Encephalocele, polycystic kidneys, and polydactyly with other defects. A necropsy case of Meckel syndrome and a review of literature.

Meckel syndrome, which is diagnosed by 2 of 3 main congenital malformations such as a occipital encephalocele, polycystic kidneys, and polydactyly, is an autosomally inherited recessive disease. We have experienced a case of Meckel syndrome and performed necropsy. Necropsy findings revealed multiple congenital malformations with occipital meningo-encephalocele and agenesis of the cerebellum, 6 digits on the hands and feet, polycystic kidneys. The criteria of Meckel syndrome is still unclear. We propose that the diagnosis of this syndrome may be accompanied by the presence of all triad of main malformations. Ninety four cases satisfying this criteria have been reported in the world literature. Several discussion were made from a review of the literature.

Granular cell tumor of the orbit.

An 18-year-old woman had a 3-month history of increasing proptosis and visual loss secondary to an inferior orbital mass. Surgical exploration revealed an encapsulated mass in the orbit. After this mass was excised good vision was restored. The diagnosis of granular cell tumor was made as a result of various microscopic studies. The location of the tumor within the orbit is unusual and its histologic origin is uncertain. Immunohistochemistry by peroxidase-antiperoxidase method revealed that this tumor had S-100-protein-positive cells and negative results for lysozyme and alpha-1-antitrypsin. This fact suggests that the origin of the granular cell tumor may be due to the Schwann cell.

Creutzfeldt-Jakob disease. An autopsy case of the panencephalopathic type and a review of the literature.

This is a case report of a purported panencephalopathic type of Creutzfeldt-Jakob (C-J) disease in a 61-year-old Japanese farmer. He died nine months after the onset of clinical symptoms. This variety of C-J disease was named and reported in 1981 by Mizutani. The characteristic feature is extensive degeneration of cerebral white matter not related to cortical damage in addition to the spongiform changes in the cortical gray matter. The case was diagnosed clinically and at post mortem marked neural loss, astrocytic macrogliosis, and degeneration of subcortical white matter were found. Senile plaques and neurofibrillary tangles were virtually absent. Degeneration of the white matter is uncommon in C-J disease, and when it occurs is usually mild, and limited in distribution.

Induction of tumors with cycasin in newborn and preweanling rats.

Comprehensive studies of carcinogenesis in newborn or preweanling SD rats were conducted under various dose schedules of cycasin (CAS: 14901-08-7) administration. When cycasin was given sc to newborn rats at day 0, tumors were detected in more than 80% of rats of both sexes; kidney tumors were by far the most common. The incidences of tumors declined in the older groups, namely, over 60% in both sexes in the 7-day group, 55% in males and 8.3% in females in 14-day rats, and 0% in 21-day groups. By multiple administration, tumor incidences elevated considerably. Administration ip of cycasin also gave rise to tumor induction in newborn rats. A total of 435 kidney tumors found in the experiments were studied pathologically. Most of them were classified as mesenchymal tumor; some of them metastasized. A few other tumors were found in the liver and colon.

Nonchromaffin paraganglioma in the nose and paranasal sinuses.

Nonchromaffin paraganglioma occurring in the nose and paranasal sinuses are extremely rare. The authors have experienced a case of nonchromaffin paraganglioma of nasal cavity which extended to the epipharynx. To our knowledge, this reported case is the fourth case that has appeared in the world literature. The patient, a 31-year-old female, had complained of a right nasal obstruction and mucopurulent rhinorrhea for two years. The right nasal cavity was filled with a polypoid mass showing no bleeding tendency or necrosis. Radiographs revealed a homogeneous shadow which occupied the entire nasal cavity and paranasal sinuses. Histopathological studies revealed that the tumor showed typical findings of nonchromaffin paraganglioma and neurosecretory granules in cytoplasm were observed by electron microscopic study. Subsequently, the tumor recurred at on the posterior epipharyngeal wall after a duration of one year and three months, and was removed completely. Since then, there was no recurrence of the tumor until the present time.

Lipoprotein lipase-like activity in the liver of mice with Sarcoma 180.

