Juxtadrenal schwannoma

Schwannoma which originated from Schwann cells is a nerve sheath tumour. Schwannomas of the urogenital tract are very rare. We present a case of a juxtadrenal schwannoma in a 66 years old female patient. To date only three juxtadrenal schwannoma cases have been described in the literature.     

Intramedullary schwannoma with extradural extension: case report

Intramedullary schwannoma without evidence of von Recklinghausen's disease is extremely rare. Only 23 cases have been reported in the literature. A 42-years-old woman with transverse type myelopathy at the level of Th 6, was referred to our department. Myelography showed diffuse swelling of the thoracic cord. Delayed myelo CT scan revealed the existence of syrinx at C 6 and Th 10 spinal cord level. On NMR spin echo image, high intensity signal with an ovoid shape was visualized in Th 7-8 spinal cord parenchyma and the syrinx was verified below the level of C 5. Based on these findings, she was diagnosed as having Th 7-8 intramedullary spinal cord tumor. Th 6 to Th 9 laminectomy followed by the complete removal of well encapsulated tumor was performed. The rostral and caudal pole of the tumor was found to be located intramedullary. The tumor at the level of Th 7-8, extended not only to the extramedullary space but also to the extradural space. Histopathological examination revealed that the specimen was Antoni A type neurinoma. She showed good recovery. Following hypotheses have been postulated on the cytogenesis of intramedullary schwannoma in the literature: Central displacement of Schwann cells during embryonic development. Schwann cells ensheathing aberrant intramedullary nerve fibers. Schwann cells along the intramedullary perivascular nerve plexus. A critical area where posterior root loose their sheath on entering the pia mater. Transformation of pial cells of neuroectodermal origin into Schwann cells. The cytogenesis of these reported cases may not be identical.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary holocord ganglioneuroblastoma: case report

The authors present a case of extensive primary intramedullary spinal CNS ganglioneuroblastoma (GNB) in a 23-year-old man. Central nervous system GNB is a poorly differentiated neuroepithelial tumor composed of neuroblasts and differentiated ganglion cells, and these lesions are extremely uncommon. Most previously reported primary intraaxial neuroblastic tumors were described in the brain. There has been only one other report of primary spinal cord CNS GNB published to date; the clinical course and prognosis for primary spinal cord tumors of this type are unknown. Similar tumor types demonstrate poor prognoses. This 23-year-old man presented after 9 months of progressive myelopathy. Admission MR imaging showed an intraaxial enhancing mass extending from C-3 to the conus medullaris, with a holocord appearance in several areas. Due to the tumor size, operative intervention was initially limited to biopsy sampling. Chemotherapy resulted in histological maturation, but initial tumor regression was temporary. The patient suffered progressive quadriparesis, and neuroimaging demonstrated slow enlargement of the tumor and an associated syrinx. Nineteen months after diagnosis, the tumor was excised to gross-total resection in a 2-stage operation. One year following resection, the patient had no radiographic recurrence and was functional in a wheelchair with minimal paresis in the upper extremities. This case represents the most extensive example of primary spinal intramedullary CNS GNB reported to date. Holocord tumors present a significant challenge to the neurosurgeon, and resection bears substantial risk of morbidity. In spinal cord CNS GNB, chemotherapy followed by complete resection may be the most effective means of tumor control.     

Primary Intracranial Malignant Schwannoma of Trigeminal Nerve. A Case Report with Review of the Literature

The authors report a rare case of primary intracranial malignant schwannoma of the trigeminal nerve occuring in a 30-year-old woman without von Recklinghausen's neurofibromatosis (VRNF). The tumour arose from the intracranial part of the left trigeminal nerve, without skull base destruction. The tumour was partially removed operatively, followed by focal 50 Gy irradiation, but unfortunately the tumour showed rapid regrowth. A second operation was attempted, but she died of cerebral infarction soon after operation. Histologically, the original tumour was characterized by the presence of foci of extremely high cellularity, pleomorphism and mitosis in an otherwise typical benign schwannoma. Immunohistochemically, the tumour cells were positive for S-100 protein even in the atypical areas, suggesting the tumour to be of Schwann cell origin. However, the recurrent tumour was composed of undifferentiated spindle cells, which were negative for S-100 protein. Thus, it is possible to consider that the S-100 protein expression could reflect the degree of differentiation of Schwann cells. Furthermore, the postoperative irradiation might have exacerbated the malignant progression in the present case.     

Primary intrasellar schwannoma: clinical, aetiopathological and surgical considerations

Schwannomas originating within the sella turcica are extremely rare. We describe the clinical, radiological and intraoperative findings of a patient with an intrasellar and suprasellar schwannoma. Gross total tumour removal was performed by a trans-sphenoidal approach. Perivascular or ectopic Schwann cells, lateral nerve plexus within the cavernous sinus, as well as Schwann cells from small nerve twigs of the dura are some of the current histopathological hypotheses for the origin of these lesions.     

