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Ovarian Dysgerminoma

Dysgerminoma of Ovary

Ovarian dysgerminoma is a common malignant tumor that comprises of ovarian germ cells. It is a disease of adolescents and young women. It is diagnosed by physical examination and imaging methods and is treated by salpingo-oophorectomy, radiation, and chemotherapy.


Presentation

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].

Abdominal Lymphadenopathy
  • 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]
Lymphadenopathy
  • Combined chemotherapy led to a radiological regression of the lymphadenopathy and coincided with a decrease in SACE concentration.[ncbi.nlm.nih.gov]
  • Further evaluation of the mass with magnetic resonance imaging revealed a solid, enhancing right ovarian mass and para-aortic lymphadenopathy; additional samples were drawn demonstrating elevated serum lactate dehydrogenase, suggestive of malignancy.[academic.oup.com]
Virilization
  • Certain subtypes may occasionally be associated with isosexual precocity, dysgenetic gonads, virilization, hyperthyroidism or carcinoid syndrome (see this term).[orpha.net]
  • 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]
  • […] production 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]
  • 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]
Fishing
  • The FISH analysis demonstrated five copies of chromosome 7 and four copies of chromosome 12 in most tumors. The copy number of chromosome 18 ranged from two to four. The X chromosome was present in three copies in most tumors.[ncbi.nlm.nih.gov]
  • We analyzed a large series of ovarian dysgerminomas by dual color FISH technologies and found 12p abnormalities in 81% of cases.[nature.com]
Down Syndrome
  • An 11-year-old girl with Down syndrome (DS) was operated for a stage I right ovary dysgerminoma. She is in good health 33 years later. Some data in the literature suggest that ovarian cancers could be slightly overrepresented in DS.[ncbi.nlm.nih.gov]
Gangrene
  • RESULT: At laparotomy, torsion of the pedicle with an intact capsule and imminent gangrene were found.[ncbi.nlm.nih.gov]
Jaundice
  • CASE: A 22-year-old woman presented with 3 days of jaundice and lower abdominal pain. Liver tests revealed marked cholestasis and high alkaline phosphatase and bilirubin levels.[ncbi.nlm.nih.gov]
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]
Peripheral Neuropathy
  • Peripheral neuropathy among women with ovarian dysgerminoma has not been so far reported in the literature. We report a case of an 18-year-old woman with an association of an abdominal relapse of an ovarian dysgerminoma and a polyradiculoneuritis.[ncbi.nlm.nih.gov]
  • Read more about ototoxicity - tinnitus and hearing loss Peripheral neuropathy Assess prior to each treatment.[eviq.org.au]
Meningism
  • Isolated meningeal recurrence of ovarian cancer is uncommon. It is generally assumed that such cases are not accompanied by prolonged survival.[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 a setting of sonographically indeterminate adnexal masses, MR imaging is used as a problem-solving tool.[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]
  • Some ovarian cancers secrete hormones leading to a variety of symptoms: Amenorrhea Hirsutism Precautious puberty Postmenopausal bleeding Irregular uterine bleeding. Emergent complications due to ovarian cysts include rupture and torsion.[lecturio.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]

Workup

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].

Right Pleural Effusion
  • Chest X-ray showed massive right pleural effusion. Abdominopelvic CT scan showed a left adnexal solid mass and ascites.[ncbi.nlm.nih.gov]
  • Meigs’ syndrome is the triad of right pleural effusion, ascites and abdominal mass mostly in ovarian sex cord/stromal tumor. It is most commonly found in fibroma but can also occur in granulosa cell tumors and sometimes even in Brenner’s tumor.[lecturio.com]
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]
  • Subsequent MRI revealed solid, enhancing right ovarian mass concerning for malignancy. Transvaginal ultrasound again showed the mass as well as para-aortic lymphadenopathy.[academic.oup.com]

Treatment

  • 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]

