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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 12  |  Issue : 2  |  Page : 143-146

Pigmented Villo-Nodular Synovitis of Shoulder Joint Leading to Humeral Head Erosion


Department of Orthopaedics, AIIMS, Raipur, Chhattisgarh, India

Date of Submission22-Nov-2020
Date of Acceptance07-Dec-2020
Date of Web Publication28-Dec-2020

Correspondence Address:
Dr. Rudra Narayan Dash
Department of Orthopaedics, AIIMS, Raipur, Chhattisgarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jotr.jotr_81_20

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  Abstract 


Pigmented villo-nodular synovitis (PVNS), also known as giant cell tumor of the tendon sheath or benign synovial histiocytoma, is a benign yet locally aggressive tumor of the synovium that affects both the large joints and the tendon sheaths. The most common site for the tumor includes knees, hip, ankle, and wrist joints; shoulder joint being a very unusual site for the occurrence of this tumor. We present here an unusual case of diffuse PVNS of shoulder joint with eroded humerus head treated with surgical synovectomy and adjuvant radiosynovectomy to prevent recurrence. This case report highlights the natural history of this rare disease due to late presentation, difficulty in the diagnosis, and management of recurrence.

Keywords: Pigmented villo-nodular synovitis, radiosynovectomy, shoulder, synovectomy


How to cite this article:
Agrawal AC, Dash RN, Kar BK, Sakale HS. Pigmented Villo-Nodular Synovitis of Shoulder Joint Leading to Humeral Head Erosion. J Orthop Traumatol Rehabil 2020;12:143-6

How to cite this URL:
Agrawal AC, Dash RN, Kar BK, Sakale HS. Pigmented Villo-Nodular Synovitis of Shoulder Joint Leading to Humeral Head Erosion. J Orthop Traumatol Rehabil [serial online] 2020 [cited 2021 Nov 27];12:143-6. Available from: https://www.jotr.in/text.asp?2020/12/2/143/305089




  Introduction Top


Pigmented villo-nodular synovitis (PVNS), also known as giant cell tumor of the tendon sheath or benign synovial histiocytoma, is a benign yet locally aggressive tumor of the synovium that affects both the large joints and the tendon sheaths. Mostly found as a solitary nodule, this entity has a rarer diffuse variety as well which presents as a multinodular lesion. The most common site for the tumor includes knees, hip, ankle, and wrist joints; shoulder joint being a very unusual site for the occurrence of this tumor.[1] The disease entity itself being very rare has an incidence of around 2 per million population. Conventionally, PVNS can mimic a wide range of malignancies both clinically and radiologically. Coupled with that the rarity of the disease, makes the chances of correct diagnosis at an early stage even more difficult to achieve. The incidence of recurrence is not clear with regard to predisposed age and sex, but it seems to be more prevalent in the age group of 30–40 years. Microscopically, the tumor shows multiple hyperplastic synovial villi along with hemosedrin-laden macrophages, foam cells as well as multinuclear giant cells, thus the name giant cell tumor of the tendon sheath.

In case of PVNS involving the shoulder joint, the usual clinical presentation of this unusual entity is in the form of a gradually increasing mild-to-moderate joint pain with or without a clinically recognisable mass along the joint. In cases of long-standing disease, the joint may become stiff along with the decreased range of motion. Other consequences of delay in surgery such as permanent erosion of bone, progressive rotator cuff tear, and neurovascular invasion are rare but lead to increased disability and poor functional outcome.[2],[3]

We present here an unusual case of diffuse PVNS of shoulder joint with eroded humerus head treated with surgical synovectomy and adjuvant radiosynovectomy to prevent recurrence.


  Case Report Top


A 38-year-old right hand dominant male, farmer by occupation without any prior history of trauma presented to us with a chief complaint of gradually progressive swelling in his right shoulder for the past 4 months along with inability to move the joint freely. There was no associated pain in the joint or anywhere else in the limb. There was no any other swelling in any other joint or anywhere else in the body. There was no history of weight loss, decreased appetite, fatigue, and evening rise of temperature. There was no associated weakness in the distal part of the affected limb.

On a plain radiograph of the affected shoulder, as shown in [Figure 1], the superomedial aspect of the humeral head was not visualized, diffuse heterogeneous swelling was present all around the shoulder joint, the rest all bones appeared normal.
Figure 1: Preoperative plain radiograph of the right shoulder showing erosion of humeral head

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Magnetic resonance imaging (MRI) evaluation yielded nonvisualization of the medial half of the head of right humerus with marginal erosion, subluxation of the gleno-humeral joint along with irregular synovial thickening and enhancement, with suggestive inflammatory changes in the surrounding muscles, the MRI was suggestive of an infective etiology over neoplastic, as shown in [Figure 2] and [Figure 3].
Figure 2: Coronal view of preoperative magnetic resonance imaging

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Figure 3: Sagital section of preoperative magnetic resonance imaging

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The blood profile showed normal hemoglobin levels along with normal total leukocyte counts but raised inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein.

