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Background: The objective of the study was to analyze the clinical features, treatment, and outcomes of primary vaginal endodermal sinus tumor (EST) in infants and children treated in a tertiary center., Methods: Clinical data of patients with pathologically confirmed primary vaginal EST in our hospital from January 1997 to December 2017 were retrospectively reviewed and analyzed., Results: A total of 21 patients were included in this study. The median age at diagnosis was 11 months (range, 4-44 months). The most common manifestations were abnormal vaginal bleeding, and a polypoid mass protruding from the vagina. Chemotherapy based on PEB (cisplatin, etoposide, bleomycin) regimen was given, and serum alpha-fetoprotein (AFP) levels dropped to normal levels after 2 to 4 cycles of chemotherapy (median, 2 cycles). After 3 to 13 cycles of chemotherapy, with a median of 5 cycles, 20 patients achieved complete remission (95.2%). The median follow-up time was 80 months (range, 4-281months). At the time of the last follow-up, 19 cases were alive without disease, and the survival rate was 90.5%., Conclusion: Vaginal EST is a very rare malignant germ cell tumor and is sensitive to chemotherapy. Conservative surgery combined with PEB chemotherapy is an effective way of treatment. Serum AFP and imaging examinations can monitor the treatment response and recurrence., (© 2022. The Author(s).)

Objective: To report diagnosis, treatment, and outcomes of vaginal yolk sac tumor (YST) cases at a single institution and review literature on vaginal YST to outline advancements in diagnosis, treatment, and survival., Design: Retrospective chart review of female patients less than 21 years of age with pathologic diagnosis of vaginal YST treated at a large children's hospital, and summary of a 100-year review of the literature on vaginal yolk sac tumor., Setting: Children's Healthcare of Atlanta, a tertiary center in Atlanta, GA., Participants: Female patients less than 21 years of age diagnosed with vaginal YST., Results: Two cases of vaginal YST at our institution are outlined. Both patients presented within the first 2 years of life with vaginal bleeding and were treated successfully with chemotherapy alone. After review of the literature, 137 cases of vaginal YST were found. The mean age at diagnosis was 11 months, and all patients presented with vaginal bleeding. Before 2000, more radical treatments were pursued, and 40% resulted in death. Since the year 2000, treatment has shifted toward chemotherapy and more conservative surgical management, with 51% of vaginal YST cases treated with chemotherapy alone with 92% of patients alive at time of publication., Conclusion: Our cases contribute to the limited literature demonstrating the efficacy of conservative management of rare cases of vaginal YST with chemotherapy alone. This case series and review of the literature provide mounting evidence that vaginal YST should be in the differential diagnosis in young girls with vaginal tumors, and conservative management of vaginal YST has excellent outcomes., (Published by Elsevier Inc.)

The mother of a 9-month-old female infant complained that her child was unable to pass urine at the same time noticing a mass protruding from the vaginal orifice.The infant had a single episode of vaginal bleeding.The primary concern of the mother was the inability of the daughter to micturate. Malignant germ cell tumour arising from an infant vagina is rare and accounts for about 3% of all paedriatic malignancies. These are also referred to as endodermal sinus tumours or yolk sac tumours, and are mostly the commonest form of infant vaginal malignancies encountered. A diagnosis of endodermal sinus tumour was established based on the histology and raised α-fetoprotein levels.These tumours had Schiller-Duval bodies which are primarily blood vessels surrounded by primordial germ cells and were periodic acid shift (PAS) positive diastase resistant hyaline globules which also stain positive with α- fetoprotein which is an important diagnostic feature. Tumours with high α-fetoprotein levels have a poorer prognosis. However, they respond satisfactorily to chemotherapy.

