Ovarian germ cell tumor overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Monalisa Dmello, M.B,B.S., M.D. [2]
Overveiw
Ovarian germ cell tumor is a disease in which malignant cells form in the germ cells of the ovary. Germ cell tumors begin in the reproductive cells of the body. Ovarian germ cell tumor are rare tumors, accounting for 2% to 3% of all ovarian cancers. The median age for diagnosis is 16 to 20 (range 6 to 40 years).[1] Ovarian germ cell tumor may be classified into 7 subtypes based on histology.[2] The most common ovarian germ cell tumor is called dysgerminoma. Abnormal gonads (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk of developing a dysgerminoma. Ovarian germ cell tumor must be differentiated from other neoplastic ovarian mass, non neoplastic ovarian mass, and adnexal mass. Symptoms of ovarian germ cell tumor include abdominal distention, acute/ sub acute abdominal pain, menstrual irregularities, and precocious puberty.[3] Physical examination of patients with ovarian germ cell tumor is usually remarkable for vaginal bleeding, abdominal distention, and adnexal mass.[3] The laboratory findings associated with ovarian germ cell tumor are the following: serum lactate dehydrogenase (LDH), human chorionic gonadotropin (HCG), CA-125, and alpha-fetoprotein (AFP). Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. CT, MRI, and ultrasound are used in combination to distinguish between the subtypes of ovarian germ cell tumors.[4][5][6] However, the prognosis is generally regarded as good.[7] The mainstay of therapy for ovarian germ cell tumor is chemotherapy and surgery.[8][9][10][11]
Classification
Ovarian germ cell tumor may be classified into 7 subtypes based on histology.[2]
Pathophysiology
The pathophysiology of ovarian germ cell tumors depends on the histological subtype. However, their origin is the primordial germ cells that transformed pathologically in different stages of development. It is difficult to distinguish subtypes of ovarian germ cell tumor on gross pathology alone. The majority of ovarian germ cell tumors have a solid and cystic appearance with areas of hemorrhage and necrosis. On microscopic pathology, ovarian germ cell tumors may be characterized by a uniform “fried egg” appearance (dysgerminoma), presence of Schiller-Duval bodies (yolk sac tumor), presence of embryonic-like neural, gastrointestinal, and/or cartilaginous tissue (teratoma), or mixed histopathological features (embryonal cell carcinoma).
Epidemiology and Demographics
Ovarian germ cell tumor are rare tumors, accounting for 2% to 3% of all ovarian cancers. The median age for diagnosis is 16 to 20 (range 6 to 40 years).[1]
Risk Factors
Abnormal gonads (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk of developing a dysgerminoma.[12]
Differentiating From Ovarian Germ Cell Tumor Other Diseases
Ovarian germ cell tumor must be differentiated from other diseases that cause ovarian mass, such as stein-Leventhal syndrome, ovary teratoma, tubal pregnancy, ovary adenocarcinoma, and tubo-ovarian abscess.[13][14]
Prognosis
Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.[7]
Staging
According to the FIGO cancer staging system, there are 4 stages of ovarian germ cell tumor.[15]
History and Symptoms
Symptoms of ovarian germ cell tumor include abdominal distention, acute/ sub acute abdominal pain, menstrual irregularities, and symptoms of precocious puberty.[3]
Ovarian Germ Cell Tumor Physical Examination
Physical examination of patients with ovarian germ cell tumor is usually remarkable for vaginal bleeding, abdominal distention, and adnexal mass.[3]
Ovarian germ cell tumor Laboratory Findings
The laboratory findings associated with ovarian germ cell tumor are the following: serum lactate dehydrogenase (LDH), human chorionic gonadotropin (HCG), CA-125, and alpha-fetoprotein (AFP).[16]