Pseudoglandular schwannoma of the cauda equina. Case report

The authors present a case of pseudoglandular schwannoma with immunohistochemical findings consistent with epithelial metaplasia. Pseudoglandular schwannoma is a rare morphological variant of benign schwannoma characterized by the presence of glandlike structures lined with Schwann cells. To the best of the authors' knowledge, this is only the fifth case of pseudoglandular schwannoma reported in the literature. Clinical, imaging, and pathological findings are described. The pathological findings were consistent with a pseudoglandular schwannoma composed of typical Schwann cells arranged in an Antoni B pattern, with numerous large pseudocystic spaces. Serial immunohistochemical studies of tissue sections revealed that the cells lining the pseudoglandular spaces were not only diffusely reactive for S100 protein, but also demonstrated focal positivity for epithelial membrane antigen and cytokeratins AE and AE3. The particular immunohistochemical features of incompletely differentiated Schwann cells in the present case give support to the metaplastic theory of the origin of glandlike structures in benign peripheral nerve sheath tumors.     

Multiple schwannoma of the penis

Schwannoma or neurilemmoma designate a tumour that originates in the Schwann cells of peripheral nerves, therefore occurring anywhere in the body. They are very rare in the penis. In the literature are reported twenty seven cases of penile schwannoma. We report a case of multiple schwannoma of the penis and make a brief literature revision about this rare pathology.     

Intraparenchymal schwannomas of the central nervous system: An additional case report and review

Intraparenchymal location of schwannomas in the central nervous system (CNS) is rare. Occasional cases involving the cerebrum, cerebellum, brain stem, and spinal cord have been reported. We report here an additional case of thoracic intramedullary schwannoma in a 42 year old woman. The literature concerning intraparenchymal schwannomas in the CNS is reviewed and discussed.     

Intrasyrinx hemorrhage associated with hemangioblastoma in epiconus

Background contextHemangioblastomas in the central nervous system are highly vascular lesions, but have low risk of hemorrhage. In spinal lesions, there are a few rare cases of intramedullary hemorrhages associated with hemangioblastoma, and their prognoses were very severe.PurposeThis is the first case of an intrasyrinx hemorrhage associated with hemangioblastoma in epiconus. We report this rare case and discuss the clinical manifestations of intrasyrinx hemorrhage caused by hemangioblastoma in epiconus.Study designA case report.MethodsThis case report presents a 45-year-old woman with intrasyrinx hemorrhage caused by hemangioblastoma in epiconus. The patient presented with a sudden onset of burning bilateral leg pain and rectal/bladder dysfunction, indicating conus medullaris syndrome. Initial magnetic resonance imaging revealed an intramedullary nodular lesion in the epiconus and holocord syringomyelia. However, follow-up magnetic resonance imaging showed intramedullary hemorrhage.ResultsThe patient underwent surgery, and an intrasyrinx hematoma was evacuated and the tumor was completely removed. Histological diagnosis was hemangioblastoma. Three months after surgery, the patient recovered from neurological deficits.ConclusionsWe present this rare case, and emphasize hemangioblastoma as the differential diagnosis in hemorrhagic spinal lesion.     

Complete surgical excision of intramedullary schwannoma at the craniovertebral junction in neurofibromatosis type-2

Intramedullary spinal schwannomas are extremely rare. We describe a case of a 13-year-old female who was found to have an intramedullary schwannoma at craniovertebral junction along with incidental bilateral acoustic neuromas, which was excised completely. To our knowledge, this is the first report of a case of intramedullary schwannoma in association with NF-2 located at the craniovertebral junction. The literature has been reviewed with emphasis on radiological imaging, histopathological diagnosis and technique to achieve complete microsurgical excision.     

Schwannoma of the tibial nerve sheath as a cause of tarsal tunnel syndrome—A case study

A schwannoma is a benign, encapsulated and non-invasive tumour whose origin is derived from Schwann cells. Its incidence in the foot and ankle is rare. The etiology, incidence, clinical presentation, histology, differential diagnoses and surgical treatment are discussed. The authors present a case of a schwannoma of the tibial nerve sheath causing tarsal tunnel syndrome of the foot. The diagnosis in this case was delayed for eight years, which unfortunately is not uncommon with pedal schwannomas.     

Multiple intramedullary lipomas with conal intramedullary dermoid: magnetic resonance appearances.

BACKGROUND CONTEXT: Intramedullary fat-containing benign childhood tumors of the cord include lipomas, dermoid cysts, and teratomas. These are embryonal tumors. Most intramedullary fat-containing tumors are solitary. Multiple intramedullary lipomas are rare and may represent a spinal lipomatous malformation. The presence of another intramedullary dermoid tumor in the same case is rare. PURPOSE: The intent of this case report is to look at magnetic resonance features and possible mechanisms of association of these fat-containing intramedullary tumors. STUDY DESIGN/SETTING: A 3-year-old male child presented with spastic quadriplegia. METHODS: Magnetic resonance imaging (MRI) of spine was done on a 1.5-T scanner in different planes. RESULTS: MRI showed multiple intramedullary spinal lipomas with an intramedullary dermoid involving the conus, cord atrophy, and subarachnoid fat droplets. CONCLUSION: Multiple intramedullary lipomas with an intramedullary dermoid represent a form of spinal lipomatous malformation. Both may represent embryogenic mesenchymal inclusions and hamartomatous growth, which can be accurately diagnosed with MRI.     

