Primary Malignant Germ Cell Tumors Research Articles

PRIMITIVE MALIGNANT GERM CELL TUMORS OF THE OVARY: ABOUT 11 CASES AND LITERATURE REVIEW

Malignant germ cell tumors of the ovary are rare entities. They mainly affect young patients and can threaten the functional prognosis.
 We carried out a retrospective study of 11 cases of primary malignant germ cell tumors of the ovary, diagnosed at the paraclinical training and research unit in Pathological Anatomy and Cytology of the CHU/JRA, within a period of 10 years, from January 2010 to December 2019. The ages of these patients varied from 2 to 63 years old with an average of 30,5 years old. In the majority of cases (7 cases), radical treatement was achieved. The guiding clinical information was in all cases an abdominopelvic mass. The right side was the most affected (6/11) and one case was bilateral. The most common histological type was dysgerminoma (4/11).

Synchronous Whole Sternal Metastasis from Primary Malignant Mediastinal Germ Cell Tumor in a Teenager

Primary malignant germ cell tumor (GCT) of the mediastinum is a rare entity with an incidence ranging from 1 to 2% of all childhood cancers. Though a few cases of bone metastasis from mediastinal GCTs have been reported, synchronous whole sternal metastasis from primary malignant mediastinal GCTs is very rare. We report such a rare manifestation in a teenager, emphasizing the need for a multidisciplinary treatment approach with neoadjuvant chemotherapy, surgical expertise for en bloc excision of the mediastinal mass along with whole sternal resection, and reconstruction in a dedicated cancer care center for better oncological outcomes.

Primary hepatic mixed germ cell tumor in an adult.

Primary hepatic mixed germ cell tumor (GCT) is very rare, and less than 10 cases have been reported. We report a case of mixed GCT composed of a choriocarcinoma and yolk sac tumor, which occurred in the liver of a 40-year-old woman. A large mass was detected by computed tomography solely in the liver. Serum β-human chorionic gonadotropin (hCG) was highly elevated, otherwise, other serum tumor markers were slightly elevated or within normal limits. For hepatic choriocarcinoma, neoadjuvant chemotherapy was administered, followed by right lobectomy. Histologic features of the resected tumor revealed characteristic choriocarcinoma features with diffuse positivity for hCG in the syncytiotrophoblasts and diffuse positivity for α-fetoprotein and Sal-like protein 4 in the yolk sac tumor components. Primary malignant GCT in the liver is associated with a poor prognosis and requires specific treatment. Therefore, GCT should be considered during a differential diagnosis of a rapidly growing mass in the liver.

English

Primary malignant extra gonadal germ cell tumours (GCT) of mediastinum are very rare tumours, comprising 1 - 4 % of all mediastinal tumours.1 Even though they are rare tumours of mediastinum, they should always be considered in the differential diagnosis of mediastinal tumours in young adults. Primary malignant GCT can be divided into 2 groups: Seminomatous and non-seminomatous tumours. Mature teratomas (non-seminomatous) are the most common primary malignant germ cell tumours of mediastinum.2 Non-seminomatous tumours tend to be more aggressive than seminomatous tumours present as heterogeneous masses with areas of necrosis and show local invasion. Seminomas usually present as lobulated homogeneous masses and rarely show signs of local invasion. These tumours usually present with asymptomatic lesions or with symptoms due to compression of adjacent structures and usually have a better prognosis than non-seminomatous tumours. Here, we present a case of primary malignant extra gonadal seminoma with sternal involvement.

