Acral myxoinflammatory fibroblastic sarcoma
Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade sarcoma. It is most commonly found in the extremities but has been reported elsewhere in the body.
Presentation[]
These lesions present as slow growing painless masses usually in the ankle, foot, wrist or hand. There is normally no overlying skin change.
Plain film X ray may show nothing or slight bone erosion. On MRI these lesions vary considerably. They tend to show the presence of fat and enhance strongly with contrast.[1]
Diagnosis[]
This is made by biopsy and histological examination.
Histologically these lesions are characterised by (1) an inflammatory background (2) a prominent myxoid component (3) enlarged atypical cells with prominent nucleoli and (4) often a bizarrely shaped nucleus. The cells stain positively for vimentin.[citation needed]
Differential diagnosis[]
This includes tenosynovitis, pigmented villonodular synovitis, giant cell tumor of the tendon sheath, ganglion cysts and other sarcomas.[citation needed]
Treatment[]
Complete excision of the lesion is the recommended treatment. Radiation may be useful for local recurrences. It is not known how useful chemotherapy is for this condition as there have been so few cases reported.[citation needed]
Prognosis[]
This lesion appears to be curable by surgery provided adequate margins are obtained. Local recurrences have been reported in ~40% of cases. Only three cases have reported metastatic disease.
Epidemiology[]
This is a rare lesion with less than 150 cases described. The male: female ratio is approximately 1. The age range varies between 4 and 87 years with the majority occurring between 35-45 years. The majority of lesions occur in the ankle, wrist foot and hand.
History[]
This lesion was first described in 1998 independently in three publications.[2][3][4]
References[]
- ^ Narváez JA , Martinez S, Dodd LG and Brigman BE (2007) Acral myxoinflammatory fibroblastic sarcomas: MRI findings in four cases. Musculoskeletal Imaging 188 (5) 1302-1305
- ^ Meis-Kindblom JM, Kindblom LG (1988) Acral myxoinflammatory fibroblastic sarcoma: a low-grade tumor of the hands and feet. Am J Surg Pathol
- ^ Michal M (1988) Inflammatory myxoid tumor of the soft parts with bizarre giant cells. Pathol Res Pract 194:529-533
- ^ Montgomery EA, Devaney KO, Giordano TJ, Weiss SW (1988) Inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells: a distinctive lesion with features simulating inflammatory conditions, Hodgkin's disease, and various sarcomas. Mod Pathol 11:384-391
External links[]
Classification |
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- Rare syndromes