Coarctation of the left pulmonary artery: effects on the pulmonary vasculature of infants.

At autopsy, two infants had unsuspected coarctation of the left pulmonary artery (CoLPA), which was produced by an extension of ductal tissue into the wall of the left pulmonary artery. The first case, a 4-month-old girl, also had a ventricular septal defect and an anomalous branching pattern of the innominate arterial trunk. Pulmonary arterial hypertensive changes were noted in the right lung. In contrast, the left lung showed thin-walled pulmonary arteries. The second case, a term female newborn, had exhibited severe unexplained respiratory distress since birth. Histologic sections of the right lung showed dilated pulmonary arteries with thinned media, whereas the left lung showed a persistent fetal arterial pattern. It is believed that the peripheral pulmonary arterial changes are age-dependent and associated with asymmetric blood flow between the right and left pulmonary arteries. CoLPA is a rare pulmonary artery defect, and early diagnosis of this abnormality is important.

Angiodysplasia of colon revisited: pathologic demonstration without the use of intravascular injection technique.

The recognition of angiodysplasia either at the time of surgery or at the time of routine gross and microscopic examination has often been difficult. Until now the primary methodology used in defining the lesion has been the intravascular injection of radiopaque dyes and other compounds. This technique, however, is prohibitively time consuming and expensive, and does not encourage routine use in surgical pathology practice. We report two cases of angiodysplasia, both occurring in unlikely areas, in which the pathologic lesion was demonstrated without the use of an intravascular injection technique. One case involved 35 cm of proximal transverse colon and the other involved 23 cm of colon distal to the ileocecal valve. A simple method of demonstrating angiodysplasia by intraluminal formalin fixation (37% concentrated, unbuffered), with tying of both resected ends for 3 hours, followed by dissection of the mucosa from the muscle wall, is described. Areas of ectatic, pericryptal, thin-walled blood vessels with adjacent dilated, engorged submucosal veins are readily seen on gross direct inspection highlighted by transillumination. Histologic sections taken from these areas show early lesions of angiodysplasia characterized by ectatic, engorged submucosal veins and some dilated venules and capillaries in the mucosal lamina propria.

Verrucous carcinoma of the leg positive for human papillomavirus DNA 11 and 18: a case report.

Human papillomavirus (HPV) types 6 and/or 11 have been associated with benign lesions, while types 16, 18, 31, and 33 are prevalent in malignant lesions. This case report describes the findings in a verrucous carcinoma of the leg, which was examined for HPV types 11, 16, and 18 by in situ DNA hybridization. The lesion gave positive results for HPV subtypes 11 and 18, a combination that, to our knowledge, has not been previously reported in this neoplasm.

Immunocytochemical estrogen and progestin receptor assays in breast cancer with monoclonal antibodies. Histopathologic, demographic, and biochemical correlations and relationship to endocrine response and survival.

Breast cancer specimens from 600 women were assayed for estrogen receptors (ER) using an immunocytochemical assay (ICA) employing the monoclonal antiestrophilin antibody H222 Sp gamma. Results showed significant correlation with biochemical ER determinations as well as with tumor grade and menopausal status. In 449 cases, results of progesterone receptor assay by ICA using the monoclonal anti-PgR antibody KD 68, also correlated significantly with biochemical PgR measurements. The ERICA/PgRICA positivity was significantly more frequent in postmenopausal white women. Colloid carcinomas were most likely to be ERICA positive and PgRICA positive whereas medullary carcinomas were most often negative. In 47 patients with advanced mammary carcinoma, results of ERICA and PgRICA were more closely related to endocrine response than those of ER and PgR by dextran-coated charcoal assay (DCC). In 339 women with Stage I or Stage II breast cancer, ERICA was significantly associated with disease-free survival. Analysis by Cox's proportional hazard model, however, showed PgRICA to be the best predictor of survival and disease-free survival in 197 women at the same stages of disease. These data indicate that ICA is more predictive of prognosis than biochemical ER and PgR. The ease of ICA performance coupled with these results indicate that the method is an acceptable substitute for DCC in analyzing breast cancers for ER/PgR.

Mucoepidermoid carcinoma of uterine cervix stage IB. Long-term follow-up, histochemical and immunohistochemical study.

