imaging spectrum of pigmented villonodular synovitis involving the head and neck region asnr 2015...

IMAGING SPECTRUM OF PIGMENTED VILLONODULAR SYNOVITIS INVOLVING THE HEAD AND NECK REGION

ASNR 2015

Abstract No: eEdE-151

Maryam Gul, MD

Ammar Chaudhry, MD

Abbas Chaudhry, BSc

Lev Bangiyev, DO

Correspondence: [email protected]

DISCLOSURES

• No relevant disclosures

OBJECTIVES

1. Brief review of head and neck anatomy of areas involved in Pigmented Villonodular Synovitis (PVNS)

2. Review clinicopathologic spectrum of acute, chronic and acute on chronic PVNS

3. Discuss spectrum of imaging (CT and MRI) findings in PVNS

4. Review mimics with emphasis on key findings differentiating these entities

5. Brief discussion of treatment, prognosis and follow up in PVNS

INTRODUCTION• Pigmented villonodular synovitis (PVNS) is a relatively synovial neoplastic disorder

of unknown etiology

• Thought to represent inflammatory or reactive synovial infiltrate

• Most commonly seen involving the larger joints in the lower extremities (knees, ankle, and hips)

• presents as a soft poorly circumscribed mass with local aggressive biological behavior and high recurrence rates

• Histopathology:

• Grossly appears as a dark pigmented, cystic-appearing mass

• Microscopy reveals: histiocytes with giant cells + hemosiderin eroding into surrounding bone without presence of atypia or mitoses

• Also seen is lymphoplasmacytic infiltrate and chondroid metaplasia

• Demographics: Most commonly seen in 2nd – 3rd decade of life; Female > Male (3:1)

IMAGING• Radiologic assessment is pivotal in the diagnosis of PVNS,

• MRI the most useful imaging modality in characterizing and documenting the extent of the lesion

• Imaging features of PVNS reflect its pathologic processes of synovial proliferation and diffuse hemosiderin staining

• Best diagnostic clue

• Monarticular hyperplastic, inflammatory process involving TMJ

• Size

• Typically 1 cm or > at diagnosis

Jaclyn J.S. Wong MBBS,, Pramit M. Phal MBBS,and David Wiesenfeld MDSs. Pigmented Villonodular Synovitis of the Temporomandibular Joint: A Radiologic Diagnosis and Case Report, 2012-01-01Z, Volume 70, Issue 1, Pages 126-134,

C.R. Herman , J.Q. Swift and E.L. Schiffman Pigmented villonodular synovitis of the temporomandibular joint with intracranial extension: a case and literature review, 2009-07-01Z, Volume 38, Issue 7, Pages 795-801

RADIOGRAPHS AND COMPUTED TOMOGRAPHY

• Radiographs and CT findings are nonspecific which can include:

• juxta-articular cortical erosion,

• cyst formation

• +/- erosions in the temporal bone and mandibular condyle with preservation of joint space,

• a lack of subchondral osteoporosis (makes RA and septic processes less likely)

• +peri-articular soft tissue swelling

• +/- periarticular calcification and chondroid metaplasia

• CT better quantifies degree of osseous destruction

• CT can reveal hyperdense synovium owing to iron deposition

• Angiography: Highly vascular, AV shunting, irregular vessels, dense tumor blush

• NOTE: “Mature” lesions are typically fibrotic and become hypovascular.



