|Year : 2020 | Volume
| Issue : 2 | Page : 153-155
Focal Pigmented Villonodular Synovitis in the Hip Joint – A Rare Case Report
QA Qureshi1, Sharmila Dudani2
1 Department of Orthopedics, Hamdard Institute of Medical Sciences and Research, New Delhi, India
2 Department of Pathology, Army College of Medical Sciences, New Delhi, India
|Date of Submission||09-Nov-2019|
|Date of Acceptance||27-Jul-2020|
|Date of Web Publication||28-Dec-2020|
Dr. Sharmila Dudani
Department of Pathology, Army College of Medical Sciences, Delhi Cantt, Delhi - 110 010
Source of Support: None, Conflict of Interest: None
Pigmented villonodular synovitis is characterized by villous/nodular proliferation of the synovial membrane affecting commonly the knee joint. Involvement of the hip ankle, shoulder, and elbow joint is distinctly uncommon. Young adults are mainly affected and present with localized pain with/without joint stiffness and swelling. Etiology thought to be due to synovial hyperplasia following chronic inflammation. The role of trauma is controversial. Radiography and computed tomography scan are not diagnostic in the early stages. MRI is useful in the diagnosis and follow-up. Histology reveals nodular proliferation lined by hyperplastic synovium beneath which are sheets of histiocytes, hemosiderin laden macrophages and osteoclast-like giant cells. Colony-stimulating factor 1 gene overexpression is seen in histiocytic cells. Syniovectomy is the mode of treatment. Total hip arthroplasty may be required for extensive disease. A high index of clinical suspicion is required to diagnose this rare disease to prevent the limitation of joint mobility and destruction.
Keywords: Hemosiderin-laden macrophages, hip joint, pigmented, villonodular synovitis
|How to cite this article:|
Qureshi Q A, Dudani S. Focal Pigmented Villonodular Synovitis in the Hip Joint – A Rare Case Report. J Orthop Traumatol Rehabil 2020;12:153-5
|How to cite this URL:|
Qureshi Q A, Dudani S. Focal Pigmented Villonodular Synovitis in the Hip Joint – A Rare Case Report. J Orthop Traumatol Rehabil [serial online] 2020 [cited 2021 Jan 20];12:153-5. Available from: https://www.jotr.in/text.asp?2020/12/2/153/305085
| Introduction|| |
Pigmented villonodular synovitis (PVNS) is a rare condition affecting 1.8 per million inhabitants characterized by villous/nodular proliferation of the synovial membrane which may affect any joint, bursa, or tendon sheath. The knee is the most common joint affected (70%), with the hip, ankle, shoulder, and elbow being uncommonly involved. It occurs in 2 forms: diffuse and focal, the latter being rare. Most cases occur in the second and third decades; however, occurrence from 7 to 80 years has been reported., Data regarding PVNS of hip are scarce because of the uncommon nature of disease. This case report highlights an unusual case of focal PVNS of hip joint occurring in a young adult male.
| Case Report|| |
A 34-year-old healthy male complained of acute pain and joint stiffness localized to the right hip joint for 2 weeks. Pain did not radiate, and swelling was absent. The patient did not give any history of trauma and did not complain of fever/chills. There was no significant medical history. Magnetic resonance imaging (MRI) done was suggestive of localized pigmented nodular synovitis of the right hip joint with moderate joint effusion. The femoral head, neck, and acetabulum had preserved articular surfaces [Figure 1]. Syniovectomy was done, and brownish irregular tissue bits measuring 2 cm × 1 cm was excised. Histology showed nodular tissue lined by mildly hyperplastic synovium with fibrosis and sparse lymphocytic infiltrate. Histiocytic aggregates, hemosiderin-laden macrophages, and osteoclast-like giant cells were present [Figure 2]. No granuloma formation/necrosis/atypia was seen. The diagnosis of PVNS was confirmed. The patient had an uneventful postoperative recovery and doing well 4 months postsurgery.
|Figure 1: Well-circumscribed, oval nodular lesion measuring 29.1 mm × 19.8 mm × 12.7 mm appearing hypointense on PD FS images and mildly hyperintense on T1-weighted images with peripheral gradient blooming on MERGE images. Fluid seen in Rt hip joint space distending the joint capsule|
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|Figure 2: (a) Nodular tissue showing sheets of histiocytes × 40, (b and c) osteoclast giant cells, histiocytic cells (×400), (d) hemosiderin-laden macrophages|
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| Discussion|| |
PVNS is a rare proliferative lesion of synovial tissue that was first described by Chassaignac in 1852. However, Jaffe coined the term of villonodular synovitis in 1941. Involvement is usually monoarticular with the knee being the most common affected joint, but the rare cases of multiple joint involvement may exist.
