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Epidermoid cyst of the testis in an adolescent: Case report and review of the evolution of the surgical management
Epidermoid Cyst of the Testis in an Adolescent: Case Report and Review of the Evolution of the Surgical Management By Ronan Lev, Yoram Mor, Ilan Leibovitch, Jacob Golomb, Marina Perelman, Zahava Heyman, and Jacob Ramon Ramat Gan, Israel
Epidermoid cyst, a tumorlike lesion, is a rare benign testicular tumor with typical, distinctive ultrasonographic appearance. When the preoperative features are highly suggestive of epidermoid cyst, testicular sparing surgery after strict oncologic guidelines is warranted. The authors report a case of a preoperatively suspected epidermoid cyst
in an adolescent and review the literature concerning the changing paradigm of management. J Pediatr Surg 37:121-123. Copyright © 2002 by W.B. Saunders Company. INDEX WORDS: Testis, testicular neoplasm, epidermoid cyst, organ preservation.
E
predominance to the right testis, similar to germ cell tumors. Bilateral occurrence is rare. Clinically, an epidermoid cyst appears as a circumscribed, firm, smooth, unilateral nontender, intratesticular mass, which is located peripherally.5 The ultrasonographic characteristics include a well-circumscribed hypoechoic mass with an hyperechogenic rim and center. The ongoing deposition of keratinized material inside the cyst creates an echogenic whorl forming an onionskin or targetlike typical appearance, seen in ultrasound scan and magnetic resonance imaging (MRI) studies.6-11 The pathologic diagnosis of epidermoid cysts is based on criteria proposed by Price.12 However, the most important parameters probably are the lack of dermal elements such as hair follicles or sebaceous glands13 and the absence of testicular intraepithelial neoplasia adjacent to the lesion.3 The histogenesis of epidermoid cysts is unclear. Through the years it has been considered to derive from a metaplastic transition or to represent a mesothelial or an epidermal inclusion cyst. Based on the epidemiologic analogy that exists between epidermoid cysts and germ cell tumors of the testis, Shah et al4 argued that epidermoid cysts are differentiated monolayer teratomas from germ cell origin. More recently, Dieckmann and Loy3 questioned the prevalent monolayer teratoma assumption implying that the lack of testicular intraepithelial neoplasia in epidermoid cyst– bearing testicles disproves their germ cell origin. Rather,
PIDERMOID CYSTS of the testis are rare testicular tumorlike lesions, constituting 1% of all testicular tumors and 3% of pediatric testicular tumors.1 Since its first report in 1942,2 more than 300 cases have been reported in the literature.3 During the last decades, major alterations occurred in the understanding of the histogenesis of epidermoid cysts consequently leading to a shift in the management schemes of these tumors from radical orchiectomy to testis preservation.3,4 Herein, we present a case of adolescent epidermoid cyst and review the changing paradigm of management. CASE REPORT A 16-year-old boy was referred to our department because of a right testicular mass found on a routine examination. Five years before this admission he underwent a right inguinal hernia repair, which was complicated by postoperative orchiepididymitis. Physical examination found a 1.5- ⫻ 1.5-cm hard, nontender mass in the posterior aspect of the right testis, a grade III left-sided varicocele, and a slight asymmetry of the testicles with the left testis being smaller than the right. Serum alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal range. High resolution testicular ultrasonography showed a well-defined hypoechoic lesion with typical peripheral hyperechogenicity (Fig 1), highly suggestive of an epidermoid cyst of the testis. Exploration of the right testis through an inguinal incision found a well-defined peripheral 1.5- ⫻ 1.5-cm mass palpated just beneath the tunica albuginea. The overlying tunica was excised, and the intact mass was enucleated using blunt dissection. Because frozen section examination of the mass was negative for malignancy, the tunica albuginea was sutured, and the testis was replaced into the scrotum. Postoperative course was uneventful. Final histologic examination was consistent with the diagnosis of epidermoid cyst (Fig 2). An ultrasound study performed 6 months postoperatively showed normal testicular parenchyma on the right side.
