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)

Twenty-nine patients with poorly differentiated neuroendocrine tumors of unknown primary site were seen at Vanderbilt Medical Center between May 1977 and February 1987. All patients had pathologic findings typical of neuroendocrine tumors by electron microscopy (22 patients), light microscopy (6 patients), or immunochemical staining (1 patient). Patient age ranged from 18 to 77 years; 21 were men and 8 were women; and 12 had no history of tobacco use. Seventeen patients had metastatic tumor at two or more locations. Common metastatic sites included the retroperitoneum, lymph nodes (particularly cervical), and mediastinum. In most patients, these neoplasms showed an aggressive biology, with rapid onset of symptoms and rapid growth of metastases. Major responses were seen in 18 of 25 patients who received combination chemotherapy; 6 patients achieved a complete response. Three patients remain disease-free more than 24 months after completing therapy. Four patients with single sites of disease received local therapy only; all remain disease-free 1 to 10 years after completion of therapy. In 4 patients, specific tumor types were eventually recognized: small cell lung cancer, 1; gastrointestinal carcinoid tumor, 2; and extragonadal germinal tumor with predominant neuroendocrine differentiation, 1. In the remaining 25 patients, no well-recognized type of neuroendocrine tumor was identified. Recognition of these tumors broadens the spectrum of neuroendocrine neoplasia, and also identifies a distinct subset of patients with poorly differentiated carcinoma of unknown primary site. Although optimal therapy remains undefined, these patients are usually responsive to combination chemotherapy, and long-term survival is attainable in a minority.
 ...
PMID:Poorly differentiated neuroendocrine carcinoma of unknown primary site. A newly recognized clinicopathologic entity. 284 95

Giant cell tumor GCT of bone remains a difficult and challenging management problem because there are no absolute clinical, radiographic, or histologic parameters that accurately predict the tendency of any single lesion to recur or metastasize. Enneking's and Campanacci's radiographic classifications and surgical staging are helpful in planning the initial surgical treatment, because they have observed that a number of the active (Stage 2) lesions and most of the aggressive (Stage 3) lesions have a higher incidence of local recurrence when treated by curettage alone. The bad reputation of curettage and bone grafting is undeserved and arose because of the indiscriminate application of this technique to lesions irrespective of their surgical stage. The ideal aim in the management of GCT is to eradicate the tumor and still save the joint. Curettage, possibly with adjuvant chemical or thermal cauterization, and with bone grafting or polymethyl methacrylate instillation, maintains the structural integrity of the bone and allows for early function. Good results with these techniques when applied to Stage 1 and many Stage 2 lesions may be expected in 70%--80% of the cases. Repetitive freezes with liquid nitrogen, though resulting in a lower recurrence rate, carry with them a not insignificant risk of local complications, require prolonged bracing, and incur the risk of late fracture. When GCTs occur in expendable bones, en bloc resection is the treatment of choice. En bloc resection of major joints requires a facility with reconstruction techniques including the use of allografts, large autogenous grafts and fusion, or custom arthroplasty. These are technically difficult procedures with many early and late complications. Patients have restricted function, and may require prolonged bracing even when uncomplicated. These techniques are therefore reserved for the Stage 3 and selected Stage 2 lesions. Hand lesions have been ineffectively treated by curettage and grafting, and are best treated by early en bloc or ray resection. Multicentric lesions should be handled as individual primary tumors would be in those locations. Radiation therapy has its major role in the treatment of giant cell tumors of the spine and sacrum that are not amenable to complete surgical resection, though long-term sarcomatous change must be looked for. Because of the complex management problem this rare tumor presents, it is recommended that management of giant cell tumor of bone, including the biopsy, the definitive surgery, and the follow-up examination, be carried out by individuals and institutions familiar with this entity.
 ...
PMID:Giant cell tumor of bone. 351 36

Forty-eight selected patients with GCT who were suspected of having residual disease after two or three chemotherapy inductions underwent an attempt at resection of this residual tumor. In 37 patients all gross disease was resected: 11 had malignant tissue, eight adult teratoma, and 18 no residual neoplasm, and 9, 7 and 17, respectively, remain free of disease. Patients in whom complete resection was not possible generally did poorly. Elevated serum tumor markers following the completion of preoperative chemotherapy indicated residual malignant disease and poor probability for complete resection. Twenty-nine percent of patients with negative preoperative markers had malignancy at the time of surgery, but disease was resectable in most of these patients. The key for success is, first, the response to chemotherapy and, second, complete resection of residual disease. It is recommended that patients with initially bulky metastases (diameter greater than 5 cm) be first managed by chemotherapy, employing successive close inductions, and subsequently explored with intent to resect residual disease. When the resected specimen shows malignant elements, the patients should receive additional inductions, otherwise, maintenance chemotherapy is employed.
 ...
PMID:Combined chemotherapy and surgery in treatment of advanced germ-cell tumors. 722 17

