Extra-abdominal desmoid tumours – further evidence for the watchful waiting policy (original) (raw)
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Acta orthopaedica et traumatologica turcica
We investigated treatment results and the role of potential prognostic factors in patients treated by surgery with or without adjuvant radiotherapy for primary or recurrent extra-abdominal desmoid tumors. The study included 38 patients (23 females, 15 males; mean age 24 years; range 5 to 61 years) who underwent surgical treatment for extra-abdominal desmoid tumors. Of these, eight patients (21.1%) already had recurrences before treatment. Involvement was in the upper extremity in 12 cases (31.6%), in the lower extremity in 22 cases (57.9%), and in the axial region in four cases (10.5%). Twenty-two patients received adjuvant radiotherapy following surgical resection. Survival was analyzed by the Kaplan-Meier method. The mean follow-up period was 7.3 years (2.5 to 228 months). Twenty patients (52.6%) developed recurrences after treatment. Of these, recurrences were already present in six patients, and adjuvant radiotherapy was administered to 11 patients (55%). Recurrences developed a...
Local control of extra-abdominal desmoid tumors: systematic review and meta-analysis
Rare Tumors, 2013
The local control of desmoid tumors constitutes a continuing treatment dilemma due to its high recurrence rates. The purpose of this systematic review was to critically examine the current treatment of these rare tumors and to specifically evaluate the local failure and response rates of surgery, radiation and systemic therapy. We comprehensively searched the literature for relevant studies across Cinahl, Embase, Medline and the Cochrane databases. Articles were categorized as surgery, radiation, surgery + radiation and systemic therapy (including cytotoxic and non cytotoxic). Methodological quality of included studies was assessed using the Newcastle-Ottawa Scale. Pooled odd ratios (OR) for comparative studies and weighted proportions with 95% confidence intervals (CI) are reported. Thirty-five articles were included in the final analysis. Weighted mean local failure rates were 22% [95% CI (16-28%)], 35% [95% CI (26-44%)] and 28% [95% CI (18-39%)] for radiation alone, surgery alone and surgery + radiation respectively. In the analysis of comparative studies, surgery and radiation in combination had lower local failure rates than radiation alone [OR 0.7 (0.4,2)] and surgery alone [OR 0.7 (0.4, 1.0)]. Weighted mean stable disease rates were 91% [95% CI (85-96%)] and 52% [95% CI (38-65%)] for non cytotoxic and cytotoxic chemotherapy respectively.
South African Journal of Oncology
Background: Desmoid tumours (DT) are rare soft tissue tumours that do not metastasise but are locally aggressive. Management options are varied and the response to treatment can be unpredictable. Aim: The aim of this study was to describe the clinical presentation, management strategies and outcomes for adult patients who were treated for DT. Setting: The study was conducted at Groote Schuur Hospital in Cape Town, South Africa, and all patients from 2003 to 2016 who presented with DT were included. Method: This was a retrospective review of records. Data collected included: demographics, DT-associated conditions, site and size of tumour, histological findings, treatment modalities, follow-up and outcomes. Results: Seventy patients with histologically confirmed DT were identified. The majority were women (86%) and 77% presented with a painless mass. The commonest site was the anterior abdominal wall (47%). Definitive surgery was performed in 46 (66%) patients, whereas 13 (19%) had definitive radiotherapy. Nine patients received adjuvant radiotherapy postsurgery for involved or close margins. Recurrence developed in 20% of patients post-surgery. In the primary radiotherapy group, one patient had disease progression. Two patients with mesenteric DT died because of bowel obstruction. Conclusion: This retrospective review of patients affected by DT at a single centre demonstrates the rarity of the condition, the unpredictable natural history and the variety of treatment options available. Many of our findings are similar to other published studies, except the mean size of DT which was bigger. Treatment outcomes following surgery or radiotherapy seem acceptable, although study limitations are noted.
