==== Front Int J Surg Case RepInt J Surg Case RepInternational Journal of Surgery Case Reports2210-2612Elsevier S2210-2612(18)30221-910.1016/j.ijscr.2018.06.019ArticleTesticular cavernous hemangioma associated with testicular torsion – case report and review of literature Tepeneu N.F. nftepeneu@yahoo.comab⁎Krafka K. karel.krafka@kabeg.ataMeglic S. spela.meglic@kabeg.ataRogatsch H. hermann.rogatsch@kabeg.atcFasching G. guenter.fasching@kabeg.ataa Department of Pediatric and Adolescent Surgery, General Hospital, Klagenfurt, Austriab University of Medicine and Pharmacy “Victor Babes’’, Timisoara, Romaniac Department of Pathology, General Hospital, Klagenfurt, Austria⁎ Corresponding author at: Department of Pediatric and Adolescent Surgery, General Hospital Klagenfurt, Feschnigstraße 11, 9020, Klagenfurt, Austria. nftepeneu@yahoo.com27 6 2018 2018 27 6 2018 49 247 250 13 4 2018 15 6 2018 21 6 2018 © 2018 The Authors2018This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Highlights • Testicular hemangioma is exceedingly rare and typically occurs in patients younger than 20 years. • Hemangiomas of the testis have a similar sonographic and magnetic resonance imaging appearance to that of malignant tumors of the testis, especially seminoma. • Clinicians and pathologists must be aware of the rare entity of testicular hemangiomas, as clinical examination and imaging studies do not often suffice to arrive at a correct diagnosis. • The association of testicular torsion with testicular hemangioma is a rarity in children. Introduction Testicular neoplasms that are derived from connective tissue, blood vessels and musculature are uncommon and intra-testicular tumors of vascular origin are extremely rare; both are benign in nature. Testicular hemangioma is exceedingly rare and typically occurs in patients younger than 20 years, the age in which a primary germ cell tumor of the testis may present, necessitating a radical approach to management with orchidectomy, although potential conservative focal partial surgical excision is desirable. Hemangiomas of the testis have a similar sonographic and magnetic resonance imaging appearance to that of malignant tumors of the testis, especially seminoma. The work has been reported in line with the SCARE criteria. Presentation of case We present a case of testicular torsion in a 15-year-old male patient who had a painful left testis for 6 days, no vomiting, no fever or dysuria. With clinical suspicion of an old testicular torsion the patient was examined by ultrasound which confirmed the clinical diagnosis. The patient underwent emergency surgical exploration. The left testis was found to be necrotic after a 360° testicular torsion and an orchiectomy was performed. Results The postoperative course was uneventful. The patient was discharged on day 4 after surgery. Histology showed a complete ischemic infarction of the testicular parenchyma as part of a ruptured intratesticular cavernous hemangioma. Discussion and conclusion Cavernous hemangioma is a rare tumor of the testicle in either childhood or adult period. The particularity of the presented case is the possible association of a cavernous intratesticular hemangioma with the torsion of the testis in a teenager. Clinicians and pathologists must be aware of the rare entity of testicular hemangiomas, as clinical examination and imaging studies do not often suffice to arrive at a correct diagnosis. Keywords Testicular torsionCavernous testicular hemangiomaTeenagerCase report ==== Body 1 Introduction Testicular neoplasms that are derived from connective tissue, blood vessels and musculature are uncommon and intra-testicular tumors of vascular origin are extremely rare; both are benign in nature. Testicular hemangioma is exceedingly rare and typically occurs in patients younger than 20 years, the age in which a primary germ cell tumor of the testis may present, necessitating a radical approach to management with orchidectomy, although potential conservative focal partial surgical excision is desirable. Hemangiomas of the testis have a similar sonographic and magnetic resonance imaging appearance with malignant tumors of the testis, especially seminoma. The International Society for the Study of Vascular Anomalies (ISSVA) divided vascular anomalies into vascular tumors and vascular malformations. Vascular tumors arise mainly due to endothelial hyperplasia and they may regress spontaneously. Vascular malformations occur by localized defects of vascular morphogenesis, secondary to dysfunction in pathways regulating embryogenesis and they usually persist in adulthood [1]. In 2000, Mazal et al. reviewed the literature and identified four histopathological types of testicular hemangiomas: cavernous, histiocytoid, capillary and papillary endothelial hyperplasia [2]. Cavernous hemangiomas are diagnosed by dilated large vessels with thin walls. Their walls may be abnormal which may not confirm to arterial or venous morphology. Developmental arrest during the stage of an undifferentiated capillary network has been postulated to result in a cavernous hemangioma. This work has been reported in line with the SCARE criteria [3]. 2 Presentation of case We present a case of testicular torsion in a 15-year-old male patient who had a painful left testis for 6 days, no vomiting, no fever or dysuria. With clinical suspicion of an old testicular torsion the patient was examined by ultrasound which confirmed the clinical diagnosis (Figs. 1 and 2).Fig. 1 The sonographic aspect of the left testicle without central perfusion and with an altered structure (GE Logiq E9 ultrasound equipment). Fig. 1Fig. 2 For comparison the sonographic aspect of the healthy right testicle with central perfusion and with a normal structure. Fig. 2 The patient underwent emergency surgical exploration. The left testis was found to be necrotic after a 360 degree testicular torsion (Fig. 3) and an orchiectomy was performed.Fig. 3 The macroscopic aspect of the removed testicle and spermatic cord. Fig. 3 The postoperative course was uneventful. The patient was discharged on day 4 after surgery. Histology showed a complete ischemic infarction of the testicular parenchyma as part of a ruptured intratesticular cavernous hemangioma (Fig. 4, Fig. 5).Fig. 4 Verhoeff–Van Gieson stain (20× magnification) – Histological aspect of the hemangioma. Fig. 4Fig. 5 Hematoxylin and eosin stain (20× magnification) – Histological aspect of the hemangioma. Fig. 5 3 Discussion Hemangiomas of the soft tissues are the most common benign tumors of vascular origin. In the pediatric population, 60% of the hemangiomas are located in the head and neck, 25% in the trunk and 15% in the extremities [4]. Testicular hemangiomas are extremely rare and they occur in diverse age groups. Four histological types of benign vascular tumors are reported: cavernous haemangiomas, capillary haemangiomas, hystiocytoid and papillary endothelial hyperplasia. Cavernous hemangioma of the testis primarily presents during childhood [5].Our case is a cavernous hemangioma of the testis. Intra-testicular form of hemangiomas is reported rarely, compared with extra-testicular genital hemangiomas. Unilateral testicular enlargement, with or without tenderness, is the main presenting symptom, which is similar to that of malignant testicular tumors on clinical presentation. Pain is a inconsistent feature [1,2,[4], [5], [6], [7]]. Our patient presented with enlargement and pain in his left testis for 6 days. These could have been because of the intratesticular hemangioma, because of the torsion of the testis or both. Rarely, testicular hemangioma can present with bleeding or ulceration. Spontaneous involution is not reported with this type of hemangioma and long term complications like infertility, hemorrhage and even necrosis can occur. It can be seen extending into the penis, anterior abdominal wall, perineum and pelvis [8,9]. An association with hemangiomas in other sites such as skin, liver, rectum and bladder has been reported by other authors [10,11]. Genital hemangioma may mimic an inguinal hernia, while subcutaneous location on the scrotum can be confused with a varicocele when it presents on the left side. Infarction associated with torsion of testis is usually analyzed with frozen section and histopathological sections are helpful only in confirming the diagnosis. All the cases of testicular cavernous hemangioma reported in the literature have run a benign course and there were no mentions of metastasis and recurrence following surgical excision [12]. Sometimes the growth of the testicular hemangioma can be rapid and it may clinically simulate an aggressive neoplasm [13]. On ultrasonogram testicular hemangiomas are often hypoechoic but sometimes hyperechoic or even heterogenous [[14], [15], [16]]. When the tumor markers are negative, the presence of a mass with variably sized calcifications is highly suggestive of cavernous hemangioma. However, serum AFP, beta-hCG and LDH are raised only in half of the patients diagnosed with testicular malignancies. The differential diagnosis must include germ cell tumors (seminoma, teratoma), adenomatoid tumor, and sex-cord stromal tumors such as Sertoli cell tumor. The worldwide incidence of testicular cancer is estimated to have doubled in the last 40 years but significant variation exists between races and countries [13]. The management of testicular masses presenting in childhood is governed by the pubertal status of the patient. Prepubescent tumours are often benign, the commonest neoplasms being yolk sac tumours and teratomas. The proportion of benign tumours has been reported as 30–70% compared with only 5% in adults, and those that are malignant have a much lower incidence of metastasis. Hemangioma can be very similar to malignant testicular tumors on clinical presentation and also by sonography and magnetic resonance imaging. Clinical appearance and diagnostic exams are usually not sufficient for the definite diagnosis of testicular hemangioma and require a histopathological examination, altough Tallen et al. reported that when combined with clinical symptoms in the case of childhood testicular neoplasms sonography predicted 75% of histologies. After including hormone and tumor marker levels, a correct preoperative diagnosis was made for all boys with germ cell, and for 75% of boys with sexcord stromal tumor [17]. Demonstration of intra-lesional blood flow can be made by Color Doppler studies; however lack of flow does not indicate the absence of cavernous hemangiomas, especially in the situation where a testicular torsion is also present. Treatment options available for testicular hemangioma are surgical excision, laser fulguration, intralesional sclerotherapy and cryotherapy. If the surgeons and pathologists are aware of it, especially with negative tumor marker findings, tissue sparing surgical treatment by means of tumor enucleation with preservation of the testis is possible if intraoperative frozen section examination can be performed. Frozen section must be performed especially if the neoplasm has significant vascular proliferation identified by Doppler sonography. Because of the benign nature of this pathology, preservation of the testis is possible if the diagnosis can be performed during the operation by frozen sections. The principles of testicle-sparing surgery is well established for testicular tumours. If the diagnosis is not definitive, orchidectomy must be performed. Testis sparing surgery is commonly done in pre-pubescent tumors as the proportion of benign tumors is relatively higher than in adults [4,13]. Testicular hemangiomas can also present as testicular torsion or associated with testicular infarction [7]. To our knowledge there is no report in the English medical literature up to this date which associates a testicular cavernous hemangioma with a testicular torsion at the age of 15 years or below. There is a report of a young man 18 years of age with a cavernous hemangioma mimicking testicular torsion [18], and also a report of a man 22 years of age with a hemangioma and testicular torsion [7]. There is a high likelihood of spontaneous testicular torsion at the patients age. Intravaginal torsion most commonly occurs in adolescents. It is thought that the increased weight of the testicle after puberty, as well as sudden contraction of the cremasteric muscles (which inserts in a spiral fashion into the spermatic cord), is the impetus for acute torsion. Testicular torsion is favoured by enlargement of the testis associated with testicular malignancy, especially in adults. One study found a 64% association of testicular torsion with testicular malignancy [19]. This is thought to be secondary to a relative increase in the broadness of the affected testicle compared with its blood supply. However, in a review of 32 patients who had been diagnosed with testicular torsion, testicular cancer was found in 2 of the 20 patients who had undergone orchiectomy, a rate of 6.4% [20]. It is difficult to tell if the cavernous hemangioma of the testis, which was unknown before the beginning of the testicular pain, was the trigger for testicular torsion or if this was just an incidental postoperative finding. Enlargement of the testis due to the hemangioma may have triggered the torsion of the testis. 