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PubMedDate not supplied

Cytodiagnosis of vaginal endodermal sinus tumor. A case report.

BACKGROUND: Endodermal sinus tumor (EST) of vaginal origin is extremely rare, and few previously published reports have described the cytology of this tumor. CASE: A high serum alpha-fetoprotein (AFP) level and magnetic resonance imaging findings in a 10-year-old female suggested EST of the lower genital tract. Laparotomy revealed EST occupying the pelvic cavity. The patient died on the 17th postoperative day due to complications from disseminated intravascular coagulation. Autopsy was performed immediately after death. The tumor originated in the posterior vaginal wall directly below the ectocervical area. Both ovaries were involved, and the tumor proliferated to occupy the cavity of the pelvis minor. A vaginal smear, obtained on two occasions, revealed severe necrosis with few cells, and a diagnosis could not be made. An imprint smear of the tumor obtained during surgery showed relatively characteristic cytologic findings of EST. Immunocytochemical and immunohistochemical studies revealed staining positive for AFP. CONCLUSION: Vaginal EST usually occurs in patients under 3 years of age. This 10-year-old patient was the oldest among the reported cases. Vaginal EST is clinically similar to sarcoma botryoides and clear cell carcinoma, and a differential diagnosis is necessary. Immunocytostaining for AFP is essential for a definitive cytologic diagnosis of EST.

Child↗
PubMed1984

Normal pregnancy and delivery following conservative surgery and chemotherapy for ovarian endodermal sinus tumor.

Management of younger patients with early stage ovarian endodermal sinus tumor who desire to have children can be a challenging problem. However, after an adequate and thorough staging procedure for the patient's tumor status, a Stage Iai endodermal sinus tumor may be treated with conservative surgery only, followed by chemotherapy. Successful pregnancy and delivery following unilateral adnexectomy and chemotherapy for ovarian endodermal sinus tumor have been reported only once [J. P. Forney, Obstet. Gynecol. 52, 360 (1977)]; the second reported case is presented here. It should be pointed out that strict follow-up and reliability of the patient are mandatory for such conservative therapy.

Adult↗
PubMed1976

[Primary combined chemotherapy following removal of an endodermal sinus tumor or yolk-sac tumor of the ovary (author's transl)].

The yolk-sac tumor or endodermal sinus tumor is a rare but highly malignant ovarian teratoma with its highest incidence in young women. The tumor is relatively resistant to radiotherapy but sensitive to combined chemotherapy and should therefore be differentiated in the diagnosis from embryonal carcinoma and radio-sensitive dysgerminomas of the ovary. It is possible that the determination of alpha-fetoprotein is of differential diagnostic value. Two patients are alive and well 8 months and 2 1/2 years following removal of a yolk-sac tumor and primary chemotherapy. One patient received chemotherapy for peritoneal recurrence after removal of the tumor and radiotherapy. She died 1 1/2 years following the primary operation. Long term chemotherapy of the yolk-sac tumor is indicated following operation, irrespective of the stage of the tumor.

Adult↗
PubMed2002

New perspectives on therapy for vaginal endodermal sinus tumors.

PURPOSE: Malignant germ cell tumors account for 3% of childhood cancers. Endodermal sinus tumor, the most common malignant germ cell tumor, requires treatment primarily with chemotherapy and surgery is reserved as a last resort. It is rare for the vagina to be the primary site of endodermal sinus tumor, and we report on our experiences with this phenomenon at a single institution. MATERIALS AND METHODS: We retrospectively reviewed the clinical features, treatment and outcomes of 3 children with vaginal endodermal sinus tumor. RESULTS: Initial treatment was combination chemotherapy, in 2 patients. alpha-fetoprotein decreased rapidly and returned to normal in both. One patient is disease-free 7 years after chemotherapy and the other patient is currently disease-free with a normal alpha-fetoprotein 3 months after induction chemotherapy. In the remaining case progressive disease developed following initial chemotherapy and subsequent salvage surgery combined with radiation, chemotherapy and ultimately autologous bone marrow transplant was performed. The patient has remained disease-free for 6.5 years since completing this extensive therapy. CONCLUSIONS: Endodermal sinus tumor of the vagina is rare. All of our patients presented with painless bleeding of no obvious source. In such cases one must maintain a high index of suspicion for possible underlying pathological conditions even if ultrasound is negative. Evaluation must include endoscopic examination of the lower genitourinary tract. Bone marrow transplant should be considered as a last therapeutic resort in salvage cases of unresponsive vaginal endodermal sinus tumor.

