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1.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 1280-1289, 2023.
Article in Chinese | WPRIM | ID: wpr-996966

ABSTRACT

@#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.

2.
Arch. argent. pediatr ; 119(6): e643-e647, dic. 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1353061

ABSTRACT

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.


Subject(s)
Humans , Female , Infant , Vaginal Neoplasms/diagnosis , Endodermal Sinus Tumor/diagnosis , Ultrasonography , Hemorrhage
3.
MedUNAB ; 23(2): 191-194, 22-07-2020.
Article in Spanish | LILACS | ID: biblio-1117955

ABSTRACT

Figuras A Y B: Radiografía de Tórax Anteroposterior (AP) y Lateral. Presencia de gran lesión al parecer del mediastino medio (Flechas blancas), con diámetros de 11 x 15 x 12 cm, de densidad homogénea, con efecto de masa y desviación de todas las estructuras (Flechas negras) y compresión sobre el lóbulo superior derecho (*), sin calcificaciones en su interior. Figura C. Tomografía computarizada de tórax con contraste corte coronal. En la región central del mediastino anterior, se identifica una masa hipodensa, heterogénea, con densidad de predominio de tejido blando, con algunas imágenes de densidad cálcica en su interior, de 14.3 x 10.2 x 12.6 (Línea amarilla) cm, que se encuentra desplazando la aorta ascendente, con trayecto tortuoso (Flecha azul), la flecha roja señala el tronco braquiocefálico. Figura D. Tomografía computarizada de tórax corte axial en ventana mediastinal. Masa hipodensa, heterogénea, con densidad de predominio de tejido blando, con algunas imágenes de densidad cálcica en su interior, la cual se encuentra desplazando y ocupando gran parte de la porción anterior del hemitórax derecho. Así mismo se encuentra desplazando estructuras mediastinales como el tronco de la pulmonar (Flecha azul), esta masa se encuentra generando atelectasia pasiva del lóbulo medio derecho (Flecha roja). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748


Figures A and B: Anteroposterior (AP) and Lateral Chest X-ray. Presence of a large mass, apparently from the middle mediastinum (white arrows), diameters of 11 x 15 x 12 cm, homogeneous density, with the mass effect on other mediastinal structures (black arrows) and compression on the right upper lobe (*), without inner calcifications. Figure C. Chest Computed Tomography, coronal section. In the anterior mediastinum it shows a hypodense, heterogeneous mass, with predominance of soft tissue density, with some calcifications inside, diameters of 14.3 x 10.2 x 12.6 (yellow line) cm, displacing the ascending aorta (blue arrow), the red arrow indicates the brachiocephalic trunk. Figure D. Computed tomography of the chest, axial section. Hypodense, heterogeneous mass, with predominance of soft tissue density, with some calcifications inside, which occupying a large parto f the anterior right hemithorax. It is also displacing mediastinal structures such as the trunk of the pulmonary artery (blue arrow), this mass is generating passive atelectasis of the right middle lobe (red arrow). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748


Figuras A e B: Radiografia de tórax (anteroposterior (AP) e lateral). Presença de grande massa, aparentemente do mediastino médio (Setas brancas), diâmetros de 11 x 15 x 12 cm, densidade homogênea, com efeito de massa em outras estruturas mediastinais (setas pretas) e compressão no lobo superior direito (*), sem calcificações internas. Figura C. Tomografia computadorizada de tórax, corte coronal. No mediastino anterior, mostra uma massa hipodensa e heterogênea, com predominância da densidade de partes moles, com algumas calcificações internas, diâmetros de 14,3 x 10,2 x 12,6 (Linha amarela) cm, deslocando a aorta ascendente (seta azul), a seta vermelha indica o tronco braquiocefálico. Figura D. Tomografia computadorizada de tórax, corte axial. Massa hipodensa, heterogênea, com predominância da densidade de partes moles, com algunas calcificações internas, que ocupam grande parte do hemitórax anterior direito. Também está deslocando estruturas mediastinais, como o tronco da artéria pulmonar (Seta azul), essa massa está gerando atelectasia passiva do lobo médio direito (Sseta vermelha). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748


