The Role of MRI in the Diagnosis of Spinal Cord Tumors.

Spinal cord tumors are uncommon, and its multiple representatives not always have pathognomonic characteristics, which poses a challenge for both patients and caring physicians. The radiologist performs an important role in recognizing these tumors, as well as in differentiating between neoplastic and non-neoplastic processes, supporting clinical and surgical decision-making in patients with spinal cord injury. Magnetic Resonance Imaging (MRI) assessment, paired with a deep understanding of the various patterns of cord involvement allied to detailed clinical data can provide a diagnosis or significantly limit the differential diagnosis in most cases. In this article, we aim to review the most common and noteworthy intramedullary and extramedullary spinal tumors, as well as some other tumoral mimics, with an emphasis on their MRI morphologic characteristics.

Primary Benign Tumors of the Spinal Canal.

Benign tumors that grow in the spinal canal are heterogeneous neoplasms with low incidence; from these, meningiomas and nerve sheath tumors (neurofibromas and schwannomas) account for 60%-70% of all primary spinal tumors. Benign spinal canal tumors provoke nonspecific clinical manifestations, mostly related to the affected level of the spinal cord. These tumors present a challenge for the patient and healthcare professionals, for they are often difficult to diagnose and the high frequency of posttreatment complications. In this review, we describe the epidemiology, risk factors, clinical features, diagnosis, histopathology, molecular biology, and treatment of extramedullary benign meningiomas, osteoid osteomas, osteoblastomas, aneurysmal bone cysts, osteochondromas, neurofibromas, giant cell tumors of the bone, eosinophilic granulomas, hemangiomas, lipomas, and schwannomas located in the spine, as well as possible future targets that could lead to an improvement in their management.

Cervical intramedullary teratoma: a case report and systematic review of the literature.

Here, we report a case of a 3-year-old female who presented to clinic with an enlarging mass in the posterior cervical midline. The mass was present since birth and demonstrated no cutaneous stigmata. Plain film, CT, and MRI of the cervical spine (C3-C5) revealed enlargement of the spinal canal, soft tissue calcification, spinal dysraphism, and an intramedullary, predominantly fatty, mass. The mass had associated calcifications and a highly proteinaceous cyst. Surgical resection of the spinal lesion was subsequently performed. Histopathological evaluation revealed a mature teratoma. Cervical spinal teratomas in the pediatric population are rare entities with few cases currently reported in the literature. We conducted a systematic review to outline the current evidence detailing cases of intramedullary spinal cord teratomas. Six articles were included for final review. All patients in the included articles underwent maximal surgical resection with one patient also receiving chemotherapy and radiation. With our report, we aim to add to the literature on cervical intramedullary spinal cord teratomas in the pediatric population.

Brachial plexus tumors extending into the cervicothoracic spine: a review with operative nuances and outcomes.

BACKGROUND: The rarity and anatomical complexity of brachial plexus tumors (BPTs) impose many challenges onto surgeons performing surgical resections, especially when these tumors invade the cervicothoracic spine. Treatment choices and surgery outcomes heavily depend on anatomical location and tumor type. METHODS: The authors performed an extensive review of the published literature (PubMed) focusing on "brachial plexus tumors" that identified invasion of the cervicothoracic spine. RESULTS: The search yielded 2774 articles pertaining to "brachial plexus tumors". Articles not in the English language or involving cervicothoracic spinal invasion were excluded. CONCLUSIONS: Recent research has shown that the most common method used to resect tumors of the proximal roots is the dorsal subscapular approach. Despite its association with high morbidity rate, this technique offers excellent exposure to the spinal roots and intraforaminal portion of the spinal nerve. The dorsal approach is used to resect recurrent lower trunk tumors and dumbbell-shaped neurofibromas, yet it is also the least common overall approach used in brachial plexus tumor resections. The ventral or anterior technique is commonly used to resect tumors at the cord to division level, and root to trunk level. Motor complications, transient nerve palsy, and bleeding are among the most common complications of the anterior supraclavicular approach. Further controlled studies are needed to fully determine the optimal surgical approach used to obtain the best outcomes and least complications for each type of brachial plexus tumor.

Giant intradural myxopapillary ependymoma: review of literature.

This report presents the longest spanning intradural myxopapillary ependymoma consisting of 23 vertebral segments in the literature. An 11-year-old boy presented with right arm pain, mid back pain and progressive paraparesis associated with urinary retention. On MRI, the patient was found to have an intradural lesion extending from C5 to S3. The patient underwent T7 and T8 laminectomies with an almost total resection except for a minimal residual adhering to the spinal cord. The patient with the largest spanning spinal cord ependymoma was managed satisfactorily without significant morbidity. A small laminectomy may be used in some occasions despite the tumour's extensive size because it may have a single point of attachment to the cord.

Rosette-forming glioneuronal tumor: an update.

