1/62. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/62. Case of the month: January 1999--fetus with echogenic mass in third ventricle.A 29-week gestational age newborn male infant was found to have an echogenic mass in the 3rd ventricle by prenatal ultrasound 2 weeks prior to delivery. At delivery he was poorly responsive and had hydrocephalus and ascites. A CT scan after birth showed cerebral infarction, amorphous tissue in the left hemisphere and numerous calcifications. Despite supportive treatment he died 4 days after birth. Postmortem examination of the brain revealed marked distortion of the architecture and a supratentorial undifferentiated neoplasm consistent with a PNET. The tumor showed extensive areas of hemorrhage and necrosis and involvement of lateral and third ventricles, brain parenchyma, and meninges.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
3/62. glioblastoma multiforme in a mature ovarian teratoma with recurring brain tumours.AIMS: We report a case study to highlight the occurrence of glioblastoma multiforme in an ovarian teratoma. methods AND RESULTS: A 10-year-old girl presented with a left frontal lobe primitive neuroectodermal tumour which was successfully treated. After 6 uneventful years, she developed glioblastoma multiforme located posterior to the site of the initial tumour. Six years later, she presented with a mature cystic teratoma containing glioblastoma multiforme. CONCLUSIONS: glioblastoma in an ovarian teratoma is an exceptional event, which might have an initial clinical presentation as a metastatic brain tumour. Alternatively, recurring glial tumours may occur in a genetically predisposed person; the role of radiation and chemotherapy in this context remains to be elucidated.- - - - - - - - - - ranking = 5keywords = brain (Clic here for more details about this article) |
4/62. Pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes.We have encountered a series of seven unusual neuroblastic pediatric central nervous system (CNS) neoplasms with a unique constellation of histologic, immunohistochemical, and ultrastructural features. The tumors presented in five girls and two boys, ages 1 to 3 years. In six cases the lesions involved the frontoparietal region, in one case the tectal plate. The tumors consisted of small to medium-sized, round to oval, hyperchromatic cells with poorly defined cytoplasmic borders. cells were found in clusters and cords set in a paucicellular fibrillar neuropil matrix. Distinctive, virtually anuclear regions of neuropil were scattered throughout the lesions. True rosettes with well-formed central lumens often filled with granular debris were present, along with perivascular pseudorosettes and occasional Homer-Wright rosettes. Mitoses and apoptosis were frequent, but large regions of confluent necrosis were absent. Immunohistochemically, the neuropil-like areas as well as the perinuclear cytoplasm of many embryonal tumor cells were positive for synaptophysin and neurofilament protein. Ultrastructurally, the tumor cells showed microtubule-containing neuronal processes, some with neurosecretory granules. While the lesions were largely glial fibrillary acidic protein (GFAP) negative, there was focal GFAP positivity consistent with divergent differentiation in one case. The clinical outcome was poor, with five patients dead from their disease 5 to 14 months after initial presentation and one patient with recurrent disease 7 months after resection and chemotherapy. The final patient is alive without recurrent disease 30 months after initial presentation. These lesions present distinctive histological features within the group of primitive neuroectodermal tumors.- - - - - - - - - - ranking = 21.245327341764keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
5/62. Primitive neuroectodermal tumor of the uterus. A case report.BACKGROUND: Primitive neuroectodermal tumor (PNET) is a rare tumor derived from fetal neuroectodermal cells. These tumors occur in the central nervous system and in peripheral locations. Histologic diagnosis is the standard since most of these tumors are detected at an advanced stage. CASE: A 17-year-old female presented with persistent vaginal bleeding. physical examination revealed a 4-cm, hard, barrel-shaped cervix. A cervicovaginal smear was obtained. The specimen was hypercellular, with small to medium-sized, round, malignant cells. A diagnosis of PNET was made from the histologic sections of the surgical specimen. CONCLUSION: When numerous small round cells in a diffuse pattern are seen on a Pap smear, the differential diagnosis is long and difficult. However, with careful evaluation of the cytologic features, a few reasonable differential diagnoses can be reached. Furthermore, with liquid-based Pap smears, material is available for immunohistochemical staining to narrow the range even more. Using all resources, including a good clinical history, a cytopathologist can give the clinician an early diagnosis for intervention and treatment.