11/68. Disappearing cystic cerebellar medulloblastoma: the ghost tumour.A case of medulloblastoma, which disappeared following steroid administration and a single sitting of radiotherapy (8Gy) is presented. At surgical exploration tumour was not found, multiple biopsies were negative. Post operative CT scan too did not show the tumour. Five months later, tumour reappeared in left cerebellar lobe in cystic and solid form. Patient was reoperated and tumour was decompressed. The biopsy showed medulloblastoma. To our knowledge, medulloblastomas are not known to be completely responsive to either of these modalities.- - - - - - - - - - ranking = 1keywords = tumour (Clic here for more details about this article) |
12/68. Medulloblastomas of the cerebellopontine angle.It is unusual for a medulloblastoma to occur in the cerebellopontine angle (CPA). Four of the total 63 cases of medulloblastoma managed in our department between 1988 to 1998, presented with a mass in the cerebellopontine angle. There were two children amongst these four patients, aged eight and nine years. The other two patients were young adults. Three patients had primary CPA medulloblastoma, while the fourth case had a recurrence in this region, following the excision of the primary vermian tumour. All the four cases were managed by gross total microsurgical excision and craniospinal irradiation. One of the two children, developed subfrontal recurrence after 1 year and 8 months of initial treatment, while the other child developed a large recurrence at primary site, within three months of post-operative irradiation. However, the adult patient developed CPA recurrence and subsequent cervical intramedullary metastasis within two and a half years of diagnosis. These three cases succumbed to their disease, between three months to thirty months of their initial management. However the fourth patient, a 20 years female with desmoplastic variant, was well at two and a half years of follow up.- - - - - - - - - - ranking = 0.125keywords = tumour (Clic here for more details about this article) |
13/68. Congenital disseminated malignant rhabdoid tumor and cerebellar tumor mimicking medulloblastoma in monozygotic twins: pathologic and molecular diagnosis.Malignant rhabdoid tumors are highly aggressive childhood tumors. Recently, all of the malignant rhabdoid tumors, whatever their location, have been related to the inactivation of the hSNF5/INI1 gene. A subset of cerebral tumors, associated with malignant rhabdoid tumors or isolated ones arising in siblings, showed similar molecular alterations. We report for the first time in monozygotic twins a congenital disseminated malignant rhabdoid tumor in one twin and a cerebellar tumor mimicking a medulloblastoma in the other. Molecular analysis revealed similar alterations for both tumors: a deletion of exon 7 of the hSNF5/INI1 gene in one allele, and a point mutation in the same exon in the other, suggesting a common genetic pathway. Analysis of constitutional dna revealed a germline mutation. These findings are in favor of a common etiology for rhabdoid tumor and a subset of brain tumors developing in infancy.- - - - - - - - - - ranking = 2.7199673033318keywords = rhabdoid (Clic here for more details about this article) |
14/68. Metastatic infiltration of the dental pulp by medulloblastoma.medulloblastoma is an infratentorial primitive neuroectodermal tumour. It is the most commonly occurring brain tumour of childhood, accounting for 15-20% of all paediatric tumours. Extracranial metastasis is rare, but may involve the skeleton. jaw lesions, however, have never been described. A case is reported of metastases of a medulloblastoma to the jaw including the dental pulp.- - - - - - - - - - ranking = 0.375keywords = tumour (Clic here for more details about this article) |
15/68. Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report.Rhabdoid tumors of the central nervous system are rare malignancies. Primary central nervous system atypical teratoid/rhabdoid tumors (ATT/RhTs) mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumormedulloblastoma (PNET-MB) but have different biological behaviors. We report a case of primary intracranial ATT/RhT in the posterior cranial fossa of a child. Preoperative radiological diagnosis was PNET-MB, but pathological diagnosis is ATT/ RhT. The case involved a 16-month-old baby boy who presented with severe headache, vomiting, and gait disturbance. He was treated by surgical resection, chemotherapy, and radiotherapy. Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB.- - - - - - - - - - ranking = 1.5110929462954keywords = rhabdoid (Clic here for more details about this article) |
