Pigmented villonodular synovitis with chondroid metaplasia, resembling chondroblastoma of the bone: a report of three cases.

We herein describe three cases of pigmented villonodular synovitis with chondroid metaplasia. Two cases involved the temporomandibular joint, whereas the remaining one case occurred in the hip joint. Histologically, the tumors showed a villous pattern and were mainly composed of histiocyte-like cells and scattered osteoclast-like multinucleated giant cells, accompanied by chondroid areas with occasional lace-like calcification. These features resembled those of chondroblastoma of the bone, with the exception of the villous pattern. The histiocyte-like cells showed positive immunoreactivity for CD68, whereas they were negative for S-100 protein. Some of the previously reported cases of chondroblastoma in the temporal bone may have actually been cases of pigmented villonodular synovitis with chondroid metaplasia. When histologically chondroblastoma-like lesions involve the temporal bone or temporomandibular joint, the possibility of pigmented villonodular synovitis with chondroid metaplasia should also be considered, in addition to chondroblastoma of the bone. The correlation between this lesion and synovial chondromatosis remains uncertain.     

Fine-needle aspiration biopsy diagnosis of "invasive" temporomandibular joint pigmented villonodular synovitis

The clinical and aspiration cytologic details of a case of temporomandibular joint pigmented villonodular synovitis are presented and correlated with imaging, surgical, histopathologic, and clinical follow-up findings; the origin of such lesions is discussed. The lesion originally presented in a 36-year-old, otherwise healthy, white man as a unilateral mass involving the temporal fossa and temporomandibular joint region. The tumor's extent was defined by magnetic resonance imaging and computed tomographic scan; there was destruction of the temporomandibular joint and erosion of the temporal cranial bones by a lesion whose maximum dimensions were estimated by imaging to be 2.75 x 3.25 cm. The lesion was initially sampled and classified by computed tomography-guided fine-needle aspiration biopsy. Following complete resection, the original diagnosis was confirmed with both hematoxylin-eosin-stained paraffin sections and immunohistochemical staining. The patient remains free of disease 7 years postoperatively.     

Fine-needle aspiration of pigmented villonodular synovitis of the temporomandibular joint masquerading as a primary parotid gland lesion

The fine-needle aspiration findings in a case of pigmented villonodular synovitis of the temporomandibular joint are presented. The characteristic cytomorphologic and clinical features of this uncommon, benign fibrohistiocytic lesion are discussed. In addition, due to the initial clinical impression of a primary parotid gland lesion, the differential diagnosis for the cytomorphologic features observed (histiocytoid cells admixed with osteoclast-like giant cells) are discussed within the context of a primary salivary gland mass. Diagn. Cytopathol., 16:47–50,1997. © 1997 Wiley-Liss, Inc.     

Pigmented villonodular bursitis/diffuse giant cell tumor of the pes anserine bursa: a report of two cases and review of literature

Pigmented villonodular synovitis (PVNS) is a benign but potentially aggressive lesion, characterized by synovial villonodular proliferation with hemosiderin pigmentation and stromal infiltration of histiocytes and giant cells. This consists of a common family of lesions, including localized and diffuse forms of pigmented villonodular synovitis, giant cell tumor of the tendon sheath (nodular tenosynovitis) and the very rare cases of extra-articular pigmented villonodular synovitis arising from the bursa (pigmented villonodular bursitis or diffuse giant cell tumor of the tendon sheath). The purpose of this paper is to present two rare cases of pigmented villonodular bursitis arising from the pes anserinus bursa. The various differentials along with a review of literature of similar lesions are also being discussed. However, as with other lesions, clinicoradiographic features along with close histological correlation is essential for diagnosis.     

Tenosynovial giant cell tumor: case report and review

Tenosynovial giant cell tumors are a group of generally benign intra-articular and soft tissue tumors with common histologic features. They can be roughly divided into localized and diffuse types. Localized types include giant cell tumors of tendon sheath and localized pigmented villonodular synovitis, whereas diffuse types encompass conventional pigmented villonodular synovitis and diffuse-type giant cell tumor. Localized tumors are generally indolent, whereas diffuse tumors are locally aggressive. Recent developments indicate that tenosynovial giant cell tumors are clonal neoplastic tumors driven by overexpression of CSF1. Herein, I report a case of intra-articular, localized tenosynovial giant cell tumor (or localized pigmented villonodular synovitis) and review the classification, histopathology, and recent developments regarding its pathogenesis.     

