35-year-old woman with progressive bilateral leg weakness

A 35-year-old woman presented with one month's history of progressive bilateral leg weakness and altered sensation. There had been no pain. She had noted urinary frequency and constipation in the previous two weeks. On examination, the patient had diffuse lower extremity weakness (2-3/5), with a T6 sensory level to pain and temperature sensation. MRI demonstrated a T4-5 intradural mass ventral to the spinal cord, with an enhancing dural tail, consistent with meningioma. At surgery an intradural, extramedullary, firm, black neoplasm was encountered, which invaded the ventral dura and elevated and distorted the spinal cord. The mass was removed, leaving only microscopic invasion of the ventral dura. There was no bone invasion. Serial sections revealed a homogeneous black tumor without necrosis. H&E stained sections showed an occasionally fascicular tumor of melanocytes and small round blue tumor spindle cells with melanin pigmentation and 1-2 mitotic figures per 10 high-powered fields. The nuclei are generally oval-shaped and elongated, with prominent nucleoli. Necrosis, hemorrhage, and nuclear and cellular pleomorphism are not present and mitotic figures are rare. Immunohistochemical staining was positive for S-100 and HMB-45. MIB-1 labeling averaged 1-2%. A diagnosis of primary meningeal melanocytic tumor was made. Primary meningeal melanocytic tumors (PMMTs) are rare; fewer than 100 cases have been described. PMMTs of the CNS consist of a spectrum of tumors ranging from well-differentiated melanocytoma to its overtly malignant counterpart, melanoma. Intermediate grade melanocytomas (IMGs) are the least common variant, comprising about 10% of PMMTs reported. IGMS occur in the spinal leptomeninges and intracranially in approximately equal proportions. IGMs are more cellular than the well-differentiated variant, with 1-3 mitotic figures per 10 HPFs and MIB-1 labeling of <6%. By contrast, melanomas contain more mitotic figures (3-15 per 10 HPF) and MIB-1 labeling rates up to 15%. Once metastasis, including drop metastasis from pigmented medulloblastomas, have been excluded, the differential includes pigmented meningiomas and schwannomas (solitary or as part of Carney complex), as well as other pigmented CNS tumors such as ependymoma and pineoblastoma and systemic diseases such as lymphoma . . . For primary CNS melanocytic neoplasms, complete tumor resection is preferred, as it leads to cure of well-differentiated and intermediate-grade melanocytomas and most melanomas. Radiotherapy is recommended for incomplete resection of IMGs and melanomas; the recurrence potential of low-grade melanocytomas is less clear and watchful waiting may be employed, since recurrent tumors may be treated surgically prior to radiation. Two months after surgery, the patient had normal sensation and strength. She was given focused radiotherapy to the region of the ventral thecal sac to 40 cGy. At one year following surgery, the patient's neurological examination is normal and she remains free of residual disease by MR examination.     

Intradural extramedullary leptomeningeal hemangioblastomatosis and paraneoplastic limbic encephalitis diagnosed at autopsy: an unlikely pair

Typical cerebellar or spinal cord hemangioblastoma is often solitary and has a benign clinical course, whereas disseminated hemangioblastomatosis is extremely rare. We report a 75-year-old man with disseminated "leptomeningeal" hemangioblastomatosis and concurrent paraneoplastic limbic encephalitis who died of this disease. The patient presented with gait problems and cognitive deficits, and was diagnosed with a cervical spinal tumor. Surgical resection achieved an apparent gross total resection, but later the patient began experiencing severe cognitive decline. His condition progressively deteriorated during the next few months, and he died 15 months after his initial surgery. Autopsy revealed disseminated extramedullary intradural hemangioblastomatosis with extensive leptomeningeal nodules involving the entire spinal cord, medulla, pons, and midbrain, as well as histologic features of limbic encephalitis. There was no evidence of von Hippel-Lindau disease, and no specific etiology was identified for the dementia. To the best of our knowledge, this is the first case of limbic encephalitis occurring in the setting of disseminated hemangioblastomatosis.     

Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature

Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.     

