Seminars in Arthritis and Rheumatism ] (2014) ]]]–]]]

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Palmar fasciitis and polyarthritis syndrome—Systematic literature review of 100 cases Bernhard Manger, MDn, Georg Schett, MD Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany

a r t i c l e in fo

Keywords: Palmar fasciitis Arthritis Paraneoplastic Ovarian cancer Adenocarcinoma

a b s t r a c t Objective: To analyse clinical, laboratory, and imaging characteristics of all patients with palmar fasciitis and polyarthritis syndrome (PFPAS) described in the literature. Method: Comparison of the clinical presentation of one patient with acute onset of PFAPS with 99 other published cases identified through a PubMed literature research. Results: Since the original description in 1982 by Medsger et al., there have been numerous case reports and small case series in the literature. In total, 73 articles in English, French and Spanish language were included in the analysis. PFPAS is a rare but characteristic paraneoplastic syndrome in rheumatology. Its distinct clinical feature is a painful swelling of both the hands caused by an inflammation of the palmar fascia, tendon sheaths and small joints of fingers and wrist, and flexion contractures develop rapidly. Since the subcutaneous tissues become indurated and hard, the illustrative term “woody hands” was coined. The most frequent underlying malignancy is ovarian cancer but adenocarcinomas of the breast, gastrointestinal tract and other organs can also cause this syndrome. A helpful diagnostic procedure in order to identify the nature of the underlying malignancy in many cases has been the determination of various serum tumour markers. In cases when a complete removal of the malignancy is possible, PFPAS can also undergo complete remission. Conclusions: Knowledge of the distinct features of this rare paraneoplastic syndrome facilitates early diagnosis and potentially life-saving therapeutic interventions. & 2014 Elsevier Inc. All rights reserved.

Paraneoplastic syndromes are cancer-associated diseases or symptoms that occur distant from the underlying malignancy. They are not directly caused by the tumour or its metastases but are mediated by humoral factors, such as hormones and cytokines, or they are a consequence of the immune response against tumour cells. Various musculoskeletal conditions can be triggered by a malignant process: periostitis, arthritis, fasciitis, myositis, panniculitis or vasculitis can be a paraneoplastic symptom and therefore of interest to the rheumatologist [1]. The observation of a patient with acute severe palmar fasciitis and polyarthritis prompted us to conduct a systematic literature research to analyse all patients from case series and single case reports described up to this date. The first description of a patient with palmar fasciitis and arthritis of finger joints in association with metastatic ovarian carcinoma by Bremer [2] dates back to 1966. However, despite its symmetric presentation, these symptoms were then designated “shoulder–hand syndrome”, a variant of reflex sympathetic

n Correspondence to: Medizinische Klinik 3, Krankenhausstr 12, D-91054 Erlangen, Germany. E-mail address: [email protected] (B. Manger).

http://dx.doi.org/10.1016/j.semarthrit.2014.03.005 0049-0172/& 2014 Elsevier Inc. All rights reserved.

dystrophy or complex regional pain syndrome. It was not until 1982, when Medsger et al. [3] reported about six postmenopausal women, who developed very similar symptoms as a consequence of malignant ovarian tumours. In this article the term “palmar fasciitis and polyarthritis syndrome” (PFPAS) was created, which allowed to recognise this as a rare but characteristic paraneoplastic disease entity in rheumatology.

Materials and methods To analyse all published patients with PFPAS, we conducted a systematic literature research. A PubMed search using “palmar” and “fasciitis” for the years 1982–2013 retrieved 76 articles. All were screened in full-text version and 58 were included in the analysis, because they reported individual patient cases. All relevant articles were retrieved and additional references quoted in these articles were checked. This led to the inclusion of 14 additional full-text articles in English, French and Spanish. These 72 articles together with the initial report by Bremer [2] describe the clinical characteristics of 99 patients with PFPAS. All cases were reviewed as full-length articles in original language, and both the

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Two months after the initial onset of musculoskeletal symptoms, CT examination showed a mass of the left ovary with signs of peritoneal and omental metastases. Hysterectomy and bilateral ovarectomy with removal of peritoneal, omental and para-aortal metastases revealed a poorly differentiated serous adenocarcinoma. The patient received postoperative chemotherapy with carboplatin and paclitaxel, but this did not influence her musculoskeletal symptoms; the tumour progressed and she died only about 2 months later. Literature review

