[Clinicopathologic analysis of Langerhans cell histiocytosis in adults].

OBJECTIVE: To explore the clinical features, pathomorphology, immunohistochemical characteristics and prognosis of Langerhans cell histiocytosis (LCH) in adults. METHODS: Twenty-three cases of adult LCH were retrieved from Ningbo Diagnostic Pathology Center during the period from January 2005 to December 2011. And their clinical presentation, pathomorphology and immunohistochemical characteristics were analyzed. RESULTS: The mean age of patients was 37.2 years (range: 20 - 58). The male-to-female ratio was 1.6:1. Of 23 LCH patients, 26 lesions were found including 14 bone tissue lesions (53.8%), followed by 4 lymph node lesions and 4 skin lesions (both 15.4%), as well as 1 soft tissue, liver, parotid and buccal lesion respectively (all 3.8%). Clinically, uni-system and unifocal disease was predominant (19 cases, 82.6%), followed by uni-system and multifocal disease (1 case, 4.3%), multi-system disease (3 cases, 13.0%). Histologically, all cases of LCH revealed diffused distribution of Langerhans cells, accompanied by a variable number of eosinophils, lymphocytes, neutrophils and multinucleated giant cells. Immunohistochemically, the expression of CD1a, Langerin, S-100 protein and CD68 was 100% (23/23), 100% (20/20), 95.6% (22/23) and 90.5% (19/21) respectively. All lesions were treated by surgical therapy. Sixteen patients were available for follow-up examination and 14 patients survived. The 3 and 5-year cumulative survival rates were 92.9% and 79.6% respectively. CONCLUSIONS: LCH of adults occurs predominantly in bone and presents mainly as uni-system or unifocal defects. Surgical excision is generally effective and the prognosis is fair.

[A clinicopathological study of perianal Paget's disease associated with internal rectal adenocarcinoma].

OBJECTIVE: To investigate the clinicopathological features and the immunohistochemical phenotype of perianal Paget's disease (PPD) associated with internal anorectal adenocarcinoma, with emphasis on the histogenesis of Paget's cells. METHODS: The clinical and pathologic features of three cases of PPD with rectal adenocarcinoma were investigated. Periodic-acid-Schiff (PAS), alcian-blue and mucicarmine staining with and without diastase digestion were performed. The immunohistochemical study was performed on selected sections by a panel of antibodies including carcinoembryonic antigen (CEA), CK7, CK8, CK10/13, CK20 and gross cystic disease fluid protein 15 (GCDFP15). RESULTS: All three cases occurred in middle to old age male patients complaining of anal bleeding. Digital physical examination revealed ulcerated or cauliflower-like masses in the anus just distal to the dentate line. Perianal skin erythematous patches were found in two cases, and small discrete granules in one case. Histologically, the anorectal neoplasm was either a moderately or poorly differentiated adenocarcinoma. Two types of Paget's cells were noted, namely the classical type characterized by a polygonal shape with vesicular nuclei and abundant pale cytoplasm, and the signet ring type characterized by eccentrically displaced nucleus. Both the rectal adenocarcinoma cells and Paget's cells showed strong positivity for PAS, AB and mucicarmine, which were resistant to the diastase digestion. Immunohistochemically, they were both positive for CEA, CK7, CK8 and CK20, but negative for CK10/13 and GCDFP15. CONCLUSIONS: The CK20(+)-GCDFP15(-) type Paget's cells in PPD were derived from the direct intraepithelial Pagetoid spread of anorectal adenocarcinomas. PPD was more frequently associated with internal carcinomas than any other type of extramammary Paget's disease. It is recommended that clinicians should carefully examine the anus or rectum in the presence of PPD to ascertain if it is associated with an internal carcinoma.