Long-term results with radiation therapy for pediatric desmoid tumors

Merchant, Thomas E.; Nguyen, Duyen Thuy; Walter, Andrew W.; Pappo, Alberto S.; Kun, Larry E.; Rao, Bhaskar N.

doi:10.1016/s0360-3016(00)00566-6

Cited by 57 publications

(41 citation statements)

References 27 publications

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“…[9][10][11] Various alternative systemic strategies have been explored, including the use of chemotherapy and noncytotoxic agents (ie, hormone therapy, anti-inflammatory agents, interferon alfa [IFN-a]). [12][13][14][15] Other open questions relating to treatment strategy concern the possibility of a no-treatment approach for patients who have nonevolving disease and the search for prognostic indicators for the purpose of patient risk stratification.…”

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confidence: 99%

Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

132

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BACKGROUND:Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence. To date, there are no general recommendations for the clinical management of pediatric AF.METHODS:The authors retrospectively analyzed 94 patients aged ≤21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).RESULTS:The 5‐year event‐free survival (EFS) and overall survival rates were 44% and 99%, respectively. Local recurrences developed in 22% of patients in Group I, in 76% of patients in Group II, and in 76% of patients in Group III. Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra‐abdominal AF died of their disease. Systemic treatment was given to 15 patients as first‐line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients. Objective responses were observed in 11 of 19 patients who received combined methotrexate plus vinblastine/vinorelbine, in 7 of 15 patients who received alkylating‐agent chemotherapy, and in 4 of 11 patients who received other therapies (tamoxifen, sulindac, interferon alfa).CONCLUSIONS:The current analysis suggested that the clinical course of AF in children may resemble that of AF in adults. Local recurrences did not affect the chance of responding to systemic therapy or the survival rate. The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy. Good responses to systemic treatments, and particularly to low‐dose chemotherapy, were observed as reported previously in adults. Cancer 2010. © 2010 American Cancer Society.

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confidence: 99%

Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

132

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“…In contrast, in another pediatric AF study, which was from the same institution reported by Rao et al 8 and included overlapping patients, 11 of 13 children (85%) developed recurrent disease after irradiation, including 6 of 8 children who received doses Ն 50 Gy. 26 To our knowledge, the role of radiotherapy in childhood AF as adjuvant treatment in patients with SP margins has not been established to date and will require further study in a prospective, randomized study. The use of chemotherapeutic and other systemic agents may be a reasonable alternative to avoid radiotherapy in the growing child, although chemotherapy also carries a risk for potentially adverse side effects, such as second malignancies, fertility problems, and cardiotoxicity.…”

Section: Discussionmentioning

confidence: 99%

“…22,25,[31][32][33] In pediatric patients with AF, the high doses of radiotherapy (55-60 grays [Gy]) necessary for tumor control harbors a large risk for growth problems and the development of secondary malignancies. 4,6,12,22,26,32 One pediatric AF study reported 11 children with partially excised or recurrent lesions who received radiotherapy and who had at least 3 years of follow-up. 6 Four of those 11 children (36%) developed recurrent disease, including 2 of 5 patients who received radiation doses Ͼ 50 Gy.…”

Section: Discussionmentioning

confidence: 99%

“…Two studies, by Merchant et al 26 and Raney et al, 27 reported patients from the same institution as Rao et al 8 and Spiegel et al, 12 respectively, with overlapping time frames. Those investigators 26,27 reported on small series of patients who were treated with radiotherapy and chemotherapy, respectively, and both reports were excluded from this review. The other 10 studies, along with the current study, reported a total of 187 patients (Table 3).…”

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confidence: 99%

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Pediatric aggressive fibromatosis

Buitendijk

Ven

Dumans

et al. 2005

Cancer

100

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Fine‐needle aspiration (FNA) biopsy has become a standard first‐line diagnostic procedure for palpable and nonpalpable nodules of the thyroid gland. Six cytologic diagnostic categories have recently been proposed to unify the terminology that is linked to proper clinical management. We report a case of follicular neoplasm diagnosed on FNA specimen that had a very artistic appearance of the microfollicle formation on both Diff‐Quik and Papanicolaou‐stained slides. Diagn. Cytopathol. 2010;38:660–662. © 2009 Wiley‐Liss, Inc.