Fig. 1. Dorsal aspects of hands of a 73-year-old patient with a sudden onset of extremely painful bilateral hand swelling with rapid development of flexion contractures.

authors classified these cases as PFPAS based on the major clinical symptom of palmar fasciitis supported by additional clinical, laboratory, imaging and histopathological data. This makes the patient recently seen in our department the 100th observation of this syndrome in the literature. In 87 patients, PFPAS was associated with an underlying malignancy [2–67], in 10 it was attributed to benign conditions [68–70] or to medication [71] and in three it remained idiopathic [72–74]. The following results will analyse the findings in all 87 cases of paraneoplastic PFPAS, the 13 cases without malignant disease will be discussed separately below.

Results Case report A 73-year-old female farmer experienced an acute onset of very painful symmetrical swelling of both the hands with stiffness and diffuse arthralgias in the elbows and the knees. In addition, she suffered from fatigue and reported a weight loss of 2 kg within the last 6 weeks. A presumptive diagnosis of initial systemic sclerosis was made but therapy with 50-mg prednisone per day had no effect at all and flexion contractures with bilateral palmar induration developed rapidly (Figs. 1 and 2). Magnetic resonance imaging showed palmar fascial and peritendinous signal enhancement and gadolinium uptake in tendon sheaths of flexor and extensor tendons.

Musculoskeletal symptoms In most of the reported cases, PFPAS presents with a sudden onset of diffuse painful swelling of both the hands with marked stiffness. Later nodular thickening of the palmar fascia develops similar to Dupuytren's contracture, but much more severe. Overall, 20% of the reported cases exhibit a similar involvement of the plantar fascia. The skin of the involved area is shiny and tight, and some case reports describe an erythematous or acrocyanotic discoloration. However, there is only one description of Raynaud's phenomenon [43], sclerodactyly is absent and capillary microscopy findings are normal. Some authors describe the palpatory findings in an advanced stage with the illustrative term “woody hands” [29,42,56]. Occasionally a “groove sign” of involved areas of the hands can be seen, which is an indentation of the skin over superficial veins, when the extremity is elevated [35,52]. The bilateral “woody hands”, flexion contractures and nodular palmar fasciitis of our patient are shown in Figures 1 and 2. The polyarthritis presents with arthralgias and synovitis of metocarpophalangeal and proximal interphalangeal joints and wrists. Progressive flexion contractures lead to a rapid loss of hand function. PFAPS in most cases shows symmetrical involvement of fascia and joints, but occasionally strictly [33,59] or predominantly [46,55,65] unilateral manifestations have been reported. Arthritides of other joints are frequent but usually considerably milder than those of hands and fingers. The shoulders are most frequently involved, which can present as adhesive capsulitis with a markedly reduced range of motion. Before the concept of PFPAS as a separate disease entity was generally accepted, often these patients were diagnosed with “shoulder–hand syndrome” [2,6,9] or reflex sympathetic dystrophy [7,10,13,17]. In eight patients carpal tunnel syndrome was present as a complication of their wrist involvement [3,23,33,35,40,41,43]. Other frequently involved joints are the elbows, knees and ankles. In four patients with paraneoplastic PFAPS, arthrocentesis was performed because of knee synovitis with effusion [33,35,38,58]. Type of underlying malignancy and epidemiology

Fig. 2. The palmar nodular fasciitis resembles Dupuytren's contracture but is in contrast much more severe and inflammatory. The atrophic scar at the tip of the index finger stems from an earlier injury and is not caused by the PFPAS disease process.

In 36.8% of women, by far the most frequent tumour type in paraneoplastic PFPAS is ovarian adenocarcinoma. Ovarian and breast cancer together with malignancies of other female reproductive organs are the underlying cause in more than half of all published cases (Table 1). This accounts for the fact that PFPAS has been described in females more than four times more frequent than in males. This observation has often been used as an argument for a pathophysiological role of female sex hormones in the pathogenesis of this paraneoplastic syndrome. However, when all patients with malignancies of female reproductive organs, breast cancer and prostate cancer are excluded from the analysis there remains only a small female preponderance of 1.3–1 for all other tumour types. Interestingly and in contrast to some other paraneoplastic syndromes, only five PFPAS patients with hematolymphatic malignancies have been described. In 73.8% of all cases, by far the most frequently occurring histological type was adenocarcinomas, for which detailed