Annals of Surgical Oncology, 2019
Background. The behavior of desmoid tumors is unpredictable and varies from spontaneous remission to symptomatic and radiologic progression. This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance. Methods. Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom. Inverse univariate Cox proportional hazard regression analyses were conducted to evaluate the course of the disease and indications for initiating treatment. Results. The study identified 168 patients with a primary desmoid tumor initially managed with active surveillance. The tumors were located in the abdominal wall (n = 61, 36%), an extremity (n = 51, 30%), chest wall (n = 30, 18%), intra-abdominal site (n = 15, 9%), or elsewhere (n = 11, 6%). Of all the patients, 36% experienced radiologic progressive disease, 36% had stable disease, and 27%
A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases
European Journal of Surgical Oncology (EJSO), 2009
Background: The management of desmoid tumours, previously based on strategies employed for sarcomas, should be reassessed, given the morbidity of interventions used in their treatment. Methods: Long-term follow-up (median 123 months) of a series of 106 treated patients with 69 primary and 37 recurrent desmoids, in order to study natural history and outcome. Results: Desmoids typically evolved actively over a median period of 3 years, and stabilised thereafter. Recurrences or progression most commonly occurred between 14 and 17 months. Risk factors for recurrence were presentation (primary vs. recurrent), gender, tumour location and resection margins. However, survival was independent from these factors, with equivalent survival whether resection had been performed or not. Tumour control and functional outcome depended on location and presentation. Functional impairment was proportional to number of operations and whether patients had received radiotherapy. Recurrences were observed in 12/23 patients after radiotherapy. Conclusion: Desmoids are relatively indolent tumours needing different approaches than sarcomas. Direct surgery is advisable only in primary lower trunk wall/girdle locations. Wait-and-see and medical treatment is preferable in other types of presentations.
Balkan Medical Journal, 2016
Background: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. Aims: To report our institutional experience and longterm results of patients with desmoid tumors who received radiotherapy. Study Design: Retrospective cross-sectional study. Methods: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. Results: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. Conclusion: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.
2021
Background This study compared the clinical and functional outcomes of patients initially treated with observation or medical treatment with those of patients treated with local treatment (surgery alone or surgery with adjuvant radiotherapy) to confirm whether observation or medical treatment is an appropriate first-line management approach for patients with desmoid tumors. Methods We retrospectively reviewed the medical records of 99 patients with histologically confirmed primary desmoid tumors treated between 1978 and 2018. The median follow-up period was 57 months. We evaluated event-free survival, defined as the time interval from the date of initial diagnosis to the date of specific change in treatment strategy or recurrence or the last follow-up. Results An event (specific change in treatment strategy or recurrence) occurred in 28 patients (28.3%). No significant difference in event-free survival was found between the first-line observation/medical treatment and local treatmen...
The management of desmoid tumors
International Journal of Radiation Oncology*Biology*Physics, 1993
Purpose: To determine the efficacy of different treatment modalities for desmoid tumors.-1s and Methods: We reviewed the treatment of 40 patients with histologically confirmed desmoid tumors seen at Duke University Medical Center between 1974 and 1990. Results: Radiotherapy was administered to 16 patients (Group I)-14 with recurrent disease s/p surgery and in-asinitial treatment. The average size of the irradiated lesions was 9.3 + 3.9 X 8.4 + 3.5 cm. With a median follow-up of 57.5 months and a median administered dose of 5400 cGy (mean 5286 cGy, range 4960-5620 cGy), local control has been obtained in 15/16 patients (94%). Complete regression (5/16), partial regression (5/16), or stable disease (5/16) was produced in 15 patients while one patient failed and was salvaged via gross total resection. Continued regression has been seen up to 60 months after treatment. Fourteen patients underwent primary gross total resection and two underwent subtotal resection (Group II). None received post-operative radiotherapy. Three of 14 patients (21%) recurred after gross total resection. All three were salvaged with subsequent gross total resection. After subtotal resection, 2/2 patients recurred. With a mean follow-up of 52 months, 14 patients are without evidence of disease, one is dead with disease (unrelated cause of death), and one was lost to follow-up after recurrence. Eight patients have been treated with combinations of chemotherapy, NSAIDS, anti-estrogens, and immunotherapy with mixed results (Group III). A subset of seven patients with retroperitoneal disease taken from all three groups had large tumor burden (mean size 17 X 15 cm), an infiltrative nature, as well as a difficult location. The disease was surgically resectable in three patients. One is without evidence of disease 9 years after gross total resection alone. Disease has been stabilized with radiotherapy in the other two patients after multiple unsuccessful surgical resections. Of four patients with unresectable disease, two are dead of disease, one died of unrelated causes with disease, and regression of disease was obtained in the other with Gamma-interferon after unsuccessful treatment with tamoxifen and vincristine, doxorubicin, and cyclophosphamide chemotherapy. Conclusion: Gross total resection is the indicated initial therapy, if it can be performed without significant disfigurement. Radiotherapy is also excellent for obtaining local control, even in patients with a large burden of recurrent disease. Doses in the range of 50 to 55 Gy give a chance of local control equal to that obtained with higher doses previously reported. Desmoid tumor, Fibromatosis, Radiotherapy.