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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In three brothers of a family with a high incidence of cancer a bilateral renal-cell carcinoma was demonstrated. In the index case there was also a cerebellar haemangioblastoma. At necropsy primary bilateral renal-cell carcinoma was demonstrated while intrarenal metastases were excluded. The cases are considered to be predominantly renal manifestations of the von Hippel-Lindau syndrome with facultative oculocerebellar involvement. Urological and cytogenetic study of other family members provided no evidence of further manifestations of the syndrome. Clinical supervision of members of such families is indicated.
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PMID:[Familial bilateral renal-cell carcinoma and cerebellar haemangioma (author's transl)]. 72 79

There are several urologic disorders which also involve the eye and orbit. We have compiled examples of these and reviewed the literature. Metastasis from genitourinary malignancy (including neuroblastoma), Wilms tumor, Reiter syndrome, tuberous sclerosis, von Hippel-Lindau disease, and oxalosis may all have ophthalmologic manifestations. Urologists need to be aware of these and obtain appropriate consultation in order to fully care for patients with these diseases.
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PMID:Ophthalmologic manifestations of genitourinary diseases. 152 62

We report a case of multiple capillary hemangioblastomas of the liver occurring in a patient with von Hippel-Lindau disease and a history of previous cerebellar and spinal hemangioblastomas. Although rare examples of this tumor have previously been recorded in the pancreas, kidney, and urinary bladder, this appears to be the first recorded case with hepatic involvement. The histology and immunohistochemical appearance of this neoplasm are identical with those of the cerebellar tumor. We believe it represents a separate primary neoplasm rather than metastatic disease.
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PMID:Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease. 189 83

Isolated capillary haemangiomata of the optic disc are uncommon; they may be associated with the von Hippel-Lindau syndrome. Renal carcinoma occurs in almost one-third of patients with this condition, and it may go unnoticed until metastases occur. Early nephrectomy offers a cure. It is important, therefore, that all patients with optic disc capillary haemangiomata as well as their relatives are referred for screening for the stigmata of this disease.
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PMID:Haemangioma of the optic disc. 240 17

In order to evaluate the prognostic factors of renal cell carcinoma (RCC), a retrospective study was carried out on 215 patients (150 men, 65 women) who had undergone surgical treatment from March 1979 to December 1986. The average age of the patients was 58 years (ranging from 15 to 85 years). The ratios of survival to tumor stage and grade, as well as to nodal and venous involvement, distant metastases at the time of diagnosis (as TNM classification) were evaluated by means of the actuarial method (life table). Corrected 5-year survival rates for stage T1-T4 were 100, 81, 68.9 and 0%, respectively. Grade 1 and grade 2 tumors had a 5-year survival rate of 66.5 and 52.8%, respectively; grade 3 had a 3-year survival rate of 46.5%. Nodal involvement at the time of diagnosis had a prediction value for the 3- and 5-year survival rate of 39.8 and 34.5%, respectively. Metastases at time of diagnosis had a 14.1% prediction value for a 3-year survival rate. Tumor extension to the vena cava (21 patients) affected survival in a significant way (55.8% at 3 years). A definite tendency towards lower stage and grade was observed in incidentally diagnosed RCC (11%). In conclusion, stage, grade, nodal and venous involvement as well as metastases at the time of diagnosis have a significant prognostic value in RCC. These data and the lack of early diagnostic methods for RCC suggest the routine use of renal ultrasonography in clinically asymptomatic patients during examination for suspected abdominal diseases and in high-risk patients (Von Hippel-Lindau disease smokers).
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PMID:Prognostic factors in renal cell carcinoma. 321 40

We present our experience with 7 patients with von Hippel-Lindau's disease. Five patients had bilateral renal tumors and 2 had pheochromocytomas. Staging is accomplished best with abdominal computerized tomography and renal angiography. Computerized tomography is the preferred method to follow the patients. Because of the tendency for these patients to have bilateral renal involvement, surgical efforts should be directed toward preserving renal parenchyma without compromising adequate tumor excision. Of the 7 patients 6 are alive 4 months to 8 years postoperatively. One patient had metastatic disease at autopsy. No patient required dialysis or renal transplantation. The relationship between the multifocal renal cysts and renal carcinomas in this disease remains unknown.
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PMID:Urological management of patients with von Hippel-Lindau's disease. 376 31