Urothelial carcinoma metastases impacting the CNS: A 20-year retrospective series

IntroductionOur group has a longstanding interest in metastases impacting the central nervous system (CNS), including spread from prostatic adenocarcinomas, thyroid carcinomas, and breast carcinomas, most of which metastasize to CNS sites at a later time after the primary tumor is well-known. However, one of the least frequent types of systemic malignancies to metastasize to brain or spine is urothelial carcinoma. Thus, few large studies from a single institution exist. Fewer still detail the interval between first diagnosis of primary tumor and CNS lesion, or whether a patient might have their first presentation of cancer in the brain or spine, thus prompting review of our 20-year experience.MaterialsCase identification via text word search of pathology databases from our adult and referral hospitals, 2002 to present. Demographic and clinical data were extracted from reports and the medical record.Results15 cases, 11 male: 4 female, age range 37–82 years were identified. Nine had metastases to brain parenchyma, 5 to vertebral column impacting spinal cord, and 2 to skull, one of which had tumor extension into right parietal lobe. Strikingly, 5 of 15 patients had had their CNS-impacting metastasis as their first presentation of neoplastic disease.ConclusionsCNS metastasis of urothelial carcinoma is a rare occurrence; nevertheless, pathologists should include urothelial carcinoma in their differential diagnosis as a type of cancer that can first present with a CNS-impacting metastasis.     

Angiodysgenetic necrotizing myelopathy (the Foix-Alajouanine syndrome)

One observation of a rare disease--angiodysgenetic necrotizing myelopathy--in a 50-year-old patient is described. The diagnosis was established at necropsy and did not confirm suspected spinal cord tumor and myelitis. The disease is angiodysplasia with resulting necrotizing myelopathy. Large dysplastic intra- and extramedullary vessels with a disturbed wall permeability, vascular malformations and necrotic foci in the spinal cord are characteristic for this condition.     

成人与儿童髓母细胞瘤的MRI特征分析

目的 分析成人与儿童髓母细胞瘤的MRI表现,提高对髓母细胞瘤的认识。方法 回顾性分析2009年1月—2017年12月广东省农垦中心医院经手术病理证实的髓母细胞瘤36例的影像学资料。将其分为两组:儿童组(＜18岁)25例,成人组(≥18岁)11例。36例患者术前行MRI平扫及增强扫描,观察并对比分析两组患者肿瘤的发生部位、瘤体最大径线、边界、囊变、实性部分强化程度、瘤周水肿、梗阻性脑积水、室管膜种植转移等影像学特征。结果 36例髓母细胞瘤均发生于幕下,儿童常见于小脑蚓部(21/25),成人病灶多位于小脑半球(9/11),差异有统计学意义(P=0.000)。儿童肿瘤最大径为(4.50±0.58) cm,其中17例肿瘤最大径超过4 cm;成年人瘤体最大径为(5.24±1.36) cm,其中8例肿瘤最大径超过4 cm;两者比较差异无统计学意义(Z=-0.895, P＞0.05)。成人组和儿童组囊变分别有16例和11例,瘤周水肿分别有4例和7例,差异均有统计学意义(P=0.034、0.008);而边界、梗阻性脑积水及室管膜种植转移方面组间差异均无统计学意义(P值均＞0.05)。36例患者髓母细胞瘤实性部分均表现为T₂WI及T₂WI-FLAIR稍高信号,T₁WI等偏低信号。12例做了弥散加权成像检查(5例儿童、7例成人),瘤体实性部分均表现为弥散受限;增强后,两组肿瘤实性区域均表现为轻度至中度强化。结论 儿童髓母细胞瘤好发于小脑蚓部,成人以小脑半球多见;儿童肿瘤囊变及瘤周水肿相对于成年人少。儿童与成人髓母细胞瘤均常表现为边界清楚肿块,实性区域轻度至中度强化,易发生梗阻性脑积水,可发生室管膜种植转移。     

Metastatic pineocytoma of the spinal cord after long-term dormancy

An unusual tumor in the spinal cord of a 57 year old man is reported. At the age of 42 years, the patient had had a pineal region tumor that subsequently disappeared completely after local radiation therapy only. Eleven and 15 years later, at the ages of 53 and 57 years, respectively, solitary spinal cord tumors were found and removed. Pathological examination revealed that these tumors were of neurogenic origin and showed the characteristic features of pineal parenchymal tumors such as pineocytoma and pineoblastoma. Based on the cytology of individual tumor cells and the absence of mitotic figures and necrotic foci, this patient was diagnosed as having metastatic pineocytoma of the spinal cord. This case suggests that distant, subarachnoidal metastasis to the spinal cord can occur in pineocytomas even after long-term dormancy.     

