Myelopathy patients studied with magnetic resonance for multiple sclerosis plaques

Seven patients with isolated spinal cord symptoms, and with evoked potential (EP) recordings and/or cerebrospinal fluid (CSF) findings supporting a demyelinating cause for their myelopathy, were examined with cervical and cranial magnetic resonance imaging (MRI). Lesions in the cervical spinal cord were detected in 6 of the patients, including 2 who also had disseminated lesions in the brain compatible with multiple sclerosis (MS). In one patient MRI of the cervical spinal cord was normal, while plaques were seen in the periventricular region of the brain and in the brain stem. Thus, in the 3 patients with cerebral plaques, MRI supported the diagnosis of MS by showing dissemination in space. In the remaining 4 patients MRI provided support for the diagnosis of MS by demonstrating the cervical spinal cord plaques while excluding other potential causes of myelopathy, such as spinal cord compression and intramedullary tumor.     

Medulloblastoma with mandibular metastasis after complete remission of the central nervous system lesions]

A 6-year-old boy presented with headache and vomiting. Brain and spinal MRI demonstrated a large mass in the cerebellar vermis and 4th ventricle and showed thick spinal subarachnoid dissemination. Suboccipital craniotomy was performed and the tumor was totally removed. The histological diagnosis was medulloblastoma. The patient subsequently received craniospinal irradiation, and also received systemic and intrathecal perfusion chemotherapy. Then complete remission was achieved 10 months after operation. Three years later, however, a swelling at the left mandibular angle appeared. A CT scan revealed osteosclerotic lesion. After biopsy was performed, the specimen was detected infiltration of medulloblastoma cells. Bone scintigram showed a single lesion and MRI of brain and spinal cord revealed no recurrence of the central nervous system lesions. He underwent local irradiation and systemic chemotherapy with ICE regimen. This is the rare case of extracranial metastasis following remission of medulloblastoma in childhood.     

Intramedullary spinal cord involvement from metastatic gastric carcinoma: a case report

Intramedullary spinal cord metastasis (ISCM) is a rarely seen complication of cancer. We report a case of ISCM from gastric cancer. A 42-year-old male presented with a rare intramedullary spinal cord metastasis from gastric carcinoma manifesting as rapidly worsening motor and sphincter disturbances. The primary tumor had been treated 2 years previously. Magnetic resonance imaging (MRI) revealed the tumor localized in the thoracic spinal cord. The tumor was totally removed. The histological diagnosis was gastric adenocarcinoma. He has been stable postoperatively. Surgical treatment may be considered for cases with even rapid-growing tumors such as gastric carcinoma with no evidence of multi-organ dissemination, especially when presenting with progressive neurological deterioration.     

Intramedullary spinal cord metastasis from salivary ductal carcinoma of the parotid gland mimicking transverse myelitis in a patient with radiologically isolated syndrome

Intramedullary spinal cord metastases (ISCMs) are rare lesions but their presence should not be underestimated in a cancer patient with rapidly progressive neurological compromise. Due to similar timing of clinical progression and imaging characteristics, these lesions may be misdiagnosed as transverse myelitis, an inflammatory disorder of the spinal cord that may be idiopathic or secondary to other diseases including infections, connective tissue disorders, nutritional deficiencies, and demyelinating disorders. We present a case of a 44 year-old male with a history of parotid gland metastatic salivary ductal carcinoma (SDC) and incidental demyelinating white matter lesions on brain magnetic resonance imaging (MRI) diagnosed as radiologically isolated syndrome with a CSF that was positive for oligoclonal bands. The patient initially presented with mid-thoracic dermatomal numbness, bilateral lower extremity weakness, and neurogenic bladder. MRI spine demonstrated an enhancing T5–7 intramedullary lesion initially diagnosed as transverse myelitis. After progressing to complete motor and sensory loss below T6 despite high-dose intravenous steroids and plasmapheresis, surgical biopsy was undertaken. Intraoperative findings revealed an intramedullary tumor for which a subtotal resection was performed. Pathology was consistent with a metastatic deposit from the patient's primary parotid SDC. The patient underwent postoperative chemotherapy but expired due to systemic disease progression seven months following surgery without neurological improvement. This is the first reported case of ISCM from a primary SDC. The median survival is 6 months for patients with ISCMs treated surgically. The goals of surgery are spinal cord decompression, functional preservation, and tissue diagnosis.     

