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Malignant Pigmented Villonodular Synovitis-A Rare Entity.

Radha Sistla1, Vidyasagar J V S1, Tameem Afroz1

  • 1Department of Anatomical Pathology and Cytology, Aware Global Hospitals, L.B Nagar, Hyderabad-500035, Andhra Pradesh. India.

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Summary

Malignant pigmented villonodular synovitis (PVNS) can metastasize to lymph nodes, a rare occurrence. Early diagnosis and wide surgical excision are crucial for managing this aggressive tumor.

Keywords:
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Area of Science:

  • Oncology
  • Pathology
  • Surgical Oncology

Background:

  • Malignant pigmented villonodular synovitis (PVNS), also known as malignant giant cell tumor of the tendon sheath (MGCTTS), is a rare and controversial neoplasm.
  • While typically a localized tumor, rare cases exhibit metastatic potential, with fewer than 20 documented instances in medical literature.

Observation:

  • A 65-year-old female patient with a history of recurrent PVNS developed inguinal swelling.
  • Imaging revealed extensive lymphadenopathy in the iliac region, indicative of metastatic disease.
  • Histopathological and immunohistochemical analysis confirmed metastatic PVNS.

Findings:

  • The patient presented with metastatic PVNS to inguinal and pelvic lymph nodes, a highly unusual presentation.
  • Despite initially bland cytologic features, the lesion demonstrated aggressive behavior with local recurrence and distant metastasis.

Implications:

  • High clinical suspicion is essential for diagnosing aggressive PVNS, even with unremarkable cytology.
  • Prompt diagnosis and wide surgical excision with clear margins are critical for potential limb salvage and improved outcomes in recurrent GCTTS.