Twelve women with mucoepidermoid carcinoma of the cervix uteri were followed for 2-15 years after diagnosis. Three patients died within 14 months. All had lymph node metastases and/or vascular involvement and exhibited tumor invasion to a depth of 1.2-3.2 cm. Mucoepidermoid carcinoma is defined as a tumor with the appearance of squamous cell carcinoma without any glandular pattern and with demonstrable intracellular mucin. The mucin is best demonstrated by alcian blue and periodic acid-Schiff-diastase. In 265 cases of squamous cell carcinoma, stage IB, lymph node metastases were present in 14%. In the cases of mucoepidermoid carcinoma, the prevalence of nodal metastases was 33%. Because mucoepidermoid carcinomas appear to be more aggressive lesions than squamous cell carcinomas are, it may be advisable to stain all cervical squamous carcinomas for mucin if they demonstrate finely vacuolated cytoplasm and lack peripheral palisading. Immunohistochemical studies for carcinoembryonic antigen (CEA), keratin, and epithelial membrane antigen were positive in all tumors to varying degrees. The detection of CEA may be of additional help in establishing a diagnosis.

Immunocytochemical localization of estrogen and progesterone receptors in human thyroid.

The presence of steroid hormone receptors has previously been suggested in thyroid tissue by biochemical means. Our studies were designed to confirm these results and to localize the specific receptor-containing cell type using a novel immunocytochemical method. Monoclonal antibodies specific to estrogen receptors (ER) and progesterone receptors (PgR) were used to localize these steroid hormone receptors in the human thyroid gland. Frozen tissue sections from surgical specimens excised from 22 patients of both sexes with benign thyroid disease were studied. The sections were incubated with rat antiestrophilin and antiprogesterone receptor antibodies and were then exposed to rabbit anti-rat IgG and to rat peroxidase-antiperoxidase complex. The reaction product was visualized with diaminobenzidine tetrahydrochloride and hydrogen peroxide. Four specimens were positive for both ER and PgR, 16 were ER-positive and PgR-negative, and two were negative for both ER and PgR. Positive reactivity was limited to the follicular lining cell nuclei and varied from focal to diffuse. The immunohistochemical findings confirmed the presence of ER and PgR in the thyroid tissue and demonstrated for the first time that these receptors are present only in the nuclei of the lining cells of the thyroid follicle. The role of steroid hormone receptors in the thyroid in health and disease remains to be explained.

Immunocytochemical detection of progesterone receptor in breast cancer with monoclonal antibody. Relation to biochemical assay, disease-free survival, and clinical endocrine response.

A new immunocytochemical assay for progesterone receptor (PgR-ICA) employing the monoclonal antibody JZB 39 was used to study tumors from two series of patients with breast cancer. In Series 1 assay results were in agreement with those of biochemistry in 76% of 338 cases (P less than 0.001) and in 54% of 101 cases in Series 2 (P less than 0.001). Agreement was better in Series 1 because it included fresher, previously untouched specimens. There were 70 patients in Series 1 with known clinical endocrine response. A negative assay correlated with disease progression in 45 of 57 patients, significantly better than with biochemistry (P = 0.013). In comparing 39 women with rapid disease progression with 39 free of disease at 5.1 years, those with PgR-ICA-positive tumors were over four times more likely to remain disease-free than those with negative results (P = 0.007). Product moment life-table analysis of 79 patients from Series 2 showed a significantly better cumulative survival for those with PgR-ICA-positive tumors (P = 0.047). These findings indicate that PgR-ICA should be of value in planning therapy and predicting disease course in breast cancer patients.

Alleged resection of the vas deferens: medicolegal implications.

New York state law requires that significant complications be reported by telephone within 24 hours, followed by a full written report within five days with the names of the alleged involved physicians. This case illustrates the potential medicolegal consequences following the mandated immediate nonsubstantiated report of an alleged bilateral vas deferens injury during a bilateral inguinal herniorrhaphy in a 7-month-old male. Review of the surgical pathologic specimen showed that both hernial sacs did indeed contain segments of vas deferens structures, which based on the findings at reexploration, were consistent with duplication or embryonic remnants of the vas deferens. The vas deferens was found to be normal and uninjured bilaterally. The legal ramifications remain unclear. Since there is no clear cut method to retract the allegation, the physician's profile may be permanently damaged.

Children with AIDS--is pathologic diagnosis possible based on chest radiographs?

In a review of the clinical, radiographic and pathologic data of 36 patients with AIDS we found 29 with pulmonary infections (Cytomegalic virus, Pneumocystis carinii, etc.) and 7 who had varying types of lymphocytic infiltration and interstitial changes on chest radiographs but did not have opportunistic infection. Pathologic diagnosis included lymphocytic interstitial pneumonitis, bronchus associated lymphoid tissue, chronic interstitial pneumonitis, desquamative interstitial pneumonitis and immunoblastic sarcoma. The chest X-rays were not predictive in making a specific diagnosis.

