Tumor del seno endodérmico ovárico bilateral en adolescente / Bilateral ovarian yolk sac tumor in adolescent i: en

Se presenta el caso clínico de una adolescente de 15 años de edad, quien fue asistida en el Hospital Provincial Pediátrico Universitario José Luis Miranda de Villa Clara, remitida desde su área de salud, por presentar dolor pélvico intenso desde hacía 3 días, náuseas y fiebre de 38,5 °C. Luego de realizados el examen clínico y los estudios complementarios pertinentes, se decidió practicar la resección completa del tumor. Durante el procedimiento se tomó muestra para estudio histológico que confirmó la existencia de un tumor del seno endodérmico ovárico, por lo cual fue reintervenida para extirpar el ovario contralateral y el epiplón infiltrados. Posteriormente se indicó poliquimioterapia según el protocolo y la evolución postratamiento fue satisfactoria.

The case report of a 15-years-old adolescent is presented, who was assisted at José Luis Miranda University Pediatric Provincial Hospital from Villa Clara, referred from her health area due to an intense pelvic pain for 3 days, nausea and fever of 38.5 °C. After carrying out the clinical exam and the pertinent laboratory tests, it was decided to practice the complete tumor resection. During the procedure a sample for histologic study was taken that confirmed the existence of an ovarian yolk sac tumor, reason why she was operated again to extirpate the contralateral ovary and the infiltrated omentum. Later on polychemotherapy was indicated according to the protocol and the post-treatment clinical course was satisfactory.

Diagnosis, treatment and prognosis of primary mediastinal yolk sac tumor in 18 patients / 中国胸心血管外科临床杂志

@#Objective    To investigate the clinical characteristics and treatment of primary mediastinal yolk sac tumor (PMYST). Methods    We collected the clinical data of PMYST patients who were admitted to the Department of Thoracic Surgery of Peking Union Medical College Hospital from September 2000 to September 2020. The clinical and pathological characteristics, treatment and prognosis of PMYST patients were retrospectively analyzed. Results     Finally 18 patients were enrolled, including 17 males and 1 female with a median age of 22.0 (16.6, 26.2) years. Patients had increased level of alpha fetoprotein (AFP). The pathological type can be single yolk sac tumor or combined with other mediastinal germ cell tumors. Chemotherapy and radical surgery were the main treatment methods. Extensive resection was feasible for patients with tumor invasion of other organs. Seven patients developed lung or pleural metastasis after operation, and 3 of them had extrapleural metastasis. One patient recurred within 1 year after surgery. All patients were followed up by telephone or outpatient department. At the last follow-up, 5 patients survived, 9 died, and 4 were lost to follow up, with a median survival of 16.8 months. The median disease-free survival was 14.9 months. The survival rates at 1, 3 and 5 years were 73.3%, 28.6% and 11.8%, respectively. Conclusion    PMYST often occurs in young-middle aged male patients. Preoperative puncture can provide an effective diagnostic basis. R0 resection, AFP returning to normal after treatment and no metastasis may be potential indicators of good prognosis. The overall prognosis of PMYST is poor, and some patients can achieve long-term survival after treatment.

Value of high-frequency ultrasound combined with alpha-fetoprotein in accurate qualitative diagnosis of pediatric testicular tumors / 中国医师杂志

