Epithelioid Angiomyolipoma: a Pathological Entity Discovered in Verona with the Endorsement of Doctor Rosai

Overview

Journal Pathologica

Specialty Pathology

Date 2021 Nov 27

PMID 34837089

Citations 1

Authors

Anna Calio

Matteo Brunelli

Stefano Marletta

Giuseppe Zamboni

Franco Bonetti

Maurizio Pea

Guido Martignoni

Affiliations

Soon will be listed here.

Abstract

In this manuscript, we summarize the main features of angiomyolipoma highlighting the recognition of epithelioid angiomyolipoma and the discovery of immunohistochemical expression of HMB45 in a group of tumors that now are referred to as as PEComas. In this scenario, Dr. Rosai believed in our intuition, demonstrating his intellectual honesty and motivated us with his experience ("") and enthusiasm for the new entities (""). He really pushed the improvement of the knowledge in this field.

Citing Articles

The Relevance of the Virchow Node and Virchow Triad in Renal Cancer Diagnosis.

Dorobantu-Lungu L, Dinca V, Gegiu A, Spataru D, Toma A, Welt L Clin Pract. 2025; 15(1).

PMID: 39851801 PMC: 11763355. DOI: 10.3390/clinpract15010018.

Case report: Hepatic epithelioid angiomyolipoma with elevated alpha-fetoprotein and a history of breast cancer.

Zhang X, Chen J, Huang B, Wang L Front Surg. 2022; 9:991228.

PMID: 36157434 PMC: 9500349. DOI: 10.3389/fsurg.2022.991228.

References

Weeks D, Malott R, Arnesen M, Zuppan C, Aitken D, Mierau G . Hepatic angiomyolipoma with striated granules and positivity with melanoma--specific antibody (HMB-45): a report of two cases. Ultrastruct Pathol. 1991; 15(4-5):563-71. DOI: 10.3109/01913129109016264. View

PRICE Jr E, Mostofi F . SYMPTOMATIC ANGIOMYOLIPOMA OF THE KIDNEY. Cancer. 1965; 18:761-74. DOI: 10.1002/1097-0142(196506)18:6<761::aid-cncr2820180614>3.0.co;2-a. View

Martignoni G, Pea M, Bonetti F, Zamboni G, Carbonara C, Longa L . Carcinomalike monotypic epithelioid angiomyolipoma in patients without evidence of tuberous sclerosis: a clinicopathologic and genetic study. Am J Surg Pathol. 1998; 22(6):663-72. DOI: 10.1097/00000478-199806000-00003. View

Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F . PEComas: the past, the present and the future. Virchows Arch. 2007; 452(2):119-32. PMC: 2234444. DOI: 10.1007/s00428-007-0509-1. View

Carbonara C, Longa L, Grosso E, Mazzucco G, BORRONE C, Garre M . Apparent preferential loss of heterozygosity at TSC2 over TSC1 chromosomal region in tuberous sclerosis hamartomas. Genes Chromosomes Cancer. 1996; 15(1):18-25. DOI: 10.1002/(SICI)1098-2264(199601)15:1<18::AID-GCC3>3.0.CO;2-7. View

Gaffey M, Mills S, Zarbo R, Weiss L, Gown A . Clear cell tumor of the lung. Immunohistochemical and ultrastructural evidence of melanogenesis. Am J Surg Pathol. 1991; 15(7):644-53. DOI: 10.1097/00000478-199107000-00005. View

Morgan G, STRAUMFJORD J, Hall E . Angiomyolipoma of the kidney. J Urol. 1951; 65(4):525-7. DOI: 10.1016/S0022-5347(17)68515-X. View

Martignoni G, Pea M, Zampini C, Brunelli M, Segala D, Zamboni G . PEComas of the kidney and of the genitourinary tract. Semin Diagn Pathol. 2015; 32(2):140-59. DOI: 10.1053/j.semdp.2015.02.006. View

Calio A, Brunelli M, Gobbo S, Pedron S, Segala D, Argani P . Stimulator of interferon genes (STING) immunohistochemical expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney. Pathology. 2021; 53(5):579-585. DOI: 10.1016/j.pathol.2020.09.025. View

10.

Pea M, Bonetti F, Martignoni G, Henske E, Manfrin E, Colato C . Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: the identification of malignant epithelioid angiomyolipoma. Am J Surg Pathol. 1998; 22(2):180-7. DOI: 10.1097/00000478-199802000-00005. View

11.

Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E . Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996; 20(6):722-30. DOI: 10.1097/00000478-199606000-00010. View

12.

Calio A, Brunelli M, Segala D, Zamboni G, Bonetti F, Pea M . Angiomyolipoma of the kidney: from simple hamartoma to complex tumour. Pathology. 2020; 53(1):129-140. DOI: 10.1016/j.pathol.2020.08.008. View

13.

Argani P, Zhong M, Reuter V, Fallon J, Epstein J, Netto G . TFE3-Fusion Variant Analysis Defines Specific Clinicopathologic Associations Among Xp11 Translocation Cancers. Am J Surg Pathol. 2016; 40(6):723-37. PMC: 4864095. DOI: 10.1097/PAS.0000000000000631. View

14.

Farrow G, HARRISON Jr E, Utz D, Jones D . Renal angiomyolipoma. A clinicopathologic study of 32 cases. Cancer. 1968; 22(3):564-70. DOI: 10.1002/1097-0142(196809)22:3<564::aid-cncr2820220311>3.0.co;2-p. View

15.

Calio A, Brunelli M, Gobbo S, Argani P, Munari E, Netto G . Cathepsin K: A Novel Diagnostic and Predictive Biomarker for Renal Tumors. Cancers (Basel). 2021; 13(10). PMC: 8157838. DOI: 10.3390/cancers13102441. View

16.

Nese N, Martignoni G, Fletcher C, Gupta R, Pan C, Kim H . Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011; 35(2):161-76. DOI: 10.1097/PAS.0b013e318206f2a9. View

17.

HOLM-NIELSEN P, Sorensen F . Renal angiomyolipoma: an ultrastructural investigation of three cases with histogenetic considerations. APMIS Suppl. 1988; 4:37-47. View

18.

Perez-Atayde A, Iwaya S, Lack E . Angiomyolipomas and polycystic renal disease in tuberous sclerosis. Ultrastructural observations. Urology. 1981; 17(6):607-10. DOI: 10.1016/0090-4295(81)90089-3. View

19.

Calio A, Brunelli M, Segala D, Pedron S, Tardanico R, Remo A . t(6;11) renal cell carcinoma: a study of seven cases including two with aggressive behavior, and utility of CD68 (PG-M1) in the differential diagnosis with pure epithelioid PEComa/epithelioid angiomyolipoma. Mod Pathol. 2017; 31(3):474-487. DOI: 10.1038/modpathol.2017.144. View

20.

Martignoni G, Bonetti F, Chilosi M, Brunelli M, Segala D, Amin M . Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney. Mod Pathol. 2011; 25(1):100-11. DOI: 10.1038/modpathol.2011.136. View