Gene expression patterns of paired bronchioloalveolar carcinoma and benign lung tissue.

A variant of adenocarcinoma, bronchioloalveolar carcinoma (BAC), has increased in incidence since 1950 and now represents 2-14% of all lung cancers. There has been concomitant diminution in the proportion of squamous cell carcinoma, the most common form of primary lung cancer. The BAC form of adenocarcinoma occurs disproportionately in women, has an earlier age of onset than conventional pulmonary carcinoma, and is not linked to smoking. The increased incidence of BAC in both smokers and non-smokers suggests that BAC may have an environmental etiology other than smoking. To explore this possibility, we compared the patterns of gene expression in paired samples of tumor and normal lung tissue from 3 patients with a pathologic diagnosis of BAC. Characterization of the gene expression patterns of the paired tissue samples was performed by oligonucleotide microarray analysis of 12,000 known genes and expressed sequence tags (ESTs). We identified 12 genes that were up-regulated > or = 2-fold in all 3 tumors and 6 genes that were down-regulated in all 3 tumors to < or = 0.20 times the baseline. These findings suggest that large scale transcriptional profiling of BAC tumors may disclose a pattern of altered cellular expression in response to genetic changes, diseases, and environmental insult; such transcriptional profiling may aid in diagnosis and therapy.

Liposarcoma in the bone marrow: a terminal event.

We report a case of metastatic liposarcoma in the bone marrow with a rapidly fatal course. In view of the poor prognosis and paucity of clinical and imaging findings in patients with high-grade liposarcoma that is metastatic to bone marrow, we propose that bone marrow examination should be performed during the patient's initial evaluation as well as follow-up examinations.

Ultrastructure of the periductal area of comedo carcinoma in situ of the breast.

We have previously shown that the different biological natures of comedo ductal carcinoma in situ (DCIS) and non-comedo DCIS may, in part, be explained by the different expression patterns of tenascin, a large extracellular matrix protein, as observed by immunohistochemical studies. In the present study, we compared 8 cases of comedo DCIS with 5 cases of non-comedo DCIS by ultrastructural analysis, focusing on the myoepithelium, basal lamina, and tenascin-positive extracellular periductal stromal matrix. Our observations show that the comedo type DCIS frequently has an altered basal lamina, a looser and more disorganized collagenous matrix, and a general increase in stromal cellularity, including fibroblasts, lymphocytes, histiocytes and small blood vessels. In addition, in comedo DCIS, the lateral intercellular spaces between large myoepithelial cells that border the basal lamina are often expanded, compared to those of non-comedo DCIS. These results identify structural characteristics of comedo DCIS that may play a role in its greater preinvasive potential. They may also provide a structural basis for the different strategies that are needed for for clinical management of comedo DCIS, compared to non-comedo DCIS.

Adenocarcinoid tumor of the ovary diagnosed during pregnancy. A case report.

BACKGROUND: Adenocarcinoid tumors are uncommon neoplasms with dual morphology, showing components of a neuroendocrine tumor with carcinoid features and an adenocarcinomatous component composed of glands lined with mucin-containing cells, some of which are goblet type. CASE: A 36-year-old woman had a left adnexal mass found during the second week of pregnancy. Sonography showed it to be increasing in size and eventually to become associated with pelvic pain. During the 20th week of gestation, an exploratory laparotomy was performed, and the left ovary and fallopian tube were excised. A diagnosis of adenocarcinoma was rendered by intraoperative frozen section. A staging procedure was then performed that included removal of the contralateral adenexa, pelvic lymph node sampling, peritoneal biopsies and partial omentectomy. The vermiform appendix and gastrointestinal tract appeared unremarkable. The patient was discharged. Permanent sections of the left ovary revealed an adenocarcinoid tumor. CONCLUSION: While reports detail ovarian metastases of adenocarcinoid neoplasms from primary appendiceal and other gastrointestinal sites, this case, in the setting of a normal appendix and negative workup for an extraovarian origin, is the fourth documented one of a primary ovarian adenocarcinoid tumor and first diagnosed during pregnancy.

Tenascin patterns of expression in duct carcinoma in situ of the breast.