A 14-month-old girl was evaluated for intermittent vaginal bleeding. Vaginoscopy revealed a friable tumor, and biopsy results confirmed a yolk sac tumor. Alpha-fetoprotein was elevated (1386 ng/mL) at diagnosis but quickly normalized with chemotherapy. The patient remained tumor-free 12 months after diagnosis without the need for radical surgery. Although rare, malignant tumors of the vagina must be included in the differential diagnosis of prepubertal girls who present with vaginal bleeding. Primary yolk sac tumor of the vagina is seen in girls less than 3 years of age and is treated with chemotherapy with or without surgical excision. Prognosis is good with appropriate treatment., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Vaginal yolk sac tumors (YSTs) are rare malignant germ cell tumors largely affecting children younger than 3 years. Because of their low incidence, there is no consensus regarding diagnosis and treatment. In this article, we describe the presentation, diagnosis, treatment, and outcomes of 16 patients with vaginal YSTs diagnosed and managed at our center., Methods: Diagnoses of YST of the vagina were confirmed by 2 experienced pathologists. All patients were treated with bleomycin, etoposide, and cisplatin (PEB) combination chemotherapy alone. Complete remission (CR) was defined by a normal serum α-fetoprotein (AFP) level, no tumor detected on computed tomography, and negative pathology results. Biochemical CR (bCR) was defined by a normal serum AFP level. Long-term follow-up was completed according to our regulations., Results: The median age of patients at diagnosis was 10 months (range, 5-44 months), and all patients presented with abnormal vaginal bleeding and/or vaginal discharge. Serum αAFP is a sensitive tumor marker, and it was markedly elevated in all patients (median 4848 ng/mL; baseline at our hospital is <20 ng/mL). Thirteen patients completed a chemotherapy regimen consisting of PEB alone without surgery. Importantly, all patients achieved CR. Patients received additional cycles of consolidation chemotherapy after bCR and there are no cases of recurrence to date., Conclusions: We propose a contemporary treatment strategy in line with current practice. First, all suspected cases of vaginal YST should be diagnosed by histopathological examination and serum AFP levels. Second, nonsurgical treatment with PEB chemotherapy should be initiated until patients achieve bCR, followed by an additional 2 cycles of consolidation therapy to optimize results and reduce the risk of remission., Competing Interests: The authors declare no conflicts of interest., (© 2017 by the International Gynecologic Cancer Society and the European Society of Gynaecological Oncology.)

Six patients (aged 3-36 mo) with vaginal tumors (rhabdomyosarcoma and endodermal sinus tumor [EST]; n = 3 each) received intraarterial chemotherapy (IAC) and intravenous chemotherapy. Patients underwent internal iliac artery infusion with cisplatin, pirarubicin, and vindesine. Intravenous chemotherapy with vindesine, ifosfamide, and etoposide was administered after 3 weeks. Vaginal tumors disappeared in all patients after 2 or 3 cycles of alternating therapy. Two patients underwent resection of pelvic metastases. Intravenous consolidation chemotherapy was applied. Four patients were disease-free at a median follow-up of 5.8 years. One patient had pelvic recurrence treated with "salvage" therapy with IAC and surgery and was disease-free for 2.5 years., (Copyright © 2016 SIR. Published by Elsevier Inc. All rights reserved.)

Vaginal yolk sac tumor is a rare malignant germ-cell tumor occurring most commonly in young girls. The treatment has evolved over the last 3 decades from radical surgery to conservative surgery with chemotherapy to chemotherapy alone. Here we present a case of a 6-month-old girl successfully treated with upfront surgery followed by chemotherapy. We include a literature review of studies on vaginal yolk sac tumor published in the last 30 years. We discuss the role of upfront surgery where possible followed by chemotherapy as a safe alternative to chemotherapy alone for the treatment of this rare malignancy.

Malignant germ-cell tumors (MGCT) are rare tumors of childhood accounting for less than 3% of pediatric malignancies. Yolk sac (endodermal sinus) tumor is one of the malignant germ cell tumor that usually involves the gonads (ovaries and testes). Its occurrence in the vagina is extremely rare. We report a 6-months old girl presented with a vaginal mass diagnosed as a yolk sac tumor. This diagnosis is confirmed by histopathologic examination, immunehistochemical studies as well as elevated serum alpha fetoprotein (AFP).

