SRU TOSHIBA RESIDENT TEACHING CASE
Ruptured Testicular Yolk Sac Tumor Joseph Zechlinski, MD, Mark Sharafinski, MD, and Gary Sudakoff, MD
CLINICAL HISTORY A 25-year-old man presented with 3 days of mild scrotal swelling and tenderness. Several hours before his evaluation in the emergency department, he awoke to discover skin ulceration, bleeding, and extrusion of pale material from his right hemiscrotum. Scrotal ultrasound was performed (Fig. 1), followed by subsequent computed tomography (CT) of the chest, abdomen, and pelvis (Fig. 2). After radical inguinal orchiectomy, pathology result demonstrated a 10-cm tumor extending to the scrotal skin.
DIAGNOSIS Yolk sac tumor resulting in testicular rupture and perforation of the scrotal wall was diagnosed.
DISCUSSION Testicular rupture is an infrequent cause of acute scrotal pain and typically presents with a history of blunt or penetrating trauma.1 This is a rare case of atraumatic testicular rupture with scrotal perforation in the setting of underlying neoplastic disease. To date, only 2 similar cases have been reported.2,3 Despite the infrequency of associated testicular rupture, neoplastic processes remain an important differential consideration in the evaluation of acute scrotal swelling or pain, especially in young adults. Testicular cancer is the most common cancer in men aged 15 to 44 years, with an incidence of 5.1 per 100,000 men in North America.4 Its incidence varies greatly by geography, being most common in Europe, Australia, and North America. Ultrasound is the most appropriate initial imaging examination in emergency conditions of the scrotum.5 Acute scrotal pathology is often divided into 4 categories (‘‘4 Ts’’): trauma, torsion, infection (‘‘testiculitis’’), and tumor.6 The sonographic findings of the testicular rupture include disruption of the tunica albuginea, altered testicular echogenicity (secondary to intratesticular fracture and/or extrusion of seminiferous tubules), and associated intrascrotal hematoma. The ultrasound findings of torsion include testicular enlargement,
Received for publication April 21, 2014; accepted April 23, 2014. Department of Radiology, Medical College of Wisconsin, Milwaukee, WI. The authors declare no conflict of interest. Reprints: Joseph Zechlinski, MD, Department of Radiology, Medical College of Wisconsin, 9200 W Wisconsin Ave, Milwaukee, WI 53226 (e-mail: [email protected]). Copyright * 2014 by Lippincott Williams & Wilkins
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hypoechogenicity (as compared with contralateral normal testis), and decreased or absent blood flow on color or power Doppler imaging. An ipsilateral hydrocele may be present. Infection, presenting as epididymitis or epididymo-orchitis, appears as an enlarged, heterogeneous epididymis or testis with increased vascularity on color Doppler imaging and often with an associated hydrocele or pyocele (Fig. 2). Severe orchitis may present as focal or diffuse testicular necrosis with or without abscess formation. Intrascrotal masses have a wide range of appearances, with sonographic sensitivity and ability to differentiate between extratesticular (usually benign) and intratesticular (usually malignant) lesions approaching 100%.7 Most (95%) primary testicular neoplasms are of germ cell origin, commonly divided into seminomatous and nonseminomatous germ cell tumors. Seminomas represent up to 50% of germ cell tumors, making them the most common testicular neoplasm.7 Less common germ cell tumors include embryonal, yolk sac, choriocarcinoma, teratoma, polyembryoma, gonadoblastoma, and mixtures thereof. Nonseminomatous germ cell tumors, such as the yolk sac tumor in this case, are more aggressive and less sensitive to chemoradiation than seminomas. On ultrasound, these lesions are typically hypoechoic, heterogeneous secondary to cystic necrosis or hemorrhage, and variably calcified. Malignant tumors larger than 1 cm are usually hypervascular or demonstrate disorganized internal vascularity. Pure yolk sac tumors are common in infants, whereas adult forms are typically found in combination with other kinds of germ cell tumor (particularly teratoma and embryonal carcinoma).8 Seminomas are very sensitive to radiation and chemotherapy, whereas nonseminomatous tumors are usually treated with a combination of surgery and chemotherapy.7 In this case, a high clinical suspicion for malignancy (based on progressive swelling of the right testis associated with hemorrhage, in the absence of trauma or significant infection) ultimately led to scrotal exploration, right inguinal orchiectomy, and scrotoplasty (Fig. 3). Laboratory analysis near the time of surgery yielded the following: >-fetoprotein, 10,393; A-human chorionic gonadotropin, 8.1; and lactate dehydrogenase, 286. A right testis mass with an area of erosion on the right lateral hemiscrotum with hemorrhage was identified operatively (Figs. 3 and 4). Scrotal and inguinal incisions were required, and although the tunica vaginalis was not violated during dissection, drainage from the scrotal sinus tract was concerning for theoretical tumor spillage. The patient underwent 3 cycles of chemotherapy consisting of bleomycin, cisplatin, and etoposide. At the 6-month follow-up, the CT of the chest, abdomen, and pelvis demonstrated no evidence of local tumor recurrence or metastatic disease. Tumor markers revealed no Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Ruptured Testicular Yolk Sac Tumor
FIGURE 1. A, Sagittal image through the right hemiscrotum reveals a large complex hematoma (yellow arrow) surrounding a poorly defined testis (green arrow). The apparent disruption of the tunica (not shown) was strongly suggestive of testicular rupture. In the absence of trauma or signs of significant infection, the possibility of an underlying tumor versus other cause of the rupture was questioned. B, Sagittal image through the left hemiscrotum demonstrates a normal left testis (T).