Prognosis

  • LDH can be considered as an enzymatic tumor marker of ovarian dysgerminoma, for diagnosis, prognosis, and surveillance.[ncbi.nlm.nih.gov]
  • These are fast growing tumors, although with excellent prognosis. 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.[symptoma.com]

Etiology

  • The exact etiology of dysgerminomas has not been determined, although recent molecular studies have implicated loss of function with potential tumor suppressor gene TRC8/RNF139 as a possible etiology. [1] Additionally, 5% of all dysgerminomas occur in[emedicine.medscape.com]
  • 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]
  • Besides dysgerminoma the other germ cell tumors found in ovaries are The exact etiology of dysgerminomas has not been determined. Typically, germ cells are encapsulated at birth within the primordial follicle.[medigoo.com]
  • Although there is still much to learn about the etiology of MRKH, there has been steady progress in recent decades with regard to efficient diagnostic modalities and proper medical management.[sciforschenonline.org]
  • Ancillary findings, such as ascites and peritoneal carcinomatosis on ultrasound, CT, or MR imaging are strong indicators of a malignant etiology for an adnexal mass.[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiology Frequency United States The incidence of dysgerminomas has remained unchanged over the last 30 years.[emedicine.medscape.com]
  • […] 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]
  • 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]
  • References: [1] [2] [3] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • […] cytoplasm and distinct, squared off cell membranes Tumor often separated by fibrous septae containing cytotoxic lymphocytes that can spill out into tumor Positive for SALL4, OCT3/4, D2-40, CD117, PLAP ICD coding ICD-10: C56 - malignant neoplasm of ovary Epidemiology[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • 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.[csh.org.tw]

Prevention

  • 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]
  • Harriet O Smith , Marianne Berwick , 4 authors Clifford R Qualls Obstetrics and gynecology 2006 1 Excerpt Adhesion prevention in gynecologic surgery.[pdfs.semanticscholar.org]
  • Read more about preventing antineoplastic induced nausea and vomiting Etoposide conversion factor Note: Etopophos (etoposide phosphate) 113.6 mg is equivalent to etoposide 100 mg. Doses in this protocol are expressed as etoposide.[eviq.org.au]

References

Article

  1. Aldhafery BF. Ovarian dysgerminoma in two sisters. J Family Community Med. 2008 Sep;15(3):127-131.
  2. Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. Radiographics. 2002 Nov-Dec;22(6):1305-1325.
  3. Ajao M, Vachon T, Snyder P. Ovarian dysgerminoma: a case report and literature review. Mil Med. 2013 Aug;178(8):e954-945.
  4. Pectasides D, Pectasides E, Kassanos D. Germ cell tumors of the ovary. Cancer Treat Rev. 2008 Aug;34(5):427-441.
  5. Brown DL, Doubilet PM, Miller FH, et al. Benign and malignant ovarian masses: selection of the most discriminating gray-scale and Doppler sonographic features. Radiology. 1998 Jul;208(1):103-110.
  6. Kurjak A, Kupesic S, Sparac V, Bekavac I. Preoperative evaluation of pelvic tumors by Doppler and three-dimensional sonography. J Ultrasound Med. 2001 Aug;20(8):829-840
  7. Cohen LS, Escobar PF, Scharm C, Glimco B, Fishman DA. Three-dimensional power Doppler ultrasound improves the diagnostic accuracy for ovarian cancer prediction. Gynecol Oncol. 2001 Jul;82(1):40-48.
  8. Kurjak A, Kupesic S, Sparac V, Prka M, Bekavac I. The detection of stage I ovarian cancer by three-dimensional sonography and power Doppler. Gynecol Oncol. 2003 Aug;90(2):258-64.
  9. Kurtz AB, Tsimikas JV, Tempany CM, et al. Diagnosis and staging of ovarian cancer: comparative values of Doppler and conventional US, CT, and MR imaging correlated with surgery and histopathologic analysis-report of the Radiology Diagnostic Oncology Group. Radiology. 1999 Jul;212(1):19-27.

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Last updated: 2019-07-11 21:05