eMedicine Specialties > Urology > Cancer, Testicle

Testicular Choriocarcinoma

Author: Michael B Williams, MD, MS, Fellow, Department of Urologic Oncology, MD Anderson Cancer Center
Coauthor(s): Paul Schellhammer, MD, Chairman, Program Director, Professor, Department of Urology, Eastern Virginia Medical School; John W Davis, MD, Assistant Professor, Department of Urology, University of Texas MD Anderson Cancer Center
Contributor Information and Disclosures

Updated: May 21, 2009

Introduction

Background

Pure choriocarcinoma of the testis is the exception to most of the rules established for testicular seminoma and all other forms of nonseminomatous germ cell tumors (NSGCTs). Like other germ cell tumors (GCTs), choriocarcinoma typically affects younger men. Unlike other cancers, choriocarcinoma metastasizes hematogenously, with the testicular primary tumor often small or even "burned-out." In most reports, the tumor responds poorly to radiation and chemotherapy and carries high mortality rate. Surgery is usually limited to radical orchiectomy for tissue diagnosis.1

Pathophysiology

Choriocarcinoma recapitulates placental tissue development. For unknown reasons, it metastasizes early via hematogenous routes to the lung, liver, and brain, among others.1

Frequency

United States

Testicular GCTs are rare, representing only 1%-2% of all malignancies in males and occurring in 1 of 250 men by age 65 years. However, these tumors represent the most common malignancy in men aged 15-35 years. The incidence rates are 3.7 cases and 0.9 cases per 100,000 per year in white males and black males, respectively. GCTs have several subtypes and frequencies, including seminoma (40%), embryonal tumor (25%), teratocarcinoma (25%), teratoma (5%), and choriocarcinoma (pure; 1%).

International

The incidence of testis cancer increased worldwide from the early 1960s to the mid 1980s. The malignancy is more common in whites than in nonwhites. The highest rates are in Denmark (8.4 cases per 100,000 per y) and Switzerland (6.2-8.8 cases per 100,000 per y), and the frequency varies across Europe. Because of its rare incidence, the international rates of pure choriocarcinoma are unknown.

Race

No racial differences in choriocarcinoma have been reported.

Age

  • Ramon y Cajal et al (1987) reported a case of pure choriocarcinoma in the oldest patient recorded, aged 63 years.2 The patient died of aspiration shortly after initiation of chemotherapy, so a determination of treatment efficacy in this age group was impossible. The second-oldest patient reported was aged 50 years.
  • In a literature review of 10,000 cases of germinal testicular cell tumors, Ramon y Cajal et al found 54 (0.5%) cases of pure choriocarcinoma. The tumors occurred most commonly in men aged 20-30 years.2
  • In a 2008 review of 50 men older than 60 years with GCT, only one was found to have a component of choriocarcinoma.3

Clinical

History

  • Unlike classic seminoma or mixed GCTs, pure choriocarcinoma is more likely to manifest as symptoms of metastatic disease and is the most common element observed in brain metastases.
  • The local tumor itself may be small and may not cause symptoms.

Physical

  • The local tumor in choriocarcinoma may be small or nonpalpable, whereas most testicular GCTs cause scrotal swelling and a palpable mass. Testicular pain, with or without radiating pain to the groin and abdomen, is possible but is more consistent with epididymitis.
  • Widely metastatic testicular GCTs, including choriocarcinoma, may also manifest as a "burned-out" local testis lesion that consists of fibrous scar with absent or minute amounts of viable tumor.
  • Physical examination findings from lung, liver, and/or brain metastases may be more pronounced than an abnormal finding on testicular examination.

Causes

  • Patients with a history of cryptorchidism are at a greatly increased risk of testis cancer (by a factor of 10-40 times). Abdominal undescended testis is associated with a greater risk than the inguinal form.
    • An abdominal testis cancer is more likely to be seminoma, while a testis surgically brought to the scrotum via orchiopexy is more likely to be an NSGCT.
    • Orchiopexy allows for earlier detection by physical examination but does not alter the risk of GCT.
    • Ten percent of patients with a GCT have a history of cryptorchidism.
    • In a series of 125 patients with a history or clinical evidence of cryptorchidism and testis tumor, 3 (2%) were pure choriocarcinoma, which is similar to the overall incidence of choriocarcinoma among GCTs.4
  • Genetic changes in the form of amplifications and deletions are observed predominantly in the 12p11.2-p12.1 chromosomal region.
    • Gain of 12p sequences is associated with invasive growth of seminomas and nonseminomas.
    • In contrast, spermatocytic seminoma shows a gain of chromosome 9, and most infantile yolk sac tumors and teratomas show no chromosomal changes.
  • Other risks include trauma, mumps, and maternal estrogen exposure.