FIGURE 2. A, Axial CT image through the scrotum reveals a large, heterogeneous mass occupying nearly the entire scrotum (outlined by yellow arrows). Two locules of free air, secondary to ulceration of the overlying skin, are visible along the left lateral margin of the mass (red arrow). B, Sagittal image through the right scrotum demonstrates a complex mass eroding through the scrotal wall (yellow arrow).
FIGURE 3. Intraoperative photograph taken after scrotal incision but before tumor resection demonstrates a large right testicular mass with ulceration of the overlying scrotum (yellow arrow). * 2014 Lippincott Williams & Wilkins
FIGURE 4. Microscopic specimen of the resected mass demonstrates microcystic and reticular patterns with the tumor cells containing vacuolated cytoplasm (yellow arrow), intercellular eosinophilic globules (blue arrow), and extracellular pink basement membraneYlike material (black arrow). Sections show vessels surrounded by a layer of tumor cells (Schiller-Duval bodies), indicating perivascular invasion (green arrow). An area of adjacent peritumoral hematoma (H) is also noted.
evidence of disease recurrence (>-fetoprotein, 4.8; A-human chorionic gonadotropin, G1.0; Fig. 4). The case presented here represents an extremely rare entity of atraumatic scrotal rupture in the setting of underlying testicular neoplasm. Very few cases of atraumatic scrotal rupture have been described in the modern literature, most often associated with infection rather than tumor. The distinction is paramount to guide appropriate therapy. In cases of scrotal enlargement without typical clinical or laboratory findings of infection, the possibility of an underlying malignant process should always be considered. www.ultrasound-quarterly.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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Ultrasound Quarterly
Zechlinski et al
REFERENCES 1. Davis JE, Silverman M. Scrotal emergencies. Emerg Med Clin North Am. 2011;29:469Y484. 2. LaMontagne AE Jr. Spontaneous rupture of a testicular tumor. J Urol. 2002;167:1787Y1788. 3. Vidyavathi K, Prabhakar K, Harendra KM. Primary testicular lymphoma with rupture: an unusual presentation. J Nat Sci Biol Med. 2013;4:232Y235. 4. Rosen A, Jayram G, Drazer M, et al. Global trends in testicular cancer incidence and mortality. Eur Urol. 2011;60:374Y379.
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& Volume 30, Number 3, September 2014
5. Remer EM, Casalino DD, Arellano RS, et al. ACR Appropriateness Criteria\ acute onset of scrotal painVwithout trauma, without antecedent mass. Ultrasound Q. 2012;28:47Y51. 6. Wittenberg AF, Tobias T, Rzeszotarski M, et al. Sonography of the acute scrotum: the four T’s of testicular imaging. Curr Probl Diagn Radiol. 2006;35:12Y21. 7. Cokkinos DD, Antypa E, Tserotas P, et al. Emergency ultrasound of the scrotum: a review of the commonest pathologic conditions. Curr Probl Diagn Radiol. 2011;40:1Y14. 8. Cao D, Humphrey PA. Yolk sac tumor of the testis. J Urol. 2011;186:1475Y1476.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Ruptured Testicular Yolk Sac Tumor Joseph Zechlinski, MD, Mark Sharafinski, MD, and Gary Sudakoff, MD
CLINICAL HISTORY A 25-year-old man presented with 3 days of mild scrotal swelling and tenderness. Several hours before his evaluation in the emergency department, he awoke to discover skin ulceration, bleeding, and extrusion of pale material from his right hemiscrotum. Scrotal ultrasound was performed (Fig. 1), followed by subsequent computed tomography (CT) of the chest, abdomen, and pelvis (Fig. 2). After radical inguinal orchiectomy, pathology result demonstrated a 10-cm tumor extending to the scrotal skin.
