Yolk Sac Tumor of the Ovary in Mosaic 46XX Turner Syndrome.

Introduction: Correlation of Turner syndrome (TS) with germ cell malignancy is acknowledge in TS patient with Y chromosome material but not otherwise. This case report wishes to highlight yolk sac tumor occurrence in patients with TS 46XX karyotype mosaicism. Case Report: A 23-year-old nulligravid woman was admitted with abdominal mass and vaginal bleeding. She had primary amenorrhea and had already been diagnosed with TS. Her karyotype was 46XX with 5% X mosaicism. Ultrasonography revealed a solid mass measuring 14.05 x 10.99 cm based on the International Ovarian Tumor Analysis (IOTA) simple rule, M1 and M2. During surgery, a solid mass originates from her left ovary measuring 20 x 15 x 15 cm with adhesion to omentum, ileum, and caecum was found. Pathology examination reveals it's an endodermal sinus tumors (EST). Discussion: TS with Y cells are closely linked with germ cell malignancy but not otherwise. It's still unclear what causes the malignancy in such cases. Conclusion: The present report illustrates a rare case of EST occurred in a TS patient with 46XX mosaicism.

Case Report: Primary endodermal sinus tumor on the abdominal wall near the right liver: a diagnostic pitfall.

Background: Endodermal sinus tumors are rare, especially extragonadal endodermal sinus tumors, which often occur in the midline of the brain, neck, chest, and abdomen. Case summary: We present the case of a 37-year-old woman with a mass on the right edge of the liver. Color Doppler ultrasound, computed tomography, and magnetic resonance imaging examinations were performed before the operation. Given these results and the elevation of alpha-fetoprotein, the diagnosis of hepatocellular carcinoma was made. Postoperative pathological examination indicated an endodermal sinus tumor on the abdominal wall near the right liver. The causes of misdiagnosis were analyzed, and the related literature was reviewed. Conclusion: Primary endodermal sinus tumors on the abdominal wall near the right liver are easily misdiagnosed as hepatocellular carcinoma due to liver compression and elevated alpha-fetoprotein. The key point of differentiation is the wide basal connection between the tumor and the abdominal wall near the liver. In addition, the enhancement mode of endodermal sinus tumors is different from the enhancement pattern of hepatocellular carcinoma. Core tip: Extragonadal endodermal sinus tumors often occur in the midline of the body. Here, we present a case of a primary endodermal sinus tumor on the abdominal wall near the right liver for the first time.

Primary endodermal sinus tumor originating from the sacral ligament: a case report and review of the literature.

BACKGROUND: Endodermal sinus tumor (EST) is a malignant tumor originating from the ovary or testis. In most case, ultrasound examination shows ovarian mass. But there is a special kind of extra-gonadal endodermal sinus tumor, which occur in organs other than gonads with insidious onset. Here we reported a case of endodermal sinus tumor, which originated from the sacral ligament presenting as an acute lower abdominal pain. CASE PRESENTATION: A 14-year-old girl was admitted to the hospital because of acute lower abdominal pain. The ultrasound showed a mass with 72 mm × 64 mm × 50 mm in Douglas, and there was no abnormality in bilateral ovaries and fallopian tubes. Laparoscopic exploration showed a large amount of blood clots in the pelvic cavity. After removal of the blood, we found rotten fish-like tissue in the left sacral ligament, rapid pathology suggested endodermal sinus tumor. After the operation, we retrospectively examined the value of alpha-fetoprotein (AFP), which was found to be elevated, and post-operative paraffin pathology confirmed the diagnosis. After four cycles of BEP chemotherapy, exploratory laparotomy was performed to remove the visible lesion, but postoperative pathology showed no abnormality. At the one-year follow-up, the patient remained recurrence-free. CONCLUSION: Extra-gonadal germ cell tumors are rarely reported. When young teenagers complain of acute lower abdominal pain with elevated AFP, but there was no lesion in bilateral ovaries and fallopian tubes, we must think about the possibility of endodermal sinus tumors. Accurate diagnosis facilitates complete resection of lesions and improves patient's outcomes.

Primary Endodermal Sinus Tumor of the Intraspinal Cavity: A Case Report and Review of the Literature.