Following this, the patient was counseled, and he underwent joint aspiration from the affected shoulder. 50 ml of sero-sanguineous fluid was aspirated and sent for bacterial culture and sensitivity, tuberculosis polymerase chain reaction, and synovial fluid analysis. All these investigations did not yield anything significant.

The patient underwent triple-phase technitium-99 bone scan which gave an impression of osteosarcoma as there was increased tracer flow and tissue hyperemia in the right upper arm and shoulder region and increased tracer uptake noted in glenoid process, acromion, coracoids process, and humerus up to mid one-third. Positron emission tomography (PET) scan revealed fluorine-18 fluorodeoxyglucose (18F-FDG) avid lytic destructive soft-tissue mass lesion over the head of right humerus and around the right shoulder, with low grade 18F-FDG avid infra-clavicular lymph nodes with no evidence of any distant metastasis anywhere else in the body.

Core needle biopsy was done, the sample yielded a normal bony tissue, with no evidence of granulomatous or neoplastic tissue in the sample. Excisional biopsy with intraoperative frozen section evaluation was planned.

Using the standard anterior approach to the shoulder joint, the tumor mass was exposed and later on full exploration and delineating the extent of tumor which seemed to extend deep into the shoulder joint. Humeral head and glenoid fossa were found to be in an eroded state. Tissue sample was sent for frozen section evaluation which described the lesion as having proliferative synovium with local papillary projections, interspersed multi-nucleated giant cells, and occasional hemosiderin-laden macrophages, suggestive of PVNS. After gaining this information intraoperatively, complete synovectomy was carried out. Immediate postoperative period was uneventful.

Follow up and further course

At 1-month follow-up, the patient had no signs of recurrence, but had limited shoulder range of motion with deltoid muscle atrophy. At 3-month follow-up, the patient came with a complaint of reemergent swelling in the right shoulder. The patient was advised MRI suspecting recurrence of disease. MRI showed a collection in the right shoulder joint space with thickened irregular incomplete septations and nodular areas within the collection suggestive of residual/recurrent disease process. The patient underwent radiosynovectomy with Samarium-153 following which the swelling subsided.

At 8 months follow-up, there is no sign of recurrence. The patient has a good range of motion of right shoulder, as shown in [Figure 4] and [Figure 5]. PET computed tomography done at this visit showed no signs of recurrence.
Figure 4: Functional outcome (External Rotation)

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Figure 5: Functional outcome (Internal Rotation)

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  Discussion Top


PVNS is a benign, locally invasive synovial proliferation of unknown etiology affecting joints, bursae, and tendon sheaths.[4] Malignancy is extremely rare.[5] Knee is the most common joint affected but cases of hip, ankle, and wrist have also been reported. PVNS affecting shoulder joint is a rare clinical entity.

Two growth forms of PVNS, varying in clinical presentation, prognosis and response to treatment have been described in the literature.[6] There is diffuse PVNS which affects the entire synovial lining of a joint is more common and considered more aggressive. Localized PVNS is rare, focal, and slow progressing.

Plain radiographs of patients with diffuse PVNS may appear normal or may reveal joint effusion, soft-tissue edema. In cases where the diagnosis and treatment is delayed sclerosis and bony erosion may be evident. MRI of the affected joint provides a useful noninvasive means of diagnosis and also delineates the extent of the lesion. MRI shows hypodense, diffuse infiltrative lesions involving soft-tissue structures and blooming artifact from hemosiderin.[7],[8] Although sensitivity of MRI is very high, PVNS is sometimes confused with soft-tissue sarcoma or rheumatoid arthritis.[9] In our case, due to delayed presentation, erosion of head of humerus and glenoid was evident in plain radiograph. This led to a suspicion of aggressive bony lesion of proximal humerus. Considering the comparatively high incidence of infective joint pathology in India, MRI was suggestive of infective etiology rather than a neoplastic one. Following intraoperative confirmation of diagnosis by frozen section examination, total synovectomy was attempted.

