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Treatment and Follow-Up Strategies in Desmoid Tumours: A Practice Guideline

Treatment and Follow-Up Strategies in Desmoid Tumours: A Practice Guideline Curr Oncol, Vol. 21, pp. e642-649; doi: http://dx.doi.org/10.3747/co.21.2112 TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS P R AC T I C E G UID E L IN E Treatment and follow-up strategies in desmoid tumours: a practice guideline † ‡ M. Ghert md ms c,* X. Yao ms c, T. Corbett md , § || A.A. Gupta md ms c, R.A. Kandel md , S. Verma md , and J. Werier md ** • Depending on individual patient preferences, sys- ABSTRACT temic therapy alone or tr alone might also be rea- sonable treatment options, regardless of whether Objectives the desmoid tumours are deemed to be resectable. We set out to Follow-Up Strategies • determine the optimal treatment options—sur- • Undergo evaluation for rehabilitation (occupa- gery, radiation therapy (tr ), systemic therapy, tional therapy or physical therapy, or both). or any combinations thereof—for patients with • Continue with rehabilitation until maximal func- desmoid tumours once the decision to undergo tion is achieved. active treatment has been made (that is, monitor- • Undergo history and physical examinations with ing and observation have been determined to be appropriate imaging every 3–6 months for 2–3 inadequate). years, and then annually. • provide clinical-expert consensus opinions on follow-up strategies in patients with desmoid tu- KEY WORDS mours after primary interventional management. Clinical practice guideline, desmoid tumours, follow- Methods up, treatment This guideline was developed by Cancer Care On- 1. INTRODUCTION tario’s Program in Evidence-Based Care and the Sarcoma Disease Site Group. The mliedne , emeasb , Desmoid tumours, also known as aggressive fibro - and Cochrane Library databases, main guideline matoses, are rare neoplasms. The global incidence Web sites, and abstracts of relevant annual meetings of desmoid tumours is 2– 4 new cases per million (1990 to September 2012) were searched. Internal 1,2 population per year . Although desmoid t umours and external reviews were conducted, with final ap - are non-malignant and non-metastasizing, and proval by the Program in Evidence-Based Care and seldom cause death, they are locally invasive and the Sarcoma Disease Site Group. exhibit a high risk for recur rence . Generally, they are asymptomatic, but they can cause significant Recommendations local and neuropathic pain, can compress local structures, and might limit function. Some tu- Treatments mours can grow to a large size; others remain stable without intervention. Clinical observation • Surgery with or without tr can be a reasonable is therefore a preferable management option in treatment option for patients with desmoid tu- patients without symptoms. mours whose surgical morbidity is deemed to Several treatments are available for patients with be low. desmoid tumour when the decision has been made • The decision about whether tr should be offered to pursue active (non-observational) treatment such in conjunction with surgery should be made by as surgery, radiotherapy (tr ), systemic therapy, or a clinicians and patients after weighing the poten- combination of those options. However, there is little tial benet fi of improved local control against the consensus about which treatment strategy leads to potential harms and toxicity associated with tr . Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e642 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. a lower recurrence rate and less long-term toxicity. 3.3 External Review Thus, the Sarcoma Disease Site Group (gds ), in as- sociation with the Program in Evidence-Based Care The epbc external review process is two-pronged: (ecbp ) of Cancer Care Ontario, decided to develop a targeted peer review obtains direct feedback on a clinical guideline for Ontario addressing two re- the draft report from a small number of specified search questions. content experts, and a professional consultation facilitates dissemination of the final guidance report 2. QUESTIONS to Ontario practitioners. • When the decision has been made to pursue active 3.3.1 Targeted Peer Review treatment for a patient with desmoid tumour, what During the guideline development process, the is the optimal treatment option—considering sur- Sarcoma gds identie fi d 9 targeted international peer gery, tr , systemic therapy, and any combinations reviewers considered to be clinical or methodology thereof—for improving clinical outcomes (that experts on the topic. Several weeks before comple- is, rates of relapse-free survival, local control, tion of the draft report, the nominees were contacted progression-free survival, response, and toxicity, by e-mail and asked to serve as reviewers. The draft and patient-reported outcomes, among others)? report and a questionnaire were sent by e-mail to • After primary treatment, what are reasonable follow- the 5 reviewers who consented to participate. The up strategies for patients with desmoid tumours? questionnaire consisted of items evaluating the methods, results, and interpretive summary used 3. METHODS to inform the draft recommendations and whether the draft recommendations should be approved as a This guideline, developed by Cancer Care Ontario’s guideline. Written comments were invited. Follow- cbep and the Sarcoma gsd , used the methods of the up reminders were sent at 2 weeks (e-mail) and at 4 practice guidelines development cycle . For this weeks (telephone call). project, the core methodology used to develop the evidentiary