Tracheal extramedullary plasmacytoma: a rare cause of chronic cough.

Operative Rigid Bronchoscopy treatment.

Primary Effusion Lymphoma: Cytological Diagnosis of a Rare Entity--Report of Two Cases in HIV-Uninfected Patients from a Single Institution.

BACKGROUND: Primary effusion lymphoma (PEL) is a Kaposi's sarcoma-associated herpesvirus (KSHV)-related large B-cell lymphoma often affecting immunocompromised adults. However, several cases in nonimmunocompromised elderly patients have been reported. It usually occurs as a massive, serosal effusion without tumoral masses and lymphadenopathies. The most frequent diagnostic materials are cytological smears, displaying large lymphocytes with variable morphological aspects but with a specific 'null-cell' phenotype associated with the expression of KSHV-related latency-associated nuclear antigen. CASES: We report 2 cases of PEL that occurred in elderly, HIV-uninfected patients. In the first case, an 83-year-old man presented with severe dyspnea of 1 week duration. Radiological images documented pericardial effusion causing cardiac tamponade, without lymphadenopathies and solid masses. In the second case, an HIV-uninfected 94-year-old man was affected by anorexia, fatigue, shortness of breath and nonproductive cough. Radiological studies revealed bilateral pleural effusion, without evidence of abnormal lymphadenopathies and lung and/or pleural masses. Thoracentesis was immediately performed. Cytological evaluations of pericardial (first case) and pleural (second case) fluid effusion showed anaplastic and immunoblastic lymphocytes, respectively, expressing LCA/CD45, CD30 and KSHV. CONCLUSION: Albeit rare, PEL should be kept in mind in the diagnostic algorithm of serosal cytological evaluation in elderly HIV-uninfected patients.

The morphological and immunohistochemical spectrum of gastric biopsies of patients with absorptive hypercalciuria.

OBJECTIVE: Idiopathic hypercalciuria is characterised by renal stone formation and vertebral osteoporosis. The syndrome displays high clinical variability with patients almost equally distributed between fasting or renal type (prevalent calcium loss) and absorptive type (prevalent increase of intestinal absorption). Absorptive hypercalciuria (AH) is characterised by hypersensitivity of calcium-sensing receptors of antral G cells with normal fasting gastrinaemia and meal hypergastrinaemia. To the best of our knowledge, no study has been published about the morphological aspects of gastric biopsies of patients with AH and the immunohistochemical findings of gastrin-producing G cells. So we studied morphologically and immunohistochemically a group of 38 patients with AH, describing their gastric findings and associated lesion. DESIGN: All 38 patients had a clinical-laboratory diagnosis of AH with normal fasting hypergastrinaemia and an abnormal rise of gastrinaemia after a standardised meal test. Their 38 antral and 27 body-fundus biopsies, and 5 normal antral and body controls, were stained with H&E, Giemsa stain, polyclonal antiserum anti-Gastrin and a monoclonal antibody anti-Chromogranin A. RESULTS: Antral biopsies of all 38 patients showed a simple (15) or linear (23) hyperplasia of G cells, whereas only 2 of 27 body biopsies showed a nodular hyperplasia of endocrine cells. In five patients with AH, we found an association with fundic gland polyps (FGPs). CONCLUSIONS: We found in all of the patients with AH a correlation between meal hypergastrinaemia and morphological antral G-cell hyperplasia. Moreover, in five patients, AH was associated with FGPs. We know from literature data that FGPs' development in Zollinger-Ellison syndrome is statistically associated with hypergastrinaemia. From our present data, we suggest that even in AH the stimulated hypergastrinaemia may have a role in polyp development.

Clear cell hidradenocarcinoma of the breast: a very rare breast skin tumor.

Hidradenocarcinoma is an uncommon malignant intradermal tumor of sweat gland origin with a predilection for the face and extremities. It is encountered equally in males and females, usually in the second half of life. These tumors tend to be locally aggressive. In our case, the tumor was located relatively superficially but without any apparent connection to the overlying skin. The typical disease course includes local and sometimes multiple recurrences, and some patients develop regional lymph node and distant metastases. These type of tumors in the parenchyma of the breast are extremely rare. We report a case of hidradenocarcinoma in a 77-year-old woman who presented with a palpable inflammatory nodule in the right breast.

Immunophenotype of sporadic and familial adenomatous polyposis associated fundic gland polyps: a mucin and MIB1 study.