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“…However, for patients with gross or microscopic residual disease or for those who have recurrence, regardless of margin status, the use of irradiation appears to improve local control. [1][2][3][4][5][6] There have also been recent attempts to define whether chemotherapy and/or hormonal therapy might also play a role in the treatment of desmoid tumors, although less than impressive results have been reported. 7,8 The decision to recommend radiation must be weighed against the knowledge that this is a 'benign' disease, and the fact that potential second malignancies may be result of intervention.…”

Section: Introductionmentioning

confidence: 99%

The Role of Postoperative Irradiation in the Treatment of Locally Recurrent Incompletely Resected Extra‐Abdominal Desmoid Tumors

Fontanesi

Mott

Kraut

et al. 2004

Sarcoma

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PrécisFor patients who undergo re-resection for recurrent extra abdominal desmoid tumor, and in whom microscopically or grossly positive margins are found, the use of postoperative radiation is not only warranted, but is critical in the ability to establish local control. We recommend total doses of at least 50 Gy for microscopic positive surgical margins and 56 Gy for gross residual surgical margins. We recommend the use of external beam irradiation alone for patients who have involvement in the hand and plantar regions, while in the remaining areas treatment using external beam irradiation, brachytherapy alone, or a combination of external beam with brachytherapy may be utilized. Abstract Background: To define the efficacy of postoperative irradiation in patients with recurrent extra-abdominal desmoid tumors in whom surgical intervention has resulted in microscopically or grossly positive surgical margins. Methods: A retrospective analysis was performed on all patients referred to the department of radiation oncology at the Detroit Medical Center with a diagnosis of recurrent extra-abdominal desmoid tumor. This analysis includes all patients seen from 1 January 1990 through 31 December 1999. A total of 11 patients were treated to 13 sites. Ten had microscopically positive margins and three had gross residual disease. Three patients were noted to have multifocal disease at the time of initial representation. Local control, survival, follow-up, and subsequent development of new tumors are measured from the last day of treatment with irradiation. Results: Thirteen sites were treated. Seven patients had received chemotherapy/hormonal therapy prior to surgery and/or irradiation. The most commonly used drug was tamoxifen (n ¼ 6). The type of radiation delivered included external beam irradiation alone (n ¼ 3), combined external beam irradiation and brachytherapy (n ¼ 4), brachytherapy alone (n ¼ 3) and 252-Cf neutron brachytherapy alone (n ¼ 3). Follow-up has ranged from 29 to 115 months (median ¼ 76 months). Three patients have failed locally at 17, 24 and 29 months. One of these was treated for gross residual disease. No patient has died of tumor-related causes. Salvage at the failed sites was possible in twom of three with re-irradiation using external neutrons and/or aggressive surgical intervention and systemic therapy. Complications were most often noted to include decrease range in motion, especially in joint areas, and skin reactions which were normal in presentation. In one site there was development soft tissue necrosis. Conclusion: Based on our experience we recommend postoperative irradiation for all recurrent extra-abdominal desmoid lesions with microscopically or grossly positive surgical margins. Furthermore, patients with recurrent desmoid tumors involving the bony structures of the hand or feet are poor candidates for brachytherapy alone. For patients with extremity lesions, brachytherapy may be a reasonable treatment option provided adequate margins around the tumor bed are covered. The continued r...

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Long-term results with radiation therapy for pediatric desmoid tumors

Cited by 57 publications

References 27 publications

Aggressive fibromatosis in children and adolescents

Aggressive fibromatosis in children and adolescents

Pediatric aggressive fibromatosis

The Role of Postoperative Irradiation in the Treatment of Locally Recurrent Incompletely Resected Extra‐Abdominal Desmoid Tumors

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