Subject(s)
Mediastinal Neoplasms , Radiology , Superior Vena Cava Syndrome , Endodermal Sinus Tumor , Hemoptysis
4.
Chinese Journal of Medical Imaging Technology ; (12): 1504-1508, 2019.
Article in Chinese | WPRIM | ID: wpr-861204

ABSTRACT

Objective: To explore CT and PET/CT features of primary mediastinal yolk sac tumor (PMYST). Methods: CT, PET/CT and clinical data of 14 patients with PMYST confirmed by percutaneous biopsy were retrospectively analyzed. All patients underwent plain scan and enhanced chest CT examination, while 7 patients underwent PET/CT examination. Results: CT showed round or lobulated masses in all 14 cases. The maximum diameter of masses was 4.10-13.50 cm, with an average of (10.65±2.27) cm. Lesions with the maximum diameter≥10.00 cm were in 13 cases (13/14, 92.86%).Plain CT showed non-uniform density or heterogeneous density, and the enhanced CT showed multiple strip, thin linear partition enhanced focus and flake non-enhanced area in 14 cases, among which moderate and mild enhancement were observed in 11 cases (11/14, 78.57%) and 3 cases (3/14, 21.43%), respectively. Cardiac envelope involvement was found in 5 cases (5/14, 35.71%), left cephalobrachial vein and/or superior vena cava involvement were detected in 3 cases (3/14, 21.43%), while the right upper and middle lobe involvement was noticed in 1 case (1/14, 7.14%).There were 3 patients (3/14, 21.43%) with right hilar and mediastinal lymph node metastasis, 1 (1/14, 7.14%) with right pleural metastasis and 1 (1/14, 7.14%) with right lower lung metastasis. Pericardial effusion (7/14, 50.00%) and pleural effusion (7/14, 50.00%) were found each in 7 cases, respectively. PET/CT showed markedly increased radioactivity uptake in all 7 cases. During following-up after treatment, stable or recovered well after operation were observed in 9 cases (9/14, 64.29%), whereas progressive or recurred after operation in 5 cases (5/14, 35.71%). Conclusion: The main imaging manifestations of PMYST include large round or lobulated non-uniform mass in the anterior mediastinum, often invading surrounding vessels, tissue and organs, mostly with lymph nodes and distant metastasis. The specific manifestations of PMYST are multiple strip-like, linear septate-like enhancement foci and patchy non-enhancement areas in enhanced CT. 18F-FDG uptake of the lesions are markedly hypermetabolic.

5.
National Journal of Andrology ; (12): 144-149, 2019.
Article in Chinese | WPRIM | ID: wpr-816845

ABSTRACT

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.

6.
Rev. chil. obstet. ginecol. (En línea) ; 83(2): 210-217, abr. 2018. graf, ilus
Article in Spanish | LILACS | ID: biblio-959506

ABSTRACT

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.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Ovarian Neoplasms/diagnosis , Abdominal Pain/etiology , Abdominal Pain/therapy , Endodermal Sinus Tumor/diagnosis , Endodermal Sinus Tumor/drug therapy , Postpartum Period , Pregnancy Complications, Neoplastic/therapy , Cytoreduction Surgical Procedures
7.
Einstein (Säo Paulo) ; 15(4): 496-499, Oct.-Dec. 2017. graf
Article in English | LILACS | ID: biblio-891429

ABSTRACT

ABSTRACT Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum (50 to 70% of all extragonadal germ cell tumors). We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.


RESUMO Os tumores de células germinativas são neoplasias raras que acometem mais frequentemente as gônadas, embora possam também ocorrer em outras localizações do corpo, destacando-se o mediastino anterior (50 a 70% de todos os tumores de células germinativas extragonadais). No presente artigo, relatamos um caso de tumor de saco vitelínico mediastinal primário, de subtipo raro e agressivo de tumor de células germinativas. Tratava-se de um homem, 38 anos, admitido no Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", com quadro de dispneia e tosse seca há 1 ano. Na investigação clínica, foi solicitada tomografia computadorizada de tórax, que mostrou volumosa massa no mediastino anterior com realce heterogêneo ao meio de contraste associada a derrame pleural. Havia ainda aumento dos níveis séricos da alfafetoproteína. Após quimioterapia neoadjuvante pré-operatória, o paciente foi submetido à ressecção cirúrgica, seguida de estudo anatomopatológico da peça, no qual demonstrou tratar-se de um tumor de saco vitelínico primário do mediastino. Os tumores de saco vitelínicos primários do mediastino têm prognóstico reservado, apesar do avanço na terapêutica com a ressecção cirúrgica e a quimioterapia à base de cisplatina. Isto se deve ao grau de invasão e de irressecabilidade na maioria dos pacientes no momento do diagnóstico.


