Spinal Cord Metastasis of a Non-neurofibromatosis Type-1 Malignant Peripheral Nerve Sheath Tumor: An Unusual Manifestation of a Rare Tumor

Summary Malignant peripheral nerve sheath tumors are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest. They arise from the spinal roots, peripheral nerves, brachial and lumbosacral plexi, cranial nerves and terminal nerve fibers within soft tissue, intestine, lung and bone. These tumors recur either locally, or metastasize distally. Most of these tumors occur in association with neurofibromatosis type 1. Spinal cord metastasis from malignant nerve sheath tumors associated with neurofibromatosis type 1 is very rare. We describe a rare case of near-total spinal cord metastasis in a patient with malignant nerve sheath tumor in the absence of neurofibromatosis, and highlight the microscopic findings and natural history of this disease process.     

The bony crescent sign -a new sign of facial nerve schwannoma

Schwannomas of the facial nerve are rare slowly growing lesions that have a predilection for the geniculate ganglion. Radiological evaluation is important in their diagnosis and in the assessment of their extent. In our series of 4 cases the facial nerve schwannoma was seen on high resolution CT as a soft tissue mass bounded anteriorly by a thin rim of bone. This bony crescent sign is a previously undescribed sign of facial nerve schwannoma which appears on the basis of this small series to be strongly indicative of the presence of this tumour. Schwannomas are relatively uncommon intracranial tumours. They most commonly involve the acoustic nerve followed in frequency by the trigeminal nerve. Other cranial nerves are rarely involved. Facial nerve schwannomas occur ring within the petrous temporal bone are very rare. Their diagnosis may be missed prospectively even when appropriate CT scans are per formed. Even in retrospect the site of abnormality may be difficult to identify, especially if there is an associated middle ear mass such as a cholesteatoma. Lesions occurring in the petrous area are all rare. The differential diagnosis includes cholesterol granuloma, epidermoid, carotid aneurysm and, very rarely, primary and secondary bone tumours. We describe a new sign associated with facial nerve schwannoma on CT, that of a bony crescent. Recognition of this sign makes those tumours arising in the region of the geniculate ganglion easy to diagnose prospectively.     

Epithelioid sarcoma of the median nerve mimicking a peripheral nerve sheath tumour

We describe a case of epithelioid sarcoma of the median nerve in a 57‐year‐old woman presenting with symptoms and signs of carpal tunnel syndrome for 2 years. The clinical examination was suggestive of a wrist ganglion compressing the median nerve. Magnetic resonance imaging (MRI) showed a 5 cm × 3 cm mass involving the median nerve in the carpal tunnel and appearances mimicked a benign peripheral nerve sheath tumour. This report illustrates a rare tumour presenting in a rare location and emphasizes the atypical clinical and MRI features that should alert the radiologist to the possibility of a rare sarcoma mimicking a benign peripheral nerve sheath tumour.     

Facial Nerve Schwannoma Involving Middle Cranial Fossa: When the Unilateral Sensorineural Hearing Loss Guide to the Correct Diagnosis

The Facial Nerve Schwannoma is a rare tumor and it seldom involved the middle cranial fossa. Facial nerve schwannoma has various manifestations, including facial palsy but unfortunately facial nerve is very resistant to compression and often facial nerve paralysis or a facial weakness are not present. We present a case of giant facial nerve schwannoma involved the middle cranial fossa without facial nerve paralysis. In these cases the unilateral hearing loss (if present) guide to a correct diagnosis.     

Multiple Ancient Schwannoma of the accessory nerve: A Case Report

We are reporting a case of multiple ancient schwannoma of the accessory nerve in a 57 year old male. Accessory schwannomas are rare with only 19 cases reported in literature so far. Ancient schwannoma is even rarer in the head and neck region with no other cases associated with the accessory nerve being reported in literature so far. Key words: schwannoma, neurilemomma, ancient, accessory nerve, neck.     

Isolated optic nerve pseudotumour

Isolated optic nerve involvement by the idiopathic inflammatory process is a rare finding and very few reports are available. Here a case of an isolated optic nerve inflammatory pseudotumour presenting with gradually progressive unilateral loss of vision is described. It showed dramatic response to a trial of steroids and its differential diagnoses are discussed.     