More on Testicular Choriocarcinoma

Overview: Testicular Choriocarcinoma
Differential Diagnoses & Workup: Testicular Choriocarcinoma
Treatment & Medication: Testicular Choriocarcinoma
Follow-up: Testicular Choriocarcinoma
Multimedia: Testicular Choriocarcinoma
References
Further Reading
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References

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  2. Ramon y Cajal S, Pinango L, Barat A. Metastatic pure choriocarcinoma of the testis in an elderly man. J Urol. Mar 1987;137(3):516-9. [Medline].

  3. Berney DM, Warren AY, Verma M, Kudahetti S, Robson JM, Williams MW, et al. Malignant germ cell tumours in the elderly: a histopathological review of 50 cases in men aged 60 years or over. Mod Pathol. Jan 2008;21(1):54-9. [Medline].

  4. Batata MA, Whitmore WF Jr, Chu FC. Cryptorchidism and testicular cancer. J Urol. Sep 1980;124(3):382-7. [Medline].

  5. Klein EA. Tumor markers in testis cancer. Urol Clin North Am. Feb 1993;20(1):67-73. [Medline].

  6. Horstman WG. Scrotal imaging. Urol Clin North Am. Aug 1997;24(3):653-71. [Medline].

  7. Vugrin D, Cvitkovic E, Posner J. Neurological complications of malignant germ cell tumors of testis: biology of brain metastases (I). Cancer. Dec 1979;44(6):2349-53. [Medline].

  8. Bredael JJ, Vugrin D, Whitmore WF Jr. Autopsy findings in 154 patients with germ cell tumors of the testis. Cancer. Aug 1 1982;50(3):548-51. [Medline].

  9. Beahrs O, Henson D, Hutter R. Handbook for staging of cancer. In: The Manual of Staging Cancer. 4th ed. Philadelphia, Pa: JB Lippincott; 1993:195-7.

  10. Prow DM. Germ cell tumors: staging, prognosis, and outcome. Semin Urol Oncol. May 1998;16(2):82-93. [Medline].

  11. Logothetis CJSamuels MLSelig DEOgden SDexeus FSwanson DJohnson Dvon Eschenbach A. Cyclic chemotherapy with cyclophosphamide, doxorubicin, and cisplatin plus vinblastine and bleomycin in advanced germinal tumors. Results with 100 patients. American Journal of Medicine. 2/1986;81:219-28. [Medline].

  12. Tatokoro M, Kawakami S, Sakura M, Kobayashi T, Kihara K, Akamatsu H. Successful management of life-threatening choriocarcinoma syndrome with rupture of pulmonary metastatic foci causing hemorrhagic shock. Int J Urol. Mar 2008;15(3):263-4. [Medline].

  13. Bodiwala D, Summerton DJ, Terry TR. Testicular prostheses: development and modern usage. Ann Royal Coll Surg Engl. 2007;89:349-53. [Medline]. [Full Text].

  14. Mead GM. Chemotherapeutic Management of Metastatic Germ Cell Testis Cancer. Risk-Adapted Therapy/Poor Risk Patients. In: Vogelzang et al, eds. Comprehensive Textbook of Genitourinary Oncology. 2nd ed. Philadelphia, Pa: Lippincott Williams & Williams; 2000:1024-31.

  15. Requena L, Sanchez M, Aguilar A. Choriocarcinoma of the testis metastatic to the skin. J Dermatol Surg Oncol. May 1991;17(5):466-70. [Medline].

  16. Saxman SB, Loehrer PJ. Chemotherapeutic Management of Metastatic Germ Cell Testicular Cancer. Overview of Initial Therapy for Metastatic Seminoma and Nonseminoma. In: Vogelzang et al, eds. Comprehensive Textbook of Genitourinary Oncology. 2000. 2nd ed. Philadelphia, Pa: Lippincott Williams & Williams; 1010-7.