Combined Modality Therapy↗
PubMed1989

Intestinal variant of ovarian endodermal sinus tumor.

A case of ovarian endodermal sinus tumor (EST) with exclusive intestinal differentiation in a 28-year-old black woman is reported. The tumor cells form irregular glands with focal early Paneth cell differentiation resembling intestinal Luberkuhn's crypts. Clinical and pathological features as well as immunohistochemical findings are described.

Adult↗
PubMed1997

Primary endodermal sinus tumor of the vulva: a case report and review of the literature.

BACKGROUND: Extragonadal endodermal sinus tumors arising in the external genitalia represent an exceedingly rare malignancy in women. Six cases of endodermal sinus tumors of the vulva have been reported to date, with three cases failing to respond to conservative surgery and vincristine-based chemotherapy. We report a seventh case of vulvar endodermal sinus tumor that was treated with radical surgery and platinum-based chemotherapy. CASE: RT is an 18-year-old female who presented with a vulvar mass that was diagnosed as endodermal sinus tumor at the time of biopsy. She was subsequently treated with modified radical vulvectomy and ipsilateral groin lymphadenectomy, followed by bleomycin, etoposide, and cisplatin chemotherapeutic regimen. She has since remained free of disease for 18 months as evidenced by serum alpha-fetoprotein and physical exam at 18 months. CONCLUSIONS: Vulvar endodermal sinus tumors represent a very small number of germ cell tumors in women. Based on the previous accounts, this disease appears to be more fatal than endodermal sinus tumor arising at other sites. These tumors also have a predilection for local metastasis. Due to the previous accounts, we chose to treat this patient with radical surgery and platinum-based chemotherapy. This treatment regimen has resulted in a disease-free state for 18 months.

Adult↗
PubMed1997

Primary endodermal sinus tumor presenting with spinal cord compression. Case report.

Endodermal sinus tumor is an uncommon malignant germ-cell neoplasm. These tumors usually present in childhood or young adulthood as testicular or ovarian masses; however, mediastinal and intracranial tumors have been described. The authors report the occurrence of a primary paraspinal endodermal sinus tumor in a 21-month-old boy who presented with thoracic spinal cord compression. A review of the literature failed to reveal a similar case. The clinical presentation, radiographic characteristics, operative findings, and patient outcome are discussed.

Endodermal Sinus Tumor↗
PubMedDate not supplied

Genetic analysis of childhood endodermal sinus tumors by comparative genomic hybridization.

Childhood endodermal sinus tumors (CEST) are a distinct category of germ cell tumors that involve the testis and extragonadal sites of young children. Recurrent deletions of 1p and 6q have been reported by classic cytogenetic analysis of a small number of cases. Comparative genomic hybridization, a technique that screens the entire genome for genetic abnormalities, is applied to additionally define the genetic changes present in CESTs. Sixteen frozen CESTs (10 testicular, 6 extragonadal) obtained from Pediatric Oncology Group-affiliated institutions or from the Cooperative Human Tissue Network were analyzed. The most common changes were gain of 20q (10 tumors), 1q (6 tumors), 11q and 22 (4 tumors each), and loss of 6q (8 tumors with common deleted region of 6q24-qter), 16q (4 tumors), and 1p (4 tumors). Localized regions of gain were identified at 8q24 (2 tumors both showing c-myc amplification by fluorescence in situ hybridization). Gain of 12p, characteristic of adolescent germ cell tumor, was present in one testicular tumor. Comparative genomic hybridization was useful in defining genetic differences between adult and childhood tumors, in determining the common regions deleted on chromosome 6, and in identifying other involved loci to be correlated with clinical parameters in future studies.

