Case 3

A 48-year-old male presented with a huge mass about the left shoulder. He had developed a small mass at the same site at age 9 that was locally excised in the Great Britain, UK and subsequently had rexcision of recurrences at ages 11,12,16,18, and 23. He then emigrated to South Africa and in the ensuing 20-years had 12 more procedures for recurrences. Four years prior to presentation he was treated with Tamoxifen with no change in its course. He sought consultation in the US and because of pain and neurologic disability a forequarter amputation was performed.
    A: Appearance at presentation.
    B: AP radiograph of the shoulder at presentation.
    C: Axial CT image cut through the mid-portion of the mass.
    D: Axial CT image cut through the distal portion of the mass.
    E: Cut surface of the axially sectioned surgical specimen corresponding to the distal CT image.
    F: A representative field from a recurrent lesion in the UK, age 16. The field contains mature heavily collaginized fibrous tissue with a low cell to matrix ratio. There is no cellular atypia nor were mitoses seen. Given his long history of recurrences without metatases a diagnosis of recurrent fibromatosis was made.
    G: A representative field from a recurrent lesion excised in South Africa, age 31. A closer look at the cellular detail in this recurrence again shows no cellular atypia or other signs of malignancy and the diagnosis of recurrent fibromatosis was repeated.
    H: A representative field from the amputation specimen. Greater magnification of more cellular areas shows less maturity of the collagen and modest cellular aypia. Occasional mitotic figures were seen.

Diagnosis: In view of the increasing clinical aggressiveness a diagnosis of low grade fibrosarcoma protruberans was made and the issue of possible malignant transformation following multiple recurrrences of fibromatosis was raised.

The patient has remained without evidence of recurrence or metastasis for five years.