MA 16.10 Treatment Outcomes of Primary Malignant Germ Cell Tumors of the Mediastinum

Primary mediastinal malignant germ cell tumors (GCT) are rare neoplasms with various histopathological findings and contain complicated clinical characteristics. Chemotherapy plays an important role in the treatment and there are some cases where a good prognosis is expected with multimodal treatment including surgical resection, pre- and postoperative chemoradiotherapy. The medical records of 27 patients who were treated in our institution between 1988 and 2014 were retrospectively reviewed. We investigated the clinical characteristics and the outcomes of treatment. All patients were male with a mean age of 30.7 years (range, 16-53 years), including 17 cases of seminoma (SGCT group) and 10 cases of non-seminoma (NSGCT group). Twenty-three patients underwent surgery and the remaining four patients received chemotherapy and/or radiotherapy without surgery. Of the 23 patients who underwent surgery, 17 cases received preoperative chemotherapy and 15 cases were treated with postoperative chemo/radiotherapy. In six patients, surgery was performed without preoperative chemotherapy due to the suspicious of thymoma, and was followed by postoperative chemo/radiotherapy. Among 24 patients whose serum tumor marker levels were measured before the treatment, 15 patients showed elevated serum tumor marker levels [five (35.7%) in SGCT group and 10 (100.0%) in NSGCT group]. Furthermore, among 20 patients whose serum tumor marker levels were measured before surgery, four patients showed elevated serum tumor marker levels [one (9.1%) in SGCT group and three (33.3%) in NSGCT group]. The median follow-up period after the treatment was 68 months (range, 4-316 months). The 5-year and 10-year survival rate was 88% and 88% in SGCT group, respectively, and 60% and 45% in NSGCT group, respectively (p=0.031). Although there was no relationship between the serum tumor marker levels before the treatment and the prognosis, the patients without elevated tumor marker levels revealed better prognosis than those with elevated one (p=0.014). The treatment for mediastinal malignant GCT in our institution was feasible with favorable outcomes. Although malignant GCT has poor prognosis especially in NSGCT group, chemotherapy which normalizes serum tumor marker levels can improve its prognosis.

C154: Clinicopathological prognostic factors for patients' survival for mediastinal primary malignant germ cell tumor

C154: Clinicopathological prognostic factors for patients' survival for mediastinal primary malignant germ cell tumor

Diagnostic accuracy of integrated (18)F-FDG PET/CT for restaging patients with malignant germ cell tumours.

Evaluation of utility of fluorine-18 fludeoxyglucose ((18)F-FDG) positron emission tomography/CT (PET/CT) for restaging patients with primary malignant germ cell tumours (GCTs). Data of 92 patients (age, 31.94 ± 10.1 years; male/female, 86/6) with histopathologically confirmed malignant GCTs (gonadal, 88; mediastinal, 4; seminomatous, 47 and non-seminomatous, 45) who underwent (18)F-FDG PET/CT for restaging (suspected recurrence/post-therapy evaluation) were retrospectively analysed. Two experienced nuclear medicine physicians reviewed the PET/CT images in consensus, qualitatively and semi-quantitatively [maximum standardized uptake value (SUVmax)]. Histopathology (if available) and clinical/imaging/biochemical follow-up (minimum of 6 months) were employed as the reference standard. (18)F-FDG PET/CT was interpreted as positive in 59 and negative in 33 patients. Local disease was seen in 5, nodal disease in 50 and distant metastasis in 22 patients. PET/CT was true positive in 49, false positive in 10, true negative in 30 and false negative in 3 patients. (18)F-FDG PET/CT showed sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 94.2%, 75.0%, 83.0%, 90.9% and 85.8% overall; 90.0%, 74.0%, 72.0%, 90.9% and 80.8% in seminomatous GCT; and 96.8%, 76.9%, 91.1%, 90.9% and 91.1% in non-seminomatous GCT, respectively. Difference in PET/CT accuracy for seminomatous and non-seminomatous GCTs was not significant (p = 0.263). PET/CT demonstrated disease in 13 patients with negative/equivocal conventional imaging findings and in 9 patients with normal tumour markers. No site- or histology-based difference was seen in SUVmax. (18)F-FDG PET/CT demonstrates high diagnostic accuracy for restaging patients with malignant GCTs. It has comparable diagnostic performance in both seminomatous and non-seminomatous malignant GCTs. The present article demonstrates high diagnostic accuracy of (18)F-FDG PET/CT for restaging both seminomatous and non-seminomatous malignant GCTs in a large patient population.