Late intrathoracic relapse of pineal germinoma connected to intraspinal canal

Extraneural metastases of intracranial germinoma are rarely reported. The authors describe the first case of metastatic lung germinoma of the thoracic spine. A 27-year-old man presented with right shoulder pain and right upper limb weakness. He had a history of repetitive radiation therapy - nine (whole-abdomen; 15Gy), 12 (whole brain; 30Gy, whole spine 42Gy) and 14 years ago (local; 32Gy) - for abdominal metastasis, temporal and fourth ventricle metastasis and spinal dissemination and metastatic pineal germinoma, respectively. Magnetic resonance imaging revealed a lung mass invading the thoracic spine that was diagnosed as a germinoma by tumor biopsy. He was treated by irradiation with 54Gy and two cycles of chemotherapy with cisplatin and etoposide. He did not have any sign of tumor eight years later.     

Treatment of an intracranial germinoma spinal metastasis

A 20-year-old male with a solitary intradural extramedullary spinal metastasis from a pineal germinoma presented with progressive spinal cord compression. This was initially treated with radiotherapy and the patient showed rapid clinical improvement with complete resolution of his neurological symptoms and signs. Subsequent systemic chemotherapy resulted in complete radiological resolution of the lesion. This case is presented to demonstrate radiotherapy and chemotherapy as a treatment option for germinoma 'drop' metastasis causing spinal cord compression, along with a review of the literature.     

Concurrent chemoradiotherapy for primary cervical spinal cord germinoma

We report a rare case of primary intramedullary germinoma in the cervical spine of a 39-year-old woman without evidence of intracranial or disseminated disease. The germinoma was treated by a biopsy and follow-up concurrent chemoradiotherapy. This is the only reported case of primary spinal cord germinoma for which concurrent chemoradiotherapy was given. Furthermore, this is only the second reported case of histologically documented primary intramedullary cervical spinal cord germinoma. The patient was disease-free and there was near-complete resolution of the pre-operative neurological deficits at the 20-month follow-up examination.     

Primary yolk sac tumor in the spinal cord

A case of a primary yolk sac tumor of the spinal cord is reported. The patient was a 17-month-old Japanese girl, who was found to have an intramedullary mass at the upper thoracic level. The preoperative diagnosis was primary glioma, but histological examination of the surgical specimen revealed a yolk sac tumor. As there were no other lesions, the spinal cord lesion was considered to be the primary tumor. Based on our previous experience, we treated this patient with low-dose irradiation followed by combination chemotherapy with cisplatin, vinblastine and bleomycin. However, the effectiveness of this therapy was poor in this particular case, and the patient died of pyelonephritis with uncontrollable imbalance of serum electrolytes. Received: 3 December 1997 Revised: 20 February 1998     

Malignant transformation in pediatric spinal intramedullary tumors: case-based update

BACKGROUND: In children, intramedullary spinal cord neoplasms are rare. These are typically low-grade neuroepithelial tumors, most commonly astrocytomas, ependymomas, and gangliogliomas. Malignant transformation, while common in recurrent adult low-grade gliomas, is an unusual event in pediatric low-grade neoplasms, specifically in intramedullary spinal cord tumors. ILLUSTRATIVE CASES: We report two cases of malignant transformation in low-grade neuroepithelial tumors of the pediatric intramedullary spinal cord. Two children with intramedullary tumors, one with a WHO grade I ganglioglioma and one with a low-grade astrocytoma, were treated surgically, diagnosed histologically, and followed through the course of their disease. Both patients' tumors transformed to higher grades without prior irradiation or chemotherapy, and without a genetic predisposition to tumorigenesis. DISCUSSION: Malignant transformation can occur in low-grade intramedullary neoplasms in children. This is a novel documented event for pediatric intramedullary spinal cord tumors and a rare event for all pediatric low-grade neuroepithelial tumors without induction by irradiation. A survey of the relevant literature reveals an underwhelming number of studies focusing on malignant transformation in children's CNS tumors relative to adults. Further investigation into molecular mechanisms of pediatric low-grade neoplasms may reveal more aggressive tumor sub-variants predisposed to malignant degeneration.     

