Endodermal sinus tumor
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]; Shivali Marketkar, M.B.B.S. [3]; Ammu Susheela, M.D. [4]
Synonyms and keywords: EST
Overview
Historical Perspective
- [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
- In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
- In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
Classification
- Endodermal sinus tumor may be classified according to histology into 10 groups:
- Reticular
- Endodermal sinus-like
- Microcystic
- Papillary
- Solid
- Glandular
- Alveolar
- Polyvesicular vitelline
- Enteric
- Hepatoid
Pathophysiology
- The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
- The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
- On microscopic histopathological analysis, Schiller-Duval bodies, [feature2], and [feature3] are characteristic findings of [disease name].
Causes
- [Disease name] may be caused by either [cause1], [cause2], or [cause3].
- [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
- There are no established causes for [disease name].
Differentiating From Endodermal sinus tumor Other Diseases
Endodermal sinus tumor must be differentiated from other diseases that cause ovarian mass, such as:
- Ovary teratoma
- Tubal pregnancy
- Ovary adenocarcinoma
- Tubo-ovarian abscess
Epidemiology and Demographics
- The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
- In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may develop [disease name].
- [Disease name] is more commonly observed among patients aged [age range] years old.
- [Disease name] is more commonly observed among [elderly patients/young patients/children].
Gender
- [Disease name] affects men and women equally.
- [Gender 1] are more commonly affected with [disease name] than [gender 2].
- The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
Race
- There is no racial predilection for [disease name].
- [Disease name] usually affects individuals of the [race 1] race.
- [Race 2] individuals are less likely to develop [disease name].
Risk Factors
- Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- The majority of patients with [disease name] remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
Ovarian germ cell tumor (endodermal sinus tumor) is surgically staged using the FIGO cancer staging system:[3]
| Stage | Finding |
| I | Growth limited to the ovaries |
| Ia | Growth limited to one ovary; no ascites present containing malignant cells. No tumor on the external surface; capsule intact |
| Ib | Growth limited to both ovaries; no ascites present containing malignant cells. No tumor on the external surfaces; capsules intact |
| Icb | Tumor either stage Ia or Ib, but with tumor on surface of one or both ovaries, or with capsule ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings |
| II | Growth involving one or both ovaries with pelvic extension |
| IIa | Extension and/or metastases to the uterus and/or tubes |
| IIb | Extension to other pelvic tissues |
| IIcb | Tumor either stage IIa or IIb, but with tumor on surface of one or both ovaries, or with capsule(s) ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings |
| III | Tumor involving one or both ovaries with histologically confirmed peritoneal implants outside the pelvis and/or positive regional lymph nodes. Superficial liver metastases equals stage III. Tumor is limited to the true pelvis, but with histologically proven malignant extension to small bowel or omentum |
| IIIa | Tumor grossly limited to the true pelvis, with negative nodes, but with histologically confirmed microscopic seeding of abdominal peritoneal surfaces, or histologic proven extension to small bowel or mesentery |
| IIIb | Tumor of one or both ovaries with histologically confirmed implants, peritoneal metastasis of abdominal peritoneal surfaces, none exceeding 2 cm in diameter; nodes are negative |
| IIIc | Peritoneal metastasis beyond the pelvis >2 cm in diameter and/or positive regional lymph nodes |
| IV | Growth involving one or both ovaries with distant metastases. If pleural effusion is present, there must be positive cytology to allot a case to stage IV. Parenchymal liver metastasis equals stage IV |
Diagnosis
Symptoms
The symptoms of endodermal sinus tumor include the following:[4]
- Abdominal distention
- Acute/sub acute abdominal pain
Physical Examination
Ovarian Germ Cell Tumor Physical Examination
Abdomen
- Abdominal distention [5]
- Abdominal tenderness
Pelvic exam
- Adnexal mass
Laboratory Findings
- An elevated concentration of serum alpha feto-protein is diagnostic of endodermal sinus tumor.
Imaging Findings
- On MRI, endodermal sinus tumor is characterized by areas of haemorrhage.
Other Diagnostic Studies
- Endodermal sinus tumor may also be diagnosed using biopsy and measurement of GATA-4, a transcription factor.
Treatment
Medical Therapy
- There is no treatment for [disease name]; the mainstay of therapy is supportive care.
- The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for [disease name].
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
- [Surgical procedure] can only be performed for patients with [disease stage] [disease name].
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1],[test 2], and [test 3].
Video
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References
- ↑ 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
- ↑ Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
- ↑ Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.