Intraspinal lumbosacral lipoma: review of literature and report of three cases.
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Biomedical subjects
Publications and source records attributed to L Das.
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A series of 22 infants and children with posterior fossa benign ependymomas treated surgically during the past 12 years is presented. All patients were operated on with posterior fossa craniotomy: visible total resection in 10, subtotal resection in 9, partial resection in 2 and biopsy only in 1. One patient (4.5%) died shortly after surgery. Only 5 patients had documented infiltration of the floor of the fourth ventricle. Postoperative radiotherapy was administered with variable radiation fields and doses. Two out of six patients who had total resection and postoperative radiation therapy did not show recurrence during at least 26 months follow-up period. However, patients with incomplete tumor resection almost invariably developed recurrence. An attempt should be made to remove posterior fossa ependymomas totally at the initial craniotomy. The risk periods for recurrence were between 1 and 2 years after subtotal resection and between 2 and 3 years after total resection. In our experience, gross recurrent tumors appear to be resistant to chemotherapy, and a combination of surgery and radiation therapy does not necessarily prevent recurrence. Newer agents or protocols of adjuvant chemotherapy are needed to explore.
A 40-day-old male infant had massive gastric teratoma. Histologically, it had immature neural elements, among the glial masses. In most of the reported series, the tumor was benign. Malignancy in gastric teratoma is extremely rare.
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Although the results of the National Wilms' Tumor Study suggest poor outcome for children with metastases to intra-abdominal, intracranial, skeletal, skin, diaphragm, or mediastinal sites, the incidence and course of mediastinal disease has not been described. Fifty-four newly diagnosed Wilms' tumor patients at Children's Hospital of Michigan (1972-1980) were studied for patterns of intrathoracic metastases. All had favorable histology; mean age (MA) at diagnosis was 4.5 years. Initial chemotherapy ranged from single agent to three-drug combination (actinomycin, vincristine, adriamycin). Intrathoracic metastases occurred in 14 (26%) from 0-28 months (median, 3.5 months) from diagnosis (MA, 5.8 years). Upon the discovery of intrathoracic metastases all received radiation (1,200-2,000 rads) to the chest, some with supplementary doses to the mediastinum. Five children with parenchymal and one with parenchymal and hilar metastases appearing 0-28 months after the time of diagnosis survive disease-free 44-127 months after metastases appeared (MA, 6.3 years). Eight children developed parenchymal and mediastinal metastases (MA, 5.4 years). Time from diagnosis to parenchymal metastases was 0-7 months; time from diagnosis to mediastinal metastases was 0-16 months; and in four, both sites appeared simultaneously. Death occurred in all eight patients 7-25 months from diagnosis. Only one child had evidence of recurrence outside the chest. Potential risk factors and influence on survival are discussed.
Osteosarcoma very rarely metastasizes to the heart. Thirteen cases have been reported in the literature so far. Diagnosis in some of these cases was made during investigation for severe cardiac failure and in most of them at autopsy. Our patient, a 13-year-old girl, showed right pulmonary metastases on chest x-ray 1 year after above knee amputation for osteosarcoma of the distal femur. Routine preoperative computerized axial tomography (CT Scan) revealed a calcified lesion in the heart in addition to the pulmonary metastases. She was very active and completely asymptomatic. Two-dimensional echocardiography, angiography, and right and left heart catheterizations were done. This revealed a large mobile metastatic lesion in the right ventricle. The intraventricular tumor was successfully removed, and 12 days later she had a second thoracotomy for removal of pulmonary metastases. Nine months after her intraventricular metastasis was removed she developed a solitary right pulmonary metastasis. This was successfully resected. Now, 10 months later, she is disease free and completely asymptomatic.
Two unusual cases of symptomatic Rathke's cleft cysts are reported. In one, the cyst was entirely suprasellar with a normal sella turcica. The gross visual failure, hypothalamic disturbances, and hypopituitarism of the patient resolved after a transfrontal aspiration and partial excision of the wall of the cyst. The second patient was a pituitary dwarf with hypothalamic dysfunction, but with normal vision. The patient had a large intrasellar cyst that exhibited marked suprasellar extension with calcification of the capsular rim. After transsphenoidal aspiration and partial excision of the wall of the cyst, the patient achieved a satisfactory recovery. The differential features between Rathke's cleft cyst and craniopharyngioma are highlighted.
A report of the demonstration of an asymptomatic intracardiac osteogenic sarcoma metastasis by computed tomography. This metastasis was successfully excised along with associated pulmonary metastases. Intensive chemotherapy and aggressive surgical excision of metastatic osteosarcoma lesions have resulted n 40%-60% long-term survival in these patients.
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Primitive neuroectodermal tumor (neuroepithelioma) is a relatively common central nervous system tumor in children. Those arising from a peripheral nerve are extremely rare in childhood. There is only one reported case in 6-year-old where the tumor arose from the sciatic nerve. A case of neuroectodermal tumor of the chest wall, arising from the intercostal nerve, in a newborn is presented. The tumor metastasized to the brain. Prominent Homer-Wright rosettes, with central eosinophilic fibrillar substance similar to that seen in neuroepithelioma of the central nervous system, were present in the primary tumor and brain metastases. Ultrastructure, as revealed by transmission electron microscopy, is also described.
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Ultrasonography was used to examine 81 children with intra-abdominal or retroperitoneal malignancies for tumor extension into the inferior vena cava (IVC). In seven of the 18 patients with Wilms tumors and three of the seven patients with hepatic tumors, the IVC ws sonographically abnormal. Venography was also performed in five patients with Wilms tumors. However, percutaneous transfemoral venacavography did not always allow differentiation between extrinsic compression and intracaval tumor. It is suggested that venacavography, when necessary, should be performed via the brachial vein route and that the catheter be advanced into the right atrium. If an injection in this chamber does not result in enough reflux into the retrohepatic IVC for adequate evaluation, the catheter should be advanced into the IVC and a second injection made. However, ultrasonography is the preferred modality for evaluation of the IVC in children because it is the most accurate method and is noninvasive. Real-time ultrasonography makes it possible to distinguish between tumor extension into the lumen of the IVC and extrinsic compression of the vessel.
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