The family of tumors derived from mesenchymal perivascular epithelioid cells (so-called PEComas) includes angiomyolipoma, lymphangioleiomyomatosis, clear cell sugar tumor of the lung, clear cell myomelanocytic tumor of ligamentum teres/falciform ligament, and abdominopelvic sarcoma of perivascular epithelioid cells. These tumors were characterized by coexpression of melanocytic (HMB-45) and muscle markers.

Members

 angiomyolipoma
 lymphangioleiomyomatosis
 clear cell sugar tumor of the lung
 clear cell myomelanocytic tumor of ligamentum teres/falciform ligament (#10976698#)
 abdominopelvic sarcoma of perivascular epithelioid cells

Localization

 digestive PEComas (#15614743#)

• jejunum (#12650518#)
• ileon (#16343800#)
• colon (#16398680#)

 common bile duct (#15252321#)
 uterus (#16327428#, #15213607#, #15043315#, #13678746#)
 uterine cervix (#15494070#)
 prostate (#12562263#)
 tigh (#11756764#)
 falciform ligament (#10976698#)
 skin (#15842631#)
 skull base (#15316324#)
 soft tissue (#15577688#)

• orbit (#15803216#)
• broad ligament (#15361222#)
• breast (#11979098#)

Variants

 malignant perivascular epithelioid cell tumor

Immunochemistry

 HMB-45+
 progesterone receptor+
 MyoD1+ (#12778001#)

 CK-
 EMA-
 melan-A-
 S100-

See also

 Tumors

• clear cell tumors
  • clear cell carcinomas
  • clear cell sarcomas

References

 Vang R, Kempson RL. Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol. 2002 Jan;26(1):1-13. PMID: #11756764#

 Tazelaar HD, Batts KP, Srigley JR. Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol. 2001 Jun;14(6):615-22. PMID: #11406665#

 Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol. 2000 Sep;24(9):1239-46. PMID: #10976698#