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 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.

     

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.     

Extragonadal germ cell tumor: the preoperative urological evaluation

Primary extragonadal germ cell tumors were diagnosed in 4 patients in whom surgical or postmortem examination of the testis failed to reveal germ cell tumor. A fifth patient underwent serial examination with testicular ultrasound without evidence of any abnormalities and he remains free of disease following chemotherapy. Based on our experience and a literature review, it is our opinion that orchiectomy is not indicated unless there is a palpable testicular abnormality, recent change in testicular size or consistency, abnormal testicular ultrasound or history of cryptorchidism, or if the primary tumor is choriocarcinoma.     

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.     

Testis sparing surgery for the treatment of a sequential bilateral testicular germ cell tumor

Standard therapy of sequential bilateral testis cancer is generally considered to be orchiectomy. We present a case of sequential bilateral testicular germ cell tumor treated with testis sparing surgery. The patient was disease free 50 months after surgery without local recurrence or distant metastases. Testis sparing surgery provides a better quality of life and may be considered a safe, feasible alternative in the treatment of carefully selected patients with bilateral testicular germ cell tumor.     

Testis-sparing surgery in an adult with bilateral synchronous seminomatous tumor

Bilateral synchronous testicular cancer is a rare occurrence and is usually associated with similar histological findings in each testicle. The standard therapy of bilateral testis cancer is generally considered to be orchiectomy. We present a case of synchronous bilateral testicular germ cell tumor treated with testis-sparing surgery. The patient was disease free for 30 months after surgery without local recurrence or distant metastases. Testis-sparing surgery provides a better quality of life and may be considered a safe, feasible alternative in the treatment of carefully selected patients with bilateral testicular germ cell tumor.     

睾丸生殖细胞肿瘤176例临床诊治分析

目的:探讨睾丸生殖细胞肿瘤的诊断、治疗及预后情况。方法:对四川大学华西医院泌尿外科1980年10月～2000年10月收住入院的睾丸生殖细胞肿瘤患者的临床病历及随访资料进行回顾性分析。结果:精原细胞瘤111例、非精原细胞瘤43例、混合性生殖细胞瘤22例,合并隐睾46例,睾丸生殖细胞肿瘤的临床分期、治疗方式以及有无隐睾都是影响睾丸生殖细胞肿瘤预后的重要因素。结论:对精原细胞瘤Ⅰ期患者单纯手术与手术+放疗疗效相当,Ⅰ期精原细胞瘤可以单纯手术治疗为主;Ⅱ期和Ⅲ期精原细胞瘤和其他类型的生殖细胞肿瘤首选治疗方式为根治性睾丸切除术辅以放射治疗;早期诊断、早期合理治疗对睾丸生殖细胞肿瘤的预后有重要意义。     

Metachronous testicular tumor developing eight years after retroperitoneal extragonadal germ cell tumor

Abstract:   A 30 year-old man was admitted to our hospital with a chief complaint of left flank pain. Computed tomography revealed a retroperitoneal tumor. Levels of lactic dehydrogenase, human β-chorionic gonadotropin, α-fetoprotein, DUPAN-2 and carbohydrate antigen 19–9 were elevated. No abnormal findings were present according to palpation and ultrasonography of the testes. The patient was diagnosed as having a retroperitoneal extragonadal germ cell tumor (EGCT) considering the elevated markers. Resection of the tumor, two cycles of neoadjuvant and one cycle of adjuvant chemotherapy (cisplatin and etoposide) were performed. The surgical specimen showed total necrotic tissue. Eight years later, the patient noticed an enlargement of his left testicle. The tumor felt elastic and firm on his left testis. Neither distant metastasis nor lymph node metastasis was present according to computed tomography. Left high orchiectomy was performed and histology revealed seminoma. Twenty-three cases have been reported previously about metachronous testicular tumor developing after retroperitoneal EGCT. We report the 24th case.     

Serum lactate dehydrogenase and its isoenzymes in men with maldescended testes

Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were determined as a screen for testicular germ cell neoplasia in 130 men with maldescended testes. A testicular tumor was found on clinical examination in 1 patient, which was revealed to be embryonal carcinoma, teratoma, yolk sac tumor and carcinoma in situ on orchiectomy. Subclinical testicular germ cell neoplasia was found on testicular biopsy in 3 men (1 with microinvasive seminoma and 2 with carcinoma in situ). These 4 patients had normal serum lactate dehydrogenase and serum lactate dehydrogenase isoenzymes. Elevated serum lactate dehydrogenase was noted in 3 men without testicular germ cell neoplasia: 1 had predominantly increased serum lactate dehydrogenase isoenzymes 1 to 3 and 2 had slightly increased serum lactate dehydrogenase isoenzymes 3 and 4. Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were not sensitive to detect testicular germ cell tumors in a subclinical stage.     

Case of gonadoblastoma in a 9-year-old boy without physical abnormalities

BACKGROUND: A 9-year-old boy was admitted to Jikei University Hospital complaining of gradual enlarging of the left scrotal contents. METHODS/RESULTS: Physical examination was significant for bilateral descended testicles. No abnormalities were detected in the testicles or along the spermatic cords. Scrotal ultrasound showed that hyperechoic shadows were recognized in the central area of the left testicle. Subsequent testicular biopsy and histopathological examination showed intratubular malignant germ cells in the testicular tubules. One week later, left orchiectomy was performed. CONCLUSIONS: Histopathological evaluation revealed gonadoblastoma. Gonadoblastoma, a rare gonadal neoplasm, is composed of germ cells and sex cord derivatives and usually occurs in phenotypically female patients with gonadal dysgenesis. To date, only three cases of gonadoblastoma have been reported in anatomically normal male patients with scrotal testicles. We report on a case of gonadoblastoma unaccompanied by a germ cell tumor in a physically normal male.     