The triglyceride lipase (TGL) activity of liver homogenates of mice with Sarcoma 180 was measured. The liver homogenate of normal or tumor-bearing mice was treated with 0.25% Triton X-100 and centrifuged at 100,000 g for 60 min, and the supernatant was applied to a heparin-Sepharose column. In normal mice, most of the TGL activities in the supernatant was eluted with 0.75 M NaCl from the column. In mice with Sarcoma 180, the TGL gave two peaks on heparin-Sepharose column chromatography, which were eluted with 0.75 M and 1.5 M NaCl, respectively. The activity in the first peak (0.75 M NaCl eluate) decreased; that in the second peak (1.5 M NaCl eluate) increased, and the ratio of the second peak to the first peak increased during tumor development. The livers of normal mice and mice on day 10 after tumor inoculation were perfused with heparin. The highest rate of the TGL release occurred within 1 min of heparin perfusion, and the bulk of heparin-releasable activity appeared within 2 min of perfusion in both normal and tumor-bearing mice. The TGL activity in liver perfusate of tumor-bearing mice, as well as that of liver homogenate, was resolved on a heparin-Sepharose column into two peaks, which were eluted with 0.75 M and 1.5 M NaCl, and most of the activity was eluted with 1.5 M NaCl. The nature of the TGL activity eluted from a heparin-Sepharose column was investigated. In both liver homogenates and liver perfusates, the first peak did not require serum for maximal activity and was relatively resistant to a high concentration of NaCl or protamine sulfate.(ABSTRACT TRUNCATED AT 250 WORDS)

Ultrastructure of mammary carcinomas in male rats induced by 7,12-dimethylbenz(a)anthracene.

The morphology of mammary tumors induced by 7,12-dimethylbenz(a)anthracene in male rats was investigated by light and electron microscopy. All tumors induced in male rats were carcinomas with prominent epithelial growth which shows a medullary or cribriform appearance. Neither mammary dysplasias nor fibroadenomas were induced in male rats. Foci of adenoid cystic carcinoma were encountered in some parts of tumors. Papillary and/or tubular patterns, which have been observed frequently in mammary carcinomas in female rats, were not prominent histologic features in male rats. Secretory activity was not remarkable. The morphology of mammary carcinomas in male rats was unchanged in primary and transplanted tumors under various hormonal conditions. This finding supports our previously published results that the mammary carcinomas in male rats are hormone-independent, although our previous biochemical study revealed that the tumors contain both estrogen and estrogen-dependent progesterone receptors.

Carcinoid tumor of the uterine cervix. A light and electron microscopic study.

A case of carcinoid tumor of the uterine cervix is reported. The patient was a 76-year-old Japanese woman with a complaint of vaginal bleeding. She was clinically diagnosed as stage IVb carcinoma of the uterine cervix. Light microscopically, the tumor was characterized by formation of solid cell nests and, in limited areas, glandular structures. The tumor cells in solid cells nests showed positive argyrophil reaction but were negative for argentaffin reaction. At the ultrastructural level, the cytoplasm of the tumor cells contain numerous neurosecretory granules, microfilaments and well-developed Golgi complexes. On the bases of histochemical and electron microscopic features of tumor cells, the tumor was diagnosed as carcinoid tumor of the uterine cervix.

Effect of pharmacological doses of estrogen on ovary-independent rat mammary carcinoma containing estrogen and progesterone receptors.

Mammary carcinoma induced in male Sprague-Dawley rats by multiple intragastric intubations of 7,12-dimethylbenz-(a)anthracene showed ovary-independent growth but contained estrogen receptors (ER) and progesterone receptors (Yoshida, Yoshida. Fukunishi, Sato, Okamoto, and Matsumoto, Cancer Res., 42: 2434-2439, 1982). Transplantable carcinoma (MT6) was obtained from dimethylbenz(a)anthracene-induced mammary carcinoma and then maintained in male rats. MT6 tumors with ER grew equally well in males, females, gonadectomized males, males given injections of bromocryptine (1 mg/day) or lisuride hydrogen maleate (50 micrograms/day), and gonadectomized males receiving smaller doses of 17 beta-estradiol (1 to 100 micrograms/2 days). However, the growth of MT6 was inhibited markedly by injection of a very large amount of 17 beta-estradiol (1 mg/2 days). Although transplanted MT6 tumors were ductal carcinoma with cribriform pattern with ER, tumors recurring after injection with 1 mg 17 beta-estradiol were found to be spindle cell carcinoma without ER, which could grow equally well in recipients treated with or without 1 mg 17 beta-estradiol. These observations suggest that the growth of ovary-independent MT6 tumors with ER and progesterone receptors is inhibited only by pharmacological doses of estrogens and that the loss of growth-inhibiting effect of pharmacological doses of estrogen of MT6 tumors occurs during high-dose estrogen treatment.

