Aggressive angiomyxoma in males. A report of four cases.

Aggressive angiomyxoma (AAM) is a rare, locally infiltrative but nonmetastasizing tumor of the pelvic and perineal soft tissues that occurs almost exclusively in adult females. The authors describe four cases of AAM in adult males that arose in the scrotum. There was some histologic variation among the tumors.

Nodular spindle-cell vascular transformation of lymph nodes. A benign process occurring predominantly in retroperitoneal lymph nodes draining carcinomas that can simulate Kaposi's sarcoma or metastatic tumor.

We describe a vasoproliferative nodular spindle-cell lesion representing a variant of vascular transformation of lymph-node sinuses and designated a nodular spindle-cell vascular transformation of lymph nodes. The lesion is most frequently identified in retroperitoneal lymph nodes excised in association with radical nephrectomies for renal cell carcinoma, but it can also be present in association with other malignant tumors. Occasionally it is seen in superficial lymph nodes in patients with no history of malignant neoplasms.

Diffuse idiopathic pulmonary neuroendocrine cell proliferation presenting as interstitial lung disease.

Pulmonary neuroendocrine cell (PNEC) hyperplasia typically occurs as an adaptive response in persons living at high altitudes and as a reactive response in the setting of lung injury. However, previous studies suggest that PNEC hyperplasia can occur in the absence of preexisting lung disease and may even give rise to airway disease through the development of pulmonary tumorlets and airway fibrosis and perhaps the release of paracrine secretions. We describe a patient with diffuse PNEC proliferation of a probable hyperplastic nature developing in the absence of a chronic pulmonary disorder who presented clinically with an interstitial lung process.

Carcinoma of the thymus with clear-cell features. Report of eight cases and review of the literature.

We report eight cases of thymic carcinoma with clear-cell features and review the five previously reported cases. The tumor was composed of cells with clear cytoplasm and rather bland nuclear features, and showed a lobular or occasionally sheet-like growth pattern. Cytoplasmic glycogen was demonstrable in 92% of cases, whereas mucin was absent in all cases tested.

Liposarcoma of the anterior mediastinum and thymus. A clinicopathologic study of 28 cases.

We studied 28 cases of anterior mediastinal liposarcoma occurring in 16 males and 12 females with a mean age of 43 years (range, 14-72). Presenting symptoms included dyspnea (four cases) and chest pain (four cases), although 11 tumors were detected incidentally by routine chest radiography. Seven cases were believed to be located within the thymus.

Desmoplastic small round cell tumor of the pleura.

Three cases of desmoplastic small round cell tumor (DSRCT) with multiphenotypic differentiation, primary in the pleura, are presented. This is a previously unrecognized site for this tumor type. Two patients were male and one female aged 29, 24, and 17 years.

Florid vascular proliferation associated with neural and neuroendocrine neoplasms. A diagnostic clue and potential pitfall.

We report the light microscopic and immunohistochemical features of vascular proliferations associated with 26 extracranial neural and neuroendocrine neoplasms including esthesioneuroblastoma, neuroblastoma/ganglioneuroblastoma, the primitive neural component of immature teratoma, mediastinal teratoma, primitive neuroectodermal tumor, intra-abdominal desmoplastic small cell tumor, Merkel cell carcinoma of the skin, and thyroid medullary carcinoma. These vascular proliferations were similar to those associated with high-grade glial neoplasms and were characterized by tufts of vessels with a glomeruloid configuration or by long cords of vessels. Immunohistochemical evaluation documented the presence of endothelial cells, perithelial cells, and basement membrane components within the foci of proliferating vessels.

Detection of circulating tumor cells in patients with localized and metastatic prostatic carcinoma: clinical implications.

Purpose: To determine the frequency with which prostate-specific antigen (PSA)-positive cells can be detected in the peripheral blood of patients with prostatic cancer in different stages and with different sensitivities to hormonal therapy.

Patients And Methods: Peripheral blood from 107 men with prostatic cancer and 27 non-prostate cancer controls was analyzed for PSA mRNA using reverse-transcriptase polymerase chain reaction (RT-PCR) and Southern blotting.

Results: The lower limit of detection was one PSA-producing cell diluted into 1 x 10(6) blood mononuclear cells.

Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor.

Desmoplastic small round cell tumor is a recently recognized distinctive tumor shown to be associated with a recurrent translocation, t(11;22)(p13;q12), and rearrangement of the genes for Ewing sarcoma (EWS) and Wilms tumor (WT1). A genomic DNA fragment containing the EWS-WT1 gene fusion has been isolated from a desmoplastic small round cell tumor, and the breakpoint has been characterized. The breakpoints involve the intron between EWS exons 7 and 8 and the intron between WT1 exons 7 and 8.