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Table 1 Location and type of malignancies in 87 patients with paraneoplastic PFAPS Location

n

Percentage

Ovary

32

36.8

Other female reproductive organs Fallopian tube (n ¼ 2) Uterus (n ¼ 3) Cervix (n ¼ 2)

7

12.4

Other urogenital organs Prostate gland (n ¼ 2) Renal pelvis (n ¼ 1) Urinary bladder (n ¼ 1)

4

4.6

Breast

8

9.2

Lung Non-small cell carcinoma (n ¼ 4) Small cell carcinoma (n ¼ 1) Adenocarcinoma (n ¼ 1)

6

6.9

17

19.6

Gastrointestinal organs Pancreas (n ¼ 7) Colon (n ¼ 3) Gastroesophageal adenocarcinoma (n ¼ 5) Hepatocellular (n ¼ 1) Bile duct (n ¼ 1)

Fig. 4. Distribution of time lag between onset of PFAPS symptoms and detection of malignancy.

Chondrosarcoma

1

1.1

Hematolymphatic malignancies Chronic lymphatic leukaemia (n ¼ 1) Hodgkins's disease (n ¼ 1) Plasmocytoma of thyroid gland (n ¼ 1) Light chain multiple myeloma (n ¼ 1)

4

4.6

Multiple malignancies Chronic myelogenous leukaemia and clitoris (n ¼ 1) Urinary bladder, lung and colon (n ¼ 1)

2

2.3

Cancer of unknown primary origin

6

6.9

histological information was available. The percentage of tumours that were characterised as poorly differentiated or undifferentiated was 24.7%. In more than two-thirds of all patients, the tumour was diagnosed already in an advanced stage of metastatic spreading. Substantial weight losses of 10 kg and more have been described in a significant number of patients. The majority of all paraneoplastic PFPAS patients experience the onset of symptoms between the sixth and eighth decade of life (Fig. 3). The median age at onset was 60.5 years. The youngest patient was 25 years old, when she was diagnosed with a serous papillary adenocarcinoma of the ovary, and the oldest had uterine adenocarcinoma at an age of 92 years [28,33]. In 71.8% of all cases, musculoskeletal symptoms preceded the diagnosis of malignancy by a median of 6 months. In five patients,

the onset of rheumatic and tumour symptoms was simultaneous, or PFPAS symptoms led to the immediate detection of an underlying neoplasm (Fig. 4). In some patients, the malignancy had already been diagnosed when musculosketal symptoms started. In these cases, PFPAS often was associated with tumour progression [5,40,58,61] or lymph node metastases [6,18,30], with intervals of 1–9 years after the initial manifestation of the neoplasm. In others, PFPAS started during chemotherapy [3,6,7,47] or occasionally even without any clinical evidence for any residual malignant tissue [4,51]. In some cases, interesting comorbidities have been reported in PFPAS patients. Three had other paraneoplastic phenomena that presented simultaneously with the musculoskeletal manifestations: dermatomyositis [16], digital clubbing [55] or bilateral uveal melanocytic proliferation [48]. Other patients had benign neoplasias in addition to their malignant process, such as thyroid [13] or parathyroid adenoma [11], or prolactinoma [18], and some presented with a history of autoimmune diseases such as Hashimoto's thyroiditis [3], multiple sclerosis [33] or rheumatoid arthritis [61]. One PFPAS patient additionally developed signs of Peyronie's disease [49], another had an achalasia-like oesophageal involvement [30]. Laboratory investigations Parameters of inflammation show a great range of variability in PFAPS. Erythrocyte sedimentation rate was less than 20 mm/h in

Fig. 3. Distribution of the age of onset of paraneoplastic PFPAS for all patients (full bars) and those with ovarian cancer (hatched bars).