A case of olfactory neuroblastoma with intracranial extension and distant metastasis]

Olfactory neuroblastoma is a rare tumor originating in the upper nasal cavity. It rarely extends intracranially. We report a clinical case of olfactory neuroblastoma with intracranial extension and distant metastasis. A 35-year-old man complained of nasal stuffiness and bleeding, headache and vomiting. Neurological examination showed anosmia and papilledema. MRI showed a huge mass that occupied the right nasal and paranasal cavities, and extended into the right frontal base. The tumor was removed totally and was histologically diagnosed as olfactory neuroblastoma. About two months after surgery, however, MRI demonstrated a rapid recurrence of the tumor in the nasal and paranasal cavities and the frontal lobe. Metastatic lesions were also seen in the right cervical lymph nodes. Chemotherapy was administered using cisplatin and etoposide. The tumor in the frontal lobe shrunk markedly but the other lesion persisted. Whole brain irradiation and local irradiation of the cervical lymph nodes were performed. The tumors became smaller but did not disappear. MRI demonstrated spinal dissemination. Irradiation of the whole spinal cord was performed. The metastatic lesions disappeared. The patient was discharged without neurological deficits, but died of pneumonia 15 months after surgery. Olfactory neuroblastoma is a slow-growing tumor and is highly radiosensitive, but it rarely extends or develops multiple distant metastases and seldom shows a short survival time, as in our case. A review of the literature documented responses in patients treated with a cisplatin-based drug combination. We recommend systemic control using cisplatin-based chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic olfactory neuroblastoma.     

Intramedullary Solitary Fibrous Tumor of Cervicothoracic Spinal Cord

Solitary fibrous tumor is rare benign mesenchymal neoplasm. The spinal solitary fibrous tumor is extremely rare. The authors experienced a case of intramedullary solitary fibrous tumor of cervicothoracic spinal cord in a 48-year-old man with right lower extremity sensory disturbance. Spinal MRI showed intradural mass lesion in the level of C7-T1, the margin between the spinal cord and tumor was not clear on MRI. A Left unilateral laminectomy and mass removal was performed. Intra operative finding, the tumor boundary was unclear from spinal cord and it had intramedullary and extramedullary portion. After surgery, patient had good recovery and had uneventful prognosis. Follow up spinal MRI showed no recurrence of tumor.     

Primary Intramedullary Spinal Sarcoma : A Case Report and Review of the Current Literatures

Primary central nervous system (CNS) sarcomas are exceedingly rare, and, to the best of our knowledge, there has not yet been a report of intramedullary sarcoma. Here, we report a primary intradural intramedullary sarcoma of the spinal cord in a four-year-old boy who presented with low back pain and a radiculopathy involving both lower extremities. The tumor showed significant enhancement on magnetic resonance (MR) images due to its extreme vascularity. Gross total tumor removal was performed with microelectrical pulse recording, and the patient also received adjuvant radiotherapy and chemotherapy. After the operation, the patient''s sensory deficits were improved. Because CNS dissemination is common, entire neuraxis evaluation is essential, although there was no evidence of dissemination in this case. The prognosis of primary CNS sarcoma is poor due to infiltrative nature and early CNS dissemination is common, and the treatment of choice is radical surgical resection. Adjuvant therapy is also beneficial with radiotherapy and chemotherapy.     

Intramedullary spinal cord glioma with intracranial seeding.

