Interactive Case Study - 10- October 2022

‎28-year-old male with a painful pink papule

Mavra Masood BA

‎Abraham Abdulhak MD

Julia R. Nunley MD

An otherwise healthy 28-year-old male presented with a 1-year history of a painful lesion on his left abdomen. He had been applying a topical benzoyl peroxide-clindamycin gel to the area for 6 months without improvement. Visual inspection revealed a 4mm well-defined, pink papule (Figure 1). Palpation further revealed a tender indurated plaque as well as 2 additional tender papules that were not visible on exam. An 8mm punch biopsy was performed on the visible papule; the histopathology is demonstrated in Figures 2-5.

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Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

Figure 6

Figure 7

What is the best diagnosis?

Cellular benign fibrous histiocytoma

Epithelioid hemangioendothelioma

Epithelioid sarcoma

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma

Spindle cell squamous cell carcinoma

Which immunohistochemical profile best defines the entity demonstrated in this ‎case?‎

FOSB+, CD34 -, ERG+, INI-1 retained

FOSB-, CD34 +, ERG -, INI-1 lost

FOSB-, CD34+, ERG +, INI-1 retained ‎

FOSB+, CD34 +, ERG +, INI-1 retained

FOSB-, CD34+, ERG+, CD31+

Which of the following agents is most commonly used as adjuvant therapy, ‎following surgery, for pseudomyogenic hemangioendothelioma (PMHE)?‎

Doxorubicin‎

Everolimus‎

Paclitaxel‎

Tazemetostat

Vincristine

Wei J, Liao Z, Zhao G, et al. Clinicopathological features of pseudomyogenic hemangioendothelioma and precision therapy based on whole exome sequencing. Cancer Commun. 2020;40(4):197-201. doi:10.1002/cac2.12020
Hornick JL, Fletcher CDM. Pseudomyogenic hemangioendothelioma: A distinctive, often multicentric tumor with indolent behavior. Am J Surg Pathol. 2011;35(2):190-201. doi:10.1097/PAS.0b013e3181ff0901
Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003;27(1):48-57. doi:10.1097/00000478-200301000-00006
Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma (pseudomyogenic hemangioendothelioma). Am J Surg Pathol. 2011;35(7):1088. doi:10.1097/PAS.0b013e31821caf1c
VanJzendoorn DGP, Sleijfer S, Gelderblom H, et al. Telatinib is an effective targeted therapy for pseudomyogenic hemangioendothelioma. Clin Cancer Res. 2018;24(11):2678-2687. doi:10.1158/1078-0432.CCR-17-3512
Requena L, Santonja C, Martinez-Amo JL, Saus C, Kutzner H. Cutaneous epithelioid sarcomalike (pseudomyogenic) hemangioendothelioma: A little-known low-grade cutaneous vascular neoplasm. JAMA Dermatology. 2013;149(4):459-465. doi:10.1001/jamadermatol.2013.3190
Doyle LA, Fletcher CDM, Hornick JL. Nuclear expression of CAMTA1 distinguishes epithelioid hemangioendothelioma from histologic mimics. Am J Surg Pathol. 2016;40(1):94-102. doi:10.1097/PAS.0000000000000511
Caballero GA, Roitman PD. Pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma). Arch Pathol Lab Med. 2020;144(4):529-5333. doi:10.5858/arpa.2018-0395-RS
Wernheden E, Trøstrup H, Pilt AP. Unusual Presentation of Cutaneous Spindle Cell Squamous Cell Carcinoma: A Case Report. Case Rep Dermatol. 2020;12(1):70. doi:10.1159/000507358
Ortins-Pina A, Llamas-Velasco M, Turpin S, Soares-de-Almeida L, Filipe P, Kutzner H. FOSB immunoreactivity in endothelia of epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia). J Cutan Pathol. 2018;45(6):395-402. doi:10.1111/cup.13141
Ramkumar S. Epithelioid Haemangioma of Bone: A Case Series and Comprehensive Literature Review Reappraising the Diagnostic Classification of All Epithelioid Vascular Neoplasms of Bone. Cureus. June 2021. doi:10.7759/cureus.15371
Yan M, Gilmore H, Bomeisl P, Harbhanjanka A. Clinicopathologic and immunohistochemical study of breast angiosarcoma. Ann Diagn Pathol. 2021;54:151795. doi:10.1016/J.ANNDIAGPATH.2021.151795
Ozeki M, Nozawa A, Kanda K, et al. Everolimus for Treatment of Pseudomyogenic Hemangioendothelioma. J Pediatr Hematol Oncol. 2017;39(6):e328-e331. doi:10.1097/MPH.0000000000000778
Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K. Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma. Pediatr Blood Cancer. 2018;65(2). doi:10.1002/pbc.26781
Cao J, Wang J, He C, Fang M. Angiosarcoma: a review of diagnosis and current treatment. Am J Cancer Res. 2019;9(11):2303. /pmc/articles/PMC6895451/. Accessed August 8, 2021.

Acknowledgements

Author Profiles

Mavra Masood

Virginia Commonwealth University School of Medicine

Mavra is a 4th year medical student at Virginia Commonwealth University School of Medicine. She earned her bachelor’s degree in global public health at the University of Virginia. She is currently applying to dermatology residency programs and is interested in public health and global dermatology.

‎Abraham Abdulhak

Department of Dermatology, Virginia Commonwealth University Health System

Abe is a chief dermatology resident at VCU Health in Richmond, VA. He earned his ‎bachelor’s degree and medical doctorate from Wayne State University in Detroit, ‎Michigan. His clinical interests include general dermatology, cutaneous oncology, Mohs ‎micrographic and reconstructive surgery. He is currently applying for a fellowship in ‎Mohs Micrographic Surgery.‎

Julia R. Nunley

Department of Dermatology, Virginia Commonwealth University Health System

Dr. Nunley is a nationally renowned complex medical dermatologist. After completing ‎medical school at Case Western Reserve University, she completed residency in internal ‎medicine, fellowship in nephrology, and a second residency in dermatology all at the ‎VCU Health System. She has authored numerous peer-reviewed scientific articles and ‎book chapters. ‎

Question:

Interactive Case Study - 10- October 2022

Acknowledgements

Author Profiles

‎28-year-old male with a painful pink papule

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