Primary malignant mediastinal germ cell tumours: A literature review and a study of 18 cases

From 1957 to 1992, 18 cases of primary mediastinal germ cell tumours were referred to the Peter MacCallum Cancer Institute (PMCI). Six were seminomas, six were mixed germ cell tumours, two were embryonal cell carcinomas, three were teratocarcinomas and one was labelled an ‘anaplastic germ cell tumour’. Two of the 18 patients were female. For seminomas, surgical (and in one case chemotherapeutic) debulking, followed by radiotherapy produced the best results. Mediastinal doses ranged from 30 to 40 Gy. Local control was achieved in those patients receiving mediastinal radiotherapy. Four patients currently survive disease-free. The non-seminomatous germ cell tumours showed a significantly poorer survival, and only two of 12 patients remain alive in remission at 110 and 130 months after diagnosis. Survival has been updated as of November 1997. Attention is focused on the anterior position of the primary germ cell tumours in the mediastinum. A review of the literature up to and including 1997 is presented.     

原发性纵隔恶性生殖细胞肿瘤临床和预后分析

目的探讨原发性纵隔恶性生殖细胞肿瘤(PMGCT)的临床病理特点、治疗方法和预后因素。方法回顾性分析29例PMGCT患者的临床资料。结果29例患者均为男性,平均发病年龄26.1岁,肿瘤均来源于前纵隔,平均最大径16.0 cm。其中原发性纵隔精原细胞瘤(PMSGCT)5例(17.2%),原发性纵隔非精原细胞瘤(PMNSGCT)24例(82.8%)。PMGCT最常见症状是憋气、咳嗽与胸痛,其治疗采用化疗、手术、放疗相结合的综合治疗模式。PMNSGCT组中化生存期为19.0个月, 1年和2年生存率分别为65.3%和28.1%。PMSGCT组均长期生存,预后优于PMNSGCT组(P= 0.008)。多因素分析结果显示,病变局限于纵隔、以顺铂为基础的联合化疗是PMNSGCT患者预后的独立影响因素。结论PMGCT以PMNSGCT为主,主要治疗手段是以顺铂为基础的联合化疗。PMNSGCT预后明显差于PMSGCT,并与病变范围、化疗与否相关。     

Non-seminomatous ovarian germ cell tumours in children

In this study, we report the results of two consecutive protocols. TGM 55 and TGM 90, of the Société Fran?aise d'Oncologie Pédiatrique ( SFOP) for patients with non-seminomatous germ cell tumours of the ovary and analyse the rationale for surgical indications. neoadjuvant or adjuvant chemotherapy. TGM 55 and 90 both utilised six drugs, bleomycin, cyclophosphamide, vinblastine, dactinomycin, etoposide and either cisplatin (TGM 55) or carboplatin TGM 90). Chemotherapy was given in ease of unresectable or incompletely resected tumour. Patients who had a complete resection of a localised tumour underwent expectant management and were only treated if progression occurred. 63 patients aged less than 18 sears old were enrolled between January 1955 and December 1994. 49 patients had alpha-fetoprotein (alphaFP) +/- beta-human chorionic gonadotropic hormone (betaHCG) secreting tumours and 14 had immature teratomas. Median follow-up for surviving patients is 60 months (range: 19-154). The 5-year overall survival is 85% +/- 5%. 13 out of 14 patients (93%) with immature teratoma are alive, including 3 of 4 patients (75%) who received chemotherapy for advanced disease. 41 patients (54%) with secreting tumours are alive, including 2 patients who required salvage treatment. Most failures occurred amongst patients with high initial alphaFP secretion ( > 15,000 ng/ml). 39 of 41 survivors (95%) in thc non-teratoma group had conservative surgery, allowing the possibility of future pregnancy. High cure rate can he achieved with a conservative approach in non-seminomatous germ cell tumour of the ovary. Whenever possible, fertility should he preserved during the primary operation in children suffering from these tumours.     

