Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Medullary carcinoma of the thyroid develops in the parafollicular C cells, the origin of which is in the neural crest. It is characterized clinically by the presence of motor diarrhea, vasomotor attacks, and immunologically by the presence of an amyloid stroma and the appearance of an anaplastic carcinoma. It is frequently associated with a pheochromocytoma (Sipple's syndrome), parathyroid adenomas and cytaneo mucosal manifestations (phacomatosis, multiple mucosal neuromas). The C cells may secrete thyrocalcitonin and the estimation of the substance may be sometimes used in pre-operative diagnosis and, above all, subsequent observation and examination for the presence of metastases. Treatment is based upon surgery (total thyroidectomy) with lymphnode dissection. Complementary treatment (radiotherapy, chemotherapy, opotherapy) is given only when surgery is inedequate or recurrences occur.
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PMID:[Medullary carcinoma of the thyroid (author's transl)]. 46 19

In 409 sufferers from various malignant tumours, we used the cytostatic Ifosfamide (ASTAZ4942) in fractionated doses. The total i.v. dose averaging 300 mg/kg bodyweight, was either spread over 5 consecutive days (5 X 60 mg/kg i.v.) or over 10 consecutive days (10 X 30 mg/kg). At the same time, most patients were irradiated, the radiation dose usually being only one tenth the antitumour dose. Infections and electrolyte imbalance were first treated before Ifosfamide therapy was instituted. Cases of advanced cerebral sclerosis, thrombopenia below 75,000/cmm, cerebral metastases, impaired renal function and inadequate cooperation of the patient were excluded from the studies. To prevent and control side effects, various premedications and adjuvants are required: Antiemetics, prevention of cystitis and infections, cardiovascular agents etc. Corticosteroids are contraindicated. Out of 360 assessable patients 101 had a full remission, 150 a partial remission, 79 were failures; 30 cases were not evaluated. Good results were seen especially in ovarian carcinoma, mammary carcinoma and microcellular bronchial carcinoma. Particularly striking is the drug's effectiveness in testicular tumours including teratomas, osteosarcomas, chondrosarcomas and myosarcomas as well as in some adenocarcinomas of the gastro-intestinal tract, particularly pancreatic carcinoma. In lymphoreticular tumours and haemoblastoses, its potency is less pronounced. The side effects of Ifosfamide are the same as those of other alkylating agents. They are reversible and can usually be controlled or even avoided by adequate preventive measures. In the order of incidence we observed: Alopecia, leukopenia, fall in haemoglobin, cystitis, intercurrent infections, nausea and vomiting as well as cerebral disorders. Since haemorrhagic cystitis considerably interferes with Ifosfamide treatment, its prevention is of essential importance. Because of possible complications and specific premedication and adjuvant measures for their control, this type of treatment should for the present only be carried out by oncologists or special cancer centres.
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PMID:Results obtained with fractionated ifosfamide massive-dose treatment in generalized malignant tumours. 78 66

In this surgical series of 100 pheochromocytomas (PH), where 91 cases were studied and followed up, the frequency of malignancy appears high (29%) on macroscopic criteria observed at the first operation (25 out 26 cases of malignant PH) or thanks to the occurrence of metastases (1 case out of 26 malignant PH). While some clinical signs are suggestive, there is no excretory profile predictive of malignancy. Isolated or predominant secretion of dopamine is not specific of malignancy event if it occurs more frequently and severely in malignant PH. Bilateral involvement, occurring in the setting of phacomatosis or of multiple endocrine neoplasia (MEN II), or in a sporadic case, is not a predictor for malignancy. Contrarily, extraadrenal involvement is a major feature indicative of malignancy. A CT scan which demonstrates possible locoregional invasion, and a MIGB scan revealing distant localizations raise the suspicion of malignancy. The preferred surgical approach is via a laparotomy, thus allowing the exploration of all possible abdominal chromaffin site and the search for intraabdominal metastases. Intraoperative MIBG scanning checks the completeness of the excision. Flow cytometric DNA ploidy studies have been performed in 25 of our patients. The follow up of benign or malignant PH, after excision "for cure" must be life-long: clinically, biologically and by MIGB scan. Persistence of the disease after surgery or recurrence with unresectable metastases can benefit from MIGB and chemotherapy.
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PMID:High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991. 147 48

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

Major syndromes in which cutaneous and extracutaneous nervous neoplasms are frequently associated include: 1) dysgenetic syndromes or phacomatoses (tuberous sclerosis and neurofibromatosis), 2) multiple schwannoma syndromes (schwannomatosis and Carney's complex), 3) multiple mucosal neuromas syndrome, 4) neurocutaneous pigmentary syndromes (Peutz-Jeghers-Touraine syndrome and neurocutaneous melanosis), and 5) sundry associations (cutaneous meningiomas and cutaneous metastases of neuroblastoma or carcinoid tumors). The early clinical and pathological recognition of these cutaneous neural and pigmentary associated lesions should stimulate the search for centrally located neural or neuroendocrine neoplasms, some of which might be life-threatening.
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PMID:Cutaneous neuropathology: neurofibromas, schwannomas and other neural neoplasms with cutaneous and extracutaneous expressions. 195 52

