Pancreatic PEComa (
sugar tumor): MDCT and EUS features.
(1) The PEComa family includes angiomyolipoma, clear cell
sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomic sites.
Comparison of melanoma antigen recognized by T cells (MART-1) to HMB-45: additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell
sugar tumor. Mod Pathol.
Primary extrapulmonary
sugar tumor (PEST): a report of four cases.
The PEComa family of tumors includes angiomyolipoma, lymphangioleiomyomatosis, clear cell
sugar tumor of the lung, and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres.
(1) In 1991, Pea and colleagues (2) first noted this unusual cell in both angiomyolipoma (AML) and clear cell
sugar tumor (CCST) of the lung.
Comparison of melanoma antigen recognized by T-cells (MART-1) to HMB-45: additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell
sugar tumor. Mod Pathol.
In the early 1990s it was recognized that renal angiomyo lipomas, clear cell
sugar tumors, and pulmonary lymphangioleiomyomatosis constitute a group of lesions that have similar histopathologic features and share the expression of melanocytic antigens.
In contrast to sclerosing hemangiomas, clear cell tumors (so-called
sugar tumors) have abundant clear cells with scant stroma, thin-walled vessels, and strong HMB-45 expression.
It also should be noted that A103 immunostaining may be seen in angiomyolipomas, lymphangioleiomyomatosis, clear cell
sugar tumors, and extra-adrenal steroid-producing tumors such as Leydig cell tumors and granulosa cell tumors.