Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 59-year-old female was admitted to our institute with coughing. A primary tumor of the diaphragm was suspected by chest X-ray, CT and angiograms preoperatively. Intraoperative findings also suggested a large primary tumor in the diaphragm with invasion to the lung, pericardium and liver. Therefore, we performed partial resection of the diaphragm, lung, pericardium and liver all together through a right thoraco-abdominal approach and the diaphragm was reconstructed using polyglycolic acid mesh. Histologically, the large tumor located mainly in the diaphragm was sarcomatous, with transposition from the carcinomatous cells to the sarcomatous cells. A large cell tumor of the lung was also confirmed. Immunohistologically, the diaphragm tumor was positively stained by keratin and by epithelial membrane antigen. In addition, desmosomes were demonstrated under electron microscopy studies. The tumor of this case was sarcoma in the diaphragm developed from a minor large cell tumor in the lung. Though commonly thought difficult to prove sarcomatous change development from large cell tumor in the lung, we were able to determine this clearly with immunohistology and electron microscopy.
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PMID:[Suspected primary tumor in the diaphragm revealing large cell carcinoma in the lung with sarcomatous change]. 216 24

The chromosomal banding pattern and the in vitro growth characteristics of a metastatic epithelioid sarcoma are described. The cultured tumor cells retained growth characteristics as well as ultrastructural and immunohistochemical properties similar to the cells of the primary tumor. Cytogenetic analysis revealed a modal range in the diploid-hypodiploid region, a finding which was corroborated by quantitative DNA determinations of both the primary tumor and a lymph node metastasis. Fourteen different marker chromosomes were identified. The most frequent clonal rearrangement was a 1p-marker resulting from a short arm terminal deletion, i.e., del (1) (p21-22). A similar 1p- marker has previously been observed in an established epithelioid sarcoma cell line. The finding of an apparently identical 1p-marker in two of two analyzed epithelioid sarcomas suggests that this rearrangement may be a primary cytogenetic abnormality in epithelioid sarcoma. An elevated ras p21 expression was demonstrated using immunohistochemical methods. The possible involvement of the N-ras gene and/or a tumor suppressor in the 1p deletion is considered.
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PMID:A cell culture, chromosomal and quantitative DNA analysis of a metastatic epithelioid sarcoma. Deletion 1p, a possible primary chromosomal abnormality in epithelioid sarcoma. 219 89

From June 1984 to September 1989, 43 patients with large vena caval tumor thrombi from retroperitoneal malignancies underwent surgical treatment with cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). The primary malignancies were renal cell carcinoma (RCC) (n = 39), renal pelvic transitional cell carcinoma (n = 1), adrenal pheochromocytoma (n = 1), and renal (n = 1) or retroperitoneal (n = 1) sarcoma. The level of the caval thrombus was either suprahepatic (n = 27), intrahepatic (n = 14), or subhepatic (n = 2). In all cases the primary tumor and caval thrombus were completely removed. Concomitant procedures included coronary artery bypass grafting (n = 5), pulmonary resection (n = 2), and hepatic lobectomy (n = 1). The time of circulatory arrest ranged from 10 to 44 minutes (mean, 23.5 minutes). There were two operative deaths (4.7%), neither of them due to to the use of DHCA. Major postoperative complications occurred in 13 patients (30.2%). There were no ischemic or neurologic complications and no cases of perioperative tumor embolization. The median postoperative hospital stay was 9 days. Twenty-two patients (51%) are alive and enjoying a good quality of life. The 3-year patient survival rates in patients with localized (n = 24) versus metastatic (n = 15) RCC are 63.9% and 10.9%, respectively (p = 0.02). We conclude that CPB with DHCA facilities excision of retroperitoneal malignancies with large caval thrombi and provides the potential for cure with low morbidity and mortality rates.
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PMID:Experience with cardiopulmonary bypass and deep hypothermic circulatory arrest in the management of retroperitoneal tumors with large vena caval thrombi. 222 13

