Endodermal sinus tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Gertrude Djouka, M.D.[2]; Monalisa Dmello, M.B,B.S., M.D. [3]; Shivali Marketkar, M.B.B.S. [4]; Ammu Susheela, M.D. [5]

For patient information, click here Synonyms and keywords: Endodermal sinus tumor(EST), yolk sac tumor.

Overview

Endodermal sinus tumor was first discovered by Dr. Gunner Telium, a Danish pathologist, in 1959. The hypermethylation of the RUNX3 gene promoter and overexpression of GATA-4, a transcription factor has been associated with the development of endodermal sinus tumor. Endodermal sinus tumor is a rare type of malignant ovarian tumor that occurs in the second decade of life. On gross pathology, solid gray-white with a gelatinous, myxoid, or mucoid appearance, necrosis, cystic changes, and hemorrhage are characteristic findings of endodermal sinus tumor. On microscopic histopathological analysis, Schiller-Duval bodies is a characteristic finding of endodermal sinus tumor. Endodermal sinus tumor must be differentiated from other diseases that cause ovarian mass, such as Stein-Leventhal syndrome. Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. ovary teratoma, tubal pregnancy, ovary adenocarcinoma, and tubo-ovarian abscess. Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population. The symptoms of endodermal sinus tumor include the following:abdominal distention, acute/sub acute abdominal pain. The mainstay of therapy for endodermal sinus tumor is chemotherapy.

Historical Perspective

• Endodermal sinus tumor was first discovered by Dr. Gunner Telium, a Danish pathologist in 1959.

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 hypermethylation of the RUNX3 gene promoter and overexpression of GATA-4, a transcription factor has been associated with the development of endodermal sinus tumor.
• On gross pathology, encaptulated, firm, smooth, round, globular, solid gray-white with a gelatinous, myxoid, or mucoid appearance, necrosis, cystic changes, and hemorrhage are characteristic findings of endodermal sinus tumor.
• On microscopic histopathological analysis, Schiller-Duval bodies (invaginated papillary structures with central vessel) is a characteristic finding of endodermal sinus tumor. The tumors are composed of irregular space lined by flattened to cuboidal cells and recticular stroma.

Causes

• There are no established causes for endodermal sinus tumor.

Differentiating Endodermal sinus tumor from Other Diseases

Endodermal sinus tumor must be differentiated from other diseases that cause ovarian mass, such as:

• Stein-Leventhal syndrome

• Ovary teratoma

• Tubal pregnancy

• Ovary adenocarcinoma

• Tubo-ovarian abscess

Epidemiology and Demographics

Age

• Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population
• Pure yolk sac tumor (endodermal sinus tumor) affects mostly children of less 2 years old.

Gender

• Endodermal sinus tumor affects men and women equally.

Natural History, Complications and Prognosis

• Endodermal sinus tumor has a poor prognosis.
• Endodermal sinus tumor is the most common malignant germ cell tumor in children.
• If left untreated, endodermal sinus tumor quickly metastasizes in other parts of the body such as the brain.
• Endodermal sinus tumor can be found in the ovaries or testicles including the chest, abdomen, and the brain.
• Ovarian germ cell tumor (endodermal sinus tumor) is surgically staged using the FIGO cancer staging system:

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:

• Abdominal distention
• Acute/sub acute abdominal pain

Physical Examination

Ovarian Germ Cell Tumor Physical Examination

Abdomen

• Abdominal distention
• Abdominal tenderness

Pelvic exam

• Adnexal mass

Laboratory Findings

• An elevated concentration of serum alpha feto-protein is diagnostic of endodermal sinus tumor.
• AFP is very important for diagnosis, disease monitoring and early metastasis.

Imaging Findings

• On MRI, endodermal sinus tumor is characterized by areas of haemorrhage and necrosis.

Other Diagnostic Studies

• Endodermal sinus tumor may also be diagnosed using biopsy and measurement of GATA-4, a transcription factor.

Treatment

Medical Therapy

• The mainstay of therapy for endodermal sinus tumor is chemotherapy.

Stage I endodermal sinus tumor

• Unilateral salpingo-oophorectomy with adjuvant chemotherapy

Stage II endodermal sinus tumor

• Unilateral salpingo-oophorectomy with adjuvant chemotherapy

Stage III endodermal sinus tumor

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy

Stage IV endodermal sinus tumor

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy

Surgery

Surgery is the mode of treatment for endodermal sinus tumor when chemotherapy is not effective:

Stage I endodermal sinus tumor

• Unilateral salpingo-oophorectomy with adjuvant chemotherapy
• Unilateral salpingo-oophorectomy followed by observation

Stage II endodermal sinus tumor

• Unilateral salpingo-oophorectomy with adjuvant chemotherapy
• Second-look laparotomy

Stage III endodermal sinus tumor

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy
• Second-look laparotomy

Stage IV endodermal sinus tumor

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy

Video

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