Endodermal sinus tumor (EST) occurs most frequently in the gonads and is relatively rare in other sites, particularly in the spinal cavity. We report a 19-year-old woman who presented with back pain and weakness of both lower extremities who was found to have an EST in the spinal canal cavity. She had severely elevated serum alpha-fetoprotein (AFP) level at presentation. Magnetic resonance imaging (MRI) revealed the mass in the spinal canal. The tumor was excised. Serum AFP returned to normal after three cycles of chemotherapy. We describe the imaging findings and the macroscopic and microscopic features of this rare tumor. EST is a relatively rare malignant germ cell tumor that usually originates in the gonads and has poor prognosis. This is a rare case of the primary EST in the spinal canal. Radiologists need to be aware of the MRI appearance of extragonadal EST.

Renal Yolk Sac Tumor Clinically Misdiagnosed as Nephroblastoma: A Case Report.

Background: Yolk sac tumor is a germ cell tumor (GCT) that occurs in infants and adolescents and affects various sites. There is a trend to treat pediatric renal tumors before a tissue diagnosis. We report a renal yolk sac tumor clinically misdiagnosed as Wilms tumor, based on ultrasound (US) and MRI.Case Report: This 21-month-old male infant was discovered to have a space occupying lesion in the right kidney. Because the tumor was large, initial radiotherapy preceded surgical resection. Histologically, the tumor was a yolk sac tumor.Conclusion: Imaging examination of renal yolk sac tumor can easily be misdiagnosed as Wilms tumor. SIOP treatment plan for Wilms tumor requires preoperative chemotherapy, which is different from the treatment regimen for yolk sac tumor. Preoperative alpha-fetoprotein could have been helpful in avoiding this clinical misdiagnosis.

Yolk Sac Tumor of the Parotid Gland in a Child and its Differentials.

Yolk sac tumors (YST) (or endodermal sinus tumors) are rare neoplasms of germ cell origin that have been reported in gonadal sites (testis and ovary). Extragonadal YST are uncommon and are extremely rare in the extracranial head-and-neck regions. Here, we present a rare case of a 1-year-old male with isolated YST of the right parotid gland initially diagnosed as malignant epithelial neoplasm with possibilities of mucoepidermoid carcinoma and epithelial-myoepithelial carcinoma.

Endodermal sinus tumor with pancreatic origin: A case report.

Endodermal Sinus tumor is a rare malignant type of germ cell tumor (GCT). Primary endodermal sinus tumor, especially in the head of pancreas, is extremely rare. The case under consideration is of a 22 year old male who presented with pancreatic mass causing postprandial nausea and vomiting, and raised AFP levels. The biopsy of the mass revealed the endodermal sinus tumor, and the pancreatic origin was confirmed by a CT scan and a negative scrotal ultrasound. As the tumor was unresectable initially, a gastrojejunostomy was done to relieve the obstructive symptoms. Whipple procedure was performed two months after 4 cycles of BEP chemotherapy to remove the residual tumor in pancreas. It is crucial to highlight this uncommon case in order to increase surgeons' and oncologists' knowledge of the malignant extra-gonadal GCTs diagnosis and treatment.

Primary vaginal endodermal sinus tumor in infants and children: experience from a tertiary center.

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.

Intracranial Seeding Following Surgery and Chemotherapy in a Child with a Spinal Cord Endodermal Sinus Tumor: A Case Report.

We herein report a case of an extremely rare spinal cord endodermal sinus tumor (EST). A nine-year-old boy presented with progressive paraparesis, hypesthesia, and urinary retention. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed multiple intradural enhancing lesions at T1 to T9 without evidence of intracranial tumors. He underwent partial resection of the lesions, and histology revealed an EST. He received chemotherapy, but 12 months after surgery, rapid tumor progression and intracranial metastases with obstructive hydrocephalus were detected. Following external ventricular drainage, the patient's condition rapidly deteriorated, and he ultimately died. EST should be considered when confronting a homogenously enhancing intradural tumor of the spine on post-contrast MRI.

Yolk Sac Tumor of the Omentum: A Case Report and Literature Review.

This is a case report of a rare finding of an extragonadal yolk sac tumor in a 37-year-old patient who presented with shortness of breath and abdominal bloating. During imaging and staging surgery, the findings were strongly suggestive of an extragonadal advanced tumor presenting with peritoneal dissemination, predominantly affecting omentum, with no clear primary origin. Histology revealed an extragonadal yolk sac tumor in a pure form outside the ovaries. Lacking an obvious origin elsewhere, the tumor was highly suspected to have truly originated from the omentum. The patient underwent surgery and four cycles of chemotherapy consisting of cisplatin, etoposide, and bleomycin. One-year outpatient follow-up thereafter showed no relapse. We herein discuss a possible site of the tumor origin and its development, as well as diagnostic challenges and disease prognosis.

