Based on their origin, ovarian tumors can be classified into three groups. Epithelial tumors, at about 60% of all tumors but making up 85% of the malignant ones, constitute the largest group; germ cell tumors (second group) and sex cord-stromal tumors (third group), together with metastatic tumors, make up the remainder [1] [2]. Dysgerminomas make up a third of malignant germ cell tumors, although they constitute only 2% of ovarian tumors [3]. Dysgerminomas correspond to the seminomas of the testis.

Dysgerminomas present with the general symptoms of adnexal tumors: tenderness and pain in the pelvic or lower abdominal area [1], feeling of fullness and early satiety, and urinary problems. Abdominal enlargement can be the consequence of hemoperitoneum or the presence of a large mass [3].

Usually present unilaterally - with about 10-20% being bilateral- and present as firm masses. These are fast growing tumors, although with excellent prognosis [3] [4].

Dysgerminoma mainly occurs in young women; those with abnormal gonads, for example with Swyer syndrome (XY gonadal dysgenesis), have an increased risk of developing dysgerminomas.

It is composed of undifferentiated germ cells, with occasional granulomas and lymphocytes being present. About five percent have syncytiotrophoblastic giant cells present, which secrete lactate dehydrogenase, alkaline phosphatase [3], and human chorionic gonadotropin (HCG) [2]. Thus, in these cases, serum HCG may be elevated, but otherwise hormonal problems are not characteristic of dysgerminomas [4].

• Virilization

  Certain subtypes may occasionally be associated with isosexual precocity, dysgenetic gonads, virilization, hyperthyroidism or carcinoid syndrome. [orpha.net]

  Sex cord-stromal tumors of the ovary 5–10% of all ovarian tumors Estrogen producing : granulosa cell tumor and theca cell tumor Androgen producing: Sertoli-Leydig cell tumor Occurs very rarely; ∼ 20% malignant transformation Production of androgens → virilization [amboss.com]

  These cells are known for their hormonal function which may lead to virilization or feminization of the opposite sex. CD56 has been proposed to identify sex cord tumors and to follow response to treatment as a marker. [lecturio.com]

  The tumors typically produce androgens, and clinical virilization is noted in 70–85% of patients. [academic.oup.com]

  Two thirds of children with these tumors present with endocrine abnormalities, such as precocious pseudopuberty, virilization, or menstrual irregularities. [glowm.com]

• Hunting

  Hunt A.B. Childs D.S. A study of ovarian dysgerminoma with emphasis on the role of radiation therapy.Surg. Gynecol. Obstet. 1961; 113: 692-698 PubMed Google Scholar Moreton R.D. Desjardins A.U. Dysgerminoma of the ovary. [gynecologiconcology-online.net]

  Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: tumor types and imaging characteristics. RadioGraphics 2001 ;21(2):475–490. Link, Google Scholar 33. Yamaoka T, Togashi K, Koyama T et al. [pubs.rsna.org]

• Peritoneal Disease

  Nodal metastases are more frequent than peritoneal disease. The patients are treated with unilateral salpingo-oophorectomy and chemotherapy. 7, 8 Dysgerminomas are bilateral in 10-35% of cases. [ncbi.nlm.nih.gov]

  Nodal metastases are more frequent than peritoneal disease. The patients are treated with unilateral salpingo-oophorectomy and chemotherapy. [7], [8] Dysgerminomas are bilateral in 10-35% of cases. [jfcmonline.com]

  The FIGO staging system for ovarian neoplasms was used. 8 Pathological stage was recorded as 1a in 14 cases, 1c in one case, 2a in one case and 3c in the remaining five cases, among which four had lymph node metastases and one showed peritoneal disease [nature.com]

  Although OMGCTs are surgically staged, preoperative imaging may be used to identify local extension, peritoneal disease, and distant metastases. Suspicious areas may be sampled during surgery. [pubs.rsna.org]

• Breast Mass

  One month later, she developed a right pelvic mass along with abdominal lymphadenopathies, peritoneal carcinomatosis, left breast mass, and left axillary node. Cytology of the breast mass was suggestive of a pure dysgerminoma. [ncbi.nlm.nih.gov]

• Adnexal Mass

  These symptoms, along with the sonographic findings of a large, solid lobulated adnexal mass in young women, strongly suggest an ovarian dysgerminoma in the differential diagnosis. [nebraska.pure.elsevier.com]