Child, Preschool↗
PubMed1979

Endodermal sinus tumor of the ovary. Clinicopathologic study of 6 cases.

6 cases of endodermal sinus tumor of the ovary are presented. In 4 patients pure endodermal sinus tumor was found microscopically. 1 patient had endodermal sinus tumor in one ovary and gonadoblastoma in the contralateral one. In another case endodermal sinus tumor was accompanied by an embryonal teratoma. Histologically, the tumor had characteristic features with meshwork of spaces and channels lined by embryonal cells, glomerulus-like structures known as Schiller-Duval bodies, solid aggregates of epithelial cells, hyaline basement membranes and round, PAS-positive small globules found both intra- and extracellulary. In 1 patient the elevated serum alpha-fetoprotein was stated. All patients were treated surgically with adjunctive radiation and/or with chemical agents. None of them were cured. The median duration of survival amounted to 8.5 months. Discussing the value of the more recent approach to diagnostic and therapeutic methods found in the literature, it must be emphasized that the demonstration of elevated serum alpha-fetoprotein in patient with that tumor lend not only further support to its yolk sac origin but also might be useful to monitor response to the therapy applied. It is also of prognostic significance by indicating the presence of residual or recurrent disease, even in its subclinical stage. Combined postoperative irradiation and triple chemotherapy according to the VAC regimen of patients can prevent recurrence and in some cases even may cause permanent remission of the neoplasm.

Adolescent↗
PubMed1986

Endodermal sinus tumor of the ovary associated with pregnancy.

The association of endodermal sinus tumor of the ovary with pregnancy is a rare event. Reported is a patient with stage Ic endodermal sinus tumor diagnosed in the 25th week of gestation. She received two cycles of combination chemotherapy consisting of vinblastine, bleomycin, and cisplatin, and delivered a healthy male infant by cesarean section at 32 weeks' gestation. She subsequently completed three more cycles of chemotherapy and remains alive and well. This is the first reported case of a patient with endodermal sinus tumor treated with combination chemotherapy during pregnancy that had a successful outcome for both mother and infant. The literature concerning the association of endodermal sinus tumor and pregnancy and the use of chemotherapy during pregnancy is reviewed.

Adult↗
PubMed1992

Primary endodermal sinus tumor of the liver detected by CT.

We report a case of primary endodermal sinus tumor of the liver. Endodermal sinus tumors are rare neoplasms which usually arise in the testis or ovary. Extragonadal endodermal sinus tumors are uncommon and primary tumors of the liver are very rare. The tumor was detected using CT and the diagnosis was made by percutaneous biopsy.

Female↗
PubMed1990

Management of advanced endodermal sinus tumor of the ovary with preservation of reproductive function.

Endodermal sinus tumor of the ovary occurs primarily in children and young adults. This tumor is classified as a germ cell tumor which comprises approximately 5% of all ovarian tumors. In the past, patients with this highly malignant tumor had low survival. However, with the evolution of effective adjunctive chemotherapeutic regimens, specifically vincristine, actinomycin D, and cyclophosphamide, survival rates have improved. In an attempt to continuously improve survival rates, other combination chemotherapeutic regimens are currently under investigation. Unlike epithelial ovarian malignancies, endodermal sinus tumor can be monitored by a specific tumor marker, alpha-fetoprotein (AFP). Serum levels of AFP can be followed from the preoperative period through the course of chemotherapy. When elevated, AFP has excellent predictability that persistent disease is present. Because this disease presents primarily in young females, preservation of reproductive function is of utmost importance. We report a case of advanced endodermal sinus tumor which was managed with conservative surgery only, followed by radical combination chemotherapy, resulting in preservation of normal reproductive function and total resolution of disease documented by second-look laparotomy.

