Retroperitoneal Extragonadal Germ Cell Tumors Research Articles

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.

Retroperitoneal germ cell tumor mimicking neoplasm of ampulla of Vater

A 20-year-old man previously healthy, presented to the emergency department for evaluation of obstructive jaundice, backache and weight loss. Abdominal ultrasonography demonstrated a dilated common bile duct (up to 2 cm), increased gallbladder dimensions (10.3 x 4.9 cm) with biliary sludge. Computed tomography of the abdomen showed a retroperitoneal tumor with 18 x 14 cm dimensions, and numerous large retroperitoneal lymph nodes. The liver demonstrated a bilateral intrahepatic bile duct dilatation. An endoscopic retrograde cholangiography was performed. The study revealed an ulcerative neoplasm of ampulla of Vater, and the cholangiogram obtained images of dilated extra-hepatic and intra-hepatic bile ducts with a large stenosis in the distal third of the common bile duct. After biopsies were analyzed, the definite diagnosis was a retroperitoneal extragonadal germ cell tumor (subtype: embryonal carcinoma) with infiltrative invasion to duodenal wall. A testicular ultrasonography ruled out a testicular tumor. Endoscopic stenting was necessary for decompress the unresectable malignant bile duct obstruction. Patient was referred for cisplatinum based chemotherapy.

Clinical outcome of extragonadal germ cell tumors: Retrospective analysis at a single institution in Japan.

345 Background: Extragonadal germ cell tumors (EGGCT) are very rare and account for only 2% to 5% of all malignant germ cell neoplasms. Although multimodality treatment, including cisplatin-based chemotherapy and postchemotherapy surgery, has improved the prognosis of patients with EGGCT, few findings are available for these tumors. We therefore performed a retrospective analysis of Japanese patients with EGGCT. Methods: We performed a retrospective review of the medical records of 34 male patients ranging in age from 18 to 62 years (median age, 30 years) treated at our institution between 1982 and 2010. Fifteen patients (44%) had primary mediastinal EGGCT, 16 patients (47%) had primary retroperitoneal EGGCT, and three patients (9%) had primary mediastinal and retroperitoneal EGGCT. Results: Twenty-six patients (76%) had nonseminomatous EGGCT, and eight patients (24%) had seminomatous histology. Surgical procedures were performed in five patients (15%) as induction treatment. Three of them had seminomatous histology and were continuously disease-free after adjuvant chemotherapy. Twenty-nine patients (85%) had received cisplatin-containing regimen as induction therapy. Twenty-three patients underwent post–chemotherapy surgery, and 14 of them (60%) had residual viable malignant cells. On univariate analysis, nonseminomatous EGGCT (P<0.01) and resistance to cisplatin (lack of achievement of CR/PRm- by induction chemotherapy; P<0.01) were identified as negative prognostic factors for survival. Twenty of 34 patients (59%) were alive without disease, and 13 patients with nonseminomatous histology died of disease progression. The overall 10-year survival rate for all patients was 53%. Conclusions: Patients with seminomatous EGGCT have a very good prognosis, while nonseminomatous histology and resistance to cisplatin were found to be negative prognostic factors.

The incidence and management of metachronous testicular germ cell tumors in patients with extragonadal germ cell tumors

ObjectivesThe optimal management of extragonadal germ cell tumor (EGGCT) and metachronous testicular germ cell tumor (MTGCT) has not been determined. Patients and methodsFifty-one consecutive patients with EGGCT were identified. Testicular palpation or ultrasonography to rule out a primary testicular tumor was performed. Pretreatment testicular biopsies were not performed. The incidence and outcome of MTGCT, and the prognosis of EGGCT were evaluated. ResultsTwenty-five and 26 patients, respectively, had mediastinal and retroperitoneal EGGCT. Fourteen and 37 patients, respectively, had seminoma and nonseminoma. Five patients developed MTGCT in patients with retroperitoneal EGGCT. The median interval from the primary treatment for EGGCT to MTGCT diagnosis was 64 months (range 15–120). The cumulative risk of developing MTGCT was 8.3% at 6 y. Five patients underwent an orchiectomy and have survived in the 16-months median follow-up period (range 4–30). Among the patients with seminomatous and nonseminomatous EGGCT, the 5-year survival rate was 84.6% and 78.3%, respectively. Among the patients with retroperitoneal and mediastinal nonseminomatous EGGCT, the 5-year survival rate was 94.7% and 58.8%, respectively. ConclusionsThe prognosis of EGGCT without testicular biopsies was sufficient. EGGCT patients, especially retroperitoneal EGGCT, need long-term follow-up for MTGCT.

