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An Important Role for Initial Observation in the Management of Paediatric Desmoid Fibromatosis
Clinical Oncology 25 (2013) e16ee16 Contents lists available at SciVerse ScienceDirect
Clinical Oncology journal homepage: www.clinicaloncologyonline.net
Letter
An Important Role for Initial Observation in the Management of Paediatric Desmoid Fibromatosis Sir d The management of desmoid fibromatosis has evolved over the last decade. Historically, primary surgery was the treatment of choice, whereas initial observation or less aggressive therapies are now considered. This tumour may stabilise, making observation reasonable. Furthermore, high postsurgical recurrence rates have been reported, even after apparent complete excision [1]. Aggressive surgery causing mutilation or functional impairment is concerning in the paediatric population. We retrospectively studied 28 cases of desmoid fibromatosis (age < 21 years, treated 2003e2010). Of 23 cases undergoing surgery, only 4 had complete resection (histological margin > 1 mm) and 3 of these recurred (median 13, range 4e14 months). Four of 19 cases with intralesional/ unknown margins, and no residual disease on imaging after surgery, remain in remission (median 50, range 3e70 months). Four cases underwent initial observation. Although 3 progressed, this was at a median of 11 months (range 4e17 months). Three of 8 cases treated with single modality hormonal or non-steroidal anti-inflammatory drugs progressed (median 12, range 7e12 months), with 5 remaining stable (median 8, range 4e108 months). In comparison, 4 of 10 children who received chemotherapy alone progressed (median 29, range 26e85 months), with 6 remaining stable (median 35, range 2e80 months). After radiotherapy, stable disease or response was seen in 8 of 11 children (median 51.5, range 12e102 months). As with previous reports, our data in this small series shows a high recurrence rate after surgery even after
complete resection. Although radiotherapy provides good control, use is often delayed in children due to associated morbidities. In our practice, an initial trial of observation or treatment with non-cytotoxic therapies has provided disease stabilisation in a subset of patients. Furthermore, regular clinical review and imaging are safe, even in this age group, in which a more aggressive course has been documented [2]. Our institution treatment algorithm for paediatric desmoid fibromatosis includes initial observation when asymptomatic and the risk of encroachment on critical structures is low. H. Holme*, C.B. Westbury*, D. Morgenstern*, G. Erturany, R. Tiraboscoz, R. Pollockx, T. Briggsx, A. Cassoni*, M. Michelagnoli* *The London Sarcoma Service, University College London Hospital, London, UK yNuffield Orthopaedic Centre, Headington, Oxford, UK zDepartment of Pathology, Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK xThe London Sarcoma Service, Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK
References [1] Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer 1999;86(10):2045e2052. [2] Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN. Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 2000;47(5): 1267e1271.
0936-6555/$36.00 Ó 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.clon.2012.09.006
Clinical Oncology journal homepage: www.clinicaloncologyonline.net
Letter
An Important Role for Initial Observation in the Management of Paediatric Desmoid Fibromatosis Sir d The management of desmoid fibromatosis has evolved over the last decade. Historically, primary surgery was the treatment of choice, whereas initial observation or less aggressive therapies are now considered. This tumour may stabilise, making observation reasonable. Furthermore, high postsurgical recurrence rates have been reported, even after apparent complete excision [1]. Aggressive surgery causing mutilation or functional impairment is concerning in the paediatric population. We retrospectively studied 28 cases of desmoid fibromatosis (age < 21 years, treated 2003e2010). Of 23 cases undergoing surgery, only 4 had complete resection (histological margin > 1 mm) and 3 of these recurred (median 13, range 4e14 months). Four of 19 cases with intralesional/ unknown margins, and no residual disease on imaging after surgery, remain in remission (median 50, range 3e70 months). Four cases underwent initial observation. Although 3 progressed, this was at a median of 11 months (range 4e17 months). Three of 8 cases treated with single modality hormonal or non-steroidal anti-inflammatory drugs progressed (median 12, range 7e12 months), with 5 remaining stable (median 8, range 4e108 months). In comparison, 4 of 10 children who received chemotherapy alone progressed (median 29, range 26e85 months), with 6 remaining stable (median 35, range 2e80 months). After radiotherapy, stable disease or response was seen in 8 of 11 children (median 51.5, range 12e102 months). As with previous reports, our data in this small series shows a high recurrence rate after surgery even after
complete resection. Although radiotherapy provides good control, use is often delayed in children due to associated morbidities. In our practice, an initial trial of observation or treatment with non-cytotoxic therapies has provided disease stabilisation in a subset of patients. Furthermore, regular clinical review and imaging are safe, even in this age group, in which a more aggressive course has been documented [2]. Our institution treatment algorithm for paediatric desmoid fibromatosis includes initial observation when asymptomatic and the risk of encroachment on critical structures is low. H. Holme*, C.B. Westbury*, D. Morgenstern*, G. Erturany, R. Tiraboscoz, R. Pollockx, T. Briggsx, A. Cassoni*, M. Michelagnoli* *The London Sarcoma Service, University College London Hospital, London, UK yNuffield Orthopaedic Centre, Headington, Oxford, UK zDepartment of Pathology, Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK xThe London Sarcoma Service, Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK
References [1] Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer 1999;86(10):2045e2052. [2] Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN. Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 2000;47(5): 1267e1271.
0936-6555/$36.00 Ó 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.clon.2012.09.006