Study of neoadjuvant chemotherapy in advanced malignant ovarian germ cell tumors at a tertiary center in western India.

OBJECTIVE: To study clinical characters and outcomes in patients of malignant ovarian germ cell tumor (MOGCT) undergoing surgery following neoadjuvant chemotherapy (NACT). METHODS: Retrospective study of patients undergoing surgery following NACT for MOGCT at our institute. Platinum based chemotherapy was given in all patients in NACT. RESULTS: Between March 2013 and February 2023, 30 patients had surgery after NACT. Patient's median age was 22 years (range, 12 to 35 years) and median follow up 42months (range, 6 to 132 months). Majority had endodermal sinus tumor (n=12), dysgerminoma (n=9) and mixed GCT (n=7). All had either International Federation of Gynecology and Obstetrics (FIGO) stage 3 (n=19) or FIGO stage 4 disease (n=11). Complete response to NACT seen in 5 patients and 23 patients had partial response. Fertility sparing surgery in 18 patients and complete surgery in 12 patients. Suboptimal surgery was seen in 4 patients. Currently, 20 of 30 patients are alive and disease free, 3 lost for follow up and 7 patients had progression after adjuvant therapy. Five patients had mortality-4 with progression and 1 with bleomycin toxicity. Fifteen of 17 eligible patients have resumed menstruation and one had successful pregnancy. Prognostic factors noted in study are stage, optimal surgery and viable tumor in histopathology. Dysgerminoma had better outcome than other histology. CONCLUSION: NACT may be a reasonable option in patients with extensive unresectable disease or in whom fertility sparing is not possible or in the poor general condition. Fertility sparing surgery can be attempted post neoadjuvant chemotherapy without adversely affecting prognosis.

Disgerminoma ovariano na gestação: relato de caso / Ovarian dysgerminoma in pregnancy: a case report

Os disgerminomas são tumores malignos de células germinativas ovarianas, são raros, geralmente acometem mulheres em idade fértil e têm bom prognóstico e sobrevida elevada. Paciente de 20 anos, primigesta com 26 semanas de gestação, foi admitida no centro obstétrico da Fundação Hospitalar Santo Antônio em Blumenau- SC com quadro de dor abdominal intensa refratária à analgesia e desconforto respiratório. Ressonância magnética demonstrou derrame pleural, moderada ascite e volumosa lesão expansiva de aspecto sólido-cístico em anexo direito. Foram realizadas salpingo-oforectomia à direita e omentectomia parcial e coletado lavado peritoneal. Anatomopatológico evidenciou disgerminoma. A paciente seguiu acompanhamento gestacional nos serviços de pré-natal de alto risco e oncologia. Devido à imaturidade fetal, manteve-se conduta expectante e, após o parto normal com 37 semanas, foi realizado estadiamento e iniciada quimioterapia adjuvante. Devido à baixa incidência e à raridade de tumores de células malignas ovarianas, relatos de casos como este são importantes para discutir as melhores estratégias de manejo clínico.(AU)

Dysgerminomas are rare malignant ovarian germ cell tumors that generally affect adolescence and early adulthood, have a good prognosis and high survival. Patient 20 years old, gestation 1, at 26 weeks of gestation, was hospitalized at the obstetric center of Fundação Hospitalar Santo Antônio in Blumenau-SC, with severe abdominal pain refractory to analgesia and respiratory discomfort. Magnetic resonance showed pleural effusion, moderate ascites and a massive expansive lesion with a solid cystic aspect in the right ovary. Right salpingoophorectomy, partial omentectomy and peritoneal lavage were collected. Anatomopathological evidence showed dysgerminoma. Patient followed gestational follow-up at high-risk prenatal and oncology services. Due to fetal immaturity, expectant management was maintained and after vaginal delivery at 37 weeks, staging was performed and adjuvant chemotherapy was started. Due to the low incidence and rarity of ovarian malignant cell tumors, case reports such as this one are important to discuss the best clinical management strategies.(AU)

Need for risk-adapted therapy for malignant ovarian germ cell tumors: A large multicenter analysis of germ cell tumors' patients from French TMRG network.