- - - - - - - - - - ranking = 17.245327341764keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/62. Primitive neuroectodermal tumor of the pancreas. An extremely rare tumor. Case report and review of the literature.Peripheral primitive neuroectodermal tumor (PPNET) is a malignant neoplasm of the peripheral nervous system and soft tissues. Representing the fourth case published we herein report a PPNET arising in the pancreas of a six year old girl. She presented with severe anemia due to ulcerative tumor growth and hemorrhage into the duodenum. From the first biopsy pancreatoblastoma was considered as histological diagnosis. Therefore pancreato-duodenectomy was successfully performed. Immunohistochemically, the tumor cells were positive for cytokeratines and several neuronal markers. Due to focal membranous staining for MIC-2 gene product and rosettes in one lymph node metastasis the diagnosis had to be altered into PPNET. This was confirmed by cytogenetic analysis. We conclude that the interpretation of histologic sample excisions from pediatric pancreatic neoplasms may be difficult and that PPNET should be included in the differential diagnosis.- - - - - - - - - - ranking = 4.0844675339599keywords = nervous system (Clic here for more details about this article) |
7/62. Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.- - - - - - - - - - ranking = 4keywords = brain (Clic here for more details about this article) |
8/62. Primary primitive neuroectodermal tumor of the kidney.Primitive neuroectodermal tumor (PNET) is a small round cell sarcoma that mainly develops in the central nervous system and soft tissues of childhood; however recently, primary occurrence of this tumor in the kidney has been reported. We experienced one case of PNET primarily arose in the kidney without metastasis. The patient was a 28-year-old man whose chief complaint was abdominal pain, especially on exercise. On computed tomography scan and magnetic resonance imaging, a solid lesion was found in the left kidney, and a left nephrectomy was performed based on the diagnosis of a tumor in the left kidney. The tumor was within the parenchyma of lower end of left kidney protruding into the abdominal cavity. Histologically, diffuse proliferation of primitive small round cells with rosette formation was found. Immunohistochemically, MIC2 gene product, neuron-specific enolase and S-100 protein were positive. No metastasis to the regional lymph nodes was found. From these observations, the tumor was diagnosed as PNET primarily arising in the left kidney. Although chromosome analysis was not performed, EWS-FLI1 chimera gene was identified by reverse transcriptase-polymerase chain reaction on the freshly frozen specimen and fluorescence in situ hybridization on paraffin sections.- - - - - - - - - - ranking = 17.245327341764keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/62. Primitive neuroectodermal tumor of cerebrum with adipose tissue.Primitive neuroectodermal tumors (PNETs) of the central nervous system are uncommon embryonal neoplasms, rarely occurring in adults. Differentiation into specific mesenchymal tissues, such as cartilage, bone, skeletal muscle, smooth muscle, or adipose tissue, is rare. We report a case of a 51-year-old woman with a PNET of cerebrum that showed extensive mature adipose tissue differentiation. This is the second case, to our knowledge, of PNET of cerebrum with adipose tissue elements that has been described.- - - - - - - - - - ranking = 17.245327341764keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/62. Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report.Primitive neuroectodermal tumors (PNET) represent about 25% of primary central nervous system tumors in childhood, but congenital PNETs are rare. Cytogenetic studies and studies on molecular pathology have identified several genetic alterations in medulloblastoma, but molecular investigations on supratentorial PNETs are infrequent. We present a male newborn with a large congenital PNET of the right cerebral hemisphere and the molecular analysis of the tumor. Tumor tissue was investigated by routine histology and immunohistochemistry. fluorescence in-situ hybridization was carried out on native tumor tissue to investigate deletions on chromosome 17p and to analyze c-Myc or N-Myc amplifications. Histologic examination revealed a primitive neuroectodermal tumor with massive extension covering almost the entire right hemisphere. Genetic analysis of the native tumor tissue of our patient excluded a deletion of chromosome 17p. An amplification of the c-Myc or N-Myc oncogene was absent using fluorescence in-situ hybridization. Despite unremarkable genetic analysis in our case prognosis was poor, suggesting that there are additional, yet unknown constitutional genetic aberrations in the pathogenesis of congenital supratentorial PNET.- - - - - - - - - - ranking = 17.245327341764keywords = central nervous system, nervous system (Clic here for more details about this article) |
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