Seventy-eight patients with soft tissue sarcoma (STS) arising in the distal extremities--wrist, hand, finger, ankle, foot, and toe--who were treated with conservation surgery and radiation therapy were studied retrospectively with respect to survival, local recurrence, functional limb preservation, complications, and distant metastasis. After a median follow-up of 7.9 years, actuarial 5-year and 10-year survival rates were 80% and 69%, respectively, and disease-free rates were 61% and 51% at the same times. Actuarial local control rates were 80% and 74% at 5 and 10 years, respectively. Fifteen patients (19%) had local recurrence, but 12 of these were salvaged. Ultimately, 53 patients (68%) retained a normal or fairly normal extremity, six (8%) needed amputation for complications, and 13 (17%) needed amputation to control recurrent disease. The functional outcome was significantly better for patients with upper extremity lesions than for those with lower extremity tumors; even for the latter, this treatment strategy was preferable to amputation. The incidence of hematogenous metastases from distal extremity sarcomas depends on the size of the primary tumor. It was concluded that conservation surgery and radiation therapy (XRT) is an acceptable treatment strategy for STS arising in distal extremities; it yielded a high rate of disease control and functional limb preservation.
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PMID:Conservative surgery and radiation therapy for soft tissue sarcoma of the wrist, hand, ankle, and foot. 224 88

One hundred seventy-five consecutive patients with soft tissue sarcoma of the limb and limb girdle were studied using univariate and multivariate analysis. The most important factor determining risk of local recurrence was the quality of treatment of the primary tumor, with wide or radical surgery plus radical radiotherapy obtaining the best local control. Tumor size, site, and histologic grade were not predictive of local recurrence. The important tumor variables predicting survival were tumor size at first presentation and histologic grade. Tumors with greater than 10 cm greatest diameter and high-grade tumors carried a poor prognosis. Local recurrence was significantly associated with reduced survival, but only when (correctly) considered as a time-dependent variable in multivariate analysis. Irradiation was also significantly associated with poor survival. Review of the literature indicates that local recurrence is believed to have little influence on survival. Since the relevant randomized controlled trials have been small, the evidence is inconclusive.
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PMID:The influence of local recurrence of extremity soft tissue sarcoma on metastasis and survival. 230 63

Primary host and tumor-related prognostic factors, the occurrence of local recurrence, and the time interval between diagnosis of the primary tumor and metastatic disease were analysed in 39 patients with metastatic Grade 4 soft tissue sarcoma. The pattern of prognostic factors and the timing of metastases were the same in patients with and without local recurrence. Thus, primary prognostic factors alone may determine the risk of metastases in high-grade soft tissue sarcoma; local recurrence per se may not influence the prognosis in these cases.
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PMID:Is there no influence of local control on the rate of metastases in high-grade soft tissue sarcoma? 231 56

BMY-28175 is a novel antitumor antibiotic produced in fermentation by Actinomadura verrucosospora. The cytotoxic effects of BMY-28175 were determined using murine and human tumor cell lines in vitro. Following 72 hour exposure, the drug had IC50 values 1.5 to 13.5 ng/ml in a microtiter assay. BMY-28175 was evaluated for antitumor activity against several experimental murine and human tumor models. The drug administered ip was active against ip implanted P388 leukemia, L1210 leukemia, B16 melanoma, M109 lung carcinoma, C26 colon carcinoma, M5076 sarcoma and Lewis lung carcinoma. In addition, BMY-28175 administered iv was active against iv implanted P388 and L1210 leukemias. BMY-28175 was active against sc implanted B16 melanoma (increased lifespan and/or inhibition of primary tumor growth) in about 60% of the tests. The growth of sc implanted M109 was inhibited by BMY-28175 in a single experiment. BMY-28175 was also active against the MX-1 human mammary xenograft implanted in the subrenal capsule of nude mice. The optimal dose for BMY-28175 in these various studies ranged from 0.16 micrograms/kg per injection with consecutive daily (qd1-9) administration, to 51.2 micrograms/kg with single dose administration. The results of these studies indicate that BMY-28175 is one of the most potent antitumor agents yet observed, with a broad spectrum of activity against tumors of murine and human origin and activity against tumors located distal to the site of drug administration.
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PMID:Experimental antitumor activity of BMY-28175 a new fermentation derived antitumor agent. 234 72

