Composite intestinal-type adenocarcinoma and small cell carcinoma of sinonasal tract.

BACKGROUND AND AIMS: Sinonasal intestinal-type adenocarcinomas (ITACs) are rare neoplasms resembling intestinal adenocarcinomas. Although several studies have documented neuroendocrine differentiation in ITACs, the combination of ITAC and small cell carcinoma has not been previously described in detail. The aim of this report is to detail the histopathological and immunohistochemical characteristics of two cases of composite ITAC with small cell carcinoma. METHODS: Two cases of composite ITAC with small cell carcinoma were routinely processed, and representative sections were stained with CAM5.2, AE1:AE3, keratin 7, keratin 20, keratin 19, CDX-2, p63, villin, chromogranin, synaptophysin and CD56. RESULTS: One tumour consisted of a mixed-type ITAC showing colonic-type and poorly differentiated adenocarcinoma with foci of "signet-ring" cells combined with small cell carcinoma. Both components stained positively with CAM5.2, AE1:AE3, CK7, CK20 and CK19, whereas only the small cell carcinoma expressed synaptophysin and CD56. Both components stained negatively with CDX-2, villin, CD99 and p63. The "signet-ring" cells stained positively with chromogranin and synaptophysin. The second tumour showed a papillary-type ITAC combined with a small cell carcinoma. The adenocarcinoma and small cell carcinoma stained positively with CAM5.2, AE1:AE3, CK7, CK19 and CK20. Only the adenocarcinoma was CDX-2 positive, whereas the small cell carcinoma expressed CD56 and synaptophysin. CONCLUSIONS: The two components of the combined ITACs and neuroendocrine small cell carcinoma show significant immunohistochemical overlap, supporting a common origin. The occurrence of a distinct neuroendocrine carcinoma combined with ITACs expands the histopathological spectrum of these tumours.

Fine-needle aspiration of a follicular dendritic-cell tumor: report of a case and review of the literature.

Follicular dendritic-cell tumors (FDCT) are rare neoplasms, well-characterized in surgical pathology material. There are, however, few cytopathology reports. We describe the fine-needle aspiration (FNA) findings of a histologically confirmed FDCT. Conventional smears and a cell block showed large spindle to oval neoplastic cells admixed with small mature lymphocytes. The neoplastic cells were present mainly in small syncytial clusters. Immunostains for CD21 and CD35, performed on the cell block, were positive in the neoplastic cells. The diagnosis was fully confirmed by the presence of typical immunohistochemical and ultrastructural features on the surgically removed tumor. The differential diagnosis of FDCT is broad and includes other tumors characterized by an admixture of large neoplastic cells and small mature lymphocytes, such as thymomas, lymphoepithelioma-like carcinomas, and interdigitating dendritic-cell tumors. It may not be possible to diagnose FDCT based on FNA material without the use of immunocytochemical and electron microscopic studies. Certain cytomorphological characteristics, however, might suggest its diagnosis and allow the practicing cytopathologist to perform confirmatory studies.

Functional adrenal cortical tumors in childhood: a study of ploidy, p53-protein and nucleolar organizer regions (AgNORs) as prognostic markers.

Prognostic markers in pediatric adrenal cortical tumors are difficult to define. We determined the ploidy, immunostaining of p53-protein and number of nucleolar organizer regions (AgNORs) in 16 such tumors and related them to clinical outcome, tumor weight (TW) and histologic Weiss' criteria. Eleven females and 5 males aged 0.4 to 15.6 years were followed for 8.7 years; 10 presented Cushing's and 6 virilization syndrome. Diploid (n = 4, x TW = 269 g, range: 17-800 g) and near-diploid tumors (n = 3, x TW = 55 g, range: 20-85 g) had good outcome, Weiss' criteria were 0-7, and p53 reactivity was negative in all. Among the aneuploid tumors (n = 9, x TW = 298 g, range: 7-1000 g), 6 had good outcome, 2 presented metastasis and 1 was lost to follow-up; Weiss' criteria were 2-8 and p53 reactivity was positive in 3 tumors (2 of them of malignant evolution). AgNORs number was not different in cases of good or poor outcome (3.65 +/- 1.9 vs 2.83 +/- 1.1). Our findings indicate that diploid and near-diploid cases had always a good outcome regardless of tumor weight. In aneuploid cases, tumor weights < 100 g had good outcome, while those > 750 g had poor prognosis. Malignant tumors were aneuploid and had reactivity to p53-protein. Good outcome in aneuploid tumors < 100 g is probably due to early treatment. The expression of p53-protein appears as a promising marker of poor prognosis. Weiss' criteria and AgNORs were not useful in the present series.

Aneuploid nucleomegaly of bronchial cells in ataxia-telangiectasia: cytologic recognition in bronchial brushings.

Ataxia-telangiectasia (AT) is an autosomal recessive disorder of childhood onset characterized by cerebellar ataxia and cutaneous and conjunctival telangiectasias, which affects many systems and organs. One histologic feature of AT is the presence of enlarged dystrophic nuclei, predominantly in satellite cells of sympathetic ganglia and dorsal roots. This paper describes the recognition of nucleomegaly of respiratory cells in bronchial brushings of a 9-year-old patient with AT. The enlarged nuclei displayed smooth nuclear contour, coarse and clumped chromatin granules, and one or two conspicuous nucleoli. The average size was 0.1015 mm in the AT case and 0.0573 mm in control cells. Ploidy analysis demonstrated an aneuploid population of cells with a DNA index of 1.31 and a S-G2M fase of 4.48% in the AT, while the control nuclei showed normal diploid values. To our knowledge, this is the first report of a description of aneuploid nucleomegaly of bronchial cells detected in bronchial smears from a patient with AT. Given that malignant transformations are usually preceded by ploidy alterations, it seems likely that the presence of an aneuploid cell population probably correlates with the increased cancer risk observed in AT patients. Cytopathologists must bear in mind these morphologic features of aneuploid nucleomegaly exhibited by certain cell populations when examining a smear from AT patients. Moreover, this finding may even represent a clue for diagnosis of AT in cases in which the disease has gone unrecognized.