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Background

The yolk sac tumor (also known as the endodermal sinus tumor) is a rare germ cell neoplasm of the ovary. Extra-gonadal variants as well as testicular variants have been reported.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS Endodermal sinus tumor
INCIDENCE/
PREVALENCE
 
AGE Childhood to early adulthood
Ovarian endodermal sinus tumor in a postmenopausal woman.

Oh C, Kendler A, Hernandez E.

Department of Obstetrics, Gynecology, and Reproductive Sciences, Temple University School of Medicine, 3401 N. Broad Street, Philadelphia, Pennsylvania 19140, USA.

Gynecol Oncol. 2001 Aug;82(2):392-4 Abstract quote.  

BACKGROUND: Ovarian endodermal sinus tumor is rare in postmenopausal women.
CASE: We report the case of a 75-year-old woman with a pure endodermal sinus tumor of the ovary.

CONCLUSION: We believe this to be the oldest patient reported with an ovarian endodermal sinus tumor. The histogenesis of this entity in older patients may be different than when it occurs in young women.
SEX  
GEOGRAPHY  
EPIDEMIOLOGIC ASSOCIATIONS  

 

DISEASE ASSOCIATIONS CHARACTERIZATION
PREGNANCY  
Yolk sac tumor of the ovary during pregnancy: A case report.

Aoki Y, Higashino M, Ishii S, Tanaka K.

Division of Molecular Genetics, Department of Obstetrics and Gynecology, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Niigata 951-8510, Japan.

Gynecol Oncol. 2005 Jul 25; [Epub ahead of print] Abstract quote  

BACKGROUND.: There is a therapeutic dilemma for the pregnant patient with malignancy requiring chemotherapy.

CASE.: We had a case of a 30-year-old pregnant woman with a yolk sac tumor who underwent right salpingo-oophorectomy at 22 weeks gestation. Chemotherapy during pregnancy was postponed because of concern with the risks of the chemotherapy to the fetus. After the operation, L2 fraction of alpha-fetoprotein (AFP) depending on the affinity for Lens culinaris agglutinin (LCA) as an indicator for a maternal source of AFP had been detectable since 30 weeks gestation and elevated rapidly with advancing gestation. Tumor recurrence was confirmed clinically at 34 weeks gestation. She was induced, delivering a healthy baby, and received seven complete chemotherapy courses (cisplatin, etoposide, and peplomycin). Currently, the mother (39 months after last chemotherapy) and child are doing well.

CONCLUSION.: AFP-L2 may be a sensitive indicator for a yolk sac tumor derived AFP, leading to an earlier detection of tumor recurrence during pregnancy.

 

PATHOGENESIS CHARACTERIZATION
GENETIC ALTERATIONS  
Genetic analysis of childhood germ cell tumors with comparative genomic hybridization.

Schneider DT, Schuster AE, Fritsch MK, Calaminus G, Harms D, Gobel U, Perlman EJ.

Clinic of Pediatric Hematology and Oncology, Heinrich-Heine-University, Medical Center, Dusseldorf, Germany.
Klin Padiatr. 2001 Jul-Aug;213(4):204-11. Abstract quote  

BACKGROUND: Germ Cell Tumors (GCTs) in children and adolescents constitute a clinically and histologically heterogeneous group of tumors. Compared to GCTs in adults, the numbers of GCTs in children analyzed with cytogenetic and molecular genetic techniques is limited. However, the data available to date reveal a pattern of cytogenetic aberrations different from that in adults. Comparative genomic hybridization (CGH) is a valuable technique for the genetic profiling of tumors that allows screening for chromosomal imbalances consistent with amplification of oncogenes and loss of putative tumor suppressor genes. As CGH does not require tissue culture, it also allows analysing archival tissue samples.

PATIENTS: This study focuses exclusively on GCTs in children younger than ten years of age and summarizes the genetic data of 51 tumors. Eighteen teratomas and 33 malignant GCTs were included. Primary sites were the testis (n=10), coccyx (n=13), mediastinum (n=20), ovary (n=5), CNS (n=2), and the face (n=1).

