1314 Cai et al. Case Report TMJ Disorders

J. Cai1, Z. Cai1, Y. Gao2 1Department of Oral and Maxillofacial Pigmented villonodular synovitis 2 Surgery, Beijing, China; Department of Oral Pathology, Peking University School & of the temporomandibular joint: Hospital of Stomatology, Beijing, China a case report and the literature review

J. Cai, Z. Cai, Y. Gao: Pigmented villonodular synovitis of the temporomandibular joint: a case report and the literature review. Int. J. Oral Maxillofac. Surg. 2011; 40: 1314–1322. # 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Abstract. Pigmented villonodular synovitis (PVNS) is an uncommon benign proliferative disorder of synovium that may involve joints, tendon sheaths, and bursae. It most often affects the knees, and less frequently involves other joints. It presents in the temporomandibular joints (TMJs) extremely rarely. The authors report an elderly female patient with PVNS of the TMJ with skull base extension, who had traumatic history in the same site. It was diagnosed through core-needle Keywords: pigmented villonodular synovitis (PVNS); synovitis; temporomandibular joint biopsy, which was not documented in the literature. Radical excision and follow-up (TMJ). for 7–8 years was recommended because of the reported malignant transformation and high recurrence rate. This case and previously reported cases in the literature are Accepted for publication 2 March 2011 reviewed and discussed. Available online 6 April 2011

The term pigmented villonodular synovi- were reported in detail (Table 1). The visits and mouth-opening for a long time tis (PVNS) was introduced by JAFFE et al. authors present an additional case of during the operation. Left preauricular in 1941 to describe a proliferative disorder PVNS affecting the TMJ, and review pre- swelling and pain occurred after the dental of unknown aetiology involving diarthro- vious cases. treatment, with progressive diminution of dial joints, tendon sheaths and bursae22. hearing in the same ear. The swelling It’s an uncommon disorder, which has an gradually increased in size, but the pain annual incidence of 1.8 cases per million Case report reduced after using anti-inflammatory 30 population . It most often affects the A 59-year-old Chinese female was drug. The patient had no complaint of 14,30 knees, including about 80% of cases . referred in February 2010 with the chief mouth opening restrict or biting pain. Other joints that may be affected by complaint of a left preauricular mass that Physical examination revealed a preauri- PVNS, in decreasing order of frequency, she had noticed for about 2 years. She cular mass measuring approximately include the hips, ankles, small joints of the reported a history of left TMJ dislocation 3.5 cm  3.5 cm, spherical, firm, immo- hands and feet, shoulders and elbows. during yawning about 5 years ago, which vable, nontender on palpation, with a Involvement of vertebral articular joints she reduced by hand by herself immedi- somewhat detectable boundary, localized 14 has also been documented . It presents ately. Although it did not recur, she occa- over the left TMJ region (Fig. 1). Max- extremely rarely in the temporomandibu- sionally felt preauricular pain. About 2 imum mouth opening was 3.7 cm. There lar joints (TMJs). The first reported occur- years ago, a pea-sized preauricular mass was slight deviation to the left when the 25 rence was in 1973 by LAPAYOWKER et al. . with pain was noticed, and the symptoms mouth was opened, with slight deviation To the authors’ knowledge, only 37 cases disappeared after using anti-inflammatory to the right when the mandible was moved of PVNS affecting the TMJ have been drug. About 1.5 years ago, she underwent forward, with a notable decrease in left reported in the English literature and 1 root canal treatment and crown restoration lateral excursion. The occlusion was nor- case in the Chinese literature; 34 of these for the left first molar, that required several mal. The patient’s medical history was Table 1. Summary of reported cases of PVNS involving the TMJ. A. Clinical features and preoperative diagnosis No. Authors/date Age (y);sex;site Clinical features Duration Bone destruction Preoperative diagnosis

