Dysgerminoma of the ovary

Dysgerminomas of the ovary are rare germ cell tumors. They are more common at puberty. Surgery followed by irradiation has resulted in excellent cure rates in early stages of this disease. This paper is a review of six patients with a diagnosis of dysgerminoma who were treated at the Department of Radiation Oncology, University of Louisville. The controversy on the use of irradiation in Stage Ia tumor is discussed in detail. A review of the literature is also done in this paper.     

Dysgerminoma of the ovary: review of 27 cases

Twenty-seven patients with pure dysgerminoma were seen at the Tata Memorial Hospital, Bombay, between January 1980 and December 1984. Of the 10 patients in stage I, 2 patients underwent a unilateral salpingoophorectomy (USO) and were kept under observation without any adjuvant therapy, while the remaining patients received adjuvant treatment following surgery. All of the 6 patients in stage II and 4 of 7 in stage III had total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) followed by postoperative radiotherapy. One patient presented in stage IV and 3 patients presented with recurrence after previous definitive treatment undertaken at other institutions. The disease free, as well as the overall survival, at 108 months, for the 24 cases primarily treated at this institution, is 81% and 88% respectively, and, for patients in stages I and II, 100% (Kaplan-Meier estimation). The need for controlled clinical trials to devise optimal therapy in the early clinical stages and use of chemotherapy for advanced stages of this highly curable entity are stressed.     

卵巢无性细胞瘤20例临床分析

本文对我院１９８４年６月～１９９４年６月共收治无性细胞瘤２０例进行临床分析。年龄最小９岁，最大４８岁，３０岁以下１４例，占７０％，占同期卵巢原发恶性肿瘤的５．４％。Ⅰａ期８例，Ⅰｂ期２例，Ⅰｃ期２例，Ⅱ期２例，Ⅲ期３例，Ⅳ期３例。全部病例均以手术为主，术后辅以放疗或化疗。除２例失访外，３例手术后短期内死亡，１５例存活，５年存活率２５％。从本组病例看，Ⅰａ期的要求保留生育能力的年轻患者，术时检查如无转移，同时行对侧卵巢楔形切除快速切片，而且有良好的随访条件者，可行单侧附件切除，术后配以化疗，待其生育后再行根治性手术。     

卵巢无性细胞瘤的治疗分析与保存生育能力的探讨

本文报道了１９７５年～１９９２年在我院治疗的卵巢无性细胞瘤４４例。其中Ⅰ期２０列，Ⅱ期８例，Ⅲ期５例，Ⅳ期１１例。２８例在我院完成首次治疗，除５例在治疗后３年内死亡外，其余均生存５至１７年，５年生存率为８２％。１６例外院术后复发转入我院治疗，按其初诊时的分期：Ⅰ期４例，Ⅱ期２例，Ⅲ期３例，Ⅳ期７例。５年生存率仅４４％。说明有效的首次冶疗相当重要。采用选择性病例作瘤侧附件切除加术后联合化疗，达到不增加死亡率的同时又保留患者的生育及卵巢功能，已经有了比较乐观的前影。     

Dysgerminoma of the Ovary with Hypercalcemia Associated with Elevated Parathyroid Hormone-related Protein

The purpose of the present paper is to present a rare case ofdysgerminoma of the ovary with hypercalcemia showing elevationof multiple serum tumor markers including parathyroid hormone-relatedprotein (PTH-rP). An 18-year-old unmarried woman, with ovariandysgerminoma showing hypercalcemia and elevated serum PTH-rP,received six courses of a combination chemotherapy consistingof bleomycin, etoposide and cisplatin after her first surgery,and had no evidence of recurrence approximately 30 months aftercompleting the chemotherapy.     

Dysgerminoma of the ovary.

