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

A case of female urethral diverticulum is reported by the authors. It is a rare disease and about 31 cases have previously been described. An analysis is made in relation to the diverticulum of the urethra and to female urethral carcinoma. Emphasis is given on the semiology and the recently acquired diagnostic procedures, as these have led to an increased number of recognized cases. In the case presented, an extended resection and a definitive urinary derivation was performed, together with chemo- and radiotherapy. A follow-up of 2.5 years showed no signs of recurrence or metastases.
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PMID:Carcinoma in diverticulum of female urethra. 10 1

Primary soft tissue sarcoma of the retroperitoneum is a rare disease. A series of 11 evaluable adult patients with retroperitoneal soft tissue sarcomas is reported. These patients were treated with complete surgery and adjuvant radiation therapy (total dose from 50 to 64 Gy) using an 18 MeV linear accelerator. After a median follow-up of 48 months (range, 6-84), 4 patients had a local-regional recurrence, 3 had distant metastases, and 4 died of progressive disease. Four-year estimated disease-free survival was 54.5% and overall survival was 70%. Treatment was well tolerated by most patients: 7 patients experienced moderate gastrointestinal toxicity, mainly nausea and diarrhea, during radiotherapy; 2 cases had weight loss > 15% at the end of the therapy; and chronic ileitis was observed in 2 cases. We conclude that adjuvant radiotherapy seems to reduce the incidence of local-regional recurrences in these patients. No radiation-induced irreversible injury was observed, but one young woman had amenorrhea after radiotherapy. Controlled clinical trials are warranted to define the role and effectiveness of adjuvant radiotherapy and/or chemotherapy in retroperitoneal soft tissue sarcomas.
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PMID:Surgical and adjuvant radiation therapy of resectable retroperitoneal soft tissue sarcomas in adults. 129 34

Primary malignant anorectal melanoma is a rare disease. A wide variety of different treatment modalities--local excision, rectal amputation, exenteration of the pelvis with or without lymph node dissection--are proposed and represent the limited experience of the individual clinic. Mean survival of between 7 and 32 months and a 5-year survival between 0% and 12% are reported. The need for histological examination of every pathologic formation of the anorectum is stressed. Local excision is preferable provided the tumor is small, complete excision is possible and tumor thickness is less than 2 mm. Inguinal lymph node dissection can be dispensed with for prophylactic purposes but is standard when metastases are proven. Four own cases are presented and discussed.
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PMID:[Anorectal malignant melanoma]. 156 37

A diagnostic and therapeutic strategy for ZES is proposed based on the vast experience (180 cases) of this rare disease gained at hospital Bichat (Paris, France). The first step is diagnostic and relies essentially a) upon measurements of gastric acid and serum gastrin in the basal state and b) upon the results of secretin test because of the overlap between ZES and duodenal ulcer disease in a large proportion of cases. In sporadic ZES cases, after adequate control of acid overproduction and attempt to localize the tumoral process(es), surgery is indicated (at the exception of patients with advanced metastatic disease). Surgery aims essentially at eradicating gastrinoma(s) as often as possible and whenever it is feasible without endangering patient's life. An apparently definite cure is attained in 60 to 80% in extrapancreatic gastrinoma(s) and 20 to 30% when gastrinomas are located within the pancreas. Liver involvement (25% of ZES cases) remains the major concern and death cause in these patients. Although liver metastases frequently stabilize and sometimes regress upon chemotherapy and chemoembolization, liver transplantation may, in the future, represent the major chance for these patients; but in this frequently slowly evolving condition, the time for liver transplantation is exceptionally difficult to settle.
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PMID:[Which diagnostic and therapeutic approach to the Zollinger-Ellison syndrome should be adopted in 1990?]. 167 64

Adrenocortical carcinoma is a rare disease which is primarily approached surgically. There have been few reports of the efficacy of radiation therapy and, for the most part, these have been anecdotal. This paper reports on the potential adjuvant role of radiation therapy after surgical excision of primary adrenal cortical carcinoma and also comments about the efficacy of palliative radiation therapy for metastases. We have identified eight patients treated for adrenal cortical carcinomas at Hahnemann University Hospital (HUH) from 1962 until the present and have also identified five patients with the same diagnosis at Philadelphia General Hospital (PGH) from 1962 until its close in 1975. These two groups are examined separately. In the PGH group, in which two patients were diagnosed at autopsy and only one patient was treated by radiation therapy, the median survival was between 0 and 1 month for Stage IV disease with the only patient surviving to 6 months being that patient receiving radiation therapy. In the HUH group, five of eight patients were treated adjunctively after diagnosis, one was not and two received palliative therapy. The median survival for treated Stage III patients was between 34 months and 7 years. The suggestion, based on a limited patient series, is that patients treated postoperatively to the tumor bed and nodal areas in Stage III disease may have improved survival over historic series and improved local control.
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PMID:Radiation therapy for adjunctive treatment of adrenal cortical carcinoma. 170 36

