Family members of non-endemic Hokkaido patients with adult T-cell leukemia (ATL) were assessed for the prevalence of ATL virus (ATLV) infection. Immunofluorescence test showed that 53 (39.8%) of 133 healthy family members of 23 ATL patients were positive for antibodies of ATLV-associated antigen (ATLA). When seropositive T-cell malignancies other than ATL and healthy controls in Hokkaido were examined, 10 of 33 family members (30.3%) of 9 patients and 3 of 18 family members (16.7%) of 5 donors had ATLA antibodies, respectively. In sharp contrast, the overall seropositivity in this northern part of Japan was 0.73%. None were seropositive (0%), when 26 family members of 6 patients with acute myeloid leukemia sero-converted by blood transfusion during the course of the disease.

A family of multiple endocrine neoplasia type I with five confirmed cases in three generations is described. All of them have primary hyperparathyroidism in common. The propositus is 51 year-old male. After a year of symptoms of gastroduodenal ulcer, he was found to have elevated levels of serum gastrin and PTH. The serial imaging studies revealed a tumor in pancreatic head, and Zollinger-Ellison syndrome was diagnosed. The gastrin level was reduced into normal range after extirpation of the tumor, but post surgical elevation of Calcium put the patient under parathyroidectomy, which normalized serum PTH and Calcium levels. His two sisters (I and II), the mother of them, and the daughter of sister I, had neither signs nor symptoms until family study showed hypercalcemia in all. Sister I is a 54 year-old female with enlarged parathyroid. The hyperparathyroidism is of chemical type, but no other endocrinological abnormality is found. The Calcium level decreased after parathyroidectomy. Sister II is a 56 year-old female. The only sign was galactorrhea. Serum PTH and Calcium decreased after parathyroidectomy. The prolactinoma was diagnosed by the increased prolactin levels and enhanced mass lesion in sella turcica. Her serum prolactin levels is now within normal range since she is on bromocryptine. The mother of the above three siblings and the daughter of the sister I are now under further study.

Acquired multidrug resistance as well as innate drug resistance are directly related to ineffectiveness and failure of the cancer chemotherapy. The mechanisms of such resistance, especially those of innate resistance, have not been fully elucidated. Drug resistant tumor cells, however, usually bear biochemical changes which are related to resistance mechanisms. New modalities with high selectivity against resistant cells could, therefore, be possible if we could target these biochemical changes. Vincristine (VCR)-and adriamycin (ADM)-resistant tumor cells (pleiotropic drug resistant cells) usually show an enhanced outward transport of these antitumor agents, and they express unique glycoproteins in the plasma membrane. By targeting for these biochemical changes characteristic to the resistant tumor cells, we establish new modality which shows high selectivity against drug resistant tumor cells. In this review, I will describe genetic origin of drug resistance, biochemical mechanisms of drug resistance and reversal of drug resistance in tumor cells. The modality to utilize calcium channel blockers which inhibit the enhanced outward transport of VCR and ADM from resistant tumor cells will be reviewed.

The association of a pheochromocytoma with von Hippel-Lindau disease is uncommon. We had a family with eight patients affected by von Hippel-Lindau disease, of whom five had hemangioblastoma of the central nervous system and seven had pheochromocytoma. As other lesions, retinal angiomatosis, spinal A-V malformation and spinal hemangioma were included in this family. In this paper five hemangioblastomas, four of whom had the association of hemangioblastoma and pheochromocytoma, are presented and clinical features of hemangioblastoma associated with pheochromocytoma are discussed, comparing to the 16 reported cases that had the association of hemangioblastoma and pheochromocytoma. The mean age of our own five cases at the onset of clinical symptoms was 32.2 years (ranging from 24 to 41 years) and that of reported 16 cases was 33.9 years (ranging from 18 to 55 years). These ages are slightly younger than that of sporadic hemangioblastoma. On the other hand, the mean age at the onset of pheochromocytoma was 28.3 years in our cases and 31.0 years in reported cases. This may suggest that hemangioblastoma when it is associated with pheochromocytoma presents its symptoms several years after the signs and symptoms of pheochromocytoma are manifested. In our cases a male to female ratio was 4:1 and in reported ones it was 9:7, showing that hemangioblastoma associated with pheochromocytoma as well as sporadic hemangioblastoma is likely to occur more in male.(ABSTRACT TRUNCATED AT 250 WORDS)

A new human endometrial adenocarcinoma cell line, Ishikawa cells, was established from an endometrial adenocarcinoma from a 39-year-old woman and has been maintained in vitro for more than 3 years. The cells were found to form a monolayer in a mosaic fashion and to tend to pile up. Population doubling time was calculated to be about 36, 29 and 27 hours at the 9th, 40th and 50th generations, respectively. The modal chromosomal number of the cells fell in a diploid range. Histology of the tumor induced in athymic nude mice showed it to be a well differentiated adenocarcinoma which closely resembled the original human tumor. Estrogen receptor and progesterone receptor were demonstrated to occur not only in the induced tumor in athymic nude mice but also in in vitro culture cells. From the fact that the cell growth was maintained in an estrogen-free medium, it appeared that the cells had no estrogen dependency.

To establish the clinical management of partial moles, cytogenetic, morphological and clinical studies of 57 partial moles, including three twin cases, were performed. The karyotypes of the 54 singletons were 46 triploid and eight diploid. By examining the genetic markers, two of the diploid moles were proved to be derived from normally fertilized conceptuses. Molar tissues from two twin cases had a 46,XX karyotype of androgenetic origin, while the normal placenta and fetus were derived from normal conceptuses. An embryo (or fetus) (26.9%) or only an umbilical cord (9.6%) was found in some triploid and diploid moles. Vessels with erythroblasts were also recognized in most cases. In addition, there was a positive correlation between cyst formation and gestational length in triploid conceptuses. These findings suggest that triploid partial moles are an altered form of missed abortion. The mole became invasive in one twin case with an androgenetic mole, but the remaining cases had uneventful courses. These results indicate that partial moles can be divided into three groups: Triploid, Diploid derived from normally fertilized conceptus, Binovular twins with androgenetic mole, and that, except for those in group 3, partial moles have no malignant potential and require no intensive follow-up.