METHODS: The experimental procedure includes differential enzymatic fluorescence labeling of tumor and control DNA followed by comparative hybridization to normal male chromosomes, karyotyping and computerized analysis of the fluorescence profiles.

RESULTS: With the exception of one testicular and two ovarian tumors, malignant GCTs in children do not show chromosomal gain of 12p, which is characteristic of GCTs in adult patients. Irrespective of the primary site, childhood GCTs show chromosomal imbalances of chromosome 1 (loss of distal 1p, gain of 1q), deletion of 4q and 6q as well as gain of 20q at a high frequency.

CONCLUSIONS: These studies will help guiding further investigations elucidating the role of putative tumor suppressor genes at e.g. 1p36 and 6q. In addition, further studies incorporated in prospective therapeutic protocols are necessary to evaluate the prognostic relevance of specific genetic aberrations.

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  

Yolk sac tumor of the ovary: radiologic-pathologic correlation in four cases.

Yamaoka T, Togashi K, Koyama T, Ueda H, Nakai A, Fujii S, Yamabe H, Konishi J.

Department of Nuclear Medicine and Diagnostic Imaging, Hitachi Medical Corporation, Japan.

J Comput Assist Tomogr. 2000 Jul-Aug;24(4):605-9. Abstract quote  

We report the radiologic and pathologic features of yolk sac tumor (endodermal sinus tumor) of the ovary in four patients.

Yolk sac tumors were shown as well-enhancing solid tumors on both CT and MR studies and were associated with prominent signal voids on MRI. A hypervascular nature was confirmed on microscopic studies.

Areas of hemorrhage were another common characteristic. These imaging findings seem to have an important implication for the diagnosis of this tumor.
LABORATORY MARKERS Elevation of serum
SERUM ALPHA FETOPROTEIN  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
Endodermal sinus tumor of the ovary: The Hacettepe University experience.

Ayhan A, Taskiran C, Bozdag G, Altinbas S, Altinbas A, Yuce K.

Hacettepe University, Faculty of Medicine, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Kadin Hastaliklari ve Dogum A.D., Hamamonu-Ankara, Turkey.
Eur J Obstet Gynecol Reprod Biol. 2005 Jul 15; [Epub ahead of print] Abstract quote  

OBJECTIVE:: The purpose of this study was to evaluate the treatment regimens used for patients with endodermal sinus tumors (EST), and also to examine the prognostic value of surgicopathological variables.

STUDY DESIGN:: Twenty-two patients treated for pure EST, and seven patients who had mixed germ cell tumors with EST components were included. Initial surgery consisting of surgical staging to achieve optimal debulking was the principal mode of therapy. Data were obtained from patients' files, a special gynecologic oncology database, and pathology records.

RESULTS:: The median age at the time of diagnosis was 18 (range 8-45). Sixteen patients had stage I (55%), and 13 (45%) had stage II-IV disease. As an adjuvant therapy 18 patients (62%) received platin-based chemotherapy, three patients (10%) had non-platin-based chemotherapy, four patients (14%) had radiotherapy, and four patients (14%) had combined radiotherapy plus non-platin-based chemotherapy. The 5-year disease-free and overall survival rates were 47 and 48%, respectively. Survival rates were dismal in patients with stage II-IV disease (P<0.001). Platin-based chemotherapy achieved significant survival benefit (P=0.03 and P<0.001, respectively). Fertility-saving surgery had an overall survival no worse than those who underwent more extensive surgery. There was no significant survival difference with respect to age, histology, and tumor size.

CONCLUSION:: Fertility-sparing surgery with a postoperative platin-based combination chemotherapy should be the selected mode of treatment.
VARIANTS  
VIRILIZATION  
Ovarian yolk sac tumor with virilization during pregnancy: immunohistochemical demonstration of Leydig cells as functioning stroma.

Arima N, Tanimoto A, Hayashi R, Hamada T, Sasaguri Y.