  […] in the assessment of the location of the tumor (e.g., differentiation of uterine from adnexal masses) and in distinguishing between a benign and malignant adnexal lesion. [ncbi.nlm.nih.gov]

  Figure 2 Pelvic CT findings—large cystic left adnexal mass (large anteroposterior diameter of 85.6 mm). [casereports.bmj.com]

  Adnexal mass during pregnancy: a review[J]. Am J Perinatol. 2015 ;32(11): 1010 – 6. doi: 10.1055/s-0035-1549216. [tandfonline.com]

• Amenorrhea

  Therefore the occurrence of post-radiotherapy amenorrhea, characterized by high serum gonadotropin levels, should not always be considered pathognomonic of precocious menopause. [ncbi.nlm.nih.gov]

  This gene is located on the Y chromosome (p11.31); mutations at this site result in phenotypic females with müllerian external genitalia, underdeveloped (streak) internal gonads, amenorrhea, and rudimentary development of breasts, pubic hair, and axillary [emedicine.medscape.com]

  […] palpable abdominal tumour. 7 It may also present as acute abdomen because of torsion (tumour twisting around its pedicle) or intra-abdominal haemorrhage (from congestion and rupture of superficial tumour vessels). 3 Some may present with vaginal bleeding, amenorrhea [jpma.org.pk]

  Two years earlier, she presented after two months of amenorrhea. A pregnancy test was negative, and a transvaginal ultrasound examination (TVU) showed a 2cm anechoic tri-chamber image in the right ovary. [jmedicalcasereports.biomedcentral.com]

• Primary Amenorrhea

  Dysgerminoma is the most common malignant ovarian germ‐cell tumor diagnosed in pregnancy 2, and may also be discovered in patients investigated for primary amenorrhea, in which case it may be associated with gonadal dysgenesis and gonadoblastoma 6. [obgyn.onlinelibrary.wiley.com]

If a pelvic mass is detected, tests for pregnancy and sexually transmitted diseases, as background information, should be obtained in reproductive age women. Determination of lactate dehydrogenase and HCG levels may help in the diagnosis in cases where these are elevated.

The most important feature to determine is whether or not the tumor is malignant. After the physical characterization of the mass, the initial imaging method is a transvaginal ultrasound. Four sonographic features that distinguish between benign and malignant pelvic masses have been proposed [5], according to which malignancy is suggested by solid material in the tumor, the presence and location of flow on color doppler imaging, free intraperitoneal fluid, and thick septations within the tumor. The most significant association with malignancy was found in cases where a solid component was present; masses that had no solid component, or had strongly hyperechoic solid parts, were almost invariably benign [5]. Necrosis inside the solid mass was also an indicator of the malignant condition [1]. Another study found papillary projections, the most reliable predictor [6].

Most authors agree that no single sonographic feature has a strong enough diagnostic value for malignancy, and a number of scoring systems have been proposed to predict disease based on morphological features [6]. Technical improvements, such as 3-dimensional ultrasonography, which help in characterizing both the internal structure of the adnexal masses as well as their vascularization, lead to better judgment [7] [8]. Combining imaging results obtained with different sonographic systems for both morphology and vascularity of the masses also increases the accuracy of diagnosis [6].

Magnetic resonance imaging performs better than either ultrasonography or computed tomography in diagnosing malignant features in ovarian tumors (although not in extraovarian pelvic masses). However, ultrasonography detected abdominal spread with higher specificity than the other two imaging methods in few cases [9].

Surgery is not just a treatment, but also an important tool for characterizing the tumor and establishing the spread of the disease, and so contributes diagnostic information for follow-up therapy [3].

• Pneumoperitoneum

  Science ®], [Google Scholar] Remarkably, as recommended by the European Society of Gynecological Oncology (ESGO), these are 4 prerequisites for surgical interventions during pregnancy as follows: a maximal laparoscopic procedure time of 90 minutes, a pneumoperitoneum [tandfonline.com]

• Ovarian Mass

  We describe three cases of ovarian dysgerminoma in which color Doppler sonography demonstrated multilobulated solid ovarian masses with prominent flow signal in the fibrovascular septa. [ncbi.nlm.nih.gov]

  LDH can also be raised making it useful in diagnosis and post-operative follow-up of ovarian dysgerminoma 6. Ultrasound May be seen as a septated ovarian mass with varying echotexture. [radiopaedia.org]

  CEA (carcinoembryonic Ag) 1.0 ng/mL (normal Radiologic studies showed a 5 × 13 × 7 cm right pelvic mass on CT, which was confirmed to be a solid enhancing ovarian mass with MRI. [academic.oup.com]