Depletion of lymphoid tissues by tilorone. Ultrastructure and synergistic interaction with antilymphocyte serum.

Antilymphocyte serum (ALS) caused lymphocyte depletion and shrinkage of thymus-dependent areas in spleen and lymph nodes of rats. Tilorone also depleted T lymphocytes, but light- and electron-microscopic examination revealed hyperplasia and vacuolation of the interdigitating cells that remained in the thymus-dependent areas, rather than shrinkage. These changes were not mediated by the adrenal gland. The combination of agents had a synergistic effect on the thymus-dependent areas in the spleen, lymph nodes, Peyer's patches, and bronchial-associated lymphoid tissue, all of which were severely depleted but maintained their normal dimensions. The combination of ALS and a tilorone analog that was capable of depleting lymph node but not spleen, gave a synergistic effect only in lymph node and not in spleen. Tilorone effects were transient, whereas ALS produced a long-lasting depletion. Administration of the two drugs at different times were synergistic only during the brief period of tilorone's effectiveness. The synergistic effects on T lymphocytes correlated well with immunosuppressive potency of the combined drugs on a T-lymphocyte-mediated auto-immune disease.

Extranodal malignant histiocytic lymphoma of the cheek: report of case.

A case of extranodal malignant histiocytic lymphoma of the cheek in a 34-year-old man is presented. Successful regression of the tumor was achieved by radiotherapy.

Renal lesions induced induced by tilorone and an analog. Ultrastructure and acid phosphatase study.

Tilorone, 2,7-bis-(diethylaminoethoxy)-fluoren-9-one hydrochloride, an interferon inducer, has been shown to cause hydropic degeneration in choroid plexus and distal convoluted tubules of kidney. Ultrastructural and histochemical studies of the renal lesion produced by tilorone and one of its analogs revealed striking accumulation of vacuoles and/or acid-phosphatase-positive lemellar bodies retricted to glomeruli, ascending limb of Henle's loop, and distal convoluted tubules.

Renal failure secondary to angiomyolipoma. Case of Forme-Fruste tuberous sclerosis.

A fifty-four-year-old woman without the clinical features of tuberous sclerosis underwent nephrectomy at age thirty-three years for angiomyolipoma, and twenty-one years later severe renal failure developed. At necropsy the remaining kidney had extensive angiomyolipomatous involvement; not until the brain was examined was the diagnosis of tuberous sclerosis made. To date, this would appear to be the third case without clinical tuberous sclerosis in which renal involvement was the sole clinical expression of tuberous sclerosis, and the seventh reported instance of renal failure due to renal angiomyolipomatous hamartomatous transformation.

Chronic granulocytic leukemia cutis treated with hydroxyurea. Report of a case.

The clinical course of a patient with chronic granulocytic leukemia cutis associated with myeloid metaplasia is described. Cutaneous manifestations of leukemia appeared only at the sites of bone marrow aspiration and vena puncture. Radiotherapy to both spleen and skin and chemotherapy with busulfan failed to control spreading cutaneous lesions. Treatment with hydroxyurea was followed by a marked reduction in spleen size, a decrease in white cell count and healing of the skin lesions.

Cytology of transitional cell carcinoma in situ of urinary bladder with extensive prostatic involvement.

Cytologic differences in the urine of 6 patients with transitional cell carcinoma in situ (CIS) of the urinary bladder with and without prostatic duct involvement are described. In the former group, there was an admixture of CIS cells along with many bizarre malignant cells which increased with time and showed malignant criteria indicative of changes more than CIS alone.

Further observations on carcinoma in situ of the urinary bladder: silent but extensive intraprostatic involvement.

Of seven patients with carcinoma in situ of the urinary bladder in the absence of an associated papillary carcinoma, five were found to have silent but extensive intraductal prostatic involvement. In three of these the carcinoma in situ was associated with microinvasion. The mean age at the time of diagnosis was 68 years. All had symptoms characteristic of carcinoma in situ, including hematuria, dysuria, and urgency. In three patients the prostatic involvement was diagnosed on transurethral resection. In two it was discovered only after radical cystectomy. The prostatic involvement was neither suspected clinically nor has it been previously emphasized. Although three patients are alive, apparently free of disease up to 15 months postcystectomy, two have died, one of disease. The importance of prostatic assessment in the evaluation of the patient with carcinoma in situ of the urinary bladder is emphasized.