Radiologic, pathologic and molecular attributes of two types of papillary renal adenocarcinomas.

OBJECTIVE: Most papillary renal tumors are not as aggressive as clear cell carcinomas and thus carry a better prognosis. However, several reports in the literature have demonstrated a subset of patients with papillary tumors that have a more aggressive biology and advanced stage at presentation. We compared several parameters of these subsets of renal tumors in an effort to characterize these lesions. PATIENTS AND METHODS: We reviewed 391 cases of nephrectomies that were performed for cancer over a 20-year period from four institutions. Of these, 41 were documented as papillary adenocarcinomas. We reviewed these cases with respect to stage at presentation, size, vascularity on (computerized tomography) CT scan, histology, and cytokeratin immunohistology. RESULTS: Thirty-two of the lesions presented in the fifth, sixth, seventh and eighth decades of life (Type I), while most of the remaining 9 tumors (Type II) presented in the fourth decade of life, and in more advanced stages. Tumor volumes ranged from 84 cm3 to 1660 cm3. Type I tumors had an average size of 515 cm3 and an enhancement on CT of 36 +/- 4 Hounsfield units, compared with Type II tumors which had an average size of 164 cm3 and an enhancement on CT of 92 +/- 8 Hounsfield units. Type II tumors also had a higher mean Fuhrman score of nuclear pleomorphism than Type I, and a greater expression of cytokeratin. CONCLUSIONS: We found that the more common Type I variant of papillary renal adenocarcinoma was less vascular on CT scan, larger in size, and had a lower amount of nuclear pleomorphism as well as decreased expression of cytokeratin 7. The more aggressive biological variant, Type II, presented in the earlier decades of life, with a smaller, but more vascular, cancer and had a greater nuclear pleomorphism. Nuclear pleomorphism still appears to have the best prognostic assessment. However, other molecular and genetic parameters of these tumors, as well as long-term survival data will be necessary to determine the significance of these findings.

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.

Spontaneous renal hemorrhage associated with renal tumors.

Spontaneous ruptures of the kidney sometimes require emergency surgery, at which time the etiology for the rupture becomes evident. Because the patient with previously existing renal pathology is asymptomatic, when these ruptures do occur one should be suspect of underlying disease. We present a case and discuss the relevant aspects of such entities.

Kaposi sarcoma presenting as a vulvar mass.

BACKGROUND: Kaposi sarcoma has become a common manifestation in people with AIDS, especially men. A few reports of Kaposi sarcoma in women with AIDS have involved nongenital areas. However, of the few patients with genital Kaposi sarcoma reported in the United States, none was believed to be human immunodeficiency virus (HIV)-positive. Genital Kaposi sarcoma associated with HIV has been reported in other parts of the world. CASE: A 29-year-old black woman presented with severe vulvar pain, vaginal discharge, and a vulvar mass. She had been diagnosed with AIDS 25 months earlier. Biopsy of the vulvar mass revealed Kaposi sarcoma; viral analysis of the tumor was positive for herpes simplex virus type 2. Sequencing of polymerase chain reaction product verified the presence of human papillomavirus 26. CONCLUSION: We report an HIV-associated Kaposi sarcoma presenting as a vulvar mass. This report should alert health care providers to include Kaposi sarcoma in the differential diagnosis of vulvar lesions.

Perforation of the ileum secondary to Enterobius vermicularis report of a rare case.

An isolated ileal perforation is presented in a 12-yr-old patient. Microscopic examination revealed granulomatous inflammation due to Enterobius vermicularis (pinworm). This is only the third documented report of intestinal perforation involving pinworms. Although infestation with this parasite is generally considered to be relatively harmless, on rare occasions, it can be a significant cause of morbidity due to its potential ability to invade the tissues.

Benign lymphoepithelial lesions of the parotid.

Persistent generalized lymphadenopathy has been well described in patients with seropositivity to the human immunodeficiency virus (HIV). Moreover, isolated enlargement of the parotid gland and parotid lymphadenopathy have been noted much more frequently over the past few years. Histologically, these lesions demonstrate follicular hyperplasia, cystic dilatation of the ducts lined by pseudo-stratified squamous epithelium, and lymphocytic infiltrates. They are generally considered to be benign lymphoepithelial lesions of the parotid or hyperplastic periparotid lymph nodes. The relationship of this entity to the AIDS-related complex (ARC) and the subsequent development of AIDS is not clear. Over the past 7 years, we have seen 50 patients with parotid enlargement in whom the diagnosis of benign lymphoepithelial lesion was made. Fine-needle aspiration was performed in 32 patients. Although not conclusively diagnostic, needle aspirates ruled out primary salivary glandular pathology. Most patients gave a history of intravenous drug abuse. HIV tests have been performed on a routine basis only in the last 2 years, and these were positive in the majority of the patients. Thirty-five patients underwent surgical excision. In the initial 20 patients, we routinely performed parotid exploration, identification of the facial nerve, and superficial parotidectomy. In the last 15 patients, we changed our surgical approach to parotid exploration and excision of the mass in the tail of the parotid. The exposure of the posterior belly of the digastric muscle, with identification and removal of the deep jugular node, has become routine. In each case, we found an enlarged lymph node in the deep jugular region, which was not clinically palpable preoperatively. The rate of surgical complications was minimal, and, after resection of the mass, patients improved symptomatically. If the patient shows obvious signs of AIDS, a nonsurgical approach with repeated aspirations should be considered, and treatment with zidovudine offered.