末梢血幹細胞移植併用化学療法と残存腫瘍全摘出による集学的治療にて完全寛解を得た後腹膜原発性腺外胚細胞腫瘍の１例

A 37-year-old man with the chief complaints of lumbago and fever is presented. Laboratory data showed the extreme elevation of alfa feto-protein (AFP), human chorionic gonadotropin (hCG) and lactate dehydorgenase (LDH). Computed tomography (CT) scan revealed a huge retroperitoneal tumor with multiple pulmonary nodules as well as left supraclavicular and left axillary lymph nodes enlargement. Although he was suspected the testicular tumor with metastasis, he had no testicular abnormalities including tumor and microlithiasis. Therefore, he was diagnosed as a retroperitoneal extra-gonadal germ cell tumor, which had poor prognosis because of multiple metastasis and the tremendous increase of hCG. Although he was treated with three cycles of bleomycin, etoposide, and cisplatin (BEP), he achieved partial response and no normalization of tumor markers. After three cycles of BEP, he was treated with four cycles of paclitaxel and ifosfamide plus cisplatin (TIP) immediately. During chemotherapy, he was treated with his peripheral blood stem cell transplantation (PBSCT) as well. After the completion of two regimens' chemotherapies, all his tumor markers returned to be normal. However, retroperitoneal tumor, left supraclavicular and axillary lymphnodes still remained. He underwent three operations including retroperitoneal lymphnode dissection with nephrectomy, left supraclavicular and axillary lymphnodes removal, respectively. All specimens had no viable cells, histologically, The patient has been quite well and free of disease for 24 months. It is concluded that even if far-advanced germ cell tumor is discovered, a more promising prognosis could be expected with intensive and aggressive treatment such as our case.

Retroperitoneal Seminoma with the 'Burned out' Phenomenon in the Testis

Hong Koo Ha, Suk Gun Jung, Sung Woo Park, Wan Lee, Sang Don Lee, Moon Kee Chung From the Department of Urology, College of Medicine, Pusan National University, Busan, Korea The rare ‘burned out’ phenomenon in germ cell tumors is known as the presence of an extragonadal germ cell tumor with a spontaneously regressed testicular tumor found in common metastatic sites, including the retroperitoneal, mediastinal, supraclavicular, cervical, and axillary lymph nodes; lung; and liver. We report a patient who presented with a retroperitoneal extragonadal germ cell tumor with a spontaneously regressed testicular tumor. (Korean J Urol 2009;50:516-519) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

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

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 beta-chorionic gonadotropin, alpha-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.

Retroperitoneal extragonadal germ cell tumor presenting as a bulky pelvic mass of the obturator fossa

Extragonadal germ cell tumors (EGGCT) are commonly found in the midline of the body. We report a rare case of a retroperitoneal EGGCT presenting as a bulky pelvic mass located between the left internal and external iliac arteries in a 29-year-old man. His alpha-fetoprotein level was 12946.2 ng/mL and the biopsy of the tumor revealed a nonseminomatous germ cell tumor. After three cycles of chemotherapy (bleomycin/etoposide/cisplatin) followed by resection of all residual tumors, one cycle of salvage chemotherapy (etoposide/ifosphamide/cisplatin) was added. The patient was disease free at 21-month follow up.

Retroperitoneal extragonadal germ cell tumor in a patient with Klinefelter's syndrome

A 22-year-old man was diagnosed with retroperitoneal seminoma associated with Klinefelter's syndrome. The tumor was 14 x 12 cm in size and surrounded the superior mesenteric artery. He received induction chemotherapy with bleomycin, etoposide and cisplatin (BEP) followed by salvage chemotherapy with paclitaxel, ifosfamide and cisplatin (TIP). The tumor considerably decreased in size and remains as a poorly defined plaque. At the time of writing, he is being followed closely and is free from progression.

Extragonadal germ cell tumors of the mediastinum and retroperitoneum: results from an international analysis.

To characterize the clinical and biologic features of extragonadal germ cell tumor (EGCT) and to determine the overall outcome with currently available treatment strategies. Of an unselected population of 635 consecutive patients treated from 1975 through 1996 at 11 cancer centers, 341 patients (54%) had primary mediastinal EGCT, and 283 patients (45%) had retroperitoneal EGCT. Five hundred twenty-four patients (83%) had a nonseminomatous germ cell tumor (GCT), and 104 patients (16%) had a seminomatous histology. After platinum-based induction chemotherapy with or without secondary surgery, 141 patients (49%) with mediastinal nonseminomas (median follow-up, 19 months; range, 1 to 178 months) and 144 patients (63%) with retroperitoneal nonseminoma (median follow-up, 29 months; range, 1 to 203 months) are alive (P =.0006). In contrast, the overall survival rate for patients with a seminomatous EGCT is 88%, with no difference between patients with mediastinal or retroperitoneal tumor location (median follow-up, 49 months; range, 4 to 193 months; respective 70 months; range, 1 to 211 months). A significantly lower progression-free survival rate was found in seminoma patients treated with initial radiotherapy alone compared with chemotherapy. Nonseminomatous histology, presence of nonpulmonary visceral metastases, primary mediastinal GCT location, and elevated beta-human chorionic gonadotropin were independent prognostic factors for shorter survival. Hematologic malignancies (n = 17) occurred without exception in patients with primary mediastinal nonseminoma. Sixteen patients developed a metachronous testicular cancer despite the use of platinum-based chemotherapy. Whereas patients with pure seminomatous EGCT histology have a long-term chance of cure of almost 90% irrespective of the primary tumor site, 45% of patients with mediastinal nonseminomas are alive at 5 years. This outcome is clearly inferior compared with patients with nonseminomatous retroperitoneal primary tumors.