MAGNETIC RESONANCE IMAGING

• T1WI

• Hypointense to isointense mass with peripheral rim of low signal

• May have areas of ↑ T1 due to lipid in foamy macrophages

• T2WI

• Hypointense lobulated mass often with peripheral rim of low signal

• Hypointensity due to hemosiderin deposition

• Hemosiderin staining causes decreased signal on both T 1 - and T 2 -weighted images

• In addition, because of its paramagnetic effects, hemosiderin will cause “blooming” or susceptibility artifact on gradient echo or echo planar imaging, leading to a loss of signal on these sequences; therefore, if the diagnosis of PVNS is in question, gradient echo sequences could help to confirm the diagnosis

• Cystic areas of synovial fluid will be hyperintense

• synovial thickening typically demonstrates enhancement1 and could result in focal areas of T 2 -

weighted hyperintensity corresponding to loculated joint fluid and adjacent osseous erosion

• T1WI C+

• Variable mild to moderate enhancement of the mass component of the lesion



Case: 61 year old female presents with right-sided headaches difficulty with mastication.

CT facial bones: In the squamous portion of the right temporal bone and adjacent right zygomatic arch, there is an expansile mixed lytic-sclerotic lesion with associated cortical destruction. There is an associated soft tissue component that appears to extend into right temporomandibular joint and in the right glenoid. The adjacent mandibular condyles appears unaffected. Differential considerations include metastasis versus primary synovial neoplasm versus osseous neoplasm.

MULTIPLE CORE TISSUE BIOPSIES WERE PERFORMED AND DIAGNOSIS OF PVNS WAS

MADE

Axial non-contrast CT of the right TMJ during biopsy needle

6-HOURS POST BIOPSY PATIENT COMPLAINED OF HEADACHE AND WAS NOTED TO HAVE DILATED RIGHT

PUPIL

CT of the head was performed that revealed interval development of acute right frontotemporoparietal convexity subdural hematoma with mass effect and right-to-left midline shift. Unchanged heterogeneous density expansile/destructive bone lesion centered in the right zygomatic/temporal suture with possible extension into the right middle cranial fossa.hich reveals

HISTOLOGY

Histology (Hematoxylin and eosin; 100x) reveals multinucleated giant cells (redarrow), mixed with foamy cytoplasmic cells (yellow arrow), lymphocytic cells (green arrow), and histiofibroblastic cells (black arrow). These findings are highly suggestive of PVNS

COMPANION CASE: 51 YEAR OLD FEMALE WITH LONG STANDING HISTORY OF LEFT JAW

PAIN AND RESTRICT MOTION

Axial CT +contrast reveals nonspecific left TMJ synovial enhancement (blue arrow) with soft tissue thickening and mild enlargement of lateral pterygoid muscle (yellow arrow)

Coronal CT in bone windows reveals prominent sclerosis of left mandibular condyle (green arrow) and glenoid fossa with focal erosions in mandibular condyle (red arrow)

MRI

Coronal GRE and Axial T 2-weighted images demonstrating prominent hypointense synovial thickening around left mandibular condyle compatible with presence of blood products

MRI

Coronal T 1-weighted image demonstrating sclerosis and hypointense capsular thickening

Axial post-contrast T1FS shows prominent synovial enhancement and osseous erosion anteriorly in mandibular condyle

TREATMENT AND PROGNOSIS

• Optimal treatment consists of complete excision of the mass and removal of the synovium and affected bony structures

• Recurrence: reports of recurrence of PVNS in all joints range from 8% to 46%.

• There are no reported cases of long-term follow-up, but when follow-up was reported in cases involving the TMJ, recurrence was observed in only 2 cases (7%)

• Localized bony destruction was common and occurred in 70% of cases.

• In 6 cases, including the present one, the lesion had extended into the middle cranial fossa 3 6 12 14 17 . Eisig et al. 14 recommend a wide excision and long-term follow-up

• Overall, the prognosis is good with adequate surgical excision

• diffuse type has a worse prognosis with an increased recurrence risk

• “malignant” variants with lymph node or distant organ metastases had poor outcomes.