BACKGROUND: Malignant ovarian germ cell tumors are rare tumors, affecting young women with a generally favorable prognosis. The French reference network for Rare Malignant Gynecological Tumors (TMRG) aims to improve their management. The purpose of this study is to report clinicopathological features and long-term outcomes, to explore prognostic parameters and to help in considering adjuvant strategy for stage I patients. PATIENTS AND METHODS: Data from patients with MOGCT registered among 13 of the largest centers of the TMRG network were analyzed. We report clinicopathological features, estimated 5-year event-free survival (5y-EFS) and 5-year overall survival (5y-OS) of MOGCT patients. RESULTS: We collected data from 147 patients including 101 (68.7%) FIGO stage I patients. Histology identifies 40 dysgerminomas, 52 immature teratomas, 32 yolk sac tumors, 2 choriocarcinomas and 21 mixed tumors. Surgery was performed in 140 (95.2%) patients and 106 (72.1%) received first line chemotherapy. Twenty-two stage I patients did not receive chemotherapy. Relapse occurred in 24 patients: 13 were exclusively treated with upfront surgery and 11 received surgery and chemotherapy. 5y-EFS was 82% and 5y-OS was 92.4%. Stage I patients who underwent surgery alone had an estimated 5y-EFS of 54.6% and patients receiving adjuvant chemotherapy 94.4% (P < .001). However, no impact on estimated 5y-OS was observed: 96.3% versus 97.8% respectively (P = .62). FIGO stage, complete primary surgery and post-operative alpha fetoprotein level significantly correlated with survival. CONCLUSION: Adjuvant chemotherapy does not seem to improve survival in stage I patients. Active surveillance can be proposed for selected patients with a complete surgical staging.

[Management of intra-abdominal dysgerminoma During pregnancy - A Case Report and Literature Review]. / Management Unterbauchraumforderungen in der Schwangerschaft ­ Fallbeschreibung eines extraovariell-intraabdominalen Dysgerminoms mit Überblick über die Literatur.

INTRODUCTION: Dysgerminomas are rare malignant germ cell tumors. They usually arise from the ovary, but case reports describing extraovarian dysgerminomas do exist. When treated adequately the disease has a good prognosis. Dysgerminomas diagnosed during pregnancy are very rare. METHODOLOGY: Report of extraovarian intra-abdominal dysgerminoma during pregnancy. Systematic literature review. CASE REPORT: A 35-year-old second gravida was diagnosed with a suspected intra-abdominal mass at 20 gestational weeks. During an exploratory laparotomy, a tumor infiltrating the transverse colon and histologically identified as a dysgerminoma was resected. Ovaries were clinically unremarkable. The induction of chemotherapy was postponed until after delivery. At 34 gestational weeks the patient underwent cesarean section and tumor debulking. Four cycles of bleomycin, etoposide and cisplatin were administered. After 12 months, cystic ovaries were found. Hysterectomy with bilateral adnexectomy was performed but no malignancy found. After 16 months, the patient was still in complete remission. CONCLUSION: We describe the first-ever published dysgerminoma in gravida primarily evolving intraabdominally and not affecting the ovaries. The decision for cytoreductive surgery, prolongation of pregnancy and postponing chemotherapy until after delivery combined the best benefit for the baby with a good maternal prognosis. Due to limited data regarding dysgerminomas in pregnancy, individual interdisciplinary concepts are mandatory.

A multicentre retrospective cohort study of ovarian germ cell tumours: Evidence for chemotherapy de-escalation and alignment of paediatric and adult practice.

BACKGROUND: Adult guidelines recommend BEP (bleomycin, etoposide, cisplatin) for all ovarian germ cell tumours, causing debilitating toxicities in young patients who will survive long term. Paediatricians successfully reduce toxicities by using lower bleomycin doses and substituting carboplatin for cisplatin, while testicular and paediatric immature teratomas (ITs) are safely managed with surgery alone. AIM: The aim was to determine whether reduced-toxicity treatment could rationally be extended to patients older than 18 years. METHODS: Multicentre cohort study was carried out in four large UK cancer centres over 12 years. RESULTS: One hundred thirty-eight patients were enrolled. Overall survival was 93%, and event-free survival (EFS) was 72%. Neoadjuvant/adjuvant chemotherapy (82% BEP) caused 27 potentially chronic toxicities, and one patient subsequently died from acute lymphoblastic leukaemia. There was no difference in histology, stage or grade in patients ≤/>18 years, and EFS was not different in these age groups (≤18:28% and >18:28%; log-rank P = 0.96). Histological subtype powerfully predicted EFS (log-rank P = 4.9 × 10-7). Neoadjuvant/adjuvant chemotherapy reduced future relapse/progression in dysgerminoma (n = 37, chemo:0% vs. no chemo:20%), yolk sac tumour (n = 23, 26.3% vs.75%) and mixed germ cell tumour (n = 32, 40%vs.70%) but not in IT (n = 42, 33% vs.15%). Additionally, we observed no radiological responses to chemotherapy in ITs, pathological IT grade did not predict EFS (univariate hazard ratio 0.82, 95% confidence interval: 0.57-1.19, P = 0.94) and there were no deaths in this subtype. CONCLUSION: Survival was excellent but chemotherapy toxicities were severe, implying significant overtreatment. Our data support the extension of reduced-toxicity, paediatric regimens to adults. Our practice-changing findings that IT was chemotherapy resistant and pathological grade uninformative strongly endorse exclusive surgical management of ovarian ITs at all ages.