From 1938 to 1977, 21 patients with pure dysgerminoma were treated at Memorial and James Ewing Hospitals, now Memorial Sloan-Kettering Cancer Center. Twelve patients were considered to have their initial therapy begun or completed at our institution. Eleven (91.7%) of 12 patients were found free of disease from 2 to 38 years (median 6 years). Nine patients were referred with recurrent disease. Only 4 (45%) could be salvaged 2 to 21 years (median 9 years). Because of the lower survival rate in the treatment of recurrent disease in this series, it is difficult to advocate overly conservative therapy initially in all early lesions. The survival rate depends on the original size of the tumor, initial adequate staging and appropriate therapy based on the extent of disease. Recommendations for a treatment protocol are proposed.     

64例髓母细胞瘤放疗的回顾性分析

目的分析髓母细胞瘤放疗疗效及预后影响因素,观察最常见的放疗副反应。方法回顾性分析接受放疗的髓母细胞瘤64例,年龄均≥4岁。术前中枢神经系统MRI检查无蛛网膜下腔播散迹象,属Chang’sM分期M0～M1期。所有病例均在外院接受了原发肿瘤的全切或次全切除手术。放疗先予全脑全脊髓照射30Gy,然后缩野至后颅窝局部加量20～25Gy,中位分次剂量为180cGy。结果所有病例均完成了放疗,全组3、5年总生存率分别为68.8%和55.7%,无病生存率分别为57.8%和51.4%,27例(42.2%)在3年内复发。通过分析年龄、性别、原发肿瘤大小、手术与放疗间隔时间对生存率影响发现,只有手术与放疗间隔时间有一定影响,手术与放疗间隔时间≤25、>25d的3年总生存率分别为81.5%、59.5%(P=0.110),3年无病生存率分别为74.1%、46.0%(P=0.030)。放疗中最常见的副反应主要为白细胞下降,92.2%的病例在治疗中出现了白细胞下降(<4.0×109/dl),其中2～3级的血液系统毒性占64.0%。结论髓母细胞瘤通过手术与放疗结合能取得较好疗效,但治疗失败率较高。手术与放疗间隔延长对总生存率和无病生存率均有影响,年龄、性别、原发肿瘤大小不是明显预后因素,放疗中最常见的副反应为血液毒性。     

Prognostic factors in women treated for ovarian yolk sac tumour: A retrospective analysis of 84 cases

Background

Ovarian yolk sac tumour (OYST) is a very rare malignancy arising in young women. Our study aimed to evaluate long-term outcomes and to identify prognostic parameters likely to help make appropriate risk-based decisions about therapy in this disease.

Methods

This retrospective study is based on prospectively recorded OYST cases at the Institut Gustave-Roussy. A univariate analysis using the logrank test evaluated possible associations between survival and patient or disease covariates. The multivariate analysis was performed using the Cox proportional hazard regression method.

Results

Between 1976 and 2006, 84 patients were registered. Since 1991, most of the patients have undergone fertility-sparing surgery. With a median follow-up of 71 months, the overall 5-year and event-free survival rates are 84% and 79%, respectively. In the multivariate model only the absence of ascites and a favourable serum AFP decline rate were significantly associated with better overall survival.

Conclusions

Patients with a poor prognosis factor such as an unfavourable serum AFP decline may be considered for aggressive treatment whereas those with good prognostic factors could be given less courses of chemotherapy.     

Carcinoid tumour of the orbital muscles: A rare occurrence

Malignant carcinoid tumours are rare and usually follow an indolent course. Metastases from these tumours are uncommon, and usually involve bone, liver and lymph nodes. Orbital metastases have rarely been described, and generally involve the choroid rather than orbital structures. Our knowledge of the response of these tumours to radiotherapy is extremely limited. Two cases of orbital metastases treated with radiotherapy are presented here and the literature on the efficacy of palliative radiotherapy for this tumour is reviewed. Recommendations are made regarding an effective radiotherapy regimen for treating orbital metastases from malignant carcinoid tumours.     

Clinical management of borderline tumours of the ovary: results of a multicentre survey of 323 clinics in Germany

The aim of this survey was to analyse the standard of care in diagnostic, surgery, chemotherapy and aftercare management for patients with borderline tumours of the ovary (BOTs) in Germany. A structured questionnaire comprising different dimensions was sent to all 1114 gynaecological departments. The questionnaire could be returned anonymously. The overall response rate was 29.0% (323 departments). Most departments were on secondary care (71.8%), tertiary care (23.2%) or university hospital (5.0%) level. Most clinicians performed not more than five BOT operations (89.2%) per year. Most departments (93.2%) used in addition to classical bimanual examination and vaginal ultrasound, tumour marker CA-125 detection, CT scan, MRI or PET-CT techniques. Departments in university and tertiary care hospitals performed more often a fresh frozen section (87 vs 64%). In young women, clinicians performed much seldom unilateral salpingo-oophorectomy (92%) and only in 53% biopsies of the contralateral ovary. Generally, biopsies of the contralateral ovary were performed in 4–53% of the patients. Chemotherapy was mostly favoured in ‘high-risk'' patients with tumour residual, microinvasion or invasive implants. Thus, a high grade of insecurity in diagnostic and therapy of BOT exists in some gynaecological departments and underlines the need for more educational and study activities.     

Dysgerminoma of the ovary: good prognosis even in advanced stages.

Dysgerminoma is a rare germ cell tumor of the ovary. It constitutes about 0.5% of all ovarian malignancies. During a 12-year period (1978-1989), 22 patients were treated at the National Cancer Institute, Cairo, Egypt. According to the FIGO classification system, 4 patients were allocated to Stage I, 2 were Stage II, 13 were Stage III, and 3 were Stage IV. The 5-year actuarial survival rate for all patients was 76%. Conservative treatment in addition to retrieval therapy upon recurrence could achieve 100% survival rate in Stage I patients. Surgery followed by irradiation resulted in a 66% 5-year actuarial survival rate in Stage III and 67% in Stage IV. Retroperitoneal lymph nodes seemed to have a better survival than peritoneal tumor extension. The adoption of an elective irradiation policy to the mediastinum and supraclavicular area seemed to have a good influence on prognosis. However, the extent of surgery performed could not be shown to affect survival.     

Risk of epithelial ovarian tumors among women with polycystic ovary syndrome: A nationwide population-based cohort study

An association between polycystic ovary syndrome (PCOS) and epithelial ovarian tumors is biologically plausible as conditions inherent to PCOS such as excessive androgenic hormones, reproductive factors and obesity are also risk factors for these hormone-sensitive tumors. However, previous studies have showed conflicting results and have various methodological limitations. This population-based cohort study investigates the association between PCOS and epithelial ovarian tumors and includes all women born in Denmark between January 1, 1940 and December 31, 1993 (n = 1 719 304). PCOS diagnoses, ovarian cancer and borderline ovarian tumor diagnoses, covariates, migration and vital status were obtained from the Danish national registers. Adjusted cox proportional hazard regression models were used to calculate hazard ratios (HRs) and 95% confidence intervals (CI) for epithelial ovarian cancer and for borderline ovarian tumors overall as well as for histological subtypes separately. During median 26 years of follow-up we identified 6490 women with ovarian cancer and 2990 women with borderline ovarian tumors. Overall, we observed no marked associations between a diagnosis of PCOS and overall epithelial ovarian cancer or overall epithelial borderline ovarian tumors, irrespective of time since diagnosis. However, we found an increased risk of ovarian cancer among postmenopausal women with PCOS (HR 2.28 95% CI 1.02-5.09) and an increased risk of serous borderline ovarian tumors (HR 2.34 95% CI 1.21-4.53) in women with PCOS compared with women without PCOS. Importantly, low statistical precision is a crucial limitation of our study and in previous studies and larger studies with longer follow-up are therefore warranted.     