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almost half of the patients (49.1%), 30.9% had a moderate increase up to 50 mm/h, 10.9% had values between 50 and 100 mm/h and 9.1% more than 100 mm/h. C-reactive protein level was elevated above 5 mg/l in only 35% of the reported cases with a maximal value of 31 mg/l [64]. Rheumatoid factors were mainly negative or weakly positive in only four cases [3,36,47,49], antibodies against citrullinated proteins have not been found. Antinuclear antibodies (ANA) tests were negative in 91.8% of all patients. Only in five ANA titres of more than 1:100 were reported, with 1:8000 and 1:10,000 being the highest values at the start of symptoms [43,64]. No ANA subtype specificities or alterations of complement factors have been found in any of the reported cases. A marked elevation of the soluble interleukin-2 receptor serum level was observed in a single case [36]. Synovial fluid analysis in four cases revealed an inflammatory constellation with leucocyte counts up to 40,000 per mm3 [35]. A predominance of polymorphonuclear cells [33], of lymphomonocytes [58] or a balanced ratio [38] has all been described. An analysis of various tumour markers is reported in the literature for 22 patients (Table 2). Of the 22 patients, 19 showed an increase in at least one of these markers, most frequently of CA125 in association with malignancies of female reproductive organs. Three patients had paraproteinemia: one patient with multiple myeloma had κ-light chains [41], and one with plasmocytoma of the thyroid gland had a monoclonal IgGλ paraprotein detected by immune electrophoresis [16]. In addition to her ovarian cancer, one patient had a history of monoclonal IgGκ gammopathy of unknown significance [64].

calcinosis, most likely unrelated to PFPAS [33,46,65]. Overall, 35.2% of all hand radiographs exhibited diffuse or juxta-articular osteopenia. Small erosions of carpal, carpometacarpal and interphalangeal joints were described in the three patients [3,43,63] and acroosteolytic lesions of distal phalanges in one [64]. The patient with light chain multiple myeloma had osteolyses of the proximal phalanges [41]. Predominantly in older publications, the results of scintigraphic bone scans are reported, which demonstrate a moderate increase of tracer uptake in smalls joints of the hands and the wrists [3,14,19,23,40] as well as in tender large joints, such as the shoulders, knees and hips [3,33,36]. Only two recent publications provide a detailed description of sonographic findings in four patients with this syndrome. Due to flexion contractures, musculoskeletal ultrasound may be difficult to perform in individual cases, but it clearly demonstrates the presence of wrist and finger synovitis as well as tenosynovitis of flexor and extensor tendons. Power Doppler signal intensity documents moderate to severe activity of the inflammatory lesions [64]. In addition, hypoechoic nodules with a diameter of 0.7–2.5 mm within the palmar fascia were described [65]. MRI studies show altered signal intensities in T1- and T2weighted images within the fascial and subcutaneous tissues corresponding to fibromatosis [43,46,60,65]. Axial T2-weighted images may demonstrate tenosynovial enhancement of flexor and extensor tendons [64,65]. Histology

Imaging In 51 of paraneoplastic PFPAS cases, there is information about conventional X-rays of both the hands and occasionally other involved joints. In 47.1% of those in whom no pathologic findings were reported, three patients had osteoarthritis and/or chondro-

Many case reports show remarkably similar findings obtained by biopsies of skin, subcutaneous and fascial tissues. The epidermis is not involved; fibroblast proliferation and marked interstitial fibrosis begins in the dermis and stretches into subcutis, fascia and peritendineum. A scattered, perivascular, predom-

Table 2 Tumour markers in patients with various malignancies and PFPAS Type of malignancy

Tumour marker

Value

Normal

References

Sigmoidal adenocarcinoma Colonic adenocarcinoma

CEA CEA CA19-9 PSA CA125 CA19-9 hCG NSE CA15-3 AFP CA125 CA125 CA125 CA27-29 CEA CA125 CA125 CA19-9 CA15-3 All markers All markers CA125 CA125 CA125 CA125 CA19-9 PSA CA125

Normal 290 mg/l 74000 U/ml 6 mg/l 1478 U/ml 451 U/ml 9.5 U/l 26.3 mg/l Elevated 8.8 mg/l 240 U/ml 120 U/ml 188.8 U/ml 65.6 U/ml Elevated 3000 U/ml 470 U/ml Normal Normal Normal Normal 120 U/ml 1516 U/ml 1477 U/ml 1410 U/ml 157 U/ml 13.6 mg/l 3238 U/ml

o5 mg/l o5 mg/l o37 U/ml o4 mg/l o35 U/ml o37 U/ml o5 U/l o12.5 mg/l o25 U/ml o5 mg/l o35 U/ml o 35U/ml o35 U/ml o38 U/ml o5 mg/l o35 U/ml o35 U/ml o37 U/ml o25 U/ml