Twenty-one cases in which sarcomas metastasized to the ovaries are reported. The patients ranged from 18 to 79 (average 42) years of age; only five of them were over 50 years old. Eleven tumors were primary in the uterus and 10 outside the genital tract. Three uterine tumors were leiomyosarcomas, and eight, endometrial stromal sarcomas. The extragenital primary tumors were leiomyosarcoma of the stomach (1) and small intestine (2), retrovesical leiomyosarcoma (1), fibrosarcoma of the anterior abdominal wall (1), sarcoma of the mesentery of smooth muscle or neural type (1), hemangiosarcoma probably primary in the heart (1), osteosarcoma of the maxilla (1), chondrosarcoma of the rib (1), and Ewing's sarcoma of the pubic bone (1). The ovarian tumors, most of which were large, were discovered at the same time as the primary tumors in 11 cases; in seven cases, the ovarian tumor was discovered 7 months to 9 years after diagnosis of the primary tumor. In three cases, the ovarian tumors were discovered 4, 7, and 10 months before detection of the primary neoplasm. Two of these tumors were endometrial stromal sarcomas, and one, an epithelioid leiomyosarcoma of the stomach. Eleven ovarian metastases were bilateral. On microscopic examination, the greatest difficulty in pathologic interpretation was posed by the metastatic endometrial stromal sarcomas because of their simulation of sex cord-stromal tumors. Features helpful in their distinction from these tumors included the frequent presence of extra-ovarian disease, bilaterality, and a characteristic content of small arteries resembling the spiral arteries of the late secretory endometrium. The other tumor that caused major diagnostic difficulty was the metastatic epithelioid leiomyosarcoma from the stomach, which had a pattern that initially suggested the solid-tubular pattern of a Sertoli cell tumor.
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PMID:Sarcomas metastatic to the ovary: a report of 21 cases. 237 88

Over the past 10 years there has been significant progress made in the recognition and treatment of soft tissue sarcomas. With the advent of CAT scans and MRI, preoperative delineation of soft tissue tumors has become readily available. The diagnostic use of these modalities in patients presenting with an ill-defined asymptomatic mass has been extremely helpful in terms of screening patients to decide whether or not a biopsy is indicated. These techniques have also provided a much clearer delineation of the anatomic extent of the primary tumor, which has been of great assistance both in radiation therapy treatment calculations as well as in preoperative surgical planning. The recognition that tumor grade is the dominant prognostic variable has resulted in the more common use of a grading system, and a more uniform reporting and stratification of end results. Recent studies with immunohistochemical staining have proven of value in determining the histogenesis of many tumors that in the past were difficult to classify accurately. Most recently the use of flow cytometry suggests that this will also be a valuable adjunct in determining tumor grade and thereby prognosis. The most recent investigations of molecular biologic evaluation of genetic DNA and RNA sequences, as well as of oncogenes are extremely interesting from a diagnostic standpoint and in demonstrating the potential of molecular biologic evaluation for understanding the origin of these tumors. Multimodality therapy with surgery, radiation, chemotherapy, or all three has resulted in a marked improvement in local tumor control for patients with soft tissue sarcomas. The combination of modalities has allowed smaller surgical excisions of the tumor and thereby preservation of the extremity and much of its function. There are currently several different methods of multimodality therapy used including neoadjuvant therapy and postoperative therapy, both of which have been proven efficacious. Chemotherapy is now playing an increased role in clinical investigation and treatment. The availability of Adriamycin, DTIC, cisplatin, and most recently ifosfamide has added several chemotherapeutic agents for use by the clinician. Combination chemotherapy and radiation is of value in the neoadjuvant setting, and several studies are now underway to determine whether postoperative adjuvant chemotherapy is of similar value in reducing systemic spread of disease. Finally, surgical resection of pulmonary metastases has been proven of value in 20% to 25% of patients who subsequently develop metastatic disease. As a result of these advances in several different treatment disciplines, the overall survival rate and quality of life of patients with soft tissue sarcoma have improved markedly over the past 10 years.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Progress in the recognition and treatment of soft tissue sarcomas. 240 92

This is a clinicopathologic study of 6 cases of alveolar soft-part sarcoma. The patients age ranges from 8 to 47 years. All patients are women. In 5 cases the localization of the primary tumor is the lower extremities. Histologically the tumors have a distinctive organoid pattern outlined by thin-walled capillaries and are composed of nests of large polyhedral cells with abundant finely granular, acidophilic cytoplasm. Two tumors, among the 4 tested cases, have cytoplasmic PAS positive diastase-resistant crystalline structures. Electron microscopic study of one tumor shows intracytoplasmic glycogen, small membrane-bound electron-dense granules, Golgi lamellae and crystalline structures. Immunoperoxidase study performed in one case reveals a positivity with antikeratin and anti-enolase (NSE) antibodies. The prognosis of this tumor is poor. The main metastasis are in the lung and in the brain. Often the patients develop metastasis before detection of the primary tumor. The histogenesis of alveolar soft part sarcoma and the identity of the characteristic crystalloids remain open for discussion.
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PMID:[Alveolar soft part sarcomas. Apropos of 6 cases and review of the literature]. 241 26


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