Wünderlich syndrome from a malignant epithelioid angiomyolipoma
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Arrabal Polo, Miguel Ángel; Arrabal-Martín, Miguel; Palao-Yago, Francisco; Jiménez-Pacheco, Antonio; García-Galvis, Olga Fernanda; Zuluaga-Gómez, ArmandoEditorial
Shaheed Beheshti Medical University. Urology and Nephrology Research Center
Materia
Kidney neoplasms Angiomyolipoma Hemorrhage Epithelioid cells Renal cell carcinoma
Date
2009Referencia bibliográfica
Arrabal Polo, M.A.; et al. Wünderlich syndrome from a malignant epithelioid angiomyolipoma. Urology Journal, 6(1): 50-53 (2009). [http://hdl.handle.net/10481/33163]
Abstract
Angiomyolipoma of the kidney
has classically been considered as
a tumor of the connective tissue
composed of fat, vascular tissue,
and smooth muscle. In most cases,
it is a tumor with benign behavior
that may appear sporadically or
associated with tuberous sclerosis
syndrome. Macroscopically, these
tumors are greyish yellow in color,
and under an optical microscope,
they are characterised by presenting
the three components described.
In recent years, several authors
have published cases of epithelioid
angiomyolipomas characterized
by a minimal presence of fat
in the tumor, positive for the
melanoma-specific antigen, HMB-
45,(1) and on occasion, positive for
desmin, melan-A, and others.(2,3)
Computed axial tomography and
nuclear magnetic resonance studies
hardly differentiate epithelioid
angiomyolipomas from renal
cell carcinoma.(3) We present a
case of malignant epithelioid
angiomyolipoma in a woman
with no tuberous sclerosis, the
debut of which was spontaneous
retroperitoneal hemorrhage causing
hypovolemic shock that required
left radical nephrectomy.