1/272. central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2]. We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/272. Gastric teratoma in an infant--a case report.Gastric teratoma is an extremely rare tumour. We report a gastric teratoma in a four month old male infant who presented with a large abdominal mass. There is no evidence of recurrence 1 1/2 years after the tumour was excised. Our case is an addition to the few cases reported in the World literature.- - - - - - - - - - ranking = 0.12201648937653keywords = tumour (Clic here for more details about this article) |
3/272. Epignathus: a germ-cell tumour presenting as neonatal respiratory distress.A full-term neonate developed acute upper airway obstruction immediately after birth secondary to a polypoidal mass in the oropharynx. After the child's airway had been secured, the mass was excised and found to be a nasopharyngeal teratoma, a rare congenital germ cell tumour which is frequently associated with other congenital malformations. It is sometimes possible to diagnose these tumours in utero, thereby enabling appropriate precautions to be taken during the delivery, otherwise if the diagnosis is unknown, then it is essential for any attending clinician to urgently secure the airway by means of either intubation or tracheostomy. Such a case is presented with a review of the possible management options. This case emphasises the fact that although many conditions are uncommon, the total incidence of rare conditions is surprisingly high, and that care needs to be taken at all times in the management of patients, in order not to overlook such life-threatening diagnoses.- - - - - - - - - - ranking = 0.36604946812959keywords = tumour (Clic here for more details about this article) |
4/272. Atypical teratoid/rhabdoid tumor of the CNS: cytopathology and immunohistochemistry of insulin-like growth factor-II, insulin-like growth factor receptor type 1, cathepsin d, and Ki-67.insulin-like growth factor (IGF)-II is a potent growth factor, normally controlled by a number of other factors, including IGF binding proteins and IGF binding protein proteases. In general, the latter increase the bioavailability of IGF by cleaving IGF binding proteins. cathepsin d (an IGF binding protein protease) was also implicated in tumor invasion. Although IGF-II was implicated in the pathogeneses of various childhood neoplasms, its significance in the pathogenesis of atypical teratoid/rhabdoid tumor of the central nervous system (ATRT-CNS) was not studied to date. We present clinicopathologic features of two cases of ATRT-CNS. In addition, formalin-fixed, paraffin-embedded tissue sections were stained immunohistochemically for IGF-II, IGF receptor type 1, cathepsin d, and Ki-67. Both tumors demonstrated diffuse strong cytoplasmic positivity for IGF-II, diffuse cytoplasmic and focal membranous positivity for IGF receptor type I, and diffuse cytoplasmic positivity for cathepsin d. The Ki-67 labeling indices were 10.0% and 1.4%. We conclude that ATRT-CNS cells express both IGF-II and IGF receptor type 1, supporting the hypothesis that autocrine/paracrine stimulation of cell growth by IGF-II might be one mechanism involved in ATRT-CNS tumorigenesis. cathepsin d expressed by the tumor cells might also be involved in both tumor cell invasion and growth. The exact pathogenesis of ATRT-CNS remains to be elucidated.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
5/272. Avascular necrosis in patients treated with BEP chemotherapy for testicular tumours.Avascular necrosis (AVN) is known to occur after combination chemotherapy for lymphomas and leukaemias that includes high dose corticosteroids, but it has been reported rarely in patients with solid tumours. We describe five recent cases in young men with testicular tumours (three of which were of good prognosis), who had been treated with chemotherapy using dexamethasone as an antiemetic. dexamethasone is a low cost and effective antiemetic, but it may be responsible for inducing AVN in patients receiving chemotherapy for solid tumours. A prospective survey of the frequency of AVN is justified to quantify the extent of the problem.- - - - - - - - - - ranking = 0.42705771281786keywords = tumour (Clic here for more details about this article) |
6/272. Mature teratoma of the lateral ventricle: report of two cases.In this paper, two cases with mature teratoma of the lateral ventricle are presented. Teratomas are rare intracranial tumours and the most common location is in the midline pineal region. Lateral ventricle as the site of location is very rare. Between the years 1975 and 1996, 120 cases were operated on for lateral ventricle tumours at the University of Ankara, Department of neurosurgery, and only two cases (% 1.6) were histologically identified as mature teratomas. It is generally accepted that benign teratomas are radioresistant and total removal of these tumours results in cure. If mature teratoma of the lateral ventricle is totally removed, as done in our cases, the prognosis is usually good.- - - - - - - - - - ranking = 0.1830247340648keywords = tumour (Clic here for more details about this article) |
7/272. Sacro-coccigeal teratoma: antenatal diagnosis and management.We report a case of sacro-coccygeal teratoma (ScT) in a 29 years old primigravida at 19 weeks of gestation. Ultrasonographic evaluation in antenatal management of these abnormalities is discussed. The main sonographic criteria appear to be two: dimension and rapid growth of the tumour. Cariotype must be performed to exclude aneuploidy, frequently associated.- - - - - - - - - - ranking = 0.061008244688266keywords = tumour (Clic here for more details about this article) |
8/272. Recurrent Wilms' tumour or retroperitoneal teratoma? A challenging case.The clinical and pathological features of a left renal tumour and a subsequent retroperitoneal tumour in a 2-year-old boy are presented. The nephrectomy specimen showed typical features of a triphasic Wilms' tumour with focal heterologous elements and intralobar nephrogenic rests. The tumour was assessed as clinical stage III and post-operative chemotherapy and radiotherapy were administered. A retroperitoneal mass, detected following completion of postoperative therapy, was mainly made up of skeletal muscle and mature adipose tissue. Nests of epithelium resembling ameloblastic nests and a unique structure reminiscent of a developing tooth were present.- - - - - - - - - - ranking = 0.48806595750613keywords = tumour (Clic here for more details about this article) |
9/272. eustachian tube teratoma and its terminological correctness.Teratomas are germ cell tumours usually found in the young and are characteristically composed of tissue foreign to the place where they arise. Two teratomas of the middle ear were first described in 1866 and since then, and to the best of our knowledge, 19 additional cases have been reported in the literature under different terms such as hairy congenital polyps, epidermoid cysts, dermoid cysts, hamartoma, cutaneous teratoma and teratomas. The difficulty in classifying germ cell tumours may explain the different terminologies encountered in the literature. The authors describe a case of teratoma of the eustachian tube presenting as a mass in the middle and external ear of a 10-week-old girl. Using this case, a review of the literature is performed in light of the new classification of germ cell tumours proposed by the world health organization (WHO). It is of note that most of the teratomas that present in the middle ear arise from, or involve, the eustachian tube.- - - - - - - - - - ranking = 0.1830247340648keywords = tumour (Clic here for more details about this article) |
10/272. Anaesthetic management of an infant with anterior mediastinal mass.A substantial mediastinal mass in a small infant can create a dilemma regarding the safest mode of airway management. To ensure safety at all times, we adopted one lung ventilation for fear of compression of the carina and/or both main bronchi. Anaesthesia was maintained at a very light plane by the use of local nerve blocks to secure the airway and epidural analgesia for surgery until the tumour was mobilized.- - - - - - - - - - ranking = 0.061008244688266keywords = tumour (Clic here for more details about this article) |
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