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Background: Although colon carcinomas consisting predominantly of neuroendocrine cells carry a worse prognosis than "routine" colon adenocarcinomas, the clinical significance of scattered neoplastic neuroendocrine cells within a typical colon adenocarcinoma remains controversial. The aim of this study was to document the frequency and clinical significance of neuroendocrine cell expression within a stage-specific group of typical adenocarcinomas of the colon., Methods: Forty-eight patients with resected stage III adenocarcinomas of the colon were selected from our institutional tumor registry. The pathologic specimens from these patients were reviewed and underwent immunohistochemical staining for chromogranin, a sensitive and specific marker of neuroendocrine differentiation. Long-term (> or = 5 years) clinical outcome was compared with the presence of neuroendocrine cell expression., Results: Twenty tumors (41.7%) stained positively for chromogranin. Twenty-two patients (45.8%) had long-term cancer-free survival, although chromogranin positivity did not correlate with this survival., Conclusion: The frequency of scattered neuroendocrine cells within colonic adenocarcinomas is high. This finding does not, however, carry the same adverse prognostic implications for cancer survival as does the presence of true neuroendocrine carcinoma of the colon.

Purpose: To determine the computed tomographic (CT), magnetic resonance (MR) imaging, and angiographic findings of papillary endolymphatic sac tumors., Materials and Methods: Clinical and imaging studies in 20 patients (aged 17-65 years) with histopathologically proved papillary endolymphatic sac tumors were retrospectively reviewed. Patients underwent CT (n = 18), MR imaging (n = 15), or angiography (n = 12). CT scans were evaluated for bone erosion and calcification; MR images, for signal intensity, enhancement patterns, and flow voids; and angiograms, for tumoral blood supply., Results: All tumors were destructive and contained calcifications centered in the retrolabyrinthine region at CT. The MR imaging appearance varied with lesion size; 12 of 15 tumors showed increased signal intensity at T1-weighted imaging. The high-signal-intensity area was circumferential in lesions 3 cm or smaller and was scattered throughout the lesion in advanced tumors. Only tumors larger than 2 cm had flow voids. The blood supply arose predominantly from the external carotid artery. Large tumors had additional supply from the internal carotid and posterior circulation., Conclusion: Papillary endolymphatic sac tumors are destructive, hypervascular lesions that arise from the temporal bone retrolabyrinthine region. Increased signal intensity at unenhanced T1-weighted MR imaging is common and may help distinguish these lesions from more common, aggressive temporal bone tumors.

Ber-EP4 is a recently characterized monoclonal antibody directed against a cell surface glycoprotein that is putatively present on human epithelial cells but lacking on the mesothelium. To investigate the diagnostic efficacy of Ber-EP4 in distinguishing adenocarcinoma from mesothelioma, we studied formalin-fixed, paraffin-embedded sections from well-documented cases of adenocarcinoma (120 cases), adenomatoid tumor (nine cases), and malignant mesothelioma (49 cases). Of the 120 adenocarcinomas, 103 (86%) showed membranous Ber-EP4 positivity, with diffuse reactivity noted in 82 cases and focal staining in 21 cases. Reactivity with Ber-EP4 was also observed in two of nine adenomatoid tumors (22%) and 10 of 49 mesotheliomas (20%). Staining in the mesotheliomas was restricted to epithelioid areas and generally focal. In one mesothelioma, however, Ber-EP4 stained the majority of neoplastic cells. In contrast to previous reports, we conclude that positivity with Ber-EP4 does not exclude the diagnosis of mesothelioma. Nonetheless, most Ber-EP4-positive mesotheliomas exhibit only focal positivity, as opposed to the extensive staining commonly observed in adenocarcinomas. Ber-EP4 has diagnostic utility in the discrimination of mesothelioma from adenocarcinoma, but it is best utilized in an antibody panel that includes other markers of carcinomatous differentiation.

We studied eight clear cell tumors of the lung (CCTL) to better define their clinical, immunohistochemical, and ultrastructural features, and to clarify their distinction from other neoplasms, particularly metastatic renal cell carcinoma. Patients ranged in age from 31 to 67 years (mean, 51 years). Seven patients had clinically benign, asymptomatic lesions measuring less than 2 cm in diameter that were devoid of necrosis. The eighth patient had a symptomatic, partially necrotic CCTL 4.5 cm in diameter that metastasized to the liver and peritoneum; the patient died of tumor 17 years after diagnosis. Ultrastructural study of seven CCTL showed interdigitating cell processes (all cases), primitive cell junctions (five of seven cases), intracytoplasmic glycogen (all cases), and rare dense core granules (two of seven cases). Immunohistochemically, paraffin-embedded sections from all eight CCTL were negative for cytokeratin (CK), epithelial membrane antigen (EMA), chromogranin, and vimentin. Focal staining was seen for S-100 protein (three of eight cases), neuron-specific enolase (three cases), synaptophysin (one case), and Leu 7 (one case). Although these findings suggest that at least some CCTL exhibit neuroendocrine differentiation, the tumor's histogenesis remains uncertain. Of more practical importance, the combined absence of CK, EMA, and vimentin in formalin-fixed, paraffin-embedded CCTL virtually precludes confusion with renal cell carcinoma. Although traditionally considered benign, CCTL larger than 2 cm that are symptomatic, and focally necrotic should be regarded as potentially malignant neoplasms.

A 29-year-old woman had a middle-ear mass that resulted in hearing loss and seventh-nerve palsy. The tumor invaded the petrous bone, the mastoid, at least one semicircular canal, and then extended into the posterior fossa. Microscopically, it consisted of complex, interdigitating papillae lined by uniform, cuboidal to low columnar cells. The cells resembled the epithelium of the normal middle ear and middle-ear adenoma, but the papillary architecture distinguished this neoplasm from the latter, nonpapillary tumors. Papillary middle-ear tumors are locally invasive. We propose the term "aggressive papillary middle-ear tumor" (APMET). Nine other examples of APMET have been reported under various diagnostic terms. All have been locally destructive with frequent intracranial invasion. Although none of the tumors has metastasized, one patient died of uncontrolled local disease. For this reason, APMET must be distinguished from nonpapillary middle-ear tumors.