1LAPAYOWKER 22;M;L PS, masticatory pain 18m TMJ Malignant PT et al. 197325 2LAPAYOWKER 58;F;L Clicking; tinnitus; hearing diminution 1y TMJ, ramus, SBICE, EAC Biopsy: fibroadipose tissue et al. 197325 Open biopsy: PVNS DINERMAN and 13 MYERS 1977 2 3BARNARD 1975 37;M;L PS, pain, LMO, MD to L 3w TMJ Infection arthritis or neoplasia 4MIYAMOTO 34;M;R PS 1.5y TMJ PT et al. 197728 5TAKAGI and 36;M;R Oppressive pain, clicking, LMO 3y TMJ NR 42 ISHIKAWA 1981 6RICKERT and 39;F;L Parotid mass 1m TMJ PT; FNA: giant cell granuloma, 33 SHAPIRO 1982 ‘brown tumour’, neoplasm 18 7GALLIA et al. 1982 47;F;L PS, LMO, masticatory pain, MD to L, 2y TMJ PVNS 10 CURTIN et al. 1983 otalgia 31 8 O’SULLIVAN et al. 1984 61;F;L Swelling in parotid area NR TMJ, coronoid process, ramus, Benigh PT 4 BERTONI et al. 1997 zygomatic arch, EAC, SBICE 11 9DAWISKIBA et al. 1989 32;M;R TMJ swelling, pain, LMO NR No FNA: PVNS 15 10 EISIG et al. 1992 50;F;R HL, mass in ear canal 2m TMJ, SBICE, EAC, mastoid, Central giant cell granuloma middle ear ossicles 41 11 SYED et al. 1993 10;F;L PS 1y No PT 17 12 FRANCHI et al. 1994 59;F;L Swelling in parotid area, pain 6m No PT. 46 13 YOUSSEF et al. 1994 41;F;L TMJ swelling, pain, LMO, decreased 6m No Incisional biopsy: PVNS lateral excursion to L 43 14 TANAKA et al. 1997 47;M;R PS, masticatory pain, LMO, MD to R 8m TMJ Giant cell-containing lesion of TMJ 32 15 RENAGA et al. 1997 70;F;L TMJ swelling, intermittent pain, LMO, 7m TMJ PVNS clicking 47 16 YU et al. 1997 48;M;R PS, pain when wide mouth opening 7m TMJ PT 8 17 CHOW et al. 1998 42;F;R Zygomatic mass 6m TMJ Giant cell tumour, chondroblastoma

38 synovitis villonodular Pigmented 18 SONG et al. 1999 57;F;R TMJ swelling 5y TMJ PT 3 19 BEMPORAD et al. 1999 37;M;R PS, pain, trismus, tinnitus, HL 4y TMJ, SBICE, maxilla, maxillary PT; Incisional biopsy: STRYJAKOWSKA sinus, carotid canal, EAC ‘brown tumour’; FNA: PVNS et al. 200540 26 20 LEE et al. 2000 59;F;R PS, decreased salivary flow 6y TMJ PT; Punch biopsy: PVNS 24 21 KISNISCI et al. 2001 45;F;L Cheek swelling, pain, LMO, MD to L NR No NR 34 22 SHAPIRO et al. 2002 36;M;R Temporal mass, clicking, some HL, NR SBICE Malignant process; CT guided popping sensation in ear FNA: benign giant cell-containing lesion 9 23 CHURCH et al. 2003 42;M;R PS, clicking, HL, mass nearly 4m TMJ, zygomatic arch, SBICE, EAC Biopsy: PVNS occluded EAC 9 24 CHURCH et al. 2003 33;M;R PS, deep pain, trismus 2y TMJ FNA: PVNS 44 25 TOSUN et al. 2004 60;M;L TMJ mass, V2/V3 numbness/paresthesia, 10y TMJ, ramus, SBICE, carotid canal, Otitis, PT; FNA: PVNS LMO, MD to L, HL, otitis media foramen ovale, EAC, sphenoid bone 1 26 AOYAMA et al. 2004 33;M;R PS, clicking > 20y, LMO NR TMJ, skull base Malignant tumour, giant cell