Recurrent rhabdomyosarcoma after adjuvant chemotherapy for stage I non‐seminomatous germ cell tumor with malignant transformation

We report a rare case of stage I non‐seminomatous testicular germ cell tumor with malignant transformation. The patient received two cycles of chemotherapy (cisplatin, bleomycin and etoposide) tailored to testicular germ cell tumors as an adjuvant therapy after orchiectomy. However, 22 months later, the patient developed a metastasis in the occipital region that consisted of solely rhabdomyosarcoma through malignant transformation of a teratoma component. This case highlights an issue related to adjuvant chemotherapy for testicular germ cell tumors with components of malignant transformation.     

Extragonadal germ cell tumor of the prostate associated with Klinefelter's syndrome

PURPOSE: We report on a case of extragonadal germ cell tumor of the prostate associated with Klinefelter's syndrome. METHODS/RESULTS: The patient was a 33-year-old man. A transrectal prostate biopsy suggested combined germ cell tumor (yolk sac tumor + teratoma). Because there was no tumor except from the prostate, we considered this case to be a primary extragonadal germ cell tumor of the prostate. The prostate tumor responded to systemic chemotherapy with cisplatin, vinblastine and bleomycin and elevated lactate dehydrogenase and alpha-fetoprotein levels normalized. In addition to chemotherapy, the patient also underwent radiation therapy. CONCLUSION: The patient has survived for approximately 4 years since the diagnosis.     

Intra-abdominal desmoid tumor following retroperitoneal lymph node dissection for testicular germ cell tumor

In the testicular cancer post-treatment setting a rapidly growing retroperitoneal mass leads to a differential diagnosis including recurrent germ cell tumor, residual mature teratoma, or sarcomatoid degeneration. We report the case of a 27-year-old man with a large abdominal mass occurring in the setting of a mixed germ cell tumor after radical orchiectomy with primary chemotherapy followed by retroperitoneal lymph node dissection. Surgical excision of this mass followed by pathological review revealed an intra-abdominal desmoid tumor. Fluorescence in situ hybridization (FISH) for isochromosome 12p failed to demonstrate a germ cell tumor origin. This is the fourth such case of an intra-abdominal desmoid tumor after retroperitoneal lymph node dissection for testicular cancer in the urologic literature. This case highlights the need for careful consideration of a desmoid tumor when a rapidly growing spindle cell tumor is encountered in a post-treatment testis cancer patient.     

Conservative management of small testicular tumors relative to carcinoma in situ prevalence

PURPOSE: We evaluated the prevalence of carcinoma in situ (CIS) in orchiectomy specimens performed for germ cell tumors smaller than 40 mm in diameter to propose an appropriate conservative approach to bilateral tumors or tumor of a solitary testis. MATERIALS AND METHODS: Of 127 patients treated with orchiectomy between 1990 and 2002, 41 who presented with a tumor of less than 40 mm in diameter were selected for histological analysis of testicular parenchyma. The morphological items assessed were CIS, spermatogenesis and Leydig cell hyperplasia. RESULTS: CIS was observed in 39 of the 41 patients (95%). CIS was evenly distributed throughout the testicular parenchyma (ie around and beyond the tumor) in all 39 cases. Spermatogenesis was observed in 12 of 41 specimens (29%), spermatogenesis without spermatozoa was noted in 14 (34%) and absent germ cells were found in 15 (37%). Leydig cell hyperplasia was observed in 24 cases (58%). CONCLUSIONS: Histological analysis of whole orchiectomy specimens showed that CIS is almost always present in testicular parenchyma adjacent to germ cell tumor. In bilateral testis cancer or cancer occurring in a solitary testis tumorectomy plus radiotherapy appears to be the appropriate treatment in patients with a small tumor and no other risk factors. In patients who wish to father a child and have preserved spermatogenesis the natural history of CIS allows the postponement of testicular radiotherapy after orchiectomy, giving the double advantage of preserving testicular endocrine function and maintaining the possibility of natural fatherhood.     

A case of intratubular germ cell tumor giving rise to seminoma in a subfertile man

A 34-year-old man visited our hospital with the complaint of right scrotal swelling. Right high orchiectomy was performed under the diagnosis of testicular tumor. Pathological examination was seminoma, pT2pN0pM0 and 3 courses of chemotherapy (cisplatin, vinblastine, bleomycin) were performed. The patient had undergone testicular biopsy for infertility at another hospital 6 years before this visit. Re-examination of the biopsy specimen revealed a intratubular germ cell tumor. No evidence of recurrence or metastasis was found 2 years after surgery.     

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.     

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.     

Case of mediastinal seminoma with testicular microlithiasis

Testicular microlithiasis is a rare condition in which calcified concretions fill the lumina of the seminiferous tubules. We report the case of a 19-year-old Japanese man with mediastinal seminoma, normal testicular physical findings and bilateral testicular microlithiasis seen on ultrasonography. Testicular needle biopsy demonstrated multiple laminated calcifications within the seminiferous tubules without any signals of a viable germ cell tumor. To our knowledge, this is only the sixth reported case of extragonadal germ cell tumor with testicular microlithiasis.