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Presented by Pedram Argani, M.D. and prepared by Doreen Nguyen, M.D.
Case 3: a 17 month female with a scalp tumor.
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Question 1 of 1
1. Question
Week 603: Case 3
This is a 17 month female with a scalp tumor.
Correct
Answer: Fibromatosis
Histology: This is a bland but infiltrative spindle cell lesion. The spindle cells form in areas well-formed fascicles with abundant collagen deposition and there are associated capillaries which stand out from the spindle cell proliferation. Other areas of this lesion have more extravasated red blood cells and a less well developed vascular pattern. The lesion demonstrates nuclear labeling for beta-catenin, which supports the diagnosis of fibromatosis.
It is likely that cases such as this have been classified in the past as “cranial fasciitis”, which may account for the beta-catenin labeling reported in such lesions.
Discussion: Fasciitis would lack the well-developed fascicles of the current lesion, and would not infiltrate or demonstrate nuclear labeling for beta-catenin. Low grade fibromyxoid sarcoma has a more whorled, curvilinear pattern, and features nuclei that are hyperchromatic. Synovial sarcoma is typically more cellular than the current lesion, and features plump spindle cells with hyperchromatic nuclei forming short intersecting fascicles.
Incorrect
Answer: Fibromatosis
Histology: This is a bland but infiltrative spindle cell lesion. The spindle cells form in areas well-formed fascicles with abundant collagen deposition and there are associated capillaries which stand out from the spindle cell proliferation. Other areas of this lesion have more extravasated red blood cells and a less well developed vascular pattern. The lesion demonstrates nuclear labeling for beta-catenin, which supports the diagnosis of fibromatosis.
It is likely that cases such as this have been classified in the past as “cranial fasciitis”, which may account for the beta-catenin labeling reported in such lesions.
Discussion: Fasciitis would lack the well-developed fascicles of the current lesion, and would not infiltrate or demonstrate nuclear labeling for beta-catenin. Low grade fibromyxoid sarcoma has a more whorled, curvilinear pattern, and features nuclei that are hyperchromatic. Synovial sarcoma is typically more cellular than the current lesion, and features plump spindle cells with hyperchromatic nuclei forming short intersecting fascicles.
