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

Background

A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells. These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumour cells or by an immune response against the tumour. Sometimes the symptoms of paraneoplastic syndromes show even before the diagnosis of a malignancy.

The symptoms may be endocrine, neuromuscular or musculoskeletal, cardiovascular, cutaneous, haematological, gastrointestinal, renal, or miscellaneous in nature. Several clinical pictures may be observed, each one specifically simulating more common benign conditions.

Rheumatological and musculoskeletal symptoms or syndromes are more frequently associated to gynaecological malignancies and lung tumours.¹ Dermatomyositis is associated with neoplasms in 7% to 34% of cases and some series show a relation with ovarian carcinoma in as many as 21.4% of women older than 40 years.^1,2 Other common cancers associated are gastric and lung tumours. Polymyositis, Raynaud syndrome, erythema nodosum, lupus-like syndrome and Eaton–Lambert syndrome are other rheumatological paraneoplastic syndromes.³

A rare musculoskeletal clinical manifestation as a paraneoplastic syndrome is complex regional pain syndrome (CRPS). Symptomatically, CRPS is a condition of the extremities, known and accepted best when it affects the upper extremity, predominantly in a regional distribution involving the distal forearm, wrist and hand, and occasionally the arm and shoulder, and manifested by four somewhat constant characteristics: intense or unduly prolonged pain, vasomotor disturbances, delayed functional recovery and various associated trophic changes. Soft tissue swelling, stiffness and muscle tightening may lead to a condition in which the hands and fingers or feet and toes contract into a fixed position. No pathophysiological mechanism has been established. CRPS type I is frequently triggered by tissue injury; the term describes all patients with the above symptoms but with no underlying nerve injury. Patients with CRPS type II experience the same symptoms but their cases are clearly associated with a nerve injury.

Palmar fasciitis and polyarthritis syndrome (PFPAS) is an uncommon paraneoplastic presentation that was first described in 1982 by Medsger and colleagues,⁴ who reported the association of this syndrome with ovarian carcinoma, and described these symptoms as a variant of reflex sympathetic dystrophy (now known as complex regional pain syndrome).

The musculoskeletal manifestations of PFPAS are similar to CRPS but is characterised by its considerably more aggressive nature and more severe presentation with diffuse articular involvements, almost always presents with bilateral inflammatory arthritis and fasciitis that is not localised to a particular limb.

Case presentation

A 49-year-old Caucasian woman was admitted to our facility in December 2007 with a suspected diagnosis of CRPS I. The patient had a progressive painful swelling and stiffness of both hands, especially the palms, which were noted by the patient to have thickened progressively since September 2007. The pain and reduced range of motion gradually extended to involve the shoulders and knees, with no improvement after initial non-steroidal anti-inflammatory and corticosteroid intrarticular treatment. She denied any previous joint diseases, Raynaud phenomenon, skin tightening, or trauma.

The physical examination on admission was remarkable for symmetrical swollen hands and fingers, painful on active and passive movement, and palmar fascial thickening with erythema. The fingers of the patient showed flexion contractures; making a fist was impossible (fig 1). Examination also revealed markedly limited bilateral shoulder restriction of movement to 45° of abduction and 50° of flexion and limited knee flexion to 90° bilaterally. No evidence of skin sclerosis or arthritis in other locations was found.

Figure 1.

The hands with maximum flexion and extension of the fingers.

The patient’s symptoms were treated orally with a diclofenac and dipiridona (metamizol) and local ointments (diclofenac), and with a programme of comprehensive rehabilitation.

Investigations

The immunological laboratory investigation failed to show any specific abnormalities (rheumatoid factor, anti-nuclear antibodies, anti-streptolysin titre, anti-neutrophil cytoplasmic antibodies, complement CH50 and C3d).

Radiography of both hands showed no signs of acute arthritis or osteoarthritis. Shoulder MRI showed subacromial and subdeltoid bursitis and echography of the palms showed fibrotic chronic tenosynovitis.

A technetium 99m bone scan showed a slight increased uptake in both shoulders, wrists, hips, knees and ankles. A CT scan of the abdomen revealed a pelvic solid mass next to the uterus, suggesting an ovarian tumour (fig 2).

Figure 2.

Scan of the pelvic mass.

Differential diagnosis

The differential diagnosis of PFPAS includes other conditions associated with contractures of the hands, as scleroderma, Dupuytren disease, eosinophilic fasciitis and also rheumatoid arthritis.

Treatment

After the patient underwent total hysterectomy and anexectomy and cytostatic treatment, the polyarthritis of the palms, shoulders and knees showed a gradual spontaneous improvement, but the contractures in both hands persisted, though slightly fewer, despite extensive physiotherapy (fig 3).

Figure 3.

Appearance of hands on maximum flexion and extension of fingers.

Discussion

PFPAS is an uncommon rheumatic disorders consisting of pain, swelling, stiffness, progressive flexion contractures of both hands and thickening of palmar fascia, with erythrosis. Other symptoms are synovitis in the shoulders, wrists, knees and ankles.

Its was first described as a paraneoplastic phenomenon with ovarian carcinoma in 1982.⁴ It has also been described in association with other malignancies, such as cancer of the breast, pancreas, lung, stomach and endometrium,^5–7 and only a few reports were not associated with malignancy.^8,9 More than 40 cases have since been reported. Sung et al¹⁰ summarises all the published cases of PFPAS associated with malignancies up to 2005, and two more cases have been described since then.^11,12

The exact aetiology of PFPAS is still not known. The underlying immunological mechanisms have not been defined. The majority of affected patients have been women, which suggests that the female hormonal state may predispose women to this syndrome as is the case in most autoimmune diseases.

The rheumatic symptoms may precede detection of the tumour by 4–23 months. Previous reports have confirmed improvement in rheumatic symptoms after chemotherapy, except for the digital contractures,¹³ but it is unclear whether this is a result of tumour necrosis, immunomodulation, or anti-inflammatory effects.

There are no specific distinctive diagnostic tests for this disorder, and the diagnosis is therefore based on clinical presentation. As long as the pathomechanisms of PFPAS are unclear, the only effective therapy is cancer treatment, which may lead to arrest or even complete resolution of the rheumatic disease.

As the most frequently reported malignancy associated with PFPAS is ovarian carcinoma (14 patients, 34%),¹³ awareness and recognition of palmar fasciitis and polyarthritis syndrome as a paraneoplastic syndrome may allow for earlier diagnosis and treatment of an occult ovarian carcinoma.

Paraneoplastic syndromes may evolve over weeks to months (more rarely, over 1–3 years) and then may stabilise, regardless of whether the patient’s symptoms improve or get worse, so an early diagnosis is vital to improve patient survival, which in ovarian carcinoma is about 25% at 5 years.

Learning points

• Rapid and correct diagnosis of any paraneoplasic syndrome may lead to clues to the underlying conditions.

• This can avoid diagnostic errors and permit earlier diagnosis and faster treatment.