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

In an audit of 17,417 necropsies from 1939 to 1989 at the Royal Women's Hospital, Melbourne, Victoria, 46 congenital tumors comprising 24 teratomas, 8 vascular tumors, 6 neuroblastomas, three rhabdomyomas, two mesoblastic nephromas, one thyroid adenoma, one hepatic adenoma and one cerebellar medulloblastoma were found. The teratomas were generally large tumors and, because of their size and location, incompatible with extrauterine life. Ten teratomas were associated with polyhydramnios, three with obstructed labour, five of the fetuses were hydropic and four had malformations at sites distant to the tumor. Twenty teratomas occurred in stillbirths, half of whom were macerated. The vascular tumors were associated with polyhydramnios in three cases and hydrops fetalis in two. The neuroblastomas were all incidental findings. Four were intra-adrenal, one was an adrenal tumor which had metastasized to the liver and the remaining case was a small paravertebral lesion. Two of the three rhabdomyomas were multiple and one of these occurred in a case of tuberous sclerosis. One of the mesoblastic nephromas occurred in a hydropic fetus who had the Arnold-Chiari malformation. The thyroid and hepatic adenomas were small incidental lesions. The cerebellar medulloblastoma had led to marked hydrocephalus. Congenital tumors have different incidence, presentation and behaviour than those in childhood or adult life. The association of congenital tumors with congenital malformations and hydrops fetalis should always be remembered.
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PMID:Survey of congenital tumors in perinatal necropsies. 128 65

After a short review of phakomatosis nosology, the paper deals with 3 clinical cases, out of which two of Recklinghausen neurofibromatosis and one of tuberous sclerosis (Bourneville's disease), all of them with pulmonary determinations, confirmed bioptically and histopathologically. A "florid" cutaneous syndrome (including all the variants of skin lesions) dating from childhood, subsequently accompanied by phenomena of psychical involution and behaviour troubles were present in two cases; retinal lesions were also associated in the third case. Visceral intrapulmonary determinations (i.e. diffuse interstitial fibrosis, and even bronchopulmonary neoplasm in one case) were registered in all cases, too. The diagnostic importance of investigating all apparatus, systems and organs which can be jointly involved in phakomatosis is emphasized. The importance of an inquiry regarding family history is also noticed. For the studied cases, the contribution of complementary (psycho-neurological, ophthalmological, orthopaedic a.s.o.) examinations--though quite valuable--took a back seat in comparison with the essential elements provided by the dermatological and histopathological examinations.
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PMID:[The respiratory manifestations in the picture of phakomatosis]. 129 1

Schwannomatosis is a rare disorder, still not quite well defined, seldom described in the literature. In this paper we report the case of male. Patient, 52 years old, who in the last 30 years developed five subcutaneous tumors within his limbs peripheral nerves, which histologically proved to be schwannomas. A brain computed tomography showed a partially calcified tumor in the left temporal lobe which most likely was a meningioma. A thorough clinical examination was unable to find signs of type I or type II neurofibromatosis. The present condition, probably a form of phacomatosis, has to be distinguished from neurofibromatosis and is considered as an independent clinical entity whose origin still awaits further detailed investigations.
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PMID:[Schwannomatosis: report of a new case]. 130 64

Although infantile spasms were initially described in 1841, remarkably little progress has been made in understanding the pathophysiology of this "peculiar form of infantile convulsions." Consequently, our ability to treat infantile spasms is limited. Infantile spasms are classified as a "generalized" seizure disorder in the international classification system, which suggests that the underlying brain abnormality causing the seizures also must be diffuse or generalized. As the classification suggests, there are many diffuse, or multifocal, brain disorders related to infantile spasms, e.g., inborn errors of metabolism, hypoxic-ischemic brain injury, and developmental brain defects such as tuberous sclerosis or Aicardi's syndrome. On the other hand, infantile spasms have been reported in which a localized brain abnormality was present, e.g., tumor, stroke, and trauma. On rare occasions, removal of a tumor has resulted in cessation of the generalized infantile spasms. This finding suggests that focal cortical abnormalities can cause infantile spasms and that removing the abnormality can stop the seizures. At University of California, Los Angeles, the Pediatric Epilepsy Surgery Program has developed new approaches to the treatment of infantile spasms. The principal underlying concepts are (a) children with medically refractory infantile spasms may have an area of cortical defect (called the zone of cortical abnormality) that causes the seizures and (b) infantile spasms are usually generalized seizures. Thus, the goal of the surgical assessment is not the identification of the focus of seizure onset but rather the identification of the zone of cortical abnormality.
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PMID:Treatment of infantile spasms: medical or surgical? 133 May 8