DISCUSSION
Epidermoid cyst of the testis usually presents as an incidental finding of a painless nodule.3 The tumor usually measures 2 to 3 cm on average, with a slight Journal of Pediatric Surgery, Vol 37, No 1 (January), 2002: pp 121-123
From the Departments of Urology, Pathology, and Imaging (Ultrasound Unit), The Chaim-Sheba Medical Center, Tel-Hashomer, RamatGan (Affiliated with Tel-Aviv University), Israel. Address reprint requests to Dr Yoram Mor, Department of Urology, The Chaim Sheba Medical Center, Ramat Gan, 52621, Israel. Copyright © 2002 by W.B. Saunders Company 0022-3468/02/3701-0027$35.00/0 doi:10.1053/jpsu.2002.29444 121
122
they promoted the distinct entity of simple epidermoid cysts, also referred to as tumorlike lesions, which are non– germ cell neoplasms, as opposed to the rare complex epidermoid cyst, which is a teratomatous differentiation of the germ cell line. This controversy regarding the histogenesis of epidermoid cysts has had a direct effect on the conflicting surgical approaches to these lesions throughout the last decades. Shah et al4 who considered epidermoid cysts as a part of the germ cell line, has advocated radical orchiectomy in all cases of epidermoid cyst of the testis to allow a thorough examination of the whole testis and a more conclusive diagnosis of the exact nature of the lesion. This radical approach has been prevalent in the early years of the century, and it may account for the fact that 83% of 141 cases of epidermoid cysts reported in the world literature between the years 1942 to 1981 have undergone radical orchiectomy.4 However, 38%3 and 39%5 of the epidermoid cysts operated on between the years 1969 to 1995 have undergone testicular-sparing surgery. The optimal management of epidermoid cysts still is controversial. Nevertheless, radical orchiectomy, which traditionally has been the gold standard therapy, now is less frequently used. Indeed, more conservative approaches based on testis-sparing surgery, which has been the mainstay in the pediatric population,14 are gaining more acceptance in the modern treatment of epidermoid cysts, because of the psychological, hormonal, and reproductive implications that orchiectomy may have in such population of young healthy men.15 A most recent literature review shows that almost all of the reports published during the last decade on this subject,
LEV ET AL
Fig 2. Histologic slide shows epidermoid cyst of the testis composed of thin fibrotic wall lined by atrophic squamous epithelium and filled with keratinous material. Adjacent seminiferous tubules are present (H&E, original magnification ⴛ40).
invariably emphasize the safety and importance of the conservative management of epidermoid cysts in all age groups.1,3,5-7,15 In addition to the shift in concepts of histogenesis, which undoubtedly have affected the approach to epidermoid cysts over the last decades, the introduction of high-resolution ultrasound imaging techniques and the characterization of the typical ultrasonographic features of epidermoid cysts have facilitated a more accurate preoperative diagnosis of these lesions6-11 allowing preoperative planning of testicular preservation. The high accuracy and specificity of intraoperative frozen sections for the detection of concomitant primary germ cell malignancy or testicular intraepithelial neoplasia in the tissues adjacent to the enucleated lesion is another factor that promoted safe testicular preservation.3,5 In summary, epidermoid cyst, classified histogenetically as a tumorlike lesion, is a rare, benign intratesticular growth, with distinctive clinical and ultrasonographic characteristics. When a diagnosis of epidermoid cyst is suggested, conservative testicular-sparing surgery is warranted. REFERENCES
Fig 1. An ultrasound scan shows a well-defined hypoechoic lesion with peripheral hyperechogenicity.
