Testicular Choriocarcinoma

Updated: Sep 07, 2019
  • Author: Michael B Williams, MD, MS; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Overview

Practice Essentials

Testicular choriocarcinoma is one of the histologic types of nonseminomatous germ cell tumors (NSGCTs), which along with testicular seminoma constitute the two major histologic groups of testicular cancers. Pure choriocarcinoma of the testis is the exception to most of the rules established for testicular seminoma and all other forms of NSGCTs.

Like other germ cell tumors (GCTs), choriocarcinoma typically affects younger men. Unlike other such 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 a high mortality rate. Surgery is usually limited to radical orchiectomy for tissue diagnosis. [1]  Chemotherapy for disseminated disease is principally with cisplatin-based regimens, particularly combination chemotherapy with bleomycin, etoposide, and cisplatin. [2]  

For patient education information, see Men's Health, as well as Testicular Cancer and Testicular Self-Exam.

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Pathophysiology and Etiology

Choriocarcinoma recapitulates placental tissue development. For unknown reasons, it metastasizes early via hematogenous routes to the lung, liver, and brain, among others. [1, 3]

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 cancer in a testis that was 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. [5]

Other risks include the following:

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Epidemiology

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 males 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. Subtypes and frequencies of GCTs include the following:

  • Seminoma (40%)
  • Embryonal tumor (25%)
  • Teratocarcinoma (25%)
  • Teratoma (5%),
  • 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. In a histologic review of 1010 orchiectomies from 1999 to 2011 from a single Mexican oncology institution, 0.6% were pure choriocarcinomas and 0.9% were mixed germ cell tumors with a predominant choriocarcinoma component. [6]

Age

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. Most patients were aged 20-30 years; the oldest patient with a pure choriocarcinoma was 63 years old. [7]  The patient died of aspiration shortly after initiation of chemotherapy, so a determination of treatment efficacy in this age group was impossible. In a 2008 review of GCTs in 50 men older than 60 years, only one was found to have a component of choriocarcinoma. [8]

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Prognosis

In most reports, choriocarcinoma carries a dismal prognosis due to its early hematogenous spread. 

Batata et al (1980) reported a 5-year survival rate of 0% (0 of 20 patients) [9]

Requena et al (1991) reported a case of pure choriocarcinoma with metastases to the skin (rare), lung, and brain; this patient was treated with a 4000-rad dose to the skull and a multi-agent chemotherapy regimen, including platinum, vinblastine, and bleomycin (PVB) and lomustine, VP-16, and VePesid; the patient's beta-hCG level normalized, and he was disease-free at 2 years [10]

Lepidini et al (1997) reported a patient treated with multi-agent chemotherapy who was disease-free at 43 months of follow-up [11]

In five cases of pure choriocarcinoma with brain metastases, all patients died, and median survival was 1 month despite treatment with multi-agent chemotherapy [12]

In a 9-year review of patients treated in multi-agent chemotherapy trials at Memorial Sloan-Kettering Cancer Center, Bosl et al (1983) reported five cases of pure choriocarcinoma and two long-term survivors [13]

A review of survival after a diagnosis of testicular germ cell cancers in Germany and the United States from 2002-2006 found that 5-year relative survival was lowest with choriocarcinomas: 80.1% in Germany and 79.6% in the US; this compared with survival rates of 93.3% and 91.0% with nonseminomas generally [14]

A review by Alvarado-Cabrero et al (2014) found that of six patients with pure testicular choriocarcinoma, all died of their disease after a median of 9.5 months; of eight patients with predominant choriocarcinoma, five died of the disease after a median of 27 months, one was alive with disease, and two were alive with no evidence of disease at 60 and 72 months of follow-up, respectively; the latter two patients were the only ones with M1a disease on presentation [6]

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