Disease and economic burden of surgery in desmoid tumors: a review

Figures & data

Table 1. Recurrence and progression rates reported in retrospective studies.

				Patients who underwent surgery			Recurrence/progression rates
Reference	Country, study years	Population characteristics	Location of the tumor	Primary	Recurrent	Duration of follow-up, median, mo	Surgery only	Surgery + radiotherapy	Active surveillance^a
All subtypes of tumors (extra-abdominal, abdominal wall, intra-abdominal)
He et al. [24]	China, 1985–2014	N = 114^b; 69% female; median age 33 y	AW: 35% Intra-A: 17% Extra-A: 48%	89	25	72.5	35/114 (31%)
Cates et al. [25]	US, 1983–2011	N = 92^b; 69% female; median age, 40 y	AW: 22% Extra-A: 78%	92	0	38.4	25/92 (27%)
Colombo et al. [26]	France and Italy, 1992–2012	N = 216^b; 63% female; median age, 41 y	Intra-A: 25% Extra-A: 75%	94	0	76 (surgery)	18/94 (19%)		28/70 (40%)
Crago et al. [27]	US, 1982–2011	N = 495; 67% females, median age, 38 y (primary disease); FAP, 3%	AW: 18% Intra-A: 20% Extra-A: 57%	382	113	60	100/439^c (23%)
de Bruyns et al. [28]	Canada, 1990–2013	N = 227; 68% female, median age, 40 y; FAP, 6%	AW: 22% Intra-A: 18% Extra-A: 61%	132		77	29/108 (27%)	4/24 (17%)	33/55 (60%)
Huang et al. [29]	China, 1987–2009	N = 214; 77% female; median age, 33 y	AW: 43% Intra-A: 6% Extra-A: 51%	153	61	55.5	42/214 (20%)
Mueller et al. [30]	Germany, 2000–2015	N = 32^b; 58% female; median age, 36 y	AW: 27% Intra-A: 15% Extra-A: 58%	26	6	65	13/32 (41%)
Nishida et al. [31]	Japan, NR	N = 85^b; 66% female; 53% > 36 y	AW: 15% Extra-A: 85%	73	12	10–42	32/85 (38%)
Peng et al. [32]	US, 1983–2011	N = 211; 68% female; median age, 36 y; FAP, 8%	AW: 21% Intra-A: 23% Extra-A: 56%	179	0	25.7	42/179 (24%)
Turner et al. [33]	Canada, 2004–2015	N = 103^b; 74% female; mean age, 43 y	AW: 30% Intra-A: 17% Extra-A: 53%	53		35	8/53 (15%)		21/50 (42%)
Yang et al. [34]	China, 1999–2019	N = 267; 70% female; 65% > 30 y	Extremity: 38% Non-extremity: 62%	207	60	NR	29/141 (21%)	24/126 (19%)
Extra-abdominal tumors
van Broekhoven et al. [35]	The Netherlands, 1989–2011	N = 132; 64% female; median age, 36 y	Extra-A: 100%	128	0	38	18/128 (14%)
Janssen et al. [11]	SLR and MA 1999–2015	N = 1,295	Extra-A: 100%	1,053	242	25.0–135.0	297/1005 (30%)	79/290 (27%)
Krieg et al. [36]	Switzerland, NR	N = 96; 645% female; mean age, 39 y	Extra-A: 100%	60		100.8	20/44 (46%)	6/16 (38%)	3/15 (20%)
Tsagozis et al. [13]	UK, 1981–2015	N = 174; 56% female; mean age, 35 y	Extra-A: 100%	174		38	44% (primary surgery), ~90% (after surgery for first or second recurrences)

AW = abdominal wall; intra-A = intra-abdominal; extra-A = extra-abdominal; FAP = familial adenomatous polyposis; MA = meta-analysis; N = number of patients; NR = not reported; SLR = systematic literature review; UK = United Kingdom; US = United States.

^aPatients who progressed under active surveillance.

^bPatients with familial adenomatous polyposis were excluded.

^cRecurrence information is not available for all patients undergoing surgery.

Table 2. Functional outcomes in patients with DT in their extremities after tumor resections.

Reference	Population	Symptoms/functional deficits	At presentation, n (%)	After treatment, n (%)
Catton et al. [46]	Patients with DT in limb or limb girdle (N = 24)	Limb amputated (grade 0)	1 (4)	5 (21)
		Functional deficit severe enough to make amputation an acceptable therapy of choice (grade 1)	1 (4)	0 (0)
		Resection of major muscle group or nerve. Major functional limitations requiring an orthopedic appliance. Chronic pain requiring regular narcotic analgesia (grade 2)	4 (17)	6 (25)
		Mild functional limitations but able to perform routine activities (grade 3)	7 (29)	3 (13)
		No functional limitations. Able to participate in prolonged activity (grade 4)	9 (38)	8 (33)
		Not recorded	2 (8)	2 (8)
Gaposchkin et al. [47]	Patients with DT of the brachial plexus (N = 15)	Limb amputated	0 (0)	1 (7)
		Upper extremity or neck pain	4 (27)
		Chronic pain syndrome		9 (60)
		Upper extremity paresthesia/sensory loss	5 (33)	7 (47)
		Upper extremity weakness	2 (13)	7 (47)
		Upper extremity decreased movement	6 (40)
		Upper extremity significant functional loss		6 (40)
		Upper extremity neurologically intact	9 (60)	3 (20)
		Painless mass	8 (53)
		Muscle spasm	2 (13)

DT = desmoid tumors; N = number of patients.

Figure 1. Changes in the percentage of cases treated with surgery as initial treatment.