GCT is considered a curable tumor with a greater than 90% overall long-term survival. Pulmonary metastasis is common in patients with disseminated disease. First-line therapy in the management of patients with pulmonary metastases from germ cell tumors is cisplatin-based chemotherapy. Pulmonary metastasectomy has an important adjuvant role in a subset of patients who have residual radiographic abnormalities or progression of disease despite optimal chemotherapy. Surgical resection of residual pulmonary and mediastinal disease provides an accurate response assessment and consolidates the chemotherapy by removal of any viable GCT. Therefore, surgical resection of all residual masses is indicated in patients with NSGCT and normalized serum value of tumor markers after definitive systemic chemotherapy. Surgical resection or biopsy is a reasonable alternative in residual seminoma > or = 3 cm in diameter.
 ...
PMID:Medical and surgical management of pulmonary metastases from germ cell tumors. 951 78

The point mutations occurring in codons 12 and 13 of Ki-ras in 78 patients with colorectal carcinoma (31 Dukes' A and B, 21 Dukes' C, and 26 Dukes' D) have been determined by allele-specific oligonucleotide hybridization and sequencing. Duplicate samples of invasive primary carcinoma, adjacent normal tissue, and available lymph node and liver metastases from the same patients were microdissected from paraffin sections. There were no differences in the mutation rate between primary carcinomas and secondary deposits: 26 of 78 (33 per cent) primary carcinomas, 10 of 32 (31 per cent) lymph node metastases, and 10 of 26 (38 per cent) liver metastases. Multiple sampling revealed frequent heterogeneity within carcinomas: 9 of 26 primaries with Ki-ras mutations also contained areas of carcinoma with only the wild-type gene, implying that Ki-ras mutation, even when present in a colonic carcinoma, may not have been necessary for establishing the malignant phenotype. Also, 2 of 26 (8 per cent) Dukes' D patients had a mutation in their primary carcinoma but none in liver metastases and 6 of 47 (13 per cent) Dukes' C and D patients had mutations in liver or lymph node metastases but none in the primary carcinoma. Such heterogeneity may modify the effectiveness of novel therapies targeting mutant Ki-ras function, such as farnesyltransferase inhibition. Mutation of codon 12 from GGT (glycine) to GTT (valine) was more prevalent in primary and metastatic deposits of Dukes' C/D carcinomas (P = 0.01) than in primary carcinomas from Dukes' A/B patients. Mutations of codon 12 to GAT, AGT, GCT and codon 13 GGC to GAC were also found, but no correlation with carcinoma aggressiveness was apparent. Follow-up of 71/78 patients (up to 12 years) revealed decreased overall survival (P = 0.001) in patients with the GGT to GTT transversion in codon 12, even when the analysis was restricted to Dukes' D cases, supporting the suggestion that this mutation may confer a more aggressive phenotype in colorectal carcinoma.
 ...
PMID:Heterogeneity of mutant versus wild-type Ki-ras in primary and metastatic colorectal carcinomas, and association of codon-12 valine with early mortality. 971 38

An unselected population of 635 consecutive extragonadal GCT patients (EGCT) treated between 1975 through 1996 at 11 cancer centers was retrospectively evaluated for clinical prognosis and biological features of this disease. Five hundred twenty-four patients (83%) had a nonseminomatous GCT, and 104 patients (16%) a seminomatous histology; 341 (54%) patients had a primary mediastinal EGCT, and 283 patients (45%) a retroperitoneal EGCT. Following platinum based induction chemotherapy+/-secondary surgery, 141 patients (49%) with mediastinal nonseminomas (median follow up period: 19 months) and 144 patients (63%) with retroperitoneal nonseminoma (median follow up period: 29 months) are alive [p=0.0006]. In contrast, the overall survival rate for patients with seminomatous EGCT is 88% with no difference between patients with mediastinal or retroperitoneal tumor location (median follow up period: 49 months). Multivariate analysis revealed nonseminomatous histology, the presence of non-pulmonary visceral metastases, primary mediastinal GCT location, and elevated beta-HCG as independent prognostic factors for shorter survival. Sixteen patients (4.1%) developed a metachronous testicular cancer despite the use of platinum based chemotherapy. The cumulative risk of developing a MTC 10-years after a diagnosis of EGCT was 10.3% (95% CI=4.9 to 15.6%), but higher among patients with nonseminomatous EGCT (14.3%; 95% CI=6.7 to 21.9%) or retroperitoneal EGCT location (14.2%; 95% CI=5.6 to 22.8%) than among patients with seminomatous EGCT (1.4%; 95% CI=0.0 to 4.2) or mediastinal EGCT location (6.2%; 95% CI=0.1 to 12.2). After a median follow-up of 51 months (range=1 to 154 months), all 16 MTC patients were alive without disease. Patients with pure seminomatous EGCT histology have a long term chance of cure of almost 90% irrespective of the primary tumor site. Patients with mediastinal nonseminomas have a five-years survival rate of 45%. This outcome is clearly inferior compared to patients with nonseminomatous retroperitoneal primaries who have a five-year survival rate of 62%.
 ...
PMID:Extragonadal germ cell tumors: relation to testicular neoplasia and management options. 1275 35