Subject(s)
Humans , Male , Adult , Testicular Neoplasms/therapy , Endodermal Sinus Tumor/therapy , Neoplasms, Germ Cell and Embryonal/therapy , Neoadjuvant Therapy , Mediastinal Neoplasms/therapy , Testicular Neoplasms/pathology , Testicular Neoplasms/diagnostic imaging , Thoracotomy , alpha-Fetoproteins/analysis , Tomography, X-Ray Computed , Endodermal Sinus Tumor/pathology , Endodermal Sinus Tumor/diagnostic imaging , Neoplasms, Germ Cell and Embryonal/diagnosis , Neoplasms, Germ Cell and Embryonal/pathology , Mediastinal Neoplasms/pathology , Mediastinal Neoplasms/diagnostic imaging , Mediastinum/diagnostic imaging
8.
Chinese Journal of Pediatrics ; (12): 298-303, 2017.
Article in Chinese | WPRIM | ID: wpr-808427

ABSTRACT

Objective@#To understand the clinical manifestation, imaging characteristics and outcomes of pulmonary malignant tumors in children.@*Method@#We retrospectively collected information about seven cases of pulmonary malignant tumors in children in our hospital from Jan 2010 to Dec 2016. The information included clinical manifestation, imaging characteristics, pathologic results, and treatment.@*Result@#(1) All the seven patients firstly visited pediatric internal medicine departments. Symptoms included cough (n=6), dyspnea (n=4), fever (n=2), anorexia (n=2), chest tightness (n=1), chest pain (n=1), lameness (n=1), abdominal distension and constipation (n=1). We did not find hemoptysis, wheezing or weight loss in those patients. Physical examinations revealed unilateral reduced breath sounds (n=5), moist rales and wheezes (n=1), and normal (n=1). Extrapulmonary signs included abdominal distension (n=2), left hip tenderness (n=1), and mass in left scrotum. We did not identify clubbing finger, anemic appearance, lymph node enlargement, or hepatosplenomegaly etc. (2) Laboratory examination results: complete blood count showed white blood cells in normal range except one case (17.44×109/L). Neutrophil percentage ranged from 0.348 to 0.767. C reactive protein ranged between<1 and 162 mg/L. Hemoglobin was normal. Three out of four cases had abnormal blood tumor markers.(3) Imaging results showed multiple nodes (n=3), multiple cystic lesions in lungs (n=2) (both with pleuropulmonary blastoma), endobronchial soft tissue mass (n=1), pulmonary round-shaped mass (n=1), and mediastinal mass (n=1). Imaging results also found atelectasis (n=3), pneumonia (n=2), pneumothorax (n=2), longitudinal diaphragmatic hernia (n=2), pleural effusion (n=1), subcutaneous emphysema (n=1). (4) All the patients underwent tumor puncture biopsy or tumor resection. Pathology revealed the final diagnosis of pleuropulmonary blastoma (n=3), endodermal sinus tumors (n=2), squamous cell carcinoma of lung (n=1), and thyroid papillary carcinoma (n=1). All of them were malignant tumors. We followed up them. Two patients died (both with pleuropulmonary blastoma) after their parents refused any medical help. Two cases were lost to follow-up. Three patients survived (followed up for 19 months, 11 months and tow months, respectively). One case with thyroid papillary carcinoma pulmonary metastasis underwent right thyroid cancer radical plus left thyroid lobe resection plus modified selective central lymph node dissection, then iodine 131 treatment. One case with endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies, resection of left retroperitoneal tumor and left testicular tumor, and six additional chemotherapies. Another one case of endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies and was discharged.@*Conclusion@#Pulmonary malignant tumors are rare diseases in children. Clinical signs are often non-specific. For those with chest CT showing multiple cystic lesions, endobronchial soft tissue mass or multiple nodes, but no significant infection manifestation or no response to anti-infection therapy, pulmonary malignant tumors should be considered. Biopsy may be needed to confirm the final diagnosis.