Multimodality treatment of malignant germ cell tumours of the mediastinum

Of 15 patients with malignant germ cell tumours of the mediastinum, 9 patients had pure seminomas and 6 had non-seminomas. Resection was radical in only 4 non-seminomas, 1 of which was resected after chemotherapy; radiotherapy was delivered to all seminoma patients as sole therapy (2 patients) or as part of combined modality therapy. All patients with non-seminomatous tumours underwent chemotherapy (cisplatin-based combination). Therapy was generally well tolerated, but 1 seminoma patient died of sepsis. Chemotherapy achieved a 71% complete response rate in pure seminoma patients and a 33% complete response rate in non-seminoma patients. 53% of patients are alive and free of disease beyond 36 months from start of any treatment. Pure seminoma patients survived longer than non-seminoma patients (3 and 5 year survivals were 67% and 33%, respectively). Although cisplatin-based chemotherapy is highly effective in pure seminomas and also in non-seminomas, a better therapeutic approach is needed in non-seminomas.     

原发纵隔恶性生殖细胞瘤32例临床分析

目的:探讨纵隔恶性生殖细胞瘤(malignant germ cell tumors,MGCT)的临床特点、治疗和预后。方法:32例纵隔MGCT患者,精原细胞瘤18例,非精原细胞瘤14例。所有患者均采用手术和(或)放疗和(或)化疗等多学科综合治疗的方法。结果:非精原细胞瘤患者中位生存期(OS)32.4个月,中位无进展生存期(PFS)18个月,5年无复发生存率和总生存率均为28.6%。精原细胞瘤患者5年无复发生存率和总生存率分别为83.3%和85.6%,中位OS和PFS均未到达。精原细胞瘤患者OS和PFS均明显好于非精原细胞瘤患者,P值分别为0.001 4和0.000 7。结论:纵隔精原细胞瘤采用多学科综合治疗方法能取得较好的治疗效果,本研究的结果与文献报道相符。纵隔非精原细胞瘤的治疗效果有待进一步提高。非精原细胞瘤是影响纵隔恶性生殖细胞瘤预后的重要因素。     

Glycolipids of human primary testicular germ cell tumours.

The glycolipid content of human non-seminomatous germ cell tumour cell lines correlates with their differentiation lineage. To analyse whether this reflects the situation in primary tumours, we studied five embryonal carcinomas, five yolk sac tumours and nine (mixed) non-seminomas, using thin-layer chromatography and carbohydrate immunostaining. We also analysed the glycolipid content of 19 seminomas to reveal their relationship with non-seminomas. Lactosylceramide (CDH) was detected in all embryonal carcinomas, but in fewer than half of the seminomas. Seminomas and embryonal carcinomas contained globoseries glycolipids, including globotriosylceramide (Gb3), globoside (Gb4), galactosy globoside (Gb5) and sialy1 galactosyl globoside (GL7). The lacto-series glycolipid Le(x) was found in all embryonal carcinomas, but only in one seminoma. Gangliosides GD3 and GT3 were detected in many seminomas, but rarely in embryonal carcinomas. Yolk sac tumours displayed a heterogeneous glycolipid profile. Compared with seminomas and pure embryonal carcinomas, differentiated non-seminomas had reduced levels of globo-series glycolipids, especially Gb3 and Gb5, whereas CDH, Le(x), GD3 and GT3 were found in the majority of cases. Thus, the glycolipid content of non-seminoma cell lines reflects the situation in primary tumours. Globo-series glycolipids are similarly expressed in seminomas and embryonal carcinomas. The expression of Gb3 and Gb5 is reduced in non-seminomas upon differentiation. Le(x) expression in non-seminomas, including embryonal carcinomas, allows discrimination from seminomas. Expression of gangliosides in seminomas might indicate their maturation from ganglioside-negative precursor cells. Reprogramming of these precursors would result in the formation of Le(x)-expressing embryonal carcinomas.     