base was the systematic review. The cbep 3.3.2 Professional Consultation is mandated to post its approved practice guidelines on Feedback was obtained through a brief online survey the Cancer Care Ontario Web site (http://www.cancer of health care professionals who are the intended care.on.ca/) for dissemination to Ontario oncologists . users of the guideline in Ontario. Clinicians in the cbep database who were identie fi d using the key word 3.1 Literature Search “sarcoma” (n = 51) were asked to rate the overall quality of the guideline and to indicate whether they For the second research question of this guide- would use or recommend it. Written comments were line, the authors agreed at the project planning invited. Participants were contacted by e-mail and stage that few or no original studies in the lit- directed to the survey Web site, where they were erature compared various follow-up strategies or provided with access to the survey, the guideline intervals, and that the Sarcoma dsg would make recommendations, and the evidentiary base. final recommendations based on existing clinical practice guidelines and the clinical experience of 4. RESULTS experts in Ontario. The systematic review for the first research question is published separately . 4.1 Literature Search Results Briefly, the medli n e and em base databases, and the Cochrane Library (January 1990 to Septem- For the first research question, 3791 citations were ber 2012); guideline Web sites; and the American identie fi d in the searches of mliedne , emeasb , and Society of Clinical Oncology and Connective Tis- the Cochrane Central Register of Controlled Trials. sue Oncology Society annual meeting abstracts A search of abstracts from the American Society of (Januar y 2009 to September 2012) were searched. Clinical Oncology and the Connective Tissue Oncol- Preplanned study selection criteria were used to ogy Society annual meetings yielded no abstracts that screen the literature retrieved. met the study selection criteria. The reference lists of the included articles were hand-searched, and no 3.2 Internal Review further eligible papers were found. The quality of the 6–51 evidence in the forty-six full-text articles and one Before this draft report was submitted for external systematic review that met the preplanned study review, it was reviewed and approved by the Sarcoma selection criteria was poor to moderate . gsd members and by the cebp Report Approval Panel For the second research question, one consensus (rap ), which has a membership of three: two oncolo- guideline was identified. That guideline— Soft Tis- gists with expertise in clinical and methodology is- sue Sarcoma, from the U.S. National Comprehen- sues and one methodologist. sive Cancer Network (version 2.2012) —provided Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e643 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS recommendations on follow-up strategies in patients feedback survey. Main concerns expressed in the with desmoid tumours. The quality of the guideline written comments were these: was assessed using the gar ee ii instrument (Ta- ble i ) . • We must be careful in emphasizing even “mi- croscopically negative margins” for not a me- 4.2 Internal Review tastasizing tumour. At times surgery is indicated to palliate, knowing that positive margins will The draft guideline prepared by the authors was cir- result. This is acceptable when decided about by culated to the Sarcoma gds members for review and an experienced sarcoma board. discussion. The authors incorporated the comments • Under Introduction in section 2, desmoids are from the gds members into the draft guideline and rarely truly asymptomatic and most patients forwarded the resulting document to the ecbp ’s rap . find them at least annoying. Experts understand The following key issues were raised by the rap : the subtlety, but given that these are guidelines for potentially less experienced providers, it • The reference for “wide margin” appears only in might be better to clarify that unless the mass is the recommendation. It is not referred to in the growing, causing true pain, or easy to remove, introduction, evidence, or discussion. observation is not only acceptable but may be • Is overall survival not an important outcome? preferable, even if mildly “symptomatic.” • Studies that compare surgery with surgery plus • Combinations of a nonsteroidal anti-ina fl mmato - tr , tr with surgery, and tr with surgery plus tr ry drug (often sulindac) and tamoxifen are com- are addressing different questions. monly used as primary treatment of desmoids. • Where does drug treatment t fi in sequentially? Is the evidence entirely anecdotal (that is, the Is it always after surgery and tr having failed, studies do not meet the criteria for inclusion in the or are there patients for whom drug treatment guideline)? If so, it may be important to mention would be a primary option? this. Similarly for the use of doxorubicin (and • There may be improvement in local regional liposomal forms) and/or dacarbazine. control. Is this a good enough outcome? • Some places are not very clear. In bullet 2 under Key Evidence, “Goy et al. showed a similar 4.3 Consensus Process result” is confusing (intended to mean similar results to Spear et al., but could be read as not Feedback received from the rap was addressed by the difference between groups); on page 3, the second authors . On July 9, 2013, the revised guideline was paragraph under Justic fi ation for Recommenda - sent to the Sarcoma gds members for final approval. tion does not seem to fit there. Of the 13 members of the Sarcoma gds , 10 cast votes and 3 abstained (77% response rate). Of the 10 who 4.4.2 Professional Consultation cast votes, all approved the document (100%). The notic fi ation e-mail was sent on September 5, 2013, and the consultation period ended on October 17, 2013. 