Objective:To explore the value of high frequency ultrasound combined with serum alpha-fetoprotein (AFP) in the accurate qualitative diagnosis of pediatric testicular tumors.Methods:The ultrasound characteristics (physical properties, calcification, alder blood flow classification) and AFP levels of 47 testicular tumors confirmed by operation and pathology were analyzed retrospectively. The tumors were further divided into two ways: malignant tumor group and benign tumor group, yolk sac tumor group and non yolk sac tumor group. The characteristics of ultrasound and the accurate qualitative diagnosis efficiency of AFP in testicular tumors were analyzed by receiver operating characteristic curve (ROC).Results:18 cases of yolk sac tumor showed solid or almost solid mass, which may be accompanied by several small anechoic areas without calcification. The Alder blood flow grade were grade 3. 29 cases of nonyolk sac tumor showed cystic, solid or mixed mass, most of them have calcification and some of them showed honeycomb echo. Alder blood flow grade were 0-3 grade. ROC curve analysis showed that the area under the curve, sensitivity and specificity of the ultrasound characteristics and AFP in the diagnosis of pediatric testicular malignancies were: solid or almost solid mass (0.894, 83.3%, 95.5%), and no calcification (0.904, 94.4%, 86.4%), Alder blood flow level 3 (0.941, 88.9%, 95.5%), AFP by best cut-off value 18.8 ng/ml (0.972, 100%, 95.5%), ultrasound features combined with AFP (0.992, 100%, 90.9%). All the testicular malignancies, such as yolk sac tumor, immature teratoma, teratoma combined with yolk sac tumor, can be identified by ultrasound features combined with AFP. Further analysis showed that the sensitivity and specificity of the diagnosis of yolk sac tumor with combined solid or almost solid and no calcification were both 100.0%, which can accurately distinguish all cases of yolk sac tumor.Conclusions:Pediatric testicular yolk sac tumor has specific ultrasound performance, high-frequency ultrasound can make a relatively accurate diagnosis, combined with serum AFP can further make a relatively accurate qualitative diagnosis of other malignant tumors of the testis in children.

Metástasis retroperitonal gigante de un tumor del saco de Yolk / Giant retroperitonal metastasis from a Yolk sac tumor

RESUMEN Paciente con antecedentes de cirugía de tumor testicular, con biopsia de tumor de saco de Yolk y con diagnóstico de un tumor metastásico retroperitoneal irresecable. Un año más tarde de este último diagnóstico es remitido a nosotros, con un aumento considerable de dicha lesión y se logra su exéresis satisfactoriamente. Se realizó una revisión de la literatura, las indicaciones y técnicas en el tratamiento de la metástasis retroperitoneal de un tumor del saco de Yolk y se presentan los resultados de un paciente diagnosticado e intervenido en el Hospital Clínico Quirúrgico "Hermanos Ameijeiras", en el año 2016. La diseminación metastásica retroperitoneal del tumor del saco de Yolk es poco frecuente, muy poco reportado a nivel mundial y con pocas experiencias en su tratamiento. Se realizó una técnica de resección de una lesión gigante con quimioterapia adyuvante posoperatoria. No hubo complicaciones relacionadas con el proceder(AU)

ABSTRACT Patient with a history of testicular tumor surgery, with a Yolk sac tumor biopsy and with a diagnosis of an unresectable retroperitoneal metastatic tumor. One year after this last diagnosis, he was referred to us, with a considerable increase in this lesion and his excision was successfully achieved. A review of the literature, indications and techniques for the treatment of retroperitoneal metastasis from a Yolk sac tumor was performed, and the results of a patient diagnosed and operated on at the "Hermanos Ameijeiras" Clinical Surgical Hospital, in the year 2016. Retroperitoneal metastatic spread of Yolk sac tumor is infrequent, very little reported worldwide and with few experiences in its treatment. A giant lesion resection technique was performed with postoperative adjuvant chemotherapy. There were no complications related to the procedure(AU)

Conservative Surgery for a young nulligravid with ovarian yolk sac tumor and concurrent contralateral mature teratoma

@#<p>Ovarian cancer is the second most common gynecologic cancer worldwide and the six most common cancer among females. Germ cell tumorbs are the most common ovarian neoplasm in the first two decades of life constituting approximately two-thirds of all ovarian tumors. Malignant germ cell tumors constitute one-third of germ cell origin tutors and two-thirds of all ovarian malignancy in this age-group. This paper presents a case of a 19 year-old nulligravid who presented at the emergency room with abdominal pain, and was intraoperatively diagnosed with yolk sac tutor of the right ovary, stage 1A mature cystic teratoma of the left ovary. She subsequently underwent unilateral salpingooophorectomy and contralateral oophorocystectomy, left. Patient is advised chemotherapy postoperatively, with Bleomycin, Etoposide and Paclitaxel. This paper discusses the incidence, risk factors, prognosis and management of yolk of sac tutor in a young nulligravid.</p>

