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Biomedical subjects

W A Albano

Publications and source records attributed to W A Albano.

At least 19 recordsLinked to original sources
PubMed1985

Hereditary nonpolyposis colorectal cancer (Lynch syndromes I and II). I. Clinical description of resource.

Hereditary nonpolyposis colorectal cancer (HNPCC) is comprised of the following: the cancer family syndrome (CFS), or Lynch syndrome II, which shows early-onset proximal colonic cancer predominance and other associated extracolonic adenocarcinomas, particularly endometrial carcinoma; and hereditary site-specific colon cancer (HSSCC), or Lynch syndrome I, which shows all of the same characteristics, except for extracolonic cancer. Nine families with CFS and two with HSSCC provided the resource that was tested for biomarkers (see companion article). All families were meticulously evaluated for genealogy and cancer verification. Biologic specimens were obtained during field visits to areas of closest geographic proximity to the families. Cancer education and recommendations for surveillance/management were provided to patients and their physicians. Additionally, 40 families (about 3000 individuals) with either CFS or HSSCC have been ascertained. Syndrome cancers were restricted to direct-line relatives as opposed to nonbloodline relatives, arguing against involvement of environmental factors. One documented clinical feature was a predilection for proximal versus distal colonic cancer in both CFS and HSSCC kindreds. This has important clinical significance in that it clarifies the need for instituting effective surveillance earlier to detect the predominantly proximal colonic cancers.

Adolescent↗
PubMed1985

Hereditary nonpolyposis colorectal cancer (Lynch syndromes I and II). II. Biomarker studies.

Nine families with the cancer family syndrome (CFS), or Lynch syndrome II, and two with hereditary site-specific colonic cancer (HSSCC), or Lynch syndrome I, were investigated for the following potential biomarkers of genotype status: in vitro tetraploidy of dermal fibroblast monolayer cultures; tritiated thymidine uptake (3HdThd) labeling of colonic mucosa; cytogenetics of peripheral blood mononuclear leukocytes; quantitative serum immunoglobulin determinations; methionine dependence in dermal fibroblasts in tissue culture; segregation analysis; and the study of gene linkage with respect to 25 landmark serum and blood group markers. Positive lod scores of 3.19 for linkage of the Jk (Kidd blood group) with CFS were obtained. Both in vitro tetraploidy and 3HdThd uptake in the distal colonic mucosal crypt compartments were positively associated with cancer risk status in CFS and HSSCC kindreds. There was a high incidence of polymorphisms of centromeric heterochromatin, including complete inversion. These findings are of particular clinical and genetic significance because HNPCC lacks premonitory signs of cancer risk. If confirmed, they could conceivably enable definition of genotype as early as birth in members of HNPCC kindreds, thereby enabling psychologic preparation and intensive cancer education for improved compliance in surveillance/management programs. These studies also provide new clues about the chromosome(s) bearing the presumed cancer gene(s). For example, CFS gene(s) may possibly be located on chromosome 2, where Jk is located. These biomarkers merit intensive study in additional HNPCC kindreds for a more complete assessment of their sensitivity and specificity. Additionally, essential aspects of previous reports involving biologic samples from these and/or similar subject kindreds are included to permit a comprehensive presentation of the combined findings of this consortium to date.

Colon↗
PubMed1984

Genetic predisposition to breast cancer.

Breast cancer risk factors are closely intertwined with the patient's cultural background, which may contribute to breast cancer aggregations within families. The difficult questions are: (1) does a truly hereditary breast cancer subset exist; (2) which familial aggregations are hereditary; and (3) is the hereditary form distinctive from its sporadic counterpart? These queries will be resolved once biomarkers are identified that show high sensitivity and specificity with genotype. The authors provide a review of this subject and will focus on their recent discovery of increased in vitro hyperdiploidy in cultured skin fibroblasts from patients with or at risk for hereditary breast cancer. The authors discuss findings from their study of family histories in 225 consecutively ascertained patients with verified breast cancer from the Creighton University School of Medicine Oncology Clinic. Findings consistent with an hereditary breast cancer syndrome were identified in 5% of the patients. Given the 112,000 new cases of breast cancer in the United States in 1982, the authors estimate that with a confidence coefficient of 0.95 between 2410 and 8790 of these individuals will manifest hereditary breast cancer. Specific surveillance/management programs should be geared to high-risk members of these families in which cancer yield will be predictable.

Adolescent↗
PubMed1984

Genetics, biomarkers, and control of breast cancer: a review.

