Retroperitoneal germ cell tumor with testicular calcification indicating tiny testicular origin: consideration of the origin of retroperitoneal germ cell tumors: report of two cases

Two cases of germ cell neoplasm retrospectively considered to have been of testicular origin are reported. Case 1. A 19-year-old male with brain, liver and retroperitoneal tumors was diagnosed with yolk sac tumor by retroperitoneal tumor biopsy. After multidisciplinary treatment, a region of calcification was detected in the left testis on scrotal sonography and left high inguinal orchiectomy was performed. Case 2. A 57-year-old male with neck, lung and retroperitoneal tumors was diagnosed with yolk sac tumor by supraclavicular biopsy. From initial examination, scrotal sonography revealed a small calcified lesion in the right testis. After chemotherapy, high inguinal orchiectomy and retroperitoneal lymphadenectomy were simultaneously performed. Pathologic evaluation of these testicular specimens revealed calcification and a fibrous scar in correspondence with the clinical diagnosis. These changes were considered as scars of the primary testicular tumor due to burned-out tumor or the result of reaction to chemotherapy. Since a primary tumor of testicular origin may exist in the extragonadal germ cell tumor, it is important to examine the intrascrotal contents in detail in the case of so-called extragonadal germ cell tumors with palpably normal testes. In such cases, there are two possible conditions, an occult testicular tumor and a burned-out testicular tumor. We briefly reviewed 42 such cases in the Japanese literature. It appears that there are very few true extragonadal germ cell tumors, and that the possibility of primary testicular origin metastasizing from viable occult testicular tumor or burned-out testicular tumor with spontaneous regression is high in retroperitoneal germ cell tumors.     

Testicular seminoma occurring 8 years after treatment of a metastatic extragonadal germ cell tumor

A 30-year-old man was admitted with a chief complaint of left-sided scrotal enlargement, and was diagnosed as having testicular seminoma after orchiectomy. Eight years earlier, he had been treated with chemotherapy for an extragonadal germ cell tumor, without orchiectomy, leading to complete remission. His histological diagnosis at that time was a germ cell tumor, composed of choriocarcinoma and embryonal carcinoma. He was followed up without testicular biopsy. Routine pretreatment testicular biopsy in patients with extragonadal germ cell tumor is controversial, but regular long-term follow up and information on the risk of developing a metachronous testicular tumor are needed after treatment of extragonadal germ cell tumors, even when there seems to be a partial or complete clinical response.     

Extragonadal retroperitoneal germ cell tumors without apparent testicular involvement A search for the source

Extratesticular germ cell neoplasms without apparent testicular primary tumors are rare. The origin of these neoplasms has been debated in the literature for decades. With the advent of effective chemotherapy for extratesticular germ cell neoplasms, the origin of these tumors becomes more than academic. We report on 3 cases of retroperitoneal germ cell neoplasms with microscopic intratesticular primary tumors. All patients with extragonadal germinal cell tumors of the testes should undergo thorough investigation for an occult testicular primary tumor despite a normal testis examination. We review previously reported cases of extratesticular germ cell neoplasms without an apparent testicular primary tumor.     

Burn out bilateral testicular tumor

Differentiating a primary retroperitoneal seminoma from a metastatic testicular tumor with an occult testicular primary or a burned out testicular cancer remains difficult. We present a case of a burned out tumor. The patient had a retroperitoneal seminoma with ultrasonically and pathologically demonstrated abnormalities in both testes, but without evidence of tumor. The patient received chemotherapy and underwent surgery of the residual retroperitoneal mass and bilateral orchiectomy. All surgical specimens were negative for testis cancer. CONCLUSION: Primary extragonadal germ cell tumors in the retroperitoneum are a rare entity. The presence of a retroperitoneal tumor with ultrasonographical abnormalities in testicular evaluation should be considered as a metastases of a burned out testicular cancer, and biopsy is mandatory. Surgical evaluation and orchiectomy should be evaluated in a individual setting.     