Germ cell tumors in dysgenetic gonads

This review describes the germ cell neoplasms that are malignant and most commonly associated with several types of gonadal dysgenesis. The most common neoplasm is gonadoblastoma, while others including dysgerminomas, yolk-sac tumors and teratomas are rare but can occur. The purpose of this review is to evaluate the incidences of these abnormalities and the circumstances surrounding these specific tumors. According to well-established methods, a PubMed systematic review was performed, to obtain relevant studies published in English and select those with the highest-quality data. Initially, the first search was performed using gonadal dysgenesis as the search term, resulting in 12,887 PubMed papers, published, from 1945 to 2017. A second search using ovarian germ cell tumors as the search term resulted in 10,473 papers, published from 1960 to 2017. Another search was performed in Medline, using germ cell neoplasia as the search term, and this search resulted in 7,560 papers that were published between 2003 to 2016, with 245 new papers assessing gonadoblastomas. The higher incidence of germ cell tumors in gonadal dysgenesis is associated with a chromosomal anomaly that leads to the absence of germ cells in these gonads and, consequently, a higher incidence of neoplasms when these tumors are located inside the abdomen. Several hypotheses suggest that increased incidence of germ cell tumors involves all or part of the Y chromosome or different genes.

Clinical characterization of testicular yolk sac tumor in children and adults / 中华男科学杂志

Objective@#To compare the clinical characteristics of simple testicular yolk sac tumor (YST) in children with those in adults so as to improve the diagnosis and treatment of the malignance.@*METHODS@#This study included 75 cases of simple testicular YST pathologically confirmed between May 2008 and July 2018, which were divided into groups A (aged <18 years, n = 64) and B (aged ≥18 years, n = 11). We analyzed the clinical data on all the cases and compared the clinical manifestations, laboratory results, pathological findings, clinical stages, treatment methods and prognostic outcomes between the two groups of patients.@*RESULTS@#The patients of group A ranged in age from 6 months to 5 years (［1.38 ± 0.89］ yr), with the tumor diameter of 0.9－6.0 (2.48 ± 1.12) cm, while those of group B from 25 to 49 years (median 34 years), with the tumor diameter of 3.5－6.3 (5.16 ± 1.32) cm, most presenting with a painless scrotal mass, 4 (6.2%) in group A and 5 (45.5%) in group B with testis pain. There were statistically significant differences between the two groups in the tumor diameter and initial manifestations (P < 0.05). All the patients were treated by radical high-level spermatectomy and orchiectomy and, in addition, 1 in group A and 3 in group B by retroperitoneal lymph node dissection (RPLND), 24 in the former and 5 in the latter group followed by chemotherapy. Elevated levels of serum alpha-fetoprotein (AFP) were observed in all the cases. Sixty-five of the patients were followed up for 10－78 (52.00 ± 23.78) months, during which 2 cases of simple metastasis, 3 cases of simple relapse, 3 cases of relapse with metastasis and 5 cases of death were found in group A, and 5 cases of simple metastasis, 1 case of simple relapse, 1 case of relapse with metastasis and 4 cases of death in group B.@*CONCLUSIONS@#There are significant differences in the clinical manifestation, biological behavior, treatment and prognosis of testicular YST between children and adults. In children, most of the testicular YST cases are at clinical stage I and preferably treated by radical high-level spermatectomy and orchiectomy with favorable prognosis. In adults, however, the tumor is highly malignant, with high incidences of recurrence and metastasis and poor prognosis, for the treatment of which the first choice is radical high-level spermatectomy and orchiectomy combined with RPLND and chemotherapy.

Primary Pelvic Peritoneal Yolk Sac Tumor in the Post-Pubertal Female: a Case Report with Literature Review

Yolk sac tumors are rare malignant germ cell neoplasms that usually arise from the gonads. Extragonadal yolk sac tumors (EGYSTs) frequently occur in the mediastinum in post-pubertal females. EGYSTs in the pelvis are extremely rare, and to date, only thirteen cases have been reported in the English literature. Among them, the primary EGYST of the pelvic peritoneum in post-pubertal females has only been reported in ten cases. The present case describes a 26-year-old female diagnosed with primary peritoneal yolk sac tumor located in the rectouterine pouch. We report clinical and tumor imaging features, including ultrasound, computed tomography (CT), magnetic resonance images (MRI), positron emission tomography-computed tomography (PET-CT), and present a review of the literature.