tumour; Biopsy: PVNS 1315 7 27 CASCONE et al. 2005 38;F;L PS, TMJ pain NR No FNA: non-specific arthritis Table 1 (Continued ) 1316 A. Clinical features and preoperative diagnosis No. Authors/date Age (y);sex;site Clinical features Duration Bone destruction Preoperative diagnosis a tal. et Cai 28 STRYJAKOWSKA 36;F;R Painful parotid lesion, radiating pain 4m No PT et al. 200540 16 29 FANG et al. 2007 44;M;R PS 2m TMJ, zygomatic arch Pleomorphic adenoma; FNA: PT 6 30 CASCONE et al. 2008 78;M;R PS, orofacial pain, LMO, MD to R NR No NR 12 31 DAY et al. 2008 38;M;L Mass in zygomatic process area, pain 1m SBICE, foramen ovale, vidian canal, Cholesterol cyst, vascular foramen rotundum bone tumour 36 32 SHKOUKANI et al. 2009 74;F;R PS, pain, LMO Few m TMJ CT-guided FNA: PVNS 5 33 CAI et al. 2009 21;F;L TMJ pain, LMO, clicking, MD 3m No Anterior disc displacement without reduction 21 34 HERMAN et al. 2009 36;M;L PS, pain, numbness, LMO, MD to L, 12–18m Zygomatic arch, glenoid Biopsy: PVNS otalgia fossa, SBICE

B. Treatment and prognosis No. Treatment Complications and prognosis Rec. (FU-period) [Rec. time] 1 CE Uneventful No 2 CE, Condy., partial Parot., craniectomy, NR No (4y) reconstructed with free graft of fascia temporalis 3 Incisional biopsy Some persistent MD to L NR (6m) 4 CE Temporary tinnitus, EAC narrowing No (2y) 5 1st–2nd: CE, Condy. Uneventful Yes [5y]; no Rec. (11y) 6 Amputated tumour from TMJ, superficial Temporary FP, limited jaw motion No (3m) Parot. 7 CE Temporary FP, occlusal discrepancy No (2y) 8 1st–3rd: mass excision, Parot., Mand., Dead 20d after last operation with Yes [1.5y, 2.5y, 2y, 4y] craniectomy; 4st: RT; 5th: surgery malignant transformation, intracranial extension and lung metastasis 9 Surgery (not specified) NR NR 10 CE, craniectomy, Condy., radical Minimal MD No (1y) mastoidectomy, repaired dura with temporal fascia graft 11 CE, superficial Parot. Temporary FP NR (8w) 12 CE NR No (1y) 13 CE, superficial Parot. Uneventful No (14m) 14 CE, craniectomy, Condy. Temporary FP No (2y) 15 CE Uneventful No (3y) 16 1st: superficial Parot.; 2st: surgical exploration NR NR 17 CE, Condy. Uneventful No (2y) 18 CE, total Parot., Condy. NR NR 19 Embolization, intradural resection, Mand., FP No (3y) total Parot., facial nerve grafting, suprahyoid neck dissection, repaired with pericranial graft and rectus myocutaneous free pedicle flap Pigmented villonodular synovitis 1317

significant for hypertension, diabetes mel- litus, hepatitis C and appendectomy. Computed tomograms (CT) showed a large mass with heterogeneous soft-tissue density in the left infratemporal fossa with the condyle as a centre. Bony destruction of the mandibular condyle, temporal skull base, the root of zygomatic arch and the anterior wall of external auditory meatus No (2y)

ndibular deviation; HL, hearing was noted (Fig. 2A and B). Magnetic resonance imaging (MRI) showed a 3.8 cm  3.5 cm  3.5 cm nodular mass in the region of the TMJ, presented as an intermediate heterogeneous signal on T1-weighted SE sequences (Fig. 2C) and

