Pigmented Villonodular Synovitis

Author: Anay Patel

Topic updated on 03/07/13 11:31pm

Introduction

PVNS is an idiopathic monoarticular reactive synovial disease
- characterized by exuberant proliferation of synovial villi and nodules
Epidemiology
- demographics
  - most commonly in adults age 30-50 but can occur at any age
    - prior test question patient ages have been 16, 30, 31, 37 years
  - equal incidence in men and women
- location
  - may be localized (intra-articular or classic form)
  - knee is the most common site of involvement (80%)
    - other involved sites include hip, shoulder, and ankle
  - can be diffuse (extra-articular extension)
    - when extra-articular known as giant cell tumor of tendon sheath
      - occurring along tendon sheaths of hands and feet
Pathoanatomy and etiology
- half of patients report prior history of trauma to afflicted region
  - thought to be a reactive process
Genetics
- 5q33 chromosomal rearrangement
- increased expression of CSF1 gene
Prognosis
- local recurrence is common

Presentation

History
- 50% of patients will have a prior history of trauma to the area
Symptoms
- pain and swelling
- mechanical pain and limited motion
- recurrent atraumatic hemarthrosis is hallmark of disorder
Physical exam
- intra-articular form (classic PVNS)
  - evaluate for joint effusion and erythema
- extra-articular form (giant cell tumor of tendon sheath)
  - palpable masses seen along tendon sheath
  - painless
  - soft

Imaging

Radiographs
- may show cystic erosion with sclerotic margins on both sides of the joint
CT
- may show cystic erosions on both sides of the joint
MRI
- provides excellent delineation of intra-articular and extra-articular disease
- low signal intensity on both T1 and T2
  - due to hemosiderin deposits
- presence of fat signal (T1) within the lesion
- can evaluate extra-articular extension of an intra-articular process
  - commonly see posterior extension outside of the knee joint
- differential diagnosis
  - similar presentation to inflammatory synovitis, synovial chondromatosis, and hemophilia

Studies

Arthrocentesis
- grossly bloody effusion
Arthroscopy (gross appearance)
- brownish or reddish inflamed synovium is typical of PVNS
- frond like pattern of papillary projections
Biopsy
- synovial biopsy should be performed if there is any doubt of the diagnosis
Histology
- mononuclear stromal cells infiltrating the synovium
- highly vascular villi with hyperplastic synovial cells
- hemosiderin stained multinucleated giant cells
- pigmented foam cells (lipid-laden histiocytes)
- mitotic figures common

Treatment

Nonoperative
- obserrvation
  - indications
    - minimal role for nonoperative treatment if disease is symptomatic
Operative
- total synovectomy
  - indications
    - in grossly symptomatic and painful disease
  - technique
    - intra-articular disease
      - techniques range from arthroscopic partial synovectomy to fully open total synovectomy
      - dependent on extent and location of disease
      - frequent recurrence is common
      - mostly due to incomplete synovectomy
    - extra-articular
      - marginal excision is adequate for giant cell tumor of tendon sheath
      - recurrence (which is common) is treated with repeat excision
  - external beam irradiation
    - when combined with total synovectomy reduces rate of recurrence to 10-20%

Techniques

Arthroscopic synovectomy
- generally performed through routine arthroscopic portals for knee, ankle, and shoulder
- technique
  - perform as thorough resection of synovium as possible
  - excellent for focal or limited PVNS
- postoperative
  - generally treat with some rest after procedure
- pros
  - minimally invasive approach
  - quick return to function
- cons
  - unable to access the posterior portions of the joint
  - unable to address extraarticular disease
Arthroscopic synovectomy combined with open posterior synovectomy (knee)
- arthroscopic portion as above to address anterior disease
- approach
  - posterior approach to the knee via transverse or S-shape incision across popliteal fossa
  - approach between heads of gastrocnemius
  - retract neurovascular bundle to access posterior joint capsule
- technique
  - disease is often see posterior and extra-articular to the knee
  - complete posterior synovectomy and resection of extra-articular disease
- pros
  - allows surgeon to fully address all diseased sections
  - allows for thorough synovectomy
- cons
  - posterior approach to the knee requires approaching neurovascular bundle
Total joint arthroplasty and synovectomy
- indicated in advanced disease with severe degenerative joint changes
- applicable to knee, hip, shoulder
Total synovectomy and arthrodesis
- indicated in severe disease of the ankle

