Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

Sporadic desmoid tumors of the abdominal wall: the results of surgery.

PURPOSE: Before the wait-and-see policy became the standard approach in abdominal wall desmoid tumors, surgery was performed on a systematic basis. Surgery remains indicated for progressing tumors but its extent is debatable. The abdominal wall is a common site of origin of sporadic desmoids, usually associated with a favorable prognosis. We analyzed the results of surgery at this specific site. METHODS: Data from 33 patients affected by sporadic desmoid tumors of the abdominal wall (31 primary, 2 recurrent) consecutively treated at our cancer center between January 2000 and September 2013 were retrospectively studied. RESULTS: Twenty-nine patients underwent surgery upfront and 1 after progression during the initial wait-and-see period. Prosthetic reconstruction of the abdominal wall was required in 28 patients. The average hospital stay was 5 days. Three patients developed surgical complications. Local recurrence-free survival was 90% at 5 and 10 years. Three patients had an uneventful childbirth during the follow-up after surgery. CONCLUSIONS: Desmoid tumors of the abdominal wall have a favorable prognosis after surgical resection, which remains a safe and effective treatment. Wild-type tumors are common, whereas the incidence of S45F mutation in the beta-catenin gene is lower than in other anatomic sites. Upfront surgery may be considered in selected women who wish to bear a child.

Surgical outcomes following resection for sporadic abdominal wall fibromatosis.

BACKGROUND: Fibromatosis can be classified according to site of origin, namely, extraabdominal, abdominal wall, or intraabdominal. This study reports on the surgical management and long-term outcomes from a single center in the management of sporadic abdominal wall fibromatosis. METHODS: Patients who underwent surgery for abdominal wall fibromatosis between 1998 and 2013 were identified from a prospectively maintained database. A retrospective review of patient demographics, tumor characteristics, surgical outcomes, operative management, and recurrence rates was performed. RESULTS: Fifty patients underwent resection of a primary sporadic abdominal wall fibromatosis; 48 were female, of whom 43 reported previous pregnancy. Twenty-seven patients (54 %) had prior abdominal surgery for other pathologies. Macroscopic clearance was achieved in all cases. The median size of tumors resected was 8 cm (range 3-15 cm). The abdominal wall defect was reconstructed with prosthetic mesh in 47 of 50 cases. No major postoperative complication was encountered. Microscopic margins were reported as clear (R0) in 21 of 50 cases. With a median follow-up of 6 years (range 1-15 years), 46 of 50 patients remain disease free, with a median disease-free survival of 5 years. Of these 46 disease-free patients, 13 had further pregnancies without complications from either the abdominal mesh repair or tumor recurrence. CONCLUSIONS: For asymptomatic sporadic abdominal wall fibromatosis, observation is an accepted first-line strategy. However, in contrast to extraabdominal fibromatosis, the preferred definitive treatment is surgical resection, which is recommended as first-line therapy in symptomatic patients, selected cases when tumors are progressing, and those with tumors >7 cm.

Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series.

BACKGROUND: Desmoid tumors are rare and exhibit a highly unpredictable natural history. We sought to analyze prognostic factors associated with recurrence in a large single-institution study of patients with desmoid tumors. METHODS: We performed a retrospective review of 177 patients with desmoid tumor who underwent macroscopically complete surgical resection, with or without the addition of radiotherapy (RT) or systemic therapy, from 1970 to 2009. We examined patterns of presentation, all known risk factors for recurrence, and their association with recurrence-free survival (RFS). RESULTS: Twenty-two patients (12 %) had intra-abdominal desmoid tumors, and 155 (88 %) had extra-abdominal tumors. Patterns of presentation included primary (n = 133, 75 %) and locally recurrent (n = 44, 25 %) disease. Treatment was surgery alone in 125 patients (71 %), surgery and RT in 36 (20 %), and surgery and systemic therapy with or without RT in 20 (11 %). Median follow-up was 40 months. Overall, the local relapse rate was 29 %, and 10-year RFS was 60 %. R0 resection status was the only predictor of freedom from local recurrence on multivariate analysis (odds ratio 0.32; 95 % confidence interval 0.15-0.66; P = 0.002). The selective use of adjuvant RT appeared to improve local control in patients with positive margins. CONCLUSIONS: For patients with desmoid tumors undergoing surgery, wide excision with negative margins should be the goal, but not at the expense of function, as fewer than half of patients with positive margins will experience recurrence.

Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients.

BACKGROUND: Desmoid tumors are rare soft-tissue neoplasms with limited data on their management. We sought to determine the rates of recurrence following surgery for desmoid tumors and identify factors predictive of disease-free survival. METHODS: Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Clinicopathologic and treatment characteristics were analyzed to identify predictors of recurrence. RESULTS: Median age was 36 years; patients were predominantly female (68 %). Desmoid tumors most commonly arose in extremities (32 %), abdominal cavity (23 %) or wall (21 %), and thorax (15 %); median size was 7.5 cm. Most patients had an R0 surgical margin (60 %). The 1- and 5-year recurrence-free survival was 81.3 and 52.8 %, respectively. Factors associated with worse recurrence-free survival were: younger age (for each 5-year increase in age, hazard ratio [HR] = 0.90, 95 % confidence interval [95 % CI] 0.82-0.98) and extra-abdominal tumor location (abdominal wall referent: extra-abdominal site, HR = 3.28, 95 % CI, 1.46-7.36) (both P < 0.05). CONCLUSIONS: Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years. Factors associated with recurrence included age, tumor location, and margin status. While surgical resection remains central to the management of patients with desmoid tumors, the high rate of recurrence highlights the need for more effective adjuvant therapies.

Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution.

BACKGROUND AND OBJECTIVE: Previous reports even large studies discussing the prognosis of desmoids have included tumors from intra- and extra-abdominal sites as well as incomplete resection. The purpose of this study was to explore prognostic factors associated with the recurrence free survival (RFS) rate in surgically treated extra-abdominal and abdominal wall desmoids. PATIENTS AND METHODS: A total of 198 consecutive desmoid patients were treated with surgery over a 20-year period at a single institution. Of these, 151 patients with extra-abdominal and abdominal wall tumors were retrospectively reviewed. One hundred thirteen patients were referred for the primary tumor and the other 38 for recurrent disease initially treated elsewhere. All patients underwent a macroscopically complete resection. RESULTS: The median follow-up interval was 102 months. Thirty-one patients (20.5%) had a local recurrence (LR). No patients died of the disease. The 5- and 10-year RFS was 79.7% and 78.5%, respectively. Admission status, gender, tumor size, margin status, location, and number, were predictors of LR in univariate analysis. Tumor size and margin status were independent prognostic factors in multivariate analysis. Positive margins were predictive of recurrence of primary disease, and also showed a trend for recurrent disease, which was not statistically significant. The selective use of adjuvant radiation did not show significant benefit over local control. CONCLUSIONS: Regardless of primary or recurrent disease, microscopically negative margins should always be the goal for extra-abdominal desmoids surgery, if no cosmetic defects or function demolition is encountered. Extra-abdominal desmoids deserve more attention and should be treated more aggressively, especially when leaving positive margins.

High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors.

