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PubMed2002

Papillary serous carcinoma of the peritoneum coexisting with or after endometrial carcinoma.

OBJECTIVE: Papillary serous carcinoma of the peritoneum (PSCP) coexisting with, or after, endometrial carcinoma (EC) is an extremely rare condition with no documented patient series. The aim of this investigation was to describe our experience in treating five patients diagnosed with PSCP synchronously with EC and two others who developed PSCP metachronously after EC. METHODS: In this retrospective study, we reviewed and analyzed the clinical and pathological data of seven patients diagnosed and managed over a 10-year period. The diagnosis of PSCP was mostly based on the inclusionary criteria of the Gynecologic Oncology Group [1]. Disease stages were determined using the FIGO criteria for epithelial ovarian cancer (EOC) and endometrial carcinomas [2]. The treatment for PSCP was similar to that for advanced EOC and immunohistochemical studies were performed using archival material for PSCP and EC in order to determine p53, Bcl-2, HER2, and estrogen and progesterone receptor (ER, PR) status. Germline mutation analyses were performed for the two most common mutations pertaining to BRCA1 and the one most common mutation pertaining to BRCA2 genes only. RESULTS: Five patients with PSCP and synchronous EC initially underwent surgical treatment. The remaining two underwent surgery originally for EC and, thereafter, for metachronous PSCP. All seven patients had advanced stages (III or IV) of PSCP and stage I only EC. At the time of analysis, four patients were alive. p53, Bcl-2, ER, and PR were found to have been expressed in various rates in both or one of the neoplasms. However, no HER2 was found to have been expressed, either in PSCP or in EC. All germline mutation analyses were negative. CONCLUSIONS: The results obtained in this study show that PSCP can occur either synchronously or metachronously with lower stage EC that is associated with advanced disease stages. We suggest that this clinical form of PSCP with synchronous or metachronous EC is a very aggressive and lethal clinical form and differs markedly from the vast majority of multiple gynecologic neoplasms of the upper genital canal diagnosed in the ovarian-endometrial group, of which EOC are mostly diagnosed as stage I diseases with high-rate cures.

Aged↗
PubMed1989

Congenital soft tissue dysplasia: a new malformation entity and concept.

We report 185 children with clinical manifestations of various conditions classically described as giant hamartoma, angiodysplasia, congenital hypertrophy, congenital trophoedema, localised gigantism (e.g. macrodactyly), etc. It is proposed to group all these conditions into a single entity: congenital soft-tissue dysplasia (CSTD). According to recent advances in fundamental embryology and cell biology, CSTD appears to be a consequence of embryonal or fetal cell biosynthetic dysregulation. The concept of the CSTD entity leads to a common protocol for clinical investigation and a common therapeutic plan, with special reference to the stability and the benign nature of the condition. Treatment should be confined to improving function rather than attempting to correct cosmetic deficits.

Adolescent↗
PubMed1976

Experimental induction of bone tumors by short-lived bone-seeking radionuclides.

Single and repeated applications of 224Ra and single applications of 227Th to more than 600 female NMRI mice 3 - 4 weeks old, and to male NMRI mice have led to a high rate of osteosarcomas. Tumor incidence is dose-related. 227Th is more carcinogenic than 224Ra which induced the highest tumor incidence of 60% after a single injection of 5 muCi per Kg body weight or more. Repeated injections of 224Ra to female mice yielded a tumor incidence of up to 92%. Most of these osteosarcomas consist of well-differentiated bone-forming osteoplastic tissue. Half of the tumors occurred in the spine, particularly in the lumbar region. In protraction experiments, multifocal osteosarcomas have been observed. Less than 10% of the mice with osteosarcoma had developed metastases in lung, spleen, liver, and kidney. The possible mechanisms of the protraction effect are discussed.

Animals↗
PubMed1992

Absence of p53 germ-line mutations in bilateral breast cancer patients.

The cause of Li-Fraumeni syndrome, a rare group syndrome of familial cancers, has recently been identified. Patients with this inherited condition are highly susceptible to specific neoplasms, including early-onset breast cancers. The available evidence links Li-Fraumeni syndrome to inherited mutations of the tumor suppressor gene p53. Moreover, somatically acquired p53 mutations and gene deletions are common feature in breast cancer of sporadic origin. These findings suggest that germline p53 mutations are important in familial and, possibly sporadic, breast tumors. We have therefore screened lymphocyte DNA from 19 unrelated bilateral cancer patients for germline p53 mutations in exons 5, 6, 7 and 8. We have however detected no germline mutations by means of the single-strand confirmation polymorphism technique in any of the lymphocyte DNAs examined and conclude that p53 mutations are not generally involved in bilateral breast cancer.

