Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemangiopericytomas are mesenchymal tumors and account for about 1% of all CNS tumors. Aggressive growth, tendency to local recurrence and relatively frequent metastases are the clinical features of these tumors. Histological characteristics are marked cellularity, vascularity and a dense net of reticular fibers. This case presents a patient with a local recurrence of a right temporal, atypical meningioma that had been operated on and irradiated elsewhere. After embolization large parts of the tumor were removed palliatively. Histological aspects of the resected tumor were consistent with a diagnosis of an atypical meningioma. Not until hepatic metastases from this tumor were found was the diagnosis re-examined and corrected to a malignant meningeal hemangiopericytoma. Surgical resection of primary tumor with frequent controls and, if needed, postoperative radiation therapy is the treatment of choice. Furthermore metastasizing atypical meningiomas should be examined critically to determine if a hemangiopericytoma is present.
 ...
PMID:[Meningeal hemangiopericytoma with liver metastasis]. 1023 2

Our purpose is to describe the incidence and clinical features of leptomeningeal dissemination (LM) in children with progressive low-grade neuroepithelial tumor (LGN). We have continuously tracked all patients with primary CNS tumors since 1986. Satisfactorily followed data were obtained on 427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998, 177 (42%) of whom developed progressive or recurrent disease. LM was identified in 13/177 (7%). The median age at initial diagnosis was 5 years and at LM diagnosis was 8.5 years. The primary tumor sites were diencephalon (6), brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1). The histologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillary astrocytoma (3), mixed glioma (1), and glioneurofibroma (1). Management included chemotherapy (2) or radiotherapy (3) or both (7); 1 patient received only radical resections of symptomatic lesions. The 5-year progression-free survival rates for patients with localized versus LM disease at recurrence were 22% (95% confidence interval [CI], 13%-25%) versus 15% (95% CI, 0.1%-36%), respectively ( P = 0.28). The 5- and 10-year overall survival rates for patients with localized disease versus LM were 87% (95% CI, 82%-92%) and 83% (95% CI, 77%-89%) versus 68% (95% CI, 39%-91%) and 68% (95% CI, 39%-91%), respectively ( P = 0.05). The 7% incidence of LM is a low estimate because patients were not routinely staged at recurrence. Tumors arising from the diencephalon appeared to predispose to LM; no other predisposing features were identified. We strongly urge that for optimum treatment planning all patients with recurrent LGN be staged with an enhanced spine and brain MRI before adjuvant therapy is initiated. The good survival of patients with LGN and LM reflects a more indolent disease than malignant CNS tumors with LM.
 ...
PMID:Leptomeningeal dissemination in children with progressive low-grade neuroepithelial tumors. 1235 55

Our aim is to evaluate the clinical features and outcomes of children with primary central nervous system (CNS) tumors who develop extraneural metastasis (ENM). We retrospectively evaluated children diagnosed with primary CNS tumors treated at our institution between 1972 and 2004. Of 1,011 patients these tumors, 10 (0.98%) developed ENM. The histopathologic diagnosis was medulloblastoma in six patients, germ cell tumors in two patients, and ependymoma and atypical teratoid rhabdoid tumor (ATRT) in one patient each. In six patients, the primary tumor was located in the posterior fossa; it had a supratentorial location in the patient with ATRT, was located in the sellar and suprasellar region in the two patients with germ cell tumors, and was found in the distal spinal cord in the patient with an ependymoma. In two patients ENM was detected at the time of diagnosis. In other patients ENM developed between 9 and 25 months after diagnosis. Metastatic sites included bone, bone marrow, lung, cervical lymph nodes, liver, and paranasal sinuses. Of the 10 patients who developed ENM, 8 died of their disease 0.27-16.2 months (median, 2.60 months) after it was detected. One patient with dysgerminoma is alive, without disease, 117.80 months after diagnosis of the ENM. One patient with germ cell tumor is alive with disease 11.3 months after diagnosis of the ENM. Systemic metastasis to other extraneural sites is extremely rare in children with intracranial tumors. In our series the rate of ENM is 0.98%. The liver and lung are the most common site for metastasis, followed by the bone and bone marrow. The outcome is poor in patients with CNS tumors with ENM.
 ...
PMID:Extraneural metastasis in intracranial tumors in children: the experience of a single center. 1664 23