1. Ross JH, Kay R, Elder J: Testis sparing surgery for pediatric epidermoid cysts of the testis [see comments]. J Urol 149:353-356, 1993 2. Dockerty MD, Priestly JT: Dermoid cysts of testis. J Urol 48: 392-400, 1992 3. Dieckmann KP, Loy V: Epidermoid cyst of the testis: A review of clinical and histogenetic considerations. Br J Urol 73:436-441, 1994 4. Shah KH, Maxted WC, Chun B: Epidermoid cysts of the testis: A report of three cases and an analysis of 141 cases from the world literature. Cancer 47:577-582, 1981 5. Heidenreich A, Engelmann UH, Vietsch HV, et al: Organ preserving surgery in testicular epidermoid cysts. J Urol 153:1147-1150, 1995 6. Eustace S, Graham D, Behan M, et al: Ultrasonographic diagnosis
EPIDERMOID CYST OF THE TESTIS
and surgical enucleation of an epidermoid cyst of the testis. Br J Urol 74:518-519, 1994 7. Fu YT, Wang HH, Yang TH, et al: Epidermoid cysts of the testis: Diagnosis by ultrasonography and magnetic resonance imaging resulting in organ-preserving surgery. Br J Urol 78:116-118, 1996 8. Malvica RP: Epidermoid cyst of the testicle: An unusual sonographic finding. AJR 160:1047-1048, 1993 9. Maxwell AJ, Mamtora H: Sonographic appearance of epidermoid cyst of the testis. J Clin Ultrasound 18:188-190, 1990 10. Cohen EL, Mandel E, Goodman JD, et al: Epidermoid cyst of testicle. Ultrasonographic characteristics. Urology 24:79-81, 1984 11. Bahnson RR, Slasky BS, Ernstoff MS, et al: Sonographic
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characteristics of epidermoid cyst of testicle. Urology 35:508-510, 1990 12. Price EB: Epidermid cyst of the testis: A clinical and pathologic analysis of 69 cases from the testicular tumor registry. J Urol 102:708713, 1969 13. Garrett JE, Cartwright PC, Snow BW, et al: Cystic testicular lesions in the pediatric population. J Urol 163:928-936, 2000 14. Snyder SM, D’angio GJ, Evans AE, et al: Pediatric oncology, in Walsh PC, Retik AB, Vaughen ED, (eds): Campbell’s Urology vol 2. Philadelphia, PA, Saunders, 1998, pp 2245-2248 15. Chitale S, Morrow DR, Jena R, et al: Conservative surgery for epidermoid cyst of the testis. Br J Urol 80:506-507, 1997
Epidermoid cyst, a tumorlike lesion, is a rare benign testicular tumor with typical, distinctive ultrasonographic appearance. When the preoperative features are highly suggestive of epidermoid cyst, testicular sparing surgery after strict oncologic guidelines is warranted. The authors report a case of a preoperatively suspected epidermoid cyst
in an adolescent and review the literature concerning the changing paradigm of management. J Pediatr Surg 37:121-123. Copyright © 2002 by W.B. Saunders Company. INDEX WORDS: Testis, testicular neoplasm, epidermoid cyst, organ preservation.
E
predominance to the right testis, similar to germ cell tumors. Bilateral occurrence is rare. Clinically, an epidermoid cyst appears as a circumscribed, firm, smooth, unilateral nontender, intratesticular mass, which is located peripherally.5 The ultrasonographic characteristics include a well-circumscribed hypoechoic mass with an hyperechogenic rim and center. The ongoing deposition of keratinized material inside the cyst creates an echogenic whorl forming an onionskin or targetlike typical appearance, seen in ultrasound scan and magnetic resonance imaging (MRI) studies.6-11 The pathologic diagnosis of epidermoid cysts is based on criteria proposed by Price.12 However, the most important parameters probably are the lack of dermal elements such as hair follicles or sebaceous glands13 and the absence of testicular intraepithelial neoplasia adjacent to the lesion.3 The histogenesis of epidermoid cysts is unclear. Through the years it has been considered to derive from a metaplastic transition or to represent a mesothelial or an epidermal inclusion cyst. Based on the epidemiologic analogy that exists between epidermoid cysts and germ cell tumors of the testis, Shah et al4 argued that epidermoid cysts are differentiated monolayer teratomas from germ cell origin. More recently, Dieckmann and Loy3 questioned the prevalent monolayer teratoma assumption implying that the lack of testicular intraepithelial neoplasia in epidermoid cyst– bearing testicles disproves their germ cell origin. Rather,