Response to chemotherapy of solitary fibrous tumour: A retrospective study

BackgroundTo report on anthracycline-based chemotherapy in a retrospective case-series analysis of solitary fibrous tumour (SFT) patients treated within the Italian Rare Cancer Network.Patients and methodsWe reviewed a set of SFT treated with chemotherapy since 2002, focusing on anthracycline, administered alone or in combination with ifosfamide. Responses to ifosfamide as a single agent were also evaluated. Pathologic diagnosis was centrally reviewed, distinguishing typical, malignant (MSFT) and dedifferentiated (DSFT) subtypes.ResultsAmong 42 SFT patients treated with chemotherapy, we selected 31 cases (mean age: 62 years; locally advanced/metastatic: 13/18; front-line/further line: 25/6; typical/MSFT/DSFT/not assessable: 1/17/12/1) who received anthracycline-based chemotherapy (anthracycline monotherapy: eight; anthracycline + ifosfamide: 23). 30 patients are evaluable for response. Best response by Response Evaluation Criteria in Solid Tumours (RECIST) was: partial response (PR): 6 (20%), stable disease (SD): eight (27%), progressive disease (PD): 16 (53%) cases. Responses were confirmed after 3 months. Median progression-free survival (PFS) was 4 (range 2–15) months, with 20% of patients being progression-free at 6 months. PR was found in 2/18 (11%) MSFT and 4/12 (30%) DSFT, with a median PFS of 3.5 and 5 months in MSFT and DSFT, respectively. 19 patients received high-dose prolonged-infusion ifosfamide (front-line/further line: 11/8; typical/MSFT/DSFT: 0/15/4) with two (10%) PR, five (26%) SD, 12 (63%) PD.ConclusionsThis retrospective series suggests that in SFT anthracyclines have a degree of antitumour activity in the range of soft tissue sarcoma chemotherapy. Ifosfamide monotherapy seemed to have lower activity. A higher response rate was observed in DSFT in comparison to MSFT. Studies on targeted therapies are ongoing.     

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.     

Dysgerminoma of the ovary with rhabdomyosarcoma. Report of a case

A 14-year-old girl with a large left ovarian mass underwent excision of the tumor which on histologic examination revealed dysgerminoma mixed with elements of rhabdomyosarcoma. A subsequent staging laparotomy revealed a solitary 1-cm nodule along the pelvic wall. This was composed of rhabdomyosarcoma. Review of literature revealed that soft tissue sarcomas of several types may on occasion be seen in association with gonadal and extragonadal mixed germ cell tumors or in spermatocytic seminoma of the testis. However, no previously published report of a sarcoma arising in a pure dysgerminoma was found in the literature.     

上皮-肌上皮癌的临床特点与疗效分析

目的 分析上皮-肌上皮癌患者局部区域复发风险和长期生存的相关因素。方法 回顾分析1999—2015年收治的18例首程治疗或首程辅助治疗于本中心完成的上皮-肌上皮癌患者生存情况。单纯手术8例(44%),术后辅助放疗9例(50%),根治性同步放化疗1例(6%)。比较各组局部区域复发率和生存率。Kaplan-Meier法计算生存率,logrank法检验。结果 中位随访时间46个月,全组5例(27.8%)患者LRR,5年LRFS率为69%,5年OS率为93%。辅助放疗组5年LRFS优于单纯手术组,但未达统计学差异(71%∶57%,P=0.569)。结论 局部区域失败是上皮-肌上皮癌治疗的主要失败模式,进一步提高LC率是改进生存的关键。     

Adenocarcinoma of the uterine cervix: A study of 37 cases

In a study of 37 patients diagnosed with cervical adenocarcinoma between 1961 and 1994, clinical and pathologic findings were evaluated. Of the 37 patients, 27 (73%) had a pure adenocarcinoma, five (13.5%) had a collision tumor and five (13.5%) had an adenosquamous carcinoma. Twenty-six patients (70.3%) were diagnosed in Stage I, and II (29.7%) patients in Stage II, III, and IV. Two patients (5.4%) were treated with simple hysterectomy alone, nine (24.3%) with simple hysterectomy followed by radiotherapy, eight (21.6%) with radical hysterectomy alone, five (13.5%) with radical hysterectomy followed by radiotherapy, nine (24.3%) with radiotherapy alone, one (2.7%) with radiotherapy followed by simple hysterectomy, and three (8.1%) received no treatment. The actuarial 5-year survival rate was 69%. It is suggested that for patients with small early-stage disease, radical hysterectomy should be primary treatment and postoperative adjuvant radiotherapy would be advocated if high-risk features are histologically demonstrated. For all other patients, radiotherapy should be primary treatment. © 1996 Wiley-Liss, Inc.     

