BACKGROUND: The real-world evidence about the efficacy of cytotoxic chemotherapy in desmoid tumors is still limited. We investigated the efficacy of chemotherapy in the treatment of recurrent or progressive desmoid tumors.
METHODS: The patients with desmoid tumors who had received cytotoxic chemotherapy between November 2007 and June 2020 in two tertiary hospitals in Korea were reviewed.
RESULTS: A total of 25 patients were included in the analysis. The most common primary tumor site was the intra-abdominal or pelvic cavity (56%), followed by the trunk and abdominal wall (24%), extremities (16%), and head and neck (4%). Sixty percent of the patients had familial adenomatous polyposis and 76% received doxorubicin plus dacarbazine. The objective response rate and disease control rate was 64% (95% confidence interval [CI]: 40.7-82.8) and 96% (95% CI: 77.2-99.9), respectively. With the median follow-up time of 55 months (95% CI: 41.0-68.2), the 3-year PFS rate was 65% (95% CI: 41.1-80.5), and the 3-year OS rate was 89% (95% CI: 63.8-97.3). Grade 3 or 4 hematologic adverse events were reported in 14 patients, all of which were manageable.
CONCLUSIONS: Our real-world evidence suggests that doxorubicin-based cytotoxic chemotherapy can be an effective treatment option for recurrent and progressive desmoid tumors with respect to favorable clinical outcomes.

UNASSIGNED: In Japanese patients with familial adenomatous polyposis (FAP), colectomy tends to be postponed or avoided.
UNASSIGNED: This study aimed to clarify the current clinical practice from a Japanese multicenter cohort study database.
UNASSIGNED: We analyzed the records of 250 patients with non-dense FAP who did not require colorectal cancer removal. The clinical outcomes were compared between patients who received colectomy (n = 142) (Group A) and those who did not receive colectomy (n = 108) (Group B).
UNASSIGNED: The colectomy rate based on the age at the final follow-up examination was 46%, 60%, 54%, 65%, at ≤29, 30-39, 40-49, and ≥ 50 years, respectively (P = .11). The development of colorectal cancer did not differ between Groups A and B (25% vs 22% P = .67); however, colorectal cancer was diagnosed at the Tis stage in 88% of the patients with colorectal cancer in Group B, and 34% of the patients with colorectal cancer in Group A (P < .01). Regarding survival, all patients in Group B were alive at the final follow-up examination. In contrast, six patients in Group A died, including three patients with desmoid tumors and one with colon cancer.
UNASSIGNED: Over one-third of patients with non-dense FAP (polyps ≤ 1000) in Japan did not receive colectomy at >30 years of age, and patients who managed without colectomy showed acceptable survival with the early diagnosis of colorectal cancer, and a very low incidence of desmoid tumor development, indicating that this approach represents a potential option for the management of selected non-dense FAP patients.

OBJECTIVE: Aggressive fibromatosis of the abdominal wall is easily misdiagnosed prior to surgery as abdominal wall endometriosis or other tumors. The authors intend to evaluate the clinical features and outcomes of aggressive fibromatosis of the abdominal wall after surgical resection.
METHODS: We retrospectively analyzed the data from 24 patients treated in the First Hospital of China Medical University from January 2011 to June 2019 and discussed the characteristic of this disease after a review of literature worldwide.
RESULTS: Most patients were female (91.7%) with a mean age of 33 years (range 26-56 years). The most common symptom was progressive abdominal wall discomfort (ie, abdominal pain) in 16 patients (66.7%). Twelve patients (50%) had a history of cesarean section. All 24 patients underwent surgical R0 resection due to progression of disease or pain, including 12 patients treated with simple tumor resection and 12 patients treated with tumor resection followed by abdominal wall tension-free repair using synthetic mesh. Postoperative complications included incisional infection in three patients (12.5%), incisional hernia and postoperative bleeding in one patient each (4.2%). The local recurrence rate was 12.5%.
CONCLUSIONS: Aggressive fibromatosis of the abdominal wall mainly occurs in young women after cesarean section or other surgeries, with a high recurrence rate. Radical resection is an optimal treatment for the patients with progressive cases, and the restoration of extensive defects of the abdominal wall can be achieved with synthetic mesh, which leads to a good restoration of abdominal wall integrity.

BACKGROUND: To determine the activity of radiotherapy in patients with inoperable desmoid-type fibromatosis (DF) a multicenter prospective phase II trial was carried out.
METHODS: Patients with inoperable progressive disease of primary, recurrent or incompletely resected lesions received a dose of 56 Gy in 28 fractions. Follow-up MRI studies were carried out every 3 months for 2 years and thereafter every 6 months. The primary end point was local control rate at 3 years, estimated by a nonparametric method for interval-censored survival data. Secondary end points were objective tumor response, acute and late toxic effect.
RESULTS: Forty-four patients (27 F/17 M) were enrolled from 2001 to 2008. Median age was 39.5 years. Main tumor sites included trunk 15 (34.1%) and extremities 27 (61.3%). Median follow-up was 4.8 years. The 3-year local control rate was 81.5% (90% one-sided confidence interval 74% to 100%). Best overall response during the first 3 years was complete response (CR) 6 (13.6%), partial response (PR) 16 (36.4%), stable disease 18 (40.9%), progressive disease 3 (6.8%) and nonassessable 1 (2.3%). Five patients developed new lesions. After 3 years, the response further improved in three patients: (CR 2, PR 1). Acute grade 3 side-effects were limited to skin, mucosal membranes and pain. Late toxic effect consisted of mild edema in 10 patients.
CONCLUSIONS: Moderate dose radiotherapy is an effective treatment of patients with DF. Response after radiation therapy is slow with continuing regression seen even after 3 years.

BACKGROUND: Intra-abdominal desmoid tumors (IADTs) are a common complication of familial adenomatous polyposis (FAP). Treatment is not standardized for advanced disease. Medical and surgical treatments may be ineffective in preventing complications, which can cause intestinal failure. Home parenteral nutrition (HPN) can be a life-saving treatment in these patients. The aim of this study was to investigate the association with HPN in FAP-IADTs.
METHODS: A retrospective review of FAP patients with IADTs at the Cleveland Clinic (CC) between 1980 and 2009 was performed. Patients and tumor characteristics were retrieved from the CC Jagelman Registry for Inherited Neoplasms and CC HPN database. Inclusion criteria were FAP-IADTs and 6-month follow up at CC. Exclusion criteria were <6-month follow-up, lack of 3-dimensional lesion or sheet desmoid, and/or incomplete medical records. Kaplan-Meier curves were analyzed for HPN and non-HPN groups.
RESULTS: One hundred fifty-four patients were included and divided into 2 groups: HPN (n = 41, 26.6%) and non-HPN (n = 113, 73.4%). The HPN group was more likely to have advanced-stage disease and significantly higher incidence of chronic abdominal pain, narcotic dependency, bowel obstruction, ureteral obstruction, deep vein thrombosis, pulmonary embolism, fistulae, and sepsis (P < .05). The need for HPN represented a strong predictor of mortality (5-year survival HPN = 72% vs non-HPN = 95%), but duration of HPN did not affect mortality.
CONCLUSIONS: HPN, although a life-saving treatment, is an independent poor prognostic factor associated with high morbidity and mortality.