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Presented by Pedram Argani, M.D. and prepared by Justin Poling, M.D.
Case 3: This is 6 year old female with a 5 cm deep thigh mass.
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1. Question
Week 566: Case 3
This is 6 year old female with a 5 cm deep thigh mass.images/poling/09092013/3_2x.jpg
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images/poling/09092013/3_20x2.jpgCorrect
Answer: Low-grade fibromyxoid sarcoma
Histology: This a remarkably bland lesion, composed of uniform fibroblastic cells with a whorled pattern and a variably dense collagenous and focally myxoid matrix. The transitions between the myxoid and collagenous areas are abrupt, and blood vessels are arranged in thin branching arcades in the myxoid areas. Mitoses and pleomorphism are not readily evident. The lesion is negative for S100 protein, CD34, actin, and EMA. By genetics, the tumor harbors t(7;16)(q34;p11) translocation, resulting in a FUS-CREB3L2 fusion oncogene.
Discussion: Perineurioma may overlap with low grade fibromyxoid sarcoma morphologically. However, perineurioma should label for EMA (which low grade fibromyxoid sarcoma may occasionally do), and would lack the hyperchromatic nuclei of the current lesion. Neurofibromas label for S100 protein, and frequently are associated with nerve trunks. Myxofibrosarcoma demonstrates far greater pleomorphism and typically affects adult patients.
Low grade fibromyxoid sarcoma will metastasize in up to 30% of cases, and these metastases may be delayed and involve unusual sites (such as other soft tissue locations).
Incorrect
Answer: Low-grade fibromyxoid sarcoma
Histology: This a remarkably bland lesion, composed of uniform fibroblastic cells with a whorled pattern and a variably dense collagenous and focally myxoid matrix. The transitions between the myxoid and collagenous areas are abrupt, and blood vessels are arranged in thin branching arcades in the myxoid areas. Mitoses and pleomorphism are not readily evident. The lesion is negative for S100 protein, CD34, actin, and EMA. By genetics, the tumor harbors t(7;16)(q34;p11) translocation, resulting in a FUS-CREB3L2 fusion oncogene.
Discussion: Perineurioma may overlap with low grade fibromyxoid sarcoma morphologically. However, perineurioma should label for EMA (which low grade fibromyxoid sarcoma may occasionally do), and would lack the hyperchromatic nuclei of the current lesion. Neurofibromas label for S100 protein, and frequently are associated with nerve trunks. Myxofibrosarcoma demonstrates far greater pleomorphism and typically affects adult patients.
Low grade fibromyxoid sarcoma will metastasize in up to 30% of cases, and these metastases may be delayed and involve unusual sites (such as other soft tissue locations).