Department of Pathology and Cell Biology, School of Medicine, University of Occupational and Environmental Health and Department of Obstetrics and Gynecology, National Kokura Hospital, Kitakyushu, Japan.

Pathol Int. 2000 Jun;50(6):520-5. Abstract quote  

A case is reported of yolk sac tumor occurring in the left ovary and complicated by pregnancy. The 22-year-old patient presented at 28 weeks gestation with virilization and elevated serum levels of testosterone and alpha-fetoprotein. The tumor showed the typical features of yolk sac tumor with a mixture of islands of Leydig cells.

The accumulations of Leydig cells were well demarcated from the cellular components of the yolk sac tumor and were distributed throughout the tumor, although with predominant localization at the periphery.

By immunohistochemistry the Leydig cells were intensely positive for vimentin and negative for cytokeratins, allowing clear distinction from the cell components of the yolk sac tumor, which were positive for cytokeratins and negative for vimentin. Testosterone was also identified in the cytoplasm of the Leydig cells. After tumor resection the testosterone and alpha-fetoprotein levels declined simultaneously; this, together with the immunohistochemical demonstration of testosterone, indicates that the Leydig cells were responsible for the endocrine manifestations. Furthermore, antibodies against inhibin alpha-subunit and calretinin could be used to detect the Leydig cells.

The present case, a combination of yolk sac tumor and Leydig cells acting as a functioning stroma and causing virilization during pregnancy, is very rare.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
CARCINOID  
Mucinous Carcinoid as an Unusual Manifestation of Endodermal Differentiation in Ovarian Yolk Sac Tumors.

Nogales FF, Buritica C, Regauer S, Gonzalez T.

From the *Department of Pathology, University Hospital, Granada, Spain; and daggerDepartment of Pathology, Medical University of Graz, Graz, Austria.

Am J Surg Pathol. 2005 Sep;29(9):1247-1251. Abstract quote  

We present, for the first time, two yolk sac tumors (YST) in women 37 and 18 years of age, one with a typical parietovisceral pattern and the other with a glandular pattern, which were associated with extensive areas of mucinous carcinoid (MC).

The tumor in the first case had numerous nodules of tubulopapillary YST that merged with well-differentiated MC. This patient responded well to chemotherapy. The tumor in the second case consisted of an AFP-positive glandular YST, with a glandulopapillary pattern closely resembling fetal lung type adenocarcinoma, coexisting with an AFP-negative, cytokeratin 20-positive, atypical MC; transitional areas between the two components were also seen. In the material from the recurrences and metastases; however, no YST was present, the atypical MC having become the predominant component including areas that had become carcinomatous. There was a poor response to various chemotherapeutic regimens. AFP levels became negative during the course of disease paralleling the disappearance of the YST component and the overgrowth of an increasingly anaplastic MC. The patient died 1 year after diagnosis.

We think that, in these cases, MC represented an unusual form of endodermal differentiation of the YST. It is important to differentiate the yolk sac and carcinoid components due to their different responses to chemotherapy and to evaluate the possibility of mucinous carcinoid developing into a highly aggressive carcinoma.
ENDOMETRIOID CARCINOMA ASSOCIATED  
Ovarian yolk sac tumor associated with endometrioid carcinoma and mucinous cystadenoma of the ovary.

Lopez JM, Malpica A, Deavers MT, Ayala AG.

Departamento de Patologia, Hospital de Clinicas "Jose de San Martin", Buenos Aires, Argentina.


Ann Diagn Pathol. 2003 Oct;7(5):300-5. Abstract quote  

The clinicopathologic and immunohistochemical findings of an unusual case of ovarian yolk sac tumor associated with endometrioid carcinoma and mucinous cystadenoma of the ovary are reported.

The tumor was detected in a 51-year-old postmenopausal woman who presented with abdominal swelling and a pelvic mass. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy. The tumor was confined to the right ovary and measured 16.0 cm in greatest dimension.