Reinnervation of the mammalian spinal cord after neonatal dorsal root crush

Summary In the adult mammal, nerve fibres do not regrow into the spinal cord after a dorsal root lesion. The elongation of dorsal root nerve fibres into the spinal cord of neonatal rats was examined: L4 and L5 dorsal roots were crushed in rat pups. After 3–6 months, the dorsal root-spinal cord junction was investigated morphologically in several long series of ultrathin cross-sections. In rats which had been operated on at birth (0–2 days old), axons from the lesioned roots could be followed into the CNS tissue of the spinal cord. In contrast to normal development, the usual short segment of CNS glia did not grow into the neonatally lesioned roots. Instead, the CNS-PNS border was located within the spinal cord. The nerve fibres, which were of normal diameter, had regrown across the PNS-CNS border and elongated further into the CNS environment of the spinal cord. In rats operated on at the end of the first postnatal week or later, the largest dorsal root nerve fibres were only half the size of those in unoperated animals and reinnervation of the spinal cord had not occurred. An astrocyte-dominated CNS segment had developed in these roots. The impact of an early neuronal lesion on the development of certain glia cells and their importance in the outcome of spinal cord reinnervation are discussed.     

Autologous adult rodent neural progenitor cell transplantation represents a feasible strategy to promote structural repair in the chronically injured spinal cord

Adult neural progenitor cells (NPCs) represent an attractive source for cell-based regenerative strategies in CNS disease. In animal models of spinal cord injury, syngenic adult NPCs, which were isolated from pooled post-mortem CNS tissue and co-transplanted together with fibroblasts, have been shown to promote substantial structural repair. The autologous transplantation of adult NPCs represents a major advantage compared with other sources of neural stem/progenitor cells. However, the feasibility of autologous NPC generation from a single biopsy in a relevant preclinical CNS disease model has yet to be demonstrated. To investigate this matter, adult Wistar rats underwent a cervical spinal cord lesion, which was followed by a minimal subventricular zone aspiration biopsy 2 days later. NPCs were isolated and propagated separately for each animal for the following 8 weeks. Thereafter, they were co-transplanted with simultaneously harvested skin fibroblasts in an autologous fashion into the cervical spinal cord lesion site. A total of 4 weeks later, graft survival, tissue replacement and axonal regeneration were assessed histologically. Animals receiving either allogenic NPCs combined with fibroblasts or autologous pure fibroblast grafts served as control groups. Within 8 weeks after the biopsy more than 3 million NPCs could be generated from a single aspiration biopsy, which displayed a differentiation pattern indistinguishable from syngenic NPC grafts. NPCs within autologous co-grafts readily survived, replaced cystic lesion defects completely and differentiated exclusively into glial phenotypes, thus paralleling previous findings with syngenic NPCs. The delayed transplantation 8 weeks after the spinal cord lesion elicited substantial axonal regeneration. These findings demonstrate that the therapeutic strategy to induce structural repair by transplanting adult autologous NPCs, after the successful propagation from a small brain biopsy into an acute CNS disease model, such as spinal cord injury, is feasible at the preclinical level.     

Spinal cord compression due to extramedullary haemopoiesis in myelofibrosis.

Extramedullary haemopoiesis resulting in spinal cord compression is rare. This report of extramedullary myeloid metaplasia in a patient with myelofibrosis serves to illustrate the value of magnetic resonance imaging (MRI) in the diagnosis and management of good neurological recovery.     

A case of medullomyoblastoma of cerebellopontine angle mimicking acoustic neuroma

Medulloblastoma is a common malignant central nervous system neoplasm found mainly in children. One the contrary, medulloblastoma of the cerebellopontine angle, the location of the tumor is very unusual. This is the the first case of the medullomyoblastoma, a rare form of medulloblastoma, occurring in the cerebellopontine angle. A 15-year-old boy experienced a sudden hearing loss in the left ear. Conservative medical treatment failed, and temporal MR imaging revealed a heterogeneously enhancing mass at the left cerebellopontine angle cistern and in the internal auditory canal; therefore, the lesion was regarded as a typical acoustic neuroma. Few days before surgery, an ipsilateral facial palsy developed, and a follow-up MR imaging showed a rapid growth of the previous lesion. The extended translabyrinthine approach permitted surgical removal. And under pathological diagnosis of malignancy, radiation therapy and series of chemotherapy was performed.     

