Everolimus for the treatment of epithelioid hemangioma: a case report
Epithelioid hemangioma is a rare benign vascular tumor manifested as slow-growing subcutaneous or dermal nodules. A previously therapeutic strategy involved surgical excision and sclerotherapy. However, no standard treatment has been established. Here, we reported an atypical case of epithelioid hemangioma that had a locally aggressive behavior and was successfully treated with everolimus, a rapamycin analog used for cancer therapy. A 1-year-old boy presented with an ulcerated lump in the left palmar region. The imaging test results, confirmed through histopathologic examination, suggested a diagnosis of epithelioid hemangioma. Targeted therapy with everolimus was administered orally because of a minimal response to initial transarterial sclerotherapy. The patient achieved a satisfactory response with a significantly reduced lesion size and improved hand function after a 2-year follow-up. This finding showed that patients with locally aggressive forms of epithelioid hemangioma may significantly benefit from everolimus as a potential targeted therapy.
Enjolras O, Mulliken JB, Kozakewich HP. Vascular tumors and tumor-like lesions. In: Mulliken JB, Burrows PE, Fishman SJ, editors. Vascular anomalies: Hemangiomas and malformations. 2nd ed. New York: Oxford University Press; 2013. p. 259-324. https://doi.org/10.1093/med/9780195145052.003.0008
Guo R, Gavino AC. Angiolymphoid hyperplasia with eosinophilia. Arch Pathol Lab Med. 2015;139(5):683-6. https://doi.org/10.5858/arpa.2013-0334-RS
Lagha IB, Souissi A. Angiolymphoid hyperplasia with eosinophilia. Treasure Island: StatPearls Publishing; 2020.
Sánchez-Fructuoso AI. Everolimus: an update on the mechanism of action, pharmacokinetics and recent clinical trials. Expert Opin Drug Metab Toxicol. 2008;4(6):807-19. https://doi.org/10.1517/17425255.4.6.807
Freixo C, Ferreira V, Martins J, Almeida R, Caldeira D, Rosa M, et al. Efficacy and safety of sirolimus in the treatment of vascular anomalies: a systematic review. J Vasc Surg. 2020;71(1):318-27. https://doi.org/10.1016/j.jvs.2019.06.217
Thabusum DA, Rajesh N, Reddy RS, Ramesh T, Ravikanth M. Epithelioid haemangioma - a diagnostic dilemma. Br J Med Med Res. 2015;6(10):1032-9. https://doi.org/10.9734/BJMMR/2015/15144
Zhang SL, Liang L, Ji Y, Wang ZM, Zhou YH. The benefit of everolimus in recurrent/epithelioid angiosarcoma patients: case reports and literature review. Oncotarget. 2017;8(55):95023-9. https://doi.org/10.18632/oncotarget.21832
Zheng JW, Zhou Q, Yang XJ, Wang YA, Fan XD, Zhou GY, et al. Treatment guideline for hemangiomas and vascular malformations of the head and neck. Head Neck. 2010;32(8):1088-98. https://doi.org/10.1002/hed.21274
Santini-Araujo E, Kalil RK, Bertoni F, Park YK, editors. Tumors and tumor-like lesions of bone for surgical pathologists, orthopedic surgeons and radiologists. London: Springer; 2015. p. 479-85. https://doi.org/10.1007/978-1-4471-6578-1
Hassan Y, Osman AK, Altyeb A. Noninvasive management of hemangioma and vascular malformation using intralesional bleomycin injection. Ann Plast Surg. 2013;70(1):70-3. https://doi.org/10.1097/SAP.0b013e31824e298d
Akdeniz N, Kösem M, Çalka O, Bilgili SG, Metin A, Gelincik I. Intralesional bleomycin for angiolymphoid hyperplasia. Arch Dermatol. 2007;143(7):841-4. https://doi.org/10.1001/archderm.143.7.841
MacKeigan JP, Krueger DA. Differentiating the mTOR inhibitors everolimus and sirolimus in the treatment of tuberous sclerosis complex. Neuro Oncol. 2015;17(12):1550-9. https://doi.org/10.1093/neuonc/nov152
Ozeki M, Nozawa A, Kanda K, Hori T, Nagano A, Shimada A, et al. Everolimus for treatment of pseudomyogenic hemangioendothelioma. J Pediatr Hematol Oncol. 2017;39(6):e328-31. https://doi.org/10.1097/MPH.0000000000000778
Uno T, Ito S, Nakazawa A, Miyazaki O, Mori T, Terashima K. Successful treatment of kaposiform hemangioendothelioma with everolimus. Pediatr Blood Cancer. 2015;62(3):536-8. https://doi.org/10.1002/pbc.25241
Franz DN, Belousova E, Sparagana S, Bebin EM, Frost M, Kuperman R, et al. Everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis complex: 2-year open-label extension of the randomised EXIST-1 study. Lancet Oncol. 2014;15(13):1513-20. https://doi.org/10.1016/S1470-2045(14)70489-9
Eisenhauer EA, Therasse P, Bogaerts J, Schwartz LH, Sargent D, Ford R, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009;45(2):228-47. https://doi.org/10.1016/j.ejca.2008.10.026
Bissler JJ, Franz DN, Frost MD, Belousova E, Bebin EM, Sparagana S, et al. The effect of everolimus on renal angiomyolipoma in pediatric patients with tuberous sclerosis being treated for subependymal giant cell astrocytoma. Pediatr Nephrol. 2018;33(1):101-9. https://doi.org/10.1007/s00467-017-3806-1
Copyright (c) 2021 Mururul Aisyi, Ayu Hutami Syarif, Dewi Aisyah Mukarramah, Rio Hermawan, Dewi Iriani
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with Medical Journal of Indonesia agree to the following terms:
- Authors retain copyright and grant Medical Journal of Indonesia right of first publication with the work simultaneously licensed under a Creative Commons Attribution-NonCommercial License that allows others to remix, adapt, build upon the work non-commercially with an acknowledgment of the work’s authorship and initial publication in Medical Journal of Indonesia.
- Authors are permitted to copy and redistribute the journal's published version of the work non-commercially (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in Medical Journal of Indonesia.