4.4 External Review Among the 14 responders (27%), 7 indicated that they had no interest in this area or were currently unavail- After approval of the document at the internal review, able to review the guideline. Table iii summarizes the the authors circulated the draft document to external key results of the feedback survey from the other 7 review participants on September 5, 2013, for review clinicians. The main written comments were these: and feedback. • One shortcoming: there is no definition of a 4.4.1 Targeted Peer Review “microscopically negative margin.” Responses were received from 4 reviewers by Octo- • Who would be recommended to follow up the ber 17, 2013. Table ii summarizes key results of the patients? altbe i Results of ereg a ii quality rating for the U.S. National Comprehensive Cancer Network guideline on soft tissue sarcoma, version 2.2012 graee ii domain score (%) Scope and Stakeholder Rigour Clarity and Applicability Editorial purpose involvement of development presentation independence 64.8 40.7 25.7 87.0 22.2 61.1 Adopted from the Standards and Guidelines Evidence Inventory of Cancer Guidelines developed by the Canadian Partnership Against Cancer (http://cancerguidelines.ca/Guidelines/inventory/index.php). Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e644 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. altbe ii Responses to eight items on the targeted peer reviewer questionnaire Item Reviewer ratings (n=4) Lowest Highest quality quality 1 2 3 4 5 Rate the guideline development methods. 0 0 0 0 4 Rate the guideline presentation. 0 0 0 1 3 Rate the guideline recommendations. 0 0 0 2 2 Rate the completeness of reporting. 0 0 0 1 3 Does this document provide sufc fi ient information to inform your decisions? 0 0 1 2 1 If not, what areas are missing? Rate the overall quality of the guideline report. 0 0 0 3 1 Strongly Strongly disagree agree 1 2 3 4 5 I would make use of this guideline in my professional decisions. 0 0 1 1 2 I would recommend this guideline for use in practice. 0 0 0 2 2 altbe iii Responses to three items on the professional consultation survey Item Ratings Lowest Highest quality quality 1 2 3 4 5 Rate the overall quality of the guideline report. 0 0 14 57 29 Strongly Strongly disagree agree 1 2 3 4 5 I would make use of this guideline in my professional decisions. 0 14 0 43 43 I would recommend this guideline for use in practice. 0 0 0 43 57 clinicians and patients after weighing the poten- 5. PRACTICE GUIDELINE tial benet fi of improved local control against the potential harms and toxicity associated with tr . The present report integrates the feedback obtained • Depending on individual patient preferences, through the external review process with the final systemic therapy alone or tr alone might also approval given by the Sarcoma gsd and the cbep rap . be reasonable treatment options, regardless of whether the desmoid tumours are deemed to 5.1 Recommendation 1: Optimal Treatment Options be resectable. • Surgery with or without tr can be a reasonable 5.1.1 Qualifying Statements treatment option for patients with desmoid tu- mours whose surgical morbidity is deemed to • Given the variability of the clinical course of des- be low. moid tumours and the potential for complications • The decision about whether tr should be offered that can arise as a result of therapy, the cases of in conjunction with surgery should be made by Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e645 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS all patients with desmoid tumours who will un- found no statistically signic fi ant differences in local dergo active treatment should be discussed by an control between the two groups at 4 or 10 years. The experienced multidisciplinary sarcoma team, and main complications of surgery included the need for the treatment plan should take into consideration reconstructive surgery, above-the-knee amputation, patient preferences. permanent disability, and chronic pain. The main • Negative margin status (defined as surgical re - radiation-related complications included healing section with microscopically negative margins) problems, fibrosis, fracture, cellulitis, and secondary should be achieved, if possible, for a patient who malignancy, among others. is managed surgically. In the v fi e studies that conducted a multivariate 10,11,18 • Young patients might have a higher risk for analysis, three included margin status in the local relapse. model, and all three showed that positive margin • The optimal dose of tr , used as a surgical adju- status led to a worse rate of local control. Four stud- vant or as primary therapy, has not been defined. ies indicated that younger age (≤30 years in three 12,18,23 11 Reported radiation doses have ranged from 10 Gy studies , ≤18 in one study ) was predictive of to 75 Gy for tr used alone, and from 9 Gy to a higher local failure rate. 72 Gy for tr used as an adjuvant to surgery. Com- In three single-arm phase ii studies, imatinib alone plication rates have been reported to increase led to a progression-free survival rate of 58% at 3 12 24 signic fi antly with doses exceeding 56 Gy . years and 55% at 2 years, with some grade 3–4 tox- • Imatinib and the cytotoxic combination of vin- icities including rash, neutropenia, myalgia, asthenia, blastine and methotrexate are associated with or a