Is carboplatin-based chemotherapy as effective as cisplatin-based chemotherapy in the treatment of advanced-stage dysgerminoma in children, adolescents and young adults?

OBJECTIVE: Dysgerminoma is the most common malignant ovarian germ cell tumor (GCT) with peak incidence during adolescence and young adulthood. Current standard of care for patients with disease that has spread outside of the ovary (advanced-stage) utilizes platin-based chemotherapy regimens. The study objective was to compare clinical outcomes between platin-based (carboplatin versus cisplatin) strategies across all age groups (children < 11 years (y), adolescents = 11-25 y and young adult women > 25 y) for advanced-stage dysgerminoma. METHODS: The Malignant Germ Cell Tumor International Consortium (MaGIC) pooled data from six GCT trials (3 = pediatric, 3 = adult) conducted internationally by pediatric and gynecologic oncology clinical trial organizations (CTOs) between 1983 and 2009. Newly diagnosed patients, with advanced-stage (FIGO IC-IV) dysgerminoma, who received either carboplatin- or cisplatin-based chemotherapy were eligible for analysis. RESULTS: 126 eligible patients were identified; 56 patients (38 = pediatric, 18 = adult) received carboplatin-based and 70 patients (50 = pediatric, 20 = adult) received cisplatin-based chemotherapy. Mean age was 20 y (range = 6-46 y). The median follow-up was 10.3 y (range = 0.17-21.7 y). The five-year event-free survival (EFS5) and overall survival (OS5) was 0.94 (95%CI, 0.88-0.97) and 0.96 (95%CI, 0.91-0.99) respectively. Survival outcomes were comparable between carboplatin-(EFS5 = 0.96 (95%CI, 0.85-0.99), OS5 = 0.96 (95%CI, 0.85-0.99)) and cisplatin-(EFS5 = 0.93 (95%CI, 0.83-0.97), OS5 = 0.96 (95%CI, 0.87-0.99)) based regimens. Across three age groups, comparison of the EFS5 (<11 y = 0.1, 11-25 y = 0.91 (95%CI, 0.82-0.96), >25 y = 0.97 (95%CI, 0.81-0.99)) and OS5 (<11 y = 0.1, 11-25 y = 0.95 (95%CI, 0.87-0.99), >25 y = 0.97 (95%CI, 0.81-0.99)) did not demonstrate any statistically significant differences in outcomes. CONCLUSIONS: Patients diagnosed with dysgerminoma have an excellent OS, across all ages, even in the context of metastatic disease. Data from three large CTOs supports the investigation of carboplatin-based regimens in the frontline treatment of all patients with advanced-stage dysgerminoma to minimize treatment-related toxicities.

Bleomycin-induced flagellate hyperpigmentation.

Bleomycin is a chemotherapeutic agent used in the treatment of different tumours. It has several side effects, including flagellate hyperpigmentation, which is a unique and a well-documented side effect of bleomycin therapy. We report a case of a 23-year-old woman with a personal history of ovarian dysgerminoma, who developed flagellate hyperpigmentation on trunk after bleomycin therapy.

Mediastinal mass following successful chemotherapy for ovary dysgerminoma: benign process or disease relapse? A case report.