Microscopic examination revealed that the tumor had a yolk sac component associated with an endometrioid carcinoma, grade I, and a mucinous cystadenoma. A background of atypical endometriosis was noted. Immunoperoxidase studies showed that the yolk sac component stained diffusely with a cytokeratin cocktail and was focally positive for alpha-fetoprotein. It was negative for keratin 7. In contrast, the endometrioid carcinoma stained positive for keratin 7 in addition to the cytokeratin cocktail, but was negative for alpha-fetoprotein.

After surgery, the patient received three cycles of chemotherapy. However, the disease progressed and the patient died 10 months after the diagnosis of the ovarian tumor.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
HEPATOCYTE PARAFFIN 1  
Hepatocyte paraffin 1 antibody does not distinguish primary ovarian tumors with hepatoid differentiation from metastatic hepatocellular carcinoma.

Pitman MB, Triratanachat S, Young RH, Oliva E.

James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02114, USA.
Int J Gynecol Pathol. 2004 Jan;23(1):58-64. Abstract quote  

Anti-hepatocyte antibody, hepatocyte paraffin 1, is a monoclonal antibody that is highly specific for normal and neoplastic hepatocytes and that can differentiate hepatocytic from nonhepatocytic tumors. This marker has been rarely studied in extra-hepatic tumors and to our knowledge has not been investigated in ovarian tumors with hepatoid differentiation.

We studied hepatocyte paraffin 1 immunoreactivity in a series of ovarian hepatoid carcinomas, ovarian hepatoid yolk sac tumors (YSTs), and hepatocellular carcinomas metastatic to the ovary to assess the potential utility of hepatocyte paraffin 1 in differential diagnosis. Hepatocyte paraffin 1 positivity was seen in three of seven ovarian hepatoid carcinomas, five of eight hepatoid yolk sac tumors, and six of eight metastatic hepatocellular carcinomas. The extent of positivity ranged from <25% to >50% of the tumor cells. There was strong coarsely granular cytoplasmic staining in all three tumor types without a distinctive staining pattern in any group. The degree of hepatic differentiation correlated with hepatocyte paraffin 1 positivity in the three groups: 83% of the well differentiated tumors, 50% of the moderately differentiated tumors, and none of the poorly differentiated tumors were positive. All ovarian hepatoid carcinomas were either immunoreactive for alpha-fetoprotein or had an elevated serum alpha-fetoprotein level; more than half of these tumors were hepatocyte paraffin 1 negative. All but one hepatocyte paraffin 1 negative hepatoid yolk sac tumor and ovarian hepatocellular carcinoma were also negative for alpha-fetoprotein. In conclusion, hepatocyte paraffin 1 is positive in primary ovarian tumors with hepatoid differentiation, with the degree of hepatocyte paraffin 1 positivity correlating with the degree of hepatoid differentiation. Hepatocyte paraffin 1, however, is not useful in distinguishing metastatic hepatocellular carcinoma from primary ovarian hepatoid carcinoma or hepatoid yolk sac tumor.
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
ENDOMETRIOID TUMORS  
Ovarian endometrioid tumors with yolk sac tumor component, an unusual form of ovarian neoplasm. Analysis of six cases.

Nogales FF, Bergeron C, Carvia RE, Alvaro T, Fulwood HR.

Department of Pathology, University Hospital, Granada, Spain.

Am J Surg Pathol. 1996 Sep;20(9):1056-66. Abstract quote  

The clinical, morphological, and immunohistochemical findings in six cases of ovarian endometrioid tumors (five endometrioid carcinomas and one carcinosarcoma) with a yolk sac tumor (YST) component are described.

The age of the patients ranged from 31 to 73 years (average, 53), and only two patients were premenopausal. Two cases were stage Ia tumors, three stage III, and one stage IV. A substantial postoperative elevation of alpha-fetoprotein (AFP) was seen in two patients and a mild increase in another two. All six patients had surgery and postoperative cisplatin-based chemotherapy regimens, four of whom died of tumor 3 to 14 months after surgery without response to treatment. Only a stage Ia patient is alive and well 1 year after surgery.