Tumor ovárico del seno endodérmico (Yolk Sac). Dolor abdominal en el puerperio inmediato como síntoma diagnóstico / Endodermal sinus ovarian tumor (Yolk Sac). Abdominal pain in the immediate puerperium as a diagnostic symptom

RESUMEN Los tumores del seno endodérmico ovárico (Yolk Sac), son neoplasias malignas de origen germinal, que se caracterizan por su diferenciación embriológica a partir de estructuras del saco vitelino. Son tumoraciones muy infrecuentes, de crecimiento rápido y que suelen desarrollarse en adolescentes y mujeres jóvenes, en edad reproductiva. Su diagnóstico se basa en la combinación de pruebas de imagen asociado a niveles plasmáticos elevados de marcadores tumorales como la alfafetoproteína. El manejo terapéutico es eminentemente quirúrgico (pudiendo ser conservador en pacientes con deseo genésico no cumplido), asociado a pautas de quimioterapia sistémica combinada con bleomicina, etopósido y platino. Exponemos el caso de una paciente que en el puerperio tardío, presenta un cuadro clínico de dolor, distensión abdominal y fiebre, siendo diagnosticada tras el tratamiento quirúrgico y el estudio histológico posterior, de un tumor del seno endodérmico ovárico.

ABSTRACT Ovarian endodermal sinus tumors (Yolk Sac), are malignant neoplasms of germinal origin, which are characterized by their embryological differentiation from yolk sac structures. These tumors are very infrequent, of rapid growth and tend to develop in adolescents and young women of reproductive age. Its diagnosis is based on the combination of imaging tests associated with high plasma levels of tumor markers such as alpha-fetoprotein. The therapeutic management is eminently surgical (with a more conservative approach reserved for patients still considering later pregnancy), associated with patterns of systemic chemotherapy combined with bleomycin, etoposide and platinum. We present the case of a patient who, in the late puerperium, presents symptoms of pain, abdominal distension and fever, being diagnosed after the surgical treatment and the subsequent histological study of a tumor of the endodermal ovarian sinus.

Yolk Sac Tumor of the Temporal Lobe: Case Report

Germ cell tumors of the central nervous system (CNS) are usually located along the midline. Yolk sac tumor is a rare germ cell tumor very uncommonly located outside the midline, and, in such cases, it can be mistaken with other primary tumors. We report a case of a 32-year-old male patient who presented with a right temporal lobe tumor suggestive of a high grade glioma. He was submitted to a right temporal lobectomy with complete tumor removal. The histological exam revealed a germ cell tumor (later confirmed to be a yolk sac tumor). The search for a primary tumor outside of the CNS (including a positron emission tomography scan) was negative, making this a primary temporal lobe yolk sac tumor. The patient was submitted to chemotherapy and radiotherapy, but died 7 months after the surgery.

Ultrasonographic features and clinical pathological characteristics in yolk sac tumors of ovary / 中国医学影像技术

Objective To explore the ultrasonographic features of yolk sac tumors of ovary.Methods Totally 12 patients (13 lesions) of the yolk sac tumors of ovary confirmed by pathology were analyzed retrospectively.Two-dimensional ultra sonography,color Doppler and spectral Doppler features were observed and combined with clinical manifestations.Results Ultrasound showed 1 case with bilateral single lesion and 11 cases with unilateral single lesion,the median size of tumors was 11.82 cm × 11.19 cm.Thirteen lesions showed liquid-solid mixed echo,and the blood flows in solid region were ≥ grade Ⅱ according to Adlefs semi quantitative method,the resistance index of blood was 0.37 0.55;Six cases combined with abdominal cavity effusion.Pathology showed 2 cases combined with omentum or rectal pouch metastasis and 1 case was tubal,ovarian artery and vein invasion.Conclusion Ultrasound images in the yolk sac tumors of ovary have characteristics and diagnosis must closely combine with clinical manifestations,some cases still need to be confirmed by pathology and immunohistochemist r y.

Ovarian Mixed Germ Cell Tumor in Childhood: A Rare Case Report.

