lewis-x-antigen and Anus-Neoplasms

Extramammary Paget's disease most commonly occurs on the female external genitalia and rarely occurs in the perianal region and male external genitalia. We present the clinical and pathologic features of 5 cases of perianal Paget's disease and review the literature.. Clinical and pathologic data were recorded for 5 cases of perianal Paget's disease. Cases were studied retrospectively with special stains, including periodic acid-Schiff, mucicarmine, Alcian blue, carcinoembryonic antigen, S100 protein, pan-keratin, gross cystic disease fluid protein-15 (GCDFP-15), lysozyme, CD15 (Leu-M1), cytokeratin 7 (CK7), and cytokeratin 20 (CK20).. Three (60%) of 5 patients had concurrent rectal adenocarcinomas. All cases reacted positively for pankeratin, although the intensity and distribution of staining varied. Both cases not associated with an underlying carcinoma showed strong GCDFP-15 and CK7 expression and an absence of CK20 expression. The 3 cases associated with an underlying malignancy demonstrated CK7 and CK20 expression and an absence of GCDFP-15 expression. All cases were negative for lysozyme and CD15 (Leu-M1).. The 5 cases reported herein demonstrate that perianal Paget's disease is a heterogeneous entity. The high frequency of associated underlying malignancies and resultant poor clinical outcomes highlight the importance of an aggressive search for a second malignancy. In some cases, perianal Paget's disease merely represents a cutaneous manifestation of an underlying rectal adenocarcinoma and demonstrates a CK7+/CK20+/GCDFP-15-/lysozyme-/Leu-M1- immunophenotype and signet ring Paget's cells. Other cases represent primary adenocarcinomas of the skin, which are associated with a CK7+/CK20-/GCDFP-15+/lysozyme /Leu-M1- immunophenotype and an excellent prognosis if adequately resected. Immunohistochemical studies, particularly CK20 and GCDFP-15, are useful adjuncts in distinguishing primary and secondary perianal Paget's disease.

Topics: Aged; Anus Neoplasms; Apolipoproteins; Apolipoproteins D; Biomarkers, Tumor; Carcinoembryonic Antigen; Carrier Proteins; Fatal Outcome; Female; Glycoproteins; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Lewis X Antigen; Male; Membrane Transport Proteins; Middle Aged; Muramidase; Paget Disease, Extramammary; Retrospective Studies

The perianal region is a very rare location for Hodgkin's lymphoma, and clinicians may often neglect the diagnosis in patients with inflammatory bowel disease.. We present a case of perianal Hodgkin's lymphoma in patient with Crohn's disease who was on long-term immunosuppression and whose symptoms would normally be attributed to Crohn's disease. Diagnosis was based on the morphological appearance of atypical cells in the lamina propria and the immunohistochemical profile of Reed Sternberg and Hodgkin's cells, showing co-expression of CD15 and CD30.. Perianal complaints in patients with inflammatory bowel disease may be a manifestation of other pathology. Hodgkin's lymphoma could be a progression in the chronically inflamed tissue in this unusual location.

Topics: Aged; Anus Neoplasms; Crohn Disease; Female; Hodgkin Disease; Humans; Ki-1 Antigen; Lewis X Antigen