BACKGROUND: Desmoid tumors are mesenchymal nonmetastasizing neoplasms. Although rare in the general population, they are a common extracolonic manifestation of familial adenomatous polyposis (FAP). Because of high tumor recurrence rates, surgery has been less than satisfactory in the treatment of desmoid tumors. In the current study, high doses of tamoxifen in combination with sulindac were used to treat severe desmoid tumors to avoid surgery. METHODS: Since 1992, 25 patients at Heinrich Heine University (Dusseldorf, Germany) were treated with a combination of tamoxifen and sulindac. In the current study, 17 patients with FAP-associated and 8 patients with sporadic desmoid tumors received 120 mg of tamoxifen and 300 mg of sulindac daily. Every 6 months, the protracted course of desmoid growth was measured by computed tomography and/or magnetic resonance imaging scans. Tumor responses were characterized as progressive disease, stable disease (SD), partial regression (PR), and complete regression (CR). RESULTS: Of the group of patients who received tamoxifen and sulindac as a primary treatment, all three patients with sporadic desmoid tumors demonstrated cessation of growth, and 10 of the 13 patients with FAP-associated tumors achieved either a PR or CR. In the sporadic desmoid tumor group, eight of nine patients developed tumor recurrences after undergoing surgery at other institutions. Of these, two patients had SD and two patients had a PR to CR. CONCLUSIONS: The patients with desmoid tumors who were managed conservatively with high-dose tamoxifen and sulindac had the best outcome. Desmoid tumor recurrence after surgery was high and in the FAP-associated tumor group, therapy with tamoxifen and sulindac was found to be less successful. Based on this experience, the authors recommended high-dose tamoxifen and sulindac as the primary treatment for patients with FAP-associated desmoid tumors. However, to our knowledge, the best approach after surgical intervention for patients with sporadic desmoid tumors remains to be determined.

Soft tissue tumors of the abdominal wall: analysis of disease patterns and treatment.

HYPOTHESIS: Abdominal wall tumors, though clinically similar, have varying degrees of biological behavior. DESIGN: Retrospective review of prospective databases. SETTING: Memorial Sloan-Kettering Cancer Center. PATIENTS: Eighty-five patients with abdominal wall soft tissue tumors. MAIN OUTCOME MEASURES: Primary endpoints included time to first local recurrence, distant metastases, and disease-related mortality. Survival analysis was performed by Kaplan-Meier method, and comparisons were made by log-rank analysis. RESULTS: Thirty-nine desmoids, 32 soft tissue sarcomas (STS), and 14 dermatofibrosarcoma protuberans (DFSP) underwent surgery directed at achieving margin-negative resection. Unlike DFSP, most STS (77%) and desmoids(87%) were deep lesions requiring full-thickness abdominal wall resection and mesh reconstruction. Median follow-up time was 53 months, 101 months, and 31 months, with 5-year local recurrence-free survival rates of 97%, 100%, and 75%, for desmoids, DFSP, and STS, respectively. Desmoid tumors resected with positive microscopic margins had higher local failure rates (68% [positive margin] vs 100% [negative margin] 5-yr local recurrence-free survival, P<.05). For STS, high grade, deep location, and size at or above 5 cm were adverse prognostic factors for disease-specific and distant recurrence-free survival (P<.05); patients experiencing local recurrence was associated with decreased 5-year relapse-free survival rates (87% [primary] vs 50% [local recurrence], P<.05). Characteristically, no DFSP or desmoid developed distant metastases. Soft tissue sarcomas had significantly lower relapse-free survival rates than DFSP or desmoids (P<.05). CONCLUSION: Abdominal wall tumors demonstrate a broad spectrum of biological behavior. Desmoids and DFSP are a local problem. High grade, size at or above 5 cm, and deep location predict distant failure and tumor-related mortality for patients with STS. Complete surgical resection is the recommended treatment approach to achieve local control. Stratification by prognostic factors will facilitate selection of patients with STS for adjuvant systemic therapies.

Desmoid disease in patients with familial adenomatous polyposis.