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.
 ...
PMID:Hemangiopericytoma in the central nervous system. A study of eight cases. 1830 45

OBJECT Patients having a primary tumor of the spinal cord, spinal meninges or cauda equina, are relatively rare. Neurosurgeons encounter and treat such patients, and need to be aware of their clinical presentation, tumor types, treatment options, and potential complications. The purpose of this paper is to report results from a series of 430 patients with primary intraspinal tumors, taken from a larger cohort of 9661 patients with primary tumors of the CNS. METHODS Extensive information on individuals diagnosed (in the year 2000) as having a primary CNS neoplasm was prospectively collected in a Patient Care Evaluation Study conducted by the Commission on Cancer of the American College of Surgeons. Data from US hospital cancer registries were submitted directly to the National Cancer Database. Intraspinal tumor cases were identified based on ICD-O-2 topography codes C70.1, C72.0, and C72.1. Analyses were performed using SPSS. RESULTS Patients with primary intraspinal tumors represented 4.5% of the CNS tumor group, and had a mean age of 49.3 years. Pain was the most common presenting symptom, while the most common tumor types were meningioma (24.4%), ependymoma (23.7%), and schwannoma (21.2%). Resection, surgical biopsy, or both were performed in 89.3% of cases. Complications were low, but included neurological worsening (2.2%) and infection (1.6%). Radiation therapy and chemotherapy were administered to 20.3% and 5.6% of patients, respectively. CONCLUSIONS Data from this study are suitable for benchmarking, describing prevailing patterns of care, and generating additional hypotheses for future studies.
 ...
PMID:Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. 2059 20

Glioblastoma multiforme (GBM) is a malignant primary brain neoplasm with poor survival. Extracranial GBM dissemination is very uncommon, but is increasingly being recognized. The imaging features of metastatic pleural GBM are not specific for a primary central nervous system neoplasm. In this case, extracranial GBM metastases presented as circumscribed posterobasal pleural nodules. Diagnoses may be established with percutaneous or thoracoscopic biopsy. Radiologists should be familiar with the possibility of the extracranial spread of GBM because as therapeutic improvements provide increased local control of the primary tumor, improving patient survival, extracranial spread of disease will be increasingly encountered on thoracoabdominal imaging studies.
 ...
PMID:Pleural metastatic disease from glioblastoma multiforme. 2063 62

Medulloblastoma accounts for only 1% of all adult CNS tumors. Likewise, recurrence of adult medulloblastoma greater than 20 years after initial diagnosis is extremely rare.We describe a case of adult medulloblastoma with late relapse of disease. The patient was 24 years old when first diagnosed and was treated with total tumor resection and craniospinal radiation. At the age of 45, an enhancing 1.3 cm intradural extramedullary spinal cord lesion at T5 was discovered on MRI. This was presumed to be recurrent medulloblastoma in the form of drop metastasis and the patient was treated with spinal radiation. Several months following treatment, at the age of 46, a follow-up MRI demonstrated an enhancing 1.4 cm intradural extramedullary spinal cord lesion at T7. The lesion was resected and histopathologic examination was most consistent with medulloblastoma, late drop metastasis. Although rare, adult medulloblastoma recurring 20 years after initial diagnosis should always be considered in the main differential diagnosis when working up CNS lesions at or outside the primary tumor site.
 ...
PMID:46-year-old man with a spinal cord mass. 2112 65

The objective of this study was to analyze the characters of the cerebellopontine angle (CPA) metastases from central nerve system (CNS) tumors. Ten patients were reviewed for the period between 2008 and 2015. The clinical and neuroimaging features, and treatment outcomes were analyzed retrospectively. The average period during primary diagnosis through the diagnosis of CPA metastases was 42.4months. Among the 10 cases, the primary tumors and metastases were found simultaneously in 3 cases, the metastases after primary tumor removal were found in 5 cases, and the metastases after stereotaxic radiosurgery were found in 2 cases. Only 4 patients presented with the symptoms and signs associated with CPA involving, one with hearing loss, one presenting facial paralysis, one suffering from tinnitus and one case with dizziness. There were 2 cases with the miliary metastases and 8 cases with massive metastases. There existed 3 cases with single CPA metastases, whereas 7 cases with multiple metastatic foci. Among the 8 cases of massive metastatic foci, 6 tumors presented the solid features and the other 2 cases exhibited cystic and solid features. In this cohort of cases, 4 cases were involved in the bilateral and 6 cases presented unilateral metastatic foci. The three CPA metastases were removed in this group, 6 case performed with radiotherapy, and 5 cases received chemotherapy. In the current group 5 patients have been dead, 3 patients kept stable and 2 cases experienced improvement. In spite of seldom previous reports regarding the metastases from CNS tumors occurring in the CPA are existent, this rare form of the disease should be considered in future evaluation as a differential diagnosis.
 ...
PMID:Metastases in cerebellopontine angle from the tumors of central nerve system. 2844 97

