Simple resection of truncal desmoid tumors: A case series

Abstract

Abstract. Desmoid tumors of the extra-abdominal and abdominal wall have been associated with morbidity due to the aggressive nature of the surgery and high recurrence rates. Surgery that does not cause functional impairment is desired for patients with desmoid tumors. In the present study, among patients with desmoid tumors who were prospectively and consecutively treated with identical conservative treatment with meloxicam, a selected patients of patients were treated with less invasive surgery than wide-resection. Out of 60 patients pathologically diagnosed with desmoid tumors, 9 patients with tumors refractory to conservative treatment and 4 patients who refused to receive this type of treatment were treated with planned simple resection. Subsequently, the clinical outcome of the patients and the mutational status of the catenin β-1 (CTNNB1) gene in the tumors were analyzed. The mean age of the 13 patients that underwent planned simple resection was 39 years, and the tumors were located in the abdominal wall in 6 cases, the chest wall in 4 cases and the neck in 3 cases. All excised specimens were evaluated and positive microscopic margins were identified; however, during the mean follow-up period of 30 months, 12/13 cases, 7 of which had T41A mutations and 5 of which had no mutations (wild-type), did not develop recurrence. Only 1 initial case with an S45F mutation in the CTNNB1 gene developed recurrence. The results of the present prospectively treated with simple resection and retrospectively analyzed study suggest that planned simple resection could serve as a therapeutic modality for extraperitoneal desmoid tumors, particularly truncal ones with a wild-type or T41A mutational status.

Risk factors of local recurrence after surgery in extraabdominal desmoid‐type fibromatosis: A multicenter study in Japan

“…A considerable number of previous studies did not detect significant differences in the results of surgery between R0 and R1 either 20,23,31‐33 . Considering these previous and present results together, surgical margin is considered to be only a weak factor in the surgical prognosis, and so R1 resection may be appropriate in selected cases depending on the location and/or CTNNB1 mutation type, thereby avoiding surgery that reduces function 34 …”

Immunohistochemical staining with non-phospho β-catenin as a diagnostic and prognostic tool of COX-2 inhibitor therapy for patients with extra-peritoneal desmoid-type fibromatosis

“…The efficacy of COX-2 inhibitor treatment was determined based on Response Evaluation Criteria in Solid Tumors (RECIST) [ 22 ] evaluated with MRI or CT at the latest follow-up or the endpoint of COX-2 inhibitor therapy as compared to that at the beginning. Patients with PD status could discontinue this therapy and select other treatment options such as low-dose methotrexate and vinblastine therapy [ 10 ] or surgery [ 23 ] with careful consideration of the tumor features, which included location and infiltrative behavior of the tumor, and individual patient’s preference. Considering characteristics of DF (locally aggressive but no metastasis), SD status with no clinical symptom impairing patient’s QOL is thought to reflect tumor dormancy.…”

Desmoid with biweekly methotrexate and vinblastine shows similar effects to weekly administration: A phase II clinical trial

“…Radiation therapy has been considered as a treatment option for refractory DF only if it does not respond to various medical treatments. Excluding operative cases 14 and patients who did not consent to low‐dose chemotherapy, we administered low‐dose MTX + VBL chemotherapy to 38 patients (Figure 1) including 23 patients enrolled in a phase II, non‐randomized, single arm study. Azzarelli and colleagues 10 reported, based on the analysis of 30 patients, a 12‐mo PFS of approximately 95%.…”

“…The treatment modality in our institution has been shifted since 2003 from surgery with wide operative margin to conservative treatment, with meloxicam, which is a nonsteroidal anti‐inflammatory drug (NSAID) and a selective COX‐2 inhibitor 5 . Patients with abdominal wall lesions, those with non‐S45F mutations and non‐limb locations, and those with paralysis or limited ROM unable to wait for the effects of drug treatment to be obtained, are considered for surgery with marginal resection 14 . Treatment results with meloxicam were initially favorable 15 , however the number of patients with PD gradually increased, necessitating a switch to a more effective subsequent treatment.…”

“…5 Patients with abdominal wall lesions, those with non-S45F mutations and non-limb locations, and those with paralysis or limited ROM unable to wait for the effects of drug treatment to be obtained, are considered for surgery with marginal resection. 14 Treatment results with meloxicam were initially favorable 15 , however the number of patients with PD gradually increased, necessitating a switch to a more effective subsequent treatment. Since March 2010 we have prospectively treated patients refractory to meloxicam treatment with low-dose MTX + VBL chemotherapy.…”