Complete surgical synovectomy is the treatment of choice for diffuse PVNS. Complete excision may be limited by inaccessibility and proximity to neurovascular bundle. Incomplete removal may be the cause of recurrence. The recurrence rate for diffuse PVNS is up to 46% without adjuvant treatment.[10] In contrast, localized PVNS treated with wide excision has an excellent prognosis and low recurrence rate.

Options for adjuvant therapy include external radiotherapy, intraarticular instillation of radioactive isotope, and cryosurgery.[11] External radiotherapy can rarely cause localized skin changes and fibrosis around the joint. Radiosynovectomy and cryosurgery may sometimes cause joint stiffness and consequent poor functional outcome. However, they do decrease the chances of recurrence.

In our case, the late presentation along with nonspecific complain, erosion of humerus head led us to suspect a more aggressive bony lesion. However, following intraoperative histopathological confirmation of diagnosis, total synovectomy was performed. At 3-month follow-up, the patient showed clinical as well as radiological signs of recurrence, for which intraarticular radioisotope (samarium - 153) instillation was performed. This case report highlights the natural history of this rare disease due to late presentation, difficulty in the diagnosis, and management of recurrence.


  Conclusion Top


Rare disorders of the shoulder such as PVNS are often diagnosed late in the disease process due to nonspecific complaints, diagnostic difficulties, and secondary bony changes consequent of long-standing disease. High degree of clinical suspicion and early use of MRI may lead to earlier diagnosis. However, histopathology examination remains the gold standard. Total surgical synovectomy is the treatment of choice in these cases. Recurrence can be addressed with radiotherapy or cryosurgery. Although benign, these tumors are locally invasive and may cause significant functional impairment. With early diagnosis, complete resection and adjuvant therapy good functional outcome are expected.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Xie GP, Jiang N, Liang CX, Zeng JC, Chen ZY, Xu Q, et al. Pigmented villonodular synovitis: A retrospective multicenter study of 237 cases. PLoS One 2015;10:e0121451.  Back to cited text no. 1
    
2.
Ji JH, Shafi M, Park SE, Kim WY. Subacromial bony erosion: A rare presentation of pigmented villonodular synovitis of the shoulder. Knee Surg Sports Traumatol Arthrosc 2009;17:534-8.  Back to cited text no. 2
    
3.
Gumina S, Carbone S, Campagna V, Castagna A, Della Rocca C, Giannicola G. Pigmented villonodular synovitis of the shoulder associated with massive rotator cuff tear treated by arthroscopic synovectomy and debridement. Musculoskelet Surg 2013;97 Suppl 1:79-84.  Back to cited text no. 3
    
4.
Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented villonodular synovitis, bursitis and tenosynovitis. Arch Pathol 1941;31:731-65.  Back to cited text no. 4
    
5.
Bertoni F, Unni KK, Beabout JW, Sim FH. Malignant giant cell tumor of the tendon sheaths and joints (malignant pigmented villonodular synovitis). Am J Surg Pathol 1997;21:153-63.  Back to cited text no. 5
    
6.
Granowitz SP, D'Antonio J, Mankin HL. The pathogenesis and long-term end results of pigmented villonodular synovitis. Clin Orthop Relat Res 1976:335-51.  Back to cited text no. 6
    
7.
Mandelbaum BR, Grant TT, Hartzman S, Reicher MA, Flannigan B, Bassett LW, et al. The use of MRI to assist in diagnosis of pigmented villonodular synovitis of the knee joint. Clin Orthop Relat Res 1988:135-9.  Back to cited text no. 7
    
8.
Kelly DW, Ovanessoff SA, Rubin J. Localized pigmented villonodular synovitis of the shoulder: A difficult diagnosis of a rare disorder. J Med Diagn Methods 2013;2:1-5.  Back to cited text no. 8
    
9.
Harris O, Ritchie DA, Maginnis R, Lamb GR, Helliwell T, Jane M, et al. MRI of giant cell tumour of tendon sheath and nodular synovitis of the foot and ankle. Foot 2003;13:19-29.  Back to cited text no. 9
    
10.
Mollon B, Lee A, Busse JW, Griffin AM, Ferguson PC, Wunder JS, et al. The effect of surgical synovectomy and radiotherapy on the rate of recurrence of pigmented villonodular synovitis of the knee: An individual patient meta-analysis. Bone Joint J 2015;97:550-7.  Back to cited text no. 10
    
11.
Serra TQ, Morais J, Gonçalves Z, Agostinho F, Melo G, Henriques M. An unusual case of diffuse pigmented villonodular synovitis of the shoulder: A multidisciplinary approach with arthroscopic synovectomy and adjuvant radiotherapy. Eur J Rheumatol 2017;4:142-4.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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