Retroperitoneal germ cell tumors with high level of serum and urine hCG in an adult: a case report

A case of retroperitoneal germ cell tumors in a 32-year-old male is reported. He was admitted to our hospital with complaints of lumbago and abdominal mass. He was diagnosed with primary retroperitoneal malignant tumor and underwent resection of the tumor with retroperitoneal lymphadenectomy. The final pathological diagnosis revealed germ cell tumors of more than one histological type in the retroperitoneal space. Serum human chorionic gonadotropin (hCG) and hCG-beta levels were very high preoperatively (4,8000 mIU/ml, 57 ng/ml, respectively) and they decreased to normal after vinblastine-bleomycin cisplatin combination chemotherapy three times postoperatively. The patient later developed a solitary lung metastasis and died in another hospital 7 months after the operation. Retroperitoneal germ cell tumors in adults are rare. One question continually arises: are they extragonadal occurrences or germ cell tumors or do they represent metastatic lesions from occult testicular tumors?     

Testicular seminoma after the complete remission of extragonadal yolk sac tumor : a case report

Background

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.

Case presentation

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.

Conclusions

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.

     

Primary or secondary extragonadal germ cell tumors?

We reviewed 16 patients treated for primary extragonadal germ cell tumors whose testes were initially negative for cancer at palpation. Residues compatible with an occult testicular primary, overlooked at the pretreatment examination, were found in 10 of 12 patients with retroperitoneal germ cell tumors, whereas the testes in all 4 patients with mediastinal germ cell tumors showed no pathological signs. Therefore, we conclude that mere palpation to exclude a testicular primary is not sufficient and the testes of patients with so-called extragonadal germ cell tumors should be examined by all available means, at least by high frequency ultrasound. Orchiectomy is advisable if a focal lesion is found.     

Primary nonseminomatous germ cell tumor of the prostate

We describe a case of a primary nonseminomatous germ cell prostatic tumor and discuss the problem of extragonadal germ cell tumors. Prognosis, staging and management of these tumors are similar to that of metastatic primary testicular germ cell tumors.     

The problems in making a diagnosis and arriving at a therapeutic procedure in a patient with retroperitoneal volk sage tumor. Case report and review of the literature

Summary The case of a 26 years old patient with a primary paraaortic extragonadal yolk sac tumor is presented. After operative reduction of the tumor polychemotherapy with PEB was performed. Following chemotherapy testicular biopsy did not show histological changes typical for testicular intraepithelial neoplasia (TIN). Three years after the initial manifestation of the yolk sac tumor, a malignant tumor of the testis with components of undifferentiated teratoma and yolk sac tumor was observed. In this paper the problems of development of testicular malignancy after polychemotherapy and the validity of a small exploratory excision of testicular tissue in retroperitoneal germ cell tumors and following chemotherapy are discussed.      

A case of retroperitoneal bulky tumor with cryptorchidism

A 37-year-old male was presented with an upper abdominal mass. A retroperitoneal bulky tumor was detected and operative biopsy revealed seminoma. The right testis was undescended and atrophic which was located at the left inguinal lesion. Pathologically, the primary tumor cells were not found in the left orchiectomied testis, but only scar and fibrosis were compatible with a burned-out testicular tumor. This patient showed partial regression by multidisciplinary therapy including combined chemotherapy with CDDP, etoposide and peplomycin, and is still on the same condition and alive for 3 years. In case of extragonadal germ cell tumor without apparent testicular involvement, it is a problem to differentiate primary extragonadal germ cell tumor from occult and burned-out testicular tumor. Testis should be examined by all means available including careful exploration.     