  17. Batata MA, Chu FC, Hilaris BS. Therapy and prognosis of testicular carcinomas in relation to TNM classification. Int J Radiat Oncol Biol Phys. Aug 1982;8(8):1287-93. [Medline].

  18. Lepidini G, Biancari F, D'Andrea V. Severe thrombosis after chemotherapy for metastatic choriocarcinoma of the testis maintaining complete remission for a long period. Scand J Urol Nephrol. Apr 1997;31(2):221-2. [Medline].

  19. Bosl GJ, Geller N, Cirrincione C. Interrelationships of histopathology and other clinical variables in patients with germ cell tumors of the testis. Cancer. Jun 1 1983;51(11):2121-5. [Medline].

  20. Azzopardi JG, Mostofi FK, Theiss EA. Lesions of the testes observed in certain patients with widespread choriocarcinoma and related tumors. Am J Pathol. 1961;38:207-225.

  21. Fleming ID, Cooper JS, Henson DE, et al. AJCC Cancer Staging Manual. 5th ed. New York, NY: Lippincott-Raven; 1997.

  22. Kodama M, Murakami M, Kodama T. Chronological transition of the age-adjusted incidence rates (AAIRs) of 20 major neoplasias from early 1960s to mid-1980s. Anticancer Res. Jan-Feb 1999;19(1B):779-87. [Medline].

  23. Looijenga LH, Oosterhuis JW. Pathogenesis of testicular germ cell tumours. Rev Reprod. May 1999;4(2):90-100. [Medline].

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  25. Richie JP. Neoplasms of the Testis. In: Campbell's Urology. 7th ed. Philadelphia, Pa: WB Saunders Co; 1998.

  26. Ro JY, Dexeus FH, el-Naggar A. Testicular germ cell tumors. Clinically relevant pathologic findings. Pathol Annu. 1991;26 Pt 2:59-87. [Medline].

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Keywords

testicular choriocarcinoma, trophoblastic malignant teratoma, trophoblastic neoplasia, testicular seminoma, nonseminomatous germ cell tumors, NSGCT, germ cell tumors, GCT

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Contributor Information and Disclosures

Author

Michael B Williams, MD, MS, Fellow, Department of Urologic Oncology, MD Anderson Cancer Center
Disclosure: Nothing to disclose.

Coauthor(s)

Paul Schellhammer, MD, Chairman, Program Director, Professor, Department of Urology, Eastern Virginia Medical School
Paul Schellhammer, MD is a member of the following medical societies: American Urological Association
Disclosure: Nothing to disclose.

John W Davis, MD, Assistant Professor, Department of Urology, University of Texas MD Anderson Cancer Center
John W Davis, MD is a member of the following medical societies: American College of Surgeons and American Urological Association
Disclosure: Nothing to disclose.

Medical Editor

Leonard Gabriel Gomella, MD, FACS, The Bernard W Godwin Professor of Prostate Cancer Chairman, Department of Urology, Associate Director of Clinical Affairs, Kimmel Cancer Center, Thomas Jefferson University
Leonard Gabriel Gomella, MD, FACS is a member of the following medical societies: American Association for Cancer Research, American College of Surgeons, American Medical Association, American Society for Laser Medicine and Surgery, American Urological Association, Sigma Xi, Society for Basic Urologic Research, Society of University Urologists, and Society of Urologic Oncology
Disclosure: GSK Consulting fee Consulting; Astra Zeneca Honoraria Speaking and teaching; Watson Pharmaceuticals Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Dan Theodorescu, MD, PhD, Paul Mellon Professor of Urologic Oncology, Department of Urology, University of Virginia Health Sciences Center
Dan Theodorescu, MD, PhD is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Urological Association, Medical Society of Virginia, Society for Basic Urologic Research, and Society of Urologic Oncology
Disclosure: Nothing to disclose.

CME Editor

J Stuart Wolf Jr, MD, FACS, David A Bloom Professor of Urology, Director of Division of Minimally Invasive Urology, Department of Urology, University of Michigan
J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology
Disclosure: Terumo Corporation Consulting fee Consulting; Gyrus-ACMI Honoraria Speaking and teaching

Chief Editor

Bradley Fields Schwartz, DO, FACS, Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine
Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists
Disclosure: Nothing to disclose.

 
 
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