Immunohistochemical methods were used to study tenascin (TN) expression in duct carcinoma in situ (DCIS) of the breast of different histologic types. We evaluated 82 lumpectomy specimens of DCIS. There were 5 cases of comedo type, 19 cases of noncomedo type, and 58 cases of mixed comedo and noncomedo type. In 44 cases, the intraductal carcinomas were associated with infiltrating (invasive) duct carcinoma. TN expression was studied by immunohistochemical methods using monoclonal mouse anti-human tenascin (DAKO-TN2M636; 1:50 dilution). Positivity was recorded on a scale of 0 to 2+ for presence of TN staining around tumor ducts and thickness of TN-stained fibrous bands. TN showed positive correlation between thick bands around comedo DCIS and thin bands around noncomedo DCIS. The TN score had statistically significant positive association with high nuclear grade (p 0.004), periductal inflammatory infiltrate of DCIS (p 0.0006), associated extensive central necrosis of DCIS (p 0.0005), and comedo DCIS (p 0.0004). TN expression in the stroma was positively associated with tumor size (p 0.00002), extensive central necrosis (p 0.02), comedo DCIS (p 0.0005), and associated invasive carcinoma (p 0.006). The TN score did not correlate with duct size, multicentric carcinoma, or associated microcalcification. These results demonstrate the different biological nature of DCIS comedo type and suggest its preinvasive potential.

Biliary tract brush cytology.

OBJECTIVE: To prospectively review brush smears obtained during endoscopic retrograde cholangiopancreatography (ERCP) primarily from the biliary tree. STUDY DESIGN: A total of 175 specimens from 147 patients were included in the study. The smears, prepared directly from the endoscopic brush, were stained by the Papanicolaou technique and analyzed for standard cytologic features. RESULTS: The smears were categorized into benign/reactive, significant atypia and suspicious/positive. The consistent features seen in suspicious or positive smears were tightly cohesive, small, three-dimensional cell clusters that formed cell balls. The cells in the clusters displayed features of malignant cells. CONCLUSION: ERCP-guided brushing is a safe diagnostic procedure for the evaluation of biliary tree lesions. Small, three-dimensional epithelial clusters with marked atypia signify malignancy and warrant the diagnosis of a malignant neoplasm even when only one or two such clusters are seen in the smears. Single cells, cytoplasmic vacuoles and prominent nucleoli are not essential for a diagnosis of malignancy.

Pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation.

An unusual case is reported of pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation in a 34-year old male. According to our knowledge, such a neoplasm has never been reported in the literature.

Primary subcutaneous mucormycosis (zygomycosis): a case report.

A case of mycormycosis presenting primarily as a subcutaneous mass of the left leg in an immunocompetent individual is described. The mass that was diagnosed initially as a non-specific foreign body granulomatous process recurred a year later. Histopathological examination of the primary and recurrent lesions revealed partly degenerated hyphae associated with acute necrotizing and chronic granulomatous inflammation. Histomorphological features of primary subcutaneous mucormycosis without predisposing factors have not been previously reported.

Multifactorial analysis of long-term follow-up (more than 5 years) of primary extremity sarcoma.

BACKGROUND: The majority of survival studies in patients with extremity soft tissue sarcoma have focused on early recurrence and mortality. There are few data addressing long-term follow-up and survival. OBJECTIVE: To analyze survival and recurrence in patients with extremity soft tissue sarcoma who survive for more than 5 years. METHODS: Patients who underwent treatment for primary tumors (July 1982 to July 1994) and were followed up for more than 5 years were the subject of study. Disease-specific and disease-free survival were determined actuarially. Significance was evaluated using log-rank testing for univariate analysis and Cox model stepwise regression for multivariate analysis. RESULTS: A total of 495 patients with primary extremity tumors were treated before July 1989 and eligible for 5-year follow-up. Of these, 282 have been followed up for more than 5 years (median follow-up, 84.4 months). Actuarial disease-specific survival of patients who survive for longer than 5 years was 79%+/-7% (+/-SEM) at 10 years, and of those who were metastasis free at 5 years was 91%+/-4% at 10 years. On univariate analysis, post-5-year disease-specific survival was influenced by positive microscopic margin and initial tumor size of 5 cm or greater. On multivariate analysis, post-5-year disease-specific survival was influenced only by positive margins. CONCLUSIONS: Based on these analyses, 21% of patients with primary extremity sarcoma who survive for 5 years will die of disease within 5 years. Even of those who are metastasis free at 5 years, 9% will die of disease within 5 years. In contrast to early mortality, tumor grade has no influence on post-5-year prognosis. Patients with positive microscopic margins are at risk for post-5-year disease-specific mortality and therefore require long-term follow-up and consideration for investigational therapy.

Malignant hemangioendothelioma presenting as multifocal intraskeletal lesions during pregnancy. A case report.