Rosette-forming glioneuronal tumor (RGNT) is a rare and distinct primary nervous system tumor. The literature on this novel neoplasm is sparse and limited to mostly case reports. Reviews on the characteristics of this tumor are fewer and far between with the latest up to a decade old. We thus provide a comprehensive review of recent literature to characterize presenting symptoms, radiological evidence, treatment options, and prognosis of this novel neoplasm. A Medline search for case reports detailing primary rosette-forming glioneuronal tumors was performed. RGNTs are a benign tumor of indolent course with mixed glial and neurocytic components. There is a slight female predominance with mean age of presentation at 23.57 years. Headaches, visual disturbances, and nausea and vomiting are the most common symptoms. Most RGNTs have solid and cystic components, arising most frequently in the fourth ventricle or cerebellum. Management is usually through surgery with gross total resection (GTR) providing better prognosis.

Report of a case of giant cell ependymoma with unusual clinical and pathological presentation.

Giant cell ependymoma (GCE) is a very uncommon variant of ependymoma, known for having varying degrees of nuclear pleomorphism. There are only 34 reported cases of GCE in the English literature. We describe an additional case of a young woman who presented with a tumor located in sacral soft tissue, which was not connected to the spinal cord and did not show additional lesions in the central nervous system. Complete tumor resection was performed and no recurrences or metastasis were detected after 5 months of follow-up. Only one of all the reported GCE was located in the sacral subcutaneous region, where ependymomas are rarely found and usually have myxopapillary histology. Ours is the second report showing microscopic features of GCE in the soft-tissue region. GCE should be considered in the differential diagnosis of lumbosacral subcutaneous tumors to avoid misdiagnosing it as a malignant lesion. Since GCE could be an extraspinal extension of an intraneural ependymoma, it would be important to evaluate whether it is connected to the spinal cord.

Intramedullary cervical abscess mimicking a spinal cord tumor in a 10-year-old girl: a case-based review.

BACKGROUND: Intramedullary spinal cord abscesses (ISCA) are a rare pathological entity with very few cases reported in the world and may be confused with an intramedullary tumor. Cervical location is the least frequently affected, except for cryptogenic spread, mostly seen in adult patients. Meticulous study of contrast-enhanced MRI pictures has a key role in the diagnosis. METHOD AND RESULTS: We present a case of a spontaneous cervical ISCA, mimicking a spinal cord tumor, in a pediatric patient with good outcome. A review of the literature shows that this is a life-threatening condition with high chances of rupture and subsequent meningitis. CONCLUSIONS: We point out that if a solitary cervical spinal cord lesion is surrounded by significant medullar edema associated with fast onset of symptoms, fever, and elevation of WBC, the possibility of a spinal cord abscess should be considered prior to surgery.

Foix-Alajouanine syndrome mimicking a spinal cord tumor.

Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Foix-Alajouanine syndrome mimicking a spinal cord tumor / Síndrome de Foix-Alajouanine simulando um tumor intramedular espinal

Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Resumo Mielopatia necrotizante subaguda (MNS) ou síndrome de Foix-Alajouanine é uma doença rara que se caracteriza por disfunção neurológica progressiva causada por uma fístula arteriovenosa espinal dural. O diagnóstico radiológico é comumente suspeitado quando aparece captação não específica de contraste e de artefatos de fluxo (flow voids) perimedulares. Raramente, a captação de contraste exibe o aspecto em anel, constituindo um grande desafio diagnóstico. Nesses casos, o principal diagnóstico diferencial é um tumor intramedular, e os pacientes são encaminhados para biópsia da lesão, atrasando o diagnóstico definitivo. Relatamos o caso de uma paciente com MNS, a qual foi submetida à biópsia da lesão em virtude de suspeita de tumor intramedular.

Gliofibroma: Report of Four Cases and Review of the Literature.

Gliofibroma is a relatively rare variant of a mixed glial-fibrous tumor more frequent in children than in adults. It has been reported to appear all along the neuraxis, with predilection for the midline. Its evolution is usually benign, although few examples have shown either multiple sites of involvement or leptomeningeal dissemination. Some authors regard it as part of the desmoplastic astrocytoma spectrum. We report here four examples of this rare condition which exemplify its histological patterns and biological behavior, and provide a review of the literature. Even though this tumor is commonly regarded as heterogeneous and with variable course, our literature review points to a set of clinical and pathological traits that are constant, such as age, location and gross and histological characteristics, as well as a predictable evolution. Currently, this tumor is not included in the WHO Classification of CNS tumors.

Meningeal melanocytoma: case report and literature review.

We report a case of a 54-year-old female with progressive chronic pain in lower extremity, paraplegia and loss of function of the anal sphincter. MRI revealed an expansive solid intradural and intramedullar lesion located at the T6 to T9 levels. Histology of the lesion showed melanocytes with the results of immunohistochemistry consistent with a melanocytoma. Melanocytic tumors are rare tumors which present a diagnostic and management challenge for the modern neurosurgeon and neuro-oncologist since MRI and CT features are non-specific and there is scant data to standardise best strategic therapy.

Tanycytic ependymoma in a 76-year-old Puerto Rican male.