Morphometric analysis of testicular Leydig cells in normal adult mice.

Stereological analysis was carried out on Leydig cells in perfusion-fixed testes of normal adult mice. In a decapsulated testis, the seminiferous tubules occupy 89.3% and the interstitial tissue makes up 10.7% of the volume of the testis parenchyma. The Leydig cells comprise 3.8% of testicular volume. There are 24.9 million Leydig cells per cm3 (or gm) of tissue. An average Leydig cell has a volume of 1,533 microns 3 and a surface area of 1150 microns 2. The smooth endoplasmic reticulum (SER) is the most prominent organelle in the Leydig cells, and has a membrane surface area of 2,428 cm2 per cm3 of fresh testis tissue, which is 8.5 times the surface area of the plasma membrane and constitutes 56.9% of the total membranes in Leydig cells. Mitochondria occupy 10.1% of the Leydig cell volume or 11.4% of cytoplasmic volume. The inner mitochondrial membrane (including tubular or vesicular cristae) provides a surface area of about 2855 microns 2/cell and is 2.26 times that of the outer membrane. There are approximately 712 cm2 of inner membranes per cm3 tissue. Mouse Leydig cells have numerous lipid droplets, which average 147 per cell and occupy 5.1% of the cell volume.

Permanent cell culture lines of rat erythroleukemia and growth inhibition by dexamethasone in vitro.

Four erythroleukemia cell lines were established in vitro from rats with primary leukemias induced by a set of intravenous injections of 7,8,12-trimethylbenz[a] anthracene (TMBA). These cell lines were grown in Dulbecco's medium with 20% inactivated fetal calf serum; they were characterized by specific consistent chromosome abnormalities, related to No. 2 chromosome, which were maintained in culture for more than 1 year. Addition of dexamethasone prevented the cell growth of 2 of the cell lines in this medium but failed to alter the growth rate of the other 2 cell lines. These findings are correlated with karyotype abnormalities involving chromosome No. 2.

Mammary carcinoma induced by a series of intragastric intubations of 7,12-dimethylbenz[a]anthracene in gonadectomized female and male Sprague-Dawley rats.

Biweekly intragastric intubations of 7,12-dimethylbenz[a]anthracene (DMBA) rapidly induce mammary carcinoma in high yield in gonadectomized female and male rats. These tumors are ovary-independent, though they contain estrogen receptors (ER). In 84.4% of 32 female rats and in 100% of 35 male rats which were gonadectomized at 27 days of age and given 10 mg of DMBA 8 times at 14-day intervals starting from 28 days of age, mammary carcinoma developed 102.4 +/- 28.1 (SD) and 107.2 +/- 25.5 (SD) days, respectively, after the first intubation of DMBA. More than 55% (55 approximately 83%) of seed tumors taken from 18 tumors induced in ovariectomized rats and 18 tumors induced in orchiectomized rats could grow in intact and ovariectomized females and intact and castrated males. Cytosol ER were found in all of 10 tumors induced in gonadectomized male and female rats.

Biological characteristics and estrogen and progesterone receptors in mammary carcinoma induced in male Sprague-Dawley rats by a series of intragastric intubations of 7,12-dimethylbenz(a)anthracene.

Biological characteristics and estrogen (ER) and progesterone (PR) receptors were studied in male mammary carcinomas induced by 7,12-dimethylbenz(a)anthracene (DMBA) in male inbred Sprague-Dawley rats (MM). DMBA-induced carcinomas in females (MF) were used as controls. In 36 of 44 female rats given 20 mg DMBA once by gastric intubation at 50 days of age, MF with microscopic characteristics of cystic papillary adenocarcinoma developed 124 +/- 49 (S.D.) days after challenge. In all of the 42 male rats given 10 mg DMBA at 14-day intervals for 14 weeks starting from 28 days of age. MM with microscopic characteristics of medullary adenocarcinoma developed 106 +/- 21 days after the first intubation of DMBA. The growth of primary MM was unaffected by orchiectomy or estrogen. Eighty to 100% of the MM transplanted in the four groups could grow in intact female rats, ovariectomized female rats, intact male rats, and castrated male rats, while the transplanted MF could grow only in intact female rats. The histology of MM was unchanged in primary and transplanted tumors under various hormonal conditions. ER were present in almost all of the hormone-independent primary and transplanted MM, although the levels for cytosol ER in MM were significantly lower than those in MF. Injection of 10 micrograms 17 beta-estradiol induced marked synthesis of PR in primary and transplanted MM, even 24 and 48 hr after the 17 beta-estradiol injection. These findings show that MM are hormone independent but, like hormone-dependent female tumors, contain ER and estrogen-dependent PR.