Late recurrence in 1263 men with testicular germ cell tumors. Multivariate analysis of risk factors and implications for management

BACKGROUND: Testicular germ cell tumors are highly curable. However, 10-30% of patients have recurrence after initial treatment. The time-course of recurrence has implications for the duration of follow-up. This study was undertaken to assess the risk and time-course of recurrence and to identify patients at higher risk of late recurrence. METHODS: The records of 1263 patients with primary testicular germ cell tumors presenting to the Royal Marsden Hospital between December 1979 and December 1993 were reviewed. In all, 255 episodes of recurrence were documented (including 44 patients with multiple recurrences) and used to calculate recurrence-free survivals. RESULTS: Fifty-three patients (15 seminomas; 38 nonseminomatous germ cell tumors [NSGCT]) had recurrence more than 2 years after initial presentation. A multivariate analysis of risk of recurrence after 2 years identified positive markers at presentation and the presence of differentiated teratomas in postchemotherapy surgical specimens as significant predictors. Very late recurrence (> 5 years) occurred mainly in patients with metastatic NSGCT (12 of 14 patients) with a 1% annual risk of recurrence between 5 and 10 years. Very late recurrence was also seen in one case of metastatic seminoma and one case of Stage I NSGCT managed by surveillance. Most late recurrences (n = 9) were detected at routine annual follow-up visits but five had recurrences with symptoms leading to an unscheduled clinic visit. CONCLUSION: Late recurrences are rare in patients with testicular germ cell tumors and follow-up to detect recurrence may not be needed after 5 years, except in those presenting with metastatic NSGCTs.     

Klinefelter's syndrome associated with mediastinal germ cell neoplasms

Several case reports have suggested an association of primary mediastinal germ cell tumor (PMGCT) and Klinefelter's syndrome (KS). In an effort to confirm this association, 22 patients with mediastinal germ cell tumors had chromosome studies performed in a prospective fashion. Five patients (22%) had karyotypic or pathologic evidence of KS. All of the patients with KS had germ cell tumors of the nonseminomatous subtype and were relatively young (median age, 15 years). The literature confirms the findings of a young median age (18 years), nonseminomatous subtype, and mediastinal location of the germ cell neoplasm. We conclude that patients with KS are predisposed to the development of mediastinal nonseminomatous germ cell cancers.     

Distribution of Testicular Tumors in Lebanon: A Single Institution Overview

Background: Testicular tumors constitute a rare type of cancer affecting adolescents and young adultswith recent reports confirming an increase in incidence worldwide. The purpose of this study was to estimatethe epidemiological characteristics and histological subtypes of testicular tumors in the Lebanese populationaccording to the WHO classification of testicular and paratesticular tumors. Materials and Methods: In thissingle institutional retrospective study, all patients diagnosed with a testicular tumor in Hotel-Dieu de FranceHospital University in Beirut between 1992 and 2014 were enrolled. The age, subtype based on the 2004 WHOclassification and body side of tumor were analyzed. Results: A total of two hundred and forty-four (244) patientsdiagnosed with a testicular tumor in our institution were included in the study. Two hundred and one patients(82.4% of all testicular tumors) had germ cell tumors (TGCT). Among TGCT, 50% were seminomatous tumors,48% non-seminomatous tumors (NST) and 2% were spermatocytic seminomas. The NST were further dividedinto mixed germ cell tumors (63.9%), embryonic carcinomas (18.6%), teratomas (15.4%) and yolk sac tumors(2.1%). The mean age for testicular tumors was 32 years. The mean age for germ cell tumors was 31 years andfurther subtypes such as seminomatous tumors had a mean age of 34 years, 28 years in non-seminomatous tumorsand 56 years in spermatocytic seminoma. Patients with right testicular tumor were the predominant group with55% of patients. Three patients (1.2%) presented with bilateral tumors. Conclusions: The distribution of differentsubgroups and the mean age for testicular tumors proved comparable to most countries of the world except forsome Asian countries. Germ cell tumors are the most common subtype of testicular tumors with seminomatoustumors being slightly more prevalent than non-seminomatous tumors in Lebanese patients.     