The clinical presentation of PVNS is nonspecific with localized pain being the most common symptom which may progress in intensity with the duration of disease. Pain may be localized to the area of the hip but may radiate to groin in some cases. Chronic swelling, limitation of joint mobility, and joint stiffness have also been reported. Our patient presented with a complaint of localized pain in the hip of a short duration without any functional limitation or swelling. This is attributable to early diagnosis as functional limitation occurs late when the intracapsular space has been significantly reduced secondary to joint invasion. The nonspecificity of clinical symptoms and noncontributory radiographic findings in early stages of disease usually delay the diagnosis. The average time between the onset of disease and diagnosis is reported as 4 years.,
No gender prevalence has been reported in the literature, but Ma et al. found females to have a higher prevalence (64%) as compared to males.
PVNS belongs to the family of tenosynovial giant-cell tumors, but the etiology is poorly understood. Many researchers believe that trauma plays a major role and synovial proliferation occurs following chronic inflammation secondary to chronic trauma., However, Flipo et al. found no such association. Recently, Trisomy 5 and 7 have been reported in the some cases of PVNS. Translocations involving colony-stimulating factor 1 (CSF1) gene have been described with t (1;2)(p13;q37) translocation, leading to the formation of COL6A3-CSF1 fusion gene. The overexpression of CSF1 gene in the neoplastic cells leads to the recruitment of inflammatory cells within the tumor. Abnormal local lipid metabolism has also been postulated.
Radiography is usually normal and not helpful in the early stages of the disease. Marginal joint erosions and cystic changes may be seen in chronic cases. CT scan is infrequently used as the synovium, and the surrounding soft tissues are poorly delineated. MRI is the preferred diagnostic modality for its accuracy in the diagnosis and follow-up postsurgery or recurrence. In a series of 122 cases of PVNS reported by Ottaviani et al., MRI was diagnostic in 83% of cases.
PVNS commonly occurs in the diffuse form. The localized form is well-defined, lobulated, and surrounded by a fibrous capsule. The lesion is composed of sheets of histiocytes which may be round/polygonal cells with a reniform nucleus and abundant pale, foamy cytoplasm admixed with hemosiderin-laden macrophages and osteoclast-like multinucleate (10–70 nuclei) giant cells. Plasma cells and lymphocytes are usually sparse. The histiocytes tend to form clusters in the periphery of the nodules and may be associated with cholesterol clefts. Occasional mitotic figures can be seen, but atypical mitoses are never present. Hyalinization of stroma may be seen in the localized form. Necrosis is always absent.
Hemosiderin-laden macrophages are an important feature differentiating PVNS from other types of proliferative synovitis. Hemosiderotic synovitis is a close differential diagnosis seen in patients with hemophilia who have the episodes of intraarticular bleeding. Here, the pigment is seen in the synovial-lining cells without any nodular proliferation and absence of both mononuclear and giant cells. In doubtful cases, immunohistochemistry for the expression of histiocytic markers such as CD68 and CD45 can be elicited.
Due to their similar histological appearance, a differential diagnosis between giant cell tumor of tendon sheath and PVNS can be difficult on biopsy. Integration with clinical and radiological data is thus essential.
Surgical treatment (open method or arthroscopic) is the best option for diffuse and localized PVNS. Synovectomy is done for excising the diseased synovium. In PVNS of the hip joint, arthroscopic approach plays a limited role. Total hip arthroplasty is done in cases of failure of surgical treatment, avascular necrosis of femoral head, femoral neck fracture, or osteoarthritis.
Recurrence has been reported in diffuse PVNS. Low-to-moderate external-beam radiation is suggested for residual disease or recurrence. Targeted therapy against CSF 1 receptor may pave the way for a novel approach in the management of this disease.
A high index of clinical suspicion and correlation with imaging/biopsy are paramount for an early diagnosis to prevent the limitation of joint mobility and destruction.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]