Paraspinal plexiform schwannoma of unknown nerve origin: A case report

Introduction and importanceSchwannomas are benign, slow-growing nerve sheath tumors of neoplastic Schwann cells. They are the most common peripheral nerve tumors in adults and are typically discovered incidentally due to their asymptomatic presentation. Despite the fact that most schwannomas are unassociated with a syndrome, their etiology is thought to be related to alterations or loss of the neurofibromatosis type two tumor suppressor gene.Case presentationWe present the case of a fifteen-year-old female who presented with a recurrent lower back/upper buttocks 9 cm mass with imaging suspicious for schwannoma. Needle biopsy revealed an S100 positive cellular schwannoma with patchy Ki-67. During surgical dissection down to the sacrum, no nerve of origin was identified.Clinical discussionSchwannomas have no pathognomonic findings on MRI and may occur at any location that Schwann cells are present; therefore, confirming a diagnosis relies on histopathology. Plexiform schwannomas are defined by a “network-like” intraneural growth pattern and are exceedingly rare in paediatric populations. A location distinct from the spinal canal is also very rare as schwannomas typically originate from the head and neck region.ConclusionPaediatric plexiform schwannomas have been rarely reported. Surgical planning relies on multiple factors such as tumor size, tumor location, pathologic features and symptomatic burden. The distinctive features of this case including an unknown nerve origin and a location outside the spinal canal provide a unique opportunity to discuss the diagnosis and management of paraspinal schwannomas and the impact on operative planning when a nerve of origin is not identified.     

Peripheral Nerve Schwannoma: A Review of Varying Clinical Presentations and Imaging Findings

A schwannoma or neurilemmoma is a benign, isolated, noninvasive, and encapsulated tumor originating from Schwann cells of the peripheral nerve sheath. The incidence of a schwannoma occurring in the foot and ankle is rare, with prevalence rate of 1% to 10%. Schwannomas have no sex predilection, and they commonly occur in patients in their fourth decade. Malignant transformation of benign schwannoma is unusual; however, it is important to note that malignant variants of schwannomas do exist and account for about 5% to 10% of all soft tissue sarcomas. We present 3 cases of benign schwannoma in the lower extremity. All 3 patients presented with varying clinical symptoms, including pain, paresthesia, weakness, and a palpable mass. A schwannoma was eventually diagnosed in all 3 patients. We discuss and review the known entities of peripheral nerve schwannoma and describe the clinical and imaging findings and therapeutic strategies for treating and diagnosing peripheral nerve schwannoma.     

Giant foot schwannoma.

Schwannoma of the foot is rare; only 12 cases have been reported. A schwannoma is a benign neurogenic tumour derived from Schwann cells. The diagnosis is often delayed because the symptoms are mainly those of compression disorders. We describe a 7cm schwannoma of the heel in a 30-year-old man. Ten years earlier a schwannoma was removed from the same site. The recurrent lesion was widely excised and a medial plantar flap was used to repair the heel.     

Intramedullary melanotic schwannoma of the cervical spinal cord: report of a case

We present a case report of a patient with an intramedullary tumor of the midcervical cord. At surgery, the lesion was found to be highly pigmented, and pathological analysis revealed a melanotic schwannoma. Intramedullary schwannomas and melanotic schwannomas are exceedingly rare. This is the second reported case of an intramedullary melanotic schwannoma of the central nervous system.     

Intramedullary melanotic schwannoma of the cervicomedullary junction

We report a case of a patient with an intramedullary tumor of the cervicomedullary junction. At operation, the tumor was noted to be highly pigmented, and pathological study demonstrated that the tumor was a melanotic schwannoma. Both intramedullary schwannomas and melanotic schwannomas in any location in the body are exceedingly rare. This is the first reported case of an intramedullary melanotic schwannoma of the central nervous system. The possible origin of this tumor is discussed.     

Intramedullary dermoid in a low lying conus tethered by a fatty filum – embryological implications

Summary Intramedullary dermoids arising within the conus medullaris are rare. We report a rare association of an intramedullary dermoid cyst located in a low lying conus tethered by a thickened, fatty filum terminale and discuss the embryological implications of this association.     

Primary angiitis of the spinal cord presenting as a conus mass: long-term remission

BACKGROUND: Primary angiitis of the central nervous system (CNS) is a rare vasculitic disorder that typically involves the brain and, less frequently, the spinal cord without involvement of the blood vessels outside the CNS. CASE DESCRIPTION: We present a case of a 52-year-old woman who developed a conus syndrome linked to an enhancing mass of her lower thoracic spinal cord, lumbar cord, and conus. Spinal cord biopsy performed for diagnostic purposes in the setting of progressive neurological deficit confirmed angiitis of the spinal cord. Therapy with steroid and cyclophosphamide was associated with long-term (3 years) clinical and imaging remission of the lesion. CONCLUSIONS: The prognosis of primary CNS angiitis is dismal with most cases progressing to death. Long-term remission is unusual. Aggressive therapy with steroid and cytotoxic agents may improve survival.