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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with renal angiomyolipomas studied angiographically are reported:--A case of tuberous sclerosis with bilateral multiple lesions, renal, hepatic and adrenal;--An isolated lesion whose preoperative diagnosis allowed the excision of the tumor. A review of the literature from 1965 to 1977 identified 242 cases of which 177 were isolated angiomyolipomas. There were 54 cases associated with tuberous sclerosis and one with von Hippel-Lindau disease. The radiologic and especially the angiographic signs are illustrated, the hemorrhagic risks are stressed, and the diagnostic features of fetal renal hamartoma are described.
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PMID:[Angiomyolipomas (hamartomas) of the kidney. Radiological study apropos of 2 cases. Review of the literature]. 72 84

A case is described of a subependymal giant-cell astrocytoma that occurred as a mural nodule within a cyst in the parietal lobe. The tumor recurred twice over a period of 47 years despite two extensive surgical resections. Neither the patient nor any of his children suffered tuberous sclerosis, a disease with which this type of astrocytoma is associated.
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PMID:Recurrent subependymal giant-cell astrocytoma in the absence of tuberous sclerosis. Case report. 75 71

Pathological type complications associated with 46 cases of neurofibromatosis in children under 12 are reported. It is noted that in 65.2% of the cases there are mental retardation, usually serious. More than 50% (24 cases) had some type of tumoration. All were benign with the exception of a suprarenal neuroblastoma that caused arterial hypertension and histological characteristics of malignancy. Fifteen tumors were located in the optica ways, one in the mediastinum, one in the abdomen, one in the paravertebral area, one which was a craneal plexiform tumor and four of the moluscum pendulum type on the eyelids or in neighbouring regions. Twelve children suffered from some type of seizures (Salaam's spasms, tonic-clonic, myoclonic, atonic and versive). Radiological abnormalities were very frequent in the simple X rays as well as in those in which contrast medium was used. In four cases malformations of the midline were observed, three of which were non-communicating cysts of the septum pellucidum, the other agenesis of the corpus callosum. Neurofibromatosis was further seen associated iwth Bourneville's syndrome, Morquio's syndrome, Batten's type of lipofuscinosis, facial or generalized hemihypertrophia and stenosis of the aqueduct. Heredity was dominant autosomic in 16 cases, the rest being due to possible recent mutations.
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PMID:[Pathological complications in 46 cases of neurofibromatosis in children (author's transl)]. 82 74

The authors present a case of pheochromocytoma observed in a 49 year old man suffering from the cutaneous form of von Recklinghausen's disease. In this patient, they noted the constant absence of the inferior abdominal cutaneous reflex on the right side, contrasting with the briskness of the other abdominal cutaneous relexes. From this they concluded that the chromaffin tumor was very probably situated in the right adrenal region. This hypothesis was confirmed by retro-pneumoperitoneum, selective arteriography and finally surgical intervention. After having reviewed the essential medical data from the literature concerning the conditions associated with pheochromocytoma and especially phacomatosis, and having looked at the problem of hypertension related to neurofibromatosis, they make a critical study of the behaviour of the abdominal reflexes in case of pheochromocytoma and emphasize the part that it is possible to draw from this in view of its' topographic diagnosis. The present case is the fourth in which one of the authors has noted the abolition of the inferior abdominal reflex on the side corresponding to the site of the pheochromocytoma.
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PMID:[Pheochromocytoma and Recklinghausen's cutaneous neurofibromatosis. Localizing value of abdominal reflexes in a case]. 82 54

Clinical, catheterization, and pathologic findings were recorded in a newborn infant with tuberous sclerosis and multiple cardiac rhabdomyomas that produced a clinical picture simulating mitral atresia and the hypoplastic left-heart syndrome. The clinical picture was due to a left atrial tumor that completely obstructed the mitral valvular orifice. Even if the diagnosis of left atrial tumor had been made, successful surgical correction was unlikely because of left ventricular rhabdomyomas, which produced severe subvalvular aortic stenosis and did not appear to be resectable. This case demonstrates the possibility that a hamartoma, such as a rhabdomyoma, occasionally can mimic the hypoplastic left-heart syndrome.
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PMID:Cardiac rhabdomyoma simulating mitral atresia. 83 Apr 88

An unusual case of cardiac rhabdomyomatosis associated with a ventricular septal defect without signs of tuberous sclerosis is described. On 3 different angiocardiographies the region of the tumor seemed to be a right ventricular aneurysm. The tumor was identified as such only on operation and its nature defined histologically. Subtotal excision of the tumor and closure of the large ventriccular septal defect and debanding (pulmonary banding at the age of 8 month) was successfully undertaken at the age of 10 years.
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PMID:[Rhabdomyomatosis of the heart and ventricular septal defect (author's transl)]. 83 22