Adult↗
PubMed1987

Elective whole-lung irradiation in osteosarcoma treatment: appearance of bilateral breast cancer in two long-term survivors.

The effect of "prophylactic" lung radiation in preventing pulmonary metastasis was studied in a subset of patients with osteosarcoma. Benefit was not noted. Thirteen of the irradiated patients in the treatment group were females; in 2 of the 13, bilateral breast cancer developed approximately 15 years later. Radiation administered to the breast of an adolescent female carries a considerable risk of causing breast cancer.

Adolescent↗
PubMed1981

Double tumors of anterior and posterior pituitary gland.

Double tumors were found in the anterior and posterior lobes of pituitary gland at autopsy in a patient who presented with progressive deterioration of mental status. The chromophobe adenoma of anterior lobe consisted of a mixture of non-immunoreactive hormone containing cells and a few prolactin (PRL) and growth hormone (GH) cells in the mid portion whereas the periphery of the tumor contained immunoreactive cells for PRL, GH, thyroid-stimulating hormone (TSH), luteinizing hormone (LH), and follicle-stimulating hormone (FSH). A microscopic focus of granular cell myoblastoma (GCM) was found in the posterior lobe. Differentiation of tumor cells into anterior pituitary cells and GCM is discussed.

Adenoma, Chromophobe↗
PubMed1986

[Unwanted sequelae of irradiation in the intestinal tract--pathogenesis, diagnosis, therapy].

The therapy of irradiation sequelae in the intestinal tract is still problematic. Within the period from 1967-1984 285 patients have been treated for late radiation injuries of the intestine. Our experiences are demonstrated and discussed with reference to the literature. Specific topics are: pathogenesis, clinical problems and aspects of conservative and operative treatment.

Abdominal Neoplasms↗
PubMed1992

Multiple granular cell tumors of the gastrointestinal tract with subsequent development of esophageal squamous carcinoma.

A 52-year-old woman initially presented to our medical center with synchronous, submucosal tumors of the esophagus, stomach, and transverse colon. The gastric and colonic tumors were resected, and both displayed infiltrating sheets of polygonal cells with coarsely granular cytoplasm and small vesicular nuclei. The neoplastic cells of both tumors were immunoreactive for S-100 protein. Ultrastructural studies revealed the lysosomal nature of the cytoplasmic granules. Although the esophageal mass was not resected, it was felt that this represented another focus of granular cell tumor of the gastrointestinal tract. Two years later, she presented with disseminated squamous carcinoma of the esophagus. At autopsy, a submucosal granular cell tumor was found adjacent to the squamous carcinoma of the esophagus. To our knowledge, this is the first reported case of synchronous granular cell tumors that involved multiple segments of the gastrointestinal tract, one of which was later associated with a squamous carcinoma of the esophagus.

Carcinoma, Squamous Cell↗
PubMed1981

Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases.

Seventy-four patients with 75 examples of granular cell tumors occurring in the gastrointestinal tract and perianal region were studied. Twenty-four were located in the esophagus, eight in the stomach, three in the small bowel, four in the appendix, twenty in the large bowel, and sixteen in the perianal region. Nine cases had multiple tumors either occurring in the same area of the bowel or in other organs, mostly the skin. Approximately 50% of the tumors occurring in the esophagus and perianal region showed varying degrees of acanthosis or pseudoepitheliomatous hyperplasia. In the perianal tumors the pseudoepitheliomatous hyperplasia was much more extensive and could be mistaken for squamous cell carcinoma. The world literature was reviewed and a total of 66 cases of granular cell tumors were reported in the area of study. In neither our 74 nor the other reported cases was there evidence of recurrence or malignant behavior of the tumors, thus confirming their benign biologic behavior.

Adolescent↗
PubMed1996

Simultaneous occurrence of osteosarcoma and osteochondroma following treatment of neuroblastoma with chemotherapy, radiotherapy, and bone marrow transplantation.

Radiation-induced bone changes and second malignancies, as well as benign tumors, following bone marrow transplantation are being reported with increasing frequency. An osteosarcoma of the fourth right rib and an osteochondroma of the left scapula developed in a long-term survivor of abdominal neuroblastoma treated with chemotherapy, local radiation, and bone marrow transplantation. All these treatment modalities are known to induce neoplasia.

Abdominal Neoplasms↗
PubMed1985

Granular cell tumors of the esophagus.

An unusual benign multicentric esophageal granular cell tumor (granular cell myoblastoma) associated with 16 other similar tumors in the skin, vulva, breast, and tongue of 1 patient is described. There was a family history of granular cell tumors in the patient's mother; this has not been previously described. The pathologic findings and controversial histogenesis of granular cell tumors are discussed in an effort to delete the erroneous term "myoblastoma" from the radiologist's vocabulary. Granular cell tumors of the esophagus are also specifically reviewed.

