Treatment of Aggressive Fibromatosis: the Experience of a Single Institution

doi:10.1016/j.clon.2009.08.012

Clinical Oncology

Volume 21, Issue 10, December 2009, Pages 775-780

https://doi.org/10.1016/j.clon.2009.08.012 Get rights and content

Abstract

Aims

Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour, although pathologically benign, and it does not metastasise, yet it can cause serious local distressing symptoms by virtue of local destruction and impairment of local function. The aim of this study was to emphasise the role of radiotherapy and adequate surgery in the treatment of fibromatosis in patients presenting with newly diagnosed or recurrent disease and to analyse our treatment results over 15 years for this rare tumour type.

Materials and methods

Fifty-four patients with confirmed diagnosis of aggressive fibromatosis treated at King Faisal Specialist Hospital between 1990 and 2006 were identified from our local cancer registry. Forty-seven patients had surgery: complete resection (R0) in 20 patients, incomplete surgery (R1/2) in 27 patients, and seven patients had biopsy only. Forty-five patients were treated with radiotherapy: 38 patients were treated with postoperative radiotherapy, three patients were treated with preoperative radiotherapy and four patients had radiotherapy as the only treatment. The radiotherapy dose ranged between 45 and 60 Gy (median 50.4 Gy). Three patients did not receive any form of treatment apart from biopsy, but were still included in the final analysis.

Results

Fifty-two per cent (28/54 patients) of our patient population had tumour recurrence when first presented to King Faisal Specialist Hospital. The median age was 29.5 years (range 2–63 years). The most common site of involvement was the extremities (28 patients). Among the 54 patients (with primary and recurrent presentation) there were 10 local recurrences, all of which were within the original primary site. The 5-year progression-free survival and overall survival rates for the whole group were 75 and 95%, respectively. Univariate and multivariate Cox regression analysis showed that the depth of invasion significantly affected progression-free survival.

Conclusion

Aggressive fibromatosis is effectively treated with surgery and postoperative radiotherapy. Patients first presenting with tumour recurrence may still have local tumour control comparable with newly diagnosed patients.

Introduction

Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour. It virtually never metastasises, but can cause local destruction and organ dysfunction through infiltration of blood vessels and/or nerves. Radical surgery is paramount in local control, but because of the tumour's infiltrative nature, positive surgical margins and, hence, tumour recurrence are common findings 1, 2. Almost 100 years ago, James Ewing proposed treating inoperable tumours with radiation [3]. At that time, the precise role of radiotherapy was still not clearly defined [4]. Radiotherapy may be given as adjuvant treatment to decrease local recurrence, especially when the surgical margin is positive, or as a single treatment in cases where surgery will result in severe morbidity or disruption of organ function [5]. Nuyttens et al.[6], in a comparative review between surgery and radiotherapy, found that local control can approach 83% for primary radiotherapy.

The aim of the present study was to analyse our treatment results in this rare tumour type and to identify the important prognostic factors that could affect treatment outcome.

Section snippets

Materials and Methods

This was a retrospective analysis of patients with primary and recurrent aggressive fibromatosis treated at King Faisal Specialist Hospital (KFSH) between 1990 and 2006.

This study was approved by the research accreditation and ethical committee at KFSH in 2007.

Overall Outcome

For the whole group (taking all patients' primary and recurrent cases at presentation), with a median follow-up of 7.3 years, there were 10 (18.5%) local recurrences, all of which were within the original primary site or within the irradiated field. The actuarial local control rate at 5 years was 75% and at 10 years 67% (Fig. 1).

The univariate regression analysis of factors potentially affecting local control is shown in Table 2. In the univariate analysis, patients with completely resected

Discussion

Aggressive fibromatosis is a locally aggressive tumour, with a local recurrence rate that may reach as high as 70–90%, especially after incomplete resection 9, 10.

The treatment of fibromatosis usually consists of adequate surgery with a wide surgical margin (the definition of this margin is still contradictory), plus or minus postoperative radiotherapy 1, 11, 12. Chemotherapy, non-steroidal anti-inflammatory drugs or tamoxifen have been used, with limited published data 13, 14. In case surgery

Conclusion

Overall survival for aggressive fibromatosis is usually high and most deaths may not be related to the disease itself. Local control rates for primary vs recurrent disease were comparable in our data set. Radiotherapy and adequate surgery are very effective in radical treatment for aggressive fibromatosis, but still more data are needed to define when and who should be treated with radiotherapy.

Acknowledgment

We would like to thank Dr Abdelmoneim Eldaly from the Biostatistics Department for his help with the statistics for this study.