16/68. Atypical teratoid/rhabdoid tumors.case reports: We describe three cases of atypical ATRT that were identified at the Children's Hospital of Eastern ontario. DISCUSSION: Over the past decade, atypical teratoid/rhabdoid tumors (ATRTs) of the central nervous system have emerged as a distinct entity. This tumor is typically misdiagnosed as a primitive neuroectodermal tumor (PNET)/medulloblastoma. The unique immunohistochemistry profile of an ATRT helps distinguish it from a PNET/medulloblastoma. This is of clinical importance because the prognosis of a patient with an ATRT is worse than that of a PNET/medulloblastoma despite aggressive surgical treatment with or without adjuvant chemotherapy and radiation therapy.- - - - - - - - - - ranking = 1.5110929462954keywords = rhabdoid (Clic here for more details about this article) |
17/68. diagnosis and management of skeletal metastases from cerebellar medulloblastoma.Four cases of cerebellar medulloblastoma with skeletal metastases have been described. Extracranial metastases from this tumour are extremely rare but the skeleton is the site most commonly involved. The metastases did not occur after ventriculo-atrial shunting, but in one of the cases, the diagnosis was made from the check X-ray taken on the day a shunt was introduced. The influence of vincristine sulphate and cyclophosphamide on the radiological appearances and management of the cases has been described. After the diagnosis of skeletal involvement was made three of the children survived over a year with cytotoxic drug treatment; the other case did not respond satisfactory and died from widely disseminated metastases of the central nervous system, 4 months after starting treatment.- - - - - - - - - - ranking = 0.125keywords = tumour (Clic here for more details about this article) |
18/68. Transient loss of speech followed by dysarthria after removal of posterior fossa tumour.The authors report three children who suffered transient loss of speech during six to eight weeks following removal of a large midline cerebellar tumour. None manifested speech difficulties immediately after surgery, but all developed mutism within 24 to 48 hours. The speech of all children slowly but completely recovered, after a period of severe dysarthria. The re-organization of speech functions is discussed in relation to the functioning of musculature.- - - - - - - - - - ranking = 0.625keywords = tumour (Clic here for more details about this article) |
19/68. medulloblastoma with extensive nodularity: a variant occurring in the very young-clinicopathological and immunohistochemical study of four cases.CASE REPORT: Four cases of medulloblastoma with extensive nodularity (MBEN) are described. The patients were 3 years of age or younger at diagnosis. Cranial CT scan disclosed multiple coalescing nodules with peculiar 'grape-like' architecture in three patients. A near total excision of the tumour was performed in all four patients. The patients are currently receiving radiation and chemotherapy. Histologically, the tumours were characterized by extreme nodularity with intranodular uniformity and low proliferative index. The internodular zones were extremely cellular, composed of undifferentiated, mitotically active cells. Bcl-2 protein expression was observed in the cellular zones but was distinctly absent within nodules. DISCUSSION: MBEN represents a variant that occurs in very young children but has a good prognosis. The favourable outcome is probably related to its spontaneous neurocytic differentiation. The pattern of Bcl-2 immunoreactivity in MBENs indicates that this protein could be a key player in the regulation of neuronal differentiation in medulloblastomas.- - - - - - - - - - ranking = 0.25keywords = tumour (Clic here for more details about this article) |
20/68. gliosarcoma occurring 8 years after treatment for a medulloblastoma.CASE REPORT: We present a rare case of a gliosarcoma occurring 8 years following treatment for a medulloblastoma. The patient was diagnosed with a medulloblastoma at the age of 13 years. We considered the possibility of a radiation-induced tumour and present evidence supporting this view. The second cerebral tumour was excised and confirmed to be a gliosarcoma. The tumour bed was re-irradiated with three-dimensional conformal radiotherapy (3DCRT). She remained well for 6 months, after which there was clinicoradiological progression. CONCLUSIONS: During long-term follow-up of patients with medulloblastomas, the possibility of radiation-induced neoplasms must be borne in mind.- - - - - - - - - - ranking = 0.375keywords = tumour (Clic here for more details about this article) |
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