Diffuse-type tenosynovial giant cell tumor in association with neurofibromatosis type 1-noonan syndrome: Possibly more than a chance relationship

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Abstract

A case of diffuse-type tenosynovial giant cell tumor arising in the left upper extremity is reported in a 23-year-old man with neurofibromatosis type 1 (NF1)-Noonan syndrome. The predominately mononuclear cellular proliferation with psammomatous calcifications had the immunohistochemical and ultrastructural features of a fibrohistiocytic neoplasm. This uncommon type of soft tissue neoplasm occurring in this unique clinical setting served to open an inquiry into the subject of non-neurogenic tumors in association with NF1 and Noonan syndrome, both manifested in our patient. Nonossifying fibroma of bone as a presumptive fibrohistiocytic tumor is an uncommon but well-documented manifestation in NF1, whereas in Noonan-like syndrome, both giant cell granuloma and pigmented villonodular synovitis are recognized as associated lesions with histologic and immunophenotypic similarities with the diffuse-type tenosynovial giant cell tumor.

Original languageEnglish
Pages (from-to)734-738
Number of pages5
JournalAmerican Journal of Surgical Pathology
Volume30
Issue number6
DOIs
StatePublished - Jun 1 2006

Keywords

  • CD68
  • Diffuse-type giant cell tumor
  • Neurofibromatosis type 1
  • Noonan-like syndrome
  • Psammoma bodies
  • Tenosynovial giant cell tumor

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