Two cases of intracranial dissemination of primary intramedullary spinal cord gliomas are reported, with a review of the literature. One patient had a post mortem confirmation and in the second, cerebral CT scan and CSF examination demonstrated the occurrence of intracranial dissemination. CSF protein was elevated on both patients and malignant cells were found late in only the one patient. Both patients had raised intracranial pressure. The mechanisms of dissemination and of raised intracranial pressure are discussed. Such dissemination may be more common than previously realised.     

58例脊髓髓内室管膜瘤临床与预后

目的 回顾分析脊髓髓内室管膜瘤的诊疗特点和影响预后的因素。方法 总结58例经手术治疗的脊髓髓内室管膜瘤患者的临床资料。结果 MRI可以明确诊断,显微神经外科手术治疗使得大多数的脊髓室管膜瘤可以被全切。力弱,麻木、感觉迟钝和过敏,疼痛,膀胱直肠功能障碍是临床主要表现,而术后这四项症状都相应改善。结论 早期手术是治疗脊髓室管膜瘤最有效的方法,术中超声波和电生理监测,椎板复位能够提高手术疗效。放疗、化疗则作为不能全切肿瘤时的辅助治疗来提高生活质量。     

松果体区生殖细胞瘤合并梗阻性脑积水的治疗分析

目的 总结松果体区生殖细胞瘤合并梗阻性脑积水的治疗经验。方法 回顾性分析2016年9月至2019年9月经脑室镜下活检术+第三脑室底造瘘术治疗的15例生殖细胞瘤合并梗阻性脑积水的临床资料。无甲胎蛋白（AFP）升高的病人,术后给予全脑全脊髓放疗（36 Gy）+肿瘤区放疗（42 Gy）;存在AFP升高的病人,术后给予全脑全脊髓放疗+EP（顺铂+足叶乙甙）方案化疗。结果 15例均成功进行脑室镜下活检术,明确诊断;其中14例第三脑底造瘘成功,1例造瘘不成功改行改行透明隔造瘘+Ommaya囊置入术。15例术后随访8~45个月,平均24个月;1例放化疗过程中出现白细胞低、发热,病情加重,家属要求自动出院后死亡;其余14例精神、发育等情况良好,均未出现脑积水复发及肿瘤复发与播散。结论 松果体区生殖细胞瘤合并脑积水,脑室镜下病变活检+第三脑室底造瘘术,既可以明确诊断,还可以治疗脑积水;术后根据进行针对性放化疗,预后良好。     

The management of bifocal intracranial germinoma in children

Objectives

Bifocal intracranial germinoma (BFG) is a tumour of the pineal and suprasellar regions, which is known to be highly radiosensitive. The definitive treatment and outcomes are not well defined, particularly in the paediatric population. We review our series of purely paediatric cases from a single institution and combine them with the limited reports in the literature to determine the results of different management strategies.

Methods

Four patients were treated at our institution with a median age of 15.3 years. A literature search identified a further 38 paediatric cases with a median age of 12.9 years.

Results

All four patients had normal serum and CSF tumour markers. One patient had a diagnosis made based on imaging findings of bifocal pineal and suprasellar lesions presenting with diabetes insipidus. Three others underwent biopsy. All had craniospinal radiotherapy, which has led to complete cure with no cases of progression at a mean follow-up of 3 years. The most common treatment modality in published cases is craniospinal irradiation. In the cases reviewed, limited radiation treatments (whole ventricle or focal) combined with chemotherapy regimens yield comparable outcomes where there is no spinal dissemination. Outcomes do not appear to be altered by biopsy in cases with negative tumour markers and characteristic imaging appearances.

Conclusion

Patients who present with a classic appearance of germinoma, negative tumour markers and diabetes insipidus probably do not require a biopsy to confirm the diagnosis. No evidence of dissemination may obviate the need for craniospinal irradiation, but good quality long-term follow-up data are required to demonstrate the benefits of combined focal radiotherapy and chemotherapy regimes.     