Intra-abdominal metastasis (IAM) of central nervous system (CNS) tumors via ventriculoperitoneal shunt (VPS) is rare but has been previously reported (e.g., germinomas and medulloblastomas). However, there has been no previous report in the literature involving meningiomas. A case of primary rhabdoid meningioma with widespread intra-abdominal carcinomatosis after placement of a VPS in a 36-year-old man is described. The patient underwent preoperative angioembolization of the tumor, craniotomy, and surgical excision, followed by postoperative gamma knife radiosurgery. Five months later, he underwent a decompressive craniectomy and surgical excision for tumor recurrence causing raised intracranial pressure and communicating hydrocephalus, necessitating placement of a VPS. One month after placement of the VPS, the patient developed abdominal distension and confusion. He was treated for a VPS infection, and the shunt was explanted. He continued to deteriorate with high output from the peritoneal drain placed at the time of shunt explantation. An exploratory laparotomy revealed multiple diffuse peritoneal and omental nodules which had the same histopathological and immunohistochemical morphology as the primary tumor. We reviewed the current literature on IAM of primary CNS tumors via VPS, which revealed that patients belonging in the pediatric age group, of the male gender, and with a primary intracranial germinoma or medulloblastoma have a higher incidence of IAM. Majority of IAM occurred within 2 years of VPS placement, and patients most commonly present with abdominal distension and ascites. Treatment after diagnosis is varied and the prognosis is poor, with more than half of the patients dying within a year. It is vital for clinicians to maintain a high index of suspicion for similar patients, as early intervention could potentially improve patient outcomes and patient expectations managed more effectively.
 ...
PMID:Widespread Intra-abdominal Carcinomatosis from a Rhabdoid Meningioma after Placement of a Ventriculoperitoneal Shunt: A Case Report and Review of the Literature. 2949 56

Intra-abdominal metastasis (IAM) of central nervous system (CNS) tumors through ventriculoperitoneal shunt (VPS) is rare but has been previously reported (e.g., germinomas and medulloblastomas). However, there has been no previous reports in literature involving meningiomas. A case of primary rhabdoid meningioma with widespread intra-abdominal carcinomatosis after placement of a VPS in a 36-year-old man is described. The patient underwent preoperative angioembolization of the tumor, craniotomy, and surgical excision, followed by postoperative gamma knife radiosurgery. Five months later, he underwent a decompressive craniectomy and surgical excision for tumor recurrence causing raised intracranial pressure and communicating hydrocephalus, necessitating placement of a VPS. One month after placement of the VPS, the patient developed abdominal distension and confusion. He was treated for a VPS infection and the shunt was explanted. He continued to deteriorate with high output from the peritoneal drain placed at the time of shunt explantation. An exploratory laparotomy revealed multiple diffuse peritoneal and omental nodules which had the same histopathological and immunohistochemical morphology as the primary tumor. We reviewed the current literature on IAM of primary CNS tumors through VPS, which revealed that patients belonging to the pediatric age group of the male gender and with a primary intracranial germinoma or medulloblastoma have a higher incidence of IAM. The majority of IAM occurred within 2 years of VPS placement, and patients most commonly present with abdominal distension and ascites. Treatment after diagnosis is varied, and the prognosis is poor, with more than half of the patients dying within a year. It is vital for clinicians to maintain a high index of suspicion for similar patients as early intervention could potentially improve patient outcomes and patient expectations managed more effectively.
 ...
PMID:Widespread Intra-abdominal Carcinomatosis from a Rhabdoid Meningioma after Placement of a Ventriculoperitoneal Shunt: A Case Report and Review of the Literature. 2968 40


<< Previous 1 2 3 Next >>