DIAGNOSIS Yolk sac tumor resulting in testicular rupture and perforation of the scrotal wall was diagnosed.
DISCUSSION Testicular rupture is an infrequent cause of acute scrotal pain and typically presents with a history of blunt or penetrating trauma.1 This is a rare case of atraumatic testicular rupture with scrotal perforation in the setting of underlying neoplastic disease. To date, only 2 similar cases have been reported.2,3 Despite the infrequency of associated testicular rupture, neoplastic processes remain an important differential consideration in the evaluation of acute scrotal swelling or pain, especially in young adults. Testicular cancer is the most common cancer in men aged 15 to 44 years, with an incidence of 5.1 per 100,000 men in North America.4 Its incidence varies greatly by geography, being most common in Europe, Australia, and North America. Ultrasound is the most appropriate initial imaging examination in emergency conditions of the scrotum.5 Acute scrotal pathology is often divided into 4 categories (‘‘4 Ts’’): trauma, torsion, infection (‘‘testiculitis’’), and tumor.6 The sonographic findings of the testicular rupture include disruption of the tunica albuginea, altered testicular echogenicity (secondary to intratesticular fracture and/or extrusion of seminiferous tubules), and associated intrascrotal hematoma. The ultrasound findings of torsion include testicular enlargement,
Received for publication April 21, 2014; accepted April 23, 2014. Department of Radiology, Medical College of Wisconsin, Milwaukee, WI. The authors declare no conflict of interest. Reprints: Joseph Zechlinski, MD, Department of Radiology, Medical College of Wisconsin, 9200 W Wisconsin Ave, Milwaukee, WI 53226 (e-mail: [email protected]). Copyright * 2014 by Lippincott Williams & Wilkins
238
www.ultrasound-quarterly.com
hypoechogenicity (as compared with contralateral normal testis), and decreased or absent blood flow on color or power Doppler imaging. An ipsilateral hydrocele may be present. Infection, presenting as epididymitis or epididymo-orchitis, appears as an enlarged, heterogeneous epididymis or testis with increased vascularity on color Doppler imaging and often with an associated hydrocele or pyocele (Fig. 2). Severe orchitis may present as focal or diffuse testicular necrosis with or without abscess formation. Intrascrotal masses have a wide range of appearances, with sonographic sensitivity and ability to differentiate between extratesticular (usually benign) and intratesticular (usually malignant) lesions approaching 100%.7 Most (95%) primary testicular neoplasms are of germ cell origin, commonly divided into seminomatous and nonseminomatous germ cell tumors. Seminomas represent up to 50% of germ cell tumors, making them the most common testicular neoplasm.7 Less common germ cell tumors include embryonal, yolk sac, choriocarcinoma, teratoma, polyembryoma, gonadoblastoma, and mixtures thereof. Nonseminomatous germ cell tumors, such as the yolk sac tumor in this case, are more aggressive and less sensitive to chemoradiation than seminomas. On ultrasound, these lesions are typically hypoechoic, heterogeneous secondary to cystic necrosis or hemorrhage, and variably calcified. Malignant tumors larger than 1 cm are usually hypervascular or demonstrate disorganized internal vascularity. Pure yolk sac tumors are common in infants, whereas adult forms are typically found in combination with other kinds of germ cell tumor (particularly teratoma and embryonal carcinoma).8 Seminomas are very sensitive to radiation and chemotherapy, whereas nonseminomatous tumors are usually treated with a combination of surgery and chemotherapy.7 In this case, a high clinical suspicion for malignancy (based on progressive swelling of the right testis associated with hemorrhage, in the absence of trauma or significant infection) ultimately led to scrotal exploration, right inguinal orchiectomy, and scrotoplasty (Fig. 3). Laboratory analysis near the time of surgery yielded the following: >-fetoprotein, 10,393; A-human chorionic gonadotropin, 8.1; and lactate dehydrogenase, 286. A right testis mass with an area of erosion on the right lateral hemiscrotum with hemorrhage was identified operatively (Figs. 3 and 4). Scrotal and inguinal incisions were required, and although the tunica vaginalis was not violated during dissection, drainage from the scrotal sinus tract was concerning for theoretical tumor spillage. The patient underwent 3 cycles of chemotherapy consisting of bleomycin, cisplatin, and etoposide. At the 6-month follow-up, the CT of the chest, abdomen, and pelvis demonstrated no evidence of local tumor recurrence or metastatic disease. Tumor markers revealed no Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Ruptured Testicular Yolk Sac Tumor
FIGURE 1. A, Sagittal image through the right hemiscrotum reveals a large complex hematoma (yellow arrow) surrounding a poorly defined testis (green arrow). The apparent disruption of the tunica (not shown) was strongly suggestive of testicular rupture. In the absence of trauma or signs of significant infection, the possibility of an underlying tumor versus other cause of the rupture was questioned. B, Sagittal image through the left hemiscrotum demonstrates a normal left testis (T).
