INTRODUCTION: Aggressive fibromatosis, also known as desmoid tumour (DT), is a locally invasive soft tissue malignancy originating from fascial planes, connective tissue, and musculoaponeurotic structures of the muscles. The symptoms greatly depend on the location and size of the tumour. CASE REPORT: A 68-year-old male patient without any comorbidities with a large, palpable mass in the abdomen underwent computed tomography (CT) of the abdomen during diagnostic examination in September 2017 in another centre. The CT scan revealed a giant intraperitoneal 30×40cm tumour without signs of infiltrating the surrounding organs and large vessels. The tumour biopsy revealed an aggressive DT. The patient was scheduled for tumour resection. Midline laparotomy was performed in the supine position under general anaesthesia. After gaining access to the abdominal cavity, 8 litres of clear ascites were evacuated. The tumour was not attached to the abdominal wall. Large omentum was freed from the DT. The perioperative finding confirmed the CT images of DT encapsulation of the medial colic artery, part of the small intestine, and transverse colon. The tumour was resected with part of the mesenteric radix, 30 cm of small intestine, and 2/3 of the transverse colon. After the DT was removed entirely, the small intestine was re-anastomosed end to end. The abdominal cavity and the liver were carefully checked for bleeding. The abdominal cavity was closed in a standard manner. RESULTS: The postoperative hospital stay was uneventful. The patient was discharged on the 7th postoperative day with prophylactic low weight molecular heparin for one month. Currently, we have five months of follow-up with no signs of DT recurrence based on CT examination. The histology of the resected tumour confirmed the diagnosis of a desmoid tumour (aggressive abdominal fibromatosis). CONCLUSION: Desmoid tumours are benign neoplasms with no metastatic potential. However, their treatment is challenging due to their aggressive growth, infiltrative behaviour, and a high tendency to recur.
- Klíčová slova
- abdominal, desmoid tumour, fibromatosis,
- MeSH
- agresivní fibromatóza * diagnostické zobrazování chirurgie MeSH
- břišní stěna * chirurgie MeSH
- laparotomie MeSH
- lidé MeSH
- mezenterium chirurgie MeSH
- počítačová rentgenová tomografie MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Desmoid tumor of the breast is a rare fibroblastic disease, a slow-growing and histologically benign lesion that accounts for 0.2% of all breast tumors. It can arise either primarily from the breast parenchyma or, by secondary invasion, from the muscolo-aponeurotic layer of the pectoral muscles. These tumors do not metastatize, have no capsule but do have the propensity to aggressively invade local tissues and organs. It has been suggested that these tumours are associated with surgical and non-surgical tissue trauma, certain hormones and genetic susceptibility. We describe two cases of breast desmoid tumor after breast augmentation. A 52-year-old caucasian woman and 38-year-old caucasian woman presented for a breast implant change. During surgery an inelastic, soft, capsulated mass, arising from the periprosthetic capsula in the area of pectoralis major muscle was found. The mass was removed en bloc together with a portion of the periprosthetic capsula, the resection including a portion of the pectoralis major muscle fascia in both cases. The results of this histological examination suggested an extra-abdominal desmoid tumor. To date both the local and the general conditions of the two patients are good, with no signs of recurrence of the lesion.
- MeSH
- agresivní fibromatóza patologie chirurgie MeSH
- dospělí MeSH
- implantace prsní náhrady * MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory prsu patologie chirurgie MeSH
- odstranění implantátu MeSH
- pooperační komplikace chirurgie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
A case- review describing a desmoid in a young female. The tumor originated at the tendinuous attachment of the m. rectus abdominis on the pubic bone, which had had two relapses. During the third--so far the latest--procedure it required left-sided hemipelvectomy with resection of the rectum and a part of the vagina. The patient was instructed about the serious character of the disease, the requirement for the radical resection and about the requirement for a careful long-term follow-up of the patient after the procedure.
- MeSH
- agresivní fibromatóza diagnóza chirurgie terapie MeSH
- dospělí MeSH
- hemipelvektomie metody MeSH
- lidé MeSH
- lokální recidiva nádoru MeSH
- musculus rectus abdominis MeSH
- nádory močového měchýře chirurgie MeSH
- nádory svalů chirurgie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
In the submitted review of the literature the authors evaluate contemporary therapeutic possibilities of mesenterial desmoids. A more detailed pattern of cytotoxic and non-cytotoxic chemotherapy, actinotherapy and hormonal therapy is presented. Surgical resection is limited by the size of the tumour and above all by early detection before the size of 15 cm is reached. In larger tumours conservative treatment by a combination of non-cytotoxic and hormonal treatment is better. Actinotherapy of the intraabdominal region is not suitable. The percentage of relapses is high, a standard therapeutic procedure has not been elaborated so far. Despite rather surprising remissions of desmoids in individual cases the general results of different groups are not encouraging. In a case-history the authors describe their experience with the treatment of a young female patient with a mesenterial desmoid which, however, was not successful.
- MeSH
- agresivní fibromatóza chirurgie terapie MeSH
- dospělí MeSH
- lidé MeSH
- mezenterium * MeSH
- peritoneální nádory chirurgie terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
The authors demonstrate in a group of 10 patients treated in the course of 15 years the rare incidence of aggressive fibromatosis of the extremities and the large number of relapses after surgery. They also emphasize the necessity of detailed preoperative examination, priority of a surgical approach, as compared with radiotherapeutic procedures, and the possibility of skeletal and vascular reconstruction after radical surgery.
- MeSH
- agresivní fibromatóza chirurgie MeSH
- dospělí MeSH
- končetiny * chirurgie MeSH
- lidé středního věku MeSH
- lidé MeSH
- lokální recidiva nádoru MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH