The Impact of Adjuvant Sirolimus Therapy in the Surgical Management of Scrotal Slow-Flow Vascular Malformations

Published:September 10, 2020DOI:



      To describe the effects of oral sirolimus administered before and after surgical resection of slow-flow vascular malformations of the scrotum in pediatric patients.


      Retrospective review of 3 patients presenting with complex lymphatic-venous malformations of the scrotum who received adjuvant oral sirolimus 3 months before and 3 months after surgical resection. Demographic data, clinical course, imaging findings, and management strategies were reviewed for each patient.


      In each of the 3 patients, there was a significant volume reduction of the lesion within the 3 months after initial dose of sirolimus. Scarce lymphatic leakage during and after surgery was reported, associated with an adequate wound healing. Two years after the last postsurgical dose of sirolimus, all patients remain asymptomatic without any lymphatic leakage or lesion recurrence.


      Combined lymphatic-venous vascular malformations of the male genitalia are rare but associated with high morbidity and challenging treatment options. Pre- and postsurgical adjuvant treatment with oral sirolimus seems to be a promising therapeutic option that provides reduction of the lesion size before surgery and improvement of postsurgical recovery and wound healing.
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        • Greene AK
        Current concepts of vascular anomalies.
        J Craniofac Surg. 2012; 23: 220-224
        • White CL
        • Olivieri B
        • Restrepo R
        • McKeon B
        • Karakas SP
        • Lee EY
        Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation—part 1, lymphatic malformation mimickers.
        AJR Am J Roentgenol. 2016; 206: 940-951
        • Foley LS
        • Kulungowski AM
        Vascular anomalies in pediatrics.
        Adv Pediatr. 2015; 62: 227-255
        • Kulungowski AM
        • Schook CC
        • Alomari AI
        • Vogel AM
        • Mulliken JB
        • Fishman SJ
        Vascular anomalies of the male genitalia.
        J Ped Surg. 2011; 46: 1214-1221
        • Akaike G1
        • Nozaki T
        • Makidono A
        • Saida Y
        • Hirabayashi T
        • Suzuki K
        A case of lymphatic malformation/lymphangioma of the scrotum.
        Acta Radiol Short Rep. 2012; 1 (231-3)
        • Adams DM
        • Trenor 3rd, CC
        • Hammill AM
        • et al.
        Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies.
        Pediatrics. 2016; 137 (e20153257): 3257
        • Romics M
        • Tasnadi G
        • Sulya B
        • Kiss A
        • Merksz M
        • Nyirady P
        Congenital lymphovascular malformations with urological symptoms: a report of two cases and review of the literature.
        Int Urol Nephrol. 2016; 48: 1771-1775
        • Johnson AB
        • Richter GT
        Surgical considerations in vascular malformations.
        Tech Vasc Interv Radiol. 2019; 22100635
        • Alomari A
        • Dubois J
        Interventional management of vascular malformations.
        Tech Vasc Interv Radiol. 2011; 14: 22-31
        • Hammill AM
        • Wentzel M
        • Gupta A
        • et al.
        Sirolimus for the treatment of complicated vascular anomalies in children.
        Pediatr Blood Cancer. 2011; 57: 1018-1024
        • Ozeki M
        • Asada R
        • Saito AM
        • et al.
        Efficacy and safety of sirolimus treatment for intractable lymphatic anomalies: a study protocol for an open-label, single-arm, multicenter, prospective study (SILA).
        Regen Ther. 2019; 10: 84-91
        • Maruani A
        • Boccara O
        • Bessis D
        • et al.
        Treatment of voluminous and complicated superficial slow-flow vascular malformations with sirolimus (PERFORMUS): protocol for a multicenter phase 2 trial with a randomized observational-phase design.
        Trials. 2018; 19: 340
        • Strychowsky JE
        • Rahbar R
        • O'Hare MJ
        • Irace AL
        • Padua H
        • Trenor CC
        Sirolimus as treatment for 19 patients with refractory cervicofacial lymphatic malformation.
        Laryngoscope. 2018; 128: 269-276
        • Adams DM
        • Ricci KW
        Vascular anomalies: diagnosis of complicated anomalies and new medical treatment options.
        Hematol Oncol Clin North Am. 2019; 33: 455-470
        • Ozeki M
        • Nozawa A
        • Yasue S
        • et al.
        The impact of Sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies.
        Orphanet J Rare Dis. 2019; 14: 141
        • Curry S
        • Logeman A
        • Jones D
        Sirolimus: a successful medical treatment for head and neck lymphatic malformations.
        Case Rep Otolaryngol. 2019; 2076798: 1-3
        • Zakliczynski M
        • Nozynski J
        • Kocher A
        • et al.
        Surgical wound-healing complications in heart transplant recipients treated with rapamycin.
        Wound Repair Regen. 2007; 15: 316-321