[14] [15]

Prostate cancer Extraovarian adenocarcinoma Ovarian adenocarcinoma

Breast cancer Hepatocellular carcinoma Endometrial adenocarcinoma Ovarian adenocarcinoma Ovarian adenocarcinoma Ovarian adenorcinoma Serous papillary carcinoma of fallopian tube Ovarian metastasis of pancreatic adenocarcinoma

Non-small cell lung cancer Pancreatic carcinoma Ovarian adenocarcinoma Ovarian adenocarcinoma Ovarian adenocarcinoma Ovarian adenocarcinoma Pancreatic carcinoma Prostate cancer Ovarian adenocarcinoma

o35 U/ml o35 U/ml o35 U/ml o35 U/ml o37 U/ml o4 mg/l o35 U/ml

CEA: carcinoembryonic antigen; hCG: human chorionic gonadotropin; PSA: prostate-specific antigen; NSE: neuron-specific enolase; AFP: alpha fetoprotein.

[18] [19] [22]

[30] [38] [40] [42] [43] [43] [44] [45]

[49] [51] [52] [54] [64] [64] [65] [66] Our patient

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inantly mononuclear cell infiltrate is also a consistent finding [5,11,25,36,43,50,54,57]. Two reports describe deposits of IgG between the dense collagenous tissue fibres detected by immunofluorescence [8,12]; however, this finding was not confirmed by other authors [18,23]. In only one case, a dermal leukocytoclastic vasculitis was described and IgM and C3 were demonstrated within vessel walls [28]. The patient with light chain multiple myeloma showed amyloid deposits in addition to fibroblast proliferation and fibrosis [41]. PFAPS associated with non-malignant conditions PFPAS has also been reported in patients with benign ovarian conditions: one in association with ovarian endometrial cyst [68] another after removal of a paraovarian cyst of paramesonephric origin [69]. In addition, one patient with PFPAS in association with meningioma has been described [70]. Seven patients developed palmar fasciitis during antituberculous chemotherapy. In most cases this could clearly be attributed to ethionamide, a tuberculostatic drug no longer used in routine treatment, and symptoms resolved with its discontinuation [71]. Finally, three cases of “idiopathic PFPAS” without detection or development of any malignant disease despite long follow-up periods were identified [72–74].

Discussion Initially, PFAPF was considered a variant of reflex sympathetic dystrophy [7,10,13,17], now complex regional pain syndrome (CRPS), or “shoulder–hand syndrome” [2,6,9] in cases with shoulder–joint involvement. Indeed, the physical findings of pain, restriction of motion and vasomotor disturbances bear resemblance with CRPS. By contrast, PFPAS leads to bilateral involvement in almost all cases, frequently follows a more inflammatory and aggressive course, and there is no trauma or neurovascular link detectable in the patient's history [9,11,57]. In 1982, palmar fasciitis was recognised as the key symptom of this paraneoplastic syndrome [3] and over time increasing numbers of cases of PFPAS without shoulder involvement were seen. Palpation of the swollen and taut hands reveals their hardness, a fact that has led to the term “woody hands” similar to “woody arms or legs” in eosinophilic fasciitis. Even a “groove sign” similar to that of eosinophilic fasciitis has been described in PFAPS [35,52]. This is due to the fact that in both diseases inflammation and fibrosis of the fascia is the predominant pathologic process. Eosinophilic fasciitis, however, spares the hands and feet and no blood or tissue eosinophilia has ever been described in PFPAS. Dupuytren's contracture also presents as nodular palmar fibrosis but lacks polyarthritis, digital swelling, and is much less painful or progressive than PFPAS [57]. In early stages of diffuse swelling confusion with systemic sclerosis is possible [21,29,46], but PFPAS does not lead to sclerodactyly and spares the tips of the fingers. Also there is usually no Raynaud's phenomenon or visceral involvement, and capillary microscopy is normal. Occasionally, ANA have been described in PFPAS, but never antibodies against centromers or topoisomerase I [43,64]. If polyarthritis is the predominant initial symptom, patients might be diagnosed with rheumatoid arthritis or RS3PE syndrome. The further course of the disease with progressive palmar fibrosis and the lack of response to steroids usually lead to a quick reversal of these diagnoses [34]. Treatment of PFPAS with non-steroidal anti-inflammatory drugs usually does not lead to sufficient pain control. The effect of glucocorticoids even in high doses is equally disappointing. In the majority of cases there is no effect at all, sometimes an