We report a case of a vaginal yolk sac tumor in a 5-month-old female infant who presented with short history of bleeding per vagina. Magnetic resonance imaging showed a mass occupying most of the vagina that had lobulated outlines and heterogeneous echo texture. The serum alpha-fetoprotein was elevated, and a biopsy revealed a vaginal yolk sac tumor. The patient was given six cycles of chemotherapy and continues to be disease-free on follow up. To preserve sexual and reproductive function, we encourage consideration of chemotherapy as a sole modality to treat this rare tumor.

Objective: Six cases of endodermal sinus tumor (EST) of the vagina were reported focusing on the results of conservative treatment and outcome of long-term follow up., Methods: We reviewed a cohort of six cases with EST of the vagina diagnosed and treated in our hospital. CT scan, detection of serum AFP level, examination under anesthesia and biopsy were performed before and after treatment to evaluate the tumor status. The diagnoses were confirmed by pathological and imaging findings. All patients were treated with bleomycin, etoposide and cisplatin (BEP) combination chemotherapy alone. Complete remission (CR) includes the normal level of serum AFP, no tumor detected by CT scan and the negative result of pathology. Long-term follow up was carried out according to our regulation., Results: The mean onset age was 18.2 months. The average tumor size was 4.5 cm. Serum AFP level was markedly elevated before treatment and decreased dramatically after chemotherapy and drop to the normal level after two to three courses of chemotherapy (average 2.5 courses). The patients received averagely six courses of chemotherapy and all the six patients obtained CR. The mean follow-up time is 75.5 months and there was no evidence of recurrent disease., Conclusion: The result of this conservative treatment for EST of the vagina is excellent. BEP chemotherapy without surgery should be considered as a good choice of treatment. Close follow up with serial determinations of serum AFP level together with imaging tests and physical examination in the first two years of follow-up are recommended., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Vaginal yolk sac (endodermal sinus) tumors were diagnosed in two girls (ages 12 and 46 months). In both, the only manifestation was persistent vaginal bleeding. Pelvic ultrasonography, computed tomography, and magnetic resonance imaging revealed solitary vaginal masses (diameter, 5 and 2 cm, respectively). Serum alpha-fetoprotein was highly elevated in one patient and normal in the other. Biopsy was performed in the first patient, and a tumor excision, in the second. Combination chemotherapy with cisplatin, etoposide, and bleomycin or carboplatin was administered to the first patient, and shortly thereafter, the tumor size decreased by more than half; serum alpha-fetoprotein was normalized after four chemotherapy cycles. After chemotherapy, magnetic resonance imaging revealed a small residual lesion, however the second biopsy revealed no viable tumor cells. In the second patient, no visible tumor was observed after chemotherapy by vaginoscopy. Both patients are well at 19 and 14 months after diagnosis, respectively.

Malignant tumors of the vagina in infants and children are rare, with primary germ cell tumor (endodermal sinus tumor [EST]) being rarer, which carries a dismal prognosis. This tumor is often clinically mistaken as botryoid rhabdomyosarcoma and, on histopathological examination, is often misdiagnosed as clear cell adenocarcinoma. Two cases of EST of the vagina in infants aged 9 and 17 months are reported. Both the patients presented with bleeding per vagina and clinically and by ultrasonography were diagnosed as sarcoma botryoides. Both were diagnosed as primary yolk sac tumors. The serum alfa fetoprotein (AFP) level was 4325 and 9328 ng/ml, respectively. One patient aged 9 months expired 2 months after receiving chemotherapy. The other, aged 17 months, was given chemotherapy followed by surgery with reduction of the AFP levels.