Trigeminal nerve root entry zone pilocytic astrocytoma in an adult: a rare case of an extraparenchymal tumor

Extra-axial cerebellopontine angle (CPA) tumors account for approximately 10% of all brain neoplasms in adults. Vestibular schwannomas are the most common, followed by meningiomas. Gliomas in the CPA are rare and quite often are the exophytic extension of primary brain stem or cerebellar tumors. We describe a pilocytic astrocytoma of the CPA that was found to arise from the proximal portion of trigeminal nerve without any anatomic continuity with the brain stem and the cerebellum. Pre-operative MRI suggested a schwannoma. The proposed origin of this extremely rare tumor is the root entry zone of the involved nerve. The tumor was completely resected via a suboccipital retrosigmoid approach.     

Primary tumor of the facial nerve: Diagnosis and management

Benign primary tumors of facial nerve are rare, difficult to diagnose due to their subtle and variable clinical manifestations and these are usually misdiagnosed as idiopathic facial nerve paralysis. A case of facial nerve sehwannoma in internal auditory meatus presenting as a tumor indistinguishable from acoustic neuroma clinically is presented here. Difficalties in patient assessment, inadequacy of diagnostic techniques presently available and surgical technique of the removal of the tumor will be discussed.     

Glomus vagale presenting as a supraclavicular mass: Magnetic resonance imaging findings

Glomus vagale are rare vascular tumours of the paraganglion cells of the vagus nerve, and they usually occur in the carotid space. Tumours can be familial, multicentric, malignant but rarely hormonally active. A rare case is reported of glomus vagale presenting as a supraclavicular mass.     

Fluid–fluid levels in intracranial schwannomas

Intracranial cystic neurogenic tumours constitute an uncommon subset of tumours with a distinct clinico-biological behaviour. The presence of fluid–fluid levels within the tumours, although rare, confirms the cystic nature of the neoplasms. Barring the acoustic schwannomas, cystic cranial nerve schwannomas are exceptionally uncommon. Imaging findings of fluid–fluid levels in two non-acoustic cranial nerve schwannomas are described; one was a cystic trigeminal schwannoma and the other was a glossopharyngeal nerve schwannoma. The causes of the fluid–fluid level and its implications are analysed.     

Metastatic hepatocellular carcinoma presenting as facial nerve palsy and facial pain

Facial nerve palsy due to temporal bone metastasis of hepatocellular carcinoma (HCC) has rarely been reported. We experienced a rare case of temporal bone metastasis of HCC that initially presented as facial nerve palsy and was diagnosed by surgical biopsy. This patient also discovered for the first time that he had chronic hepatitis B and C infections due to this facial nerve palsy. Radiation therapy greatly relieved the facial pain and facial nerve palsy. This report suggests that hepatologists should consider metastatic HCC as a rare but possible cause of new-onset cranial neuropathy in patients with chronic viral hepatitis.     

胃肠神经源性肿瘤临床与病理

胃肠道神经源性肿瘤较为罕见,本文复习了我院普外科1975年7月—1994年7月间收治并经手术后病理证实的胃肠道神经源性肿瘤17例,对其临床病理及免疫组织化学分析进行讨论,认为免疫组化是判别其组织起源的必要手段,手术切除是唯一的治疗方法。     

Epithelioid sarcoma of the sciatic nerve perineural sheath: a mimic of nerve sheath tumor

We report herein a rare case of epithelioid sarcoma, in a 39-year old lady involving the sciatic nerve. Clinically and radiologically it stimulated a nerve sheath tumor. Involvement of a nerve by an epithelioid sarcoma is extremely uncommon. To the best of our knowledge, this is the first case of an epithelioid sarcoma involving the sciatic nerve and needs documentation.     

Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy

Malignant peripheral nerve sheath tumours (MPNST) are a rare variety of soft tissue sarcomas (STS) arising from major peripheral nerve branches and typically located in the lower extremity, chest wall or the retroperitoneum. It is a biologically aggressive neoplasm for which the treatment of choice is surgery, but usually requires a multimodality approach, having been generally labelled as chemoresistant. We present a case of MPNST located intracranially with a good response to chemotherapy.     