Isolated clinical syndrome of primary aldosteronism in a patient with adrenocortical carcinoma. Case report and review of the literature.

Primary carcinoma of the adrenal cortex is an extremely rare neoplasm, accounting for an estimated 0.05-0.2% of all malignancies.

Standardization of the surgical pathology report: formats, templates, and synoptic reports.

The most important routine vehicle for communication in surgical pathology is the specimen report. Although accuracy, clarity and thoroughness are the main goals, significant variability in format and content exists. In an effort to make reports more consistent, several mechanisms are useful and amenable for use in a computerized environment.

Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit.

The distinction of solitary fibrous tumors (SFTs) from histologically similar neoplasms relies heavily on a characteristic microscopic appearance. No discriminating ultrastructural or immunohistochemical features are known. We evaluated 22 SFTs and 118 other tumors often considered in the differential diagnosis for immunoreactivity using a monoclonal antibody directed against the human hematopoietic progenitor cell antigen, CD34.

Mixed acinar-endocrine carcinomas of the pancreas.

Acinar cell carcinoma is a rare pancreatic neoplasm that may contain scattered endocrine cells in as many as 40% of cases. In addition, unusual tumors exist in which the acinar and endocrine components each constitute a significant proportion (> 25%) of the neoplasm; we propose to designate them as "mixed acinar-endocrine carcinomas." In a study of five such cases, we found one case with segregated areas of acinar and endocrine cells that were identifiable in routinely stained sections and four cases with morphologically uniform cell populations where the divergent differentiation was only detected immunohistochemically.

Myogenic regulatory protein expression in adult soft tissue sarcomas. A sensitive and specific marker of skeletal muscle differentiation.

Myogenic regulatory protein (MyoD1) is a DNA-binding nuclear protein that initiates myogenesis in mesenchymal stem cells. Its expression has proved a very sensitive marker of myogenic differentiation in malignant tumors of childhood. In this study, the reliability of MyoD1 expression as marker of skeletal muscle differentiation has been tested in 38 cases of adult-type sarcomas.

Renal myxoma. A report of two cases and review of the literature.

Renal myxomas are rare neoplasms. Seven cases have been reported, of which only two are convincingly diagnosed as myxoma; the remaining cases exhibit features of sarcoma, fibroepithelial polyp, or myxolipoma. We report two additional cases; one in a 52-year-old man and another in a 68-year-old woman.

Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases.

Fifteen cases of a distinctive type of low-grade angiosarcoma of the skin are described. Most tumors presented in the second to fourth decades of life, the youngest patient being 9 years old and the oldest 78 (mean age, 36 years). There was no sex predilection.

p53 mutations in human bladder cancer: genotypic versus phenotypic patterns.

The objective of this study was to characterize the pattern of p53 mutations in bladder cancer. The sensitivity and specificity to detect these mutations using clinical material was assessed for the following assays: immunohistochemistry, restriction-fragment-length polymorphism, single-strand-conformation polymorphism, and sequencing. Discrepancies of reported results aimed at the identification of genetic alterations in the p53 gene may be due to differences in methodology, as well as to deficient morphological evaluation of the source of tissue utilized.

Salivary gland anlage tumor ("congenital pleomorphic adenoma"). A clinicopathologic, immunohistochemical and ultrastructural study of nine cases.

Salivary gland anlage tumor (SGAT) is a polypoid lesion of the nasopharynx that presents with respiratory distress at birth or within the first few days or weeks of life. Among our nine cases, there was a male predilection (7M:2F). All tumors were in the midline and attached to the posterior pharyngeal wall by a delicate pedicle.

Analysis of nuclear and mitochondrial DNA alterations in thyroid and renal oncocytic tumors.

The search for mitochondrial DNA (mtDNA) defects in oncocytic neoplasms has been the subject of several recent studies. We have performed qualitative and quantitative analysis of nuclear and mitochondrial DNAs in a series of 19 renal and 12 thyroid oncocytic tumors to identify specific alterations that might predict the clinical and biological behavior of these tumors. Allelic losses were seen in 2 of 19 renal tumors and 5 of 12 thyroid tumors.

Standardized reporting of surgical pathology diagnoses for the major tumor types. A proposal. The Department of Pathology, Memorial Sloan-Kettering Cancer Center.

A set of standardized surgical pathology forms for the reporting of the major tumor types is presented. The purpose of this proposal is to ensure that the essential morphologic information is incorporated into all reports in a thorough and consistent fashion.