Palmar fascitis and polyarthritis as a paraneoplastic syndrome associated with ovarian carcinoma: a case report

Abstract

This case report concerns a 49-year-old woman whose physical examination was remarkable for symmetrical swollen hands, fingers and palmar fascial thickening with erythema. The fingers showed flexion contractures. Examination also revealed markedly limited bilateral shoulder and limited knee flexion.The patient's symptoms were treated with rehabilitation. The immunological laboratory investigations were normal. A technetium scan showed a slightly increased uptake in both shoulders, wrists, hips, knees and ankles. CT revealed a pelvic solid mass next to the uterus. After the patient underwent a total hysterectomy and anexectomy, the polyarthritis showed a gradual improvement but the contractures in the hands persisted.Palmar fasciitis and polyarthritis syndrome (PFPAS) is an uncommon rheumatic disorder consisting of pain, swelling, stiffness, progressive flexion contractures of both hands and thickening of palmar fascia, with erythrosis. It was first described as a paraneoplastic phenomenon with ovarian carcinoma in 1982.The characteristic hand deformities of PFPA should alert the clinician to search for an underlying malignant disease.

Paraneoplastic Palmar Fasciitis and Polyarthritis Syndrome in a Patient With Advanced Prostate Cancer

“…1,7,23,25,28 However, in most cases, x-rays of the joints show no abnormalities (as was the case in our patient). 2,6,9,11,16,17,19,21,29 As the disease progresses, contractures develop and x-ray films of the hands may show flexion deformities of the MCP and PIP joints. 3,8,15 In some instances, PIP joints may be affected preferentially with relative sparing of MCP joints, although this is uncommon.…”

“…The efficacy of systemic steroids in patients with PFPAS is somewhat disappointing, with infrequent improvement of the polyarthritis and no effect on the joint contractures. 1–4,6–9,12,14,16–23,25,30,31 Results have been largely disappointing with the use of conventional disease-modifying anti-rheumatic drugs (DMARDs), such as methotrexate, 12,19,21,29 hydroxychloroquine, 6,14 D-penicillamine, 17,27 and sulfasalazine. 14 More recently, TNF inhibitors 12 have been attempted as a second-line treatment or in combination with corticosteroids.…”

“…Surgery, chemotherapy, and radiotherapy for specific underlying oncologic disorders seem to be the only treatment with a positive effect on the inflammatory component of PFPAS. 1,5,8,10–12,16,22–24,26,28,30 However, contractures are often irreversible and usually persist despite anti-inflammatory, DMARD, and anti-cancer therapies. 1,2,4–9,12,14,16–19,23,25,27,30,31 In a few cases, flexion contractures modestly improved with effective anti-neoplastic therapy.…”

“…Since that seminal report published 30 years ago, only 48 additional cases of PFPAS have been described in the literature, and these have been associated with a number of different neoplasms, including gynecologic malignancies (ovarian and fallopian tube cancers), 2–16 pancreatic adenocarcinoma, 17–20 non—small cell and small cell lung carcinoma, 3,20–22 breast carcinoma, 23–26 prostate carcinoma, 27 gastric cancer, 28 colon cancer, 20 transitional cell carcinomas of the urinary tract, 6,20,29 carcinoma of unknown origin, 17 and hematologic malignancies 17,30 (Appendix Table 1). …”

Palmar fasciitis and polyarthritis, a rare paraneoplastic syndrome related to ovarian cancer

“…PFPAS is therefore considered to be a seronegative syndrome . Radiographs of the joints appear generally normal, suggesting that PFPAS is a nonerosive form of arthropathy, while joint MRI and technetium‐99m bone scans may show nonspecific signs of arthritis such as synovial hyperaemia and joint oedema …”