SKIN - July 2021 - 1227 proof returned SKIN July 2021 Volume 5 Issue 4 Copyright 2021 The National Society for Cutaneous Medicine 422 BRIEF ARTICLE Angiosarcoma Clinically Mimicking a Targetoid Hemosiderotic Hemangioma Hannah Nam, BA1, Jordan Lim, MB BCh BAO1, Alexandra Flamm, MD1 1Penn State Health, Hershey Medical Center, Department of Dermatology, Hershey, PA Angiosarcoma is a rare, aggressive soft- tissue sarcoma of endothelial cell origin with increased likelihood for local recurrence, metastasis, and poor prognosis. Angiosarcomas can affect any organ in the body; however, nearly 50% of all cases are of its cutaneous form often of the head and neck1. The five year survival of cutaneous angiosarcoma ranges from 10-30%2. The variation in clinical presentation and the lack of disease awareness contribute to the difficulty in diagnosis3; however, early detection and intervention is critical for improved prognosis. The size of lesion at presentation is an important prognostic factor for cutaneous angiosarcoma3, and a delayed diagnosis may lead to substantial challenges in treatment4. Perez et al. reported that a tumor size less than 5cm was associated with a significantly improved overall and recurrence-free survival compared to a tumor size greater than 5cm5. Despite early detection, the neoplasm can often be more advanced than is apparent from initial physical exam2. This case report emphasizes the value of early clinical suspicion for atypical, rapidly changing vascular lesions. A 69-year-old white man with no history of skin cancer presented with a 2cm vascular targetoid lesion with a central 5mm firm dermal purple nodule and surrounding ecchymosis on his right flank (Figure 1). The patient noticed the lesion approximately three months prior which then progressively grew. No palpable axillary or inguinal lymph nodes were noted. Review of systems was ABSTRACT Angiosarcoma is a rare malignant entity that typically presents as a rapidly progressing ecchymotic or cellulitis-like lesion with subsequent bleeding or ulceration. Atypical presentations have been described, such as cases mimicking rosacea, making the diagnosis more challenging. Due to the high rate of local recurrence, metastasis, and disease-specific mortality, early identification and treatment is critical. Here we describe a 69-year-old man with a vascular targetoid lesion clinically consistent with a targetoid hemosiderotic hemangioma (THH); however, biopsy was consistent with an angiosarcoma. Early diagnosis of this lesion resulted in prompt wide local excision and no detection of metastasis. This case report highlights the importance of thorough evaluation of new onset vascular lesions and the unique THH-like presentation of an angiosarcoma. INTRODUCTION CASE REPORT SKIN July 2021 Volume 5 Issue 4 Copyright 2021 The National Society for Cutaneous Medicine 423 Figure 1. 2cm targetoid vascular lesion on right flank unremarkable. A 5mm punch biopsy of the lesion was performed. Histopathologic evaluation revealed vascular proliferation within the superficial to mid dermis composed of enlarged and pleomorphic endothelial cells. Many of the endothelial cells formed small irregular ectatic vascular units, with crack-like spaces. Scattered mitotic figures along with single scattered enlarged endothelial cells with background hemorrhage were observed (Figure 2). The endothelial cells were highlighted on CD31 immunostaining and negative with SOX-10, Melan-A, and HHV-8 immunostaining (Figure 3). Mib-1 immunostaining revealed an increased proliferation index. This was consistent with a diagnosis of an angiosarcoma. A full body PET scan demonstrated no areas of concern for metastasis. A wide local excision with 2.5cm margins followed by reconstruction with a posteriorly-based advancement flap was completed. The tumor was completely excised with negative margins. This case was discussed at a multidisciplinary tumor board where surgical treatment alone was determined to be sufficient for this localized, small lesion. Monitoring will consist of PET scans every six months for two years Cutaneous angiosarcoma is an aggressive malignant lesion frequently presenting as a raised purplish-red papule on the head and neck. Early presentations can often be dangerously mistaken as a benign vascular lesion, leading to delayed diagnosis, delayed treatment, and worse prognosis6. Histological examination of angiosarcoma include irregular vascular channels and atypical endothelial cells that may appear normal, pleomorphic, and hyperchromatic7. Immunohistochemistry may be positive for CD34 and CD317.The local recurrence rates of cutaneous angiosarcoma