BACKGROUND: Malignant hemangioendothelioma is a neoplasm of vascular origin characterized by irregular vascular channels lined with atypical endothelial cells. CASE: A gravida at 32 weeks' gestation presented with diffuse back and lower leg pain and was diagnosed with multifocal malignant hemangioendothelioma of bone. Computed tomography of the chest also demonstrated a small right atrial density. Three weeks later the patient became septic, and cesarean section was performed. After several cycles of chemotherapy, clinical improvement was noted. However, disease progression was noted thereafter, and the patient died one and a half years after the diagnosis. CONCLUSION: Our case was a primary multifocal malignant hemangioendothelioma of bone arising during pregnancy. Considering the absence of pulmonary involvement, it is unlikely that the skeletal lesions represented metastatic deposits from a cardiac primary. With such extensive skeletal disease, the right atrial density probably was a metastatic deposit.

Synchronous Paget's disease of the vulva and breast.

We report the first case of synchronous Paget's disease of the vulva and breast diagnosed within a period of 7 months. This is the fourth case of mammary Paget's disease associated with either preceding or following vulvar Paget's disease documented in the literature. With consideration of increasing age of the population, and the fact that vulvar Paget's disease affects elderly women and that breast cancer is the most common malignancy of women in the United States, one can anticipate the occurrence of these two conditions to be more prevalent in the future.

Lipoma of the mitral valve in a child.

Echocardiographic examination, performed in an asymptomatic 5 year old boy for a perimembranous ventricular septal defect (VSD), revealed a 6 x 6 mm tumor on the anterior leaflet of the mitral valve. Clinically, it was diagnosed as a benign myxoma. Histopathologic diagnosis of the excised mitral valve tumor was benign lipoma. The third case of mitral valve lipoma in the pediatric age group is reported along with a review of the literature. Echocardiogram remains the most sensitive diagnostic modality in the early detection of such neoplasms.

Cytodiagnosis of a primary effusion lymphoma. A case report.

BACKGROUND: Primary effusion lymphoma (PEL), commonly described in AIDS patients, is a unique subset of lymphoma in which the neoplastic lymphocytes proliferate exclusively in serous cavities. CASE: A 27-year-old male, HIV positive for five years and with multiple opportunistic infections in the past, was admitted for sudden-onset shortness of breath caused by a pleural effusion. Cytologic examination of the pleural fluid revealed medium to large atypical lymphocytes with a high mitosis rate, suspicious for lymphoma. Further diagnostic tests, such as immunophenotypic analysis and cytogenetic and molecular studies, confirmed the diagnosis of PEL. CONCLUSION: Cytopathologists and cytotechnologists should be aware of this new entity since additional studies are required for a definitive diagnosis.

Primary sarcomas of the mediastinum: results of therapy.

UNLABELLED: Primary sarcomas of the mediastinum are rare, and data concerning treatment and results of therapy are sparse. OBJECTIVE: To assess presentation, management, prognostic factors, and survival in mediastinal sarcomas. METHODS: We reviewed our experience with 47 patients with the diagnosis of primary sarcoma of the mediastinum. Data were collected from a computerized institutional database and medical records. Survival was analyzed by Kaplan-Meier method and comparisons of survival by log rank test. RESULTS: The median age of 47 patients with mediastinal sarcoma was 39 years (range 2.5 to 69 years), with a male/female ratio of 1.6. The most common complaints were chest/shoulder pain (38%) and dyspnea (23%). The most common tumor types were malignant peripheral nerve tumor (26%), spindle cell sarcoma (15%), leiomyosarcoma (9%), and liposarcoma (9%). Operation was the primary treatment modality in 72% of cases (n = 34); 22 sarcomas (47%) were completely resected. The overall 5-year survival was 32%. High-grade lesions had a significantly decreased survival (5-year survival = 27%) compared with low-grade tumors (5-year survival = 66%) (p = 0.05). The overwhelming factor determining survival was the ability to completely resect the tumors (5-year survival 49% for complete resection; 3-year survival 18% for incomplete or no resection) (p = 0.0016). Despite complete resection, local recurrence occurred in 64% of cases. CONCLUSION: Because the overall survival for patients with mediastinal sarcomas is 32% and the local recurrence is 64% for tumors completely resected, aggressive adjuvant therapy should continue to be systematically explored.

Intraoral tumor of Chievitz in a child.

Juxtaoral organs known as organs of Chievitz are intramuscular embryonic structures found close to the angle of the mandible near the insertion of the pterygomandibular raphae. They are considered of neuroepithelial origin with no known function. We describe the first tumor of the organ of Chievitz which presented intraorally in a child. Immunohistochemically, the Chievitz nests showed positive reaction for vimentin, cytokeratins, and epithelial membrane antigen and ultrastructurally demonstrated cytoplasmic processes and intermediate filament bundles. These observations, together with light microscopic features, suggest that the epithelial nests of the organ of Chievitz are meningothelial rather than neuroepithelial.