A sinonasal yolk sac tumor in an adult.

Yolk sac tumors (YSTs), which are also called endodermal sinus tumors, are malignant tumors of germ cell origin. These tumors usually occur in the gonads, but 20% of cases have been reported at extragonadal sites. The head and neck is a rarely affected region that accounts for just 1% of all malignant tumors of germ cell origin. In addition, YSTs arise mostly in childhood. We present a rare pathologically pure case of primary adult YST in the sinonasal area. A 45-year-old male patient presented with a rapidly growing mass in the nasal cavity, which caused nasal obstruction and bloody post-nasal drip. The histopathologic features indicated pure YST, and immunohistochemical analysis revealed positive reactivity for Sal-like protein 4 and alpha-fetoprotein. Herein, we discuss the clinical, radiologic, and histologic features of this YST and review other cases of sinonasal YST in adults.

Tumor del saco vitelino primario de vagina en una niña. A propósito de un caso / Primary vaginal yolk sac tumor in a girl. Case report

Las neoplasias ginecológicas en niñas son raras. Representan menos del 5 % de todos los cánceres en pediatría. No existen estadísticas sobre la incidencia de tumores de vagina en esta etapa de la vida. Se presenta el caso de una niña de 9 meses con sangrado por genitales. La ecografía evidencia una masa sólida en vagina, y la vaginoscopia, un tumor friable. Presenta valores de α-fetoproteína elevados, por lo que se diagnostica tumor de saco vitelino, confirmado por biopsia. Se realiza tratamiento quimioterápico. A menos de 1 año del diagnóstico, se encuentra en remisión completa. Este caso resulta de interés no solo por la rareza, sino también porque el diagnóstico rápido de tumor de saco vitelino permite mejorar los resultados y la sobrevida de las pacientes

Gynecological neoplasms in girls are rare and represent only less than 5 % of all childhood tumors. There are no statistics on the incidence of vaginal tumors at this stage in life. We present a 9-month-old girl evaluated for genital bleeding. Ultrasound reveals a vaginal solid mass and vaginoscopy reports a friable tumor. AFP is elevated. A yolk sac tumor is confirmed by biopsy she receives chemotherapy. Within a year after diagnosis, she remains tumor-free. This is a case of interest, not only because of its rarity, but also because a rapid diagnosis of a yolk sac tumor improves outcomes and patient's survival rates.

Primary Yolk Sac Tumor in the Cerebellar Hemisphere: A Case Report and Literature Review of the Rare Tumor.

Yolk sac tumor (YST) is one of rare malignant germ cell tumors (GCTs). Primary intracranial YST, also endodermal sinus tumor (EST), is a quite rare type of brain tumor. Here, we report a case of YST, review the relevant literature, and propose a treatment strategy for this rare tumor. A 6-year-old boy initially manifested symptoms of dizziness and vomiting. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large irregular oval tumor in the cerebellar hemisphere. We subtotally removed the tumor by microsurgery through the left suboccipital approach. Immunohistochemical staining showed that alpha fetoprotein (AFP) was positive and the Ki-67 proliferation index was high (60%), suggesting a germ cell tumor. After 3 months of follow-up, neither recurrence of tumor nor complications were found in the patient. The diagnosis of YST should be confirmed on the basis of clinical manifestations, neuroimaging and pathological findings. Gross total resection (GTR) is an ideal treatment for YST. However, due to the location of the tumor, GTR is usually difficult, and the rate of postoperative complications is high. This reported case shows that subtotal resection can be a good treatment strategy for YST.

[Primary vaginal yolk sac tumor in a girl. Case report]. / Tumor del saco vitelino primario de vagina en una niña. A propósito de un caso.

Gynecological neoplasms in girls are rare and represent only less than 5 % of all childhood tumors. There are no statistics on the incidence of vaginal tumors at this stage in life. We present a 9-month-old girl evaluated for genital bleeding. Ultrasound reveals a vaginal solid mass and vaginoscopy reports a friable tumor. AFP is elevated. A yolk sac tumor is confirmed by biopsy she receives chemotherapy. Within a year after diagnosis, she remains tumor-free. This is a case of interest, not only because of its rarity, but also because a rapid diagnosis of a yolk sac tumor improves outcomes and patient's survival rates.

