Sporadic Case of Desmoid Tumor in Outcomes of Lombotomic Nephrectomy

Abstract

The desmoid tumor is a rare tumor with an incidence of 2-4 cases per million people each year, and represents 0.03% of all cancers. The tumor is composed of fibrous tissue that produces masses of well-differentiated hard elastic consistency. According to their site of onset, the desmoid tumors are classified in abdominal, intra-abdominal, and extra-abdominal. The abdominal cases develop inside the abdominal muscles of the abdominal wall upright, especially in women in their 2nd - 4th decade of life, particularly in those who have been pregnant. A 66-year-old patient underwent nephrectomy in 2006 for the detection of a massive tumor in the right kidney (EI: pT1bNx). The patient came to our observation for the radiological tracking (CT) of a solid lesion of 4 cm below the right arch, 2 years after surgery. For this reason it was decided to refer the patient to a series of percutaneous biopsies. The report describes a histologic lesion of fibromatosis. After one year a new CT exam showed a significant increase of the size of the lesion, with a diameter of 11.6 x 7.9 cm, and abdominal involvement to ascending colon. Given the discrepancy between the CT data and the histological report, it was decided to refer the patient to a lombotomic exploration and the subsequent removal of the lesion, which appeared of hard, elastic consistency and well capsulated. The final histology test confirmed the fibromatosis lesion. The desmoid tumor is a rare tumor characterized by the proliferation of fibrotic tissue. The tumor is composed of well-differentiated fibrous tissue and has a hard-elastic consistency. Regarding the development of dermoid tumors, several risk factors were identified, including extra-abdominal fibromatosis, genetic factors, endocrine factors. Other causes may arise from trauma or abdominal injury in surgical outcomes of appendectomy, laparotomy and other surgical scars (scar fibromatosis) or genetic predisposing factors. The surgical resection of dermoid tumors should be the therapy of choice, complete and radical, to cover the possible excision of a wide margin of surrounding structures concerned, and those arrangements should ensure a low rate of relapse. However, in cases of inoperable cancer due to extension, anti-estrogen therapy may have an important therapeutic and well-tolerated effect, besides being relatively non-toxic, even at high doses. A close follow-up is indicated, however, and warmly recommended.