BACKGROUND: Ovarian dysgerminoma is a rare tumor that affects adolescent girls and young women. Due to its high radio-chemosensitivity, prognosis is normally excellent. Relapses occur in less than 20% of early stage disease, but are more frequent in advanced disease. It is known that some benign mediastinal processes may mimic tumor relapse, particularly in young patients. This is the case of physiologic thymic hyperplasia, which occurs as a rebound phenomenon after chemotherapy in young women with ovarian dysgerminoma. Until now, no cases of dysgerminoma with benign mediastinal mass have been published. CASE: A young woman with bulky ovarian dysgerminoma, who obtained complete disease remission after chemotherapy, subsequently developed a mediastinal mass which was initially confused with a mediastinal relapse. CT scan features (close thymic location, homogeneous hypodensity, absence of infiltration of mediastinal structures) and subsequent PET/CT scan (homogeneous glucose uptake and a typical inverted V morphology) supported the diagnosis of thymic hyperplasia. No further invasive procedures were performed. 34 months from the diagnosis the patient is in good physical condition with no signs of relapse. CONCLUSIONS: Our case underlines the importance of knowing the age- and treatment-related incidence of physiologic thymic hyperplasia in young women with ovarian dysgerminoma in order to reduce the potential pitfalls and to avoid unnecessary invasive diagnostic procedures.

Bilateral synchronous testicular germ cell tumours in a patient with bilateral cryptorchidism.

Bilateral testicular tumours are rare, and 80% of bilateral tumours are metachronous. The incidence of testicular tumours is high in cryptorchidism. Synchronous bilateral testicular tumours are rare, and bilateral synchronous testicular tumours in bilateral cryptorchidism extremely rare, probably not reported previously.

Residual tumor after the salvage surgery is the major risk factors for primary treatment failure in malignant ovarian germ cell tumors: a retrospective study of single institution.

BACKGROUND: Malignant ovarian germ cell tumors are rare, and knowledge of their prognostic factors is limited, with little available randomized data. This study was conducted to evaluate the clinicopathologic characteristics of malignant ovarian germ cell tumors and to determine the association of their prognostic factors to primary treatment failure. METHODS: The medical records of 57 patients with stages I to IV malignant ovarian germ cell tumor were retrospectively reviewed, and their clinicopathologic and treatment-related data were collected and analyzed. RESULTS: The median age at the diagnosis was 23.3 years (range: 8-65 years), and the median follow-up period was 108 months (range: 48-205 months). The histological types of the tumors were immature teratoma (n = 24), dysgerminoma (n = 20), endodermal sinus tumor (n = 8), mixed germ cell tumor (n = 4), and choriocarcinoma (n = 1). 66.7% of the patients had stage I disease; 5.2%, stage II; 26.3%, stage III; and 1.8%, stage IV. After the initial surgery, 49 patients (86%) received cisplatin-based chemotherapy. The five-year survival rate was 96.5%. There were six primary treatment failures, with two of the patients dying of the disease, and the median time to the recurrence was 8 months. The histological diagnosis (P < 0.0001), tumor stage (P = 0.0052), elevation of beta-hCG (P = 0.0134), operation methods (P = 0.0006), and residual tumor after the salvage surgery (P < 0.0001) were significantly associated with the risk of primary treatment failure in the univariate analysis. In the multivariate analysis, the residual tumor after the salvage surgery was the only significant variable associated with primary treatment failure (P = 0.0011, Hazard ratio = 29.046, 95% Confidence interval 3.832-220.181). CONCLUSION: Most malignant ovarian germ cell tumors have excellent prognoses with primary treatment, and good reproductive outcomes can be expected. Because primary treatment failure is associated with the residual disease after the salvage surgery, knowledge of the presence or absence of this risk factor may be helpful in risk stratification and individualization of adjuvant therapy in malignant ovarian germ cell tumors. Further large-scale prospective studies to confirm these results should be performed.

Ovarian dysgerminoma associated with fibrosarcoma: a case report.

A 35-year-old woman presented with abdominal pain and weight loss. Pelvic computed tomography showed a 15 cm mass in the left ovary. Grossly, the removed ovary was completely replaced by a solid tumor mass. On histological analysis (100 sections), the lesion showed the typical morphological features of dysgerminoma (20%) admixed with a major (80%) fibrosarcoma component. Tumors did not have well-demarcated boundaries with a close intermingling of both cell types. Despite surgery and combination chemotherapy, the disease progressed rapidly and the patient died of disease 18 months after diagnosis. Review of the literature showed that soft tissue sarcomas of several types may occasionally be associated with gonadal and extragonadal mixed germ-cell tumors or with spermatocytic seminoma of the testis. However, no previously published report of an ovarian fibrosarcoma associated with a pure dysgerminoma was found in the literature.

Malignant germ cell tumors associated with Swyer syndrome.