We report different treatment options (currently used and on trial) for a patient with a gastrointestinal carcinoid tumor and metastases in the liver, and discuss the advantages of using internal radiotherapy with 131I-MIBG rather than other treatments. According to the literature, this pathology has a poor prognosis, and considering the significant efficacy of this radiopharmaceutical treatment on symptoms, tumor reduction, and biochemical parameters, it seems to be under used. There are currently no standard treatment options. We present a management strategy for gastrointestinal carcinoid tumors with 131I-MIBG.
 ...
PMID:[Radiation therapy with 131I-MIBG is still relevant for metastatic carcinoid tumors]. 1629 66

The clinicopathologic features of 46 patients with germ cell tumors with sarcomatous components (GCTSC) involving either the primary site or their metastases were studied. There were 43 men and 3 women aged 17 to 74 years. Twenty-three tumors arose in the mediastinum, 2 in the retroperitoneum, and 21 in the gonads. The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1). The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1). Immunohistochemical studies were carried out in 34 cases with appropriate results supporting the diagnoses. Metastases containing both GCT and SC were observed in 6 cases, metastases of SC alone in 4, and metastases containing only GCT elements in 3. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. Clinical follow-up was available in 40 patients (1 to 96 mo; mean=24 mo). Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo). Comparison of these patients with an age-matched and stage-matched control group of patients with GCT without SC showed statistically significant differences in survival between the 2 cohorts (P <or=0.001). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.
 ...
PMID:Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. 1772 Nov 91

Primary giant cell tumor of soft tissue (GCT-ST) arising in breast is exceedingly rare. We report a case of a 60-year-old woman with a primary breast giant cell tumor that appeared histologically identical to giant cell tumor of bone and had a clinically malignant course. The patient presented with a cystic mass of the breast, suspected on imaging to be an organizing hematoma, possibly related to previous injury. Histopathological evaluation revealed a neoplasm composed of mononuclear cells admixed with osteoclast-like giant cells resembling giant cell tumor of bone. Immunohistochemical staining was positive for CD68, smooth muscle actin, and vimentin, but was negative for a panel of epithelial and additional muscle markers. These features were most consistent with GCT-ST, an uncommon neoplasm of low malignant potential. Despite aggressive surgical treatment achieving clear surgical margins, the patient expired with pulmonary metastases within a year of her initial presentation. This case demonstrates the difficulty of predicting clinical behavior of GCT-ST of breast on the basis of histological features and depth of tumor alone. To our knowledge, this is the first case report of a GCT-ST arising in the breast associated with a fatal outcome. The distinction of this entity from other more common primary breast tumors with giant cell morphology is also emphasized.
 ...
PMID:Giant cell tumor of soft tissue arising in breast. 1787 21

Gingival metastases from embryonal carcinoma are very rare and often associated with widespread disease and poor prognosis. Because of their indistinct clinical appearance, they may be difficult to discriminate from more frequent gingival hyperplastic or reactive lesions. The case of a 35-year-old man who presented with a swelling in the left maxillary gingiva, extending from the first premolar to the second molar is reported. This medical history revealed that, 2 years previously, he had been diagnosed with a testicular mixed germ cell tumor (GCTs), for which he had undergone right inguinal orchidectomy and chemotherapy, leading to complete remission. Histology revealed a metastatic embryonal carcinoma. Imaging of the chest and abdomen showed this was the only site of metastasis. He is currently undergoing chemotherapy and responding well. This case draws attention to the multiple diseases that may present as gingival masses and stresses the difficulty of making a correct diagnosis. It is emphasized that in some mixed cases of testicular GCT it may be the more aggressive component that metastasizes, without being clearly apparent.
 ...
PMID:Metastatic embryonal carcinoma in the maxillary gingiva. 1850 54


1 2 3 Next >>