We present a patient who had a late recurrence of ovarian dysgerminoma with successful treatment outcome. A 25-year-old woman was diagnosed with a stage IC ovarian dysgerminoma in 1983 and did not undergo adjuvant treatment. [ncbi.nlm.nih.gov]

When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. [my.clevelandclinic.org]

Both radiation and chemotherapy are highly effective treatment modalities for dysgerminoma. [medigoo.com]

Prognosis When treated promptly and appropriately, these tumors have excellent prognosis with return of normal menstrual function and fertility rates and no increase in teratogenicity. [orpha.net]

However, recent literature suggests that late recurrence may be associated with a poorer prognosis and bleomycin/etoposide/cisplatin (BEP) chemotherapy may play only a limited role in its management. [ncbi.nlm.nih.gov]

As a rule, any peritoneal involvement carries a poor prognosis. No correlation exists between tumor size and prognosis. [emedicine.medscape.com]

Etiology The exact etiology for this tumor type has not been elucidated. Epidemiology Frequency The US incidence of dysgerminomas has remained unchanged over the last 30 years. [emedicine.medscape.com]

The etiology and pathogenesis of these tumors are not clearly defined, but some studies have shown that etiology is related to genetic origin. [ejournal.unsrat.ac.id]

Etiology They are rapidly growing neoplasms that arise from germ cells in the ovary. Diagnostic methods Diagnosis relies on clinical findings, serum tumor markers and imaging. [orpha.net]

Immunohistochemistry vimentin + PLAP + (placental-like alkaline phosphatase) CD117 (c-kit) + inconstant + to - : cytokeratins, desmin, GFAP, CEA and S-100 protein Etiology and evolution the vast majority have no precursor lesion may arise from gonadoblastoma [humpath.com]

Epidemiology Frequency The US incidence of dysgerminomas has remained unchanged over the last 30 years. [emedicine.medscape.com]

Summary Epidemiology The annual incidence is 1/200000 females in Europe. Ovarian germ-cell tumors represent 15-20% of all ovarian tumors, 3% of which are malignant. [orpha.net]

[…] apparently unilateral, but occult foci of dysgerminoma can be detected by biopsy of the contralateral ovary in 10% of cases monotonous proliferation of primitive germ cells connective tissue septa containing variable amounts of chronic inflammatory cells Epidemiology [humpath.com]

Hepatology (15) Dentistry (15) Endocrinology, Metabolism (14) Genetics (13) Radiology and Imaging (12) Neurosurgery (12) Anatomy (11) Physiology and Pathophysiology (10) Respiratory Medicine (10) Medical Chemistry and Biochemistry (9) Dermatology (6) Epidemiology [portal.mefanet.cz]

Oncology, Radiation Therapy (22) Neurology (20) Infectology (17) Microbiology (16) Gastroenterology and Hepatology (15) Dentistry (15) Endocrinology, Metabolism (14) Genetics (13) Radiology and Imaging (12) Neurosurgery (12) Anatomy (11) Physiology and Pathophysiology [portal.mefanet.cz]

Pathophysiology Typically, germ cells are encapsulated at birth within the primordial follicle. If they somehow escape encapsulation, cell death usually occurs. [emedicine.medscape.com]

Although rare, dysgerminomas are important beyond mere incidence because they affect women of reproductive age (ie, Pathophysiology: Typically, germ cells are encapsulated at birth within the primordial follicle. [www1.cgmh.org.tw]

This case illustrates the potential for surgical site implant of an ovarian dysgerminoma; surgeons should follow strict guidelines when performing laparoscopic procedures for ovarian malignancies in order to prevent this type of incident. [ncbi.nlm.nih.gov]

Following is the information for prevention of Ovarian Dysgerminoma: The cause of Ovarian Dysgerminoma is unknown; hence, there are no known methods to prevent the tumor occurrence In individuals having Ovarian Dysgenesis, both the ovaries are prophylactically [dovemed.com]

Prevention To help reduce your chance of orchitis: Get vaccinated with the MMR vaccine. Practice safe sex, including wearing a latex condom to prevent STIs. Get prompt treatment for any infections. [winchesterhospital.org]

Harriet O Smith, Marianne Berwick, +4 authors Clifford R Qualls Obstetrics and gynecology 2006 1 Excerpt Adhesion prevention in gynecologic surgery. [pdfs.semanticscholar.org]

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