Surveillance for stage I testicular germ cell tumours: results and cost benefit analysis of management options

Between 1979 and 1996 303 men with stage I testicular germ cell tumours (120 seminoma and 183 non-seminomatous germ cell tumours (NSGCT)) were enrolled onto a programme of surveillance. In our institutions the frequency of computed tomography (CT) scans is reduced compared with other centres. For all 303 men, the median follow-up is 5.1 years (range: 0.1-21.7 years) and there have only been 3 deaths (1 from disease, 1 from neutropenic sepsis and 1 from secondary leukaemia). 52/183 (28%) patients with NSGCT and 18/120 (15%) patients with seminoma have relapsed. The relapse-free survival at 5 years is 82% for seminoma and 69% for NSGCT (Logrank P=0.004). All men who relapsed, except 1 man with NSGCT, were in the International Germ Cell Cancer Collaborative Group good or intermediate prognosis group at relapse. Half of the seminoma relapses presented with symptoms and 31% of the NSGCT relapses. The remaining relapses were detected serologically or radiologically by the surveillance programme. 5 men (2%) on surveillance, 3 with initial diagnosis of seminoma and 2 with NSGCT, have developed second contralateral testis tumours (all stage I seminomas). In a well motivated centre a policy of surveillance for stage I testicular germ cell tumours (both NSGCT and seminoma) is associated with a low mortality rate (3/303, 1%) and may have the advantage of sparing overtreatment with potentially toxic therapies in this group of young men.     

Malignancy in giant cell tumor of bone

BACKGROUND: The term malignant giant cell tumor embraces multiple entities and therefore can be confusing. The goals of the current study were to define the clinicopathologic and histologic features of malignancy in giant cell tumors and to clarify the terminology. METHODS: The authors reviewed all cases from the Rizzoli Institute (Bologna, Italy) of primary (PMGCT) and secondary (SMGCT) malignancy in giant cell tumors. PMGCT is a high-grade sarcoma that arises side by side with benign giant cell tumors. SMGCT is a high-grade sarcoma that occurs at the sites of previously treated giant cell tumors of bone. RESULTS: The authors report 5 PMGCTs and 12 SMGCTs; half of the SMGCTs were postradiation sarcomas. Patient age ranged from 20 to 68 years (median, 62 years) for PMGCT and from 30 to 77 years (median, 40 years) for SMGCT. The average latent period between diagnosis of giant cell tumor and diagnosis of SMGCT was 9 years (range, 3-15 years) for patients with postradiation SMGCT and 19 years (range, 7-28 years) for patients with SMGCT resulting from spontaneous transformation. The histologic classification of high-grade sarcomas in the PMGCT group was osteosarcoma in four cases and malignant fibrous histiocytoma in one case. In the SMGCT group, the histologic classification was osteosarcoma in nine cases, fibrosarcoma in two cases, and malignant fibrous histiocytoma in one case. The outcomes associated with all malignancies in giant cell tumors were poor, with the worst outcome associated with postradiation SMGCT. CONCLUSIONS: Malignancies in giant cell tumors of bone always are high-grade sarcomas with a poor prognosis. These lesions must be distinguished from benign giant cell tumors of bone. SMGCT usually is easy to diagnose upon malignant clinicoradiographic presentation. In contrast, PMGCT often mimics giant cell tumors both clinically and radiographically. In addition, upon histologic examination, PMGCT shows areas of conventional giant cell tumor, which can lead to difficulties in making the correct diagnosis.     

Primary malignant mediastinal germ cell tumors: a study of eleven cases and a review of the literature

From 1957 to 1988 eleven cases of primary mediastinal germ cell tumor were referred to the Peter MacCallum Cancer Institute (PMCI). Four were seminomas, three were mixed germ cell tumors, two were embryonal carcinomas and two were teratocarcinomas. Two of the eleven patients were female. For seminoma, surgical debulking and post-operative irradiation produced the best results. Mediastinal doses ranged from 30 to 37.5 Gy. Local control was achieved in all cases; two patients survive disease-free. The non-seminomatous germ cell tumors showed a significantly poorer survival with only one of seven patients remaining alive in remission at 15 months. One other case of non-seminomatous tumor remains alive but in relapse at 23 months. Attention is focused on the anterior position of primary germ cell tumors in the mediastinum. A review of the literature is presented.     

