Daniela Zambonin

A multidisciplinary approach to desmoid tumors. When intra-abdominal fibromatosis degenerates into an abscess, which is the right treatment?

INTRODUCTION Desmoid tumors are rare benign tumors that originates in the fibrous sheath or musculo-aponeurotic structure. Histologically benign, they tends to invade locally and to be recurrent. PRESENTATION OF CASE We report a rare case of an intra-abdominal desmoid tumor in a patient affected by familial adenomatous polyposis, which degenerated into abscess. Male, 38 years, was hospitalized for abdominal pain, bowel obstruction and fever. The computed tomography showed a big dishomogeneous mass occupying the whole mesentery with internal massive liquefaction. The mass extended from the epigastrium for 13cm up to L3. On the right mesogastric side a solid, thick mass of about 2cm, with a length of 4.5cm, was identified; it was not cleavable from the wall and from some of the loops. We decided to perform a computed tomography-guided percutaneous drainage. Two hundred ml of purulent necrotic material was aspirated, and washing with antibiotic solution was carried out. Cytological examination of fluid drainage showed histiocytes and neutrophils. At follow-up, the patients clinical condition had improved. An abdominal ultrasound showed a substantial reduction in the diameter of the mass. DISCUSSION Diagnosis and treatment of desmoids tumor in patients with familial adenomatous polyposis may be difficult, especially when desmoids are located intra-abdominally and in the mesentery. Seldom will desmoid tumors be complicated by abscess formation. CONCLUSION The management of desmoids tumors is not easy and the choice of the best treatment may be difficult due to the different possible anatomical presentations.

Pylephlebitis and Crohn’s disease: A rare case of septic shock

Highlights • We describe a rare case of septic shock due to mesenteric pylephlebitis in a 47 years old man affected with Crohn’s disease.• The occurrence of mesenteric pylephlebitis in Crohn’s disease is extremely rare, but to be considered in presence of a septic shock without clinical signs of peritonitis.• Computed tomography scan confirmed the diagnosis and medical therapy led to a complete clinical remission of the mesenteric pylephlebitis.• Optimizing pylephlebitis management, we performed an elective surgery aiming to bowel sparing and minimizing the intra and post-operative complications.

First description of the association of two rare diseases with familial adenomatous polyposis (FAP)

Dear Editor: Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease due to mutations in the tumor suppressor adenomatous polyposis coli (APC) gene. Clinically, FAP is characterized by the development of multiple adenomatous polyps throughout the bowel, and from more than 20 years, its association with a high risk of intestinal cancer and with a variety of extraintestinal manifestations is well known. Even though many scientific papers documenting the genetic bases of phenotypic heterogeneity in FAP have been published and in spite of the presence of many studies concerning the association between FAP and the development of other organic manifestations in addition to the principal tumors related to this syndrome, we report the first description of an association between FAP and Kienböck’s disease and atrial myxoma, respectively.

About the Colorectal Cancer Screening and Surveillance in Crohn's Colitis

Dear Sir, Weread withinterest thecommendablestudy by Dr Basseri 1 and colleagues that focuses on the risk of developing Colorectal Cancer (CRC) in patients with Crohns Colitis (CC). They analyzed results of screening and endoscopic surveillance in 411 patients followed from 1992 to 2009 at their institution. The study faces a yet unsolved problem and we would be pleased to suggest some observations born from a recent published clinical experience. 2 Literature shows that screening and endoscopic surveillance can be helpful in finding an early stage of CRC in patients with CC, but the efficacy in improving CRC related survival for this setting of patients is not demonstrated. This is because studies include small and heterogeneous series of patients, very often with selection bias, resulting in a low statistical evidence. It is a problem of difficult solution due to the small number of patients analyzed, that does not spare even this study. The authors affirm that the incidence of dysplasia and cancer was lower than in similarly designed study by Friedman andGillen. 3,4 They explainthatbecauseofthelowerfrequency of extensive colitis in the patient cohort (55%) compared to that of the aforementioned studies (90 and 100%), thus suggesting the extent of inflammation as risk factor for CRC development. Nevertheless, such a big difference could be explained by a dissimilarity in the inclusion criteria regarding screening and surveillance that it does not make the studies comparable. As consequence, the assertion that incidence was lower seems not correct. Moreover, the study does not consider the activity of the disease nor the relation with structuring or penetrating disease as possible variables. Additionally the lack of a control group, as well as data concerning the incidence of sporadicCRCingeneralpopulationofthesamegeographic area and its frequency of localization (distal or proximal large bowel) might create difficulties of interpretation. In this point of view the activity of the disease may result more important than the extension of the involved mucosa as a risk factor for malignant transformation, particularly if we consider the reported mean age at diagnosis of carcinoma (N55 yr). In order to incorporate the current results to clinical practice, much more knowledge would be required and the study, even if interesting and well written, does not seem to add more to the present literature.