Isolated limb perfusion using tumour necrosis factor α and melphalan in patients with advanced aggressive fibromatosis.

Tumour necrosis factor (TNF) α and melphalan-based isolated limb perfusion (TM-ILP) is a limb-saving treatment modality for soft tissue tumours. This study reports the results of TM-ILP treatment in patients with aggressive fibromatosis. Institutional databases of three European centres were searched. All patients who received TM-ILP treatment for aggressive fibromatosis between 1990 and 2012 were included. Twenty-five patients received 28 TM-ILP treatments. The median age of patients was 28 (i.q.r. 19-34) years and median hospital stay was 8 (7-12) days. Median follow-up was 84 (34-114) months. A complete response was achieved after two TM-ILP treatments, and a partial response after 17 treatments in 16 patients. Functional outcome was good; 16 patients had no physical limitations, and six patients had some limitations but did not need medical aids. Amputation was prevented in all but three patients.

A phase I trial of PF-03084014, an oral reversible γ-secretase inhibitor (GSI), in solid tumor malignancies shows drug tolerability in patients. Evidence of Notch pathway inhibition was demonstrated in peripheral blood. A surprisingly high rate of response was seen in desmoid tumors, a rare but often locally aggressive sarcoma.

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.