Malignant giant cell tumor of tendon sheath. Report of a case

In a patient with pigmented villonodular synovitis of the right knee joint, there occurred a malignant giant cell tumor of tendon sheath. There was clinical evidence of metastasis after the second local recurrence and the recurrent tumors were studied enzyme cytochemically and electron microscopically. Ultrastructurally, the malignant tumor consisted of three principal cell types; histiocyte-like cells, fibroblast-like cells, and intermediate cells, with unique attendance of myofibroblasts. This may be the first report of the presence of myofibroblasts in malignant giant cell tumor of tendon sheath. Enzyme cytochemistry revealed various functional properties of histiocytes.     

MALIGNANT GIANT CELL TUMOR OF TENDON SHEATH Report of a Case

In a patient with pigmented villonodular synovitis of the right knee joint, there occurred a malignant giant cell tumor of tendon sheath. There was clinical evidence of metastasis after the second local recurrence and the recurrent tumors were studied enzyme cytochemically and electron microscopically. Ultrastructurally, the malignant tumor consisted of three principal cell types; histiocyte-like cells, fibroblast-like cells, and intermediate cells, with unique attendance of myofibroblasts. This may be the first report of the presence of myofibroblasts in malignant giant cell tumor of tendon sheath. Enzyme cytochemistry revealed various functional properties of histiocytes. ACTA PATHOL. JPN. 35 : 699–709, 1985.     

膝关节置换与关节镜治疗膝关节色素沉着绒毛结节性滑膜炎

背景：外科治疗色素沉着绒毛结节性滑膜炎能够最大程度的切除病变滑膜,最大限度的恢复关节功能。目的：探讨人工全膝关节置换与关节镜下滑膜切除治疗膝关节色素沉着绒毛结节性滑膜炎的膝关节功能和复发率。方法：回顾性分析中南大学湘雅医院骨科2006年12月至2011年12月收治的34例膝关节色素沉着绒毛结节性滑膜炎行手术治疗患者,治疗后病检均为弥漫性色素沉着绒毛结节性滑膜炎,其中24例患者行膝关节镜下滑膜切除,10例色素沉着绒毛结节性滑膜炎患者行人工全膝关节置换,治疗后根据病情行辅助放疗。膝关节镜下滑膜切除组采用Lysholm功能评分,人工全膝关节置换组采用美国膝关节协会评分,比较两组患者治疗前后膝关节功能,随访观察比较两组间的膝关节功能恢复情况及复发率。结果与结论：34例膝关节色素沉着绒毛结节性滑膜炎患者均获有效随访,随访时间12-66个月,平均41.3个月。统计学分析结果表明关节镜下滑膜切除组治疗后膝关节Lysholm功能评分为(86.3±10.3)分,明显高于治疗前的(55.5±13.2)分(t=3.81,P =0.016,P < 0.05)。人工全膝关节置换组美国膝关节协会评分由治疗前的(40.7±2.2)分提高到(90.2±1.1)分(t =6.27,P < 0.01);关节镜下滑膜切除组美国膝关节协会评分由治疗前的(34.2±3.9)分提高到(80.8±1.9)分 (t =16.58,P < 0.01)。证实,关节镜下滑膜切除结合辅助放疗治疗膝关节色素沉着绒毛结节性滑膜炎可取得较好效果,人工全膝关节置换治疗晚期膝关节弥漫型色素沉着绒毛结节性滑膜炎可较好恢复膝关节功能,且复发率较低。中国组织工程研究杂志出版内容重点：人工关节;骨植入物;脊柱;骨折;内固定;数字化骨科;组织工程全文链接：     

Giant cell tumor of bone express p63.

p63 contributes to skeletal development and tumor formation; however, little is known regarding its activity in the context of bone and soft tissue neoplasms. The purpose of this study was to investigate p63 expression in giant cell tumor of bone and to determine whether it can be used to discriminate between other giant cell-rich tumors. Seventeen cases of giant cell tumor of bone were examined to determine the cell type expressing p63 and identify the isoforms present. Total RNA or cell protein was extracted from mononuclear- or giant cell-enriched fractions or intact giant cell tumor of bone and examined by RT-PCR or western blot, respectively. Immunohistochemistry was used to evaluate p63 expression in paraffin embedded sections of giant cell tumor of bone and in tumors containing multinucleated giant cells, including: giant cell tumor of tendon sheath, pigmented villonodular synovitis, aneurysmal bone cyst, chondroblastoma, and central giant cell granuloma. The mononuclear cell component in all cases of giant cell tumor of bone was found to express all forms of TAp63 (alpha, beta, and gamma), whereas only low levels of the TAp63 alpha and beta isoforms were detected in multinucleated cells; DeltaNp63 was not detected in these tumors. Western blot analysis identified p63 protein as being predominately localized to mononuclear cells compared to giant cells. This was confirmed by immunohistochemical staining of paraffin-embedded tumor sections, with expression identified in all cases of giant cell tumor of bone. Only a proportion of cases of aneurysmal bone cyst and chondroblastoma showed p63 immunoreactivity whereas it was not detected in central giant cell granuloma, giant cell tumor of tendon sheath, or pigmented villonodular synovitis. The differential expression of p63 in giant cell tumor of bone and central giant cell granuloma suggest that these two tumors may have a different pathogenesis. Moreover, p63 may be a useful biomarker to differentiate giant cell tumor of bone from central giant cell granuloma and other giant cell-rich tumors, such as giant cell tumor of tendon sheath and pigmented villonodular synovitis.     