Malignant germ cell tumors of the vagina represent a critical site for local treatment. We report on our experience with two vaginal endodermal sinus tumors. Treatment with PEB regimen induced both tumor regression and alpha fetoprotein normalization. One patient had a residual lesion that was biopsied twice and no viable tumor cells were ever found. Both patients remained alive and disease-free, without having had surgical procedures except for biopsies. Platinum-based chemotherapy is able to achieve complete remissions and should be considered for vaginal endodermal sinus tumors.

Background: Mixed yolk sac tumors of the ovary are biologically aggressive even in early stage disease., Case: A 41-year-old woman presented with a large pelvic mass and anterior vaginal wall tumor. At surgery vaginal biopsies were performed followed by an exploratory laparotomy with resection of the mass. Pathology of the ovary revealed a primary yolk sac tumor associated with poorly differentiated endometrioid and undifferentiated carcinoma with vaginal metastasis only. She was initiated on bleomycin, etoposide, and cisplatin, with three additional cycles of etoposide and cisplatin. Initially the patient experienced a complete response, however her disease recurred and she currently is dead of her disease., Conclusion: To our knowledge this is the first case of a mixed ovarian germ cell tumor with vaginal metastasis.

Endodermal sinus tumor (EST) of the vagina is a rare malignancy usually diagnosed before 3 years of age. Historically, the approach to therapy has included radical surgical resection, with adjuvant irradiation, and chemotherapy. An infant presented with vaginal bleeding, imaging evidence of a vaginal mass and an elevated alpha-fetoprotein level. Examination under anesthesia with vaginal biopsies confirmed the diagnosis of an EST (yolk sac) tumor of the vagina. After 5 cycles of chemotherapy, the alpha-fetoprotein had normalized and repeat vaginal biopsies for suspected residual disease was negative for malignancy. To allow preservation of sexual and reproductive function, chemotherapy as a sole modality of treatment for EST should be considered.

A 7-month-old infant was noted to have vaginal bleeding that was accompanied by a discharged tumor fragment. The histological diagnosis was endodermal sinus tumor. Her serum alpha-fetoprotein (AFP) was increased to 358.7 ng/mL, and magnetic resonance imaging showed a 1.8 x 1.0 cm tumor in the vagina. She received combination chemotherapy with cyclophosphamide, pirarubicin, carboplatin, and etoposide. The tumor in the images disappeared and the serum level of AFP returned to the normal range after 2 cycles. Treatment was complete without surgical or radiological therapy. More than 45 months after the completion of chemotherapy, she is alive without signs of recurrence.

Yolk sac tumors are common in children. By virtue of being chemosensitive, they are amenable to cure by chemotherapy alone and radical surgery is often not required. Yolk sac tumors occurring in the vagina are rare and thus may not be recognized early or may be inadvertently subjected to radical surgery. The authors report a case that presented to them after radical surgery with elevated Alpha-fetoprotein level is reported. The management of this case and review of the relevant literature are discussed here.

Objectives: To describe the clinical characteristics and the role of chemotherapy in endodermal sinus tumor of the vagina., Method: Two patients with endodermal sinus tumor of the vagina were presented focusing on the clinical manifestations and outcome of the chemotherapy., Results: Patient's age was quite young, 2 and 3 years old respectively. Vaginal bleeding and a polypoid and fragile tumor of the vagina were main clinical features. Elevated serum alpha-FP was found before chemotherapy and dropped dramatically to normal if the tumor was sensitive to chemotherapy. Diagnosis was made by pathology and alpha-FP immunohistochemical staining. Both two patient was well responded to cisplatin vincristine bleomycin (PVB) and cisplatin etoposide bleomycin (PEB) chemotherapy. Clinical and pathological complete remission was obtained after 2-3 courses of chemotherapy without radical surgery and radiotherapy., Conclusions: Endodermal sinus tumor of vagina in infant was very sensitive to the chemotherapy. Serum alpha-FP was very useful in diagnosis and monitoring of the disease.

Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.