Intramedullary subependymoma of the cervical spinal cord: a case report with immunohistochemical study

Spinal subependymomas, which have a relatively benign nature, are very rare tumors. It is difficult to distinguish spinal subependymomas from other intramedullary spinal tumors based on neuroradiological findings. A case of cervical intramedullary subependymoma in a 63-year-old female is reported. The diffused enlargement of the spinal cord at C2 level involved the lesion with isointensity on a T1-weighted MRI and relatively high intensity on a T2-weighted MRI. Enhancement in the small part of the tumor was observed on a T1-weighted MRI with gadolinium administration. The tumor occupied the left side of the spinal cord, and was totally removed through a laminoplasty of C2. Immunohistochemistry was useful for pathological diagnosis. The clinical feature of this patient is described with the review of literatures.     

Primary germinoma of the spinal cord: a case report with 28-year follow-up and review of the literature

Germ cell neoplasms occur in extra-gonadal midline locations of the retroperitoneum, mediastinum, pineal gland, areas of the suprasellar cistern, and rarely in the spinal cord. We recently reviewed a case of an unresectable lumbar spinal cord tumor in a 16-year-old female previously diagnosed as metastatic poorly differentiated carcinoma. An extensive evaluation for a primary neoplasm at that time was unsuccessful and the patient was treated with local radiation therapy. Recently, additional histochemical and immunocytochemical studies were performed on the archival formalin-fixed paraffin-embedded material and the clinical history was reviewed. These ancillary studies (including positive immunohistochemical staining for placental alkaline phosphatase) support a diagnosis of intramedullary germinoma of the conus medullaris. This patient has enjoyed 28 years of disease free survival which reflects the radiosensitive nature of this neoplasm. These data lend support to the existence of a primary germinoma in the spinal cord and illustrate the utility of using histochemical stains and immunohistochemistry to assist in diagnosing this treatable neoplasm.     

Diagnostic and therapeutic strategy for confounding radiation myelitis

We report a case of confounding radiation myelitis to demonstrate the usefulness of surgical biopsy in ensuring the correct diagnosis and to avoid unnecessary treatment. The patient was a 40-year-old man with a history of epiglottis carcinoma and sarcoidosis. Six months after radiation therapy and chemotherapy for epiglottis carcinoma, he noticed paresthesia and dysesthesia in the left arm and leg. Two months after that, he complained of severe neck pain and rapidly progressing weakness in all extremities. MRI showed an enhanced intramedullary lesion with extensive edema in the cervical spinal cord. Radiation myelitis, intramedullary spinal tumor, and neurosarcoidosis were considered as differential diagnoses. Spinal cord biopsy with laminectomy was performed and radiation myelitis was diagnosed. After the surgery, the lesion was significantly decreased in size even though corticosteroid therapy was rapidly tapered. We emphasize that a spinal cord biopsy is indicated to obtain a pathological diagnosis and to make a clear treatment strategy for patients with associated diseases causing lesions of the spinal cord.     

Multiple sclerosis]

Longitudinally extensive spinal cord lesions that extend over three vertebral segments on magnetic resonance imaging (MRI) are significantly more common in opticospinal multiple sclerosis (OSMS) than in conventional MS (CMS) in Japanese. In Japanese patients with MS, concentrations of serum vascular endothelial growth factor (VEGF) were significantly higher in MS patients in relapse than in controls or MS patients in remission, irrespective of clinical subtype. VEGF at relapse showed a significant positive correlation with the length of the spinal cord lesions on MRI. The IL-17/IL-8 system in cerebrospinal fluid (CSF) was markedly activated in OSMS, and both cytokines correlated with CSF/serum albumin ratio and the spinal cord lesion length on MRI. IL-17 is produced by autoreactive memory T cells and induces IL-8 production in a variety of cells. IL-8 acts as a chemokine for neutrophils as well as T cells. In autopsied spinal cords from OSMS patients, many neutrophils were seen to infiltrate the severely damaged spinal cord lesions; something that might be explained by intrathecal activation of IL-17/IL-8 axis in this condition. Our results, therefore, suggest that an increase in both serum VEGF and CSF IL-17 and IL-8 might contribute to the formation of longitudinally extensive spinal cord lesions through an increase of vascular permeability and the reinforcement of neutrophilic inflammation.