Endodermal Sinus Tumor (Endodermal Sinus Tumors)

An unusual and aggressive tumor of germ-cell origin that reproduces the extraembryonic structures of the early embryo. It is the most common malignant germ cell tumor found in children. It is characterized by a labyrinthine glandular pattern of flat epithelial cells and rounded papillary processes with a central capillary (Schiller-Duval body). The tumor is rarely bilateral. Before the use of combination chemotherapy, the tumor was almost invariably fatal. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1189)

Also Known As:

Endodermal Sinus Tumors; Yolk Sac Tumor; Tumor, Endodermal Sinus; Tumor, Yolk Sac; Tumors, Endodermal Sinus; Tumors, Yolk Sac; Yolk Sac Tumors

Networked: 725 relevant articles (35 outcomes, 38 trials/studies)

Relationship Network

Disease Context: Research Results

Neoplasms: 1324753
- Neoplasms by Histologic Type: 2
  - Germ Cell and Embryonal Neoplasms: 4454
    - Mesonephroma: 4
      - Endodermal Sinus Tumor: 725

Related Diseases

1.	Neoplasms (Cancer)
2.	Embryonal Carcinoma
3.	Germ Cell and Embryonal Neoplasms (Germ Cell Tumor)
4.	Choriocarcinoma
5.	Germinoma

Experts

1.	Ulbright, Thomas M: 7 articles (02/2016 - 04/2004)
2.	Cheng, Liang: 6 articles (10/2022 - 05/2010)
3.	Shen, Keng: 5 articles (01/2019 - 12/2008)
4.	Young, Robert H: 5 articles (08/2015 - 01/2004)
5.	Idrees, Muhammad T: 4 articles (01/2022 - 02/2012)
6.	Looijenga, Leendert H J: 4 articles (01/2020 - 02/2002)
7.	Cao, Dengfeng: 4 articles (01/2013 - 10/2009)
8.	Einhorn, Lawrence H: 3 articles (12/2021 - 04/2004)
9.	Spreafico, Filippo: 3 articles (01/2021 - 08/2002)
10.	Terenziani, Monica: 3 articles (01/2021 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Endodermal Sinus Tumor:

1.	Cisplatin (Platino)FDA LinkGeneric 08/01/1983 - "[Peripheral nerve lesions associated with cis-diamminedichloroplatinum (II) treatment--study of a case with yolk sac tumor of the pineal body]." 01/01/2020 - "Cisplatin resistance of ovarian yolk sac tumors (oYST) is a clinical challenge due to dismal patient prognosis, even though the disease is extremely rare. We investigated potential association between cisplatin resistance and cancer stem cell (CSC) markers in chemoresistant oYST cells and targeting strategies to overcome resistance in oYST. " 01/01/2011 - "Cisplatin is used as a key drug for ovarian yolk sac tumor (YST), but relapse may occur. " 01/01/2011 - "Establishment of cisplatin-resistant ovarian yolk sac tumor cells and investigation of the mechanism of cisplatin resistance using this cell line." 01/01/1989 - "Endodermal sinus tumor treated with high dose cisplatin."
2.	alpha-Fetoproteins (alpha-Fetoprotein)IBA 01/10/2015 - "Elevated alpha-fetoprotein (AFP) ≥ 10,000 ng/mL was associated with worse outcome, whereas pure yolk sac tumor (YST) was associated with better outcome, although neither met criteria for statistical significance. " 04/01/2005 - "A panel of immunostains (AE1/AE3, OCT-3/4, and alpha-fetoprotein) is helpful in the differential with yolk sac tumor in especially problematic cases. " 08/01/1976 - "[An experimental study on alpha-fetoprotein producing yolk sac tumor (author's transl)]." 08/01/1975 - "A study on alpha-fetoprotein and endodermal sinus tumor." 06/01/1974 - "Yolk sac tumor and alpha-fetoprotein: clinicopathological study of four cases."
3.	Bleomycin (Blenoxane)FDA LinkGeneric 08/01/1996 - "Treatment of endodermal sinus tumor in children using a regimen that lacks bleomycin." 01/01/2015 - "Herein, we describe the case of a 20-year-old woman treated with bleomycin for an ovarian yolk sac tumor and review the prominent features of this form of dermatitis. " 01/01/2015 - "Bleomycin-Induced Flagellate Erythema in a Patient Diagnosed with Ovarian Yolk Sac Tumor." 05/01/2007 - "Bleomycin induced hyperpigmentation with yolk sac tumor." 08/01/1996 - "The results of this protocol suggests that a cisplatin-containing regimen that lacks bleomycin is active in childhood endodermal sinus tumors."
4.	Etoposide (VP 16)FDA LinkGeneric 07/01/2003 - "To investigate the differences between two rat yolk sac tumor cell lines with different radiosensitivities in etoposide sensitivity and in the sensitizing effect of etoposide in combination with irradiation. " 03/01/2000 - "One had resection of residual mediastinal yolk sac tumor, followed by oral VP-16. " 04/01/1990 - "This case indicated that a combination chemotherapy CDDP and etoposide combined with surgery could play an active role increasing long-term survival rates in mediastinal yolk sac tumors." 01/01/2011 - "Management of a Type I Hypersensitivity Reaction to IV Etoposide in a Woman with a Yolk Sac Tumor: A Case Report." 06/01/2010 - "To investigate the differences between two rat yolk sac tumor cell lines with different radiosensitivities and p53 status in sensitivity to high-LET heavy-ion beam and in sensitizing effect of etoposide or cisplatin in combination with heavy-ion beam. "
5.	Vinblastine (Vinblastine Sulfate)FDA Link 08/01/1987 - "The combination of cisplatin, vinblastine, and bleomycin was administered to 13 previously untreated patients with pure endodermal sinus tumor of the ovary. " 03/01/1985 - "Three patients with advanced and recurrent endodermal sinus tumor (EST) of the ovary were treated with modified vinblastine, actinomycin-D, and bleomycin (VAB-3) regimen. " 01/01/1981 - "Cisplatin, vinblastine, and bleomycin for endodermal sinus tumor of the ovary." 09/01/1980 - "Three women with advanced pure endodermal sinus tumors were treated with a combination of bleomycin, vinblastine, and cis-platinum (VBC). " 09/01/1980 - "Bleomycin, vinblastine, and cis-platinum in the treatment of advanced endodermal sinus tumor."
6.	GlypicansIBA 11/20/2023 - "Glypican-3 (GPC3) is highly expressed in testicular yolk sac tumor (TYST). " 12/01/2020 - "Immunohistochemistry (SALL4, glypican 3) revealed no residual yolk sac tumor. " 05/01/2010 - "OCT4 marked the nuclei of 6 cases, among which 5 cases also showed glypican 3 expression indicative of an admixed yolk sac tumor component. " 12/01/2009 - "All yolk sac tumors showed positive glypican-3 staining (1+ in 6, 2+ in 6, 3+ in 7, and 4+ in 8). " 10/01/2009 - "Yolk sac tumor (YST) was identified by morphology and glypican 3 staining in 20 testicular mixed GCTs. "
7.	Tumor Biomarkers (Tumor Markers)IBA 09/01/2021 - "The diagnostic and prognostic value of tumor markers in giant mediastinal endodermal sinus tumor with prolonged survival: Twelve-year follow up after radical resection." 04/01/2009 - "Children with stage I yolk sac tumors should be monitored closely with periodic AFP tumor marker evaluation and imaging according to the primary dissemination (e.g., ultrasound, chest x-ray, and computed tomography). " 01/01/1992 - "The role of second look laparotomy and tumor markers in the follow-up of endodermal sinus tumor of the ovary. " 06/01/2020 - "The pathological diagnosis was mature teratoma in 17, immature teratoma in 9, yolk sac tumor in 3, and others in 6. The preoperative diagnosis, which was made based on the imaging findings and the serum tumor marker values, matched with the pathological diagnosis in 29/35 (83%). " 01/01/2001 - "So, AFP is probably more universal than believed, although it is generally a popular and useful tumor marker for hepatocellular carcinomas and yolk sac tumors."
8.	PlatinumIBA 09/01/2001 - "Sustained complete remission in a patient with platinum-resistant ovarian yolk sac tumor." 04/01/2014 - "With a multidisciplinary treatment approach using platinum-based regimens, the outcome is similar to those of any yolk sac tumor. " 12/18/2019 - "We summarized the experience of seven primary mediastinal yolk sac tumors treated with platinum-based chemotherapy and extended resection in Peking University First Hospital. " 04/01/2019 - "We report a case of a young man with an unresectable primary yolk sac tumor of the liver, who had a platinum-refractory disease that progressed despite 2 lines of chemotherapy. " 01/01/2018 - "We report on seven cases of primary mediastinal yolk sac tumor treated with platinum-based chemotherapy and extended resection. "
