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Malignant Pigmented Villonodular Synovitis-A Rare Entity

INTRODUCTION: Malignant pigmented villonodular synovitis (PVNS) or Malignant giant cell tumour tendon sheath (MGCTTS) is a controversial and debatable lesion. Few case reports have indicated the potential for metastasis.1These aggressive cases are designated malignant giant cell tumour tendon sheath...

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Autores principales: Sistla, Radha, J.V.S, Vidyasagar, Afroz, Tameem
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Indian Orthopaedic Research Group 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4719277/
https://www.ncbi.nlm.nih.gov/pubmed/27298991
http://dx.doi.org/10.13107/jocr.2250-0685.214
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author Sistla, Radha
J.V.S, Vidyasagar
Afroz, Tameem
author_facet Sistla, Radha
J.V.S, Vidyasagar
Afroz, Tameem
author_sort Sistla, Radha
collection PubMed
description INTRODUCTION: Malignant pigmented villonodular synovitis (PVNS) or Malignant giant cell tumour tendon sheath (MGCTTS) is a controversial and debatable lesion. Few case reports have indicated the potential for metastasis.1These aggressive cases are designated malignant giant cell tumour tendon sheath or malignant PVNS. Less than 20 cases are described in literature. We report a case of 65 year old lady who was diagnosed eight years back as pigmented villonodular synovitis. She had multiple local recurrences and now presented with lymphnodal metastases, which is extremely rare. CASE REPORT: Sixty five year old lady presented with swelling in left inguinal region of six months duration. She gave a past history of swelling around medial condyle of left femur eight years back. Swelling was excised three times. At the time of third recurrence, swelling was extensive, infiltrating surrounding tissues and underlying bone, encasing femoral and popliteal vessels for which she underwent an above knee amputation. She now presented with inguinal swelling measuring 5.7×3.0 cms. Positron Emission Tomography Scan (PET-CT) revealed multiple enlarged left common iliac, internal and external iliac nodes, largest measuring 7.0 cms. Both the inguinal and pelvic nodes were excised. Lesion was diagnosed as metastatic deposits of Malignant pigmented villonodular synovitis based on morphological and Immunohistochemical findings. CONCLUSION: It is important to have a high index of clinical suspicion because these lesions can have an aggressive behaviour even with bland cytological features. Our experience suggests that in a recurrent lesion for GCTTS. A wide surgical excision with safe surgical margins and close follow up with radiological evaluation might help to diagnose these lesions early and be amenable to limb salvage surgeries.
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spelling pubmed-47192772016-06-13 Malignant Pigmented Villonodular Synovitis-A Rare Entity Sistla, Radha J.V.S, Vidyasagar Afroz, Tameem J Orthop Case Reports Case Report INTRODUCTION: Malignant pigmented villonodular synovitis (PVNS) or Malignant giant cell tumour tendon sheath (MGCTTS) is a controversial and debatable lesion. Few case reports have indicated the potential for metastasis.1These aggressive cases are designated malignant giant cell tumour tendon sheath or malignant PVNS. Less than 20 cases are described in literature. We report a case of 65 year old lady who was diagnosed eight years back as pigmented villonodular synovitis. She had multiple local recurrences and now presented with lymphnodal metastases, which is extremely rare. CASE REPORT: Sixty five year old lady presented with swelling in left inguinal region of six months duration. She gave a past history of swelling around medial condyle of left femur eight years back. Swelling was excised three times. At the time of third recurrence, swelling was extensive, infiltrating surrounding tissues and underlying bone, encasing femoral and popliteal vessels for which she underwent an above knee amputation. She now presented with inguinal swelling measuring 5.7×3.0 cms. Positron Emission Tomography Scan (PET-CT) revealed multiple enlarged left common iliac, internal and external iliac nodes, largest measuring 7.0 cms. Both the inguinal and pelvic nodes were excised. Lesion was diagnosed as metastatic deposits of Malignant pigmented villonodular synovitis based on morphological and Immunohistochemical findings. CONCLUSION: It is important to have a high index of clinical suspicion because these lesions can have an aggressive behaviour even with bland cytological features. Our experience suggests that in a recurrent lesion for GCTTS. A wide surgical excision with safe surgical margins and close follow up with radiological evaluation might help to diagnose these lesions early and be amenable to limb salvage surgeries. Indian Orthopaedic Research Group 2014 /pmc/articles/PMC4719277/ /pubmed/27298991 http://dx.doi.org/10.13107/jocr.2250-0685.214 Text en Copyright: © Indian Orthopaedic Research Group http://creativecommons.org/licenses/by-nc-sa/3.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc-sa/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Sistla, Radha
J.V.S, Vidyasagar
Afroz, Tameem
Malignant Pigmented Villonodular Synovitis-A Rare Entity
title Malignant Pigmented Villonodular Synovitis-A Rare Entity
title_full Malignant Pigmented Villonodular Synovitis-A Rare Entity
title_fullStr Malignant Pigmented Villonodular Synovitis-A Rare Entity
title_full_unstemmed Malignant Pigmented Villonodular Synovitis-A Rare Entity
title_short Malignant Pigmented Villonodular Synovitis-A Rare Entity
title_sort malignant pigmented villonodular synovitis-a rare entity
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4719277/
https://www.ncbi.nlm.nih.gov/pubmed/27298991
http://dx.doi.org/10.13107/jocr.2250-0685.214
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