Wide Excision of a Desmoid Tumor of the Anterior Abdominal Wall with Subsequent Reconstruction.

Desmoid tumors are benign tumors of mesenchymal origin with a complex course of the disease, due to absence of a capsule, infiltrative growth, heterogeneous structure and shape, the true extent of which is difficult to assess before surgery. Despite its benign nature, the recurrence rate reaches 25-50% of cases, and surgical treatment is often accompanied by a number of difficulties with a decrease in the quality of life of patients. In the presented clinical case, a wide excision of desmoid tumor was performed, followed by reconstruction, which ensured a good postoperative result while preserving oncological principles.

Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

Abstract Objective: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. Aim: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. Methods: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. Results: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. Conclusion: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

Role of Nonsteroidal Anti-inflammatory Drug in Treatment of Extra-abdominal Desmoid Tumors

BACKGROUND: We retrospectively reviewed the outcomes of patients who had been treated with meloxicam for the extra-abdominal desmoid tumors and evaluated the correlation between clinical outcome and clinic pathological variables. METHODS: Twenty patients treated with meloxicam were followed up every 3 to 6 months. Meloxicam administration was planned at 15 mg/day orally for 6 months. RESULTS: Of the 20 patients evaluated, according to Response Evaluation Criteria in Solid Tumors criteria, there were five patients with partial response (25.0%), eight with stable disease (40.0%), and seven with tumor progression (35.0%). The cumulative probability of dropping out from our nonsurgical strategy using meloxicam was 35.0% at 1 year and 35.0% at 5 years. CONCLUSIONS: The present study suggests that conservative treatment would be a primary treatment option for this perplexing disease even though we were not able to determine that the use of a cyclooxygenase-2 inhibitor would have an additional influence on the natural course of a desmoid tumor.

Tumor desmoide abdominal / Abdominal desmoid tumor

Los tumores desmoides son infrecuentes, presentan crecimiento localmente agresivo, no suelen dar metástasis pero con frecuencia desarrollan un crecimiento infiltrante que amenaza la vida y puede conducir a una gran morbi-mortalidad. Representan el 0.03% de todos los tumores. En base a la experiencia, los expertos recomiendan la administración de dosis altas de tamoxifeno y sulindac como tratamiento primario para los pacientes con tumores desmoides asociados a poliposis adenomatosa familiar (PAF). Sin embargo, el mejor enfoque después de la intervención quirúrgica para pacientes con tumores desmoides esporádicos, aún no se ha determinado (AU)

Desmoid tumors are infrequent, locally aggressive growth, do not usually metastasize but often develop an infiltrating growth that threatens life and can lead to great morbidity and mortality. They represent 0.03% of all tumors. Based on experience, experts recommend the administration of high doses of tamoxifen and sulindac as a primary treatment for patients with desmoid tumors associated with familial adenomatous polyposis (FAP). However, the best approach after surgical intervention for patients with sporadic desmoid tumors has yet to be determined (AU)

Desmoid Tumor and Duodenal Adenoma in a Patient with Familial Adenomatous Polyposis: A Case Report / 대한소화기내시경학회지

Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited disorder due to germline mutation of the tumor suppressor adenomatous polyposis coli (APC) gene. Multiple large bowel polyps usually develop in adolescence or early adulthood with inevitable progression to colorectal carcinoma. It is well known that patients with FAP are at considerable risk of developing extracolonic manisfestations of the disease. Particularly, desmoid tumors of the abdominal cavity, and duodenal adenomas and carcinomas are the most serious ones. Desmoid tumors and duodenal carcinomas are major causes of death in those patients in whom a prophylactic (procto) colectomy has been performed. We report the case of a 38-year-old man with desmoid tumor and duodenal adenoma developing after total colectomy with ileostomy due to FAP, and literatures were reviewed.