Swyer syndrome is characterized by a higher risk of developing genital malignancies. In this disorder, the most common is gonadoblastoma and dysgerminoma but also, in rare cases, choriocarcinoma. The prognosis in choriocarcinoma is poor. The early diagnosis of dysgenetic gonads is necessary in view of the risk of malignancies. It can be difficult due to its different clinical masks. When the neoplasm precedes the diagnosis of gonadal dysgenesis, adequate oncological treatment should be introduced with parallel gonadectomy. We present a case of 14-year-old female with 46, XY karyotype with choriocarcinoma in one gonad and dysgerminoma in the second one.

Atypical presentation and management dilemma of mixed gonadal dysgenesis.

OBJECTIVE: To describe a case of 45,X/46,XY mixed gonadal dysgenesis complicated by malignancy with possible metastasis. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A 15-year-old female with primary amenorrhea, short stature, and a vaginal septum. INTERVENTION(S): Resection of transverse vaginal septum and laparoscopic bilateral gonadectomy. RESULT(S): The patient had dysgerminoma arising from gonadoblastoma in the left gonad and gonadoblastoma in the right gonad. No normal gonadal tissue could be identified. Postoperative computed tomography scan results were suspicious for lung metastases, but the patient opted for conservative management without chemotherapy. CONCLUSION(S): Mixed gonadal dysgenesis involves inherent malignancy risk and complex psychosocial issues, which necessitate a multidisciplinary approach to diagnosis and treatment.

Para-aortic lymph node metastasis in malignant dysgerminoma of the ovary.

Dysgerminomas comprise approximately 2-5% of all ovarian malignancies and mostly affect young adolescent women. Primary comprehensive surgery and adjuvant chemotherapy consisting of bleomycin, etoposide, and cisplatin (BEP) are the current recommended treatment options, the latter reserved for advanced stages (FIGO II-IV). We report two patients aged 20 and 26 years who presented with an initial FIGO stage IA, but inadequately assessed. Both were subsequently diagnosed with recurrent malignant dysgerminoma and para-aortic lymph node metastasis. Neither had received comprehensive staging at initial surgery nor adjuvant radio or chemotherapy. Both needed extensive surgery and multiagent chemotherapy for survival and belong to the small percentage of FIGO IA dysgerminoma patients showing a relapse. Comprehensive initial surgery including systematic para-aortic lymphadenectomy and adjuvant chemotherapy at tertiary referral centers is needed to minimize the treatment burden.

Nodular lung schistosomiais lesions after chemotherapy for dysgerminoma.

We report an unusual case of pulmonary schistosomiasis in a traveler to Mali that was diagnosed 16 months after primary infection, one month after she finished chemotherapy for a malignant tumor. Serologic analysis showed marked eosinophilia. Our case emphasizes the need to detect parasitic infections in cancer patients with unexplained eosinophilia, particularly in immigrants and travelers to tropical countries.

[A case of PEP(BEP)-resistant ovarian dysgerminoma successfully treated by VeIP therapy].

Ovarian germ cell tumors are malignant tumors which commonly develop during childhood, and which are sensitive to chemotherapy. We have had a case of germ cell tumors which showed resistance to first-line PEP(BEP)chemotherapy. As second-line chemotherapy, VeIP therapy was used, because it is possible that this therapy is effective against recurrent testicular germ cell tumors. The patient was fourteen years old. She experienced acute abdominal pain and visited the hospital, where she was diagnosed with torsion of an ovarian tumor. An emergency laparotomy and right salpingoophorectomy were performed, the pathological diagnosis being stage Ia ovarian dysgerminoma G1. She was followed for two years until her serum hCG-CTP elevated to 1.4 mIU/mL. An MRI revealed an abnormal signal in the left ovary, so we diagnosed this as a recurrence of the dysgerminoma. Then she received chemotherapy PEP(BEP), but after eight months of PEP (BEP), her serum hCG-CTP was again elevated to 14.5 mIU/mL. A recurrence was detected with an MRI and PET-CT, and another laparotomy was performed. The recurrent region was detected in the left ovary. A left ovarian cystectomy was performed in which CDDP ip was used. After the operation, the patient again underwent chemotherapy. VeIP (vinblastine+ifosfamide+cisplatin)was chosen as the second-line regimen. After 6 courses of this therapy, she had a follow-up operation. No recurrence region was found in the pelvic area. She remains without recurrence of this disease 24 months after VeIP therapy. This case suggests that VeIP therapy might be an effective second-line therapy for patients with PEP(BEP)-resistant ovarian dysgerminoma.

Trombosis venosa cerebral en un paciente con disgerminoma en tratamiento quimioterápico / Cerebral venous thrombosis in a chemotherapy patient with dysgerminoma

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