r; CE, complete excision; Condy., condylectomy; low signal on T2-weighted TSE sequences (Fig. 2D). The mass extended superiorly into the middle cranial fossa. The smooth margin between the mass and the temporal lobe suggested that the lesion was intra- cranial but extradural. The patient underwent ultrasound- guided core-needle biopsy with an 18 G cutting needle. A histopathological diag- nosis of PVNS was made (Fig. 3). In March 2010, the patient underwent complete excision of the mass through a temporofrontal approach by a multidisci- plinary team, consisting of members of the Maxillofacial Surgery, TMJ, and Neurosur- Uneventful No (11y) Temporary FP; MD No Temporary HL NR (3m) Preauricular concavity; temporary FP; MD to R Minimal MD; FP No (2y) gery Departments. A coronal incision was made with preauricular and frontal exten- sion (Fig. 1B). The frontotemporal scalp flap was elevated. The temporal branches of the facial nerve were dissected and pro- tected. The parotid gland was retracted, and the mass was exposed in the infratemporal fossa, with erosion to the inferior aspect of the left temporal bone (Fig. 1C). The root of the zygomatic arch was resected to expose the mass, then temporal craniectomy and condylectomy was performed to resect the mass completely. The middle cranial fossa floor, the gle- noid fossa, and the portion of zygomatic arch involved were resected. The dura mater was intact, and the cranial bone defect was covered by a temporal muscle pedicle flap rotated inferiorly. Grossly, the mass was red-brown with pliable texture, shaggy, not encapsulated, with a red- brown and yellow-brown cut surface and firmly adhering to the TMJ capsule (Fig. 1D). The final diagnosis, based on lateral pterygoid and temporalis muscles Parot., Condy., partial Mand. tympani with titanium mesh temporal muscle pedicle flap sural nerve grafting, Condy.,with reconstructed costochondral graft histopathological examination, was PVNS. The patient presented with hearing loss in the left ear, facial expression weakness in the region of the upper division of the : F, female; M, male; L, left; R, right; y, year(s); m, month(s); w, week(s); PS, preauricular swelling or mass; LMO, Limitation of mouth opening; MD, ma facial nerve, slight deviation of the mand- ible to the left immediately after the opera- tion. The occlusion was normal, and the maximum mouth opening was 23 mm Abbreviations loss; EAC, external auditory canal; FNA, fine-needle aspiration; NR, not reported; SBICE, skull base with intracranial extension; PT, parotid tumou Parot., parotidectomy; Mand., mandibulectomy; RT, radiation therapy; FP, facial paralysis; FU, follow-up; Rec., recurrence. 3334 Removed lesions with arthroscopy CE, craniectomy; repaired with cranial bone, Uneventful No (13m) 32 CE, tracheostomy tube placement, superficial 2728293031 CE, superficial Parot. 1st–2nd: Parot., CE CE CE CE, repaired temporal bone defect and tegmen Uneventful Uneventful NR Uneventful No (1y) Yes [15m]; no Rec. (1y) No (2y) No (20m) 212223242526 CE Embolizaiton, CE, craniotomy Wide excision, partial Mand, Wide craniectomy excision CE, Condy., temporal craniotomy, CE, partial Condy., Parot. craniotomy, covered dura by Uneventful MD to R Uneventful Uneventful Uneventful No (3y) No (6y) No (7y) No (2y) No (1y) 20 Remove mass and involved facial nerve, when she was discharged from the hospi- [(Fig._1)TD$IG]1318 Cai et al.

Fig. 1. (A) Before operation; (B) 10 days postoperation; (C) the mass exposed during operation; (D) the mass resected. [(Fig._2)TD$IG]

Fig. 2. Preoperative CT: soft-tissue window coronal section (A) and bone window axial section (B). Preoperative coronal MRI: T1-weighted SE sequences (C) and T2-weighted TSE sequences (D). [(Fig._3)TD$IG] Pigmented villonodular synovitis 1319