A rare case of extramedullary spinal cord germinoma

A rare case of spinal cord germinoma in a 20-year-old Japanese female is presented. The tumor was detected at the level of T11 and L3 by myelography. Histological findings of the resected tumor were identical to germinoma, which is characterized by polygonal tumor cells, lymphocytic infiltrate, and granuloma. Tumor cells and lymphocytes reacted with antibodies for placental alkaline phosphatase and CD45, respectively. A few multinucleated giant cells, negative immunohistochemically for human chorionic gonadotropin were also seen. The present case is the 10th case of spinal cord germinoma and is thought to be the first case of such a tumor located extramedullary. Diagnosis of such a rare tumor in an unusual location needs proper histological, ultrastructural and immunohistochemical evaluation.     

Intradural extramedullary tuberculoma of the spinal cord: a case report

Intradural extramedullary (IDEM) tuberculoma of the spinal cord is uncommon entity and moreover, few reports have been documented on concurrent IDEM and intracranial tuberculomas. Authors report a case of IDEM spinal tuberculoma having intracranial lesion simultaneously. A 49-year-old woman suffered from paraparesis and urinary incontinence while being given medical treatment for tuberculous meningitis. Magnetic resonance imaging (MRI) revealed an IDEM mass lesion between the T1 and T2 spinal levels, and multiple intracranial tuberculous granulomas. Surgical resection of the IDEM tuberculoma followed by anti-tuberculous medication resulted in good outcome.     

Tanycytic ependymoma: two case reports and review of the literature

Two cases of tanycytic ependymoma are reported, one in a woman aged 33 years and one in a man aged 36 years. The woman presented with lesions at L2 and L3 in the spinal cord and clinical symptoms suggested spinal cord compression, such as movement and sensory dysfunction. The man had a lesion located in the lateral ventricle and presented with headache, vomiting, and impaired vision. Both of these patients developed gradual increases in the intensity of their symptoms over a few months prior to admission. A spinal cord MRI of the woman showed a well-defined, solid cystic mass measuring 1.0 × 6.5 cm at L2 and L3 of the spinal cord. The mass showed low signal intensity on T2-weighted images in the intervertebral disc with equal signal intensity T1-/T2-weighted images. The signal was uniform and enhanced scanning showed a light strengthening of signal. A brain MRI of the man showed a well-defined mass measuring 4 cm in diameter invading the right ventricle. The mass showed low signal intensity on right T1-weighted images and high signal intensity on T2-weighted images. Histopathologically, the tumors were rich with significant and uniform proliferation of long spindle cells, which were arranged in fasciculate and knitted patterns with bipolar and spindled processes focally forming perivascular pseudorosettes. The cell nuclei showed minor pleomorphism. A few tumor cells centered on the vessels resulted in a nuclear-free zone, which was characteristic of ependymoma. Immunohistochemically, the tumor cells were positive for GFAP, EMA, and CD99, but negative for S-100. A review of the cases reported in the literature shows that tanycytic ependymoma occurs more often in the spinal cord. As it can resemble pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be differentially diagnosed to exclude some of the benign spindle cell tumors of the central nervous system.     

Intradural disc herniation at L5-S1 mimicking an intradural extramedullary spinal tumor: a case report

Intradural lumbar disc herniation is a rare pathological entity. The pathogenesis of intradural lumbar disc herniation is not known clearly. Intradural disc herniations usually occurred at the L4-L5 levels but have also been reported at other levels. However, intradural disc herniation at L5-S1 is quite rare. There are approximately nine reports in the English literature of intraradicular disc herniation at L5-S1. We described a 61-yr-old man with suspected intradural mass at the level of L5-S1 space. The patient presented with pain in the lower back and both lower legs for 4 months and a sudden exacerbation of the symptoms for 3 days. Gadolinium-enhanced magnetic resonance imaging (MRI) demonstrated a large disc herniation at the L5-S1 level with an intradural component. L5 and S1 laminectomy was performed, and dura was swollen and immobile. Subsequent durotomy was performed and an intradural disc fragment was removed. The patient had full recovery in 3 months. Intradural lumbar disc herniation must be considered in the differential diagnosis of mass lesions in the spinal canal. Contrast-enhanced MRI scans are useful to differentiate a herniated disc from a disc space infection or tumor.     