Between 1979 and 1989 21 renal tumors (8 girls and 13 boys) were diagnosed and treated in the Pediatric Hospital of the University of Erlangen. Additionally, there was evidence of nephroblastomatosis in 5 children with Beckwith-Wiedemann Syndrome and hemihypertrophy. One of these infants developed a Wilms' tumor at the age of 3 1/2 years. The most frequent tumor was the Wilms' tumor, which was diagnosed in 14 children. Wilms' tumor are sonographically well delineated, round or oval tumors which often enclose small cysts (72%) but rarely calcifications (5%) and show inhomogenous liver like echogenicity. Metastasis in liver, spleen or abdomen occurred in 2 infants. The most frequent renal tumor in neonates was the mesoblastic nephroma (3 infants). All mesoblastic nephromas were well delineated round tumors with inhomogenous echo-texture and equal or increased echogenicity in comparison to the liver. They often enclosed small cysts but no metastasis or calcifications. Angiomyolipomas of the kidneys could be diagnosed in two children with tuberous sclerosis. These tumors were echogenic nodules spread all over the kidney. We found multilocular nephroblastomas with multiple irregularly delineated cysts in one child. In an other child multiple renal lymphomas simulating solid tumors with liver-like echogenicity could be found.
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PMID:[Ultrasound differential diagnosis of kidney tumors in childhood]. 217 4

The fate of tuberous sclerosis (TS) patients after renal transplantation (RT) for end-stage renal failure remains to be defined. We report three patients with a posttransplantation follow-up averaging 54 months and review 6 previously published cases. Three women, aged 27-46 years, received a cadaver kidney 26-67 months after starting dialysis. None had mental retardation, 2 had suffered from seizures during infancy and 2 had intracranial calcification; neurological involvement was equally mild in the 6 reported patients. Currently, 16-84 months after RT, our 3 patients are fully rehabilitated with a well-functioning graft (serum creatinine 1.2-1.7 mg/dl). Results of RT are also satisfactory in the 4 other reported cases for whom a follow-up is available, except for 1 death unrelated to the initial disease. Neurologic disorders did not progress. Renal cell carcinoma was discovered in one removed kidney, and cells suggestive of malignant transformation in another case. No metastases were discovered up to 4 years later. No neoplastic transformation was observed up to 7 years after RT in the 3 patients who retained their native kidneys. TS patients with end-stage renal failure are good candidates for RT. The probably small risk of neoplastic transformation of native kidneys warrants a close monitoring by CT scan of the few patients who have not undergone bilateral nephrectomy.
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PMID:Outcome of patients with tuberous sclerosis after renal transplantation. 231 13

The tuberous sclerosis is a rare hereditary disease which mostly becomes manifest in a triad of symptoms: epilepsy, mental defects and skin changes of the adenoma sebaceum Pringle type. There may be an atypical clinical picture without neurological or psychiatric signs, in 50% of cases also without skin changes. Bone changes in tuberous sclerosis are asymptomatic and present in up to 50% of patients. Their finding in patients without basic known symptoms may be the cause of mistaking it for osteoplastic metastases. A case like that was described in the case history part of the communication. The knowledge of the possibilities of these changes in tuberous sclerosis may considerably ease differential diagnosis by suitable indication of computer tomography of the brain with the finding of typical calcifications.
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PMID:[Bone changes in tuberous sclerosis (Bourneville-Pringle disease)]. 263 24

Ganglioneuroblastoma a transitional tumor of sympathetic origin has not yet been described as involving orbit. It is characterized by a mixture of cells ranging from primitive neuroblast to well differentiated ganglion cells within a neurofibromatous tissue. The prognosis is uncertain, as the tumor may either undergo maturation into a ganglioneuroma or may metastasize widely and rapidly as in neuroblastoma. We may postulate a relationship between ganglioneuroblastoma and Recklinghausen's neurofibromatosis in view of the development of the tumor in conjunction with the phacomatosis.
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PMID:[Ganglioneuroblastoma of the orbit]. 392 90

Eight patients with a tumor of the exocrine pancreas were treated or seen in consultation at the Children's Hospital Medical Center over the last 30 years. There were five girls and three boys ranging in age from 15 months to 18 years (average age: 8 years). Tumors were surgically resectable in six of eight cases--three by modified Whipple procedure and three by local resection. The average diameter of tumor was 13.6 cm (range: 3.8-20 cm). There were three adenocarcinomas of duct cell type, two acinar cell carcinomas, two acinar cell adenomas, and a papillary-cystic tumor of low-grade malignancy. One of the adenocarcinomas arose in a field of orthovoltage radiation and another occurred in a patient with tuberous sclerosis. Five of eight patients died, four with regional or distant metastases documented at autopsy. The patient with the papillary-cystic tumor experienced several local recurrences but was alive 14 years after initial surgery. Data from the literature indicate that acinar cell tumors are proportionately more common in children than in adults and have a somewhat better prognosis than tumors of duct cell type. Further reporting of cases with long-term follow-up is encouraged in order to gain clearer perspective of nonendocrine tumors of the pancreas in childhood.
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PMID:Tumors of the exocrine pancreas in children and adolescents. A clinical and pathologic study of eight cases. 630 69


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