Clinical profile and problems of management of 108 cases of germ cell tumours of testis at Institute Rotary Cancer Hospital, All India Institute of Medical Sciences New Delhi 1985-1990.

A retrospective analysis of 108 cases of primary germ cell tumours of testis seen over a 6 year period at Institute Rotary Cancer Hospital of All India Institute of Medical Sciences, New Delhi is presented. There were 45 (42%) cases of seminoma and 63 (48%) of non-seminomatous germ cell tumours (NSGCT). The median age at presentation was 35 and 30 years respectively. Almost half (56) patients presented in advanced stage (stages IIc-IV). Tumours in undescended testis formed an important subgroup (14%). The standard approach of treatment was radiotherapy in stages I & II seminomas and chemotherapy in bulky seminomas and metastatic NSGCT. Chemotherapy protocols used were VAB-6 and PVB. Although a policy of surveillance has been practised for stage I NSGCT, it is debatable whether it is universally suitable for our patients. The results of treatment in low volume disease are comparable to that in the west but the management of bulky disease requires a more aggressive approach. Unfortunately only 74 out of 108 (68.5%) patients were able to complete the treatment prescribed. Most of the defaulters were from the chemotherapy group because of inability to afford the drugs. The probability of survival of those who completed treatment was 0.77 at 4 years. Since testicular tumours are largely curable, a more vigorous policy of detection, follow up and treatment needs to be pursued. Better screening of children with undescended testis will reduce cancer in this group. Failure to provide chemotherapy to all patients is particularly unfortunate for a curable disease like testis cancer.     

Primary germ cell tumors in the mediastinum: a 50-year experience at a single Japanese institution

BACKGROUND: Primary germ cell tumors (GCT) of the mediastinum share similar clinical and biologic characteristics, which are different from their testicular counterpart. The purpose of the current study was to review the authors' institutional experience of mediastinal GCT, emphasizing the clinical spectrum, time trends of treatment, and recent advances in therapeutic modalities for malignant GCT. METHODS: Between 1951 and 2000, 129 patients (70 males and 59 females) underwent surgical treatment for GCT, which accounted for 16.0% of the mediastinal tumors during the same period. There were 95 patients with mature teratomas, 13 patients with seminomas, and 21 patients with nonseminomatous germ cell tumors (NSGCT) with median ages of 26.4 years, 27.6 years, and 28.5 years, respectively. RESULTS: Adult patients with mature teratomas were less symptomatic (33.3%) than pediatric patients (52.4%). All patients with mature teratoma were cured by resection alone. Eight of the 13 patients (61.5%) with seminoma were symptomatic and 10 of 13 patients (83.3%) survived after surgery and radiation with/without chemotherapy. Nineteen of 21 patients (90.5%) with NSGCT had dyspnea, chest pain, and superior vena cava syndrome. Before 1985, patients received radical resection and/or chemoradiotherapy. However, all patients died due to disease progression, with a median survival period of 7.6 months. After 1986, six of eight patients received cisplatin-based chemotherapy, including three who received additional high-dose chemotherapy with a supporting peripheral blood stem cell transplantation until tumor markers normalized. Five patients who underwent salvage resection are currently disease free with a median survival period of 58.3 months. CONCLUSIONS: The institutional experience indicates the benign nature of mediastinal mature teratomas and the excellent prognosis for patients with seminomas after resection. An improved survival advantage was ensured with cisplatin-based preoperative chemotherapy in patients with NSGCT.     

GPR30 is overexpressed in post-puberal testicular germ cell tumors

GPR30 is a 7-transmembrane G protein-coupled estrogen receptor that functions alongside traditional estrogen receptors to regulate cellular responses to 17β-estradiol and environmental estrogens. In this study, we have evaluated by immunohistochemical analysis GPR30 expression in post-puberal testicular germ cell tumours (30 seminomas, 5 teratomas, 12 embryonal carcinomas, and 20 intratubular germ cell tumors). The GPR30 protein expression was detected at high level in all intratubular germ cell tumours, seminomas, and embryonal carcinomas, whereas in teratomas the expression was low. The immunohistochemical data were further confirmed by Western blot analysis. GPR30 protein expression has also been analyzed in GC1 and TCam-2 cell lines, respectively derived from immortalized type B murine spermatogonia and human seminoma. Our results indicate that GPR30 could be a potential therapeutic target; the design of a specific GPR30 inhibitors could be a useful molecular target to block neoplastic germ cells with a high proliferative rate for the treatment of TGCTs.     