Complications

Recurrence
- recurrence is the most frequent complication for both intra-articular and extra-articular disease
  - 30-50% recurrence rate despite complete synovectomy
  - rates can be reduced with addition of external beam radiation

IBank

	Location	Xray	CT	CT	B. Scan	MRI	Gross	Histo(1)
Case B	Knee
Case A	Ankle

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Qbank (3 Questions)

TAG

(SBQ07.5) A 45-year-old male presents with a 6-month history of recurrent knee effusions and pain. There is no history of trauma. Exam shows a large joint effusion, decreased range of motion, and a nodularity surrounding the knee. MRI and arthroscopic intra-articular picture are shown. What is the most likely diagnosis? Topic

Review Topic

FIGURES: A

1. Synovial sarcoma
2. Rheumatoid nodule
3. Septic arthritis
4. Synovial chondromatosis
5. Pigmented villonodular synovitis

PREFERRED RESPONSE ▶

DISCUSSION: The history, examination, MRI, and arthroscopic intra-articular image are most consistent with a diagnosis of pigmented villonodular synovitis. PVNS is a benign condition which can predispose patients to early arthritis. It should be noted that the MRI shows nodular findings in both the anterior and posterior portions of the knee. Arthroscopic image shows the deep red fronds classic for PVNS secondary to hemosiderin deposition in the synovium. These findings are specifically reviewed in the article by Bhimani et al where they focus on the history, presentation, imaging appearance, and treatment modalities for PVNS and focus on the MRI appearance of these lesions.

Treatment for symptomatic PVNS includes synovectomy. Often this can be accomplished arthroscopically in the anterior portion of the knee, but the synovial nodules in the posterior knee often require a formal posterior arthrotomy to remove.

True intra-articular malignancies are quite rare, and despite the misnomer, synovial sarcoma is NOT an inter-articular process. Rather, synovial sarcoma tends to occur around, but not within, joint. While rheumatoid nodules, synovial chondromatosis, and lipoma arborescens are all frequent intra-articular lesions, PVNS is the most likely diagnosis based on the MRI and arthrotomy image.

DePonti et al report their 10 year experience with arthroscopic treatment of PVNS of the knee. They found that localized PVNS responded well to lesional excision but that diffuse PVNS required an extended synovectomy to accomplish stable local control of the lesion.

Chin et al report their results on 40 patients with diffuse PVNS. They divided their treatment into surgery alone, surgery and intra-articular radiation, and surgery with external beam radiation. They found the knee scores and range of motion improved post-operatively in all groups, and there was suggestion that intra-articular adjuvant radiation improved the rates of local control.

REFERENCES:

1. De Ponti A, Sansone V, Malcherè M. Result of arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthroscopy. 2003 Jul-Aug;19(6):602-7. Review. PMID:12861198 (Link to Abstract)

2. Chin KR, Barr SJ, Winalski C, Zurakowski D, Brick GW. Treatment of advanced primary and recurrent diffuse pigmented villonodular synovitis of the knee. J Bone Joint Surg Am. 2002 Dec;84-A(12):2192-202. PMID:12473708 (Link to Abstract)

3. Bhimani MA, Wenz JF, Frassica FJ. Pigmented villonodular synovitis: keys to early diagnosis. Clin Orthop Relat Res. 2001 May;(386):197-202. PMID:11347835 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Michael Hughes MD, John Badylak MD,

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TAG

(OBQ09.221) A 24-year-old man presents with pain and a mass in the anterior ankle. Plain radiographs are normal, MRI is shown in Figure A (arrrows indicate the mass), and biopsy is shown in Figure B. What is the most likely diagnosis? Topic

Review Topic

FIGURES: A

1. Aneurysmal bone cyst
2. Periosteal osteosarcoma
3. Chondroblastoma
4. Myositis ossificans
5. Pigmented villonodular synovitis (PVNS)

PREFERRED RESPONSE ▶

DISCUSSION: The MRI and biopsy are diagnostic for pigmented villonodular synovitis (PVNS), a tumor like condition that is locally aggressive but without risk for metastatic spread. The MRI demonstrates an intra-articular mass without bone destruction. The biopsy shows a nodular pattern of giant cells, diffuse proliferation of mononuclear cells of synovial- or histiocytic-type, and hemosiderin. These components of the biopsy make the diagnosis of PVNS. Treatment is complete excision (synovectomy) of the lesion.