37 cases of cerebral hemispheric tumours are presented. These make up 23,1% of all the intracranial tumours observed over a period of 11 years in a Child Neurology Service. 19 cases were males and 18 females. Their ages were between 16 days and 7 1/2 years. Vomiting and headache were usually the first symptoms followed by seizures, frequently of the focal kind. Motor difficulties used to appear later. 69.6% of the cases presented a malfunctioning focus on the E.E.G. on the side of the tumor. The simple cranial X-Rays showed firstly widening of the fronto-parietal sutures, intracranial calcifications were seen in some of the cases with ependymonas and piloid astrocytoma in patients with Bourneville's disease. Pneumoencephalography as well as carotid angiography, radioisotope examination and computerized tomography gave us very positive results in the localization and determination of the size of the tumor. The ependymomas showed pathological vascularization regularly. The nature of the tumours corresponded to: 14 cases of ependymoma, 8 cases of astrocytoma I and II types, 1 case of astrocytoma of types III and IV, 3 cases of plexus papilloma, 2 cases of meningioma, 1 case of sarcoma of the basal ganglia, 1 case of teratoma, 3 cases of indifferentiated malignant tumours, 4 cases not proven. There was a 20% survival of patients five years later.
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PMID:[Cerebral tumours in infancy (author's transl)]. 90 Jun 62

The familial occurrence of brain tumors are exceedingly rare except in cases with phacomatosis. We encountered pituitary adenomas in two sisters of a family, so far presenting no evidence of multiple endocrine adenomatosis (MEA). Case 1, K. O. a 26-year-old woman was admitted to our Hospital on September 10, 1970 with visual acuity and field disturbance, irregular menstruation and acromegaly. Neurological examination: Her visual acuity was Vd 0.6 and Vs 0.3, visual field was bitemporal hemianopsia, and ther was papilledema bilaterally. She had left exophthalmos and left abducens palsy. Roentgenogram of the skull, brain scanning, cerebral angiogram, pneumoencephalogram suggested the presence of a pituitary tumor. On Sep. 17, 1970, through a left frontotemporal craniotomy the tumor was removed subtotally. The pathological diagnosis was pituitary adenoma (chromophobe). Case 2, M. T. a 31-year-old woman, sister of case 1, was admitted to the Hospital on September 19, 1973, with mild headache, left visual field disturbance and amenorrhea. She had a child, and a past history of pulmonary tbc. Neurological examination: Her visual acuity was Vd 1.2 and Vs 0.03, and visual field of the right eye was temporal lower quandrant anopsia. There was optic nerve atrophy in the left eye. Plain X-ray craniogram, brain scanning, cerebral angiogram and pheumoencephalogram suggested the presence of a pituitary tumor. On Sep. 28, 1973, a right frontal craniotomy was performed. The tumor tissue with capsule was removed subtotally. The pathological diagnosis was pituitary adenoma (mixed type). In the literatures about familial brain tumors with histological diagnosis, glioma and glioblastoma are common, meningioma is relatively rare. Pituitary adenoma with no evidence of MEA is exceedingly rare. The two sisters presented in this paper, have no evidence of hyperparathyroidism, pancreas adenoma and peptic ulcer. So, we consider, at present, these cases should not be field in MEA.
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PMID:[Familial occurrence of pituitary adenoma (author's transl)]. 94 79

Cardiac rhabdomyomas are rare tumors of infancy. In a series of 36 patients 78 percent were under 1 year of age, and only one patient was over age 15 years. Ninety percent of the rhabdomyomas were multiple and occurred with nearly equal frequency in the right and left ventricles. Although reportedly infrequent in the atria, rhabdomyomas involved either one or both atria in 30 percent of patients. In 50 percent of patients at least one of the tumor masses was intracavitary and obstructed 50 percent or more of one of the cardiac chambers or valve orifices. Symptoms referable to obstruction of intracardiac blood flow were present in nine patients, none of whom had tuberous sclerosis, and all of whom would appear to have been good surgical candidates. Histologically the rhabdomyomas were composed of classic "spider cells". Electron microscopic studies revealed scattered bundles of myofibrils ringing these cells and radiating toward the center; glycogen was present both free in the cytoplasm and within mitochondria. Distinct intercellular junctions resembling intercalated discs with well defined desmosomes and nexuses were present. Many of the cells contained leptofibrils, arranged either peripherally or in spiraled clusters in the center of the cell. Rhabdomyomas derive from cardiac muscle cells and appear to represent hamartomas rather than true tumors.
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PMID:Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study. 95 67

Cellular and humoral immunity, and lymphoid organ pathology, have been investigated in 10 institutionalized patients with tuberous sclerosis and 10 institutionalized matched controls without the disease. Type and incidence of infections and tumours were reviewed for each group, as was current medication. Elevated serum IgM levels were found in the patients with tuberous sclerosis, but no immunological deficiency of either cellular of humoral immunity was found, nor was there a difference in infection between the groups. Only patients with tuberous sclerosis had evidence of neoplasia. No morphological or histological abnormalities of lymph nodes, spleen or thymus were present. Explanations for the difference between tuberous sclerosis and ataxia telangiectasia are discussed, together with the effect of immuno-surveillance on the development of malignancy.
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PMID:Immunological status in tuberous sclerosis. 95 13


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