FIGURE 2. A, Axial CT image through the scrotum reveals a large, heterogeneous mass occupying nearly the entire scrotum (outlined by yellow arrows). Two locules of free air, secondary to ulceration of the overlying skin, are visible along the left lateral margin of the mass (red arrow). B, Sagittal image through the right scrotum demonstrates a complex mass eroding through the scrotal wall (yellow arrow).
FIGURE 3. Intraoperative photograph taken after scrotal incision but before tumor resection demonstrates a large right testicular mass with ulceration of the overlying scrotum (yellow arrow). * 2014 Lippincott Williams & Wilkins
FIGURE 4. Microscopic specimen of the resected mass demonstrates microcystic and reticular patterns with the tumor cells containing vacuolated cytoplasm (yellow arrow), intercellular eosinophilic globules (blue arrow), and extracellular pink basement membraneYlike material (black arrow). Sections show vessels surrounded by a layer of tumor cells (Schiller-Duval bodies), indicating perivascular invasion (green arrow). An area of adjacent peritumoral hematoma (H) is also noted.
evidence of disease recurrence (>-fetoprotein, 4.8; A-human chorionic gonadotropin, G1.0; Fig. 4). The case presented here represents an extremely rare entity of atraumatic scrotal rupture in the setting of underlying testicular neoplasm. Very few cases of atraumatic scrotal rupture have been described in the modern literature, most often associated with infection rather than tumor. The distinction is paramount to guide appropriate therapy. In cases of scrotal enlargement without typical clinical or laboratory findings of infection, the possibility of an underlying malignant process should always be considered. www.ultrasound-quarterly.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
239
Ultrasound Quarterly
Zechlinski et al
REFERENCES 1. Davis JE, Silverman M. Scrotal emergencies. Emerg Med Clin North Am. 2011;29:469Y484. 2. LaMontagne AE Jr. Spontaneous rupture of a testicular tumor. J Urol. 2002;167:1787Y1788. 3. Vidyavathi K, Prabhakar K, Harendra KM. Primary testicular lymphoma with rupture: an unusual presentation. J Nat Sci Biol Med. 2013;4:232Y235. 4. Rosen A, Jayram G, Drazer M, et al. Global trends in testicular cancer incidence and mortality. Eur Urol. 2011;60:374Y379.
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& Volume 30, Number 3, September 2014
5. Remer EM, Casalino DD, Arellano RS, et al. ACR Appropriateness Criteria\ acute onset of scrotal painVwithout trauma, without antecedent mass. Ultrasound Q. 2012;28:47Y51. 6. Wittenberg AF, Tobias T, Rzeszotarski M, et al. Sonography of the acute scrotum: the four T’s of testicular imaging. Curr Probl Diagn Radiol. 2006;35:12Y21. 7. Cokkinos DD, Antypa E, Tserotas P, et al. Emergency ultrasound of the scrotum: a review of the commonest pathologic conditions. Curr Probl Diagn Radiol. 2011;40:1Y14. 8. Cao D, Humphrey PA. Yolk sac tumor of the testis. J Urol. 2011;186:1475Y1476.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