Bickels et al describe their experience with 7 patients with PVNS and the success of adjuvant therapy. They found a high rate of complications following intra-articular injection of yttrium 90 in the ankle joint. As such, they do not recommend this treatment and like many continue to support synovectomy for symptomatic lesions.

Sharma et al describe 14 cases of PVNS of the foot and ankle evaluating the clinical, radiographic, and histopathology factors which influence local recurrence. They found a 14.3% recurrence rate with standard therapy including amputation for appropriate cases and more importantly identified incomplete synovectomy as a possible contributor towards local recurrence.

Incorrect answers:
1) Aneurysmal bone cyst - this lesion is extra-osseous. ABCs occur within the bone.
2) Periosteal osteosarcoma - the pathological section shows no signs of osteoid or malignant cells
3) Chondroblastoma - again, this lesion is extra-osseous. Chondroblastoma occurs within the bone.
4) Myositis ossificans - typically doesn't occur this close to the joint and would appear different on pathological section.

REFERENCES:

1. Bickels J, Isaakov J, Kollender Y, Meller I. Unacceptable complications following intra-articular injection of yttrium 90 in the ankle joint for diffuse pigmented villonodular synovitis. J Bone Joint Surg Am. 2008 Feb;90(2):326-8. PMID:18245592 (Link to Abstract)

2. Sharma H, Jane MJ, Reid R: Pigmented villonodular synovitis of the foot and ankle: Forty years of experience from the Scottish bone tumor registry. J Foot Ankle Surg 2006;45:329- 336 PMID:16949531 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Derek Moore, Ben Taylor MD,

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(OBQ08.31) Intralesional surgical resection is considered standard of care for which of the following conditions? Topic

Review Topic

1. High grade sarcoma surrounding a major nerve
2. Intermediate grade solitary fibrous tumor
3. Atypical lipomatous tumor
4. Tibial adamantinoma
5. Pigmented villonodular synovitis

PREFERRED RESPONSE ▶

DISCUSSION: Surgical margins include intralesional (you cut into the tumor), marginal (you resect directly at the interface between tumor and normal tissue), wide (you resect the tumor with a cuff of normal tissue surrounding the tumor), and radical (you resect the entire compartment from which the tumor arose). Intralesional excisions are not acceptable for high or intermediate grade tumors. Atypical lipomatous tumor, while a low grade tumor, has a propensity for local recurrence and as such is removed using either marginal or wide excision depending on the associated morbidity based on tumor location. Pigmented villonodular synovitis is treated with synovectomy (either open or arthroscopically), which is considered an intralesional excision.

REFERENCES:

1. Simon, M. Surgical margins. In Simon, M. Springfield D (EDS): Surgery for bone and soft tissue tumors. Philadelphia, PA. Lippincott-Raven, 1998, pp 77-92

2. Frassica, F. Orthopaedic Pathology. In Miller, MD (ED). Review of Orthopaedics, Ed 2. Philadelphia, PA. WB Saunders 1996, pp 292-335.

3. Schwartz, RS (ED): Orthopaedic knowledge update: Musculoskeletal tumors 2. Rosemont, American Academy of Orthopaedic Surgeons, 2007, pp 265-269.

Question Authors:

Patrick O'Donnell MD/PhD,

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(OBQ04.30) A 35-year-old man presents to your office complaining of vague knee pain and a fullness surrounding his patellar tendon. MRI and histology are shown. What is the name of the analogous histologic disease when located away from the synovial lining of joints? Topic