secondary cancer (clear cell renal carcinoma) ; manageable toxicities. Results are considered and methotrexate plus vinblastine led to a progression- reasonable enough to merit discussion as an free survival rate of 67% at 10 years, with 93% of option for previously untreated patients or after patients developing grade 3 or 4 leukopenia . failure of surgery or tr (or both). However, other combinations of nonsteroidal anti-inflammatory 5.1.3 Justification for Recommendation 1 drugs (often sulindac); tamoxifen or doxorubicin Despite the large number of publications reporting (and its liposomal forms), with or without da- outcomes in patients treated for desmoid tumours, carbazine; and dacarbazine alone have, among the data are of poor-to-moderate quality, and no other agents, occasionally been used as primary studies with a multivariate analysis compared the treatment of desmoids in some clinical centres. effectiveness of surgery with that of chemotherapy Because of small patient numbers, studies of or the effectiveness of tr compared with that of che- these latter chemotherapy options did not meet motherapy. Because of the absence of good-quality our criteria for inclusion in the guideline. evidence, little consensus has developed among clinicians about the optimal treatment for patients with desmoid tumours. Because desmoid tumours 5.1.2 Key Evidence are non-malignant and non-metastasizing, and sel- Of v fi e retrospective comparative studies that con - 10 –12,18,23 dom cause death , local relapse is the main concern. ducted a multivariate analysis , one did not Surgery, tr , systemic therapy, and the combination of find a signic fi ant difference in patient characteristics surgery and tr have all shown moderate success in at baseline . Three single-arm phase ii studies of 16,24,29 achieving local control in these patients. Therefore, systemic therapy serve as the primary evidence in the authors’ judgment, all of these modalities can base for the recommendations. be considered reasonable options, and the decision Spear et al. reported that, compared with surgery to use one modality over another should be made alone, surgery plus tr led to a higher local control in conjunction with experts on a multidisciplinary rate at 5 years (72% vs. 69%, p = 0.03). At 6 years in sarcoma team. Given the trade-offs between the pos- patients with microscopically positive margins, Goy et sible benet fi s and the potential harms of the various al. reported results similar to those of the Spear et al. treatment options, patients should, as a part of the study (78% vs. 32%, p = 0.02). Ballo et al. reported treatment decision-making process, be informed of that, compared with surgery alone, surgery plus tr or the absence of definitive data favouring a particular tr alone both led to a higher local control rate at 10 type of treatment plan, and patient preferences should years (75% vs. 76% vs. 62%, p = 0.04). be taken into consideration. In a comparison of surgery plus tr with surgery alone for patients with primary desmoid tumours, 5.2 Recommendation 2: Optimal Follow-Up Sorensen et al. did not find a signic fi ant difference Strategies at 5 years in the rate of relapse-free survival (78% vs. 69%, p = 0.10) or of local control (82% vs. 68%, Cancer Care Ontario supports adoption of the follow-up p > 0.05). strategy recommendations of the National Comprehen- When surgery plus tr was compared with tr sive Cancer Network’s Soft Tissue Sarcoma guideline alone, Guadagnolo et al. (primary desmoid tumours) (version 2.2012). Specic fi ally, it is recommended that and Rϋdiger et al. (recurrent desmoid tumours) Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e646 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. patients with desmoid tumours who have received 9. CONFLICT OF INTEREST DISCLOSURES primary treatment in Ontario The ecbp is supported by the Ontario Ministry of • undergo evaluation for rehabilitation (occupa- Health and Long-Term Care through Cancer Care tional therapy or physical therapy, or both). Ontario. All work produced by the ecbp is editorially • continue with rehabilitation until maximal func- independent from its funding source. MG declared tion is achieved. that she has published a systematic review on local • undergo history and physical examinations with control in patients with extra-abdominal desmoid appropriate imaging every 3–6 months for 2–3 tumours in Rare Tumours . The remaining authors years, and then annually. declared they had no financial or professional con - flicts of interest. 5.2.1 Qualifying Statement If active treatment is not pursued, it is acceptable 10. REFERENCES for patients to be followed annually by a sarcoma surgeon or any other member of the sarcoma team. 1. Pakos EE, Tsekeris PG, Goussia AC. 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[Current ver- Based Care, Department of Oncology, McMaster sion available online at: http://www.nccn.org/professionals/ University, Hamilton, ON. physician_gls/pdf/sarcoma.pdf (free registration required); D epartment of Radiation Oncology, Juravinski cited June 15, 2014] Cancer Centre, Hamilton, ON. 54. Canadian Partnership Against Cancer (cacp ). Home > Treat- D ivision of Haematology/Oncology, Hospital for ment and support > Professionals > Clinical guidelines > Sick Children, Toronto, ON. || Guidelines resource centre. Hamilton, ON: cacp ; 2010. Department of Pathology and Laboratory Medi- [Available online at: http://cancerguidelines.ca/Guidelines/ cine, Mount Sinai Hospital, Toronto, ON. inventory/index.php; cited February 18, 2013] D epartment of Medical Oncology, The Ottawa 55. Wood TJ, Quinn KM, Farrokhyar F, Deheshi B, Corbett T, Hospital Regional Cancer Centre, Ottawa, ON. Ghert MA. Local control of extra-abdominal desmoid tumors: ** Department of Orthopaedic Surgery, The Ottawa systematic review and meta-analysis. Rare Tumors 2013;5:e2. Hospital Regional Cancer Centre, Ottawa, ON. Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e649 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Oncology Multidisciplinary Digital Publishing Institute