Adolescent↗
PubMedDate not supplied

Endodermal sinus tumor (yolk-sac carcinoma) of the orbit.

A 15-month-old boy with rapidly progressive proptosis had an unusual malignant germ-cell tumor of the orbit and nasopharynx identified as an endodermal sinus tumor (yolk-sac carcinoma). Endodermal sinus tumors arising in extra gonadal locations are highly malignant and generally lethal. The boy was treated with radiation and three-drug chemotherapy and has survived more than eight years.

Exophthalmos↗
PubMed2003

Successful management of endodermal sinus tumor of the ovary associated with pregnancy.

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.

Adult↗
PubMedDate not supplied

Diagnostic value of hyaline globules in endodermal sinus tumor: report of two cases.

Two cases of ovarian endodermal sinus tumor are reported. The cytologic preparations were obtained from ascitic and/or autopsy specimens. The findings on Papanicolaou-stained smears included clustering of malignant cells, pleomorphism of nuclei and cytoplasmic vacuolization and thus resembled those seen in cases of mucinous cystoadenocarcinoma of the ovary. Hyaline globules, characteristic of the alpha-fetoprotein-synthesizing (AFP-synthesizing) cells found in endodermal sinus tumor, were observed inside the cells with periodic-acid-Schiff (PAS) stain. The presence of PAS-positive hyaline globules can be regraded, therefore, as a diagnostic clue to endodermal sinus tumor in an appropriate clinical setting and in the presence of AFP production.

Adult↗
PubMed1979

Value of tumor markers in the treatment of endodermal sinus tumors and choriocarcinomas in the pineal region.

A case of embryonal carcinoma in the pineal region of a 17-year-old boy is presented. The tumor included elements of choriocarcinoma and endodermal sinus tumor, and the use of human chorionic gonadotropin and alpha-fetoprotein as tumor markers is discussed. The markers were demonstrated both within the tumor and in the cerebrospinal fluid (CSF) and blood. The patient was treated with a postoperative program of irradiation and cancer chemotherapy, and at follow-up examination 20 months after operation no signs of residual tumor were present. It is suggested that human chorionic gonadotropin and alpha-fetoprotein should be measured in the blood and CSF before the treatment of midline tumors.

Adolescent↗
PubMed1989

Immunohistochemical differentiation of clear-cell carcinoma of the female genital tract and endodermal sinus tumor with the use of alpha-fetoprotein and Leu-M1.

The morphologic differentiation between clear-cell carcinoma and endodermal sinus tumors is difficult at times. To improve the accuracy of the diagnosis, the authors studied nine ovarian and eight vaginal clear-cell carcinomas and seven endodermal sinus tumors of the ovary by immunohistochemical methods with the use of antibodies to alpha-fetoprotein and Leu-M1. Sixteen (94.1%) of the 17 clear-cell carcinomas and two (28.5%) of the seven endodermal sinus tumors reacted for Leu-M1, whereas six (85.7%) of the seven endodermal sinus tumors and three (17.6%) of the 17 clear-cell carcinomas stained for alpha-fetoprotein. Three clear-cell carcinomas and two endodermal sinus tumors showed immunoreactivity for both markers. No reactivity for either of these markers was present in one endodermal sinus tumor and one clear-cell carcinoma. All 13 tumors that stained only for Leu-M1 proved to be clear-cell carcinomas, and the four that reacted exclusively for alpha-fetoprotein were endodermal sinus tumors. Therefore, the authors concluded that positive immunostaining for Leu-M1 and negative immunostaining for alpha-fetoprotein support the differential diagnosis of clear-cell carcinoma, whereas a positive reaction for alpha-fetoprotein and a negative reaction for Leu-M1 favor a diagnosis of endodermal sinus tumor. However, positive or negative staining for both markers appears to have no diagnostic value.

Adenocarcinoma↗