Spontaneous ureteric rupture secondary to an invasive desmoid tumour.

Spontaneous ureteric rupture is a rare entity that presents as an extravasation of urine from the ureter without previous surgery, ureteric manipulation and external trauma of the ureter. To our knowledge this is the first reported case of ureteric perforation secondary to a desmoid tumour of the mesentery.

In this article we present our personal experience in the successful treatment of six cases of sporadic desmoids, five in females of child bearing age, and all in the anterior abdominal wall and one extra abdominal in a child aged 13 y in the gluteal region (Case 6). It is very interesting and unique to see two desmoid tumours developing in the same patient (Case2)one in each of the Rectus abdominal muscles (Right and left)

We report the clinical case of a patient who showed an “accelerated” form of polyposis, with development of major lesions within the first decade of life. The patient belongs to a familial adenomatous polyposis family-already described in 2001-featured by profuse polyposis at an early age of onset and desmoid tumors in the majority of affected individuals (of both sexes). The family was characterized by an uncommon mutation of the APC gene. In 2010, at age 9, the patient was operated of total colectomy and endorectal pull-through of the small intestine owing to profuse colorectal adenomatosis. New desmoids developed in 2011 and 2012, and required chemotherapy. Further analysis of the APC gene in the proband revealed several polymorphisms. One of these (c.398A>G) had not been previously reported, nor was present in two other affected members of the family. The clinical case, and the practical implications for therapy, are discussed according to the most recent theories of colorectal cancer development. Long-term treatment with Cox-2 inhibitors might represent a good option for this patient.

The objective was to determine the usefulness of the tumor vessel sign for differentiating the groups of circumscribed hypervascular abdominopelvic mesenchymal tumors. We enrolled a total of 4 groups such as patients with gastrointestinal stromal tumor (GIST) (26 patients), those with paraganglioma (9 patients), those with primary sarcomas (13 patients, excluding those with liposarcoma), and those with desmoid tumor (6 patients). The reviewers evaluated the presence of tumor vessels capable of directly tracing from the tumor margin to the named vessels. Twenty-five of 26 GIST cases, all of the paragangliomas, all of the primary sarcomas, and all of the desmoid tumors showed a positive sign. The presence of the sign is considered to be helpful for differentiating the 4 groups of tumors.

We report the case of a 69-year-old man who presented to our hospital with an acute pulmonary embolism. The patient had a past history of colic surgery for a polyp with a high-grade dysplasia. Pulmonary angiography showed partial occlusion of the right superior lobe artery and partial occlusion of the middle lobe artery. The patient was given thrombolytic therapy. Abdominal computerized tomography revealed a mesenterial giant mass with compression of the inferior vena cava (IVC). A biopsy of the mass, confirming aggressive fibromatosis. This report underlines the potential of imaging investigations of abdomen and vena cava if pulmonary embolism is suspected, especially when there is no evidence of peripheral venous thrombosis or other predisposing factors.

This report describes an 18-year-old woman presenting with abdominal distension, left flank pain, and hypertension. She had a huge abdominal mass, diagnosed as a mesenteric desmoid-type fibromatosis, causing compression of the left external iliac vessels and ureter, as well as elevated renin concentration and hypertension. After surgical removal of the mass, all signs improved including hypertension.