4 Conclusion Cavernous hemangioma is a rare tumor of the testicle in either childhood or adult period. The particularity of the presented case is the possible association of a cavernous intratesticular hemangioma with the torsion of the testis in a teenager. Clinicians and pathologists must be aware of the rare entity of testicular hemangiomas, as clinical examination and imaging studies do not often suffice to arrive at a correct diagnosis. Conflicts of interest We have nothing to declare in any of these categories. Funding We have nothing to declare in any of these categories. Ethical approval There is ethical approval from the ethical committee of Land Kärnten number A20/18. Consent Written informed consent was obtained from the patient’s parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Author contribution Tepeneu N.F. – writing of the paper, study concept and design. Krafka K. – data collection. Meglic S.– data collection. Rogatsch H. – data analysis. Fasching G. – study concept. Registration of research studies researchregistry3921. Guarantor Prim. Univ.-Doz. Dr. Günter Fasching, LL.M. ==== Refs References 1 Talmon G.A. Stanley S.M. Lager D.J. Capillary hemangioma of the testis Int. J. Surg. Pathol. 19 2011 398 400 19147509 2 Mazal P.R. Kratzik C. Kain R. Susani M. Capillary haemangioma of the testis J Clin. Pathol. 53 August (8) 2000 641 642 11002773 3 Agha R.A. Fowler A.J. Saetta A. Barai I. Rajmohan S. Orgill D.P. for the SCARE Group The SCARE statement: consensus-based surgical case report guidelines Int. J. Surg. 34 2016 180 186 27613565 4 Ermis B. Otken A. Zulfikar B. Yılmaz K. Ilter M. Sogut A. Hemangiomas Turkiye Klinikleri J. Med. Sci. 16 1996 423 426 5 Mazzella F.M. Sieber S.C. Lopez V. Histocytoid hemangioma of the testis: a case report J. Urol. 153 1995 743 744 7861527 6 Chavan D. Javalgi A.P. Scrotal hemangioma: a case report J. Clin. Diagn. Res. 8 12 2014 ND03 ND04 7 Minagawa T. Murata Y. Testicular cavernous hemangioma associated with intrascrotal testicular torsion: a case report Hinyokika Kiyo 55 2009 161 163 19378830 8 Rastogi R. Diffuse cavernous hemangioma of the penis, scrotum, perineum, and rectum: a rare tumor Saudi J. Kidney Dis. Transpl. 19 4 2008 614 618 18580022 9 Froehner M. Tsatalpas P. Wirth M.P. Giant penile cavernous hemangioma with intrapelvic extension Urology 53 1999 414 415 9933067 10 Keret D. Kam I. Ben-Arieh Y. Scrotal cavernous haemangioma with a family history of cutaneous angiomata J. R. Soc. Med. 83 1990 402 403 2380971 11 Yanai S. Tsutsumi H. Hotsubo T. Development of a testicular haemangioma after interferon therapy for hepatic haemangiomas: a case report Eur. J. Pediatr. 156 1997 784 786 9365068 12 Liu B. Chen J. Luo J. Zhou F. Wang C. Xie L. Cavernous hemangioma of the testis mimicking a testicular teratoma Exp. Ther. Med. 6 1 2013 91 92 23935725 13 Bonetti L.R. Schirosi L. Sartori G. Lupi M. De Gaetani C. Trani N. Maiorana A. Rapidly-growing hemangioma of the testicle clinically simulating an aggressive neoplasm. A case report Pathologica 101 June (3) 2009 123 125 19886547 14 Suriawinata A. Talerman A. Vapnek J.M. Hemangioma of the testis: report of unusual occurrences of cavernous hemangioma in a fetus and capillary hemangioma in an older man Ann. Diagn. Pathol. 5 2001 80 83 11294992 15 Atkin G. Miller M. Clarkson K.S. Testicular capillary haemangioma in a child J. R. Soc. Med. 94 2001 638 640 11733594 16 Vital R.J. de Mattos L.A. de Souza L.R.M.F. da Silva Figueirêdo S. Szejnfeld J. Sonographic findings in non-neoplastic testicular lesions Radiol. Bras. 40 1 2007 17 Tallen G. Hernáiz Driever P. Degenhardt P. Henze G. Riebel T. High reliability of scrotal ultrasonography in the management of childhood primary testicular neoplasms Klin Padiatr. 223 May (3) 2011 131 137 Epub 2011 Apr 1 21462100 18 Bharthi S. Ap N. Cavernous hemangioma of the testis mimicking as torsion of testis: a case report Ann. Pathol. Lab. Med. 2018 C40 C42 19 Barthold J.S. Abnormalities of the testis and scrotum and their surgical management McDougal W.S. Kavoussi L.R. Novick A.C. Partin A.W. Peters C.A. Campbell- Walsh Urology 10th ed. 2012 WB Saunders Philadelphia, PA 642 645 20 Uguz S. Yilmaz S. Guragac A. Topuz B. Aydur E. Association of torsion with testicular cancer: a retrospective study Clin. Genitourin. Cancer 14 February (1) 2016 e55 e57 26500052