Extensive pigmented villonodular synovitis with markedly pigmented lymphadenopathy and its implication for differential diagnosis with malignant melanoma

A 51-year-old male presented with a 5 cm left knee mass. Fine needle aspiration revealed large epithelioid cells with prominent nucleoli and abundant cytoplasmic pigment, consistent with malignant melanoma. Left inguinal lymphadenopathy was present, which was suspicious for metastatic disease by ultrasound examination. A dark perianal skin lesion was also identified, therefore raising the possibility of a primary melanoma. The knee and perianal lesions were resected and inguinal sentinel node biopsy was performed. In the specimen from the knee, there were clusters and fascicles of spindle and epithelioid cells with prominent nucleoli. Many of the cells displayed abundant, granular, brown, cytoplasmic pigment. The lymph node showed clusters of similar cells located in the subcapsular sinus. Immunohistochemical study showed that the cells expressed CD68, but failed to express S-100, MART-1, and gp100. The cytoplasmic pigment was positive for iron staining. The final diagnosis was pigmented villonodular synovitis. This case illustrates that pigmented villonodular synovitis may present with lymphadenopathy, mimicking a malignant process, including melanoma. Immunohistochemical studies may be essential for establishing the correct diagnosis.     

Extrarenal primitive malignant tumor with rhabdoid features: fine-needle aspiration cytology, immunocytochemistry, and electron microscopy of a case

We describe a malignant pelvic tumor with rhabdoid-like features that arose in the sacroiliac region of a 15-year-old girl. Fine-needle aspiration (FNA) cytology showed large cells with glassy cytoplasm, globular hyaline cytoplasmic inclusions, and prominent nucleoli. Immunocytochemical methods applied to FNA material revealed immunoreactivity for cytokeratin, epithelial membrane antigen, and vimentin; the cells failed to react with antibodies to desmin. This distinct appearance and phenotype was most consistent with a diagnosis of extrarenal malignant rhabdoid tumor. Electron microscopy confirmed this interpretation, showing whorled filamentous aggregates in the cytoplasm of the tumor cells.     

Temporomandibular joint neoplasms and pseudotumors

Neoplasms and pseudotumors of the temporomandibular joint (TMJ) are very uncommon. Early recognition of such will prevent therapeutic delay and may have a dramatic impact on patient morbidity and mortality. Included in rare TMJ lesions are the following: 1) synovial chondromatosis, 2) osteochondroma, 3) osteoma, 4) osteoblastoma, 5) pigmented villonodular synovitis, 6) ganglion, 7) synovial cyst, 8) simple bone cyst, 9) aneurysmal bone cyst, 10) epidermal inclusion cyst, 11) hemangioma, 12) nonossifying fibroma, 13) Langerhans cell histiocytosis, 14) plasma cell myeloma, 15) sarcoma.     

Rapidly destruction of the hip joint associated with enlarged iliopsoas bursa in a patient with refractory rheumatoid arthritis

A case of refractory rheumatoid arthritis with a rapid destruction of the hip joint and an enlarged iliopsoas bursa is presented. Rapidly destructive coxarthrosis, chondrocalcinosis, suppurative arthritis, and pigmented villonodular synovitis were the differential diagnoses. Radiological examination showed that rheumatoid arthritis was most likely diagnosis. The patient was treated with total hip arthroplasty and etanercept, with good results.     

Primary synovial epithelioid sarcoma of the knee: distinctly unusual location leading to its confusion with pigmented villonodular synovitis

Epithelioid sarcoma (ES) is a rare malignant soft tissue tumor occurring in the distal extremities of young adults and is characterized histologically by nodules of epithelioid cells showing central necrosis. Intra‐articular ES is extremely rare; only four cases have been reported, but their radiologic and histologic documentation of intra‐articular origin have been imprecise. We report the first radiologically and histologically well‐documented case of primary synovial ES. A 59‐year‐old woman presented with pain followed by swelling of her right knee for 6 months. MRI revealed an entirely intra‐articular nodular synovial mass in the lateral part of the right knee joint in a background of diffusely thickened synovium. Synovectomy was performed under the clinical impression of pigmented villonodular synovitis (PVNS), a diagnosis erroneously confirmed by the reporting pathologist. The tumor rapidly recurred 3 months afterward and the diagnosis of primary synovial ES was made. Despite above‐knee amputation, the tumor continued to spread proximally to the retroperitoneum. She developed multiple lung metastases and died 20 months after initial presentation. The nodular aggregates of tumor cells with central necrosis resulted in diffuse polypoid synovial thickening mimicking tuberculous synovitis and PVNS. The tumor cells showed positive staining for EMA, CK19, CD34, and complete loss of INI1 staining, establishing the diagnosis of primary synovial ES. The ES spread from the synovium to and along the joint capsule, and then extra‐articularly into the soft tissue surrounding the knee joint, with lymphovascular permeation. Such pattern of spread calls for radical surgical excision as the treatment of choice.     

