Radiology Page
Metastatic “Burned-Out” Germ Cell Tumor of the Testis A 20-year-old man presented with a 3-week history of right upper quadrant abdominal pain radiating to the back. Physical examination, blood count and routine chemistry studies were normal. Right upper quadrant ultrasound (US) showed an 8.5 cm right upper abdominal mass. Contrast enhanced abdominal computerized tomography (CT) revealed the mass as retroperitoneal with central necrosis and a hepatic dome mass (fig. 1). There were also several pulmonary nodules suspicious for metastatic disease. Given the patient’s age, gender and imaging findings, blood work and scrotal US were ordered to evaluate for an underlying testicular tumor. Scrotal US showed a sub-centimeter cyst in the right testicle and an adjacent sub-centimeter hypoechoic area with a punctate echogenic focus (fig. 2). The remaining testicular parenchyma and left testis were normal. b-human chorionic gonadotropin (HCG) level was markedly elevated to more than 149,000 IU/L, lactic dehydrogenase was 599 U/L
Figure 1. Coronal reconstructed CT image shows right retroperitoneal mass, peripheral contrast enhancement and central necrosis (long arrow). Hypervascular liver dome lesion (short arrow) and right lower lobe pulmonary nodule (arrowhead) are also seen.
0022-5347/14/1923-0936/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
936
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RESEARCH, INC.
and a-fetoprotein was normal. Based on these findings, diagnosis was germ cell tumor (GCT) with metastases to the retroperitoneal lymph nodes, liver and lungs. Given a concern for choriocarcinoma based on the b-HCG level, a head CT was ordered and was negative. Upon referral to urology a 1 to 2 cm palpable right testicular mass was noted on physical examination. The patient underwent 4 cycles of neoadjuvant chemotherapy with bleomycin, etoposide and cisplatin, followed by right orchiectomy with retroperitoneal lymph node dissection and resection of the liver mass. Surgical pathology of the right testis showed a regressed testicular germ cell tumor with scar and focal residual teratoma component. There was no lymphovascular invasion. Pathological examination of the retroperitoneal mass, wedge resection of the liver and multiple lymph nodes demonstrated only organizing hemorrhage and necrosis consistent with a treated tumor. Despite high risk mixed germ cell cancer, the patient remains without evidence of disease more than 2 years after initial treatment. The most common tumor of the testis is GCT, which occurs in young men with 85% presenting between the ages of 15 and 44 years. Men with a personal history of testicular GCT, family history of testicular GCT in first degree relatives or history of cryptorchidism are at a greater risk for GCT but these tumors can develop in the absence of risk factors.1 Most patients with testicular GCT present with a palpable painless testicular lump in the body of the testis. Less commonly, patients present with symptoms related to metastatic disease. Testicular tumors metastasize by lymphatic and hematogenous routes. Ipsilateral retroperitoneal lymph nodes are the most common site for metastatic disease but metastases can also spread to the lungs, liver, supraclavicular lymph nodes and bones. Rarely, patients can present with metastatic symptoms of palpable enlarged lymph nodes, backache, right upper quadrant pain or pulmonary complaints such
http://dx.doi.org/10.1016/j.juro.2014.06.038 Vol. 192, 936-937, September 2014 Printed in U.S.A.
RADIOLOGY PAGE
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Figure 2. Sagittal gray scale (A) and color Doppler (B) US of right testis shows intratesticular cyst (arrow) and adjacent sub-centimeter, poorly marginated, hypoechoic area (arrowheads) with punctate echogenic focus (A) and some internal vascularity (B).
as dyspnea and hemoptysis, without an appreciable testicular mass.1 Ultrasound of the scrotum is indicated in young males with a palpable scrotal abnormality to determine if an intratesticular mass is present. Scrotal US is also indicated for a young man with an incidental retroperitoneal mass to assess for a primary testis tumor. The sensitivity of US in detecting an underlying intratesticular mass is almost 100%. US findings of GCTs vary, as pure seminomas are typically well-defined solid, hypoechoic lesions, while mixed GCTs are heterogeneous and ill-defined with cystic components and echogenic foci, particularly if teratoma elements are present.2 Rarely, extratesticular germ cell tumors are identified in the absence of a primary lesion. These “primary extratesticular” GCTs are thought to arise from primordial rests of germ cell tissue.3 Some patients with extratesticular GCT and a normal scrotal physical examination will have subtle intratesticular abnormalities identified by US such as echogenic foci, small hypechoic areas or calcifications. These findings have been shown to correspond histopathologically to a fibrous scar and are thought to represent a regressed or “burned out” testicular neoplasm. Burned-out GCT is an entity in which the testicular lesion undergoes changes and spontaneously regresses without therapy. Up
to 10% of retroperitoneal GCTs may be of this type. Tumor burnout can be due to immunological regression or as a result of ischemia. Any retroperitoneal lymph node enlargement, especially with cystic degeneration, should raise suspicion of an underlying germ cell tumor in a young man. Ultrasound of the testes and evaluation of testicular tumor markers (b-HCG, a-fetoprotein and lactic dehydrogenases) in these patients are warranted regardless of whether a testicular mass is palpated. The suspected affected testis should be removed to decrease risk of persistent or recurrent disease.4,5 Jawad M. Qureshi, Myra Feldman and Hadley Wood Cleveland Clinic Imaging Institute Cleveland, Ohio 1. Horwich A, Nicol D and Huddart R: Testicular germ cell tumours. BMJ 2013; 347: f5526. 2. Dogra V, Gottlieb R, Oka M et al: Sonography of the scrotum. Radiology 2003; 227: 18. 3. Rajiah P, Sinha R, Cuevas C et al: Imaging of uncommon retroperitoneal masses. Radiographics 2011; 31: 949. 4. Tasu JP, Faye N, Eschwege P et al: Imaging of burned-out testis tumor: five new cases and review of the literature. J Ultrasound Med 2003; 22: 515. 5. Comiter CV, Renshaw AA, Benson CB et al: Burned-out primary testicular cancer: sonographic and pathological characteristics. J Urol 1996; 156: 85.
Metastatic “Burned-Out” Germ Cell Tumor of the Testis A 20-year-old man presented with a 3-week history of right upper quadrant abdominal pain radiating to the back. Physical examination, blood count and routine chemistry studies were normal. Right upper quadrant ultrasound (US) showed an 8.5 cm right upper abdominal mass. Contrast enhanced abdominal computerized tomography (CT) revealed the mass as retroperitoneal with central necrosis and a hepatic dome mass (fig. 1). There were also several pulmonary nodules suspicious for metastatic disease. Given the patient’s age, gender and imaging findings, blood work and scrotal US were ordered to evaluate for an underlying testicular tumor. Scrotal US showed a sub-centimeter cyst in the right testicle and an adjacent sub-centimeter hypoechoic area with a punctate echogenic focus (fig. 2). The remaining testicular parenchyma and left testis were normal. b-human chorionic gonadotropin (HCG) level was markedly elevated to more than 149,000 IU/L, lactic dehydrogenase was 599 U/L
Figure 1. Coronal reconstructed CT image shows right retroperitoneal mass, peripheral contrast enhancement and central necrosis (long arrow). Hypervascular liver dome lesion (short arrow) and right lower lobe pulmonary nodule (arrowhead) are also seen.
0022-5347/14/1923-0936/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
936
j
www.jurology.com
AND
RESEARCH, INC.
and a-fetoprotein was normal. Based on these findings, diagnosis was germ cell tumor (GCT) with metastases to the retroperitoneal lymph nodes, liver and lungs. Given a concern for choriocarcinoma based on the b-HCG level, a head CT was ordered and was negative. Upon referral to urology a 1 to 2 cm palpable right testicular mass was noted on physical examination. The patient underwent 4 cycles of neoadjuvant chemotherapy with bleomycin, etoposide and cisplatin, followed by right orchiectomy with retroperitoneal lymph node dissection and resection of the liver mass. Surgical pathology of the right testis showed a regressed testicular germ cell tumor with scar and focal residual teratoma component. There was no lymphovascular invasion. Pathological examination of the retroperitoneal mass, wedge resection of the liver and multiple lymph nodes demonstrated only organizing hemorrhage and necrosis consistent with a treated tumor. Despite high risk mixed germ cell cancer, the patient remains without evidence of disease more than 2 years after initial treatment. The most common tumor of the testis is GCT, which occurs in young men with 85% presenting between the ages of 15 and 44 years. Men with a personal history of testicular GCT, family history of testicular GCT in first degree relatives or history of cryptorchidism are at a greater risk for GCT but these tumors can develop in the absence of risk factors.1 Most patients with testicular GCT present with a palpable painless testicular lump in the body of the testis. Less commonly, patients present with symptoms related to metastatic disease. Testicular tumors metastasize by lymphatic and hematogenous routes. Ipsilateral retroperitoneal lymph nodes are the most common site for metastatic disease but metastases can also spread to the lungs, liver, supraclavicular lymph nodes and bones. Rarely, patients can present with metastatic symptoms of palpable enlarged lymph nodes, backache, right upper quadrant pain or pulmonary complaints such
http://dx.doi.org/10.1016/j.juro.2014.06.038 Vol. 192, 936-937, September 2014 Printed in U.S.A.
RADIOLOGY PAGE
937
Figure 2. Sagittal gray scale (A) and color Doppler (B) US of right testis shows intratesticular cyst (arrow) and adjacent sub-centimeter, poorly marginated, hypoechoic area (arrowheads) with punctate echogenic focus (A) and some internal vascularity (B).
as dyspnea and hemoptysis, without an appreciable testicular mass.1 Ultrasound of the scrotum is indicated in young males with a palpable scrotal abnormality to determine if an intratesticular mass is present. Scrotal US is also indicated for a young man with an incidental retroperitoneal mass to assess for a primary testis tumor. The sensitivity of US in detecting an underlying intratesticular mass is almost 100%. US findings of GCTs vary, as pure seminomas are typically well-defined solid, hypoechoic lesions, while mixed GCTs are heterogeneous and ill-defined with cystic components and echogenic foci, particularly if teratoma elements are present.2 Rarely, extratesticular germ cell tumors are identified in the absence of a primary lesion. These “primary extratesticular” GCTs are thought to arise from primordial rests of germ cell tissue.3 Some patients with extratesticular GCT and a normal scrotal physical examination will have subtle intratesticular abnormalities identified by US such as echogenic foci, small hypechoic areas or calcifications. These findings have been shown to correspond histopathologically to a fibrous scar and are thought to represent a regressed or “burned out” testicular neoplasm. Burned-out GCT is an entity in which the testicular lesion undergoes changes and spontaneously regresses without therapy. Up
to 10% of retroperitoneal GCTs may be of this type. Tumor burnout can be due to immunological regression or as a result of ischemia. Any retroperitoneal lymph node enlargement, especially with cystic degeneration, should raise suspicion of an underlying germ cell tumor in a young man. Ultrasound of the testes and evaluation of testicular tumor markers (b-HCG, a-fetoprotein and lactic dehydrogenases) in these patients are warranted regardless of whether a testicular mass is palpated. The suspected affected testis should be removed to decrease risk of persistent or recurrent disease.4,5 Jawad M. Qureshi, Myra Feldman and Hadley Wood Cleveland Clinic Imaging Institute Cleveland, Ohio 1. Horwich A, Nicol D and Huddart R: Testicular germ cell tumours. BMJ 2013; 347: f5526. 2. Dogra V, Gottlieb R, Oka M et al: Sonography of the scrotum. Radiology 2003; 227: 18. 3. Rajiah P, Sinha R, Cuevas C et al: Imaging of uncommon retroperitoneal masses. Radiographics 2011; 31: 949. 4. Tasu JP, Faye N, Eschwege P et al: Imaging of burned-out testis tumor: five new cases and review of the literature. J Ultrasound Med 2003; 22: 515. 5. Comiter CV, Renshaw AA, Benson CB et al: Burned-out primary testicular cancer: sonographic and pathological characteristics. J Urol 1996; 156: 85.