More has been written about the epidemiology of breast cancer than possibly any other form of cancer affecting mankind. However, in the face of this intense interest, only a paucity of attention has been given to the role of genetics in its etiology. This review represents an attempt by the investigators to provide a comprehensive coverage of hereditary breast cancer. Included are pertinent endogeneous and exogeneous risk factors, which in certain circumstances, may significantly influence the role of primary genetic factors. Hereditary breast cancer is heterogeneous. When discussing the subject, therefore, one must be precise relevant to the particular heterogeneous form of concern, based on differing tumor associations. It is probably not appropriate to discuss "hereditary breast cancer" without qualification of the specific hereditary breast cancer syndrome of concern; i.e., the SBLA syndrome, breast/ovarian cancer syndrome, and others. This reasoning also applies to attempts at linking biomarkers to hereditary breast cancer. Finally, in addition to ongoing discussions on the cardinal principles that associate with hereditary forms of breast cancer, its frequency, and new developments in biomarkers, we have provided surveillance/management programs that embrace those facets of the natural history of this disease.

Adult↗
PubMed1984

Hepatic artery infusion: surgical approach.

Hepatic artery infusion chemotherapy for the treatment of nonresectable liver cancer is gaining popularity among the medical community. A lack of knowledge of the variants in the anatomy of the arterial blood supply to the liver may lead to inadequate hepatic perfusion, or inadvertent perfusion of extrahepatic organs. A review of 70 hepatic artery angiograms on patients referred to the liver program at the Creighton Cancer Center was performed to integrate angiographic findings, surgical techniques, and clinical decision making.

Angiography↗
PubMed1984

Breast cancer, genetics, and age at first pregnancy.

Hereditary breast cancer shows a distinctive natural history characterised by an earlier age of onset, excess bilaterality, vertical transmission, heterogeneous tumour associations, and improved survival when compared to its sporadic counterpart. To date, very little attention has been given to interrelationships between breast cancer risk factors and genetics. In the general population, early age of first term pregnancy has been generally accepted as protective against breast cancer. In addition, recent findings suggest that an early age of first pregnancy may be associated with an earlier age of breast cancer diagnosis. We studied the age at first pregnancy and age at onset of breast cancer among 162 females at 50% genetic risk, 72 of whom had already developed the disease. We then compared them to 154 consecutively ascertained breast cancer patients from the Creighton Cancer Center. In the hereditary subset (1) early first term pregnancy did not alter the frequency of breast cancer; (2) early age at first term pregnancy was not associated with an earlier age at cancer diagnosis; and (3) age of breast cancer onset in nulliparous females was not significantly lower than that in females having at least one term pregnancy. We speculate, therefore, that in our hereditary population, pregnancy does not influence the natural history of breast cancer in the same way that it does in the population at large.

Adult↗
PubMed1983

Phenotypic variation in the familial atypical multiple mole-melanoma syndrome (FAMMM).

The familial atypical multiple mole-melanoma syndrome (FAMMM) is characterised by an autosomal dominantly inherited susceptibility to multiple atypical moles which show variable colouration ranging from black to brown, tan, red, or pink, with occasional variegation. These compound naevi may be macular or papular, with regular or irregular borders, and measure 1 cm or more in size. They may be few in number or absent or may exceed 100 in a given patient. They are located predominantly on areas not exposed to the sun. Dysplastic changes in melanocytes, fibroplasia, focal chronic inflammatory cell infiltrate, and new blood vessel formation of the papillary dermis characterise their histopathology. These findings are not uniformly present. Because of these distinctive features, coupled with their propensity for transformation to cutaneous malignant melanoma, little attention has been given to the possibility of either minimal or absent cutaneous expression of the phenotype or more diverse neoplastic involvement in this disease. These latter phenomena, which we ascribe to the pleiotropic effects of the cancer-prone FAMMM genotype, were observed in a single FAMMM kindred, the subject of this report.

Adult↗
PubMed1982

Natural history of hereditary cancer of the breast and colon.

The natural history of 106 patients from eighteen families manifesting hereditary breast cancer syndromes, and 117 affected patients from twenty families manifesting nonpolyposis hereditary colon cancer were evaluated. Findings were compared with the American College of Surgeons (ACS) long-term audits for breast and colon cancer respectively. The cardinal features of hereditary cancer were observed within the study group, including: (1) a significant younger age of onset (49 years, breast; 46 years, colon); (2) an excess of proximal lesions in the hereditary colon series (49%); and (3) an excess of bilaterality in the hereditary breast cancer patients. The clinical stage at presentation was similar for the hereditary and ACS audit patients. Five-year survival was significantly improved (P less than .05) for both hereditary cancer populations as compared to the ACS audits (67% hereditary breast cancer and 52% nonpolyposis hereditary colon cancer). Improved survival in hereditary colon and breast cancer patients may have a bearing on the design of future clinical protocols.

