Korean J Leg Med.  2015 Aug;39(3):84-87. 10.7580/kjlm.2015.39.3.84.

[Secondary publication] Traumatic Rupture of a Huge Renal Angiomyolipoma in a Tuberous Sclerosis Patient

• Ha H
• Park JH
• Cho NY

• KMID: 2044954
• DOI: http://doi.org/10.7580/kjlm.2015.39.3.84

Abstract

A renal angiomyolipoma (AML) is an unusual benign tumor that is composed of adipose tissue, smooth muscle, and abnormal blood vessels. AMLs can occur sporadically or can be associated with tuberous sclerosis. A ruptured renal AML may manifest as lift-threatening hypovolemic shock or sudden death. Herein, the authors present an autopsy case of traumatic rupture of a renal AML in a patient with tuberous sclerosis.

Keyword

Angiomyolipoma; Tuberous sclerosis; Rupture; Autopsy

MeSH Terms

Adipose Tissue
Angiomyolipoma*
Autopsy
Blood Vessels
Death, Sudden
Humans
Muscle, Smooth
Rupture*
Shock
Tuberous Sclerosis*

Figure

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  Fig. 1. The scene.

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  Fig. 2. There are many grayish tan papules on the nose and cheeks in a butterfly distribution.

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  Fig. 3. (A) A well circumscribed, yellowish gray and focally calcified tumor (1.5×1 cm) is located at the wall of lateral ventricle. (B) Histologically, the tumor is composed mainly of elongated tumor cells forming streams and occasional polygonal cells with abundant, glassy cytoplasm (H&E, ×200).

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  Fig. 4. (A) A well-encapsulated mass with nodular appearance is seen in the upper pole of left kidney. The cut surface of the mass is mostly grayish yellow and firm, and shows extensive hemorrhage. (B) Histologically. the tumor is composed of adipose tissue, smooth muscle and vessels (H&E, ×100).

Reference

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4. Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol. 1998; 13:624–8.