Treatment and Follow-Up Strategies in Desmoid Tumours: A Practice Guideline

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Curr Oncol, Vol. 21, pp. e642-649; doi: http://dx.doi.org/10.3747/co.21.2112 TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS P R AC T I C E G UID E L IN E Treatment and follow-up strategies in desmoid tumours: a practice guideline † ‡ M. Ghert md ms c,* X. Yao ms c, T. Corbett md , § || A.A. Gupta md ms c, R.A. Kandel md , S. Verma md , and J. Werier md ** • Depending on individual patient preferences, sys- ABSTRACT temic therapy alone or tr alone might also be rea- sonable treatment options, regardless of whether Objectives the desmoid tumours are deemed to be resectable. We set out to Follow-Up Strategies • determine the optimal treatment options—sur- • Undergo evaluation for rehabilitation (occupa- gery, radiation therapy (tr ), systemic therapy, tional therapy or physical therapy, or both). or any combinations thereof—for patients with • Continue with rehabilitation until maximal func- desmoid tumours once the decision to undergo tion is achieved. active treatment has been made (that is, monitor- • Undergo history and physical examinations with ing and observation have been determined to be appropriate imaging every 3–6 months for 2–3 inadequate). years, and then annually. • provide clinical-expert consensus opinions on follow-up strategies in patients with desmoid tu- KEY WORDS mours after primary interventional management. Clinical practice guideline, desmoid tumours, follow- Methods up, treatment This guideline was developed by Cancer Care On- 1. INTRODUCTION tario’s Program in Evidence-Based Care and the Sarcoma Disease Site Group. The mliedne , emeasb , Desmoid tumours, also known as aggressive fibro - and Cochrane Library databases, main guideline matoses, are rare neoplasms. The global incidence Web sites, and abstracts of relevant annual meetings of desmoid tumours is 2– 4 new cases per million (1990 to September 2012) were searched. Internal 1,2 population per year . Although desmoid t umours and external reviews were conducted, with final ap - are non-malignant and non-metastasizing, and proval by the Program in Evidence-Based Care and seldom cause death, they are locally invasive and the Sarcoma Disease Site Group. exhibit a high risk for recur rence . Generally, they are asymptomatic, but they can cause significant Recommendations local and neuropathic pain, can compress local structures, and might limit function. Some tu- Treatments mours can grow to a large size; others remain stable without intervention. Clinical observation • Surgery with or without tr can be a reasonable is therefore a preferable management option in treatment option for patients with desmoid tu- patients without symptoms. mours whose surgical morbidity is deemed to Several treatments are available for patients with be low. desmoid tumour when the decision has been made • The decision about whether tr should be offered to pursue active (non-observational) treatment such in conjunction with surgery should be made by as surgery, radiotherapy (tr ), systemic therapy, or a clinicians and patients after weighing the poten- combination of those options. However, there is little tial benet fi of improved local control against the consensus about which treatment strategy leads to potential harms and toxicity associated with tr . Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e642 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. a lower recurrence rate and less long-term toxicity. 3.3 External Review Thus, the Sarcoma Disease Site Group (gds ), in as- sociation with the Program in Evidence-Based Care The epbc external review process is two-pronged: (ecbp ) of Cancer Care Ontario, decided to develop a targeted peer review obtains direct feedback on a clinical guideline for Ontario addressing two re- the draft report from a small number of specified search questions. content experts, and a professional consultation facilitates dissemination of the final guidance report 2. QUESTIONS to Ontario practitioners. • When the decision has been made to pursue active 3.3.1 Targeted Peer Review treatment for a patient with desmoid tumour, what During the guideline development process, the is the optimal treatment option—considering sur- Sarcoma gds identie fi d 9 targeted international peer gery, tr , systemic therapy, and any combinations reviewers considered to be clinical or methodology thereof—for improving clinical outcomes (that experts on the topic. Several weeks before comple- is, rates of relapse-free survival, local control, tion of the draft report, the nominees were contacted progression-free survival, response, and toxicity, by e-mail and asked to serve as reviewers. The draft and patient-reported outcomes, among others)? report and a questionnaire were sent by e-mail to • After primary treatment, what are reasonable follow- the 5 reviewers who consented to participate. The up strategies for patients with desmoid tumours? questionnaire consisted of items evaluating the methods, results, and interpretive summary used 3. METHODS to inform the draft recommendations and