Adrenal hemorrhage in the adult.

Bilateral adrenal gland hemorrhage was found in 22 of 2,000 (1.1%) consecutive general hospital autopsies. Clinical features of these 22 patients with diffuse or focal bilateral adrenal hemorrhage have been analyzed and compared with previous series. In our experience, patients with this postmortem finding rarely manifest features of adrenocortical insufficiency and appear instead to die as a consequence of concomitant overwhelming illness, such as septicemia, body surface burns or cardiovascular catastrophe. Two-thirds of the current series had impaired renal function at the time of adrenal hemorrhage and three patients had pituitary gland necrosis. Experimental and clinical observations indicate that the "stressed" adrenal gland--under substantive endogenous or exogenous ACTH stimulation--is unusually susceptible to hemorrhage. While our own experience indicates that bilateral adrenal hemorrhage can infrequently be implicated as a factor contributing to patients' death, the possibility of adrenocortical insufficiency must be considered in patients at risk for adrenal hemorrhage. Such patients are those who are azotemic and have bacteremia, burns, or recent cardiovascular catastrophe, particularly when the latter is managed with anticoagulant administration. In these patients, unexplained clinical deterioration or the appearance of findings consistent with adrenocortical insufficiency mandate measurement of serum cortisol concentration and institution of stress-level corticosteroid replacement therapy until a diagnosis of acute adrenocortical insufficiency can be established or refuted.

Luteoma of pregnancy: ultrastructural features.

The ultrastructural configuration of the cells in one case of pregnancy luteoma was studied by conventional electron microscopy. The fine structure of these cells conforms closely to that of steroid hormone producing cells in other human organs, such as the adrenal cortex, testicular interstitium, and corpus luteum, particularly in terms of the presence of abundant smooth endoplasmic reticulum, dispersed Golgi apparatus, and tubular cristae in the mitochondria. Similarities were noted between the pregnancy luteoma cells and, as described by other authors, the luteinized granulosa and theca cells of the human corpus luteum and the cells of spontaneous mouse luteoma. There was a more pronounced resemblance to the granulosa cells of the corpus lutemum than to the luteinized theca cells or the mouse luteoma cells but with considerable overlap among all of them. Distinctive features, not previously described, were noted in the pregnancy luteoma cells in the form of deep cytoplasmic invaginations of the cell membrane closed by tight junctions, and peculiar pleated sinuous membranous arrays within the cytoplasm. The significance of these structures is not known. In addition mesenchymal cells of ambiguous differentiation were noted in the stroma of the tumor, suggesting that the stimulus to luteinized cell proliferation may transcend the specific ovarian mesenchymal cells and may also be exerted on the supporting stroma. On the basis of previous reports concerning the biochemical and biosynthetic patterns of sex steroid hormone production by these tumors, it is concluded that although the latter recapitulate the endocrine functions of the normal ovarian stroma rather than those of the corpus luteum, the cytoarchitecture of these tumors covers a much broader spectrum of differentiation, which may come to resemble closely although not exclusively that of luteinized granulosa cells.

Primary vulvar neoplasia: a review of in situ and invasive carcinoma, 1935-1972.

This survey reports the past 38 years of experience with 192 cases of carcinoma of the vulva seen and treated at the Johns Hopkins Hospital. The review stresses the increased incidence of in situ neoplasia and the importance of individualization of therapy. Also the changing concepts in terminology (eg, the leukoplakic vulvitis of the past is the dystrophy of the present) suggest that the precursory alterations of previous discussions must be reviewed in the light of such an altered nomenclature. Features of epidemiologic and histologic importance are discussed.

PIP: Vulvar neoplasia as seen at the Johns Hopkins Hospital during a 38-year period (1935-1972) is reviewed. Of 1385 tissue specimens, 1053 were benign and 332 were malignant lesions. Of the malignant lesions, 246 were squamous cell carcinoma, both in situ and invasive. Of these, 192 were treated and followed and are reported on. There were 71 cases of in situ and 121 of invasive cancer. Of the patients, 64% were white and 36% were nonwhite, which corresponds to the patient population treated at the hospital. Ages of patients ranged from 21 to 86 years, with a median of 50 years. Of those with invasive cancer, 75% were postmenopausal and none was under the age of 30 years. There was a 29% incidence of nulliparity and a 64% incidence of obesity. Diabetes was noted in 64%. Syphilis was discovered in 26%. These patients had a high risk of being exposed to other venereal infections, particularly herpes which is suspect as a precursor of neoplasia. Vurrucous carcinoma are also likely to be of viral origin. Other malignancies were also present in 20% of patients. There was 1 case of chronic clyphocytic leukemia. Presenting symptoms were a lump, a white patch, pruritus, or bleeding. Pruritus was present in 46%. A leukoplakialike appearance was noted in most of the in situ lesions. Multicentric foci of origin were demonstrated in 35%, mostly in the in situ cases. Of the invasive cancers 60% were well differentiated and 11% were verrucous. Multiple histologic patterns were present in many cases. In 167 patients (67%), the initial treatment was surgical. Postoperative radiation was used in 30% of those with invasive cancer. Local recurrences followed in 22%. In those with multicentric foci the recurrence rate was 48%. 44 patients were known to have died, mostly from other causes. Survival was directly related to the stage of the disease at the time of initial diagnosis and treatment.

