the extent of extracranial involvement. There was no operative mortality. Dysfunction of the 8th, 9th, 10th, or 11th nerve did not improve in any patient after tumor removal. In contrast, 12th nerve palsy improved in two of three patients after tumor removal. The nerve of origin was identified in five cases; those were from the ninth nerve in three and from the 11th nerve in two.     

Angioma of the jugular foramen case report

Summary We describe a patient with a neoplasm closely mimicking a glumous jugulare tumour with regard to both clinical and radiological presentation. The tumour was removed by the transmastoid route. Only after histological examination of the excised neoplasm could the diagnosis of angioma be made. No other case of this nature has been found in the literature.     

Amyloidomas in the cerebellopontine angle and jugular foramen. Case report

The 47-year-old man reported here showed large encapsulated masses in the left cerebellopontine angle and 6 years later in the enlarged left jugular foramen. Histologically, the tumors demonstrated a large deposit of amyloid composed of immunoglobulin light chain-derived proteins (AL). There was no evidence of chronic inflammatory or infectious processes or immunoglobulin abnormalities.     

Meningiomas of the jugular foramen.

Meningiomas of the jugular foramen manifest the same signs and symptoms as glomus jugulare tumors. They arise from arachnoid cells lining the jugular bulb and grow slowly, infiltrating the temporal bone and posterior fossa. These lesions, however, are more clinically treacherous than glomus tumors. Meningiomas infiltrate surrounding bone and nerve tissue and require wide margins of resection to prevent recurrence. Eight of these lesions have been managed in the past 5 years at the House Ear Clinic using modern imaging and skull base techniques. Two have recurred after "total" microsurgical removal. Presentation, radiologic evaluation, and management guidelines are reviewed.     

Microsurgical anatomy of the jugular foramen.

The authors conducted an autopsy study of 50 jugular foramina and surrounding tissue, using the dissecting microscope. Anatomical findings from this study are presented.     

Stereotactic radiosurgery for jugular foramen schwannomas.

BACKGROUND: Jugular foramen schwannomas pose difficult management problems because of the surgical risk of lower cranial neuropathy. The indications and results of stereotactic radiosurgery are not well documented. METHODS: We reviewed our 10-year experience in the management of 17 patients who had jugular foramen schwannomas managed with the gamma knife. Thirteen patients previously had undergone surgery (range, 1-6 resections). Four patients had multiple cranial nerve deficits before microsurgical resection; 12 developed multiple lower cranial nerve palsies after resection. Four patients underwent radiosurgery based on imaging criteria alone. Conformal dose planning (tumor margin dose of 12-18 Gy) successfully encompassed the irregular tumor volumes in all patients. RESULTS: Follow-up varied from 6 to 74 months. Tumor size decreased in eight patients, remained stable in eight, and increased in one patient during the average follow-up interval of 3.5 years. Six patients improved and 10 others retained their preradiosurgery clinical status. One patient had an increase in tumor size and clinical deterioration 6 months after radiosurgery and underwent microsurgical resection. No patient developed new cranial nerve or other neurological deficits after radiosurgery. CONCLUSIONS: We believe that gamma knife radiosurgery is an effective alternative to microsurgical resection for patients who have small tumors and intact lower cranial nerve function. It is also effective for patients who have residual or recurrent tumors after microsurgical resection.     

Angiomatous type of jugular foramen meningioma with neck extension: differential diagnosis from paraganglioma and schwannoma

BACKGROUND: Meningiomas involving the jugular foramen and parapharyngeal space are extremely rare. They most commonly occur intracranially and then extend to the extracranial region through the foramen of the skull base, such as jugular foramen. Clinically, these tumors mimic the more common glomus jugulare tumor. Preoperative diagnosis can be correct on the basis of the characteristic imaging findings. METHODS: A 52-year-old woman was seen with a left neck mass and mixed-type hearing loss. She underwent physical examination, MRI, high-resolution CT, and angiography. RESULTS: Physical examination revealed a retro-tympanic, pulsatile red mass in the left ear, and mild bulging of the left oropharyngeal wall. The patient was found to have the spreading, carpet-like, meningioma with extracranial extensions via jugular foramen to parapharyngeal space. Preoperative imaging strategy allowing accurate preoperative diagnosis is discussed. CONCLUSIONS: Accurate distinction between meningioma and glomus tumor or schwannoma is possible in most cases, with attention to fine radiologic detail.     