whether the draft recommendations should be approved as a This guideline, developed by Cancer Care Ontario’s guideline. Written comments were invited. Follow- cbep and the Sarcoma gsd , used the methods of the up reminders were sent at 2 weeks (e-mail) and at 4 practice guidelines development cycle . For this weeks (telephone call). project, the core methodology used to develop the evidentiary base was the systematic review. The cbep 3.3.2 Professional Consultation is mandated to post its approved practice guidelines on Feedback was obtained through a brief online survey the Cancer Care Ontario Web site (http://www.cancer of health care professionals who are the intended care.on.ca/) for dissemination to Ontario oncologists . users of the guideline in Ontario. Clinicians in the cbep database who were identie fi d using the key word 3.1 Literature Search “sarcoma” (n = 51) were asked to rate the overall quality of the guideline and to indicate whether they For the second research question of this guide- would use or recommend it. Written comments were line, the authors agreed at the project planning invited. Participants were contacted by e-mail and stage that few or no original studies in the lit- directed to the survey Web site, where they were erature compared various follow-up strategies or provided with access to the survey, the guideline intervals, and that the Sarcoma dsg would make recommendations, and the evidentiary base. final recommendations based on existing clinical practice guidelines and the clinical experience of 4. RESULTS experts in Ontario. The systematic review for the first research question is published separately . 4.1 Literature Search Results Briefly, the medli n e and em base databases, and the Cochrane Library (January 1990 to Septem- For the first research question, 3791 citations were ber 2012); guideline Web sites; and the American identie fi d in the searches of mliedne , emeasb , and Society of Clinical Oncology and Connective Tis- the Cochrane Central Register of Controlled Trials. sue Oncology Society annual meeting abstracts A search of abstracts from the American Society of (Januar y 2009 to September 2012) were searched. Clinical Oncology and the Connective Tissue Oncol- Preplanned study selection criteria were used to ogy Society annual meetings yielded no abstracts that screen the literature retrieved. met the study selection criteria. The reference lists of the included articles were hand-searched, and no 3.2 Internal Review further eligible papers were found. The quality of the 6–51 evidence in the forty-six full-text articles and one Before this draft report was submitted for external systematic review that met the preplanned study review, it was reviewed and approved by the Sarcoma selection criteria was poor to moderate . gsd members and by the cebp Report Approval Panel For the second research question, one consensus (rap ), which has a membership of three: two oncolo- guideline was identified. That guideline— Soft Tis- gists with expertise in clinical and methodology is- sue Sarcoma, from the U.S. National Comprehen- sues and one methodologist. sive Cancer Network (version 2.2012) —provided Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e643 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS recommendations on follow-up strategies in patients feedback survey. Main concerns expressed in the with desmoid tumours. The quality of the guideline written comments were these: was assessed using the gar ee ii instrument (Ta- ble i ) . • We must be careful in emphasizing even “mi- croscopically negative margins” for not a me- 4.2 Internal Review tastasizing tumour. At times surgery is indicated to palliate, knowing that positive margins will The draft guideline prepared by the authors was cir- result. This is acceptable when decided about by culated to the Sarcoma gds members for review and an experienced sarcoma board. discussion. The authors incorporated the comments • Under Introduction in section 2, desmoids are from the gds members into the draft guideline and rarely truly asymptomatic and most patients forwarded the resulting document to the ecbp ’s rap . find them at least annoying. Experts understand The following key issues were raised by the rap : the subtlety, but given that these are guidelines for potentially less experienced providers, it • The reference for “wide margin” appears only in might be better to clarify that unless the mass is the recommendation. It is not referred to in the growing, causing true pain, or easy to remove, introduction, evidence, or discussion. observation is not only acceptable but may be • Is overall survival not an important outcome? preferable, even if mildly “symptomatic.” • Studies that compare surgery with surgery plus • Combinations of a nonsteroidal anti-ina fl mmato - tr , tr with surgery, and tr with surgery plus tr ry drug (often sulindac) and tamoxifen are com- are addressing different questions. monly used as primary treatment of desmoids. • Where does drug treatment t fi in sequentially? Is the evidence entirely anecdotal (that is, the Is it always after surgery and tr having failed, studies do not meet the criteria for inclusion in the or are there patients for whom drug treatment guideline)? If so, it may be important to mention would be a primary option? this. Similarly for the use of doxorubicin (and • There may be improvement in local regional liposomal forms) and/or dacarbazine. control. Is this a good enough outcome? • Some places are not very clear. In bullet 2 under Key Evidence, “Goy et al. showed a similar 4.3 Consensus Process result” is confusing (intended to mean similar results to Spear et al., but could be read as not Feedback received from the rap was addressed by the difference between groups); on page 3, the second authors . On July 9, 2013, the revised guideline was paragraph under Justic fi ation for Recommenda - sent to the Sarcoma gds members for final approval. tion does not seem to fit there. Of the 13 members of the Sarcoma gds , 10 cast votes and 3 abstained (77% response rate). Of the 10 who 4.4.2 Professional Consultation cast votes, all approved the document (100%). The notic fi ation e-mail was sent on September 5, 2013, and the consultation period ended on October 17, 2013. 