Testicular seminoma after the complete remission of extragonadal yolk sac tumor : a case report
© Kuroda et al; licensee BioMed Central Ltd. 2004
Received: 31 May 2004
Accepted: 16 November 2004
Published: 16 November 2004
Between 2% and 5% of malignant germ-cell tumors in men arise at extragonadal sites. Of extragonadal germ cell tumors, testicular carcinoma in situ (CIS) are present in 31–42% of cases, and CIS are reported to have low sensitivity to chemotherapy in spite of the various morphology and to have a high likelihood of developing into testicular tumors. A testicular biopsy may thus be highly advisable when evaluating an extragonadal germ cell tumor.
A 36-year-old man was diagnosed as having an extragonadal non-seminomatous germ cell tumor, that was treated by cisplatin-based chemotherapy, leading to a complete remission. In the meantime, testicular tumors were not detected by means of ultrasonography. About 4 years later, a right testicular tumor was found, and orchiectomy was carried out. Microscopically, the tumor was composed of seminoma.
We herein report a case of metachronous occurrence of an extragonadal and gonadal germ cell tumor. In the evaluation of an extragonadal germ cell tumor, a histological examination should be included since ultrasonography is not sufficient to detect CIS or minute lesions of the testis.
Between 2% and 5% of malignant germ-cell tumors in men arise at extragonadal sites . Cytogenetically most extragonadal germ-cell tumors (EGGCTs) i.e., the seminomas and non-seminomas, are similar to their testicular counterparts [2, 3]. But there are CIS in 31–42% of EGGCT patients' testes . Ultrastructural studies indicate that CIS originate from rather primitive cells and can develop into different categories of germ cell carcinomas. Furthermore, since CIS are reported to respond poorly to chemotherapies, a metachronous development of testicular cancer will possibly occur, in spite of the various morphologies of testicular cancer [5, 6]. In the present case, the etiology of a metachronous appearance of EGGCT and testicular cancer is discussed.
There are reports that approximately 4% of patients with EGGCT develop a metachronous testicular cancer despite the use of cisplatin-based chemotherapy , and the cumulative risk of developing a metachronous testicular cancer 10 years after a diagnosis of EGGCT is 10.3% . However, there is disagreement over whether EGGCT is a primary disease or metastatic from the burned-out primary testicular lesion. Actually, burned-out tumors have been detected in 76% of cases of EGGCT . CIS is also found via biopsy in 31–42% cases [4, 10]. Testicular CIS is thought to have resistance to systemic chemotherapies and to develop later to metachronous testicular cancer. In the present case, the EGGCT was a non-seminomatous germ cell tumor including a yolk sac component, whereas the testicular cancer was a seminoma. We believe CIS was present at the time of the treatment of EGGCT and testicular CIS is so primitive that it could differentiate into any type of germ cells. It is also possible that these metachronously developing germ cell tumors developed independently. A testicular biopsy could clarify the relationship between these tumor cells and the expansion of the disease. In the present case no biopsy was done, but an ultrasonic examination ruled out the possibility of testicular CIS. Giwercman et al.  emphasized the necessity of histological examinations of the testis upon an evaluation of EGGCT [12–14] and also urged a careful follow-up for patients with EGGCT who do not have simultaneous testicular cancer.
On the other hand, there is an opinion that any patients with retroperitoneal masses should undergo scrotal ultrasound. Comiter et al.  showed definite pathological evidence of a burned-out testicular carcinoma in 5 of 6 patients (83%) with presumed extragonadal germ cell tumors and concluded that scrotal ultrasound studies are useful for the evaluation of the palpably normal testes . Kitahara et al. reviewed the incidence of scrotal echogenic leisions with testicular cancers or burned-out tumors of 22 EGGCT patients and found echogenic changes in 17 patients (77.3%) . This means that disease was overlooked by ultrasonic examinations in 22.7% of cases.
In our case, it is possibile that metachronous testicular cancer oriented in testicular CIS, grew from a burned-out tumor, or was independent of the EGGCT. We should have performed testicular biopsies at the time of the diagnosis of EGGCT and reflect the strategies of treatments of EGGCT.
Our proposed surveillance protocol of EGGCT is as below.
At the time of the diagnosis
Self-check of the testis
Scrotal ultrasound study
every 3 months
every 6 months
every 3 months
every 6 months
every 3 months
every 6 months
As we mentioned, the overall risk of development a testicular tumor is not so high(4–10.3%).
The side effect of CIS therapy (whether irradaition, orchiectomy or chemotherapy) are significant, especially concerning fertility and androgen production.
Testicular tumors early detected by adequate surveillance respond well to treatments.
Testicular biopsy is not entitled to detect all the CIS.
We thank the patient for giving consent for publication of this case report.
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