The tumors were large (average, 17 cm). Benign endometrioid lesions were found in the homolateral ovary in two cases and in the contralateral ovary in another two. All cases had endometrioid ovarian carcinomas (EOC) of various types admixed with typical YST components. Immunohistochemically, EOC areas differed from YST in their positivity for OC 125, CA 19.9, and nuclear estrogen and progesterone receptors and in their negativity for AFP, which was conspicuously positive in the YST areas.

The clinicopathological profile of ovarian endometrioid tumors with YST also differs from that of YST in that it occurs in the same age range as EOC, it shows coexistence of benign endometrioid lesions, and it has a poor response to chemotherapy. The histological pattern in transitional areas may be difficult to differentiate from "endometrioid-like" (enteroblastic) YST and clear cell tumors. Ovarian endometrioid tumors with YST component should be considered a variant of endometrial carcinoma. Its recognition is necessary in view of its unusually aggressive behavior and poor prognosis.
HEPATOID CARCINOMA  
Hepatoid carcinoma of the ovary: characteristics of its immunoreactivity. A case report.

Tsung JS, Yang PS.

Department of Pathology and Laboratory Medicine, Sun-Yat Sen Cancer Center and Institute of Pharmacy, School of Medicine, National Yang-Ming University Taipei, Taiwan.
Eur J Gynaecol Oncol. 2004;25(6):745-8. Abstract quote  

Alpha-fetoprotein producing tumors other than hepatoma and germ cell tumors have been widely reported, especially in carcinoma with hepatoid differentiation (hepatoid carcinoma). Hepatoid carcinoma has mostly been found in the stomach, but also occurs in many other organs.

A rare case of hepatoid carcinoma of the ovary is presented. A 57-year-old Taiwanese woman was admitted because of lower abdominal pain. Magnetic resonance imaging showed a 10 cm right adnexal mass. She underwent a total hysterectomy and bilateral salpingo-oophorectomy with omentectomy. A right ovarian mass measuring 13 x 9 x 8 cm was found.

Microscopic examination showed characteristic features for hepatoid carcinoma. Immunohistochemical staining was performed on the tumor using a panel of eight markers (AFP, p-CEA, CD10, Hep Par 1, thyroid transcription factor-1, CK7, CK19 and CK20). This study contradicts the theory that hepatoid carcinoma derives from the surface epithelium of the ovary. Hepatoid carcinoma of the ovary commonly contains a population of clear cells, which may lead to the misdiagnosis of yolk sac tumor or clear cell adenocarcinoma that may arise in many anatomic sites. Histologically, it is also difficult to distinguish hepatoid carcinoma from hepatoid yolk sac tumor.

In such cases, demonstration of CD 10, Hep Par 1, membraneous patterns of p-CEA and CK7 would be invaluable for characterizing the tumor as hepatoid carcinoma. More studies are needed to confirm this observation.
SERTOLI-LEYDIG CELL TUMORS  
Hepatocytic differentiation in retiform Sertoli-Leydig cell tumors: distinguishing a heterologous element from Leydig cells.

Mooney EE, Nogales FF, Tavassoli FA.

Department of Gynecologic and Breast Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.


Hum Pathol. 1999 Jun;30(6):611-7. Abstract quote  

Sertoli-Leydig cell tumors (SLCT) of the ovary are rare sex cord-stromal neoplasms. A minority of SLCT are characterized by a pattern resembling that of the rete ovarii and frequently have a range of homologous and heterologous tissues. Approximately 20 cases of SLCT have been reported to have elevation of serum alpha-fetoprotein (AFP) levels, or tissue immunoreactivity for AFP, a protein usually associated with germ cell neoplasms, especially yolk sac tumor.

We identified hepatocytic differentiation in five cases of retiform SLCT (RSLCT), and confirmed immunohistochemically that these cells are hepatocytes rather than Leydig cells. Hepatocytes are positive for keratins (AE1/3 and Cam 5.2), AFP, and ferritin, negative for vimentin, and show weak to moderate staining for inhibin. Leydig cells are negative for keratins, positive for vimentin, and intensely positive for inhibin. Immunohistochemistry is needed to distinguish hepatocytic differentiation from Leydig cells with certainty.