Ancient schwannoma masquerading as a thyroid mass.

Schwannomas are benign, encapsulated nerve sheath cell neoplasms. Cervical sympathetic chain (CSC) schwannomas are rare, with less than 50 cited cases in the literature. CSC schwannomas may mimic a number of parapharyngeal masses. We report a rare variant, "ancient" schwannoma, which presented cytologically and radiologically as a thyroid mass. This is the first report of a CSC schwannoma mimicking a thyroid mass and the first report of an ancient schwannoma of the CSC.     

Sinonasal schwannoma with secondary changes

Schwannomas of the Sinonasal tract are very rare, representing less than 4% of schwannomas arising in the head and neck [1]. The most frequent site is the acoustic nerve; other locations include the scalp, oral cavity, pharynx, larynx, trachea, parotid gland, and middle ear. Secondary changes in a Schwannoma are a very rare entity. We report a case of Sinonasal schwannoma with pseudoangiomatous changes. The purpose of this article is to highlight the possibility of unusual presentation of a sinonasal schwannoma     

Right vocal cord palsy following open heart surgery

Injury to right recurrent laryngeal nerve with unilateral vocal cord paralysis is rare after thoracic surgery. The mechanism of injury to right recurrent laryngeal nerve during open heart surgery is discussed and a case is illustrated.     

非返性喉返神经的术前诊断及术中损伤的预防

背景与目的：非返性喉返神经是喉返神经罕见的解剖变异,容易导致甲状腺术中喉返神经的损伤。该研究总结非返性喉返神经的术前诊断和术中损伤预防的经验。方法：回顾性分析11例存在非返性喉返神经患者的临床资料,结合文献,探讨非返性喉返神经的术前诊断方法和术中注意事项。结果：术中证实11例均为1型非返性喉返神经,其中7例术前CT提示存在气管食管后锁骨下动脉,1例术中切断后行端端吻合。结论：非返性喉返神经是罕见的解剖变异,术前CT检查可提前判断非返性喉返神经的存在,术中采用精细化被膜操作技术,可减少神经损伤的发生。     

Progressive loss of vision in patients with high-grade non-Hodgkin's lymphoma

Three cases of double-sided neuritis of the optic nerve in patients with lymphomas are described. Two patients with lymphoblastic lymphoma had no other signs of central nervous system (CNS) relapse. All three cases responded to high doses of corticosteroids and/or radiotherapy, suggesting a lymphomatous cause of the papillitis. Optic nerve involvement is reported to be rare in lymphomas, but may become more prominent with aggressive systemic therapy controlling manifestations outside the CNS. Possible causes of optic neuritis in patients with lymphoma are discussed and therapeutic measures are suggested.     

Management of a Sporadic Malignant Subfrontal Peripheral Nerve Sheath Tumor

Summary Malignant subfrontal (olfactory) peripheral nerve sheath tumors (MPNSTs) are exceedingly rare. Although meningiomas are the most common subfrontal extra-axial lesions, it is important to recognize that MPNSTs, which are radiographically similar to meningiomas, can also be present in this location. MPNSTs require more aggressive surgical and postoperative management than meningiomas. In this paper, we describe a patient with a subfrontal MPNST with unusual histological characteristics and present a review of the literature. A 49-year-old woman presented with chronic sinusitis and progressive headaches. A neurological examination revealed left-sided anosmia. Brain-imaging studies revealed a large left subfrontal mass with extension into the frontal and ethmoid sinuses and the nasal cavity. The patient underwent both a bifrontal transbasal craniotomy and a transnasal approach for an attempt at total resection of both the intradural and extradural components of the MPNST. The patient was treated postoperatively with radiation therapy, and had no evidence of recurrence at her follow-up examination 1-year after treatment. Subfrontal PNSTs are extremely rare and usually benign. The specific cell and nerve of origin for these tumors remains unknown. Our case shows that these rare lesions can present as a malignant variant and thus require aggressive surgical and postoperative management to provide long-term tumor control.