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Interactive Case Study - June 2026

40-year-old female with recurrent papule
Ebonee S. Lunceford, BS, BSN, RN 
Miki Lindsey, MD 
David Ullman, MD 
 
 
A 40-year-old female presents with a red papule on her right lateral abdomen. The lesion was previously biopsied at an outside institution and diagnosed as a hemangioma. The lesion has since recurred and the patient presents for an excision.
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What is the best diagnosis?
Epithelioid hemangioendothelioma
Kaposi sarcoma
Kaposiform hemangioendothelioma
Postradiation angiosarcoma
Retiform hemangioendothelioma
What molecular abnormalities are associated with this entity?
Activating mutations in GNAQ and GNA11
C-myc amplification
There are no known molecular abnormalities associated with this neoplasm
WWTR1-CAMTA1 gene fusion
YAP1 rearrangements
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  2. Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica. Siapec Servizi Srl; 2021 Apr;113(2):70–84. PMCID: PMC8167394

  3. Ji Y, Chen S, Yang K, Xia C, Li L. Kaposiform hemangioendothelioma: current knowledge and future perspectives. Orphanet J Rare Dis. Springer Science and Business Media LLC; 2020 Feb 3;15(1):39. PMCID: PMC6998257

  4. Liu Q, Jiang L, Wu D, Kan Y, Fu F, Zhang D, Gong Y, Wang Y, Dong C, Kong L. Clinicopathological features of Kaposiform hemangioendothelioma. Int J Clin Exp Pathol. 2015 Oct 1;8(10):13711–13718. PMCID: PMC4680544

  5. Hung J, Hiniker SM, Lucas DR, Griffith KA, McHugh JB, Meirovitz A, Thomas DG, Chugh R, Herman JM. Sporadic versus radiation-associated angiosarcoma: A comparative clinicopathologic and molecular analysis of 48 cases. Sarcoma. Hindawi Limited; 2013 Sep 3;2013:798403. PMCID: PMC3776386

  6. Shah S, Rosa M. Radiation-associated angiosarcoma of the breast: Clinical and pathologic features. Arch Pathol Lab Med. Archives of Pathology and Laboratory Medicine; 2016 May;140(5):477–481. PMID: 27128306

  7. Snyder ML, Lyle S, Dinh T, Stead J, Kannler C. Primary cutaneous epithelioid hemangioendothelioma with lymph node metastasis. JAAD Case Rep. Elsevier BV; 2020 Nov;6(11):1125–1128. PMCID: PMC7591333

  8. Tsuchihashi K, Baba E. Epithelioid hemangioendothelioma-its history, clinical features, molecular biology and current therapy. Jpn J Clin Oncol. Oxford University Press (OUP); 2024 Jul 7;54(7):739–747. PMID: 38555494

  9. Bell BM Jr, Syed A, Carmack SW, Thomas CA, Layton KF. Disseminated Kaposi sarcoma with osseous metastases in an HIV-positive patient. Proc (Bayl Univ Med Cent). Informa UK Limited; 2016 Jan;29(1):52–54. PMCID: PMC4677855

  10. Batash R, Crimí A, Kassem R, Asali M, Ostfeld I, Biz C, Ruggieri P, Schaffer M. Classic Kaposi sarcoma: Diagnostics, treatment modalities, and genetic implications - A review of the literature. Acta Oncol. MJS Publishing, Medical Journals Sweden AB; 2024 Oct 16;63:783–790. PMCID: PMC11495121

  11. Antonescu CR, Dickson BC, Sung YS, Zhang L, Suurmeijer AJH, Stenzinger A, Mechtersheimer G, Fletcher CDM. Recurrent YAP1 and MAML2 gene rearrangements in retiform and composite hemangioendothelioma. Am J Surg Pathol. Ovid Technologies (Wolters Kluwer Health); 2020 Dec;44(12):1677–1684. PMCID: PMC7773139

  12. Hogeboom-Gimeno AG, van Ravensteijn SG, Desar IME, Hillebrandt-Roeffen MHS, van Cleef PHJ, Bonenkamp JJ, Flucke U, Versleijen-Jonkers YMH. MYC amplification in angiosarcoma depends on etiological/clinical subgroups - Diagnostic and prognostic value. Ann Diagn Pathol. Elsevier BV; 2023 Apr;63(152096):152096. PMID: 36610315

  13. Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med. American Association for the Advancement of Science (AAAS); 2011 Aug 31;3(98):98ra82. PMID: 21885404

  14. Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HPW, Bischoff J, Greene AK, Warman ML. Somatic activating mutations in GNAQ and GNA11 are associated with congenital hemangioma. Am J Hum Genet. 2016 Apr 7;98(4):789–795. PMCID: PMC4833432

 

Acknowledgements

 

Author Profiles

Ebonee S. Lunceford, BS, BSN, RN

The Dermatology Group

Ebonee Lunceford graduated with a B.S. in biochemistry and molecular biology from the University of South
Carolina and a B.S.N. in nursing from Northeastern University. She is currently completing a Masters of Art in Biomedical sciences and plans on attending medical school in the future.

 

Miki Lindsey, MD

Columbia Skin Clinic

Dr. Miki Lindsey is dermatopathologist with Columbia Skin Clinic dermatology practice in South Carolina. She completed her medical school, Anatomic and Clinical Pathology residency, and Dermatopathology fellowship at the University of Arkansas for Medical Sciences.

 

David Ullman, MD

The Dermatology Group

Dr. Ullman is a board-certified dermatopathologist. He completed both his pathology residency and dermatopathology fellowship at the University of Alabama at Birmingham. He is currently in private practice in Columbia, SC.

 

 

 

 

 

 

 

Question:

 

 

 

 
 

40-year-old female with recurrent papule

Category

Tumors > Vascular

Description

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