Malignant Leydig cell tumors (LCT) are rare. Only 32 cases of malignant LCT (as evidenced by metastatic spread) were reported. Generally metastatic spread occurs within 2 years of the primary LCT, and the patient dies within 2 years of the discovery of metastatic disease. The tumor is highly resistant to both radiation and chemotherapy. It also has a great propensity for recurring after surgical resection. A case is reported of a patient whose metastatic disease occurred 8 years after his primary LCT had been resected. He was treated with doxorubicin and mitotane without response. The clinical features of this case are highlighted, and a review of the literature describing treatment of this rare disease is presented.
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PMID:Treatment of malignant Leydig cell tumor. 191 69

The salivary gland most frequently involved with secondary cancer is the parotid gland. Metastases are responsible for 21-42% of malignant parotid tumors. Malignant melanoma and squamous cell cancer are the two most common tumors to metastasize to the parotid gland. Metastatic adenocarcinoma to this site has rarely been reported. Secondary renal cell carcinoma has been reported in many structures, including the brain, sinonasal tract, lungs, abdomen, genitourinary tract, bone, soft tissue, and lymphatics. Histologically proven parotid metastasis has been previously reported in only one patient. Two patients have recently been diagnosed and treated at our institution for this rare disease. The clinical presentation of each patient was quite different. One patient presented with parotid and pulmonary metastases seven years after resection of a renal tumor. Another patient had resection of a parotid mass revealing an occult metastasis from a renal cell carcinoma. Further evaluation revealed a locally extensive asymptomatic hypernephroma. The survival from the time of discovery of the parotid metastasis was 46 months for the former patient, while the latter patient is alive after 20 months. Differentiation of these tumors from vascular disorders (aneurysm or arteriovenous fistula) required selective angiography and computed tomography. Surgical excision via superficial parotidectomy with facial nerve preservation is necessary for palliation, particularly to avoid massive hemorrhage which may occur upon tumor extension into the oropharynx.
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PMID:Parotid gland metastasis from renal carcinoma. 224 76

Carcinoma of the breast in children is an extremely rare disease, only 44 patients have been reported in the world literature. We report on a case of breast cancer in a 9-year-old girl, who was admitted to the hospital because of a four-month history of a painless mass in the right breast. Clinical examination and mammography resulted in a strong suspicion of malignancy. The histology corresponded to juvenile secretory carcinoma, the histological type without the characteristics of prepubertal cancer cases. Electron microscopy demonstrated intracellular large vacuoles and acini filled with secretory material. The mitotic index was low, the highest measured value was 4 mitotic figures per 10 high power fields corresponding to 2.78 mitotic figures per mm2 of epithelium in the microscope field. Flow cytometry showed that the neoplastic cells were diploid and had a low S-phase fraction. A simple mastectomy including axillary evacuation was performed. No lymph node or distant metastases were found. Postoperative radiation treatment or chemotherapy were not administered. Our patient has been in follow-up for six years without recurrence. In this case simple mastectomy with axillary evacuation seems to have given a cancer-free development into adulthood. Earlier reports have also shown a favourable outcome in these tumours.
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PMID:Carcinoma of the breast in children. 232 26

A case of malignant paraganglioma arising from the posterior mediastinum and presenting with a chest wall metastasis is reported with immunocytochemical and ultrastructural documentation. The pathobiologic features of this rare disease are reassessed with a review of the literature. Cytoarchitectural features do not provide reliable information regarding malignant potential. However, evidence of local invasiveness at surgical exploration and/or lack of initial surgical control are likely to be associated with synchronous or metachronous metastatic disease. Although there are clinicopathologic differences warranting distinction between benign paragangliomas arising in the anterior and posterior mediastinal compartments, there is no apparent pathobiologic difference with regard to the malignant group.
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PMID:Malignant paraganglioma of the posterior mediastinum. A case report and review of the literature. 240 59

Bazex acrokeratosis was found in a 79-year-old man with squamous cell carcinoma of the lung. The psoriasiform skin lesions preceded the tumor diagnosis by 8 months. The symptoms, the course of the disease, and histological findings are presented. Acrokeratosis paraneoplastica is a rare disease observed only in patients either with cancer of the upper aerodigestive tract or cervical lymph-node metastases.
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PMID:[Paraneoplastic acrokeratosis (Bazex syndrome)]. 295 19


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