AW = abdominal wall; DT = desmoid tumors; Extra-A = extra-abdominal.

^a Source: Penel et al. [37]

^b Source: Nishida et al. [50]

^c Source: Sobczuk et al. [51]

^d Source: Anneberg et al. [22]

Table 3. Costs of STS surgeries in the US.

Reference	Population	Type of surgery/source	Type of costs/charges	Costs/charges per patient
Bateni et al. [52]	Medicare patients (N = 3,913, age≥66 y) with truncal or extremity STS in 1992–2011	Planned and unplanned excisions (including first and subsequent resections)/SEER-Medicare database	Costs for first excisions or re-excisions of STS tumors include costs of surgeries, hospitalizations, and services within 30 days after discharge; adjusted to 2022 USD	Planned excisions, total mean actual payments: $36,138 Unplanned excisions, total mean actual payments: $23,080
Mundinger et al. [53]	Patients (N = 13; average age, 55 y) undergoing STS resections in 1998–2004	Limb-sparing upper-extremity STS resection and functional reconstruction/single-center database	Total charges include routine, operating room, pharmacy, tests, supplies, therapy, and miscellaneous charges; adjusted to 2022 USD	Proximal upper extremity, total mean charges: $29,658 Distal upper extremity, total mean charges: $41,598
Lau et al. [54]	Adults (N = 181; age≥18 y) who underwent resection of spinal tumors (primary or metastatic) in 2008–2013	Surgery for primary tumors (chordomas, chondrosarcomas, giant-cell tumors, nerve sheath tumors, schwannomas, neurofibromas, and hemangiomas)/single-center database	Direct hospital costs for patient’s admission included surgical supplies, services, room and care, and pharmacy from 2008–2013	Mean direct hospital costs for surgery of primary tumors: $55,801
Alamanda et al. [55]	Patients (N = 304, age≥18 y) who underwent surgical resection of STS in 2006–2008	Primary excisions and re-excisions of extremity STS/single-center database	Billed charges include provider, technical (supplies, operative staff, imaging), and miscellaneous charges from 2006–2008	Total median charges, primary excisions: $40,231 Total median charges, secondary excisions: $44,770

N = number of patients; SEER = Surveillance, Epidemiology, and End Results; STS = soft-tissue sarcoma; USD = United States dollars.

Table 4. Costs of amputations in the US.

Reference	Population	Source	Type of costs/charges	Costs per patient
Franklin et al. [56]	Veterans with diabetes and LEA	VHA clinic users with diabetes in 2010	Diabetes-related LEA expenditures due to inpatient medical, inpatient surgical, and outpatient care were estimated using data from the VHA system; cost adjusted to 2022 USD	Total mean direct medical costs for toe amputation: $54,046 Total mean direct medical costs for amputation below the knee: $92,588 Total mean direct medical costs for amputation above the knee: $107,819
Labovitz et al. [57]	Patients discharged with T2D and Charcot foot undergoing LEA	Discharge files from the California Office of Statewide Health Planning and Development (OSHPD) from 2009 to 2012	Acute care costs were calculated by adjusting the inpatient charges by the hospital’s cost-to-charge ratio. This estimated cost was then adjusted to 2022 USD	Inpatient acute care mean costs, minor LEA: $30,573 for Charcot diagnosis at admission to $43,030 for delayed Charcot foot diagnosis Inpatient acute care mean costs, major LEA: $31,502 for Charcot diagnosis at admission to $41,585 for delayed Charcot foot diagnosis
Berger et al. [58]	Patients with CAD or PAD undergoing nontraumatic amputations	Optum Integrated Database from 2009 to 2016	Costs were assessed over the first year of follow-up and include inpatient, outpatient, and prescription drug costs; adjusted to 2022 USD	Total mean healthcare costs for the first year after amputation in CAD patients: $70,944 Total mean healthcare costs for the first year after amputations in PAD patients: $82,095
Desai et al. [59]	Patients (age≥50 y) with lower-extremity PAD undergoing major nontraumatic LEA	Health insurance claims using the Optum Clinformatics Data Mart database (January 2014 to June 2019)	All claims associated with a primary diagnosis code or procedure code for the event during an inpatient or emergency department visit and up to 1 y after the event; adjusted to 2022 USD	Major nontraumatic LEA, mean cost per patient-year: $112,751
Gomez et al. [60]	California workers with injuries resulting in amputations	Claims data reported to the California Department of Industrial Relations Workers’ Compensation Information System from 2007 to 2018	Medical costs were calculated as the sum of medical, pharmaceutical, and durable medical equipment dollars paid over the duration of the closed claims during 2007–2018	Median costs for amputations: Above elbow: $14,855 Below elbow: $30,825 Partial hand: $7,785 Above knee: $18,390 Below knee: $20,901 Partial foot: $21,469 Multiple: $46,637
Lo et al. [61]	Patients with diabetes or PAD undergoing LEA	Systematic review of studies published from 2013 to 2019	Medicare claims adjusted to 2022 USD	Mean costs of LEA procedure due to PAD: $21,916 Total projected lifetime healthcare costs after LEA: $953,274

CAD = coronary artery disease; LEA = lower-extremity amputation; major LEA = amputation at or proximal to the ankle; minor LEA = amputation of the foot or partial foot; PAD = peripheral artery disease; T2D = type 2 diabetes; US = United States; USD = United States dollars; VHA = Veterans Health Administration.

Note: costs reported for a period of time and not for a single year were not adjusted to 2022 USD because of the lack of information on how the costs were estimated for the whole period in the original source.

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