Ependymoma is a slowly growing tumor in children and young adults originating from the wall of the ventricles or from the spinal canal that is composed of neoplastic ependymal cells. Tanycytic ependymoma is a rare variant of ependymoma usually arising in the intra medullary spine. The World Health Organization classifies the tanycytic ependymoma as a grade II tumor. The diagnosis of tanycytic ependymoma is challenging since the morphology of the lesions resemble those found in schwannoma and astrocytomas. In the present study, we show a case of a 76 years old male with a progressive paraparesis for 8 years, due to a spinal tumor. Radiological and histological studies were used to classify the tumor as tanycytic ependymoma. Therefore, it is important to be aware of tanycytic ependymoma and its immunohistochemistry profile in older patients, especially within the Caribbean Hispanic population. To our knowledge this is the oldest patient known to have this rare tumor and the first case reported in Puerto Rico.

Disseminated glioneuronal tumor with neuropil-like islands of the spinal cord: a distinctive entity.

BACKGROUND: Glioneuronal tumor with neuropil-like islands (GTNI) was recently added as a novel lesion in the most recent update of the World Health Organization classification of tumors of the central nervous system in 2007. Since this tumor's initial description, approximately 28 cases of GTNI have been published. In this report, we describe the ninth case of a spinal GTNI in the world literature. METHODS: We report a case arising in a 2-year-old female patient who presented with headaches associated with intermittent vomiting due to a tetraventricular hydrocephalus. RESULTS: After ventriculoperitoneal shunt placement, the patient presented with lower extremity motor weakness and sensory disturbance. A dorsolumbar spine magnetic resonance imaging scan revealed an intramedullary spinal neoplasm involving T12 through L2 in association with the thick linear enhancement of the spinal cord surfaces. A brain magnetic resonance imaging scan demonstrated focal leptomeningeal enhancement in the Sylvian fissures, the basal cistern, tentorium, and multiple small cystic-like lesions extending on the cerebellar surface, brainstem, and temporal lobes. The patient underwent a T11-L2 laminectomy for a gross total tumor resection. Histology revealed a World Health Organization grade II GTNI. CONCLUSIONS: GTNI is a rare type of glioneuronal tumor that has recently been described in the literature. The outcome of this case seems to have an unfavorable clinical course despite their low-grade morphology. However, the combination of gross total resection and adjuvant chemo-radiotherapy can enhance chances for longer survival among children with spinal GTNI associated with meningeal dissemination, and a clinical follow-up of a large series will be necessary to evaluate the long-term prognosis.

Mortality is higher in patients with leptomeningeal metastasis in spinal cord tumors.

UNLABELLED: Spinal cord tumors are a rare neoplasm of the central nervous system (CNS). The occurrence of metastases is related to poor prognosis. The authors analyzed one series of metastasis cases and their associated mortality. METHODS: Clinical characteristics were studied in six patients with intramedullary tumors with metastases in a series of 71 surgical cases. RESULTS: Five patients had ependymomas of which two were WHO grade III. The patient with astrocytoma had a grade II histopathological classification. Two patients required shunts for hydrocephalus. The survival curve showed a higher mortality than the general group of patients with no metastases in the CNS (p<0.0001). CONCLUSION: Mortality is elevated in patients with metastasis and greater than in patients with only primary lesions. The ependymomas, regardless of their degree of anaplasia, are more likely to cause metastasis than spinal cord astrocytomas.

Mortality is higher in patients with leptomeningeal metastasis in spinal cord tumors / Mortalidade é mais elevada na disseminação metastática leptomeníngea em tumores da medula espinhal

Spinal cord tumors are a rare neoplasm of the central nervous system (CNS). The occurrence of metastases is related to poor prognosis. The authors analyzed one series of metastasis cases and their associated mortality. METHODS: Clinical characteristics were studied in six patients with intramedullary tumors with metastases in a series of 71 surgical cases. RESULTS: Five patients had ependymomas of which two were WHO grade III. The patient with astrocytoma had a grade II histopathological classification. Two patients required shunts for hydrocephalus. The survival curve showed a higher mortality than the general group of patients with no metastases in the CNS (p<0.0001). CONCLUSION: Mortality is elevated in patients with metastasis and greater than in patients with only primary lesions. The ependymomas, regardless of their degree of anaplasia, are more likely to cause metastasis than spinal cord astrocytomas.

Tumores da medula espinhal são neoplasias raras do sistema nervoso central (SNC). A ocorrência de metástases é relacionada a pior prognóstico. Os autores analisaram uma série de casos de metástases e a mortalidade relacionada. MÉTODO: Foram estudadas as características clínicas em seis pacientes com metástases tumorais numa série de 71 casos operados. RESULTADOS: Cinco pacientes tinham ependimomas e dois dos quais foram grau III pela classificação da OMS. O paciente portador de astrocitoma tinha classificação histopatológica de grau II. Dois pacientes necessitaram de derivação devido à hidrocefalia. A curva de sobrevivência mostrou mortalidade mais elevada no grupo de pacientes com disseminação pelo SNC (p<0,0001). CONCLUSÃO: A mortalidade, além de elevada em pacientes com metástases, é maior do que em pacientes apenas com lesão primária. Os ependimomas, independentemente do seu grau de anaplasia, costumam causar mais metástases do que os astrocitomas medulares.