Association of intratubular seminoma and intratubular embryonal carcinoma with invasive testicular germ cell tumors

The classification of intratubular germ cell neoplasia of the testis includes an unclassified type (IGCNU), in addition to various other intratubular lesions that show specific forms of differentiation, such as intratubular seminoma and intratubular embryonal carcinoma. Although IGCNU is recognized as a precursor lesion for testicular germ cell tumors, the relationship between differentiated types of intratubular germ cell neoplasia and invasive germ cell tumors of the testis is not well established. The aim of the present study was to examine the association between invasive testicular germ cell tumors and intratubular neoplastic lesions, with particular emphasis on differentiated types of intratubular germ cell neoplasia. The seminiferous tubules adjacent to 42 testicular germ cell tumors were evaluated for the presence of various forms of intratubular germ cell neoplasia. IGCNU was observed in 37 (88%) of 42 cases, whereas intratubular seminoma and intratubular embryonal carcinoma were seen in 19% and 7% of the cases, respectively. Intratubular seminoma was associated primarily with seminomas or mixed germ cell tumors with a seminomatous component, but was also present in a case of a nonseminomatous germ cell tumor. Intratubular embryonal carcinoma was associated exclusively with nonseminomatous germ cell tumors. All cases of intratubular embryonal carcinoma were identified morphologically and exhibited histologic features corresponding to traditional definitions of this lesion. No examples of intratubular embryonal carcinoma as defined by CD30 expression alone in the absence of an intratubular proliferation were observed. The presence of intratubular seminoma in a nonseminomatous germ cell tumor suggests that it is a true preinvasive lesion rather than a manifestation of intratubular spread of an established invasive seminoma. The low incidence of intratubular embryonal carcinoma supports the theory that most nonseminomatous germ cell tumors evolve initially as seminomas, rather than directly from a differentiated intratubular neoplastic lesion.     

Primary testicular seminoma in a patient with a history of extragonadal non-seminomatous germ cell carcinoma

Extragonadal germ cell carcinoma represents between 3% and 5% of all germ cell carcinomas. A metachronous primary germ cell carcinoma is exceedingly rare in these patients. We report the eighth case, which occurred in a 29-year-old man who presented with testicular seminoma 7 years after his initial presentation with extragonadal non-seminomatous germ cell carcinoma. The seven other patients also presented with extragonadal non-seminomatous germ cell carcinoma, followed subsequently by testicular seminoma in 6 patients and non-seminomatous germ cell carcinoma in the seventh. The mean time to presentation was 8 years. Although rare, this case emphasizes the need for long-term surveillance, including testicular evaluation of patients with a history of extragonadal germ cell carcinoma.     

Histology in mixed germ cell tumors. Is there a favorite pairing?

PURPOSE: Mixed germ cell tumors account for approximately 30% to 50% of testicular tumors. To our knowledge a systematic review with statistical analysis of the associations of histological subtypes in mixed germ cell tumors has not been done previously. It was our impression that such associations exist. Delineating concordant histological types may provide insight into the ontogeny of testicular tumors and also have important clinical implications. MATERIALS AND METHODS: We retrospectively reviewed the testis cancer data base at our institution. The primary tumor of orchiectomy specimens was examined in 2589 patients. Of these patients mixed histology was noted in 1765 (68.2%). ORs were calculated for all possible combinations of teratoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma and seminoma. In addition, we evaluated the association of various histological types with teratoma at post-chemotherapy retroperitoneal lymph node dissection. RESULTS: Of 10 possible combinations of histological types in the primary tumor, positive correlations were noted in 4. The strongest correlation was found between teratoma and yolk sac tumor (OR 2.58, p <0.001). Teratoma or yolk sac tumor in the testis was associated with teratoma in the pathology specimen at post-chemotherapy retroperitoneal lymph node dissection. CONCLUSIONS: The strongest associations of histological subtypes in mixed germ cell tumors were seen between yolk sac tumor and teratoma. Similar associations are seen in late relapse and in some cases of prepubertal tumors. Further study of these associations may prove valuable in understanding the biology and clinical behavior of germ cell tumors.     

Testicular recurrence from "primary" retroperitoneal germ cell tumor

Extragonadal germ cell tumors are a rare clinical entity. We present the case of an extragonadal germ cell tumor followed by testicular recurrence more than 4 years later. This presents an interesting dilemma as to whether this was recurrence of the original tumor or a new, primary tumor. The patient's outcome and surveillance protocol for these tumors are presented.     

