scholarly journals GERM CELL TUMOURS OF THE OVARY IN CHILDREN AND ADOLESCENTS: A CLINICAL STUDY OF 109 PATIENTS IN A SPECIALIZED CANCER CENTRE

2018 ◽  
Vol 4 (2) ◽  
Author(s):  
Irfan Ul Islam Nasir ◽  
Muhammad Fahd Shah ◽  
Awais Amjad Malik ◽  
Abdul Wahid Anwer ◽  
Amir Ali Syed ◽  
...  

Objective: Paediatric ovarian germ cell tumours (GCTs) are rare tumours withmalignant tumours extremely rare.Methods: All the paediatric patients who received treatment for histology proven ovarian GCT at Shaukat Khanum Memorial Cancer Hospital from January 2006 to December 2014 were retrospectively reviewed. Patients over the age of 18 years were excluded from the study. A total of 109 patients were included in the study. A set of parameters were identified to record initial clinical presentation and examination, imaging and laboratory investigations including tumour marker levels. Decisions of multidisciplinary team meetings, surgical treatment, neo adjuvant, adjuvant chemotherapy and radiation data retrieved. Data analysiscarried out using SPSS 20.Results: In total 109 girls presented to our hospital during the study period, most of them above the age of 5 years, with dysgerminoma being the most common followed by yolk sac tumour. Most of the patients received treatment outside our hospital and were referred here for chemoradiotherapy. Fertility preserving surgery was the most commonly performed surgical procedure with a mean follow-up of 50.4 months and >75% overall 5-year survival.Conclusion: Regardless of histologic types, the outcomes of GCT can be improved with a multidisciplinary approach.Key words: Dysgerminomas, germ cell tumours, ovarian tumours

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi210-vi210
Author(s):  
Bo Li ◽  
Jiayi Wang ◽  
Youqi Li ◽  
Xiaoguang Qiu

Abstract PURPOSE Intracranial germ cell tumours (iGCTs) originating from the basal ganglia/thalamus (BG/T) region are rare. We report the findings from a large single-centre series to explore the clinical features of this disease. PATIENTS AND METHODS Patients diagnosed as having BG/T iGCTs from January 2000 to December 2017 at our hospital were screened. Newly diagnosed patients who had histology-proven/tumour marker-elevated disease and received at least radiotherapy were eligible for analysis. RESULTS Of the 401 patients screened, 211 were eligible. The median age was 12 years (range, 5–41 years). The median duration of follow-up for surviving patients was 68 months (range, 12–189 months). In germinoma patients (n = 112), the 5-year disease-free survival (DFS) and overall survival (OS) were 94.9% and 98.2%, respectively. Multivariate analysis showed that the dose of radiotherapy could predict the DFS (hazard ratio [HR] 0.991, 95% confidence interval [CI] 0.983–0.998, p = 0.015). In non-germinomatous GCT (NGGCT) patients (n = 99), the 5-year DFS and OS were 83.8% and 88.6%, respectively. Multivariate analysis showed that irradiation field (whole-brain radiotherapy vs. local radiotherapy, HR = 0.163, 95% CI 0.031–0.897, p = 0.037; cranial spinal radiotherapy vs. local radiotherapy, HR = 0.095, 95% CI 0.009–1.031, p = 0.053) and chemotherapy cycle (>3 vs. ≤3) (HR 0.234, 95% CI 0.055–0.996, p = 0.049) were independent factors for DFS. The median Z-score for height and body mass index (BMI) in patients ≤19 years of age (n = 69) at the last follow-up were -0.45 (range, -2.67 to 3.41) and 0.59 (range, -4.73 to 3.1), respectively. CONCLUSION BG/T GCTs had unique characteristics. Radiation dose was an independent factor influencing the DFS in cases of germinoma. In NGGCTs, extended field radiation and more chemotherapy cycles yielded better disease control. Physical development after treatment was within the normal range for most patients.