Malignant ovarian tumours in childhood in Britain, 1962-78

The files of the Childhood Cancer Research Group and of the Oxford Survey of Childhood Cancers were scrutinized for all the ovarian neoplasms registered in England, Scotland and Wales in children under age 15 years throughout the period 1962-78. Among 172 cases confirmed as malignant ovarian tumours, 145 (84%) were tumours of germ cell origin (54 dysgerminomas, 36 malignant teratomas, 26 endodermal sinus tumours, 4 embryonal carcinomas, 2 pure choriocarcinomas, 20 mixed germ cell neoplasms, 3 gonadoblastomas), 13 (8%) were epithelial carcinomas (3 serous or undifferentiated, 10 mucinous), 9 (5%) were sex-cord stromal tumours (3 granulosa cell, 3 Sertoli-Leydig, 3 unclassified) and 5 (3%) were other miscellaneous tumour types. Less than 10% of the neoplasms occurred at age less than 5 years, approximately 20% from 5-9, and greater than 70% from 10-14 years. Germ cell neoplasms of greater malignancy (immature teratomas, endodermal sinus tumours) occurred in a significantly higher proportion at younger age (less than 10 years) than dysgerminomas (P = 0.01). The overall incidence (approximately 1.7 cases per 10(6) per annum) did not show any noticeable trend over the 17-year period considered. The clustering of two confined cases and, possibly, a third case, of germ cell neoplasms in three generations of the same family pointed to a genetic component in the aetiology of some of these neoplasms. A large number of sex related and mental or neurological abnormalities was also reported in case children. The 10-year survival rates, determined by the life-table method were: epithelial carcinomas 73%, sex-cord stromal tumours 44%, dysgerminomas 73%, malignant teratomas 33%, endodermal sinus tumours 39%, embryonal carcinomas 25%, other germ cell neoplasms 30% and gonadoblastomas 100%. Apart from cell-type, factors associated with prognosis were clinical stage (in all types), size and degree of histological differentiation (in malignant teratomas, but only when stage was not allowed for). The adoption of efficacious polychemotherapy regimens completely changed the prognosis of germ cell tumours other than dysgerminomas (from 29% to greater than 85% disease-free survivors in the present series).     

An analysis of surveillance for stage I combined teratoma--seminoma of the testis.

We analysed 973 patients with stage I testicular tumours presenting between 1983 and 1994. The median ages at presentation for non-seminomatous germ cell tumour (teratoma) were 27 years, seminoma 36 years and combined tumour 33 years. These differences were statistically significant (Mann-Whitney P < 0.05), suggesting that combined tumours may have a separate natural history. We, therefore, analysed all stage I patients managed with surveillance (530 in total) post orchidectomy. The actuarial 5 year relapse-free survival and anatomical patterns of relapse were identical for non-seminomatous germ cell tumour (NSGCT) and combined tumour and both were statistically distinct from seminoma (P = 0.01, log-rank test, chi-square test P = 0.001). The association of seminoma within a histologically confirmed NSGCT has no influence on the clinical outcome.     

An immunohistological study of testicular germ cell tumours using two different monoclonal antibodies against placental alkaline phosphatase.

Using two monoclonal antibodies directed against placental alkaline phosphatase (H17E2 and D20L) the immunohistological staining of testicular germ cell tumours was compared with that of a wide range of normal and malignant tissues. All seminomas and malignant teratomas tested gave strong positive labelling with H17E2 but were either negative or only patchily positive with D20L. Neither antibody gave any positive reaction on the normal tissues tested. All other malignancies were negative with both antibodies apart from two cases of ovarian and one case of endometrical cancer (strongly stained by H17E2) and three cases of colonic carcinoma (weakly and patchily stained by both H17E2 and D20L). This indicates that germ cell neoplasms generally express a form of placental alkaline phosphatase recognised by antibody H17E2.     