Including the cases in this report, hepatocytic differentiation has been associated with a retiform pattern in SLCT in 14 of 25 cases (56%). The association of these two patterns appears to be characteristic of a relatively primitive sex cord-stromal neoplasm.

 

PROGNOSIS CHARACTERIZATION
Prognostic factors of patients with yolk sac tumors of the ovary.

Nawa A, Obata N, Kikkawa F, Kawai M, Nagasaka T, Goto S, Nishimori K, Nakashima N.

Department of Obstetrics and Gynecology, Nagoya University School of Medicine, Japan.
Am J Obstet Gynecol. 2001 May;184(6):1182-8. Abstract quote  

OBJECTIVE: Our purpose was to evaluate the prognostic factors in yolk sac tumors of the ovary.

STUDY DESIGN: We performed a retrospective review of 47 patients with yolk sac tumors of the ovary from 1979 to 1997.

RESULTS: Twenty-two patients had pure yolk sac tumors and 25 had germ cell tumors with yolk sac tissue as a component of the disease. The 5-year survival rate in stages I, II, III, and IV was 95%, 75%, 30%, and 25%, respectively. Patients with stage I disease had a more favorable prognosis than those with stage III and IV disease (P <.001). All patients who did not respond to chemotherapy died of this disease within 36 months of the first treatment. Chemotherapy regimens that included cisplatin gave better results than those without cisplatin (P <.05). The difference in prognosis was significant in cases in which the size of residual tumor was <2 cm in diameter (P <.01) and in cases in which ascites was either absent or <100 mL in volume (P <.05). Coexistence of other components of ovarian germ cell tumors in histologic specimens, preoperative serum alpha-fetoprotein level, fertility-sparing surgery, dissection of intrapelvic nodes, and p53 status had no significant correlation with the prognosis in this study.

CONCLUSIONS: Staging and tumor-reductive surgery strongly affected the prognosis of this disease. Tumor-reductive surgery is advisable when ascites is minimal. Cisplatin-based chemotherapy after surgery was superior to chemotherapy without cisplatin; however, p53 status seemed to have no impact on chemosensitivity in yolk sac tumors of the ovary.

Malignant germ cell tumors of the ovary: 20-year report of LAC-USC Women's Hospital.

Curtin JP, Morrow CP, D'Ablaing G, Schlaerth JB.

Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, and Department of Pathology, University of Southern California School of Medicine, Los Angeles, California, USA.

Int J Gynecol Cancer. 1994 Jan;4(1):29-35. Abstract quote  

Thirty-six patients with malignant germ cell tumors (GCT) of the ovary were treated during the period from 1971 to 1990. Twenty-five of these patients underwent initial surgery at this institution; 11 were referred after initial surgery.

Endodermal sinus tumor (EST) was the most common subtype (N = 13), followed by immature teratoma (IMT) N = 11), dysgerminoma (N = 9), and mixed germ cell tumor (N = 3). Eight of nine patients with dysgerminoma and 14 of 27 patients with non-dysgerminomatous germ cell tumors had stage I disease. Surgical staging resulted in a change of stage in 10 of 31 patients; seven were upstaged, and three were downstaged. Thirty-five of 36 patients are alive with no evidence of disease at a median follow-up of 47 months (range 12-210 months). Two of nine patients with dysgerminoma received postoperative radiotherapy; two additional patients required radiotherapy for recurrence. Twenty-five of 27 patients with non-dysgerminomatous tumors were treated with combination chemotherapy.

Second-look laparotomy was done in 18 patients, four of whom had positive results. Three of the four patients with positive second-look laparotomy results remain free of disease after salvage chemotherapy; the fourth patient died of progressive grade 3 IMT. Nineteen of 27 patients undergoing fertility-preserving surgery have resumed normal menses, and four of these have delivered normal infants. The other eight patients remain on oral contraceptives.

