PROBLEMATIC ISSUES IN THE EVALUATION OF PHENOTYPIC PROGNOSTIC MARKERS IN OVARIAN GERM-CELL TUMORS

Ovarian germ-cell tumors represent about 15-20% of all ovarian tumors. They are characterised with high histological and clinical heterogeneity. Ovarian germ-cell tumors can be both benign and malignant. Nowadays, the prognostic markers of ovarian germ-cell tumors are less studied. In particular, there is only limited information about the phenotypic determinants of the malignant transformation of benign germ-cell tumors, as well as lack of information about the relapse development after treatment of malignant germ-cell tumors. In presented review article, the detailed cahracteristics of ovarian germ-cell tumors and the problematic issues in the determination of their prognostic features are discussed.

Pure dysgerminoma of the ovary: a study of 31 cases

Malignant germ cell tumors of the ovary are rare tumors characterized by their heterogeneity and occurring mostly in young women. Dysgerminoma is the most common type of these tumors. This was a retrospective study of 31 patients with pure dysgerminoma of the ovary diagnosed in Salah Azaiez institute of Tunis in Tunisia between 1970 and 2012.The median age was 22 years old. Abdominal pain was the most complaint in 45.1% of cases. An abdomino-pelvic mass was found in 83.8% of cases. Surgery was performed in all patients. The median tumoral size was 13.7 cm. Sixty four-point five percent of the patients underwent a conservative surgery. The tumor was classified stage I in 51.6% of the cases, stage II in 9.7% of the cases, stage III in 35.5% of the cases and stage IV in 3.2% of the cases. Fourteen patients received platinum-based adjuvant chemotherapy, and 10 patients had a radiotherapy. We have noticed 2 cases of recurrence and 2 cases of metastasis. Five-year and ten-year overall survival was 89.4%. Five-year disease free survival was 85.2% and ten-year disease free survival was 66.3%. Dysgerminomas of the ovary have a good prognosis. The two significant prognostic factors are the stage and the postoperative residual disease.

Uncommon Metastasis of Ovarian Dysgerminoma: A Case Report and Review of the Literature

Ovarian malignant germ cell tumors (OMGCT) represent less than 10% of all ovarian tumors. Dysgerminoma is the most common malignant primitive germ cell tumor in young women, known for its curability and low propensity to invade and metastasize when diagnosed early. Herein, we report an unusual type of ovarian dysgerminoma (OD) metastasis with a brief review of the literature, lacking similar reported cases. To our knowledge, although there are several case reports of dysgerminoma metastases with variable anatomic location and presentation, vaginal metastasis has not been previously described. The local or systemic relapse together with local and distant metastasis is considered as an independent predictor of poor survival in patients with OD. In light of the absence of mutations status, our patient successfully responded to therapy. Currently, the patient remains in clinical remission. A specific follow-up plan is ongoing knowing that ovarian dysgerminomas tend to recur most often in the first 2–3 years after treatment.

Malignant Germ Cell Tumors and Their Precursor Gonadal Lesions in Patients with XY-DSD: A Case Series and Review of the Literature

The risk of gonadal germ cell tumors is increased over the lifetime of patients with XY-disorders of sex development (XY-DSD). The aim of this study was to evaluate clinical features and histopathological outcome after gonadectomy in patients with XY-DSD to assess the risk of malignant transformation to gonadal germ cell tumors. Thirty-five women treated for XY-DSD at our hospital between 2003 and 2020 were enrolled in this study. Twenty-seven (77%) underwent prophylactic gonadectomy, 10 (29%) at our department and 17 (48%) at external hospitals. Eight (23%) patients didn´t receive gonadectomy. Of the patients who underwent a surgical procedure at our hospital, two patients were diagnosed with a unilateral seminoma, one patient with a bilateral and one patient with a unilateral Sertoli cell adenoma. According to these findings, preventive gonadectomy in patients with XY-DSD should be taken into consideration. Guidelines concerning the necessity of gonadectomy to avoid malignant transformation are still lacking. The risk of malignant germ cell tumors from rudimentary gonads has not been investigated sufficiently to date, as it is mostly based on case series due to the rarity of the condition. In our study we retrospectively analyzed patients who partly underwent bilateral gonadectomy, aiming to fill this gap. Concerning the ideal point of time for gonadectomy, further studies with a higher number of patients are needed.

Could MicroRNAs Be Useful Tools to Improve the Diagnosis and Treatment of Rare Gynecological Cancers? A Brief Overview

Gynecological cancers pose an important public health issue, with a high incidence among women of all ages. Gynecological cancers such as malignant germ-cell tumors, sex-cord-stromal tumors, uterine sarcomas and carcinosarcomas, gestational trophoblastic neoplasia, vulvar carcinoma and melanoma of the female genital tract, are defined as rare with an annual incidence of <6 per 100,000 women. Rare gynecological cancers (RGCs) are associated with poor prognosis, and given the low incidence of each entity, there is the risk of delayed diagnosis due to clinical inexperience and limited therapeutic options. There has been a growing interest in the field of microRNAs (miRNAs), a class of small non-coding RNAs of ∼22 nucleotides in length, because of their potential to regulate diverse biological processes. miRNAs usually induce mRNA degradation and translational repression by interacting with the 3′ untranslated region (3′-UTR) of target mRNAs, as well as other regions and gene promoters, as well as activating translation or regulating transcription under certain conditions. Recent research has revealed the enormous promise of miRNAs for improving the diagnosis, therapy and prognosis of all major gynecological cancers. However, to date, only a few studies have been performed on RGCs. In this review, we summarize the data currently available regarding RGCs.

Gynecologic Malignancies in Children and Adolescents: How Common is the Uncommon?

The aim of this study is to assess the projected incidence and prognostic indicators of gynecologic malignancies in the pediatric population. In this population-based retrospective cohort study, girls ≤18 years with ovarian, uterine, cervical, vaginal and vulvar malignancies diagnosed between 2000 and 2016 were identified from the Surveillance, Epidemiology and End Results (SEER)-18 registry. The Kaplan–Meier method was used to analyze overall survival (OS). The age-adjusted annual incidence of gynecologic malignancies was 6.7 per 1,000,000 females, with neoplasms of the ovary accounting for 87.5%, vagina 4.5%, cervix 3.9%, uterus 2.5% and vulva 1.6% of all gynecologic malignancies. Malignant germ-cell tumors represented the most common ovarian neoplasm, with an increased incidence in children from 5–18 years. Although certain subtypes were associated with advanced disease stages, the 10-year OS rate was 96.0%. Sarcomas accounted for the majority of vaginal, cervical, uterine and vulvar malignancies. The majority of vaginal neoplasms were observed in girls between 0–4 years, and the 10-year OS rate was 86.1%. Overall, gynecologic malignancies accounted for 4.2% of all malignancies in girls aged 0–18 years and the histologic subtypes and prognosis differed significantly from patients in older age groups.