Tumeur germinale primitive à localisation vertébrale dorsale chez un adulte : une observation exceptionnelle

Vertebral primary malignant germ cell tumors are rarely located in thoracic spine. We report the case of a 44-year-old female, in which a symptomatology including dorsal rachidial pain, intercostal neuralgia, straight and transit disorder revealed a thoracic vertebral primary malignant germ cell tumor. The sole location of a vertebral primary malignant germ cell tumor in adult, exceptionally reported in the literature, prompted us to relate this observation.

Malignant germ cell tumours of childhood: new associations of genomic imbalance

Malignant germ cell tumours (MGCTs) of childhood are a rare group of neoplasms that comprise many histological subtypes and arise at numerous different sites. Genomic imbalances have been described in these tumours but, largely because of the paucity of cases reported in the literature, it is unclear how they relate to abnormalities in adult MGCTs and impact on potential systems for classifying GCTs. We have used metaphase-based comparative genomic hybridisation to analyse the largest series of paediatric MGCTs reported to date, representing 34 primary tumours (22 yolk sac tumours (YSTs), 11 germinomatous tumours and one metastatic embryonal carcinoma) occurring in children from birth to age 16, including 17 ovarian MGCTs. The large dataset enabled us to undertake statistical analysis, with the aim of identifying associations worthy of further investigation between patterns of genomic imbalance and clinicopathological parameters. The YSTs showed an increased frequency of 1p- (P=0.003), 3p+ (P=0.02), 4q− (P=0.07) and 6q− (P=0.004) compared to germinomatous tumours. Gain of 12p, which is invariably seen in adult MGCTs, was present in 53% of primary MGCTs of children aged 5–16 and was also observed in four of 14 YSTs affecting children less than 5. Two of these cases (14% of MGCTs in children less than 5) showed gain of the 12p11 locus considered to be particularly relevant in adult MGCTs. Gain of 12p showed a significant association with gain of 12q. Conversely, MGCTs without 12p gain displayed a significantly increased frequency of loss on 16p (P=0.04), suggesting that this imbalance may contribute to tumour development in such cases. This data provides new insight into the biology of this under-investigated tumour group and will direct future studies on the significance of specific genetic abnormalities.

Unusual scapular metastasis as initial manifestation of advanced nonseminomatous germ cell tumor of the mediastinum

Primary malignant germ cell tumors of the mediastinum are relatively rare, occurring predominantly in young male adults, and have a poor prognosis. We present a case of a 27-year-old man who initially experienced a persistent, intractable painful sensation over the right lower scapula despite taking an analgesic agent for 2 months. A scapular x-ray film and a whole-body bone scan showed an expansile osteolytic lesion. Excisional biopsy of the scapula revealed a metastatic carcinoma, suggestive of nonseminomatous germ cell tumor origin. Further examination of the whole abdomen and bilateral testes were negative. Chest computed tomography and magnetic resonance imaging showed a primary tumor mass in the anterior mediastinum. Chemotherapy with cisplatin, bleomycin, and etoposide was administered for six courses. The mediastinal tumor mass was markedly reduced in size and remission without evidence of tracer uptake by [(18)F]fluorodeoxyglucose positron emission tomography examination. Six months after chemotherapy, the patient received advanced surgical intervention to remove the mediastinal tumor, the pathologic features of which were similar to the previous scapular lesion. He was doing well at 1-year follow-up.

Management of primary malignant germ cell tumor of the mediastinum.