A case of extragonadal germ cell tumor with inferior vena caval tumor thrombus]

We report a case of retroperitoneal extragonadal germ cell tumor with tumor thrombus in the inferior vena cava. The patient referred to our hospital with lumbago. Computed tomography (CT) showed a bulky mass in the retroperitoneum. The levels of alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (beta-HCG) in the serum were elevated. Histological examinations indicated embryonal cell carcinoma. Bilateral testicles did not contain any palpable mass upon careful palpation. No tumor mass was detected in the bilateral testicles on ultrasonography. Clinically, the diagnosis was a retroperitoneal extragonadal germ cell tumor associated with para-aortic lymph-node involvement. After the combination chemotherapy (BEP 1 course and EP 3 courses), the tumor mass was reduced in size and the tumor marker was normalized. Retroperitoneal lymph node dissection (RPLND) was performed and tumor thrombus in the inferior vena cava was resected. There was no involvement of the viable cells in the resected tumor. The patient has been in good condition with no evidence of disease.     

A case of bilateral testicular tumor

A case of bilateral germ cell tumor of the testis is reported. In 1988, a 36-year-old male presented with painless swelling of left scrotal contents. Right orchiectomy and retroperitoneal lymph node dissection for embryonal carcinoma had been performed 5 years earlier. Left orchiectomy was performed and its histological finding was seminoma and embryonal carcinoma. Evaluations including CT scan and Ga scintigraphy revealed no metastasis. Postoperatively, the patient was treated with PVB therapy. Previous reports of bilateral germ cell testicular tumor were reviewed, and the age distribution, interval, and histological classification of these cases are discussed.     

Extragonadal germ cell tumor originating in iliac fossa

Extragonadal germ cell tumors usually originate in midline body structures. We describe the first case of an extragonadal germ cell tumor that arose atypically in the right iliac fossa, a nonmidline pelvic region. Based on this case report and a review of the literature, we suggest a modification of Abell's criteria for the diagnosis of retroperitoneal extragonadal germ cell tumors.     

A presumed extragonadal germ cell tumor that turned out to be a gastric cancer—a case report

A solely retroperitoneal mass in males in combination with elevated serum Alpha-Fetoprotein (AFP) and beta-human choriogonadotropin (β-HCG) levels is highly indicative of a metastatic testicular cancer. Although testicular cancers are rare, they represent the most common diagnosed cancer in males between 14 and 40 years. However, in cases without evidence of a primary testicular tumor, the rare diagnosis of a retroperitoneal extragonadal germ cell tumor (EGCT) must be assumed. Here, we describe the first published case of a 66-year-old man presenting with this typical clinical picture and the diagnosis of an AFP and β-HCG producing advanced gastric cancer with retroperitoneal lymph node metastases mimicking a primary retroperitoneal EGCT. The final diagnosis was only made by gastroscopy performed after a CT-guided retroperitoneal lymph node biopsy revealed an adenocarcinoma, suggesting an upper gastrointestinal tract primary origin. However, a specific initial anamnesis and also in the primary staging, including a full-body CT-scan there was no hint for another primary tumor. Only the slightly unusual extension of the retroperitoneal mass up to the ligamentum hepatoduodenale and the pylorus, as well as the atypical age made us question our initial diagnosis. This extraordinary case is of special clinical interest to all practising physicians and once again highlights the importance of keeping rare differential diagnosis such as AFP-producing gastrointestinal tumors in mind.     

Testicular germ cell tumor seven years after a retroperitoneal germ cell tumor.

A 44-year-old male was diagnosed in August 1980 as having a retroperitoneal germ cell tumor (classic seminoma with anaplastic areas). After treatment with cisplatin-based chemotherapy, he reached complete clinical and pathological remission. Eighty-eight months later, in December 1987, he was diagnosed as having a testicular mixed germ cell tumor (embryonal carcinoma with anaplastic seminoma areas) after right orchiectomy. The potential mechanisms by which the latter tumor could have developed are discussed.