Document Detail

Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease.
MedLine Citation:
PMID:  10757395     Owner:  NLM     Status:  MEDLINE    
The clinical and pathologic features of 50 cases of diffuse-type tenosynovial giant cell tumor (D-TGCT), also known as extraarticular pigmented villonodular tenosynovitis (PVNTS), are presented. Patients' ages ranged from 4 to 76 years (median, 41 yrs), with a slight female predominance (28 women, 22 men). By definition, all lesions presented as predominant soft tissue masses, with or without an associated articular component. Tumor sites included the wrist (9 cases), knee (8 cases), thigh and foot (6 cases each), finger (5 cases), ankle (3 cases), hand, elbow, toes, buttock, paravertebral region (2 cases each), lower leg, sacrococcygeal area, and retroperitoneum; 27 cases were described as entirely extraarticular. Tumors showed infiltrative margins and, in most cases, a sheet-like growth pattern. Striking variation in the number of osteoclast-like giant cells, foamy cells, amount of hemosiderin, and in the degree of stromal hyalinization were responsible for a wide morphologic spectrum. In addition to the predominant histiocyte-like cells, we identified in most cases a subpopulation of large dendritic, desmin-positive cells showing characteristic, but potentially misleading, cytologic features, including abundant eosinophilic cytoplasm, large vesicular nuclei, paranuclear eosinophilic inclusions, and occasional nuclear inclusions. Follow-up information was available for 24 patients, with a duration ranging from 6 months to 30 years (mean, 55 mos). Local recurrence occurred in eight cases (33%), between 4 months and 6 months after surgery (median, 15 mos) and was repeated in five cases; recurrence did not appear to correlate with morphologic parameters. Six cases showed atypical histologic features and four of these contained areas of sarcomatous change. Among the latter, one of three cases with available follow up developed pulmonary metastases and died after 35 months. In addition, one histologically benign lesion gave rise, after two local recurrences, to inguinal and iliac lymph node metastases. Despite this exceedingly uncommon event, we think most cases of D-TGCT are best regarded as benign but locally aggressive neoplasms with significant recurrent potential and should be treated, when possible, by wide excision. Atypical features such as increased mitotic activity, necrosis, spindling of the mononucleate cells, and cytologic atypia are not indicative of malignancy when present individually. This study also confirms the existence of malignant tenosynovial giant cell tumors, some of which are characterized by aggressive behavior.
N S Somerhausen; C D Fletcher
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of surgical pathology     Volume:  24     ISSN:  0147-5185     ISO Abbreviation:  Am. J. Surg. Pathol.     Publication Date:  2000 Apr 
Date Detail:
Created Date:  2000-04-19     Completed Date:  2000-04-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707904     Medline TA:  Am J Surg Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  479-92     Citation Subset:  IM    
Department of Pathology, Brigham & Women's Hospital and Harvard Medical School, Boston, Massachusetts, USA.
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MeSH Terms
Antigens, CD / metabolism
Antigens, Differentiation, Myelomonocytic / metabolism
Child, Preschool
Desmin / metabolism
Giant Cell Tumors / metabolism,  pathology*,  surgery
Immunoenzyme Techniques
Lymph Nodes / pathology
Lymphatic Metastasis / pathology
Middle Aged
Neoplasm Proteins / metabolism
Neoplasm Recurrence, Local / pathology
Soft Tissue Neoplasms / metabolism,  pathology*,  surgery
Synovial Membrane / pathology*,  surgery
Synovitis, Pigmented Villonodular / metabolism,  pathology*,  surgery
Tenosynovitis / metabolism,  pathology*,  surgery
Reg. No./Substance:
0/Antigens, CD; 0/Antigens, Differentiation, Myelomonocytic; 0/CD68 antigen, human; 0/Desmin; 0/Neoplasm Proteins

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