Primary mediastinal malignant germ cell tumors (GCTs) are rare and have a worse prognosis than their gonadal counterparts. Although multimodality treatment is a standard therapeutic strategy in mediastinal GCTs, the clinical implications of surgical intervention remain unclear. Forty-eight patients with primary mediastinal malignant GCT who were treated at the National Cancer Center Hospital, Tokyo, from 1962 to 2002 were studied retrospectively with regard to their histology and clinical profile. Mediastinal GCT occurred predominantly in young males, with a mean age of 28.8 years at the time of diagnosis. There were 46 males (96%) and two females (4%). Histologically, seven patients (15%) were diagnosed as having pure seminoma and 41 (85%) had non-seminomatous GCT. Treatment consisted of surgery alone in nine patients, surgery followed by chemotherapy in two, and chemotherapy followed by surgery in 20. The other 17 patients received chemotherapy and/or radiotherapy without surgery. Of these latter 17 patients, 14 developed progressive disease and three were followed up with a sustained partial response. Among the 31 patients who underwent surgery, complete resection was performed in 27 (87%) and incomplete resection was performed in four (13%). Twelve (41%) patients had elevated serum tumor marker levels preoperatively. Among the 20 patients who received preoperative chemotherapy, viable cells were found in the resected specimen in six (30%). With regard to tumor recurrence in patients with surgical intervention, the preoperative serum tumor marker levels and the presence of viable cells in the resected specimen were significantly associated with recurrence. There was no significant association between surgical curability and recurrence. The 5-year overall survival rate in all 48 patients was 45.5%. Surgical intervention for mediastinal GCT may be needed to remove a chemotherapy-refractory tumor or to assess the pathological response to chemotherapy to determine the indications for further chemotherapy.

Primary Malignant Germ Cell Tumours of the Mediastinum: Results of Multimodality Treatment

Primary malignant mediastinal germ cell tumours are rare and considered to have poorer prognosis compared with those arising from gonads. Eighteen patients with primary mediastinal germ cell tumour were treated over an 11-year period; 9 had seminoma and 9 non-seminoma. Eight patients, 4 each with seminoma and non-seminoma underwent initial tumour excision and the rest had biopsy only. All patients received cisplatin-based chemotherapy. All patients with seminoma received consolidation radiotherapy to mediastinum. Three patients with non-seminoma received radiotherapy following partial response. All 9 patients with seminoma achieved complete response at the end of chemotherapy. Two patients with NSGCT had complete response to chemotherapy, 5 partial response and 2 no response. Two patients who underwent resection of the residual tumour mass are surviving free of disease. Addition of radiotherapy or second-line chemotherapy did not bring about any added response in partial and non-responders. Nine out of 9 patients with seminoma and 4/9 with non-seminoma are surviving disease-free at a median follow-up of 48 months (range 16–153 months). Mediastinal seminoma has excellent prognosis with cisplatin combination chemotherapy, whereas non-seminoma carries poor prognosis, and aggressive chemotherapy with resection of residual masses may improve the outcome. The role of additional radiotherapy and initial tumour debulking needs further evaluation.

Primary malignant mediastinal germ cell tumours: improved prognosis with platinum-based chemotherapy and surgery.

A retrospective analysis was performed of 18 patients with primary malignant germ cell tumours of the mediastinum treated with platinum-based chemotherapy between 1977 and 1990. All seven patients with pure seminoma were treated initially with chemotherapy and four of these patients received additional mediastinal radiotherapy. Only one patient relapsed; his initial therapy had included radiotherapy and single-agent carboplatin and he was successfully salvaged with combination chemotherapy. With a follow-up of 11 to 117 months (median 41 months) all seven patients with seminoma remain alive and disease free giving an overall survival of 100%. Eleven patients had malignant non seminoma; following chemotherapy eight of these had elective surgical resection of residual mediastinal masses. Complete remission was achieved in nine (82%) patients, however, one of these patients died from bleomycin pneumonitis. With a follow-up of 12 to 113 months (median 55 months) eight of 11 (73%) patients with malignant mediastinal teratoma remain alive and disease free.

CT in Primary Malignant Germ Cell Tumors of the Retroperitoneum

Malignant germ cell tumors may exist as a primary entity in the retroperitoneum. In a CT study of 14 males with this condition (2 seminomas and 12 non-seminomatous tumors) all masses were large, lobulated and of mixed density. Fat plane obliteration against adjacent structures was frequent. The aorta was embedded in 9 patients and the inferior vena cava was affected in 7, 2 of whom had signs of compromised caval blood flow. Distant metastases were found in the lungs (7 patients), liver (n = 4), posterior mediastinum (n = 3), and in brain and supraclavicular lymph nodes in one patient each. Serum biomarkers were elevated in 11 patients. An extragonadal germ cell tumor should be considered when CT of the abdomen reveals a large retroperitoneal mass with mixed density.

