Advanced aggressive fibromatosis: Effective palliation with chemotherapy

Figures & data

Table I. Previous treatments for patients receiving MTX/VBL.

Patients	Primary site	Previous operation(s)	Radiotherapy (RT)	Previous systemic treatment
1	Pelvis	Y	N	Toremifene for 9 months to PD
2	R upper limb	Y	Y	Toremifene for 6 months to PD
3	R neck	N	N	none
4	R lower limb	Y	Y	Toremifene for 24 months to SD
5	Abdomen	Y	N	Tamoxifen for 3 months. R not known
6	L neck/brachial plexus	Y	Y	Tamoxifen for 39 months to SD
7	R lower limb	Y	Y	Tamoxifen for 6 months to SD
8	Pelvis	Y	Y	none
9	L neck	N	Y (radical)	Toremifene for 3 months to PD
10	L neck/chest wall	Y	N	Toremifene for 2 weeks
11	Abdomen	Y	N	Ifos/Dox 1# and DTIC+Dox 2# to PR
12	Perineum	Y	Y	Tamoxifen for 6 months. R not known
13	L shoulder girdle	Y	Y	Toremifene for 6 months to PD
14	L neck	Y	Y	Etoposide #2 to PD, toremifene for 3 months to PD, DTIC/Dox #3 to SD
15	Pelvis	Y	Y	none
16	L abdominal wall	N	N	Tamoxifen for 3 months to PD
17	L neck	Y	Y	Tamoxifen + Sulindac for 4 months to PD
18	L paraspinal area	Y	Y	Toremifene for 3 months to PD

Abbreviations: Y: yes; N: no; PR: partial response; SD: stable disease; PD: progressive disease; #: cycle of; R: response.

Table II. Previous treatments for patients receiving PLD.

Patients	Primary site	Previous operation(s)	Previous radiotherapy (RT)	Previous systemic treatment
1	Perineum	Y	Y	- Tamoxifen/Pred/Cyclo/MTX for 3 months to SD, ifosfamide × 4# to SD
2	L paraspinal area	Y	Y	- Toremifene 3 months to PD, MTX/Vinblastine for 6 months to SD, Imatinib 3 weeks PD, rechallenge MTX/Vinblastine 4 months to SD
3	R lower limb	N	N	- Sulindac/vit C >12 months to SD, Imatinib 12 months to SD
4	Head and neck	N	N	Diclofenac 8 months to PD
5	Abdomen	Y	Y	Tamoxifen <2 months to PD
6	R SCF	N	N	Tamoxifen to SD
7	R axilla/brachial plexus	Y	N	Tamoxifen pre-op failed
8	Chest wall	N	N	Tamoxifen 2 months to SD
9	Abdomen (Gardner's)	Y	N	Tamoxifen and sulindac-poor tolerance
10	R upper limb	N	N	Diclofenac 3 months + tamoxifen 6 months to PD
11	R paraspinal area/brachial plexus	Y	Y	Tamoxifen for 16 months to SD but ongoing pain
12	Chest wall	Y	N	Tamoxifen + diclofenac 5 months to PD
13	L shoulder/chest wall	Y	N	Tamoxifen for 12 months to PD
14	Pelvis	N	N	Tamoxifen and diclofenac for 7 months to PD

Abbreviations: Y: yes; N: no; PR: partial response; SD: stable disease; PD: progressive disease; #: cycle of; R: response; MTX: methotrexate; Pred: prednisolone; Cyclo: cyclophosphamide; SCF: supraclavicular fossa.

Figure 1. Fibromatosis of the right inguinal region. PLD 40 mg/m² × 6 February 2006 to July 2006. Tumour shrinkage and symptomatic relief. Images: May 2006, November 2006, August 2008.

Figure 2. Fibromatosis of brachial plexus PLD 50 mg/m² × 6 – August 2007 to January 2008, late partial remission, markedly improved mobility of hand and shoulder, with reduced analgesic requirement. Images: August 2007, September 2008, March 2009.

Table III. Treatment with PLD.

PLD cycles	Starting dose mg/m^2	Response RECIST	Response symptomatic	TTP months (m)	Toxicity	Dose reduction mg/m^2
5	50	SD	yes	9 m	Fatique 2, PPE 1	40 at 3#
4	50	SD	yes	20 m	Mucositis 1, diarrhoea 1	no
6	50	PR	yes	45 + m	Mucositis 2, PPE 2	40 at 2#
6	50	PR	yes	27 + m	no	no
4	50	SD	yes	27 + m	Mucositis 2, PPE 2, fatique 1	40 at 3#
6	50	PR	yes	27 + m	no	no
6	40	SD	yes	30 + m	Mucositis 1, PPE 1	35 at 4#
5	50	SD	yes	9 m	Mucositis 1, PPE 3	40 at 4#
6	50	PR	yes	16 + m	Mucositis 1, fatique 1	no
6	50	SD	yes	12 + m	Mucositis 1, PPE 2	40 at 3#
6	50	SD	yes	18 + m	Mucositis 1, PPE 1	no
6	50	SD	no	11 + m	no	no
ongoing	50	ongoing	yes	ongoing	Mucositis 3, PPE 2	40 at 3#
ongoing	40	ongoing	no	ongoing	Hypersensitivity reaction cycle 1#	no

Abbreviations: Pts: patients; PR: partial response; SD: stable disease; PD: progressive disease; #: cycle of PLD; PPE: palmar plantar erythema.