关节镜在不同膝关节滑膜病变中的诊疗分析

目的观察关节镜在不同膝关节滑膜病变中的诊断、治疗作用,分析不同病变的临床疗效。方法选取50例关节镜下诊断、治疗的膝关节滑膜病变患者为研究对象,术前拟诊为类风湿性关节炎10例,色素沉着绒毛结节性滑膜炎11例,膝关节慢性感染5例,慢性非特异性滑膜炎12例,膝关节滑膜结核5例,半月板损伤4例,不明原因3例,记录关节镜对膝关节滑膜病变的诊治效果。结果关节镜结合病理检查结果,术后10例患者更正临床诊断,所有伤口均一期愈合,无严重并发症发生。出院后所有患者均获得随访,6例色素沉着绒毛结节性滑膜炎,2例类风湿性关节炎,1例滑膜结核,1例慢性非特异性滑膜炎术后复发,其余患者术后膝关节功能均获得显著改善,总有效率80.0%。结论关节镜检查有利于明确诊断,而且微创可彻底切除病变的滑膜组织,耐受性好。     

Pathology of the synovium

Synovium is specialized mesenchymal tissue that is essential for the appropriate function of the locomotor apparatus. It is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. In this article, the normal microscopic anatomy of synovium is briefly reviewed. Synovial proliferative disorders, including pigmented villonodular synovitis, giant cell tumor of tendon sheath, hemosiderotic synovitis, and fatty infiltration of the synovial membrane are discussed. Additionally, the subjects of intrasynovial cartilaginous lesions (primary and secondary synovial chondromatosis) and crystal deposition diseases are reviewed. Finally, the response of synovial tissues to implanted foreign materials that are used in large and small joint arthroplasty are described.     

Malignant giant cell tumor of synovium and locally destructive pigmented villonodular synovitis: ultrastructural and immunohistochemical study and review of the literature

The first reported case of an intraarticular malignant giant cell tumor of synovium studied with electron microscopic and immunohistochemical examination is presented, together with a case of diffuse intraarticular pigmented villonodular synovitis with extensive bone destruction. The malignant case was dominated by uniform cells positive for histiocytic markers, the fine structure showing a gradual change from cells dominated by organelles serving a secretory function to cells with phagocytic activity. The reported cases of giant cell tumor of the tendon sheath indicate that the pertinent histologic changes regarding malignancy are an increase in cell polymorphism and in the number of mitoses, and a decrease in the number of multinucleated giant cells.     

Ameloblastic fibrosarcoma: a cytologist's perspective

Fine-needle aspiration cytology of a case of ameloblastic fibrosarcoma (AFS), an unusual odontogenic tumor related to ameloblastoma (AB), was performed in a 25-year-old female with a 1 cm swelling in the left lower orbital region along with involvement of zygomatic region. Aspiration of the tumor yielded a cellular sample composed predominantly of mesenchymal element and few clusters representing epithelial component showing tall columnar cells with peripheral palisading. Detailed cytomorphological features of AFS are discussed along with differential diagnosis from other tumors such as AB, desmoplastic AB, odontogenic fibroma, ameloblastic fibrodentinoma and ameloblastic fibro-odontoma, ameloblastic fibroma.     

PIGMENTED VILLONODULAR SYNOVITIS A Clinicopathologic Study of 52 Cases

Clinicopathologic, enzyme histochemical and electron microscopic findings in 52 patients with pigmented villonodular synovitis (PVS) are reported. The lesion was by far the most common in the knee joint (48%), followed by the ankle joint (25%). As to sex incidence, there seemed to be no predilection (46% in men, 54% in women). Microscopically, the PVS showed thin or thick villous projections of the involved synovial membrane, associated with or without nodular formation. The nodule of PVS consisted essentially of a proliferation of histiocyte-like cells with phagocytic activities. Another characteristic feature was large clefts and pseudoglandular or alveolar spaces lined by synovial cells. Enzyme histochemical studies revealed that the lesional cells had functional properties of macrophages. Electron microscopically, the lesion consisted essentially of histiocyte-like and flbroblast-like cells, together with intermediate cells and myofibroblasts.