Intramedullary epidermoid cyst. A case report

The authors add to the literature a case report of a 32-year-old man with an intramedullary epidermoid cyst at the level of D 3/4, that was successfully operated on. There are several previous reports in the literature, but only five of these include MRI studies.     

Intracerebral schwannoma: case report and review

A case of intraparenchymal schwannoma of the brain in a 4-year-old girl is presented. Most of the small number of intracranial schwannomas unassociated with cranial nerves, reported have occurred in children or young adults. There is no male or female predominance. Most of the parenchymatous schwannomas are located supratentorially. The presence of a cyst together with the tumor appears to be characteristic of intraparenchymal schwannoma of the brain.     

Malignant solitary fibrous tumor of the lumbar spinal root mimicking schwannoma: a case report

Background contextMalignant solitary fibrous tumors (SFTs) arising from the spinal cord are extremely rare and poorly understood mesenchymal neoplasms. To date, only one malignant SFT located in the spinal canal of the sacrum has been described, but none arising from the lumbar nerve root have been reported. Although most SFTs with benign histological features can be treated by complete surgical excision alone, malignant SFTs may require adjuvant therapy. However, systemic chemotherapy and radiotherapy have not been shown effective in patients with malignant SFTs.PurposeTo describe a patient with a malignant SFT arising from the lumbar nerve root.Study designA case report and review of literature.MethodsWe describe the clinical course of the patient and the radiological and pathological findings of the tumor. The effect of systemic chemotherapy was evaluated and the relevant literature was reviewed. This work has no disclosure of funding and was approved by the Institutional Review Board of Gifu University.ResultsThe tumor had been resected previously at another hospital, but it recurred and showed multiple metastatic lesions on both lungs within 3 months. Although the patient received systemic chemotherapy, both primary and metastatic lesions were found to be stable disease according to Response Evaluation Criteria in Solid Tumors. The patient died due to cachexia 6 months after her first visit.ConclusionThis patient presented with a highly unusual tumor. Even if a tumor is a dumbbell-shaped mass, similar to a neural tumor, SFT should be considered in the differential diagnosis.     

Pancreatic schwannoma: A rare case and a brief literature review

IntroductionPancreatic schwannoma (PS) is an extremly rare benign tumor. Less than 50 cases of pancreatic schwannoma have been described in the English literature over the past thirty years.Presentation of case reportA 63-year-old female underwent left modified radical mastectomy 2 years ago due to breast cancer. During her routine check-up, a 65 × 63 × 55 mm measured calcified, well-demarcated, cystic-mass having septations and calcifications that localized to the pancreatic head was detected by abdominal computerized tomography. She was asymptomatic and her tumor markers were in normal ranges. A standard Whipple procedure was performed, and the histo-pathological diagnosis of the resected specimen was reported as ancient schwannoma with clear surgical margins. Patient’s postoperative course was eventful. She had a biliary leakage after surgery which was managed conservatively. She is under follow-up.DiscussionPancreatic schwannoma also known as neurilemoma or neuroma is a slowly growing, encapsulated, mostly benign tumor with smooth well-delineated margins that originates from myelin producing schwann cells located on the nerve sheath of the peripheral epineurium of either the sympathetic or parasympathetic autonomic fibers. PS’s are extremly rare. The head of pancreas being involved in the vast majority of cases (40%), followed by its body (20%). Management of pancreatic schwannomas remains largely controversial. Both enucleation and radical surgical resections have revealed great therapeutic efficiency. with a well prognosis without recurrences.ConclusionAlthough rare, PS’s should be considered in the differential diagnosis of the other solid or cystic masses of the pancreas.     

Intramedullary spinal cord metastasis in breast cancer: clinical features, diagnosis, and therapeutic consideration

Intramedullary spinal cord metastases (ISCMs) are very rare, but can cause devastating complications from underlying breast cancer. We report the case of a woman with known metastatic breast cancer and progressive neurologic deterioration caused by an ISCM. The epidemiology, pathogenesis, clinical presentation, diagnostic considerations, and therapeutic options are discussed.     