PIDERMOID CYSTS of the testis are rare testicular tumorlike lesions, constituting 1% of all testicular tumors and 3% of pediatric testicular tumors.1 Since its first report in 1942,2 more than 300 cases have been reported in the literature.3 During the last decades, major alterations occurred in the understanding of the histogenesis of epidermoid cysts consequently leading to a shift in the management schemes of these tumors from radical orchiectomy to testis preservation.3,4 Herein, we present a case of adolescent epidermoid cyst and review the changing paradigm of management. CASE REPORT A 16-year-old boy was referred to our department because of a right testicular mass found on a routine examination. Five years before this admission he underwent a right inguinal hernia repair, which was complicated by postoperative orchiepididymitis. Physical examination found a 1.5- ⫻ 1.5-cm hard, nontender mass in the posterior aspect of the right testis, a grade III left-sided varicocele, and a slight asymmetry of the testicles with the left testis being smaller than the right. Serum alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal range. High resolution testicular ultrasonography showed a well-defined hypoechoic lesion with typical peripheral hyperechogenicity (Fig 1), highly suggestive of an epidermoid cyst of the testis. Exploration of the right testis through an inguinal incision found a well-defined peripheral 1.5- ⫻ 1.5-cm mass palpated just beneath the tunica albuginea. The overlying tunica was excised, and the intact mass was enucleated using blunt dissection. Because frozen section examination of the mass was negative for malignancy, the tunica albuginea was sutured, and the testis was replaced into the scrotum. Postoperative course was uneventful. Final histologic examination was consistent with the diagnosis of epidermoid cyst (Fig 2). An ultrasound study performed 6 months postoperatively showed normal testicular parenchyma on the right side.
DISCUSSION
Epidermoid cyst of the testis usually presents as an incidental finding of a painless nodule.3 The tumor usually measures 2 to 3 cm on average, with a slight Journal of Pediatric Surgery, Vol 37, No 1 (January), 2002: pp 121-123
From the Departments of Urology, Pathology, and Imaging (Ultrasound Unit), The Chaim-Sheba Medical Center, Tel-Hashomer, RamatGan (Affiliated with Tel-Aviv University), Israel. Address reprint requests to Dr Yoram Mor, Department of Urology, The Chaim Sheba Medical Center, Ramat Gan, 52621, Israel. Copyright © 2002 by W.B. Saunders Company 0022-3468/02/3701-0027$35.00/0 doi:10.1053/jpsu.2002.29444 121
122
they promoted the distinct entity of simple epidermoid cysts, also referred to as tumorlike lesions, which are non– germ cell neoplasms, as opposed to the rare complex epidermoid cyst, which is a teratomatous differentiation of the germ cell line. This controversy regarding the histogenesis of epidermoid cysts has had a direct effect on the conflicting surgical approaches to these lesions throughout the last decades. Shah et al4 who considered epidermoid cysts as a part of the germ cell line, has advocated radical orchiectomy in all cases of epidermoid cyst of the testis to allow a thorough examination of the whole testis and a more conclusive diagnosis of the exact nature of the lesion. This radical approach has been prevalent in the early years of the century, and it may account for the fact that 83% of 141 cases of epidermoid cysts reported in the world literature between the years 1942 to 1981 have undergone radical orchiectomy.4 However, 38%3 and 39%5 of the epidermoid cysts operated on between the years 1969 to 1995 have undergone testicular-sparing surgery. The optimal management of epidermoid cysts still is controversial. Nevertheless, radical orchiectomy, which traditionally has been the gold standard therapy, now is less frequently used. Indeed, more conservative approaches based on testis-sparing surgery, which has been the mainstay in the pediatric population,14 are gaining more acceptance in the modern treatment of epidermoid cysts, because of the psychological, hormonal, and reproductive implications that orchiectomy may have in such population of young healthy men.15 A most recent literature review shows that almost all of the reports published during the last decade on this subject,
LEV ET AL
Fig 2. Histologic slide shows epidermoid cyst of the testis composed of thin fibrotic wall lined by atrophic squamous epithelium and filled with keratinous material. Adjacent seminiferous tubules are present (H&E, original magnification ⴛ40).