BACKGROUND: Fundic Gland Polyps (FGPs) are small sessile (2-5 mm) usually multiple polyps arising in the gastric, acid-secreting mucosa, described both in a sporadic form, prevalently in middle aged females, and associated with familial adenomatosis coli (FAP)-Gardner's syndrome and their attenuated variants (syndromic form). AIMS: We performed an immunohistochemical study on 5 syndromic (4 cases without and 1 case with dysplasia) and 28 sporadic FGPs, using monoclonal antibodies (MoAbs) against normal epitopes of fundic mucosa (Ck20, the surface gastric mucin M1, EMA, ChA), H. pylori and HLA-DR(Ia) antigens, CEA and mucin epitopes, and the Ki67 (MIB1) proliferation antigen, in order to establish the immunophenotype of FGPs; find any possible differences between sporadic and syndromic polyps. RESULTS: Ck20 and M1 were positive on surface and foveolar epithelium of controls, whereas sporadic and syndromic FGPs showed an enhanced deep positivity below foveolar necks ("foveolar metaplasia"); EMA was strongly positive on parietal cells, highlighting intracytoplasmic canaliculi. Chromogranin-positive cells in FGPs were alike controls, except for a sporadic case with micronodular hyperplasia. Ck7, as expected, was negative in controls, whereas the 5 syndromic FGPs and 25 of 28 sporadic FGPs showed a diffuse superficial and deep expression. H. pylori anti-serum gave negative results on all cases, and only 3 sporadic FGPs showed epithelial expression of HLA-DR(Ia). Syndromic FGPs were CEA negative, whereas 32% of sporadic FGPs expressed it. FGPs showed a neoexpression of the mucin oncofetal epitopes syalil-Tn (3/5 syndromic, 82% sporadic) CA19.9 and CA50 (4/5 syndromic, 14% sporadic). MIB1-labelling index of surface (30.5%) and deep (37.1%) compartments of the 4 syndromic FGPs without dysplasia was enhanced, with high statistical significance (p < 0.0001) both in comparison to controls (16.9% superficial stain only) and sporadic FGPs (15.8% surface, 19.5% deep labeling indexes). Moreover, the MIB1 labeling-index of the syndromic case with dysplasia (60.8% surface, 56.6% deep labeling indexes) was further enhanced in comparison with the other 2 syndromic cases. CONCLUSIONS: Sporadic and syndromic FGPs showed a neoexpression of Ck7, CEA, and mucin epitopes. As these markers are normal antigens of fetal stomach, FGPs showed a fetal, "immature" immunophenotype. The only difference we found between syndromic and sporadic polyps was a statistically significant enhanced MIB1-labelling index expression by syndromic FGPs, further enhanced in the syndromic FGP with dysplasia.

Sporadic fundic gland polyps: clinico-pathologic features and associated diseases.

BACKGROUND: Fundic gland polyps have been described either in association with genetic polyposis syndromes of the colon, or in a sporadic form. In the first case they are diagnosed during family screening in asymptomatic subjects, while sporadic FGP patients often complain of upper gastrointestinal symptoms. So far, no great attention has been paid to the clinical presentation of these patients, so we undertook a clinico-pathologic study to further delineate: the clinical presentation at 1st examination; the associated gastrointestinal conditions; a possible role of omeprazole; Helicobacter pylori (H. pylori) colonization, the presence of intestinal metaplasia and dysplasia. METHODS: We followed-up for a 9-year period with endoscopies a case series of 70 patients with sporadic FGPs, recording endoscopic data, symptoms, associated gastrointestinal conditions, previous therapies, histopathological findings. RESULTS: The prevalence of the present series was 0.36%. The patient prevalence and number of polyps by age classes rose in women (maximum value in perimenopausal age), while was constant in males. We observed a frequent association between FGPs and esophageal conditions (34%), namely hiatus hernia-reflux esophagitis, significantly higher than in our endoscopic population (15%). Five patients had an isolated colonic adenoma. Only one patient had received long term omeprazole therapy. H. pylori was negative in all 70 FGPs, and in 15 samples of antral mucosa. No metaplastic or dysplastic lesions were seen. CONCLUSION: Sporadic FGP patients frequently complain of epigastric pain, burning, dyspepsia, probably related to the frequently associated esophageal pathology, namely reflux esophagitis-hiatus hernia (34%). Prevalence of FGPs and polyps number are linked to female sex (maximum rise for both values in perimenopausal age). No link with omeprazole therapy was seen. FGPs patients appear to be protected from H. pylori colonization and ultimately from the development of intestinal metaplasia-dysplasia-gastric cancer. Nonetheless, they are apparently more prone to colonic adenomas. So, every sporadic FGP patient should undergo colonic surveillance.