9.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 09/01/1986 - "Cure of stage IV endodermal sinus tumor of the ovary with pulsed cyclophosphamide therapy." 09/01/1986 - "A case of Stage IV endodermal sinus tumor of the ovary treated with excision of the primary ovarian tumor followed by adjuvant pulsed Endoxan (cyclophosphamide) therapy is described. " 10/01/1997 - "The diagnosis was yolk sac tumor (stage I A), and postoperative chemotherapy (vincristine, actinomycine-D, cyclophosphamide) according to the UK-CCSG was provided. " 02/01/1975 - "Endodermal sinus tumor: a report of two sustained remissions treated postoperatively with a combination of actinomycin D, 5-fluorouracil, and cyclophosphamide." 07/01/1994 - "[A case of recurrent yolk sac tumor of testis in a 3-year-old child successfully treated by VAC (vincristine, actinomycin-D, cyclophosphamide) therapy]."
10.	Cadherins (E-Cadherin)IBA 01/01/2012 - "In conclusion, N-cadherin expression is very helpful in the identification of yolk sac tumors. " 07/01/2018 - "Two pure yolk sac tumors and 20 out of 34 mixed GCTs with yolk sac components were positive for N-cadherin. " 01/01/2012 - "Of the 83 cases with yolk sac tumor, 99% were positive for N-cadherin. " 09/01/2017 - "E-cadherin (p = 0.016) and HMGA2 (p = 0.002) expression was significantly higher in immature teratomas and yolk sac tumors compared with dysgerminomas. " 09/01/2017 - "Vimentin (p < 0.001) and Zeb1 (p = 0.029) staining was significantly higher in immature teratomas compared with yolk sac tumors and dysgerminomas, whereas no significant differences were observed for N-cadherin and P-cadherin. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 07/01/1989 - "It is indicated that surgery combined with multi-drug chemotherapy is the best management and high-dose DDP, VAC and AcFuCy are effective in treating pure endodermal sinus tumor of the ovary. " 01/01/2013 - "Sustainable complete remission in recurrence yolk sac tumor patient treated with tandem high-dose chemotherapy and autologous stem cell." 10/01/2010 - "The Yolk sac tumor requiring chemotherapy with good outcome. " 12/01/1993 - "[A case of stage IIIB2 infantile yolk sac tumor of testis achieved complete remission by "COMPE" chemotherapy]." 02/01/1985 - "A case of endodermal sinus tumor of the infant vagina is reported with long-term survival after successful therapy by surgery, chemotherapy, and radiation. "
2.	Combination Drug Therapy (Combination Chemotherapy) 05/01/1996 - "Combination chemotherapy with VAC or PVB dramatically improved the prognosis of ovarian endodermal sinus tumor but it should be emphasized that the favorable results could be obtained only when the treatment is given on time and in optimal cycles. " 10/15/1979 - "A case report of a patient with endodermal sinus tumor is presented in which a long-term remission was achieved by unilateral adnexectomy and combination chemotherapy. " 05/01/1996 - "To evaluate the role of combination chemotherapy and the optimal cycles of treatment in improving the prognosis of ovarian endodermal sinus tumor, and to study the relationship between the type of surgical management and the outcome of the disease. " 05/01/1995 - "The treatment results of 63 cases of ovarian endodermal sinus tumor admitted to Peking Union Medical College Hospital from August 1977 through April 1992 by combination chemotherapy were reported. " 12/01/1991 - "An asymptomatic 11-year-old Japanese boy with an endodermal sinus (yolk sac) tumor of the anterior mediastinum was successfully treated with cisplatinum-based combination chemotherapy and subsequent surgical excision. "
3.	Radiotherapy 12/31/2022 - "Dramatic response to local radiotherapy in a refractory metastatic mediastinal yolk sac tumor patient harboring a germline BRCA2 frameshift mutation: a case report." 05/01/2021 - "Role of Radiotherapy in Recurrent Intra-Abdominal Yolk Sac Tumor." 04/26/2016 - "Successful radiotherapy in postoperative recurrence of a primary mediastinal yolk sac tumor: A case report." 11/01/2014 - "A combination of surgical resection and radiotherapy is recommended for the yolk sac tumor." 05/01/1993 - "Endodermal sinus tumor of the vagina: radiation therapy and progeny."
4.	Therapeutics 07/01/1991 - "The cooperative therapy study MAKEI 83/86 included an examination of the prognostic value of the AFP in children and adolescents with extracranial non-testicular yolk sac tumors. " 08/01/2002 - "New perspectives on therapy for vaginal endodermal sinus tumors." 11/01/1997 - "[Problems in diagnosis and therapy planning in a patient with retroperitoneal yolk sac tumor. " 07/01/1990 - "Intracranial and intraspinal dissemination from pineal yolk sac tumor treated by PVB therapy--case report." 06/01/1985 - "We have treated prospectively 5 children with stage I endodermal sinus tumor with limited surgery and no adjuvant therapy. "
5.	Lymph Node Excision (Lymph Node Dissection) 09/01/1997 - "However, a problem on the need and efficacy of lymph node dissection in mediastinal yolk sac tumors has been not enough discussed in the past. " 04/01/2012 - "For yolk sac tumor, the difference of survival rates between patients with and without retroperitoneal lymph node dissection (RPLND) was calculated. " 09/01/1997 - "[The necessity of mediastinal lymph node dissection for yolk sac tumor of the anterior mediastinum]." 09/01/1984 - "Yolk sac tumor of the testicle: is retroperitoneal lymph node dissection necessary?" 11/01/1981 - "Young children with yolk sac tumors are not immune to metastatic disease and should be treated with an extended unilateral lymphadenectomy for staging and appropriate treatment regimens."