Germ cell tumor is a rare malignancy accounting for 3% of all pediatric tumor. The mixed germ cell tumor is still rare. They affect mainly young girls and women, Due to this, the conservation of reproductive potential is a great concern. We report a rare case of a 7-year-old girl who presented with peripheral pseudoprecocious puberty having mixed malignant ovarian germ cell tumor of both yolk sac tumor with dysgerminoma.

Persistent Müllerian duct syndrome of mixed anatomical variant (combined male and female type) with mixed germ cell tumor of left intra‑abdominal testis.

Persistent Müllerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism characterized by retention of Müllerian duct derivatives in a phenotypically and karyotypically male patient. Deficiency of anti‑Müllerian hormone (AMH) secretion or resistance to AMH action due to defective AMH‑II receptor is presumed to cause such syndrome in the majority of cases. About 158 PMDS cases have been reported so far, out of which 31 cases are associated with testicular neoplasms. Herein, we describe an interesting case of young male initially diagnosed and treated for inguinal hernia, but finally diagnosed as “PMDS of mixed anatomical variant (combined male and female type) with mixed germ cell tumor of left intra‑abdominal testis” comprising components of seminoma and yolk sac tumor and treated successfully.

Endometrioid like yolk sac tumor of the testis with small teratomatous foci: A case report and review of the literature.

I have reported a case of endometrioid like yolk sac tumor of the testis in a 20-yearold boy. Endometrioid like yolk sac tumor is a rare tumor. A few cases have been reported in ovary. In case of male, a case of pure glandular endometrioid like yolk sac tumor is reported in a 43 years male in right undescended testis and another case of abdominal metastasis showing endometrioid pattern from mixed testicular germ cell tumor comprising of teratoma and embryonal carcinoma. My patient was a 20-year-old male presented with painless enlargement of right testis. Grossly the tumor was glistening creamish white with a multicystic appearance. Histopathological examination showed the tumor to be composed of glandular elements resembling early secretory endometrium, foci of keratinized thin squamous epithelium and a single focus of benign cartilage. The glandular elements show immunohistochemical positivity for AFP, cytokeratin 7 (CK7) and epithelial membrane antigen (EMA).

CT diagnosis of pediatric testicular yolk sac tumor in five cases / 实用放射学杂志

Objective To analyze the clinical characteristics and CT manifestations of pediatric testicular yolk sac tumor,to im-prove the CT diagnostic level of the disease.Methods Five pediatirc patients with testicular yolk sac tumor was retrospectively en-rolled in this study.All cases underwent spiral CT scan and enhancement scan prior to operation.Furthermore,the clinical and ima-ging data of these 5 cases were analyzed.Results Image features were unilateral testicular mass with uneven density,including patchy necrosis area,no obvious calcification and fatty density on CT scan.Obvious uneven reinforcement was revealed in the sub-stantial part of the masses after enhancement scan,no obvious reinforcement was revealed in necrotic area.Homogeneous enlarge-ment of the spermatic cords was demonstrated in all 5 cases,and the density was uniform.Obvious reinforcement was revealed in all affected spermatic cords,and the degree of the reinforcement was more obvious than those normal sides.The preoperative diagnosis of 5 cases was stage I malignant germ cell tumor Yolk sac tumor.The diagnosis of all 5 cases was basically in the line with surgical and pathological results.Conclusion CT scan is not only valuable in determining the nature of the tumor,but also helps to tumor management.

Primary yolk sac tumor of the cerebellar vermis: A case report.

Extragonadal germ cell tumors are rare and comprise 2-5% of all germ cell tumors. Seminoma/dysgerminoma, teratoma, yolk sac tumor (YST) and mixed germ cell tumors are few of the extragonadal germ cell tumors arising within the brain. Of these, primary pure YSTs arising in the brain are extremely rare and arise in the pineal (62%) and suprasellar (31%) regions. Primary YST occurring in the cerebellum is exceedingly rare, associated with a very poor prognosis and has been reported in less than 10 cases in literature. We herein report a case of primary pure yolk sac tumor in the cerebellar vermis in a 2-year-old child.

