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Abstract

Pigmented villonodular synovitis (PVNS) is a benign disease that rarely undergoes malignant transformation. Th ere are two types of disease: localized (nodular tenosynovitis) and diff used (pigmented villonodular synovitis/tenosynovitis) with intra- or extra-articular locations. Th e second one is limited to synovium of the burse (PVNB) or tendon sheath (PVNTS). Th e intraarticular lesions are usually located in the knee, hip, ankle and elbow joints. Histologically, PVNS is a tenosynovial giant cell tumor, characterized by proliferation of two types of mononuclear cells — predominantly small, histiocyte-like cells and larger cells with dense cytoplasm, reniform or lobulated nucleus, with accompanying multinucleated giant cells and macrophages overloaded with hemosiderin that give typical image on MRI — currently selected as a gold standard for its diagnosis. Th e classic X-ray and CT are non-specifi c but similar to ultrasound should be used to evaluate disease progression and treatment response if radiotherapeutic and pharmacological methods were selected for treatment. An open arthroscopic surgery could also be applied in selected cases.

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Authors and Affiliations

Artur Fałek
Joanna Niemunis-Sawicka
Katarzyna Wrona
Grzegorz Szczypiór
Ludomira Rzepecka-Wejs
Katarzyna Cięszczyk
Maciej Burdan
Michał Puderecki
Paulina Burzec
Barbara Marzec-Kotarska
Justyna Szumiło
Franciszek Burdan
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Abstract

Tenosynovial Giant Cell Tumor (TGCT) is a group of typically benign lesions arising from the synovium of joints, bursae and tendon sheaths. Depending on their growth pattern and clinical course, they are divided into localized and diffuse types. It is predominantly caused by a mutation in the stromal cells of the synovial membrane leading to overexpression of the colony stimulating factor 1 that recruits CSF1R-expressing cells of the mononuclear phagocyte lineage into the tumor mass. The lesions contain mainly histiocyte-like and synovial cells accompanied by varying numbers of multinucleated giant cells, mononuclear cells, foam cells, inflammatory cells and hemosiderin deposits. The gold standard for detect-ing and monitoring the disease is MRI, where the characteristic hemosiderin accumulation can be best appreciated, but it is a histological examination that is most conclusive. The main treatment is surgical resection of all pathological tissue, but radio- and chemotherapy are also viable options for certain groups of patients.
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Authors and Affiliations

Monika Kager
1
Richard Kager
2
Paulina Fałek
3
Artur Fałek
3
Grzegorz Szczypiór
4
Joanna Niemunis-Sawicka
4 5
Ludomira Rzepecka-Wejs
6
Elżbieta Starosławska
1
Franciszek Burdan
1 7

  1. Radiology Department, St Johns’ Cancer Center, Lublin, Poland
  2. Public Independent Clinical Hospital Number 4, Lublin, Poland
  3. Independent Public Healthcare, Puławy, Poland
  4. MRI Unit of Maritime Medical Center, Gdańsk, Poland
  5. 2nd Radiology Department, Medical University of Gdańsk, Gdańsk, Poland
  6. Goris-Med, Gdańsk, Poland
  7. Human Anatomy Department, Medical University of Lublin, Lublin, Poland

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