after surgical excision have been reported to range from 35-86%8. A retrospective study conducted at the Mayo Clinic reported a 5-year-recurrence free survival of 16%, a 5-year local regional control of 18%, and a 5-year overall survival of 38% among patients treated for cutaneous angiosarcoma 9. Another study conducted among patients diagnosed with angiosarcoma of the face and scalp reported an even higher recurrence rate of 93.8%8. Imaging including MRI, CT, and PET scan is crucial for staging and management of angiosarcoma. Due to its rare incidence, the optimal treatment is not currently established9. Wide local excision with negative margins is the gold standard; however, currently, there are no specific margins recommended by the NCCN guidelines. The multifocal nature of the lesion and propensity for tissue infiltration often result in positive resection margins, recurrence, and metastasis of the tumor10 DISCUSSION SKIN July 2021 Volume 5 Issue 4 Copyright 2021 The National Society for Cutaneous Medicine 424 Figure 2. 5mm bunch biopsy of right flank. H&E stain A) 4x showing an atypical vascular proliferation and background hemorrhage within the dermis B) 40x highlighting the atypical endothelial cells demonstrating mitotic figures, arranged singly and forming small irregular vessels Figure 3. CD31 Staining highlighting the atypical endothelial cells and vessels (10x) and an additional peripheral ecchymotic ring. THH lesions typically occur on the proximal extremities and trunk. Several studies have reported a cyclical variation in palpability, In SKIN July 2021 Volume 5 Issue 4 Copyright 2021 The National Society for Cutaneous Medicine 425 In contrast, targetoid hemosiderotic hemangioma (THH), also known as hobnail hemangioma, is a benign vascular lesion that often presents as a solitary annular violaceous papule surrounded by a pale ring size, and color of the lesions, which contributes to the challenge in diagnosis11. Histological findings of THH include non- circumscribed dermal vascular proliferations that are usually found within the superficial and mid dermal layers of the skin11. The vascular channels are lined by a single layer of “hobnail-like” endothelial cells. Excision of a THH lesion is mostly curative, and malignant transformation, recurrence or systemic metastases have not been reported12. In this case we demonstrate a unique clinical presentation of angiosarcoma in which a targetoid vascular lesion was identified, a finding more commonly associated with THH. The wide variety of clinical presentations of THH and angiosarcoma can make initial diagnosis challenging; however, early clinical suspicion is critical due to their vastly different prognoses. Conflict of Interest Disclosures: None Funding: None Corresponding Author: Alexandra Flamm, MD Assistant Professor of Dermatology & Pathology Department of Dermatology Penn State Hershey Medical Center 500 University Drive Hershey, PA 17033 Phone: 717-531-8307 Fax: 717-531-4702 Email: aflamm@pennstatehealth.psu.edu References: 1.Gaballah, A. H. et al. Angiosarcoma: clinical and imaging features from head to toe. Br. J. Radiol. 90, 20170039 (2017). 2.Mendenhall, W. M., Mendenhall, C. M., Werning, J. W., Reith, J. D. & Mendenhall, N. P. Cutaneous angiosarcoma. Am. J. Clin. Oncol. 29, 524–528 (2006). 3.Holden, C. A., Spittle, M. F. & Jones, E. W. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 59, 1046– 1057 (1987). 4.Vora, R., Anjaneyan, G. & Gupta, R. Cutaneous angiosarcoma of head and neck. Indian J. Dermatol. 59, 632 (2014). 5.Perez, M. C. et al. Cutaneous angiosarcoma: a single-institution experience. Ann. Surg. Oncol. 20, 3391– 3397 (2013). 6.Dossett, L. A., Harrington, M., Cruse, C. W. & Gonzalez, R. J. Cutaneous angiosarcoma. Curr. Probl. Cancer 39, 258–263 (2015). 7.Selim, A., Khachemoune, A. & Lockshin, N. A. Angiosarcoma: a case report and review of the literature. CUTIS-NEW YORK- 76, 313 (2005). 8.Ogawa, K. et al. Treatment and prognosis of angiosarcoma of the scalp and face: a retrospective analysis of 48 patients. Br. J. Radiol. 85, e1127–33 (2012). 9.Patel, S. H. et al. Angiosarcoma of the scalp and face: the Mayo Clinic experience. JAMA Otolaryngol. Head Neck Surg. 141, 335–340 (2015). 10.Pawlik, T. M. et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer 98, 1716–1726 (2003). 11.Bingham, L. G. & Davis, D. M. Clinicopathologic challenge. International Journal of Dermatology vol. 47 991–992 (2008). 12.Kakizaki, P., Valente, N. Y. S., Paiva, D. L. M., Dantas, F. L. T. & Gonçalves, S. V. C. B. Targetoid hemosiderotic hemangioma - Case report. An. Bras. Dermatol. 89, 956–959 (2014). CONCLUSION