Fig. 3. Photomicrograph of a pathology specimen (haematoxylin–eosin stain). (A) High-power: showing mononuclear histiocytic cells (white arrows), multinucleated giant cells (hollow arrows), foam cells, deposits of hemosiderin, and abundant capillary vessels. (B) Low-power: showing histocytic cell infiltration into the synovial layers. tal. Following discharged, the patient the joint was essential for the development use was often neglected when the lesion underwent a course of physical therapy of this lesion and that trauma was often the was considered a type of tumour. The 45 including a home exercise for facial func- initiating factor . In 1966, MCCOLLUM history relating to chronic trauma, such tion and maintenance of occlusion with a et al. repeated the experiment by intra- as dental treatment, oral surgery, TMJ prosthetic appliance. Ten months after articular injection of blood and saline treatment, dislocation of TMJ, bruxism surgery, the patient was followed up by solution in dogs, suggesting that PVNS and malocclusion, should be recorded as telephone. No facial paralysis, mandibular might be produced by repeated trauma to well as acute trauma. Despite that, the deviation, malocclusion or preauricular the joint27. They found that the presence of malignant transformation and metastases concavity were noted. The maximum blood was not necessary for the develop- of PVNS4 might support the theory of mouth opening was 43 mm. Hearing was ment of PVNS, but that the lesion could neoplasm. To date, the aetiology of the improved, approaching normal. The cor- subside after a 6-month rest period27.In entity remains elusive and controversial. onal scar was invisible and covered by 1969, SINGH et al. repeated the experiment The 34 cases reported in the literature hair. As the disorder often recurs, long- using iron-dextrin, blood, plasma, gum are summarized in Table 1 and Fig. 4. All term close follow-up was required. acacia, and saline in rhesus monkeys with the cases presented monoarticular lesion, less frequency and volume, but only the without predilection for sex or site. The iron-dextrin and blood group presented a age of the patients ranged from 10 to 78 Discussion PVNS-like lesion, suggesting that the iron years; the average was 44.1 years. The PVNS is an uncommon benign prolifera- content of the inoculum was the important duration of initial symptom ranged from 3 tive disorder of the synovium that may factor in eliciting the changes37. More weeks to 10 years, and the average was involve the joints, tendon sheaths, and than 80% of PVNS cases involve the about 19.9 months. Eighty-eight per cent bursae. Two forms of PVNS are identified knees, upper extremities are seldom (30/34) of cases reported mass or swelling, depending on the area involved: the dif- involved, suggesting a predilection for 57% (17/30) of which were in the pre- 14,30 fuse form that affects the entire synovial weight bearing joints .MYERS and auricular area, which might be confused membrane of a joint or bursa; and the MASI reported that 88% of localized PVNS with parotid tumour. Masses in the ear localized form that affects tendon sheaths and 59% of diffused PVNS had a positive canal were found in two cases on physical or only part of the joint lining. traumatic history, and all the trauma-posi- examination. The masses were immova- The cause and pathogenesis remain tive localized cases and 38% of the dif- ble, firm, nontender or mildly tender on controversial but several possibilities have fused cases had suffered from acute palpation in most cases. Fifty-six per cent been suggested, including disturbances of trauma30. Bloody fluid was obtained in (19/34) of cases presented pain, that could lipid metabolism, neoplasm, inflamma- most of the cases undergoing synovial be oppressive, masticatory, intermittent, 19,21,38 tion, haemorrhage, and trauma . fluid aspiration, and yellow fluid was [(Fig._4)TD$IG]progressive, radiating or deep pain. Prior to 1941, the diseases resembling obtained in few cases30. In the present villonodular synovitis were referred to case there was an acute traumatic history as benign synovioma, giant cell tumour (TMJ dislocation) about 5 years ago and of joints, xanthoma of joints, and hemor- chronic traumatic history (dental treat- 27 rhagic villous synovitis . In 1941, JAFFE ment) about 1.5 years ago, that might be et al. recognized the similar histologic associated with PVNS. The symptoms pattern of these disorders and termed them could be relieved after using anti-inflam- pigmented villonodular synovitis, which matory drugs, suggesting that inflamma- has gained general acceptance22. He con- tion might occur in the protophase and/or sidered it to be a benign inflammatory in the process of PVNS. Of the 34 cases in state of the synovium of uncertain aetiol- Table 1, only 4 referred to a traumatic 22 1,2,5,17 ogy . In 1954, YOUNG and HUDACEK history, but none was positive . None reproduced PVNS-like lesion in dogs by of the case reports referred to the effect of repeated haemorrhage into the joint, and using anti-inflammatory drugs. The trau- considered that repeated haemorrhage into matic history and anti-inflammatory drug Fig. 4. Age distribution of the 34 cases. 1320 Cai et al.