4.4 External Review Among the 14 responders (27%), 7 indicated that they had no interest in this area or were currently unavail- After approval of the document at the internal review, able to review the guideline. Table iii summarizes the the authors circulated the draft document to external key results of the feedback survey from the other 7 review participants on September 5, 2013, for review clinicians. The main written comments were these: and feedback. • One shortcoming: there is no definition of a 4.4.1 Targeted Peer Review “microscopically negative margin.” Responses were received from 4 reviewers by Octo- • Who would be recommended to follow up the ber 17, 2013. Table ii summarizes key results of the patients? altbe i Results of ereg a ii quality rating for the U.S. National Comprehensive Cancer Network guideline on soft tissue sarcoma, version 2.2012 graee ii domain score (%) Scope and Stakeholder Rigour Clarity and Applicability Editorial purpose involvement of development presentation independence 64.8 40.7 25.7 87.0 22.2 61.1 Adopted from the Standards and Guidelines Evidence Inventory of Cancer Guidelines developed by the Canadian Partnership Against Cancer (http://cancerguidelines.ca/Guidelines/inventory/index.php). Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e644 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. altbe ii Responses to eight items on the targeted peer reviewer questionnaire Item Reviewer ratings (n=4) Lowest Highest quality quality 1 2 3 4 5 Rate the guideline development methods. 0 0 0 0 4 Rate the guideline presentation. 0 0 0 1 3 Rate the guideline recommendations. 0 0 0 2 2 Rate the completeness of reporting. 0 0 0 1 3 Does this document provide sufc fi ient information to inform your decisions? 0 0 1 2 1 If not, what areas are missing? Rate the overall quality of the guideline report. 0 0 0 3 1 Strongly Strongly disagree agree 1 2 3 4 5 I would make use of this guideline in my professional decisions. 0 0 1 1 2 I would recommend this guideline for use in practice. 0 0 0 2 2 altbe iii Responses to three items on the professional consultation survey Item Ratings Lowest Highest quality quality 1 2 3 4 5 Rate the overall quality of the guideline report. 0 0 14 57 29 Strongly Strongly disagree agree 1 2 3 4 5 I would make use of this guideline in my professional decisions. 0 14 0 43 43 I would recommend this guideline for use in practice. 0 0 0 43 57 clinicians and patients after weighing the poten- 5. PRACTICE GUIDELINE tial benet fi of improved local control against the potential harms and toxicity associated with tr . The present report integrates the feedback obtained • Depending on individual patient preferences, through the external review process with the final systemic therapy alone or tr alone might also approval given by the Sarcoma gsd and the cbep rap . be reasonable treatment options, regardless of whether the desmoid tumours are deemed to 5.1 Recommendation 1: Optimal Treatment Options be resectable. • Surgery with or without tr can be a reasonable 5.1.1 Qualifying Statements treatment option for patients with desmoid tu- mours whose surgical morbidity is deemed to • Given the variability of the clinical course of des- be low. moid tumours and the potential for complications • The decision about whether tr should be offered that can arise as a result of therapy, the cases of in conjunction with surgery should be made by Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e645 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). TREATMENT AND FOLLOW-UP STRATEGIES IN DESMOID TUMOURS all patients with desmoid tumours who will un- found no statistically signic fi ant differences in local dergo active treatment should be discussed by an control between the two groups at 4 or 10 years. The experienced multidisciplinary sarcoma team, and main complications of surgery included the need for the treatment plan should take into consideration reconstructive surgery, above-the-knee amputation, patient preferences. permanent disability, and chronic pain. The main • Negative margin status (defined as surgical re - radiation-related complications included healing section with microscopically negative margins) problems, fibrosis, fracture, cellulitis, and secondary should be achieved, if possible, for a patient who malignancy, among others. is managed surgically. In the v fi e studies that conducted a multivariate 10,11,18 • Young patients might have a higher risk for analysis, three included margin status in the local relapse. model, and all three showed that positive margin • The optimal dose of tr , used as a surgical adju- status led to a worse rate of local control. Four stud- vant or as primary therapy, has not been defined. ies indicated that younger age (≤30 years in three 12,18,23 11 Reported radiation doses have ranged from 10 Gy studies , ≤18 in one study ) was predictive of to 75 Gy for tr used alone, and from 9 Gy to a higher local failure rate. 72 Gy for