Six cases of concurrent bilateral renal cancer are reported. These cases represented 3.7% of 163 renal cancers in our institute during the period 1965-1983. Five of the six cases were examined by CT. One earlier case received only selective renal arteriography. Three patients who had extrarenal metastases at the initial presentation showed poor prognoses and died within a year. The other three showed better prognoses. One had bilateral multiple renal cell cancer which was not associated with von Hippel-Lindau disease. One survived 4.5 years after unilateral nephrectomy and chemotherapy. One received unilateral total embolization combined with partial embolization of the contralateral kidney and is still well 1.5 years after initial diagnosis, without evidence of remote metastases. The smaller tumors of bilateral renal cancer were early in stage and were detected by CT. CT is thought to be the best method for detecting small asymptomatic tumors amenable to conservative surgery. Angiography is thought to be essential for the planning of embolization and partial nephrectomy. Although it is an interesting question whether each tumor has a multicentric origin or whether one is a metastasis of another, no criteria seem to exist which are completely satisfactory for making this distinction.
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PMID:Bilateral synchronous renal cancer. 652 38

Pulmonary metastases from a carotid body paraganglioma developed in a patient with von Hippel-Lindau disease. On electron microscopic examination, these metastases consisted solely of chief cells that contained membrane-bound, dense-core granules and formed numerous cytoplasmic processes. These data support the hypothesis that the chief cell is the sole neoplastic cell in paragangliomas. Finally, to our knowledge, this clinical association of a malignant carotid body paraganglioma and pheochromocytoma in von Hippel-Lindau disease is unique.
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PMID:Metastatic carotid body paraganglioma in von Hippel-Lindau disease. An electron microscopic study. 689 35

A total of 65 patients with von Hippel-Lindau disease underwent surgery for renal cell carcinoma (54 bilaterally and 11 unilaterally) at 8 medical centers. Only 1 patient presented with metastatic disease. Radical nephrectomy and nephron sparing surgery were performed in 16 and 49 patients, respectively. Mean posttreatment followup was 68 months. The 5 and 10-year cancer-specific survival rates for all patients were 95% and 77%, respectively. The corresponding rates for patients treated with nephron sparing surgery were 100% and 81%, respectively. Of the latter patients 25 (51%) had postoperative local tumor recurrence but only 2 had concomitant metastatic disease. Survival free of local recurrence was 71% at 5 years but only 15% at 10 years. End stage renal failure occurred in 15 patients (23%): 6 underwent renal transplantation (5 are alive with satisfactory renal function and no evidence of malignancy) and 9 were treated with chronic dialysis (6 are free of tumor). Our results indicate that nephron sparing surgery can provide effective initial treatment for patients with renal cell carcinoma and von Hippel-Lindau disease. These patients must be followed closely, since most will eventually have locally recurrent renal cell carcinoma. When removal of all renal tissue is necessary to achieve control of malignancy, renal transplantation can provide satisfactory replacement therapy for end stage renal disease.
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PMID:Treatment of renal cell carcinoma in von Hippel-Lindau disease: a multicenter study. 775 24

A 51-year-old patient with severe back pain had undergone resection of a benign cerebellar tumour when aged 15 years. In addition, polycystic kidney disease was diagnosed 24 years ago, bilateral phaeochromocytoma 2 years ago, and for 4 months before the present admission he had been on haemodialysis. The family history indicated autosomal dominant inheritance of the polycystic renal disease. His general condition was found to have deteriorated, he had pain on pressure over the upper thoracic and lower lumbar vertebrae, and the kidneys were enlarged on palpation. There were increased concentrations of calcium (3.01 mmol/l), parathormone (2.0 ng/l), carcinoembryonic antigen (13.5 micrograms/l) and TPA (69 U/l). Computed tomography demonstrated cystic and solid parts of much enlarged kidneys. Biopsy revealed a poorly differentiated clear-cell renal carcinoma. Further information concerning the previously removed brain tumour showed this to have been an haemangioblastoma of the cerebellar tonsils indicating the diagnosis of v. Hippel-Lindau disease. Nine other family members had been affected, but none had the full-blown picture of the disease. The patient died 3 weeks later from the rapidly advancing tumour. Autopsy showed the bilateral renal carcinoma, bilateral phaeochromocytoma and metastases to the sternum, femurs, vertebrae and liver.
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PMID:[The von Hippel-Lindau syndrome. Its differential diagnosis from cystic kidneys in adulthood]. 778 10


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