Necrotizing pancreatitis and multisystem organ failure associated with toxoplasmosis in a patient with AIDS.

Extraneural manifestations of toxoplasmosis often are not recognized antemortem in patients with AIDS. We describe a patient who was seropositive for human immunodeficiency virus and presented with lethargy, abdominal tenderness, rapidly progressive ventilatory failure, rhabdomyolysis, myoglobinuria, and disseminated intravascular coagulation. Although the diagnosis of pancreatitis was not considered while the patient was alive, an autopsy demonstrated pancreatic necrosis associated with toxoplasmal cysts. No other infection was evident. This case suggests that Toxoplasma gondii can cause severe pancreatitis in patients with AIDS.

Steroid hormone receptor immunohistochemistry and amplification of c-myc protooncogene. Relationship to disease-free survival in breast cancer.

BACKGROUND: It is important to develop parameters that aid in prognosticating which patients with breast cancer are more likely to have a rapid disease course and therefore might benefit from early aggressive therapies. METHODS: Specimens from two groups of women, deliberately selected because their clinical courses differed greatly, were studied to detect amplification of the protooncogenes c-myc, int-2, and C-erbB-2/neu by slot-blot assay, the estrogen receptor (ER), and the progesterone receptor (PR) by both biochemical and immunohistochemical procedures (ERICA and PRICA). One group of 50 patients had a prolonged disease-free interval after initial surgery (mean, 6.4 years); the other group of 52 women had had rapid disease recurrence (mean, 1.4 years) or progression (5 patients died of disease within 1 year of diagnosis). The patients were selected from 1700 consecutively accessioned cases if they fit the study criteria and sufficient tissue was available for oncogene hybridization studies. RESULTS: The two groups differed statistically by stage, number of involved axillary lymph nodes, ERICA and PRICA results (P = 0.001), and amplification of c-myc (P = 0.003). The percentage of patients with rapid disease recurrence and progression increased from 0-93% when risk factors changed from best case (ERICA and PRICA results, positive; c-myc, not amplified; and axillary nodes, not involved) to worst case (ERICA and PRICA findings, negative; c-myc, amplified; and axillary nodes, involved). CONCLUSIONS: Women with these worst-case parameters were more likely to have a recurrence sooner and rapidly progressive disease. They might benefit from early aggressive therapeutic measures.

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.

Diminished survival of young blacks with adenocarcinoma of the prostate.

The survival rates of 117 black and white patients treated by primary radiation for carcinoma of the prostate at the State University of New York Health Science Center at Brooklyn and Kings County Hospital Center were analyzed according to age and race. In addition, stage, grade, and delay time in seeking medical attention were analyzed. Survival was similar in both young (less than 60 years) and old (greater than or equal to 60 years) patients, with 45% and 41% 5-year-survival rates, respectively. Survival was better in white patients, 48% 5-year survival, than in blacks, 35% 5-year survival. Black patients presented with higher stage disease than whites (p less than 0.01). This trend was even greater in young black males, who had higher grade (64% versus 11%; p less than 0.04) and higher stage tumors (p less than 0.05). In addition, young blacks delayed seeking medical attention greater than 3 months 72% of the time, as compared to 0% in white young males (p less than 0.005). A survival difference was also seen in young blacks as compared with young whites: 3.9-year median survival versus 6.0-year median survival, respectively.

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.

Comparative yield of blood culture for fungi and mycobacteria, liver biopsy, and bone marrow biopsy in the diagnosis of fever of undetermined origin in human immunodeficiency virus-infected patients.

The diagnostic yield of mycobacterial blood cultures, bone marrow biopsy, and liver biopsy for determining the cause of unexplained fever was compared prospectively in eight men and four women with serologic evidence of human immunodeficiency virus infection and fever of undetermined origin. Mycobacterial infection was found in 8 of the 12 patients (Mycobacterium tuberculosis in 3 and Mycobacterium avium in 5). Mycobacteria were isolated from the blood of 6 of these 8 patients. The mean interval from blood culture inoculation to growth was 28 days. Acid-fast organisms or granulomas were seen in four bone marrow and six liver specimens. Liver biopsy revealed acid-fast bacilli in a higher percentage of cases (75%) than did bone marrow biopsy (25%). Mycobacterial blood culture is a relatively slow method that occasionally fails to diagnose mycobacterial infection. In febrile patients infected with human immunodeficiency virus, liver biopsy is the most rapid method of diagnosing mycobacterial infection.

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.

Diffuse cystic angiomatosis of the breast.

Vascular tumors of the breast are extremely rare, and the majority are malignant. In this report, the presentation of a diffuse, benign, cystic, vascular tumor that histologically was angiomatosis of the breast is described. The clinical presentation, magnetic resonance imaging (MRI) appearance, and histologic features of this rare lesion are reviewed. This case is of particular interest because the tumor recurred during pregnancy and underwent massive enlargement despite negative hormone receptor values. Angiomatosis is prone to local recurrence, so complete excision with histologically negative margins is the treatment of choice.