2020 ◽  
Vol 121 (3) ◽  
pp. 181-193
Author(s):  
Zeynep Oruc ◽  
Senar Ebinç ◽  
M. Ali Kaplan

Rare tumours of the testis includes a wide variety of tumours. We aim to present clinical and histological characteristics of our patients with rare tumours of the testis. The medical records of 33 patients who were treated and followed-up for testicular rare tumours in our center between 2007 and 2020 were retrospectively reviewed. Of all the 243 testicular tumours, 222 cases (91.4%) were germ cell tumours and 21 cases (8.6%) were non-germ cell tumours. Thirty-three rare tumours of the testis including rare germ cell tumours and non-germ cell tumours were detected. The mean age of the patients at diagnosis was 34 years (range 18–68 years). The histological types of rare testicular tumours were as follows: teratoma 4.5% (n=11), sex-cord stromal tumours 4.5% (n=11), paratesticular tumours 3.2% (n=8), and the others [lymphoma 0.4% (n=1), mesothelioma 0.4% (n=1) and choriocarcinoma 0.4% (n=1)]. The median duration of follow-up was 32 months (range 1 to 256 months). None of the patients with non-metastatic disease stage developed recurrence after having received appropriate therapy. Metastatic disease was documented in 9 cases at the time of diagnosis (five patients with teratomas, two patients with Leydig cell tumour, one patient with choriocarcinoma and rhabdomyosarcoma). The most common subtypes of testicular rare tumours in our center was teratoma and sex-cord stromal tumours. Because of testicular rare tumours have different biological features and different clinical outcomes, the management of each tumour requires a different approach.


2016 ◽  
Author(s):  
P. Veena

Introduction: Germ cell tumours comprise approximately 15-20% of all ovarian tumours. Two third of ovarian tumours in first two decades of life are germ cell tumours. Majority of ovarian germ cell tumours are benign teratomas. The malignant germ cell tumours are usually solid and arise from totipotent germ cells. Over the past 3 decades the clinical outcome of women with ovarian germ cell tumours (OGCT) have significantly improved mainly due to development of more effective chemotherapy regimens. Objective: To study the clinic pathological features, treatment and survival of women with ovarian germ cell tumours. Methods: This is a retrospective descriptive study taken from the case files of patients with histo-pathologically proven ovarian germ cell tumours who were treated in JIPMER over 8 years from 2007 to 2014. Results: There were totally 63 patients with ovarian germ cell tumours over 8 years who were treated in JIPMER. The age at presentation varies from 12 years to 65 years with a median age of 26.5 years. Three were pre pubertal and 1 was post-menopausal. Twenty two women (34%) were unmarried and 5 were pregnant at the time of presentation. Forty eight (76%) of them did not have any menstrual abnormalities. Pain abdomen (55%) was the most common presentation. Ten of them presented with acute abdomen of which 8 were torsion, 1 was ruptured dermoid and 1 was infected dermoid. Another 6 patients had torsion which was diagnosed only during surgery. Majority (68%) were benign tumours (dermoid) and among malignant tumours, there were 6 dysgerminomas, 5 immature teratomas, 5 mixed germ cell tumours and 4 yolk sac tumours. Almost half (22 out of 43) of women with benign tumours were <25 years whereas 3/4th (14 out of 20) of women with malignant germ cell tumours were <25 years. The most common tumour marker which was elevated was alpha feto protein (8) followed by LDH (5). Fertility sparing surgery (salpingo-ovariotomy) was commonly performed which was 95% (41/43) in benign tumours and 60% (8/20) in malignant tumours. Contra lateral ovary was biopsied in only 5 patients with suspected involvement (negative on final HPR). Out of 20 women with malignant ovarian tumours 7 were in advanced stage (Stage III). Majority of them recovered well from surgery, only 12% had post-operative febrile morbidity and one patient had subclavian vein thrombosis on post op D9 which required anticoagulants. 7 of 20 women received chemotherapy (BEP) for 4 cycles. No serious side effects of chemotherapy were noted in these women. 3 out of 20 women with malignant germ cell tumour were lost to follow up. No recurrences have been found in rest of the women and there are no deaths till last follow up. Conclusion: Advances in the field of medicine like effective chemotherapy regimens, improved imaging, precise surgical staging and fertility sparing surgical procedures enable women not only to preserve the reproductive function but also to improve their quality of life.


2007 ◽  
Vol 100 (1) ◽  
pp. 30-32 ◽  
Author(s):  
Ramachandran Venkitaraman ◽  
Bernadette Johnson ◽  
Robert A. Huddart ◽  
Chris C. Parker ◽  
Alan Horwich ◽  
...  

2010 ◽  
pp. 461-467
Author(s):  
George Samandouras

Chapter 8.14 covers germ cell tumours, including germinoma, embryonal carcinoma, yolk sac tumour, choriocarcinoma, and teratomas.