External Beam Radiotherapy in the Treatment of Gastroenteropancreatic Neuroendocrine Tumours: A Systematic Review

Aims

External beam radiotherapy (EBRT) is infrequently used to treat gastroenteropancreatic neuroendocrine tumours (GEPNETS), with little published data to date. We carried out a systematic review to assess the activity of EBRT for GEPNETS.

Male breast cancer: A retrospective study from a regional cancer center in northern India

Over a 7-year period from 1987 to 1993, 41 male breast cancer patients were seen in the breast cancer clinic of the Institute Rotary Cancer Hospital (IRCH) at the All India Institute of Medical Sciences (AIIMS). Their mean age was 54.2 years; and duration of symptoms ranged from 1 to 84 months with a mean of 15.1 months. Breast lump was the commonest presenting symptom. Fine needle aspiration cytology (FNAC) was the commonest diagnostic procedure. The TNM stage distribution was stage I, 5; stage II, 13; stage III, 17; and stage IV, 6. Radical mastectomy (25/36) was the commonest surgical procedure. Locoregional radiotherapy was given in 15 patients. Thirty patients received systemic adjuvant therapy (chemotherapy or tamoxifen, or a combination of the two). Local or distant recurrence occurred in 8 patients (8/31, 28.3%). Actuarial overall and disease-free survival was 100% and 80.1% at 2 years and 91.7% and 66.7% at 4 years, respectively. On univariate analysis, axillary lymph node status and age were found to affect disease-free survival significantly. Advanced stage of disease at presentation is common in Indian patients and will continue to influence treatment policies. Neoadjuvant chemotherapy needs to be evaluated for locally advanced tumors to improve outcome. Multicentric studies are necessary to define the relative roles of tamoxifen and chemotherapy for adjuvant treatment. © 1996 Wiley-Liss, Inc.     

Clinical Analysis of 57 Patients with Ovarian Dysgerminoma

Objective  Ovarian dysgerminoma is an uncommon ovarian malignancy, Its clinicai features are special and there are many factors affecting its prognosis. If treated properly, the patient can be cured. Otherwise it may endanger the patient’s life. The aim of this study is to investigate the clinical features and factors related to prognosis of ovarian dysgerminoma. Methods  Data from 57 patients with pure ovarian dysgerminoma were analyzed retrospectively. The patients were admitted to the Cancer Center, Sun Yat-sen University from January 1.1964 to December 31, 2000. Results  The main clinical features were abdominal mass (56.1% ), abdominal pain (21.1% ), abdominal swelling (17.5%.), vaginal bleeding (5.3% )and genital tract abnormalities (5.3%). Twenty-six patients had stage I diseases, 8 stage II.9 stage III.1 stage IV and 13 recurrent and persistent diseases. The uterus was involved in 41.2% of patients with stage II -III diseases. Combined modality was given to 52 cases and a single-method treatment to 5 cases. The total overall 5 and 10-year survival rates for stages I-IV was 80.1 % and 70.0% respectively. The 5-year survival rate for stage I was 100%, stage II 55.2%. stage III 55.6% and stage IV 0%; for recurrent and persistent diseases, 72.7%. The stage I group of 12 patients. received adnexectomy and 14 patients underwent hysterectomy and adnexa removal. There was no significant difference between the 5 and 10-year survival rates (all 100%). Of the 23 patients in the stage I group to whom oniy chemotherapy was given after operation, 19 cases received 3 or more courses and were well without recurrence; 4 patients received only one course and one of them recurred 21 months after the operation. In the group of stages II and III cases, the 5-year survival rate was 86.7% for those whose chemotherapy courses were 3≥ 4 and 25.0% for patients who received less than 4 courses of chemotherapy (P<0.05). Conclusions  The prognosis of ovarian dysgerminoma is closely related to the disease stage and treatment modality. A fertility-preserving operation can be considered in early -staged patients, but caution needs to be exercised in the middle to late staged cases. Good results can be achieved with an operation-based combined modality in recurrent patients.