Luteomas of pregnancy.

Since its original description in 1963, the pregnancy luteoma has been a controversial entity both clinically and pathologically. The most important questions which have been asked are: Is the luteoma a true neoplasm or does it represent simply an abnormal response to the hormones of pregnancy? What is the relation between the tumor and certain endocrinopathies that are associated with these lesions? This study of 20 cases collected from the files of the Emil Novak Ovarian Tumor Registry and the Pathology Section of the Baltimore City Hospitals directs its attention to these critical clinicopathologic features.

Hydronephrosis, renal agenesis, and associated genitourinary anomalies in ACI rats.

The ACI rat provides a congenital mammalian model for the spontaneously occurring defects of renal agenesis and hydronephrosis. These anomalies appear to be part of a spectrum of abnormalities caused by a mesonephric duct defect. These same anomalies also exist in man probably more commonly than previously appreciated.

Pulmonary tumor emboli and cor pulmonale in primary carcinoma of the lung.

Tumor embolization was found at autopsy in the pulmonary arteries of 33 of 331 patients dying with primary carcinoma of the lung (other than oat cell carcinoma). Venous or lymphatic vascular involvement, or both, was greater frequency of tumor embolization htan epidermoid carcinomas (16.5% vs. 5.8%). In 16 of the 33 cases, diffuse, obliterative intimal fibrosis was found in small arteries affected by the tumor embolization. Enlargement of the right cardiac ventricle was present in 26 of the 33 cases. Dyspnea as the initial symptom or as one of the major symptoms, occurred in 21 patients. The average interval between onset of dyspnea and death was 4 weeks. It is concluded that pulmonary arterial embolization is a relatively frequent complication of primary lung carcinoma, particularly adenocarcinoma, which may lead to the developemnt of pulmonary hypertension and cor pulmonale Dyspnea, occurring de novo in patients with primary lung carcinoma is an ominous sign and should alert the clinician to the possibility of arterial pulmonary tumor embolization.

Meningeal carcinomatosis.

An anatomopathologic study of 18 cases of pure meningeal carcinomatosis is presented. In five of these cases, the brain, spinal cord, choroid plexuses, cerebral vessels, and prevertebral soft tissues, including the lumbosacral nerve plexuses and ganglia, were examined microscopically in an attmept to determine the routes of tumor spread. Our results suggest that the malignant cells reach the cerebrospinal leptomeninges via perineural, endoneural, and perivascular lymphatics and sheaths through the intervertebral and possibly cranial foramina. Involvement of the choroid plexuses appears to be secondary to, rather than the avenue for, leptomeningeal carcinomatosis, with the tumor cells reaching the choroid plexuses via the perivascular sheaths of choroidal vessels. Leptomeningeal carcinomatosis was the only manifestation of metastatic spread beyond regional lymph nodes in about 40% of all reported cases in which this information is available. This implies that radiation or other forms of local therapy to the cerebrospinal leptomeninges may provide an effective means of palliation in many of these cases.

Subependymal and intraventricular hemorrhage in neonates. Early diagnosis by computed tomography.

Computed tomography was used to evaluate subependymal and intraventricular hemorrhage in 11 formalin-fixed brains obtained at autopsy from newborn infants from a population with a known high incidence of cerebral hemorrhage. After scanning, the brains were step-sectioned at levels corresponding to those of the scans. Subependymal and/or intraventricular hemorrhage was demonstrated in 6 of the 11 brains. There was a very close correlation between the scan images and the gross pathological findings in the affected brains. The authors conclude that computed tomography is an accurate method for the diagnosis of neonatal cerebral hemorrhage.