Collagenous sprue: don´t forget connective tissue in chronic diarrhea evaluation.

Collagenous sprue is a rare disease of the small bowel characterized by mucosal atrophy and excessive subepithelial collagen deposition. The etiology remains unclear and the diagnosis is based upon patient´s clinical picture and anatomopathological findings. Clinically, collagenous sprue is characterized by persistent diarrhoea, severe malabsorption, multiple nutrient deficiencies and progressive weight loss. Differential diagnosis includes celiac disease, which is mandatory to rule out because of their frequent association. Glutenfree diet is the first therapeutic step, but it usually is not effective. However, recent studies show high success rates with immunomodulators, mainly corticosteroids. We report the case of a patient presenting with chronic diarrhea and severe malabsorption who was diagnosed with collagenous sprue, with no response to gluten free diet, but with excellent response to budesonida.

[Enteropathy-associated T-cell lymphoma: a diagnostic challenge]. / Linfoma T asociado a enteropatía: un reto diagnóstico.

Diagnosis of refractory celiac disease (CD) is based on exclusion of other disorders, persistence of malabsorptive symptoms and villous atrophy, despite a strict gluten-free diet for at least 6-12 months. Detection of alterations in the intraepithelial lymphocyte population is crucial for diagnosis. A subgroup of patients with refractory CD may develop severe complications such as enteropathy-associated T cell lymphoma (EATL). We present the case of a patient with longstanding silent CD who developed EALT, highlighting the challenge posed by the diagnosis and treatment of this entity.

CD10 expression in pure stromal and sex cord-stromal tumors of the ovary: an immunohistochemical analysis of 101 cases.

CD10 has been recently advocated as a good immunohistochemical marker for endometrial stromal tumors. Metastatic endometrial stromal tumors to the ovary and primary endometrioid stromal sarcomas may show overlapping histological features with pure stromal and sex cord-stromal tumors (SCSTs). We investigated CD10 expression in a large series of pure stromal and SCSTs of the ovary to ascertain whether CD10 may aid in this differential diagnosis. Archival material from 11 fibromas, 10 thecomas, 10 sclerosing stromal tumors (SSTs), 10 adult granulosa cell tumors (AGCTs), 4 luteinized AGCTs, 9 juvenile granulosa cell tumors (JGCTs), 9 Sertoli cell tumors, 9 Sertoli-Leydig cell tumors, 11 sex cord tumors with annular tubules, 10 steroid cell tumors (StCTs), and 8 fibrosarcomas of the ovary were immunostained for CD10. The percentage of cells stained (<5%, 5%-39%, 40%-75%, and >75%) and intensity of staining (1+, 2+, 3+) were evaluated. CD10 was expressed in 7 of 10 thecomas (4 with 5%-75% and mostly 1+), 9 of 10 SSTs (7 with 5%-39% + cells, mostly 1+), 9 of 10 AGCTs (<5%-39%, four 1+, five 2+), 1 of 4 luteinized AGCTs (<5% and 1+), 8 of 9 JGCTs (mostly <5% to 39% and +1), 4 of 9 Sertoli cell tumors (either focal or >75% with variable intensity), 4 of 9 Sertoli-Leydig cell tumors (mostly <10% with variable staining), with the Leydig cells being positive in only 1 tumor (1+ and <5%), and 7 of 10 StCTs (4 tumors with more than 75% + cells, from 1+ to 3+). All fibromas, all but 1 fibrosarcoma (<5% and 1+), and all sex cord tumors with annular tubules were CD10 negative. CD10 expression was frequently seen in StCTs, SSTs, and thecomas of the ovary, although the latter 2 categories usually showed only faint immunoreactivity. In conclusion the frequency and intensity of CD10 immunoreactivity in pure stromal and sex cord-stromal ovarian tumors are low and contrast with the typical strong and diffuse immunostaining seen in endometrial stromal tumors; however, faint CD10 positivity is consistent with the diagnosis of ovarian SCST. Steroid cell tumors are often positive for CD10, but these tumors do not pose problems in differential diagnosis with endometrial stromal tumors. CD10 may play a useful role in aiding the differential between endometrial stromal tumors in the ovary and SCST and stromal tumors.

Renal variant of Anderson-Fabry disease and bilateral renal cell carcinoma.

Anderson-Fabry disease (AFd) is an X-linked metabolic disease with clinical manifestations secondary to accumulation of glycosphingolipids in various tissues. We report the first case in which a patient with renal variant of AFd and chronic renal failure developed bilateral conventional renal cell carcinoma. His metabolic disorder was diagnosed only after histopathologic study of the kidney specimen resected because of the tumoral lesion. There is no clear etiologic relation between the metabolic and neoplastic disease. As AFd is not common or well known and its clinical manifestations tend to be nonspecific, the disorder is often unrecognized, misdiagnosed, or diagnosed late in life. The pathologist should be aware of this disorder when evaluating a kidney specimen from patients with chronic renal failure of unknown cause.