We conclude that surgical staging provided important information in this study of 36 patients with ovarian germ cell malignancies. As noted by many previous reports, combination chemotherapy (developed during the past 20 years) has dramatically improved prognosis for this group of patients. Second-look laparotomy (SLL), especially for patients with advanced disease, was able to identify patients requiring additional therapy. Most patients with this disease can retain their normal, uninvolved ovary with preservation of normal menstrual and reproductive function.
PREGNANCY ASSOCIATED  
Successful management of endodermal sinus tumor of the ovary associated with pregnancy.

Shimizu Y, Komiyama S, Kobayashi T, Nakata K, Iida T.

Department of Obstetrics and Gynecology, Saiseikai-Utsunomiya Hospital, Utsunomiya-shi, Tochigi, Japan.

Gynecol Oncol. 2003 Mar;88(3):447-50. Abstract quote  

BACKGROUND: Pregnancy complicated by endodermal sinus tumor of the ovary has rarely been reported.

CASE: A 32-year-old pregnant woman was found to have an ovarian tumor. At 19 weeks of gestation, tumorectomy was performed and a diagnosis of primary endodermal sinus tumor of the ovary (stage Ic) was made. Pregnancy was continued without postoperative chemotherapy. At 36 weeks of gestation, she underwent cesarean section combined with second-look laparotomy. A normal infant was delivered and there were no signs of recurrence. Subsequently, three courses of combination chemotherapy with bleomycin, etoposide, and cisplatin were administered. There was no evidence of recurrence at 27 months after initial treatment.

CONCLUSIONS: Successful management of endodermal sinus tumor of the ovary in a pregnant woman is reported.

 

TREATMENT CHARACTERIZATION
GENERAL  
CHEMOTHERAPY  
Sustained complete remission in a patient with platinum-resistant ovarian yolk sac tumor.

Jeyakumar A, Chalas E, Hindenburg A.

Oncology/Hematology Division, Department of Medicine, Winthrop University Hospital, Mineola, New York 11501, USA.
Gynecol Oncol. 2001 Sep;82(3):578-80. Abstract quote  

BACKGROUND: Yolk sac tumors of the ovary are generally very responsive to chemotherapy; however, they are difficult to manage in the setting of platinum resistance where treatment options are limited and outcomes are poorer.

CASE: We present a 39-year-old woman who had a platinum-resistant yolk sac ovarian tumor. She achieved complete remission on an innovative regimen of docetaxel, gemcitabine, and thalidomide.

CONCLUSION: The combination of docetaxel, gemcitabine, and thalidomide might be an active regimen for platinum-resistant ovarian nondysgerminomas and further investigation of this combination is warranted.
SURGERY  
Ovarian endometrioid-like yolk sac tumor treated by surgery alone, with recurrence at 12 years.

Kommoss F, Schmidt M, Merz E, Knapstein PG, Young RH, Scully RE.

Department of Pathology, University of Mainz, Mainz, D-55101, Germany.

Gynecol Oncol. 1999 Mar;72(3):421-4 Abstract quote.  

We describe the case of a stage Ia endometrioid-like yolk sac tumor (YST) of the ovary, which was originally misdiagnosed as a malignant struma ovarii and not treated with adjuvant chemotherapy.

After 12 years, a contralateral dermoid cyst was excised along with a small omental nodule of partially necrotic and calcified endometrioid-like YST. No tumor was detected in several other biopsy specimens, and a peritoneal lavage was negative for tumor cells. Since there was no evidence of remaining tumor and the serum alpha-fetoprotein (AFP) level was normal after the second operation, the patient was followed. Serial serum AFP levels remained normal for 4 months. At a second-look laparotomy after 4 months, a small tumor nodule was removed from the cul-de-sac.

Postoperatively, the patient received three cycles of BEP chemotherapy. The long disease-free interval after the first operation in spite of the presence of occult spread to the omentum and to the pouch of Douglas in this case indicates that some endometrioid-like YSTs may have an indolent course.

The present case underscores the importance of careful surgical staging and of long-term follow-up in cases of primitive germ cell tumors of the ovary.

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Last Updated September 16, 2005

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