Fifty-three per cent (18/34) of cases pre- Other temporary diagnoses included giant All 34 cases in Table 1 underwent sur- sented symptoms of the TMJ that mimic cell-containing lesion (4), arthritis (2), gery. Thirty-one cases received complete temporomandibular disorders. Twenty- neoplasia (2), giant cell granuloma (2), or wide excision, of which 28 cases were seven per cent (9/34) of the cases pre- malignant tumour (2), ‘brown tumour’ followed-up and 3 patients suffered recur- sented auricular symptoms, including (2), chondroblastoma (1), otitis media rences (11%) (3/28), that might have been hearing loss, otalgia, tinnitus, a popping (1), cholesterol cyst or vascular bone due to incomplete resection. It is difficult sensation in the ear, and otitis media. Two tumour (1) or anterior disc displacement to assess the true recurrence rate because cases presented with numbness and/or without reduction of TMJ (1). Only 32% there are not many long follow-up reports. paresthesia of the face21,44 and in one case (12/14) of the cases had been diagnosed as The recurrence rate is 17% (3/18) account- decreased salivary flow was noted on PVNS before operation. Although MRI is ing for the cases who were followed up for physical examination. a sensitive and specific method for the no less than 2 years. Only one case under- Owing to its aggressive infiltrative diagnosis of PVNS, histopathology is defi- went radiotherapy (RT) for three recur- behaviour, 74% (25/34) of the lesions nitive. Fine-needle aspiration (FNA) and rences. The lesion regressed after RT, but were associated with bony destruction in biopsy are helpful for the diagnosis of recurred again with malignant transforma- images and/or on intraoperative views, PVNS1,3,7,9,11,21,26,36,44,46, however mis- tion and lung metastasis 4 years after RT, that might cause a diagnostic dilemma. diagnoses might occur if the specimen and the patient died 20 days after the last Sixty-eight per cent (23/34) of cases could not represent the lesion3,7,16,21, operation. Only one case underwent eroded the TMJ. The surrounding struc- especially FNA, which is not a histopatho- arthroscopic treatment to remove the tures including skull base (10), auditory logic examination, but a cytological exam- lesion. Although some patients presented organs (6), zygomatic arch (4), mandibu- ination. In Table 1A, only 56% of FNA postoperative symptoms, most had an lar ramus (3), coronoid process (1), max- and 75% of biopsies diagnosed PVNS uneventful recovery. Only six cases had illa (1) and/or sphenoid bone (1) were before a definitive operation, so multiple permanent complications, including man- destroyed. The lesions extended into the FNA and/or biopsy might be necessary. dibular deviation, facial paralysis, crania in eight cases, which resulted in CT-guided FNA was carried out in two decreased strength, and preauricular con- difficulty in completely excising the mass. cases1,36, but none of the 34 cases had used cavity. These lesions were of soft tissue origin, so ultrasound-guided core-needle biopsy, Owing to the infiltrative, aggressive early changes might not be detectable on which is micro-traumtic, easy to operate behaviour and high recurrence rate, the plain film radiography. CT and MRI could and locate, and sufficient for histopatho- treatment recommended in most of the reveal the borders and extension of the logical diagnosis. There were two cases literature is complete excision with syno- lesion, as well as local bony invasion. The with simultaneous PVNS and synovial vectomy and/or capsulectomy. Simple most sensitive and specific method in the chondromatosis of the TMJ, which made excision should result in a complete cure diagnosis of PVNS is MRI, which is the the diagnosis more elusive. for the localized form of PVNS, and RT optimum radiographic method for judging The typical pathologic features of probably has no place in the treatment of the extent of the tumour before operation PVNS are indicated by the name14,22,29. these solitary lesions, and the recurrence and recurrence afterwards. The most char- Grossly, the synovium has a mossy or (range 0–27% in all joints) may be due to acteristic finding is nodular intra-articular nodular texture, spongy cut surfaces, and incomplete excision19,35. Diffuse PVNS masses of low signal intensity on T1-, T2-, a rusty, red-brown, or yellow-brown col- poses a diagnostic and therapeutic problem. and proton density-weighted sequences, our. Microscopically, the synovium is Where possible, complete synovectomy is that are considered to be due to a para- composed of fingerlike or rounded masses indicated but the recurrence rate after such magnetic effect attributed to heavy hemo- of fibrous stroma covered by hyperplastic surgery is quite high, ranging from 8% to siderin pigmentation23. The appearance of lining cells. Large numbers of foamy 46% in all joints5,19,35. Long periods of PVNS on MRI is variable, depending on macrophages in the stroma account for follow-up (more than 7–8 years) and reg- the relative proportion of the lipid, hemo- the yellow colouration, whilst the rusty ular MRI examinations after operation are siderin, fibrous stroma, pannus, fluid, and or brown colour is due to hemosiderin required to obtain the optimal outcome35. cellular elements23. Areas of high signal deposits in the stroma, cytoplasm of Postoperative RT for high-risk or incom- intensity on T2-weighted images corre- macrophages and synovial lining cells. pletely resected PVNS should probably be spond to a loculated cyst of joint The hemosiderin is a breakdown product considered as an effective treatment for fluid3,12,23, and the accumulation of lipid of haemoglobin, the presence of which local tumour control, complications, such in foamy macrophages may give rise to signifies old haemorrhage. Multinucleate as stiffness, pain, pathologic fracture of the areas of high signal intensity on T1- giant cells varying in number, are derived joint, may occur19,20,39. weighted SE images, similar to subcuta- from macrophages and synovial lining Three secondary and five primary neous fat23. CT scans are useful for deter- cells, and may also contain hemosiderin. malignant PVNS cases were reported by mining the extent of bony destruction that Mitotic figures with a normal configura- BERTONI et al. in 1997. Sixty-three per cent often occurs. tion are easily found in the proliferating (5/8) of them had local recurrence after The differential diagnoses of PVNS fibroblasts, macrophages, and synovial surgery; 50% (4/8) suffered lung, inguinal, with tuberculosis septic arthritis, malig- lining cells. If malignant PVNS develops, and/or spine metastases, and were dead nant synovioma, haemophilia, rheumatoid it would present the following histological within 3 years even though wide excision arthritis, gout, synovial chondromatosis, characteristics4: a nodular, solid infiltra- and RT were carried out4. carcinoma of the external or middle ear, tive growth pattern of the lesion; large, In view of the infiltrative nature, high cartilagenous disruption or abnormality plump, round or oval cells with deep recurrence, malignant transformation and have been documented in areas other than eosinophilic cytoplasm and indistinct bor- metastasis, wide or complete excision of the TMJ25.InTable 1A, 38% (13/34) of ders; large nuclei and prominent nucleoli; the entity is necessary, and close observa- the cases had been diagnosed as parotid and necrotic areas. Atypical mitoses are tion and regular MRI examination are tumour because of the site of the mass. seen occasionally. required for more than 7–8 years. Pigmented villonodular synovitis 1321