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improvement of tenderness and swelling but persistence of fibrosis has been reported. Only in a few instances, glucocorticoids had a favourable effect [4,10,38,39]. Also all attempts with other disease-modifying anti-rheumatic drugs, such as methotrexate [51,57,66], hydroxychloroquine [33,64], sulfasalazine [64], D-penicillamine [11,18] or adalimumab [66] were disappointing. At the time of manifestation of PFPAS the malignancy in most cases has already advanced to a late metastatic stage. Therefore it is not surprising that the general outcome is poor and many patients die from their tumour within 1 year after diagnosis. In 70 patients, we have information about the influence of various antitumour therapies on the musculoskeletal symptoms. Almost half of them (48.6%) do not show any response to therapy, whereas 41.4% have a moderate improvement normally consisting in a reduction of inflammation but persistence of fibrosis and contractures. Only in seven cases a complete remission of paraneoplastic PFPAS has been seen. In three of them, a surgical resection of the primary tumour in a pre-metastatic stage was possible (colon, stomach and lung cancers) and the following fast regression of PFPAS manifestations provided the ultimate proof of their paraneoplastic nature [10,36,48]. In four additional cases, a good response of the tumour to chemotherapy was paralleled by a remission of musculoskeletal symptoms [8,21,37,45]. The pathogenesis of this paraneoplastic syndrome is unknown. Because fibroblast proliferation and increased production of extracellular matrix components is a key histologic feature of PFPAS, the role of soluble stimulators of fibroblast activity, such as transforming growth factor β or connective tissue growth factor (CTGF), has repeatedly been proposed [3,57,67]. Indeed a significantly increased CTGF serum level has been found in one patient with PFPAS [52], but this observation still needs to be confirmed in other patients. An interesting observation was made in a patient with gastroesophgeal carcinoma, who received postoperatively an experimental treatment with the broad-spectrum matrix metalloproteinase (MMP) inhibitor marimastat in a Phase III study. During this therapy, PFPAS symptoms appeared, regressed with interruption and reappeared with the reintroduction of marimastat. This could indirectly suggest that a decreased activity of certain MMPs may be involved in the pathogenesis of PFPAS [39]. The role of immune mechanisms in this syndrome remains obscure. The detection of IgG deposits in the involved tissues in some cases [8,12] was not confirmed by others [18,23], but lymphocytic infiltration is a regular histological feature and the observation of a markedly elevated soluble interleukin-2 receptor serum level in one case also points in the direction of lymphocyte activation [36]. Cross-reactivity of the cellular immune response against tumour cells might play a role in the pathogenesis of the inflammation of the palmar fascia and adjacent structures.

Conclusion PFPAS is a rare but characteristic paraneoplastic syndrome in rheumatology. Its distinct feature is an acute painful swelling of both the hands caused by palmar fasciitis, tenosynovitis and arthritis of small finger and wrist joints. Flexion contractures develop rapidly and carpal tunnel syndrome can occur. Because of the induration and hardening of subcutaneous tissues, the illustrative term “woody hands” was coined. Larger joints can also get involved in the course of the disease. The most frequent underlying malignancy is ovarian cancer, but adenocarcinomas of the breast, gastrointestinal tract and other organs can also cause this syndrome. Inflammatory parameters can be elevated but autoimmune serology does not provide any additional diagnostic information. The determination of serum tumour markers can in contrast be helpful to identify the underlying malignancy. At least

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one tumour marker (most frequently CA125) has been reported elevated in 19 out of 22 patients reported in the literature. In the majority of PFAPS cases, the tumour is already present in an advanced stage and musculoskeletal symptoms are irreversible. When a complete removal of the malignancy is possible; however, PFPAS can also undergo complete remission. This systematic literature review summarises the clinical, laboratory and imaging characteristics of all 100 patients described so far in order to facilitate diagnosis and clinical management of patients with this syndrome.

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Palmar fasciitis and polyarthritis syndrome-systematic literature review of 100 cases.

To analyse clinical, laboratory, and imaging characteristics of all patients with palmar fasciitis and polyarthritis syndrome (PFPAS) described in the...
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