CT in Primary Malignant Germ Cell Tumors of the Retroperitoneum

Malignant germ cell tumors may exist as a primary entity in the retroperitoneum. In a CT study of 14 males with this condition (2 seminomas and 12 non-seminomatous tumors) all masses were large, lobulated and of mixed density. Fat plane obliteration against adjacent structures was frequent. The aorta was embedded in 9 patients and the inferior vena cava was affected in 7, 2 of whom had signs of compromised caval blood flow. Distant metastases were found in the lungs (7 patients), liver (n=4), posterior mediastinum (n=3), and in brain and supraclavicular lymph nodes in one patient each. Serum biomarkers were elevated in 11 patients. An extragonadal germ cell tumor should be considered when CT of the abdomen reveals a large retroperitoneal mass with mixed density.

Malignant germ cell tumors of the mediastinum

A review of 56 cases of primary malignant germ cell tumors of the mediastinum revealed that, as with benign teratomas, the tumors occurred in young adults (mean age 29 years) but that the sex distribution differed (86% male and 14% female). A single germ cell element was found in 37 (66%) of the tumors, and various combinations were present in the remaining 19 (34%). The tumors were classified among five recognized types of germ cell tissues. There were 24 seminomas (22 pure and two with mature teratomas), 17 embryonal carcinomas (nine pure and eight with mixtures), five teratomas, seven choriocarcinomas (three pure and four with mixtures), and three pure yolk sac tumors. Most (86%) of the patients were symptomatic at the initial examination, with chest pain, cough, and loss of weight being the most frequent presenting symptoms. The standard posteroanterior and lateral roentgenograms were the most helpful diagnostic tool, showing evidence of an anterior mediastinal mass in 53 patients. The diagnosis was established by surgical exploration of the mediastinum or by biopsy of a lymph node in 55 patients. Of the 55, 24 (43.6%) had complete resection of the tumor and 31 (56.4%) had incomplete resection or biopsy alone. The overall prognosis for mediastinal germ cell tumors is poor, partly because the tumors are far advanced at the time of diagnosis but also because some of the tumors that contain embryonal cell carcinoma, choriocarcinoma, and yolk sac elements are very aggressive. Factors that were prognostic in patients with seminoma--such as age, presence of the superior vena caval syndrome, lymphadenopathy, evidence of hilar disease on the chest roentgenogram, and resectability--were not predictive in patients with other types of malignant germ cell tumors. Although aggressive combination chemotherapy may represent a significant treatment modality for nonseminomatous mediastinal tumors, the present study spanned many years in which no chemotherapy was available. Patients in the later years of the study received combination chemotherapy with various treatment regimens. No conclusions concerning specific chemotherapy, therefore, can be derived from this study.

Management of primary germ cell tumors of the mediastinum

Twenty-eight patients with primary malignant germ cell tumors (GCT) of the mediastinum were treated at the University of California at Los Angeles and The Johns Hopkins Hospital in the past 30 years. Of 11 patients with pure seminomas, nine (82%) are free of disease from 6 months to 15 years following therapy. The primary treatment modality in these patients was mediastinal radiation; one patient with metastatic disease had a complete remission and prolonged survival following combination chemotherapy. Seventeen patients had GCT with nonseminomatous elements. Only three (18%) are alive and free of disease. One patient treated only surgically is alive at 15 years and two patients treated with combination chemotherapy and operation are alive and free of disease at 6 months and 3 years. When analyzed by a Kaplan-Meier actuarial survival estimate, patients with nonseminomatous GCT who were treated with cisplatin-bleomycin-based chemotherapy had a median survival of 14.0 months whereas those treated with chemotherapy regimens not employing these agents had a median survival of 4.0 months (generalized Wilcoxon test, p = 0.0495). Patients with pure seminomas are effectively treated with radiation therapy. Patients with nonseminomatous tumors have a much poorer prognosis and deserve aggressive multimodality therapy with cisplatin-bleomycin-based chemotherapy.

Bilateral Primary Malignant Germ Cell Tumors of the Testes: Report of 2 Cases

Bilateral Primary Malignant Germ Cell Tumors of the Testes: Report of 2 Cases