Peripheral primitive neuroectodermal tumor of the jugular foramen: case report

OBJECTIVE AND IMPORTANCE: Peripheral primitive neuroectodermal tumor (pPNET) is a rare type of tumor, most commonly found in the limbs of children and young adults. The authors report an extremely rare case of pPNET located at the jugular foramen whose clinical course demonstrated rapid progression. CLINICAL PRESENTATION: A 23-year-old man presented with a 2-month history of progressive hoarseness and dysphagia. The neuroradiological appearance of the lesion was a jugular foramen tumor. INTERVENTION: The patient underwent a partial resection of the tumor through a far lateral suboccipital craniotomy. After surgery, the patient experienced an unexpected deterioration in consciousness. Magnetic resonance images on postoperative Day 18 revealed rapid and large expansion of the residual tumor into the posterior fossa. No adjuvant therapy was administered, and the patient died 6 weeks after diagnosis. CONCLUSION: The pathological diagnosis of the surgical specimen was pPNET, according to the findings of hematoxylin and eosin and immunohistochemical stainings. To the best of our knowledge, this is the first reported case of pPNET at the jugular foramen.     

Meningiomas of the jugular foramen

Primary meningiomas of the jugular foramen are extremely rare. There is controversy regarding the management of these tumors. The objective of this article is to analyze the treatment and results in a series of ten cases. From a series of 107 patients that had been operated on for jugular foramen tumors between 1987 and 2005, ten had meningiomas. The clinical, histological and imaging findings, as well as the outcome of these cases, were reviewed. A high incidence of malignant or aggressive tumors (six cases) was found. Gross total resection (Simpson I and II) was achieved in five patients. Four patients with meningotheliomatous meningiomas are alive, with a mean follow-up time of 71.8 months (6.5 years). Two patients (one with anaplastic type and one with papillary type) died in the immediate postoperative period. Four patients (two with papillary type, one with microcystic type and one with anaplastic type) died because of disease progression, with a mean survival time of 35 months. Radical removal of benign jugular foramen meningiomas is possible. The incidence of postoperative deficit of cranial nerves is higher than in other benign tumors of the jugular foramen. A high incidence of aggressive (malignant) tumors was observed in this series.     

颈静脉孔神经鞘瘤的显微外科治疗

目的　研究颈静脉孔神经鞘瘤的手术切除效果。　方法　回顾性分析采用显微外科手术治疗颈静脉孔神经鞘瘤２０ 例,其中 Ａ 型肿瘤位于桥小脑角１２ 例; Ｂ型肿瘤原发于颈静脉孔,向颅内生长３ 例,采用乳突后枕下或后正中钩型切口开颅; Ｃ 型肿瘤位于颅外,并向颈静脉孔生长１ 例, Ｄ 型肿瘤由颈静脉孔向颅内外生长,呈哑铃型４ 例,取颈乳突联合入路。　结果　肿瘤全切除１１ 例,次全切除９ 例次,大部切除５ 例次,无手术死亡率。　结论　根据肿瘤生长方式选择良好显露肿瘤的手术入路是切除肿瘤的关键,而防止脑神经损伤是切除肿瘤的重要环节     

Surgical outcomes of lateral approach for jugular foramen schwannoma: postoperative facial nerve and lower cranial nerve functions

The lateral surgical approach to jugular foramen schwannomas (JFS) may result in complications such as temporary facial nerve palsy (FNP) and hearing loss due to the complicated anatomical location. Ten patients with JFS surgically treated by variable methods of lateral approach were retrospectively reviewed with emphasis on surgical methods, postoperative FNP, and lower cranial nerve status. Gross total removal of the tumors was achieved in eight patients. Facial nerves were rerouted at the first genu (1G) in six patients and at the second genu in four patients. FNP of House–Brackmann (HB) grade III or worse developed immediately postoperatively in six patients regardless of the extent of rerouting. The FNP of HB grade III persisted for more than a year in one patient managed with rerouting at 1G. Among the lower cranial nerves, the vagus nerve was most frequently paralyzed preoperatively and lower cranial nerve palsies were newly developed in two patients. The methods of the surgical approach to JFS can be modified depending on the size and location of tumors to reduce injury of the facial nerve and loss of hearing. Careful manipulation and caution are also required for short facial nerve rerouting as well as for long rerouting to avoid immediately postoperative FNP.     

胃神经鞘瘤:附9例报告

目的 分析胃神经鞘瘤的临床特点、诊断和治疗方法。方法 回顾分析笔者近15年来收治的9例胃神经鞘瘤的临床资料。结果 临床主要表现是上腹部隐痛(9例)、腹部肿块(5例)和上消化道出血(3例)。9例均手术治疗，但无1例术前确诊。1例恶性胃神经鞘瘤术后只生存了8个月，良性的手术效果良好。结论 胃神经鞘瘤无特异性临床表现，术前确诊困难，误诊率高，一经确诊，应及早手术治疗。