Primary extragonadal yolk sac tumor:a clinicopathologic analysis of 40 cases / 临床与实验病理学杂志

Purpose To investigate the clinicopathological features, histogenesis, morphological characteristics, immunophenotypes and differential diagnosis of primary extragonadal yolk sac tumor ( eYST) . Methods Clinicopathological data, morphological charac-teristics and immunophenotypes results of 40 cases of eYST were retrospectively studied and the relevant literatures were reviewed. Re-sults All 40 patients, which included 24 male and 16 female, aged from 6 months to 42 years with a 12 years average age and 17 (42.5%) cases were over 12 years old. Mediastinum, sacrococcygeal, retroperitoneal, pineal gland and vagina were involved in 16 (40.0%), 12(30.0%), 5(12.5%), 4(10.0%) and 3(7.5%), respectively. All 40 cases included 32(80.0%) cases pure YSTs, while other 8(20.0%) cases contained other one or two types of germ cell tumor (GCT) components. Conclusions Primary eYST is rare, mediastinum and sacrococcygeal are the most common anatomic sites for eYST, and these mediastinal tumor patients are overwhelmingly confined to adult males, whose average age are significantly older than that in sacrococcygeal, retroperitoneal, pineal gland and vaginal tumor patients (P<0.05), while other sites eYST are restricted to prepubertal children. Adult eYST contain other types of GCT components in some cases, and children's counterparts are always pure YST. Extragonadal eYST manifestate pleomorphic histological features, which combine with immunohistochemical markers is definite value for diagnosis, differential diagnosis.

CT Findings of Intrarenal Yolk Sac Tumor with Tumor Thrombus Extending into the Inferior Vena Cava: A Case Report

Yolk sac tumor (YST) is a rare germ cell neoplasm of childhood that usually arises from the testis or ovary. The rare cases of YST in various extragonadal locations have been reported, but the primary intrarenal YST is even more uncommon. Here, we report a case of a primary intrarenal YST with tumor thrombus of the inferior vena cava and left renal vein in a 2-year-old boy, with an emphasis on the CT features. To our knowledge, this is the first reported case of an intrarenal YST with intravascular involvement.

Tumor del saco vitelino puro con síndrome de Turner mosaico X0/XY

El síndrome de Turner es una afección genética por falta de un cromosoma X. Algunos casos se presentan con mosaicos XY, aumentando el riesgo de tumores gonadales de comportamiento maligno. Es importante determinar la presencia del cromosoma Y para realizar la extirpación profiláctica de las gónadas disgenésicas. Presentamos el caso de una paciente con síndrome de Turner, mosaico 45,X0/46,XY, asociado a tumor del saco vitelino puro gigante. Esta asociación es un caso extremadamente raro de tumor maligno en una paciente con disgenesia gonadal mixta.

Turners syndrome is a genetic disorder caused by lack of an X chromosome. In some cases it is present with XY mosaicism, with increased risk of malignant gonadal tumors. It is important to determine the presence of the Y chromosome to perform prophylactic removal of the dysgenetic gonads. We present the case of a patient with Turners syndrome 45,X0/46,XY mosaicism associated to pure giant yolk sac tumor. This association of malignancy in a patient with mixed gonadal dysgenesia is extremely rare.

Primary omental yolk sac tumor

Extra-ovarian yolk sac tumor arising in the omentum is extremely rare. As yolk sac tumor originated from the omentum has been rarely reported, its clinical information is very limited. The authors encountered a case of yolk sac tumor originated from the omentum, and reported the case herein. A 32-year-old woman was presented with developed low abdominal distension for a month. Magnetic resonance imaging findings were suggestive of ovarian malignancy with ascites and peritoneal seeding nodules. Explorative laparotomy was performed and then the findings from frozen biopsy of omentum were suggestive of poorly differentiated tumor though whether it was primary or metastatic was uncertain. Thus, staging laparotomy were performed. Histopathology confirmed that the tumor was a yolk sac tumor of omentum origin. Then, 6 cycles of postoperative adjuvant chemotherapy at intervals of 3 weeks were performed using bleomycin, etoposide, and cisplatin regimen. Four-year outpatient follow-up thereafter showed no relapse.