References (27)

J.C. Acker et al.
The management of desmoid tumors
Int J Radiat Oncol Biol Phys
(1993)
M.A. Spear et al.
Individualizing management of aggressive fibromatoses
Int J Radiat Oncol Biol Phys
(1998)
M.T. Ballo et al.
Radiation therapy in the management of desmoid tumors
Int J Radiat Oncol Biol Phys
(1998)
R. Miralbell et al.
Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy
Int J Radiat Oncol Biol Phys
(1990)
C.N. Catton et al.
Aggressive fibromatosis: optimisation of local management with a retrospective failure analysis
Radiother Oncol
(1995)
E. Stoeckle et al.
A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases
Eur J Surg Oncol
(2009)
H. Suit et al.
Radiation treatment of benign mesenchymal disease
Semin Radiat Oncol
(1999)
H.D. Suit
Radiation dose and response of desmoid tumors
Int J Radiat Oncol Biol Phys
(1990)
J.A. Jelinek et al.
The efficacy of radiotherapy as postoperative treatment for desmoid tumors
Int J Radiat Oncol Biol Phys
(2001)
J.J. Reitamo et al.
The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor
Am J Surg
(1986)

R.A. Zlotecki et al.

External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis

Int J Radiat Oncol Biol Phys

(2002)

A.D. Stockdale et al.

Radiotherapy and conservative surgery in the management of musculo-aponeurotic fibromatosis

Int J Radiat Oncol Biol Phys

(1988)

B.A. Guadagnolo et al.

Long-term outcomes for desmoid tumors treated with radiation therapy

Int J Radiat Oncol Biol Phys B

(2008)