Las neoplasias ginecológicas en niñas son raras. Representan menos del 5 % de todos los cánceres en pediatría. No existen estadísticas sobre la incidencia de tumores de vagina en esta etapa de la vida. Se presenta el caso de una niña de 9 meses con sangrado por genitales. La ecografía evidencia una masa sólida en vagina, y la vaginoscopia, un tumor friable. Presenta valores de α-fetoproteína elevados, por lo que se diagnostica tumor de saco vitelino, confirmado por biopsia. Se realiza tratamiento quimioterápico. A menos de 1 año del diagnóstico, se encuentra en remisión completa. Este caso resulta de interés no solo por la rareza, sino también porque el diagnóstico rápido de tumor de saco vitelino permite mejorar los resultados y la sobrevida de las pacientes.

The diagnostic and prognostic value of tumor markers in giant mediastinal endodermal sinus tumor with prolonged survival: Twelve-year follow up after radical resection.

We report a case of 16 -year male who presented with nonproductive cough, chest pain, and hemoptysis. His chest -x-ray and computerized tomographic scan (CT) of the thorax with contrast enhancement revealed a large mediastinal mass mostly occupying the left hemithorax. Percutaneous CT scan-guided biopsy of the mediastinal mass was reported as an endodermal sinus tumor (EDST). Serum alpha-fetoprotein levels were markedly raised (120,000 ng/ml). After completion of chemotherapy repeat, CT scan of the thorax revealed a large residual mass. Radical resection of the tumor was carried out. Twelve-year post-surgical resection follow-up with serial serum alpha-fetoprotein (AFP) tumor marker levels and CT scan of the thorax showed no recurrence.

Pure yolk sac tumor of testis with lung metastasis in an adult patient - case report.

Pure yolk sac tumor is the most frequently seen testicular tumor in childhood; however it is usually a component of mixed germ cell tumor in adults. Pure yolk sac tumors are quite rare in adults. Pure yolk sac tumor in adults has been categorized as yolk sac tumor, post-pubertal type. We report a case of pure yolk sac tumor of right testis with lung metastasis in a 37-year-old man who was successfully treated by high inguinal orchidectomy and BEP combination chemotherapy.

Laparoscopic uncontained power morcellation-induced dissemination of ovarian endodermal sinus tumors: A case report.

BACKGROUND: Endodermal sinus tumors (ESTs), which arise primarily in children and adolescents, account for 20% of malignant ovarian germ cell tumors, but constitute only 1% of all ovarian malignancies. Treatment of ESTs consists of surgical staging with fertility-sparing surgery and chemotherapy. CASE SUMMARY: A 15-year-old nulliparous patient was diagnosed with disseminated ovarian ESTs after laparoscopic unilateral salpingo-oophorectomy using uncontained power morcellation for treatment of a ruptured solid adnexal mass in another hospital. Exploratory laparotomy; total abdominal hysterectomy, right salpingo-oophorectomy, and lymphadenectomy were performed with optimal debulking, and surgical stage 3C was assigned to the patient. CONCLUSION: In 2014, the Food and Drug Administration noted that power morcellation was probably associated with a risk of disseminating suspected cancerous tissue. Furthermore, the use of power morcellation to remove solid adnexal mass is considered a contraindication because of the potential for a malignant tumor. This case report aims to warn of the dangers of using uncontained power morcellation to treat solid adnexal masses.

Yolk Sac Tumour of the Ovary in Pregnancy Treated With Surgery and Chemotherapy: A Case Report.

BACKGROUND: Ovarian carcinoma diagnosed in pregnancy is rare. Treatment should take both mother and fetus into consideration. CASE: We present the case of a patient diagnosed with a stage IC1 yolk sac tumour of the ovary at 15 weeks gestation, who underwent surgical staging and adjuvant chemotherapy during pregnancy. Intrauterine growth restriction was diagnosed and the patient delivered by cesarean at 36 weeks gestation for obstructed labour. CONCLUSION: Yolk sac tumour of the ovary in pregnancy with concomitant chemotherapy is uncommon. Adverse outcomes, including restricted fetal growth, are possible and their identification may help guide timing of delivery.

Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature.

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.