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Presented by Dr. Pedram Argani and prepared by Dr. Jason Kern
Case 3: This is a 2 year old female with a lesion on the forearm
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1. Question
Week 615: Case 3
This is a 2 year old female with a lesion on the forearmCorrect
Answer: Infantile fibromatosis
Histologic Description: This is a fairly bland but infiltrative spindle cell lesion. The spindle cells show minimal mitotic activity, and are associated with collagen production. The supporting capillary vasculature is prominent, as is typically seen in the fibromatosis.
Differential Diagnosis: Infantile fibrosarcoma may overlap in some cases with infantile fibromatosis, and most of these variant lesions appear to be cellular variants of infantile fibromatosis genetically. However, infantile fibrosarcoma typically demonstrates poorly formed fascicles of spindle cells, greater mitotic activity and necrosis. Low grade fibromyxoid sarcoma typically has focal nodules of myxoid areas associated arcade-like vasculature in the background of a more hyalinized fibroblastic proliferation showing nuclear hyperchromasia. Low grade fibromyxoid sarcoma labels for MUC4. Rhabdomyosarcoma typically demonstrates more coarse chromatin and eosinophilic cytoplasm, and would label for myogenic markers like myogenin.
Fibromatosis in infants often does not demonstrate nuclear beta-catenin expression.
Incorrect
Answer: Infantile fibromatosis
Histologic Description: This is a fairly bland but infiltrative spindle cell lesion. The spindle cells show minimal mitotic activity, and are associated with collagen production. The supporting capillary vasculature is prominent, as is typically seen in the fibromatosis.
Differential Diagnosis: Infantile fibrosarcoma may overlap in some cases with infantile fibromatosis, and most of these variant lesions appear to be cellular variants of infantile fibromatosis genetically. However, infantile fibrosarcoma typically demonstrates poorly formed fascicles of spindle cells, greater mitotic activity and necrosis. Low grade fibromyxoid sarcoma typically has focal nodules of myxoid areas associated arcade-like vasculature in the background of a more hyalinized fibroblastic proliferation showing nuclear hyperchromasia. Low grade fibromyxoid sarcoma labels for MUC4. Rhabdomyosarcoma typically demonstrates more coarse chromatin and eosinophilic cytoplasm, and would label for myogenic markers like myogenin.
Fibromatosis in infants often does not demonstrate nuclear beta-catenin expression.