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Presented by Fred Askin, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 4: The patient is a 54 year-old male with a large mass identified adjacent to the left kidney.
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Week 231: Case 4
The patient is a 54 year-old male with a large mass identified adjacent to the left kidney.images/7 15 05 case 4 1.jpg
images/7 15 05 case 4 2.jpg
images/7 15 05 case 4 3.jpgCorrect
Answer: Angiomyolipoma
Histology: Angiomyolipoma is a neoplasm composed of varying admixtures of blood vessels, peculiar smooth muscle, and adipose tissue. The smooth muscle cells are often intimately associated with the outer portion of the muscular wall of the constituent blood vessels, and the muscle cells are arranged in a radial configuration. The tumors may range widely in their histology and in varying proportions of fat and smooth muscle. Lesions with pronounced epithelioid features are considered to have malignant potential.
Discussion: Angiomyolipoma has a characteristic constituent of peculiar epithelioid or spindle cells that have some features suggestive of smooth muscle. These cells stain with S-100 related protein and a variety of melanoma antigens, including HMB-45. Angiomyolipoma is now considered to be one of the lesions of the periepithelial cell, and thus is a “PEComa”.
Reference(s):
– 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, 26: 1-13.
– Murphy WM, Grignon DJ, Pearlman EJ, Editors. Tumors of the Kidney, Bladder, and Related Urinary Structures. Washington, D.C., American Registry of Pathology, 2004.Incorrect
Answer: Angiomyolipoma
Histology: Angiomyolipoma is a neoplasm composed of varying admixtures of blood vessels, peculiar smooth muscle, and adipose tissue. The smooth muscle cells are often intimately associated with the outer portion of the muscular wall of the constituent blood vessels, and the muscle cells are arranged in a radial configuration. The tumors may range widely in their histology and in varying proportions of fat and smooth muscle. Lesions with pronounced epithelioid features are considered to have malignant potential.
Discussion: Angiomyolipoma has a characteristic constituent of peculiar epithelioid or spindle cells that have some features suggestive of smooth muscle. These cells stain with S-100 related protein and a variety of melanoma antigens, including HMB-45. Angiomyolipoma is now considered to be one of the lesions of the periepithelial cell, and thus is a “PEComa”.
Reference(s):
– 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, 26: 1-13.
– Murphy WM, Grignon DJ, Pearlman EJ, Editors. Tumors of the Kidney, Bladder, and Related Urinary Structures. Washington, D.C., American Registry of Pathology, 2004.