• long-term follow-up involves serial computed tomography or MRI for a minimum of 7 to 10 years



DIFFERENTIAL DIAGNOSIS• Giant Cell Tumor

• Benign intra-osseous neoplasm arising from multinucleated giant cells

• In H&N, most commonly involves sphenoid & temporal bones

• Expansile mass with benign "eggshell" wall on bone CT

• Multiple lytic lesions may have "bubbly" appearance

• May have hypointense rim on T1 and T2 MR

• Marked homogeneous or heterogeneous enhancement on CECT or T1 C+ MR

• Calcium Pyrophosphate Dihydrate Deposition Disease

• Metabolic disease resulting in peri/intraarticular chondrocalcinosis

• Diffusely calcified mass involving TMJ on CT

• Associated remodeling, erosion, or mass effect on condyle

• 50% have involvement of multiple joints

• Hypointense T1, heterogeneously hypointense T2 MR

• Heterogeneously enhancing on T1WI C+ MR

• Chondrosarcoma• Chondroid malignancy involving TMJ or petro-occipital fissure

• Chondroid calcification + bone destruction on bone CT

• Hypointense to isointense on T1, hyperintense on T2 MR

• Heterogeneously enhancing on CECT or T1WI C+ MR

• Synovial Chondromatosis

• Synovial metaplasia with formation of foci of hyaline cartilage

• Multiple calcified "loose bodies" in superior joint space of TMJ

• Bone CT: Calcified nodules surrounding mandibular condyle

• Associated degenerative changes of condyle

• MR: Multiple hypointense nodules in superior joint space T1

• Joint space expansion & effusion T2

• Enhancing synovium on T1WI C+ MR

Giant Cell Tumor

CPPD Syndovial Chondromatosis

ADD’L DIFFERENTIAL DIAGNOSIS• Synovial sarcoma

• Lobular mass with + presence of amorphous calcification, T1 iso-to-hyperintense, T2 hyperintense ++ heterogeneous enhancment +cysts

• Septic arthritis

• + periaritcular demineralization, characterized by more cortical destruction and poorly defined margins; +/- concurrent osteomyelitis;

• Rheumatoid arthritis

• +demineralization, +erosions and narrowing of the joint space; late stages +condylar head destruction

• Gout

• usually polyarticular; T1/T2 intermediate synovium w/o loss of joint space until end stage; +biriferingent crystals in polarized microscopy

• Osteoarthritis

• Elderly, asymptomatic, -soft tissue swelling, -erosions

• Carcinoma of skin (SCC, melanoma, basal cell), MSK (osteosarcoma, fibrosarcoma, rhabosarcoma), external auditory meatus or middle ear carcinoma, parotid, etc

• +direct extension is an uncommon, but important, + with evidence of bony destruction contiguous with the auditory canal

• NOTE:

• do NOT misdiagnosed as a parotid mass; upto 20% of pre-auricular swelling is initially misdiagnosed as a parotid mass

• Another important mimic is TMJ osteonecrosis from bisphosphonate use, which can lead to TMJ arthrosis that in end-stage can mimic PVNS

TMJ synovial sarcoma

Septic Arthritis

Gout

CONCLUSION

• Pigmented villonodular synovitis is a rare cause of aggressive mixed lytic-sclerotic lesion with or without an associated mass which can cause cranial neuropathies and ipsilateral brain parenchymal abnormalities secondary to mass effect.

• TMJ PVNS must be considered in patients who present with the warning symptoms of preauricular swelling, trismus, or altered sensation.

• Patients with an early diagnosis and adequate resection have had good long-term results; thus, patients should be promptly referred to an oral and maxillofacial surgeon with TMJ expertise for a thorough clinical assessment and radiologic and histopathologic investigations.

• Combination of CT and MRI can help narrow the differential diagnosis and guide biopsy.

• MRI is a highly sensitive and specific noninvasive modality that aids the PVNS diagnosis and surgical planning.

• It provides precise localization of the mass and its extent relative to the surrounding structures. Long-term follow-up is required for a minimum of 7 to 10 years with serial computed tomography or MRI to monitor for recurrence.

imaging spectrum of pigmented villonodular synovitis involving the head and neck region asnr 2015...

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