Histopathologic reproducibility of thyroid disease in an epidemiologic study.

An investigation of the long-term effects of childhood scalp irradiation demonstrated a significantly increased risk of thyroid tumors in the irradiated population. Because of the complexity of thyroid cancer diagnosis, a histopathologic slide review of 59 of the 68 patients (irradiated and nonirradiated) with thyroid disease was undertaken. The review revealed 90% agreement (kappa = +0.85, P less than 0.01) between the original and review diagnosis. Four of 27 cases previously diagnosed as malignant were reclassified as benign, yielding a cancer misdiagnosis rate of 14.8%. All four of the misdiagnosed cancers were of follicular or mixed papillary-follicular type. As a result of the histologic review, the ratio of malignant to benign tumors decreased from 2.55 to 1.75. Since disagreement in diagnosis was similar in the irradiated and nonirradiated groups, the relative risk of radiation-associated neoplasms did not change substantially. The histopathologic review shows that although there were some problems in diagnostic reproducibility, they were not statistically significant and did not alter our previous conclusions regarding radiation exposure. However, a 15% reduction in the number of malignancies might affect epidemiologic studies with an external comparison as well as geographic or temporal comparisons.

Histopathological features of the skin in hypopituitarism and Laron-type dwarfism.

Skin biopsies from children and adolescents with various syndromes of somatomedin deficiency revealed changes in structure and in distribution of the dermal elastin fibers as compared with a group of seven healthy control subjects. In 18 patients with isolated growth hormone deficiency (IGHD) and in 11 with multiple pituitary hormone deficiencies (MPHD), the number of elastin fibers was reduced; the individual fibers were shorter and slimmer than usual, and frequently arranged in groups dispersed in various directions. Skin biopsies from six patients with Laron-type dwarfism (LTD) revealed mostly thickened elastin fibers, frequently arranged in irregular bundles, but the number of elastin fibers was normal in prepubertal patients and reduced only in postpubertal patients. The reduction of elastin fibers in these patients was less prominent than in IGHD. The finding of more numerous elastin fibers in LTD than in IGHD is suggestive of a direct nonsomatomedin-mediated effect of human growth hormone on skin elastogenesis.

Ovarian endometrioid tumors mimicking Sertoli and Sertoli-Leydig cell tumors: Sertoliform variant of endometrioid carcinoma.

We have encountered four cases of an unusual variant of well-differentiated endometrioid carcinoma that was predominantly composed of tubules, solid or hollow, as well as cord-like areas histologically mimicking Sertoli and Sertoli-Leydig cell tumors. The two features most helpful in differential diagnosis were the presence of areas of tumor with the typical confluent pattern of endometrioid carcinoma, and the presence of mucin at the apical borders of the tumor cells and/or within glandular lumina. Other features that were helpful if present, but were observed only in one case each, were foci of squamous metaplasia or the presence of ciliated epithelium. In two cases, ultrastructural studies showed well developed microvilli and perinuclear microfilaments confirming the endometrioid nature of the neoplasm. The patients varied from 22-74 years in age. All tumors were confined to a single ovary, and no tumor is known to have recurred or metastasized. One of the patients died at age 80, six years following operation, presumably without evidence of recurrent neoplasm or metastases. Two other patients are living and well, one and 14 years after diagnosis. In one patient follow-up is short. The clinicopathologic features of this variant of endometrioid carcinoma are reviewed with emphasis on differential morphologic features.

Tricuspid stenosis due to intravenous leiomyomatosis--a call for caution: case report and review of the literature.

Extension of a benign intravenous leiomyomatous tumor from the pelvis into the right atrium resulted in severe tricuspid stenosis in a 28-year-old woman. Absence of the Budd-Chiari syndrome and other abdominal manifestations led to a misdiagnosis of a primary right atrial tumor, with a fatal outcome during operation. In our search of the English literature, this was only the second case found in which extensive intravenous leiomyomatosis interfered with the tricuspid valve mechanism. In this report, the clinical picture, etiology, and treatment are discussed, along with the literature on this rare entity.

Malignant cervical teratoma of the fetus.

Cervical teratomas have been defined and one new case is now added to the 116 cases previously reported. We have been able to find only six reports of malignant teratoma in the literature so far. The problem of diagnosis and obstetric management are discussed briefly. The incidence of appearance of the tumor is described and the likelihood of hydramnios or of acute obstructive symptoms of the trachea and esophagus at birth are discussed. The differential diagnosis includes cystic hygroma, congenital goitre, bronchial cyst, lymphangioma, neuroblastoma, parotid tumor and carcinoma of the thyroid. The object of this review is to present a synoptic description of these tumors, their clinical manifestations, management, treatment and prognosis, so that it may afford the attending obstetrician a guideline in understanding this rare entity.