2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi197-vi197
Author(s):  
Bo Li ◽  
Youqi Li ◽  
Jiayi Wang ◽  
Xiaoguang Qiu

Abstract BACKGROUND Growth problems are common in patients with intracranial germ cell tumours. However, the characteristics were not fully profiled, especially when stratified by orientation. Thus, we conducted this study. METHODS Patients newly diagnosed, ≤19 years, with confirmed pathology or tumour markers elevation were included. Patients with bifocal lesions or lost to follow-up were excluded. WHO AnthroPlus software, which was developed for the global application of the WHO Reference 2007 to monitor growth in children aged 5–19 years, was used. Based on age, sex, and height, the Z-scores of height(ZSOH) were calculated. A ZSOH< -1 indicates a slower physical development than that in peers, while a ZSOH >1 indicates faster growth than peers. RESULTS Among the 200 included patients, 75 had primary lesions originating from the sellar/suprasellar region(S/SS), 73 from the pineal gland(PG), and 52 from the basal ganglia/thalamus region(BG/T). At initial diagnosis, the median ZSOH in S/SS was -0.66(-3.76–3.05), which was significantly lower than that in PG(0.76,-2.23–5.19) and BG/T(0.64,-3.05–5.32)(p=0.001). However, the difference in ZSOH between PG and BG/T was comparable(p=0.61). In patients with S/SS who had paired data(n=36), the median ZSOH decreased from -0.22(-3.76–3.05) at diagnosis to -1.01(-3.42–1.89) at the last follow-up(p=0.001). The median ZSOHs in PG(n=38) and BG/T(n=28) remained in the normal range at last follow-up although the changes were statistically significant(p< 0.001). Linear regression analysis showed that, in S/SS, age at diagnosis(β=0.115,p=0.033), sex(β=1.337,p=0.002), radiotherapy dose(β=-0.061,p=0.007), and number of chemotherapy cycles(β=-0.177,p=0.038) were correlated with changes in median ZSOH. In BG/T, only the radiotherapy dose(β=-0.064,p=0.041) and number of chemotherapy cycles(β=-0.551,p=0.026) were correlated. However, none of the above were validated in PG. CONCLUSIONS Compared to intracranial germ cell tumours in the PG or BG/T, the growth problems in S/SS was more prominent and aggravated after treatments, especially in those with younger age, female sex, higher radiotherapy dose, and more chemotherapy cycles.


2020 ◽  
Vol 13 (1) ◽  
pp. e232114
Author(s):  
Megha Kansara ◽  
Garima Yadav ◽  
Meenakshi Gothwal ◽  
Pratibha Singh

Yolk sac tumours of the ovary are rare and highly malignant germ cell tumours, which comprise of only 10%–15% of all malignant germ cell tumours. They have various clinical presentations most common being subacute pelvic pain and feeling of lump but sometimes high-grade fever can be one of the rare presentations. Here, we present a case report of a 26-year-old nulliparous woman with 36 weeks gravid uterus size advanced stage yolk sac tumour of one ovary with fever as main clinical presentation and overt hypothyroidism. We did staging laparotomy with total abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy with multiple peritoneal biopsies. Postoperatively, we had started adjuvant chemotherapy. Since yolk sac tumours are highly aggressive tumours as they rapidly increase in size, their early diagnosis and appropriate surgical management is required particularly in young women where fertility sparing surgery is possible in early stage with good prognosis.


2014 ◽  
Vol 15 (13) ◽  
pp. 1442-1450 ◽  
Author(s):  
Karim Fizazi ◽  
Lance Pagliaro ◽  
Agnes Laplanche ◽  
Aude Fléchon ◽  
Josef Mardiak ◽  
...  

2014 ◽  
Vol 4 (2) ◽  
pp. 134-135
Author(s):  
Tumay Ipekci ◽  
Yigit Akin ◽  
Burak Hoscan ◽  
Ahmet Tunckiran

Testicular neoplasm usually occur in men aged between 15 and 35. These are solid organ tumours and also should be operated when there is a suspicious clinical findings. Testis tumours are levelled after histopathology evaluation. The medical, surgical and follow-up strategies of well know testis tumours, such as seminomas, non-seminom germ cell tumours, have been established. In case of testis tumours rare entities may occur as rhabdomyosarcoma.We here presented a rare case of   alveolar subtype of rhabdomyosarcoma in testis with long term follow-up.


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