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In those patients who refuse surgery or are not surgical candidates, multiple treatment and management are available, ranging from observation and monitoring to radiation and chemotherapy. Radiation therapy may be used postoperatively, preoperatively, or as the sole treatment [15]. Pharmacologic agents with antiestrogens and prostaglandin inhibitors result in objective response rates of approximately 40–50%; the duration of response is variable [16].
Desmoid tumors are rare, aggressive fibroblastic soft tissue tumors. We report a case of a 20-year-old male with recurrence of desmoid tumor of the left shoulder after initial complete resection 3 years prior. Pathology revealed fibromatosis desmoid type. After complete resection of tumor at 17-year-old, no symptoms were reported after 3 years of first resection, when he developed numbness and weakness of the left upper extremity. Magnetic resonance imaging demonstrated an enlarging arteriovenous malformation at the left shoulder with possible recurrence. Excision was performed, and after tissue examination evidence of recurrent fibromatosis desmoid type was found without arteriovenous malformation. Recurrence after complete resection could be as high as 70%. Early recurrence recognition is essential as tumor growth before the infiltration of the aggressive tumor makes the resection more difficult and increases surgical risk of complications.
Four different treatment strategies in aggressive fibromatosis: A systematic review
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As expected, local recurrence was more common after surgery with positive margins compared to negative margins (Fig. 2A, Supplementary Table 1). Adjuvant radiotherapy after positive margins did not improve the local control rate (p = 0.549) [7–9,11,12,14,15,17–19,21,24,26,28,30–33,35]. Between the treatment groups radiotherapy and observation, irrespective of surgical margins, no significant difference existed in terms of local control (p = 0.355).
The treatment approach for aggressive fibromatosis is changing. Although surgery is the mainstay in common practice, recent literature is reporting a more conservative approach. We compared the local control rate for surgery, surgery with radiotherapy, radiotherapy alone and a wait and see policy in a systematic review.
A comprehensive search of the databases PubMed/Medline, Embase and Cochrane, of the medical literature published in 1999 till March 2017 was performed by two reviewers, including articles about extra abdominal aggressive fibromatosis without the genetical variants. A total of 671 studies were assessed for eligibility, and 37 studies were included for analysis, representing 2780 patients.
The local control rates for surgery alone, surgery and radiotherapy, radiotherapy alone and observation were 75%, 78%, 85% and 78%, respectively. For patients with recurrent disease observation had a better local control rate than surgery alone (p = 0.001). In the observation group, stabilization of the tumor was seen in median 14 (range 12–35) months. The time to local recurrence in the treatment group was median 17 (range, 11–52) months.
A watchful conservative first line approach with just observation and closely monitoring, by means of physical examination and MRI, appears to be justified in a subgroup of patients without clinical symptoms and no possible health hazards if the tumor would progress.
The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series
2017, The Lancet Child and Adolescent Health
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Our case series included children with unselected desmoid-type fibromatosis, and occurrence in the head and neck region was more frequent than that reported in adults (26% vs 7%).10 The sex ratio in our study (male:female 1·04) is consistent with that in previous paediatric studies (0·88–1·56),2,5,24 whereas in adults the tumour is more common in women than in men (0·23–0·53).9,10,31 Desmoid-type fibromatosis could be stimulated by oestrogen secretion in women, mainly during pregnancy.
In 2005, the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) proposed a conservative treatment algorithm—consisting of an initial wait-and-see strategy, non-mutilating surgery, and minimal-morbidity chemotherapy (in the case of tumour progression)—for paediatric patients with desmoid-type fibromatosis. We aimed to investigate the outcomes of this algorithm.
In this case series, patients (<25 years) with desmoid-type fibromatosis from 57 centres in eight countries were prospectively registered through a web-based system. Diagnosis was based on histological analysis of the tumour specimen after biopsy or surgery, and we classified patients by tumour site, clinical stage (TNM system), and post-surgical stage (Intergroup Rhabdomyosarcoma Study system). Progression-free survival was defined as the time from diagnosis until disease progression (clinical or radiological progressive disease, relapse, or death from any cause).
From Oct 1, 2005, to July 31, 2016, 173 patients (median age 11·4 years [IQR 4·0–14·1], 88 [51%] male patients) were registered. After excluding patients with missing data, 54 (35%) patients had no immediate therapy (wait-and-see strategy), 47 (31%) had immediate surgery, and 53 (34%) had immediate chemotherapy after diagnosis. 5-year progression-free survival was 36·5% (95% CI 27·8–45·2) overall, 26·7% (14·2–41·0) in the wait-and-see group, 41·2% (25·8–55·9) in the surgery group, and 42·8% (27·2–57·6) in the chemotherapy group (overall log-rank p=0·17; wait-and-see vs surgery p=0·12; wait-and-see vs chemotherapy p=0·13). In multivariable analysis, large tumour size (>5 cm) was associated with worse progression-free survival (hazard ratio 2·25, 95% CI 1·34–3·76; p=0·0021). Apart from one patient in the chemotherapy group who died from a secondary tumour (head and neck anaplastic embryonal rhabdomyosarcoma), all patients were alive at the time of analysis. 13 (8%) patients had biopsy only (no further treatment), 65 (42%) had chemotherapy only, 31 (20%) had surgery only, 36 (23%) had both chemotherapy and surgery, and nine (6%) had radiotherapy in addition to other therapies.
In paediatric patients with desmoid-type fibromatosis, the EpSSG conservative strategy did not compromise outcomes and could be adopted to reduce treatment burden.
S Wisnia and la Città della Speranza Foundation.
Diagnostic Pathology: Gastrointestinal
2015, Diagnostic Pathology: Gastrointestinal
Facial paediatric desmoid fibromatosis: A case series, literature review and management algorithm
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Stratifying the risk of recurrence in paediatric DF is difficult, due in part to the low incidence of the disease and the multiple therapeutic techniques employed, the absence of correlation between recurrence and clinicopathological findings and also the seemingly unpredictable nature of the disease. Although individual studies have identified several potential prognostic factors (for example, trisomy 8,24 depth of invasion,36 large numbers of slit like blood vessels37 and the presence of undifferentiated mesenchymal cells39), the only consistent association is with adequacy of surgical resection margins.3,5,14,38 Outcomes of treatment are generally good.
Desmoid fibromatosis (also known as infantile or aggressive fibromatosis) is a rare soft tissue tumour that is occasionally seen in children. Although histologically benign, its growth pattern is highly aggressive often showing invasion of surrounding musculature and bone.
Frequently found in cosmetically sensitive areas, complete excision can present a challenging problem. However, incomplete surgical excision is associated with high recurrence rates and although the disease responds to chemo and radiotherapy, both carry significant risks in young children.
The management of four paediatric desmoid fibromatoses occurring in the midface is discussed. The recent and pertinent literature is comprehensively reviewed and an algorithm for the management of paediatric desmoid fibromatoses is proposed.
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Original ArticleTreatment of Aggressive Fibromatosis: the Experience of a Single Institution

Abstract

Aims

Materials and methods

Results

Conclusion

Introduction

Section snippets

Materials and Methods

Overall Outcome

Discussion

Conclusion

Acknowledgment

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Radiother Oncol

Eur J Surg Oncol

Semin Radiat Oncol

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Am J Surg

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys B

Original Article
Treatment of Aggressive Fibromatosis: the Experience of a Single Institution