Age Factors↗
PubMed1982

Phenotypic variation in hereditary adenomatosis: unusual tumor spectrum.

Familial adenomatous polyposis coli is a heterogeneous disease with respect to genetics as well as its colonic polyposis and cancer phenotype. The authors report a family with variation in phenotypic expression of polyps ranging from isolated polyps to florid carpeting of the entire colonic mucosal surface. In addition to early-onset colonic cancer, a patient with isolated polyps had a seminoma and subsequently developed gastroesophageal cancer. His son, also with isolated colonic polyps, manifested a pararectal rhabdomyosarcoma. The significance of these variations can only be assessed fully through the study of cancer of all anatomic sites in many additional families.

Adolescent↗
PubMed1982

Total colectomy and the cancer family syndrome: report of a case.

A case is reported of a 47-year-old man with a family history consistent with Cancer Family Syndrome (CFS), who manifested proximal colon carcinoma. On the authors' recommendation, with the knowledge that the colon in CFS shows an inordinate lifelong susceptibility to malignant neoplastic transformation, a total abdominal colectomy was performed. The resected colon was found to contain an additional occult carcinoma distal to the primary lesions. Knowledge of this phenomena is critical to the management of CFS.

Adenocarcinoma↗
PubMed1982

Genetic/epidemiological findings in a study of smoking-associated tumors.

While the etiology of bronchogenic carcinoma remains enigmatic, an expanding array of carcinogenic pollutants in interaction with host susceptibility factors has been implicated. We have studied family histories of cancer in a consecutive series of cancer patients from two university oncology clinics in Nebraska. Particular attention has been given to carcinoma of the lung and other putative smoking-associated cancers (oral cavity, esophagus, urinary bladder, pancreas). Smoking histories were obtained for relatives of an overlapping series of patients with these tumor sites. Findings revealed that, although a significant cohort effect was observed with respect to smoking habits for both relatives of lung cancer probands and for relatives of probands with other smoking-associated tumors, a corresponding trend for lung cancer frequency was observed only for relatives of lung cancer probands. This result suggests the importance of host factors in combination with environmental exposures in determining lung risk. A cohort trend for lung cancer was also apparent among relatives of breast cancer probands, but not for relatives of colon cancer probands, suggesting the possibility of an intrinsic association between carcinomas of the breast and lung. We believe that further elucidation of host factor susceptibility in lung cancer may have important etiological and preventive implications.

Breast Neoplasms↗
PubMed1982

Surveillance and management of patients at high genetic risk for ovarian carcinoma.

The present study is concerned with surveillance/management programs for hereditary ovarian cancer syndromes. These syndromes lack distinguishing premonitory physical signs or biomarkers; therefore, in these genotypic settings, ovarian cancer risk must be assessed by analysis of the patient's pedigree. The authors describe 10 families showing a hereditary proclivity to ovarian carcinoma and/or associated cancer(s) in accord with their respective cancer-prone genotypes. Cancer education, genetic counseling, and surveillance should be instituted early. In addition to bimanual pelvic examination, ultrasound should be tested for its possible efficacy as a screening technique. The option of prophylactic bilateral oophorectomy and hysterectomy is thoroughly discussed with highly selected candidates. The authors believe that the aggressive management approach proposed for ovarian carcinoma is warranted for high-risk members of cancer-prone families, wherein the risk for ovarian cancer may approach 50%.

Adult↗
PubMed1981

Familial breast cancer and its recognition in an oncology clinic.

Family history of cancer was evaluated for 79 breast cancer probands from among a series of consecutively ascertained cancer patients undergoing treatment in the authors' Oncology clinic. Cancer prevalence for each family was quantified by using a statistic that accounts for variable size and age structure among families. To test the null hypothesis that cancer risk is independent of family membership, the distribution of this statistic for families in their original configuration was compared with the distributions observed when relatives were randomly assigned to families in 99 random permutations of family membership. The results indicated significant heterogeneity for cancer risk among relatives of breast cancer probands, which suggests that the isolation of high risk families can provide a meaningful resource for in-depth studies in breast cancer genetics. Using an objective criterion for selection of such families, high risk for early onset breast cancer was confirmed in a prospective follow-up study fof four families from the original resource.

Adult↗