Review Topic

FIGURES: A

1. Fibrous dysplasia
2. Hemangioma
3. Synovial sarcoma
4. Giant cell tumor of bone
5. Giant cell tumor of tendon sheath

PREFERRED RESPONSE ▶

DISCUSSION: The history, MRI, and histology suggest pigmented villonodular synovitis of the knee. However, the question asks what is the analogous histologic disease process of PVNS when located away from the synovial lining of joints, which is giant cell tumor of tendon sheath. Features that help make the diagnosis of PVNS in this case include the intra-articular location, signal uptake on MRI, and the diagnostic hemosiderin laden macrophages on histology. The reference by Abdul-Karim et al. describes the histologic similarities between giant cell tumor of tendon sheath and PVNS. Fibrous dysplasia would show a fibrous histeocytic background with the characteristic chinese letters. Hemangiomas show vascular structures on histology. There is no cellular atypia or high nuclear to cytoplasmic ratio as would be seen with synovial sarcoma and the characteristic giant cell tumor of bone would show the spindle cell background with associated multinucleated giant cells. In addition, none these lesions "change" names with anatomic location. In the reference by MCCarthy et al, they discuss the differential diagnosis and treatment of synovial based lesions including PVNS. They argue that because PVNS is a difficult disease to cure, if a patient is relatively asymptomatic treat first with observation followed by synovectomy if pain continues.

REFERENCES:

1. Diseases of synovial membrane, in McCarthy EF, Frassica FJ (eds): Pathology of Bone and Joint Disorders with Clinical and Radiographic Correlation. Philadelphia, PA, WB Saunders, 1998, pp 307-315

2. Abdul-Karim FW, el-Naggar AK, Joyce MJ, Makley JT, Carter JR. Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathologic and flow cytometric DNA analysis. Hum Pathol. 1992 Jul;23(7):729-35. PMID:1319390 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

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Videos

	A Case Presentation of PVNS of the Hip - Dr. Kamron Izadi Pathology - Educational Presentation - Pigmented Villonodular Synovitis A narrated case report of a 31-year-old male who presented with painful right hi...	3/7/2013 48 views 0
	Arthroscopic Excision of Focal PVNS in the Knee - Dr. John Hardy Pathology - Surgical Techniques - Pigmented Villonodular Synovitis In this narrated video Dr. Hardy arthroscopically removes a small focal area of...	3/7/2013 32 views 0

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ABOS II: Pigmented Villonodular Synovitis

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Evidence & References Show References

Level of Evidence 4 (Case Series)

Chin KR, Barr SJ, Winalski C, Zurakowski D, Brick GW. Treatment of advanced primary and recurrent diffuse pigmented villonodular synovitis of the knee. J Bone Joint Surg Am. 2002 Dec;84-A(12):2192-202. PMID:12473708 (Link to Abstract)
Bickels J, Isaakov J, Kollender Y, Meller I. Unacceptable complications following intra-articular injection of yttrium 90 in the ankle joint for diffuse pigmented villonodular synovitis. J Bone Joint Surg Am. 2008 Feb;90(2):326-8. PMID:18245592 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

De Ponti A, Sansone V, Malcherè M. Result of arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthroscopy. 2003 Jul-Aug;19(6):602-7. Review. PMID:12861198 (Link to Abstract)
Bhimani MA, Wenz JF, Frassica FJ. Pigmented villonodular synovitis: keys to early diagnosis. Clin Orthop Relat Res. 2001 May;(386):197-202. PMID:11347835 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Simon, M. Surgical margins. In Simon, M. Springfield D (EDS): Surgery for bone and soft tissue tumors. Philadelphia, PA. Lippincott-Raven, 1998, pp 77-92
Frassica, F. Orthopaedic Pathology. In Miller, MD (ED). Review of Orthopaedics, Ed 2. Philadelphia, PA. WB Saunders 1996, pp 292-335.
Schwartz, RS (ED): Orthopaedic knowledge update: Musculoskeletal tumors 2. Rosemont, American Academy of Orthopaedic Surgeons, 2007, pp 265-269.

Undefined

Sharma H, Jane MJ, Reid R: Pigmented villonodular synovitis of the foot and ankle: Forty years of experience from the Scottish bone tumor registry. J Foot Ankle Surg 2006;45:329- 336 PMID:16949531 (Link to Abstract)
Diseases of synovial membrane, in McCarthy EF, Frassica FJ (eds): Pathology of Bone and Joint Disorders with Clinical and Radiographic Correlation. Philadelphia, PA, WB Saunders, 1998, pp 307-315
Abdul-Karim FW, el-Naggar AK, Joyce MJ, Makley JT, Carter JR. Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathologic and flow cytometric DNA analysis. Hum Pathol. 1992 Jul;23(7):729-35. PMID:1319390 (Link to Abstract)

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Pigmented Villonodular Synovitis

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