PURPOSE: The aim of this retrospective study was to review the clinical features, and surgical and medical management of patients with familial adenomatous polyposis-associated desmoid tumors. METHODS: From 1980 to 1997, 97 of 780 patients with familial adenomatous polyposis developed desmoid disease. Clinical and demographic data; operative notes; and histologic, radiologic, and follow-up reports were retrieved from patients' medical records. Risk factors for desmoid disease, such as prior surgery, age at desmoid tumor diagnosis, pregnancy, and family history were sought. The outcome after noncytotoxic and cytotoxic therapy was evaluated with respect to improvement of symptoms. RESULTS: There were 38 males with a mean age of 32.1 years and 59 females with a mean age of 29.1 years. A family history of desmoid tumors was found in 41 patients (42 percent), and a history of pregnancy was documented in 33 females (56 percent). The most common clinical presentation was small-bowel obstruction (58 percent). One-half of the desmoids were located in the mesentery, and 32 percent were located in the mesentery and the abdominal wall. Desmoids developed after colectomy in 77 cases (80 percent), after a mean time of 4.6 years. Partial resection of desmoid tumor was performed in 46 patients (47 percent), resection of extra-abdominal desmoid tumors was performed in 17 cases (17 percent), and biopsy only was performed in 34 patients (35 percent). Postoperative morbidity was 23 percent after desmoid tumor resection. Eight patients (8 percent) died of their intra-abdominal desmoid. Mean follow-up time was 5.3 years. Sulindac, tamoxifen, or toremifene therapy was able to alleviate symptoms in only 4 of 31 patients. Symptomatic improvement was noted after chemotherapy in six of ten patients with extremely complex desmoids. CONCLUSION: Desmoid disease was found in 12.4 percent of our patients with familial adenomatous polyposis. In view of the high rate of morbidity, indication for surgery should be limited mainly to acute or chronic small-bowel obstruction, because resection triggers a high recurrence rate. Noncytotoxic therapy was not effective for progressive desmoid tumors, whereas chemotherapy was effective in aggressive cases of intra-abdominal desmoid tumors.

Fibromatoses: clinical and pathological features suggestive of recurrence.

BACKGROUND/PURPOSE: Fibromatoses represent a spectrum of nonneoplastic spindle cell tumors frequently identified in childhood. Although they may be locally aggressive, they do not metastasize. The authors have reviewed their experience of these lesions in an attempt to identify clinical and pathological features suggestive of recurrence. METHODS: Clinical records and pathological specimens from 55 cases of fibromatosis diagnosed and treated at British Columbia's Children's Hospital from 1982 to 1995 were reviewed. RESULTS: Thirty boys and 25 girls with ages ranging from 1 month to 14 years were identified. Eighteen tumors (33%) were congenital. Diagnoses were as follows: musculoaponeurotic fibromatosis (n = 27), infantile myofibromatosis (n = 10), Dupuytren-type fibromatosis (n = 7), fibromatosis colli (n = 7), and digital fibroma (n = 4). Clinical follow-up showed a survival rate of 98%. The single mortality occurred in an infant born with an intraabdominal visceral myofibromatosis, which proved unresectable. Fifteen cases recurred (27%), and nine cases (16%) of infantile myofibromatosis spontaneously regressed. All remaining cases were controlled successfully by surgical resection. CONCLUSIONS: Clinical features suggestive of recurrence included (1) presentation at greater than 5 years of age, (2) extremity location of disease, and (3) incomplete surgical resection. Pathological features suggestive of recurrence were (1) microscopic evidence of tumor at resection margins, (2) mitotic index of 5 or more per 10 high-power fields, and (3) areas of necrosis and inflammation within the tumor.

CT appearances of desmoid tumours in familial adenomatous polyposis: further observations.

Intra-abdominal desmoid tumours represent a major cause of morbidity and mortality in patients with familial adenomatous polyposis (FAP), and such patients are also liable to develop musculoskeletal desmoids. We have reviewed the CT appearances of 44 desmoid lesions (28 intra-abdominal and 16 musculoskeletal) in 20 patients with FAP. We found a considerable heterogeneity in the CT appearance of musculoskeletal and intraabdominal desmoids, with respect to their density, definition and change in size or density on follow-up, not only between different patients but also in patients with multiple lesions, who rarely showed identical appearances of all lesions. In some cases, mesenteric tumours may initially present as ill-defined soft tissue infiltration of mesenteric fat, becoming larger and more mass-like with time. On medical treatment, shrinkage was seen infrequently in musculoskeletal desmoids, and not at all with mesenteric lesions. CT evidence of bowel involvement by intra-abdominal lesions was frequent, most commonly appearing as 'tethering' or encasement of bowel loops. The presence of a large mesenteric mass (> 10 cm diam.), multiple mesenteric masses, extensive small bowel involvement and/or bilateral hydronephrosis were associated with ultimate death.