August 2004: 64-year-old man with intermittent paresthesia of the abdomen and of the legs

A 64-year-old-man had a 2-year history beginning with a sense of abdominal "constriction." Additional slowly rising symptoms, such as tingling of the legs, mild gait ataxia and painful micturition, led to MRI investigation of the spinal cord. A fusiform enlargement of the cord extending from T5 to T8 was shown. The space occupying lesion infiltrated diffusely the spinal cord. A contrast medium enhancing exophytic tumor pellet was approached via a 2-level laminoplasty and resected. Biopsies were taken from different exophytic tumor areas whereas the intramedullary part was spared. The histologic examination confirmed the typical pattern of a pilocytic astrocytoma in all specimens. In our surgical experience with 226 intramedullary tumors and with 117 patients affected by intracranial pilocytic astrocytoma this case is unique because of its combination of tumor location, growth pattern and age of the patient.     

Spinal epidural metastasis of cerebral oligodendroglioma

A 50-year-old male patient with right frontal oligodendroglioma underwent subtotal resection on three separate occasions and, 10 months later, exhibited right frontal oligodendroglioma and extracranial metastasis. Spinal magnetic resonance imaging (MRI) demonstrated the existence of an enhancing mass lesion with evidence of posterior epidural compression at the 10th-11th thoracic level, not involving the vertebrae. A bone scan of the spine appeared normal, but showed evidence of hot uptake in the pelvis and femur. This report concerns a patient who developed a fatal and clinically unexplained, pancytopenia 3 months after the removal of a spinal epidural oligodendroglioma. Oligodendroglioma with metastasis outside the central nervous system is extremely rare, and only a few cases have previously been reported. A brief review of the literature with an emphasis on the mechanisms of tumor cell dissemination is presented.     

Granulozytäres Sarkom (sog. Chlorom) als mögliche Ursache einer Rückenmarkskompression

Granulocytic sarcomas (so-called chloromas) are rare extramedullary tumorlike proliferates of myelogenous precursor cells that may de novo precede acute leukemia or coincide with the first manifestation or relapse of acute myeloid leukemia. Rarely, such tumors represent the initial manifestation of a blast crisis in the course of a chronic myeloproliferative disease, such as chronic myeloid leukemia. If they occur in aleukemic patients incorrect diagnoses may result. Differential diagnostic considerations are being discussed by presenting the case of a 58-year-old man who experienced spinal cord compression by an isolated epidural mass lesion.     

Spinal pleomorphic xanthoastrocytoma companied with periventricular tumor

Pleomorphic xanthoastrocytoma (PXA) is a low grade tumor that occurs in supratentorial area of children and young adult. In the previous reports, PXA of spinal cord or multicentre was extremely rare. A 60-year-old patient of spinal PXA and periventricular tumor presented with waist pain and weakness of double legs for one month. Neuroimaging showed that a lesion at the level of L2-L3 and periventricular tumor. Postoperative microscopy indicated that WHO grade II PXA. Photomicrograph of the lesion showed spindle cells, marked nuclear and cytoplasmic pleomorphism, with foamy cytoplasm. Immunohistochemical staining showed that GFAP and S-100 were positive. This is a rare case of synchronous multicentric PXA. Physicians should be realized multicentric dissemination by meninges or cerebrospinal fluid in PXA patients. It is important to describe the particular case in order to better understanding of clinical features.     

Spinal nerve sheath myxoma (neurothekeoma)

Nerve sheath myxoma (NSM) is a rare, benign tumor of predominantly cutaneous location. They usually arise from small cutaneous nerves in the head, neck, and extremities, but exceptionally they arise from spinal nerve roots. Only three cases of intraspinal NSM have been reported. Herein is describe two additional cases of spinal NSM. The patients were 64-year-old and 31-year-old men who presented with low back pain. Spine magnetic resonance imaging showed small intradural extramedullary masses at the L2-3 level. Both tumors had typical histological features of myxoid-type NSM. The tumors had a strong immunoreactivity for vimentin, S-100 protein, and neuron-specific enolase and focal expression of epithelial membrane antigen and phosphorylated neurofilament. Ultrastructural observation of tumor cells with perineurial, fibroblast-like, and Schwann-cell differentiation suggests an origin from nerve sheath precursor cells.