invariably emphasize the safety and importance of the conservative management of epidermoid cysts in all age groups.1,3,5-7,15 In addition to the shift in concepts of histogenesis, which undoubtedly have affected the approach to epidermoid cysts over the last decades, the introduction of high-resolution ultrasound imaging techniques and the characterization of the typical ultrasonographic features of epidermoid cysts have facilitated a more accurate preoperative diagnosis of these lesions6-11 allowing preoperative planning of testicular preservation. The high accuracy and specificity of intraoperative frozen sections for the detection of concomitant primary germ cell malignancy or testicular intraepithelial neoplasia in the tissues adjacent to the enucleated lesion is another factor that promoted safe testicular preservation.3,5 In summary, epidermoid cyst, classified histogenetically as a tumorlike lesion, is a rare, benign intratesticular growth, with distinctive clinical and ultrasonographic characteristics. When a diagnosis of epidermoid cyst is suggested, conservative testicular-sparing surgery is warranted. REFERENCES
Fig 1. An ultrasound scan shows a well-defined hypoechoic lesion with peripheral hyperechogenicity.
1. Ross JH, Kay R, Elder J: Testis sparing surgery for pediatric epidermoid cysts of the testis [see comments]. J Urol 149:353-356, 1993 2. Dockerty MD, Priestly JT: Dermoid cysts of testis. J Urol 48: 392-400, 1992 3. Dieckmann KP, Loy V: Epidermoid cyst of the testis: A review of clinical and histogenetic considerations. Br J Urol 73:436-441, 1994 4. Shah KH, Maxted WC, Chun B: Epidermoid cysts of the testis: A report of three cases and an analysis of 141 cases from the world literature. Cancer 47:577-582, 1981 5. Heidenreich A, Engelmann UH, Vietsch HV, et al: Organ preserving surgery in testicular epidermoid cysts. J Urol 153:1147-1150, 1995 6. Eustace S, Graham D, Behan M, et al: Ultrasonographic diagnosis
EPIDERMOID CYST OF THE TESTIS
and surgical enucleation of an epidermoid cyst of the testis. Br J Urol 74:518-519, 1994 7. Fu YT, Wang HH, Yang TH, et al: Epidermoid cysts of the testis: Diagnosis by ultrasonography and magnetic resonance imaging resulting in organ-preserving surgery. Br J Urol 78:116-118, 1996 8. Malvica RP: Epidermoid cyst of the testicle: An unusual sonographic finding. AJR 160:1047-1048, 1993 9. Maxwell AJ, Mamtora H: Sonographic appearance of epidermoid cyst of the testis. J Clin Ultrasound 18:188-190, 1990 10. Cohen EL, Mandel E, Goodman JD, et al: Epidermoid cyst of testicle. Ultrasonographic characteristics. Urology 24:79-81, 1984 11. Bahnson RR, Slasky BS, Ernstoff MS, et al: Sonographic
123
characteristics of epidermoid cyst of testicle. Urology 35:508-510, 1990 12. Price EB: Epidermid cyst of the testis: A clinical and pathologic analysis of 69 cases from the testicular tumor registry. J Urol 102:708713, 1969 13. Garrett JE, Cartwright PC, Snow BW, et al: Cystic testicular lesions in the pediatric population. J Urol 163:928-936, 2000 14. Snyder SM, D’angio GJ, Evans AE, et al: Pediatric oncology, in Walsh PC, Retik AB, Vaughen ED, (eds): Campbell’s Urology vol 2. Philadelphia, PA, Saunders, 1998, pp 2245-2248 15. Chitale S, Morrow DR, Jena R, et al: Conservative surgery for epidermoid cyst of the testis. Br J Urol 80:506-507, 1997