Continuous high-dose intravenous desferrioxamine treatment for iron overload.

We describe a patient with heavy posttransfusion iron overload, to whom continuous high-dose i.v. desferrioxamine was administered shortly before his death. The administration of nearly 500 g of this agent was followed by urinary excretion of 5.4 g of iron. The chelator therapy lost its efficiency in the last of five courses and the patient eventually died as a result of cardiac and hepatic damage related to iron overload. The theoretical and practical implications of these observations are discussed.

Experimental limb dysmorphogenesis as a model of chemical injury response in undifferentiated embryonic tissues: a light and electron microscopical study.

A histopathological and ultrastructural analysis of the early stage of limb dysmorphogenesis from 1 hr to 48 hr, following citral administration is presented as a model for studying the reaction to injury of undifferentiated embryonic tissues. Early lesions such as cloudy swelling, hydropic degeneration, enlargement of the extracellular space and retraction of protoplasmic extensions were observed 1 hr after treatment, affecting both epiderm and mesenchyme of the limb. Few pyknotic cells were encountered during the early stages. Cell necrosis and autophagocytosis became more conspicuous at 12 hr post-injection. About the same time the first signs of healing were observed, charcterised by heterophagocytosis and return of mitotic activity. The epiderm being more differentiated, regenerates ad integrum and more rapidly than the mesenchyme. The evolution of these pathological events, which proceed in a spatio-temporal cascade, seem to reflect the pharmacokinetic gradient of the cell-teratogen interactions. The type of limb malformation will depend mainly on the extent of injury and/or healing of the mesenchymal mass, rather than on the injury and/or healing of the surface ectoderm. It is concluded that the reaction to chemical injury of undifferentiated tissues partially mimics the destructive and repair process in adult tissues, but differs in lacking some basic constituents such as oedema, monocyte exudation and granulation tissue formation.

Selective oocyte degeneration and impaired fertility in rats treated with the aliphatic monoterpene, Citral.

Mature female rats treated with Citral (3-7-dimethyl-2,6-octadienal) either topically for 60 or 100 days or by 6 i.p. injections (at 4-5 day day intervals) showed a marked decrease in the number of normal follicles per section, because oocytes tended to degenerate although the follicular cells remained normal. The reproductive performance after Citral treatment was impaired: there was a reduction in implantation number and litter size and an increased post-implantation fetal wastage. None of the young survived after 100 days of topical Citral treatment. It is suggested that Citral directly affects the oocytes.

Pulmonary alveolar proteinosis associated with fanconi's anemia.

A 16-year old patient with Fanconi's anemia developed pre-leukemia and pulmonary infiltration which was found upon autopsy to be pulmonary alveolar proteinosis. The question whether the pulmonary alveolar proteinosis was due to the marked leukopenia and an eventual defective phagocytic ability of the leukocytes is raised.

Cysts of the female external genitalia in the newborn infant.

Nineteen cases of cysts of the external female genitalia were diagnosed over a two-year period, giving an incidence of six per 1,000 female infants. The embryology, morphology, and differential diagnosis are discussed. No treatment was given and follow-up at two months of age revealed complete resolution of the cysts, thereby establishing their benign course in the newborn period.

Glycogen metabolism in the placenta of streptozotocin diabetic rats.

Administration of STZ prior to mating, induced significant impairment in glycogen metabolism in term rat placentae. Glycogen retention was observed in the treated placentae, while glycogen synthetase activity was reduced. The glycogenolytic pathway seemed to proceed mainly through amyloglucosidase enzyme whose activity increased threefold. Glucose-6-phosphatase showed a moderate activation, mainly in the labyrinth zone, while phosphorylase was slightly inhibited. These changes were accompanied by a decrease in placental insulin levels. The possible defensive role played by the placenta in cases of maternal hyperglycemia was postulated.

Incipient histopathological lesions in citral-induced microphthalmos in chick embryos.

Microphthalmos was indeed experimentally in 3-day-old chick embryos by intraamniotic injection of citral. Various degrees of this particular microphthalmic syndrome were observed. Severe cases were always accompanied by destructive changes in the ipsilateral part of the head. The corneal epithelium lost its continuity and the lens showed, even in the early stages, severe degenerative changes with spherophakia. The neural retina seemed to be affected later and showed hyperplasia and fold formation, while the retinal pigment epithelium was only mildly affected. The mesenchyme behaved differently according to its location: the superficial corneal and ciliary mesenchyme underwent hyperplasia, while the deeper choroidal mesoderm showed edematous changes only. This temporal and spatial distribution of pathological changes in the eye structures favors the assumption that citral acts locally. The experimental microphthalmic syndrome is not only due to the direct effect of the exogenous teratogenic factor on the ocular anlagen but is also the result of subsequent adaptive changes of these structures to the new environmental condition.