Review article: the histological assessment of disease activity in ulcerative colitis.

BACKGROUND: In patients with ulcerative colitis (UC), mucosal healing has emerged as a major therapeutic goal, and is usually assessed endoscopically. Histological healing does not correlate very well with endoscopic mucosal healing in UC and persistent histological inflammation might be a better predictor of future clinical relapse than the endoscopic appearance alone. AIM: To define how histological assessment of disease activity should be best done in UC. METHODS: Electronic (PubMed/Embase) and manual search. RESULTS: At least 18 histological indices to assess disease activity in UC have been described, though none are fully validated. However, histological assessment is increasingly used as a secondary endpoint in clinical trials in UC. After reviewing and discussing existing histological scoring systems for UC activity, we describe features of histological response and define three grades of activity: (i) histological healing - complete resolution of abnormalities; (ii) quiescent disease, - lack of mucosal neutrophils but chronic inflammation may remain; (iii) active disease - presence of neutrophils plus possible epithelial damage. It is recommended that two biopsies are taken from each colonic segment which should include always biopsy of the rectum and the most affected segments. There is to date no agreed preferable scoring system but the Geboes Index is the best validated (kappa for interobserver variation 0.59-0.70). CONCLUSION: Histological assessment of disease activity in UC is increasingly used, but needs to be carefully defined.

Benign intraductal papillary-mucinous neoplasm of the pancreas associated with spontaneous pancreaticogastric and pancreaticoduodenal fistulas.

Invasive intraductal papillary-mucinous neoplasms (IPMNs) of the pancreas may be associated with pancreaticogastric fistulas as shown by case reports. We report the case of a benign IPMN associated with pancreaticogastric and pancreaticoduodenal fistulas. A 70-year-old woman was admitted with intestinal obstruction. Computed tomography and MRI showed a large dilatation of the main pancreatic duct (>1 cm) with intraductal nodules, and pancreaticogastric and pancreaticoduodenal fistulas. Several features in imaging were present to support a malignant IPMN, so that the patient underwent a pancreaticoduodenectomy. The histopathological examination of the surgical specimen showed a benign IPMN. This case proves that a benign IPMN can cause pancreaticogastric and pancreaticoduodenal fistulas, probably resulting from mechanical factors.

Composite renal cell carcinoma with clear cell renal cell carcinomatous and carcinoid tumoral elements: a first case report.

Renal endocrine tumours are extremely rare, and carcinoid tumoral elements in renal cell carcinoma have never been reported. This is the first report of a composite renal cell carcinoma containing a clear cell renal cell carcinoma associated with carcinoid tumoral elements, in a patient with synchronous metastatic disease. In the absence of specific radiological and clinical manifestations, typical morphological features as well as an immunostaining profile of neuroendocrine differentiation were identified by microscopy. Secondary nodal and liver localisations were characterised by carcinoid elements only. Despite antiangiogenic therapy, liver metastasis progressed, suggesting that adjuvant therapy cannot be based on the presence of the clear cell renal cell carcinoma component. In this context, extensive tissue sampling is recommended to reveal the endocrine component that is the most aggressive element of such a composite carcinoma.

[Primary leiomyosarcoma of the epididymis: a case report with review of the literature]. / Léiomyosarcome de l'épididyme : à propos d'un cas avec revue de la littérature.

Primary epididymal leiomyosarcoma is uncommon: only 16 cases has been reported in the literature. We present an additionnal case in a 78-year-old man, treated for a prostatic adenocarcinoma by gonadorelin (LH-RH) analogue, who had an epididymal tumor. A right orchidectomy with high ligation of the spermatic cord was performed. The diagnostic of primary leiomyosarcoma of the epididymis was made. The patient is dead 2 years later with no recurrence of disease. A review of reported cases is made.

Reactive nodular fibrous pseudotumor: a first report of gastric localization and clinicopathologic review.

Reactive nodular fibrous pseudotumor (RNFP) of the gastrointestinal tract is a distinct benign lesion, which could originate from a reactive proliferation of multipotential subserosal cells. This is the first case to be reported in the stomach. It was fortuitously discovered in a 60-year-old man with history of bulbar ulcer and gastritis. Gross examination revealed three lesions in the gastric wall and an adjacent lesion in the lesser omentum. Histologically, lesions were composed of a proliferation of spindle and stellate cells in a dense collagenic hyalinized background containing a mononuclear cell inflammatory infiltrate with numerous lymphoid aggregates and plasma cells with perivascular disposition. Immunohistochemistry showed staining for cytokeratins (AE1/AE3), vimentin and smooth muscle actin, without staining for the neurofilament and S100 proteins, synaptophysin, calretinin, CD117 (c-kit), CD34, desmin, caldesmon or anaplastic lymphoma kinase (ALK-1). Complete excision was performed, and no evidence of disease was found 4 months later. After analysing clinical, morphological and immunohistochemical features of this entity, the main differential diagnoses will be discussed, including calcifying fibrous pseudotumor, which shares morphological characteristics with RNFP, but which immunohistochemistry and the ultrastructural study suggest that it may be a result of another reactive process.