Adult↗
PubMed1977

Multiple oncogenesis of neural crest cells by steroids in suckling mice.

In suckling mice injected with steroids, multiple tumors occurred in the sites where neural crest cells normally are present. It is speculated that the phase of the cell cycle of the neural crest cells may have something to do with its cell surface and cellular phenotypic expression in the system mediated by cyclic AMP.

Animals↗
PubMed1991

Clinicopathologic features of the flat adenoma.

One hundred twenty-eight small flat adenomas (SFAs) were collected from 101 patients, and the clinicopathologic features were investigated. There were 91 adenomas with mild atypia, 20 with moderate atypia, and 17 with severe atypia. SFAs were found more often in males than in females, with a ratio of 3.4:1, and the malignancy rate in females (31.8 percent) was higher than in males (9.3 percent). About 38 percent of the patients had a history of colorectal carcinoma, and 65 percent had a history of colorectal neoplasms. Of 37 patients whose family history was traced, 21 had cancer families. SFAs were prone to be found in patients with a history of colorectal neoplasms and a cancer family. Malignancy rate increased with increasing size. The overall malignancy rate was 13.3 percent, which was considerably higher than that of ordinary small polypoid adenomas (2.8 percent). SFAs were situated more proximally (30.9 percent) than ordinary adenomas; however, there was no relationship between site and malignancy. All the lesions showed tubular adenomas, and there was no villous feature. A central depression was noted in 20 lesions, more frequently in adenomas with higher atypia. All but one adenoma with severe atypia showed a component of lower atypia, supporting the adenoma-carcinoma sequence.

Adenoma↗
PubMed1997

What is the risk factor for metachronous colorectal carcinoma?

PURPOSE: The purpose of this study was to determine the risk factors for developing metachronous colorectal carcinoma and to determine an adequate postoperative colonoscopic surveillance. METHODS: Two hundred eighty-four patients, examined by routine colonoscopy after resection for colorectal carcinoma, were reviewed. Clinical and pathologic factors were assessed by multiple logistic regression analysis. RESULTS: One hundred eighty-three patients with synchronous adenoma or carcinoma at the initial operation had a significantly higher incidence of both metachronous adenoma and carcinoma than the 101 patients without a synchronous lesion. Other clinical factors including age, gender, tumor stage, tumor site, and tumor grade were not significant for an increased incidence of metachronous carcinoma. The presence of synchronous lesions proved to be the only risk factor (relative risk, 3.293; P = 0.0155) for developing metachronous carcinoma. Metachronous carcinoma was detected in 30 patients (10.6 percent) and completely removed from all patients. Mucosal carcinoma was found in 25 patients (8.8 percent) and invasive carcinoma in 5 patients (1.8 percent). All five invasive carcinomas were detected in asymptomatic patients having synchronous lesion. Four patients required a second operation for metachronous carcinoma more than 13 months following the first. CONCLUSION: The risk factor for developing metachronous carcinoma is the presence of synchronous adenoma or carcinoma at the initial operation. To detect metachronous carcinoma at a curable stage, annual colonoscopic surveillance should be performed for high-risk patients.

Adenoma↗
PubMed1996

Establishment of a hereditary nonpolyposis colorectal cancer registry.

INTRODUCTION: Hereditary nonpolyposis colorectal cancer (HNPCC) is an autosomal dominant condition characterized by early age of onset colorectal cancer, right-sided predominance, excess of synchronous and metachronous colonic neoplasms, and extracolonic cancers. The purpose of this study is to report clinical characteristics of HNPCC families in our registry. METHODS: This is a retrospective review of medical records of patients with a significant history of colorectal cancer and interviews with their families. RESULTS: Three hundred one people with cancer in 40 HNPCC families were identified. In 284 of 301 (94 percent) people, 363 cancers were identified. Colorectal cancer only was identified in 182 people (64 percent) and, in conjunction with extracolonic tumors, in another 31 people (11 percent). Extracolonic cancer alone was noted in 71 people (25 percent). Median age at diagnosis of colorectal cancer was 48 (range, 17-92) years. In patients with documented pathology, right-sided tumors predominated (55 percent), synchronous and metachronous tumors were noted in 53 percent, and synchronous of metachronous adenomas were documented in 51 percent of people. Generational anticipation was also noted. CONCLUSION: This study demonstrates and confirms characteristics that have been described in HNPCC. Namely, early age of onset of colorectal cancer, right-sided predominance, multiple synchronous and metachronous neoplasms, increased extracolonic cancers, and generational anticipation.

Adolescent↗