26例原发性纵隔恶性生殖细胞瘤的诊治

目的:探讨原发性纵隔恶性生殖细胞瘤的诊治及外科手术的作用.方法:对26例收治的原发性纵隔恶性生殖细胞瘤的临床资料进行回顾性分析.结果:22例手术治疗患者中,11例根治性切除,10例姑息性切除,1例探查,手术并发症发生率及死亡率分别为18.2%和9.1%,其中12例术后给予以顺铂为主的联合化疗,4例予以放疗.手术治疗患者术后病理为无性生殖细胞瘤12例,精原细胞瘤5例,未成熟畸胎瘤5例.3例未成熟畸胎瘤及1例胚胎癌患者明确诊断后未手术而给予放疗或放、化疗.本组26例患者中仅2例精原细胞瘤生存满5年,17例已证实死亡,除2例手术死亡外均死于肿瘤复发转移.结论:原发性纵隔恶性生殖细胞瘤的治疗应强调以化疗为主的综合治疗,外科切除只宜做为阶段性的辅助手段,手术时机把握应以具体患者情况而定.     

Platinum-based chemotherapy of primary extragonadal germ cell tumours: the Hellenic Cooperative Oncology Group experience.

Extragonadal germ cell tumours (EGCT) are uncommon, most frequently arise in the mediastinum and retroperitoneum and have variable responses to platinum-based chemotherapy. A retrospective analysis was performed on 38 patients with EGCT treated with cisplatin-based (CDDP) or carboplatin-based (CBDCA) chemotherapy between 1984 and 1998. Twenty-four patients had nonseminomatous germ cell tumours (NSGCT) and 14 seminoma. Twenty-two tumours arose in the mediastinum (13 nonseminomas, 9 seminomas) and 16 in the retroperitoneum (11 NSGCT, 5 seminomas). Initial surgery included complete resection in 1 patient, biopsy in 27 patients and debulking surgery in 10 patients. Complete response rates with chemotherapy +/- surgery were as follows: mediastinum 14 of 21 (66.66%) patients (8 of 12-75% NSGCT, 6 of 9-66.66% seminomas) and retroperitoneum 14 of 16 (87.5%) patients (9 of 11-81.81% NSGCT, 5 of 5-100% seminomas). One patient who underwent complete resection of a mediastinal malignant teratoma combined, received PVB chemotherapy on an adjuvant basis and remains alive and disease-free. Three additional seminoma patients who achieved partial response after chemotherapy remain alive and disease-free following mediastinal radiotherapy. All 14 patients with extragonadal seminomas remain alive with no evidence of disease at a median follow-up of 49 months (range 7-164), giving an overall survival of 100%. Nine of 13 (69.23%) patients with mediastinal NSGCT are long-term disease-free at a median follow-up of 43.5 months (range 7-152). Nine of 11 (81.81%) patients with retroperitoneal NSGCT remain alive and disease-free at a median follow-up of 56 months (range 14-110). Complete surgical resection of residual mass was undertaken in 10 patients (3 seminomas, 7 nonseminomas). The histology revealed necrosis/fibrosis in 6 patients (3 seminomas, 3 NSGCT) and viable cancer in 4 patients. Patients who had viable malignant cells in the resected specimens received two more courses of VelP chemotherapy. None of our patients had relapsed at the time of this analysis. None of our 6 patients who underwent testicular biopsy (1 patient) or orchiectomy (5 patients) due to suspicious ultrasound of the testis were found to have testicular tumour or fibrotic scar. In conclusion, this retrospective analysis showed significant responses in patients with either mediastinal or retroperitoneal NSGCT treated with CDDP- or CBDCA-based chemotherapy +/- surgery. All patients with extragonadal seminomas remain alive with no evidence of disease, regardless of the site at presentation.