The authors report on a case of tuberous sclerosis diagnosed in the neonatal period on the basis of intracardiac tumor, rib anomalies and cerebral calcifications. At 4.5 months of age the infant presented acute abdominal pains which led to the discovery (ultrasound identification confirmed by CT scan) of a giant ectasia of the whole abdominal aorta. The infant died 2 days later from the rupture of this aortic aneurysm.
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PMID:[Abdominal aortic aneurysm and Bourneville's tuberous sclerosis]. 133 62

Dysplastic gangliocytoma (Lhermitte-Duclos disease) is a rare entity. Usually presenting as a posterior fossa mass, dysplastic gangliocytoma is not a true neoplasm but a hard-to-characterize lesion that may represent an abnormality of cell migration or a phacomatosis. Previous reports of CT findings are rare in the radiologic literature, and high-field (1.5 Tesla) MR images have never been described in the pediatric age group. We present a case of dysplastic gangliocytoma in a one-year-old boy with CT and MR findings.
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PMID:Dysplastic gangliocytoma (Lhermitte-Duclos disease): CT and MR imaging. 140 48

We prospectively analyzed MR studies of 32 patients with intractable complex partial seizures, who later underwent resection of EEG-proven seizure foci. Twenty-seven patients were imaged at 1.5, 4 at 0.35, and 1 at 0.5 Tesla. Correlation was obtained on all patients with EEG and surgical pathology. Of 19 patients with mesial temporal sclerosis (MTS), 13 exhibited an MR abnormality at the site of pathology as determined by EEG and surgery. Abnormalities included increased (8) or decreased (1) signal intensity, distortion of gray-white interface (1) and temporal lobe atrophy (3). Six patients had no abnormalities at the site of the diseased focus. MR was more sensitive in patients with other structural lesions which included tumor, encephalitis, polymicrogyria, cryptic vascular malformation, and tuberous sclerosis. Prior studies indicate some usefulness of MR in intractable seizure patients, but many report relative insensitivity of MR in defining an abnormality (as low as 11%). Our results demonstrate the usefulness of high-resolution thin-section multiplanar MR using cardiac gating or flow compensation techniques in this patient population.
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PMID:Intractable complex partial seizure: correlation of magnetic resonance imaging with pathology and electroencephalography. 141 38

Periungual fibromas may be acquired lesions or they may be associated with tuberous sclerosis or von Recklinghausen's Disease. Such tumors are comparatively rare benign dermatological entities. Periungual fibromas associated with the posterior nail fold have a potential for inducing deforming nail deformity. Pain resulting from the growth is due to concentration of pressures which may be placed on the nail matrix and contiguous soft tissue structures. This is further complicated by compression developed within the toe box of the shoe. The preferred treatment in symptomatic cases is that of complete surgical excision of the entire lesion. The present study presents such a case, together with histopathological features of this type of growth, with special emphasis on the subcellular features of the neoplasm.
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PMID:The cytological and biochemical implications of periungual fibroma. 143 Aug 31

Five patients were diagnosed as having angiomyolipoma. Evidence or signs of tuberous sclerosis were observed in no patient. In 2 patients the presenting symptoms was massive retroperitoneal hemorrhage which required emergency surgery. The kidney could be preserved in 4 cases. One underwent nephrectomy for synchronous clear cell renal tumor. CT and US afford a diagnostic accuracy of nearly 100 percent. According to the data reported in the literature, tumors larger than 4 cm are amenable to treatment by conservative surgery. Smaller tumors can be followed carefully. However, surgical exploration is warranted not only in patients with pain and hemorrhage, but also in those cases with complex lesions less than 4 cm.
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PMID:[Renal angiomyolipoma: a cause of massive retroperitoneal hemorrhage]. 146 78

Renal angiomyolipoma, a rare benign renal neoplasm constitutes less than 3 percent of all renal neoplasm. Two rare cases of renal angiomyolipoma were reported from the Department of Pathology, Dr. S.N. Medical College, Jodhpur in last fifteen years. Both were not associated with tuberous sclerosis. The relevant literature on the subject is reviewed.
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PMID:Renal angiomyolipoma (report of 2 cases). 151 29


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