Incidence of metachronous testicular cancer in patients with extragonadal germ cell tumors.

The frequency of subsequent testicular cancer (referred to as metachronous testicular cancer) in men who have had previous testicular cancer is relatively high. The rate of metachronous testicular cancer in men with extragonadal germ cell tumors (EGCTs), however, is largely unknown. We conducted a retrospective study of EGCT patients to determine the incidence, cumulative risk, and specific risk factors for metachronous testicular cancers. A standardized questionnaire about patient characteristics, the extent of EGCT disease, any second malignancies, and treatments received was completed for 635 patients with EGCTs identified from the medical records of 11 cancer centers in Europe and the United States from 1975 through 1996. Comparisons with age group-specific data from the Saarland, Germany, population-based cancer registry were used to calculate the standardized incidence ratio (SIR). The Kaplan-Meier method was used to analyze survival data and cumulative risk. All statistical tests were two-sided. Sixteen EGCT patients (4.1%) developed metachronous testicular cancers, with a median time between diagnoses of 60 months (range, 14-102 months). The risk of developing metachronous testicular cancers was statistically significantly increased in patients with EGCTs (observed = 16; expected = 0.26; SIR = 62; 95% confidence interval [CI] = 36 to 99) and in subsets of EGCT patients with mediastinal location (SIR = 31; 95% CI = 8 to 59), retroperitoneal location (SIR = 100; 95% CI = 54 to 172), and nonseminomatous histology (SIR = 75; 95% CI = 43 to 123). The cumulative risk of developing a metachronous testicular cancer 10 years after a diagnosis of EGCT was 10.3% (95% CI = 4.9% to 15.6%) and was higher among patients with nonseminomatous EGCTs (14.3%; 95% CI = 6.7% to 21.9%) and retroperitoneal EGCTs (14.2%; 95% CI = 5.6% to 22.8%) than among patients with seminomatous EGCTs (1.4%; 95% CI = 0.0% to 4.2%) and mediastinal EGCTs (6.2%; 95% CI = 0.1% to 12.2%). Patients with EGCTs, particularly those with retroperitoneal or nonseminomatous tumors, but also those with primary mediastinal EGCTs, are at an increased risk of metachronous testicular cancer.

Burned-Out Primary Testicular Cancer: Sonographic and Pathological Characteristics

Purpose Rarely, a testicular scar is discovered in a patient with a presumed extragonadal germ cell tumor. Of 6 patients originally diagnosed with retroperitoneal extragonadal germ cell tumors who had echogenic foci on scrotal sonography 5 had definite histological evidence of a regressed primary testicular cancer. Materials and Methods Six men 21 to 36 years old presented with palpably normal testes and a presumed retroperitoneal extragonadal germ cell tumor. After chemotherapy each patient underwent retroperitoneal lymph node dissection and ipsilateral orchiectomy. The entire testis was submitted for histological evaluation and all calcifications were identified. Results Scrotal sonography revealed an echogenic focus or foci in all cases, which corresponded to intratubular hematoxyphilic bodies in 2. In 3 cases the echogenic foci were intratubular psammoma bodies close to a fibrous scar with hemosiderin deposition, 1 of which contained a focus of intratubular germ cell neoplasia. The hematoxyphilic bodies appeared larger and more intensely echogenic on sonography than the psammoma bodies. The remaining case had stromal calcifications near the rete testis. Conclusions The hematoxyphilic bodies and fibrosis with hemosiderin deposits are believed to represent remnants of testicular carcinoma. Our finding of intratubular germ cell neoplasia provides further proof that testicular carcinomas regress. In 5 of 6 patients (83 percent) with presumed extragonadal germ cell tumors we showed definite pathological evidence of a burned-out testicular carcinoma. With a presumed retroperitoneal germ cell tumor and palpably normal testes, sonographic demonstration of an echogenic lesion in the absence of a hypoechoic mass probably represents a burned-out primary neoplasm.

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.

1002 - Retroperitoneal Extragonadal Germ Cell Tumors Associated with Unrecognized Testicular Cancer

1002 - Retroperitoneal Extragonadal Germ Cell Tumors Associated with Unrecognized Testicular Cancer