Clinical Question: Is CRPS-I associated with malignancy?
Clinical Bottom line: Yes, CRPS-I has been reported
to be associated with various cancers and may manifest as
a paraneoplastic syndrome.
ISI Current contents
The Cochrane Library, Issue 2, 2002. Oxford: Update Software.
Citations used for evidence:
Goldberg MI, Kennedy SF. Reflex sympathetic dystrophy--recognition
and management in gynecologic oncology. Gynecol Oncol. 1979
Medsger TA, Dixon JA, Garwood VF. Palmar fasciitis and
polyarthritis associated with ovarian carcinoma. Ann Intern
Med. 1982 Apr;96(4):424-31
Michaels RM, Sorber JA.Reflex sympathetic dystrophy as
a probable paraneoplastic syndrome: case report and literature
review. Arthritis Rheum. 1984 Oct;27(10):1183-5
Taggart AJ, Iveson JM, Wright V. Shoulder-hand syndrome
and symmetrical arthralgia in patients with tubo-ovarian
carcinoma. Ann Rheum Dis. 1984 Jun;43(3):391-3.
Goldberg E, Dobransky R, Gill R. Reflex sympathetic dystrophy
associated with malignancy. Arthritis Rheum.1985 Sep;28(9):1079-80.
Summers CL, Shahi M. Epithelioid sarcoma presenting as
the reflex sympathetic dystrophy syndrome. Postgrad Med
J. 1987 Mar;63(737):217-20.
Prowse M, Higgs CM, Forrester-Wood C, McHugh N. Reflex
sympathetic dystrophy associated with squamous cell carcinoma
of the lung. Ann Rheum Dis. 1989 Apr;48(4):339-41.
Regan M, Foley-Nolan D, McCarthy G, Coughlan RJ. Reflex
sympathetic dystrophy associated with large cell lung carcinoma. Ann Rheum Dis. 1989 Dec;48(12):1031
Ameratunga R, Daly M, Caughey DE. Metastatic malignancy
associated with reflex sympathetic dystrophy. J Rheumatol.
Malane SL, Sau P, Benson PM. Epithelioid hemangioendothelioma
associated with reflex sympathetic dystrophy. J Am Acad
Dermatol. 1992 Feb;26(2 Pt 2):325-8.
Derbekyan V, Novales-Diaz J, Lisbona R. Pancoast tumor
as a cause of reflex sympathetic dystrophy. J Nucl Med.
Olson WL. Reflex sympathetic dystrophy associated with
tumour infiltration of the stellate ganglion. J R Soc Med. 1993 Aug;86(8):482-3
Ku A, Lachmann E, Tunkel R, Nagler W. Upper limb reflex
sympathetic dystrophy associated with occult malignancy. Arch Phys Med Rehabil. 1996 Jul;77(7):726-8.
the Demographics Study
the Results Study
- Age: Average – 55 yrs; Range 27-79
- Gender: Male – 6; Female – 16
- Cancer Type – Carcinoma – 18; Other – 4
- Cancer Location: Reproductive tract – 9, Lung – 5, Extremity
– 3, Other – 5
- Metastasis detected - 9; Not detected – 13
- RSD location: Unilateral – 12 Bilateral - 10
Upper limbs involved – Right – 14; Left – 10
Lower limbs involved – Right – 2; Left – 6
- Treatment – Sympathetic block – 9;Physical Therapy – 6;
Corticosteroids – 12
- Symptomatic improvement:
Some Pain relief – 13; No improvement –5; Disability worsened
– 3; Not available – 1
- Satisfied IASP criteria for diagnosis of CRPS-I – 9 out
of 22 cases
- The average age of RSD patients in the series is 55 years
with the youngest being 27 years and the oldest being 79
years old, suggesting that RSD with cancer can occur at
- Majority of the patients are observed to be females. This
may be due to a generally higher prevalence of RSD in women.
- Carcinomas from the reproductive tract and lung were the
most common types of malignancy associated with concurrent
RSD. Metastases were not reported as detected in majority
of the cases.
- The majority (80%) of patients with ovarian cancers are
reported to have presented with bilateral upper extremity
- The RSD location was unilateral in most cases with the
majority confined to the upper limbs. Right and left sides
were involved equally.
- Majority of these cancer patients with RSD were treated
with corticosteroids. Sympathetic blocks and physical therapy
was administered less frequently than corticosteroids.
- Twelve out of the total 22 cases of malignancy with concurrent
RSD have not reported any noxious event or a cause of immobilization
prior to the onset of RSD.
- Pain and edema were the most commonly associated symptoms.
- Consistent with the review on this topic by Mekhail and
Kapural1, in the majority of cases with malignancy and concurrent
RSD/CRPS-I, the upper limbs were affected with RSD/CRPS-I.
- While vulvar and cervical carcinomas were associated with
RSD/CRPS-I in the lower limbs, ovarian malignancies were
consistently associated with upper limbs and in most cases
- The aim of this evidence report is to corroborate the
coexistence of RSD/CRPS-I and malignancy, and to characterize
the demographic and disease profile of patients reported
to have had both conditions. There is no evidence of any
specific treatment that is different from routine therapy
that is effective in managing patients with concurrent RSD/CRPS-I
- About 40% of the reported patients met the current IASP
criteria for CRPS-I. In more than 50% of patients, a noxious
event or immobilization was not reported.
1. Mekhail N, Kapural L. Complex regional pain syndrome
type I in cancer patients. Curr Rev Pain. 2000;4(3):227-33.
Appraised by: Prabhav Tella, July 2002