tr used as an adjuvant to surgery. Com- In three single-arm phase ii studies, imatinib alone plication rates have been reported to increase led to a progression-free survival rate of 58% at 3 12 24 signic fi antly with doses exceeding 56 Gy . years and 55% at 2 years, with some grade 3–4 tox- • Imatinib and the cytotoxic combination of vin- icities including rash, neutropenia, myalgia, asthenia, blastine and methotrexate are associated with or a secondary cancer (clear cell renal carcinoma) ; manageable toxicities. Results are considered and methotrexate plus vinblastine led to a progression- reasonable enough to merit discussion as an free survival rate of 67% at 10 years, with 93% of option for previously untreated patients or after patients developing grade 3 or 4 leukopenia . failure of surgery or tr (or both). However, other combinations of nonsteroidal anti-inflammatory 5.1.3 Justification for Recommendation 1 drugs (often sulindac); tamoxifen or doxorubicin Despite the large number of publications reporting (and its liposomal forms), with or without da- outcomes in patients treated for desmoid tumours, carbazine; and dacarbazine alone have, among the data are of poor-to-moderate quality, and no other agents, occasionally been used as primary studies with a multivariate analysis compared the treatment of desmoids in some clinical centres. effectiveness of surgery with that of chemotherapy Because of small patient numbers, studies of or the effectiveness of tr compared with that of che- these latter chemotherapy options did not meet motherapy. Because of the absence of good-quality our criteria for inclusion in the guideline. evidence, little consensus has developed among clinicians about the optimal treatment for patients with desmoid tumours. Because desmoid tumours 5.1.2 Key Evidence are non-malignant and non-metastasizing, and sel- Of v fi e retrospective comparative studies that con - 10 –12,18,23 dom cause death , local relapse is the main concern. ducted a multivariate analysis , one did not Surgery, tr , systemic therapy, and the combination of find a signic fi ant difference in patient characteristics surgery and tr have all shown moderate success in at baseline . Three single-arm phase ii studies of 16,24,29 achieving local control in these patients. Therefore, systemic therapy serve as the primary evidence in the authors’ judgment, all of these modalities can base for the recommendations. be considered reasonable options, and the decision Spear et al. reported that, compared with surgery to use one modality over another should be made alone, surgery plus tr led to a higher local control in conjunction with experts on a multidisciplinary rate at 5 years (72% vs. 69%, p = 0.03). At 6 years in sarcoma team. Given the trade-offs between the pos- patients with microscopically positive margins, Goy et sible benet fi s and the potential harms of the various al. reported results similar to those of the Spear et al. treatment options, patients should, as a part of the study (78% vs. 32%, p = 0.02). Ballo et al. reported treatment decision-making process, be informed of that, compared with surgery alone, surgery plus tr or the absence of definitive data favouring a particular tr alone both led to a higher local control rate at 10 type of treatment plan, and patient preferences should years (75% vs. 76% vs. 62%, p = 0.04). be taken into consideration. In a comparison of surgery plus tr with surgery alone for patients with primary desmoid tumours, 5.2 Recommendation 2: Optimal Follow-Up Sorensen et al. did not find a signic fi ant difference Strategies at 5 years in the rate of relapse-free survival (78% vs. 69%, p = 0.10) or of local control (82% vs. 68%, Cancer Care Ontario supports adoption of the follow-up p > 0.05). strategy recommendations of the National Comprehen- When surgery plus tr was compared with tr sive Cancer Network’s Soft Tissue Sarcoma guideline alone, Guadagnolo et al. (primary desmoid tumours) (version 2.2012). Specic fi ally, it is recommended that and Rϋdiger et al. (recurrent desmoid tumours) Current OnCOlOgy —VOlume 21, n umber 4, August 2014 e646 Copyright © 2014 Multimed Inc. Following publication in Current Oncology, the full text of each article is available immediately and archived in PubMed Central (PMC). GHERT et al. patients with desmoid tumours who have received 9. CONFLICT OF INTEREST DISCLOSURES primary treatment in Ontario The ecbp is supported by the Ontario Ministry of • undergo evaluation for rehabilitation (occupa- Health and Long-Term Care through Cancer Care tional therapy or physical therapy, or both). Ontario. All work produced by the ecbp is editorially • continue with rehabilitation until maximal func- independent from its funding source. MG declared tion is achieved. that she has published a systematic review on local • undergo history and physical examinations with control in patients with extra-abdominal desmoid appropriate imaging every 3–6 months for 2–3 tumours in Rare Tumours . The remaining authors years, and then annually. declared they had no financial or professional con - flicts of interest. 5.2.1 Qualifying Statement If active treatment is not pursued, it is acceptable 10. REFERENCES for patients to be followed annually by a sarcoma surgeon or any other member of the sarcoma team. 1. Pakos EE, Tsekeris PG, Goussia AC. 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Journal

Current OncologyMultidisciplinary Digital Publishing Institute

Published: Aug 1, 2014

Keywords: clinical practice guideline; desmoid tumours; follow-up; treatment

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