Choriocarcinomatous change with immunocytochemically HCG-positive cells in the gastric carcinoma of the males.

Gastric choriocarcinoma is a rare tumor and attracts interest because of its controversial pathogenesis. The present study reports a choriocarcinomatous change with immunocytochemically hCG-positive cells in the gastric carcinoma. The patients were males, one was 41 years old and the other 42 years old. The tumor of both cases consists of adenocarcinoma and choriocarcinoma. A sequential process of morphological transition of the adenocarcinoma to the choriocarcinoma can be traced. Indirect immunoperoxidase stain (PAP method) for human chorionic gonadotropin (hCG) demonstrates the localization of hCG in the syncytiotrophoblasts. Small number of cytotrophoblasts are weakly positive. None of the components of adenocarcinoma of both cases is positive for hCG. Human placental lactogen is not demonstrated in both cases. Stain for pregnancy specific beta-1 glycoprotein is weakly positive in the adenocarcinoma of one case but not in the choriocarcinoma. In one case, the concentration of hCG was 19.9 mIU/ml in the preoperative serum and decreased to 1.2 mIU/ml after gastrectomy. HCG production by gastric carcinoma was discussed with regard to possible pathogenesis of gastric choriocarcinoma.

Argentaffin carcinoid tumor of the rectum.

A case of rectal carcinoid tumor in a 79-year-old Japanese man is reported. The tumor, 1.5 x 0.9 cm, was localized in both mucosa and submucosa of the rectum with neither invasion nor metastasis. Microscopically, neoplastic cells were mainly arranged in rosette-like and trabecular structures within thin fibrous stroma. Histochemically, both argyrophil and argentaffin reactions were positive. At the ultrastructural level, two distinctive types of neurosecretory granules were found in the cytoplasm of tumor cells.

Effects of cholesterol-rich diet and hypolipidemic drug (clofibrate, CPIB) on the adrenal cortex in rats--ultrastructural stereological and biochemical analysis.

Stereological analysis at the electron microscope level was performed on zona fasciculata and zona reticularis of the adrenal cortex of male rats maintained on normal rat chow, clofibrate (hypolipidemic drug), cholesterol-rich diet or cholesterol-rich diet plus clofibrate. The purpose of this study is to know whether the alteration in the serum cholesterol level is accompanied by an alteration in the ultrastructure and steroidogenic activity of the adrenal cortex, and to compare these changes with those of Leydig cells reported previously. The corticosterone level in both serum and the adrenal tissue of cholesterol-fed rats remained unchanged. Treatment of the cholesterol-fed rats with clofibrate caused a significant rise in the serum corticosterone level but the concentration in the adrenal remained unchanged. Lipid droplets increased in number and volume significantly in both cortical zones in all experimental groups as compared with the control group. But neither cholesterol feeding nor clofibrate treatment of normal rats caused a change in the volume or surface area of smooth endoplasmic reticulum (ER). Treatment with clofibrate on the cholesterol-fed rats resulted in a decrease in the volume of smooth ER in both cortical zones. Mitochondria showed no change except for a decrease in volume and inner membrane surface area of zona reticularis of cholesterol-fed rats. Peroxisomes did not proliferate but rather decreased in clofibrate-treated rats. These changes in the adrenal cortex were different from those in Leydig cells. Possible reasons for these differences were discussed.

Adamantinoma of the tibia -- a case report and a statistical review of reported cases.

A case of adamantinoma of the tibia is reported. A 70-year-old man complained of a painful swelling over the lower front of the right tibia. Roentgenograms showed an oval well-circumscribed, multilocular cystic lesion. Curettage and bond grafting were performed, and 17 months later the patient is in good condition. Microscopically the tumor tissue consisted mainly of epithelial islands with an palisading at the periphery and a loose reticular pattern in the center of the tumor cell nests, showing a close resemblance to the ameloblastoma of the jaw bones. Electron micrographs revealed the epithelial nature of the tumor cells, l.e., a continuous basal lamina, desmosomes and bundles of microfilaments, probable tonofilaments. One hundred fifty six cases of this peculiar primary skeletal bone tumor previously reported in the literature were reviewed and discussed.