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osseous PEComa

PEComa in bone

PEComas other than angiomyolipoma, lymphangioleiomyomatosis, and clear cell sugar tumor of the lung are relatively rare, and PEComas presenting in bone are especially rare.

Both the primary and metastatic PEComas can present in bone, although both are rare.

It may be suggested that primary PEComa of bone tends to involve lower extremities.

Histologically, they show similar cytomorphology, nested architecture, and characteristic vessels as PEComas at other sites.

In addition, a significant subset of the primary bone lesions seem to be malignant.

References

- PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Yamashita K, Fletcher CD. Am J Surg Pathol. 2010 Nov;34(11):1622-9. Review. PMID: #20975340#