Low-grade cribriform cystadenocarcinoma of salivary glands is a recently described rare tumor with favorable prognosis. This study reports the case of 50-year-old woman with swelling lasting for 9 months in the right parotideomasseteric area. Grossly, the tumor was well circumscribed and dominated by cystic space. Microscopically, the neoplasm consisted of well-demarcated islets, some of them cystically dilated. The architecture of islets varied from solid to cribriform and micropapillary without comedo-type necroses. The tumor cells featured no significant cytologic atypia. Immunohistochemically, luminal cells showed expression of cytokeratins (CK), CK7, CK18, and S100 protein. In addition, immunostains for CK5/6, CK14, p63 protein, and smooth muscle actin displayed a continuous rim of myoepithelial cells around all tumor nests. In contrast, detection of CK20, hormonal receptors (androgen, estrogen, and progesterone), epidermal growth factor receptor and Her-2/neu oncoprotein was negative. The patient is free of disease for 2 years. The relationship between low-grade cribriform cystadenocarcinoma and salivary duct carcinoma is discussed.
Biphasic squamoid alveolar renal cell carcinoma (BSARCC) has been recently described as a distinct neoplasm. Twenty-one cases from 12 institutions were analyzed using routine histology, immunohistochemistry, array comparative genomic hybridization (aCGH) and fluorescence in situ hybridization. Tumors were removed from 11 male and 10 female patients, whose age ranged from 53 to 79 years. The size of tumors ranged from 1.5 to 16 cm. Follow-up information was available for 14 patients (range, 1 to 96 mo), and metastatic spread was found in 5 cases. All tumors comprised 2 cell populations arranged in organoid structures: small, low-grade neoplastic cells with scant cytoplasm usually lining the inside of alveolar structures, and larger squamoid cells with more prominent cytoplasm and larger vesicular nuclei arranged in compact nests. In 9/21 tumors there was a visible transition from such solid and alveolar areas into papillary components. Areas composed of large squamoid cells comprised 10% to 80% of total tumor volume. Emperipolesis was present in all (21/21) tumors. Immunohistochemically, all cases were positive for cytokeratin 7, EMA, vimentin, and cyclin D1. aCGH (confirmed by fluorescence in situ hybridization) in 5 analyzable cases revealed multiple numerical chromosomal changes including gains of chromosomes 7 and 17 in all cases. These changes were further disclosed in 6 additional cases, which were unsuitable for aCGH. We conclude that tumors show a morphologic spectrum ranging from RCC with papillary architecture and large squamoid cells to fully developed BSARCC. Emperipolesis in squamoid cells was a constant finding. All BSARCCs expressed CK7, EMA, vimentin, and cyclin D1. Antibody to cyclin D1 showed a unique and previously not recognized pattern of immunohistochemical staining. Multiple chromosomal aberrations were identified in all analyzable cases including gains of chromosomes 7 and 17, indicating that they are akin to papillary RCC. Some BSARCCs were clinically aggressive, but their prognosis could not be predicted from currently available data. Present microscopic, immunohistochemical, and molecular genetic data strongly support the view that BSARCC is a distinctive and peculiar morphologic variant of papillary RCC.
Some renal epithelial neoplasms, such as renal angiomyoadenomatous tumor, clear cell papillary renal cell carcinoma and renal cell carcinoma with smooth muscle stroma, contain a variably prominent smooth muscle stromal component. Whether or not this leiomyomatous stroma is part of the neoplastic proliferation has not been firmly established. We studied the clonality status of 14 renal cell carcinomas with a prominent smooth muscle stromal component (four renal angiomyoadenomatous tumors/clear cell papillary carcinomas, five clear cell carcinomas, two papillary carcinomas, and three renal cell carcinomas with smooth muscle rich stroma) using the human androgen receptor assay (HUMARA). We found the leiomyomatous stromal component in all analyzable (8/14) cases to be polyclonal and therefore reactive rather than neoplastic. Based on morphological observations, we propose that the non-neoplastic leiomyomatous stromal component is likely derived from smooth muscle cells of large caliber veins located at the peripheral capsular region or within the collagenous septae of the tumors.
E 5 9What ' s known on the subject? and What does the study add? Surgical treatment of renal cell carcinoma (RCC) with tumour thrombus extending into the right atrium remains, despite its complexity and specifi c technical aspects, the only radical therapeutic option.This single-centre study, unique in size for this rare condition, reports early and late results over a period of 18 years. All patients were operated on using a standardised protocol with use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Overall and cancer-specifi c cumulative survival was better than in other reports.
OBJECTIVE• To evaluate the long-term results of radical surgical management of renal cell carcinoma (RCC) with tumour thrombus extension (TTE) level IV into the right atrium (RCC/TTE IV) in a large singleinstitution series.
PATIENTS AND METHODS• Radical complex urological and cardio-surgical procedure was performed over a period of 18 years (1993 -2010) on 21 patients with RCC/TTE IV. A radical nephrectomy was performed followed by sternotomy, institution of cardiopulmonary bypass and extraction of the intracardiac tumour thrombus under direct visual control during deep hypothermic circulatory arrest (DHCA).• Perioperative and postoperative variables, and long-term overall and cancer-specifi c survival using the Kaplan -Meier method were analysed.
RESULTS• In all patients, precise removal of tumour thrombus was accomplished in a bloodless fi eld during DHCA.• The mean ( SD ) duration of circulatory arrest was 16 (6) min at a mean hypothermia of 20 (3) ° C. In-hospital mortality was 9.5% (two patients).• The median survival (including in-hospital mortality) was 25 months.• In Kaplan -Meier analysis, 2-and 5-year overall cumulative survival rate was 57 (95% confi dence interval, CI 36 -78)% and 37 (95% CI 15 -58)%, respectively.• Cancer-specifi c cumulative survival was 68 (95% CI 49 -89)% at 2 years and 51 (95% CI 28 -74)% at 5 years.
CONCLUSIONS• Late outcome after radical surgical treatment in patients with RCC and TTE reaching up to the right atrium justifi es this extensive procedure.• Cardiopulmonary bypass with DHCA allows safe and precise extirpation of all intracaval and intracardiac tumour mass.
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