Chronic intramedullary abscess (Streptococcus sanguis). A case report and review of the literature

OBJECTIVE: Chronic intramedullary spinal cord abscesses are very rare. We present here a new case of this pathology, revealed by a progressive spinal cord compression. This case is particular because of the long follow up before surgery (two years). CASE REPORT: This 69-year-old patient presented a gradually progressive paraparesis and sensory loss associated with leg pain. The past medical history was dominated by a epidermoid cancer of the tongue. MRI showed an intramedullary mass at T10. On the T1-weighted sequences, the lesion was isointense with the spinal cord, Gadolinium infusion showed homogeneous enhancement of the lesion. T2-weighted sequences showed decreased signal within the lesion and an increased signal peripherally (edema). Corticosteroid infusions relieved the symptoms so the patient declined surgery. Two episodes of regressive paraplegia were treated by the same infusions. The patient accepted surgery after a third episode. RESULTS: The intervention was performed two years after the first signs and revealed a hard white mass attached to the spinal cord. Pathologic examination showed infectious inflammation. Bacteriological cultures found a Streptococcus sanguis. Two years later the patient could walk; a minimal deficit persists. CONCLUSION: First, the diagnosis was evoked but not retained because of the long course. The corticosteroid infusions gave relief and did not increase the deficit. The MRI findings remained unchanged. Intramedullary spinal cord abscesses are very rare. This pathology must be recognized because without treatment morbidity is high with a potentially fatal outcome.     

Pancreatic schwannoma: Report of a case

(Received for publication on June 24, 1998; accepted on Mar. 11, 1999)     

A Large Intramedullary Neurofibroma in the Thoracic Spinal Cord: Case Report

Neurofibromas are occasionally present in spinal roots; however, an intramedullary neurofibroma is especially rare. Although a few cases of intramedullary neurofibromas in cervical spinal cord have been reported, to the best of our knowledge, there are no reports of intramedullary neurofibromas in thoracic spinal cord, and moreover, no reports have clearly reported immunohistochemical findings. We report a rare case of a large intramedullary neurofibroma in the thoracic spinal cord and show immunohistochemical examination of the tumor. A 52-year-old man presented with a 2-year history of progressive gait disturbance. Neurological examinations demonstrated complete motor and sensory deficit of his legs. Magnetic resonance imaging of the thoracic spine demonstrated an intramedullary enhancing mass within the spinal cord between T4 and T5 levels. The patient underwent T3–T6 laminectomy surgery. The dura mater was opened to reveal fusiform dilatation of the spinal cord and a midline myelotomy was performed. An intramedullary mass was revealed and could be resected totally. Histopathological examination revealed that the tumor cells exhibited spindle-shaped and wavy nuclei with abundant collagen, which resembled schwannoma or fibrous meningioma. By immunohistochemical examination, some tumor cells were positive for S-100 proteins; however, most tumor cells were strongly positive for CD34. From these pathological findings and immunohistochemical reactions, we diagnosed the intramedullary tumor as a neurofibroma.     

A case of pancreatic schwannoma showing increased FDG uptake on PET/CT

IntroductionSchwannomas are neoplasms originating from Schwann cells in nerve sheath, but pancreatic schwannomas are rare.Presentation of caseA 59-year-old female incidentally pointed out a 2-cm-sized solid mass in the pancreas head by computed tomography (CT) which was performed for other reasons. Magnetic resonance imaging (MRI) showed a mass with hypointensity on T1-weighted images and a heterogenous hyperintensity on T2-weighted images. Magnetic resonance cholangiopancreatography (MRCP) showed no abnormal findings in the main pancreatic duct. The mass of the pancreas head showed a significantly increased uptake on fluorin-18 fluorodeoxyglucose positron emission tomography CT (FDG-PET/CT). Based on the radiological findings, a malignant potential tumor was preliminarily considered. Subtotal stomach-preserving pancreatoduodenectomy (SSSPD) was performed to make a definite diagnosis. The resected specimen showed a well-encapsulated circumscribed mass, measuring 2.0 cm, in the pancreas head. Histopathological examination revealed proliferation of spindle cells showing interlacing and palisading patterns in the tumor. The proliferating cells showed no atypia and few mitoses. Immunohistochemically, the spindle cells were positive for S-100 protein. The Ki67 labeling index was approximately 2%. Based on these findings, a diagnosis of benign schwannoma of the pancreas was made.ConclusionAlthough pancreatic schwannoma is a rare neoplasm, it is important to take into account this tumor in the differential diagnosis of pancreatic tumors. In addition, we should be aware that FDG-PET/CT shows abnormal accumulation in a benign pancreatic schwannoma.     

