1/4. Malignant rhabdoid tumour of the third ventricle.A 16-year-old boy presented to the All india Institute of Medical Sciences hospital with signs and symptoms of raised intracranial pressure. A CT scan showed obstructive hydrocephalus from a large posterior third ventricle mass lesion. The patient underwent a subtotal resection of the tumour, followed by radiotherapy. Histological, ultrastructural and immunohistochemical features of the tumour were consistent with primary malignant rhabdoid tumour. The age of presentation and location of the lesion were unusual.- - - - - - - - - - ranking = 1keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
2/4. Multicentric atypical teratoid/rhabdoid tumors occurring in the eye and fourth ventricle of an infant: case report.Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive malignant tumors found in infants and young children. The tumor is characterized by the presence of a rhabdoid cell component in all cases, but the histological origin is still unclear. Recently, germline mutation of the hSNF5/INI1 gene has been reported in association with AT/RTs. The authors report a rare case of an intraocular AT/RT followed by a fourth ventricular tumor. The results of immunohistochemical studies of the surgical specimens revealed the presence of an AT/RT and from this finding the neural origin was inferred. A novel missense mutation of the hSNF5/INI1 gene was demonstrated by dna analysis. High-dose chemotherapy with stem cell rescue was effective in treating this patient. The immunohistochemical relationship between rhabdoid cells and the neurogenic zone, which has not been described in AT/RTs, is of great interest in view of the nature of rhabdoid cells.- - - - - - - - - - ranking = 0.29771203140438keywords = rhabdoid (Clic here for more details about this article) |
3/4. Intraventricular rhabdoid tumor.Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication.- - - - - - - - - - ranking = 0.26049802747884keywords = rhabdoid (Clic here for more details about this article) |
4/4. Atypical teratoid/rhabdoid tumor of the velum interpositum presenting as a spontaneous intraventricular hemorrhage in an infant: case report with long-term survival.Atypical teratoid/rhabdoid tumors (ATRT) of infancy are highly malignant neoplasms that are most common in the first 2 years of life. We present the case of a 3-month-old girl who presented with the acute onset of generalized seizures and was found to have a large spontaneous intraventricular hemorrhage. The blood masked an underlying ATRT of the velum interpositum in the midline of the lateral ventricles and roof of the third ventricle, the first reported case in this location. Serial imaging studies and two ventriculoscopic biopsies were required to establish the diagnosis of the tumor in this unique location and in the midst of an evolving hematoma. After surgical resection, the patient received adjuvant chemotherapy. At 4-year follow-up, the child is neurologically intact, meeting normal developmental milestones, and imaging studies show no evidence of tumor. ATRT were previously associated with an extremely poor prognosis, but more recent evidence with complete surgical resection and adjuvant chemotherapy shows extended survival in some cases, supporting an aggressive and comprehensive approach to give these patients the best chance for a good outcome. Spontaneous brain hemorrhage in a full-term infant requires a diligent and persistent search to rule out an underlying neoplasm.- - - - - - - - - - ranking = 0.18607001962774keywords = rhabdoid (Clic here for more details about this article) |