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Long-term follow-up of von Recklinghausen neurofibromatosis. Survival and malignant neoplasms.

To document the natural history of von Recklinghausen neurofibromatosis, we followed up a nationwide cohort of 212 affected patients and families identified in Denmark 42 years ago. We obtained follow-up information on 99 percent. Because all 76 probands were identified through hospitals, they may include a disproportionate number of severe cases of neurofibromatosis. To diminish this effect of selection bias, we distinguished between the probands and their affected relatives. In a comparison with the general population, survival rates were significantly impaired in relatives with neurofibromatosis, worse in probands, and worst in female probands. Malignant neoplasms or benign central nervous system tumors occurred in 45 percent of the probands, giving a relative risk of 4.0 (95 percent confidence limits, 2.8 to 5.6) as compared with expected numbers. Multiple primary neoplasms were found in 15 probands, but only 1 relative. Compared with the general population, male relatives with neurofibromatosis had the same rate of neoplasms, whereas female relatives had a nearly twofold higher rate (relative risk, 1.9; 1.1 to 3.1). Nervous system tumors were disproportionately represented. We conclude that patients with severe neurofibromatosis requiring hospitalization often have a poor prognosis, but incidentally diagnosed relatives may have a considerably better outcome.

Brain Neoplasms↗

Five primary synchronous neoplasms of the gastrointestinal tract.

Multiple primary malignant neoplasms in a single patient have been well documented in the literature over the past hundred years. The lesions can be limited to a single organ or involve multiple organ systems. It is relatively common for patients with colorectal carcinoma or carcinoid tumors to have more than one primary neoplasm. Colonic lesions can be synchronous or metachronous in presentation and colonic or extracolonic in location. We present a patient with five primary synchronous neoplasms of the gastrointestinal tract, involving the stomach, small bowel, and colon. The patient had no evidence of metastatic disease and underwent resection of all the lesions. This case illustrates the need for a thorough search for additional neoplasms in the treatment of patients with cancer.

Adenocarcinoma↗

[Multiple primary malignant cancer and radiation induced cancer--a point of view of radiation oncology].

The cases of multiple primary malignant neoplasm and radiation induced cancer were discussed based on the experiences of Tokyo Women's Medical College in which radiotherapy has been mainly used for cancer. These cases have been increasing in number and it seems to be that the problems of them shall be gradually developed in future, because survival time of cancer patients become longer due to improvement of cancer treatment. But if the mechanisms of cancer etiology on which radiation and other many things influence, would not be clear, radiotherapy have been still principal post in cancer treatment. Systemic multidisciplinary treatment of cancer must be considered and we are looking forward to establishing it.

Adult↗

Plasma cell neoplasia in a single host: a mosaic of different protein-producing cell types.

The peritoneal plasma cell neoplasias that develop in strain BALB/c mice after the injection of adjuvant-staphylococcus mixtures or mineral oil alone appear in the form of multiple nodules in the mesentery and on peritoneal surfaces. Experiments were done to determine if these nodules were metastases or multiple primary neoplasms. Nodules or pieces of masses were transplanted subcutaneously by the trochar method or by insertion of tissue under the kidney capsule from 6 primary cases and parallel transplant lines were established. The serum and urinary protein abnormality (a stable heritable characteristic) of each of the various transplant lines was characterized by agar gel electrophoresis and immunoelectrophoresis. Different protein-producing lines were found in 3 cases; in one case 5 different protein-producing lines were isolated. Two different lines were found for each of the other 2 cases. When transplantation studies were begun early, it was demonstrated that the nodules were multiple primary plasma cell neoplasms; when delayed, only one protein-producing plasma cell neoplasm was found.

Animals↗

Second primary malignancy in endometrial carcinoma patients.

An epidemiologic study of multiple primary malignant neoplasms in endometrial cancer patients in Israel is presented. During the 7-year period of the study, 104 patients (10.3%) out of 1007 patients diagnosed as having endometrial cancer had another primary cancer. There was a significant difference in the incidence of multiple primary cancers between various ethnic groups, women of European/American origin having higher incidence than women of Asian/African origin. A significantly higher than expected incidence of second primary cancers occurred at the following sites: breast (relative risk, 4.1), ovary (relative risk, 11.6), cervix (relative risk, 5.1), and colon (relative risk, 5.9). The prognosis was mainly influenced by the site of the second primary cancer. The increased incidence of multiple primary malignancies justifies a high level of alertness to the possibility of second primary cancer in endometrial cancer patients.

Adult↗

Collision metastasis of bladder and prostate carcinoma to a single pelvic lymph node.

Genitourinary organs are at greater risk for multiple primary malignant neoplasms because of the high incidence of these tumours as primaries among all other organs. We present a case of prostate and bladder carcinoma metastasized to a single pelvic lymph node, called a "collision" metastasis, in a patient with four primary tumours.

Adenocarcinoma↗

Patterns of multiple primary tumours in patients treated for cancer during childhood.

One hundred and sixty one children who have developed more than one primary neoplasm have been identified. Children with tumours of the central nervous system, retinoblastoma and leukaemia were those most frequently observed to develop a second malignancy whilst osteosarcoma was the most common second tumour. The patterns of second neoplasms appear to be changing and a recent increase in the number of children with leukaemia and lymphoma who develop second primary tumours has been observed. In this series, the two most frequent associations of tumours were retinoblastoma followed by osteosarcoma and the combination of acute leukaemia with a tumour of the central nervous system. Genetic factors which may have contributed to the development of the second primary tumour were identified in 53 patients (33%), 33 of whom had the genetic form of retinoblastoma. In an analysis of the treatment of 151 patients, for whom the interval between the two neoplasms was greater than 12 months, the second malignancy was considered to be 'radiation associated' in 93 (61%). Fifty children (33%) had been treated with either single or multiple agent chemotherapy which included an alkylating agent in 38. Forty five children had received a combination of chemotherapy and radiotherapy and of these, 10 developed leukaemia as their second tumour. Of the 19 secondary leukaemias, 16 have occurred in patients treated since 1970.

Adolescent↗

Multiple distinct intracranial tumors: association of pinealoma and craniopharyngioma. Case report.

We report the case of a patient with multiple primary brain neoplasms. A pinealoma was treated with radiotherapy and surgical resection in 1983. In 1987, a craniopharyngioma was resected surgically. Various possible determinants of multiple brain tumors have been described in the literature and are discussed. The possibility of an unknown underlying pathologic mechanism predisposing to multiple malignancies must be considered, but we are unable to conclude that this particular occurrence is more than a random chance finding.

Adult↗

Multiple primary cancers in 5,456 autopsy cases in the National Cancer Center of Japan.

Multiple primary malignant neoplasms in 5,456 consecutive autopsy cases from 1962 to 1981 in the National Cancer Center were analyzed. There were 285 (5.2%) double primary cancers, 58 (1.1%) triple or more, and 65 (1.2%) minute cancers of the thyroid or prostate gland. Higher incidence of second cancer is observed in cancers of the oropharynx, intestine, larynx, uterus, bladder, and thyroid. Organ association between the two cancers was present in certain organs; for example, there was a tendency for upper gastrointestinal tract cancers to be associated with lower gastrointestinal tract cancers. Influence of histologic subtypes in multiple cancer cases is discussed for lung cancer.

Adult↗