Benign esophageal schwannoma: Report of a case

(Received for publication on Mar. 5, 1999; accepted on Jan. 7, 2000)     

Multifocal clear cell meningioma in the spine: A case report

Clear cell meningioma is a rare morphologic form of meningioma. This case report describes a very rare case of multifocal clear cell meningioma in the thoracic and lumbar spine. A 9 year old patient presented with back pain and popliteal pain, which were aggravated by trunk motion and knocking. Magnetic resonance imaging (MRI) suggested a meningioma at the L2 level. An intradural extramedural tumor attached to the cauda equina was surgically removed and pathological diagnosis was clear cell meningioma. The patient began to complain of back pain at the Th12 level 4 months postoperatively. Retrospective analysis of MRI before the first operation revealed the small tumors at Th12 and L5 levels; comparison with a new MRI showed the rapid growth in 4 months. Adjunctive surgical resection was carried out and the back pain disappeared. These tumors had the same pathological findings. In conclusion, in young patients with suggested meningioma in the lumar spine, clear cell meningioma should also be considered, and the possibility of multifocal occurrence and postoperative recurrence must be taken into account.     

精索神经鞘瘤一例报告

回顾性分析我院收治的1例精索神经鞘瘤患者的临床资料。本例患者行精索肿物切除术,术后恢复良好。术后病理确诊为精索神经鞘瘤。精索神经鞘瘤属于罕见病例,最佳治疗方式是手术切除,术后应严密随访。     

Primary spinal malignant schwannoma: Clinical,histological and cytogenetic findings

Of the 19 patients who presented between 1980 and 1990 to the Department of Neurosurgery, University of Hamburg, F. R. G. with malignant schwannoma, 5 patients suffered from primary malignant spinal schwannoma (mean age 44 years, only one patient with von Recklinghausen's disease). Here we report the clinical, histological, and cytogenetic features of the five cases with primary malignant spinal schwannoma and discuss the prognostic aspects of this rare tumor.     

Intramedullary spinal cord gangliocytoma: case report and a review of the literature

We present a case of intramedullary spinal gangliocytoma in a 19-year-old woman who presented with a long history of hyperhidrosis of the face and neck areas and intermittent pain of the hands. The tumor involved the cervico-thoracic region of the spinal cord from C7-T3. The patient underwent debulking of the lesion and remains neurologically stable at two years follow-up. A discussion of this case is followed by a review of the literature surrounding this rare clinicopathological entity.     

Intramedullary spinal cord tumour associated with neurofibromatosis type 1

Summary We investigated the characteristic features of intramedullary spinal cord tumour associated with neurofibromatosis type 1 (NF-1). We have experienced 44 cases of pathologically confirmed intramedullary spinal cord tumour. Diagnosis of NF-1 was done according to the criteria set by the National Institute of Health Consensus Development Conference. Within the described population NF-1 was diagnosed in two patients. Both the patients were male and histopathologically the tumours were anaplastic astrocytoma and glioblastoma multiforme respectively. 6 cases of NF-1 associated with intramedullary spinal cord tumour based on the above diagnostic criteria have so far been reported, including our 2 cases. Of these 6 cases, 5 were male and in one sex was not described. The tumour was an astrocytoma in all 6 cases. This finding suggested that intramedullary spinal cord tumour associated with NF-1 tends to occur predominantly in males and that histopathologically the tumour is likely to be an astrocytoma. We conclude that the criteria proposed by the National Institute of Health Consensus Development Conference are contributory in making an accurate pre-operative pathological diagnosis of intramedullary spinal cord tumour associated with NF-1.     

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)     

Intramedullary tuberculomas. Five cases

Tuberculosis of the central nervous system is uncommon. The intramedullary localization is exceptional. We report five cases of intramedullary tuberculosis observed in four women and one man with a mean age of 43 years. Two patients had a prior history of tuberculosis. Spinal cord compression was found clinically in all cases. The spinal MRI visualized the tuberculoma in all patients; two had a double tumor. Complete removal of the tuberculoma was possible in only one patient. Outcome was stationary for four patients.