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Case Report Clinical Pathology

M. Zhang1, K. Matsuo1, Y. Yamashita2, T. Takahashi2 Leiomyomatous hamartoma of 1 Division of Oral Pathology, Department of Biosciences, Kyushu Dental College, Kitakyushu, Japan; 2Division of Oral and the midline maxillary gingival Maxillofacial Reconstructive Surgery, Department of Oral and Maxillofacial Surgery, presenting as a congenital Kyushu Dental College, Kitakyushu, Japan epulis: a case report with an immunohistochemical study

M. Zhang, K. Matsuo, Y. Yamashita, T. Takahashi: Leiomyomatous hamartoma of the midline maxillary gingival presenting as a congenital epulis: a case report with an immunohistochemical study. Int. J. Oral Maxillofac. Surg. 2011; 40: 1322–1326. # 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Abstract. An otherwise-healthy 2-year-old Japanese female presented with a polyp- like lesion on the palatal surface at the incisive papilla. The appearance of the lesion was similar to that of a congenital epulis. The histological findings showed proliferating mesenchymal components that contained mainly smooth muscle admixed with collagen fibres, nerve fibres, small vessels and mucous salivary glands. The immunohistochemical staining findings for a-smooth-muscle actin, desmin and S-100 protein were all positive. The histological diagnosis was therefore Accepted for publication 10 May 2011 leiomyomatous hamartoma, based on clinical microscopic observations. Available online 22 June 2011