Abdominal desmoid-type fibromatosis – a case report and an overview of treatment options

Abstract

Fibromatosis (desmoid, desmoid tumour) is a tumour belonging to the group of fibrous tissue proliferative diseases. Although the tumour cannot produce distant metastases, it shows local malignancy by invading adjacent tissues, as well as high recurrence rates after local resection. The incidence of fibromatosis is 2-4 cases per 1 million inhabitants per year. Three types of fibromatosis have been distinguished: extra-abdominal, abdominal wall and intra-abdominal type. Although the most common types are sporadic, coexistence with familial adenomatous polyposis (FAP), known as the Gardner’s syndrome, is observed. Available therapeutic options include surgical treatment, watchful waiting, hormone therapy, non-steroid anti-inflammatory drugs, chemotherapy and radiation therapy. This case report aims to draw attention to the difficulties in selecting an appropriate treatment option, limited surgical treatment, the psychological aspect of the disease, as well as the need to actively search for modern diagnostic and therapeutic solutions. We present a case of a 26-year-old patient who was ultimately diagnosed with a desmoid tumour invading the abdominal wall and internal abdominal organs. The patient presented to a county hospital due to suspected gastrointestinal obstruction. It was found from medical history that the patient had palpated a tumour of the abdominal wall 9 months before admission.