CT
It is difficult to distinguish ovarian germ cell tumors on CT alone. Dysgerminoma often appears as multilobulated solid masses with prominent fibrovascular septa while, mature ovarian teratoma may demonstrate fat (areas with very low Hounsfield values), fat-fluid level, calcification (sometimes dentiform), Rokitansky protuberance, and tufts of hair.[4][5][6]
MRI
It is difficult to distinguish subtypes of ovarian germ cell tumor on MRI alone. The majority of ovarian germ cell tumors have a solid and cystic appearance with areas of hemorrhage and necrosis. On MRI, ovarian germ cell tumors may be characterized by T2: the septae are often hypointense or isointense T1 C+ (Gd): the septae often show marked enhancement (dysgerminoma), the presence of a prominent solid component containing calcifications and small foci of fat (mature teratoma), areas of haemorrhage can also be seen (yolk sac tumor).[4][5][6]
Ultrasound
It is difficult to distinguish ovarian germ cell tumors on ultrasound alone. Both solid and cystic lesions with calcification may be present. Dysgerminoma often appears as a hypoechoic mass while other ovarian germ cell tumors often have variable echogenicity. Ovarian teratoma may be further characterized by the presence of sebaceous and hair components arising from the rokitansky protuberance.[4][5][6][17]
Medical Therapy
The mainstay of therapy for ovarian germ cell tumor is chemotherapy.[8][9][10][11]
Surgery
Surgery is the mainstay of treatment for ovarian germ cell tumors.[8][9][10][11]
References
- ↑ 1.0 1.1 Updates in the Management of Ovarian Germ Cell Tumors. http://meetinglibrary.asco.org/content/31-132. URL Accessed on November 11, 2015
- ↑ 2.0 2.1 Cellular Classification of Ovarian Germ Cell Tumors. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_. URL Accessed on November 4, 2015
- ↑ 3.0 3.1 3.2 3.3 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
- ↑ 4.0 4.1 4.2 4.3 Ovarian dysgerminoma. http://radiopaedia.org/articles/ovarian-dysgerminoma. URL Accessed on November 11, 2015
- ↑ 5.0 5.1 5.2 5.3 Mature (cystic) ovarian teratoma. http://radiopaedia.org/articles/mature-cystic-ovarian-teratoma. URL Accessed on November 11, 2015
- ↑ 6.0 6.1 6.2 6.3 Ovarian yolk sac tumour. http://radiopaedia.org/articles/ovarian-yolk-sac-tumour. URL Accessed on November 11, 2015
- ↑ 7.0 7.1 Ovarian Germ Cell Tumors Treatment.http://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq. URL Accessed on November 11, 2015
- ↑ 8.0 8.1 8.2 Stage I Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_33. URL Accessed on Nov 5, 2015
- ↑ 9.0 9.1 9.2 Stage II Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_43. URL Accessed on Nov 5, 2015
- ↑ 10.0 10.1 10.2 Stage III Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_54. URL Accessed on Nov 5, 2015
- ↑ 11.0 11.1 11.2 Stage IV Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_65. URL Accessed on Nov 5, 2015
- ↑ Kliegman, Robert (2011). Nelson textbook of pediatrics. Philadelphia, PA: Elsevier/Saunders. ISBN 978-1-4377-0755-7.
- ↑ Shaaban AM, Rezvani M, Elsayes KM, et al. Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. Radiographics. 2014;34(3):777-801.http://pubs.rsna.org/doi/pdf/10.1148/rg.343130067
- ↑ 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;22(6):1305-25.http://www.ncbi.nlm.nih.gov/pubmed/12432104
- ↑ Stage Information for Ovarian Germ Cell Tumors. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_8. URL Accessed on November 5, 2015
- ↑ Ovary: Germ cell tumors. http://atlasgeneticsoncology.org/Tumors/OvarianGermCellID5067.html. URL Accessed on November 10, 2015
- ↑ Immature ovarian teratoma. http://radiopaedia.org/articles/immature-ovarian-teratoma. URL Accessed on November 11, 2015