9.
Journal of Practical Radiology ; (12): 1175-1177,1185, 2015.
Article in Chinese | WPRIM | ID: wpr-600556

ABSTRACT

Objective To investigate the charateristic CT features of pediatircs reproductive system of endodermal sinus tumor. Methods Thirteen cases with pediatircs reproductive system of endodermal sinus tumor confirmed by surgery and biopsy pathology all underwent CT and contrast-enhanced CT scans,and theire imaging features were then ananlyzed.Results On CT scan,7 cases of testicular or vagina endodermal sinus tumor of children appeared soft tissues mass with uneven density,small patchy hypodense shadows,and ill-defined boundaries.Contrast-enhanced CT scans reveal the solid masses were obvious uneven continuous enhance-ment,but small patchy hypodense shadows were not enhanced,local envelope were enhanced.CT scans reveal that 6 cases of ovaries endodermal sinus tumor showed the large cystic solid mass,predominantly cystic mass,including 2 cases of punctate and patchy cal-cification,and contrast-enhanced CT scans showed that solid part were enhanced obviously and with obvious strengthening vessels shadows.1 case of right ovaries endodermal sinus tumor with fat density shadow and small patchy calcification.Its pathological di-agnosis was the endodermal sinus tumors with mature cystic treatomas.Conclusion CT image has an advantage in the diagnosis of endodermal sinus tumor of the children's reproductive system,especially combined with serum AFP determination.

10.
Clinical Pediatric Hematology-Oncology ; : 30-37, 2015.
Article in Korean | WPRIM | ID: wpr-788540

ABSTRACT

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.


Subject(s)
Adolescent , Female , Humans , Infant , Infant, Newborn , Pregnancy , Age Distribution , Choriocarcinoma , Cisplatin , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Germinoma , Gonads , Hematopoietic Stem Cell Transplantation , Neoplasms, Germ Cell and Embryonal , Ovum , Pediatrics , Prognosis , Puberty , Spermatozoa , Teratoma , Biomarkers, Tumor
11.
Clinical Pediatric Hematology-Oncology ; : 30-37, 2015.
Article in Korean | WPRIM | ID: wpr-13546

ABSTRACT

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.


Subject(s)
Adolescent , Female , Humans , Infant , Infant, Newborn , Pregnancy , Age Distribution , Choriocarcinoma , Cisplatin , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Germinoma , Gonads , Hematopoietic Stem Cell Transplantation , Neoplasms, Germ Cell and Embryonal , Ovum , Pediatrics , Prognosis , Puberty , Spermatozoa , Teratoma , Biomarkers, Tumor
12.
Clinical Pediatric Hematology-Oncology ; : 41-45, 2014.
Article in English | WPRIM | ID: wpr-788502

ABSTRACT

Endodermal sinus tumor is a type of germ cell tumor that is relatively common in children. An important problem concerns secondary neoplasms after treatment. We report a case of osteosarcoma that developed five years after treatment of intrapelvic endodermal sinus tumor. The patient was a seven-year-old girl who presented with right thigh and knee joint pain. The patient had been diagnosed with endodermal sinus tumor five years previously and treated with a regimen consisting of high dose cisplatin, etoposide, bleomycin and surgery but without radiotherapy. We detected a mass shadow on the right distal femur that proved to be osteoblastic osteosarcoma by incisional biopsy. The patient received surgical treatment after chemotherapy that included high dose methotrexate. The follow-up bone scan revealed no abnormal uptakes. There has been no evidence of recurrence eighteen months of follow-up after chemotherapy.


Subject(s)
Child , Female , Humans , Biopsy , Bleomycin , Cisplatin , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Femur , Follow-Up Studies , Knee Joint , Methotrexate , Neoplasms, Germ Cell and Embryonal , Neoplasms, Second Primary , Osteoblasts , Osteosarcoma , Radiotherapy , Recurrence , Thigh
13.
Clinical Pediatric Hematology-Oncology ; : 41-45, 2014.
Article in English | WPRIM | ID: wpr-111168

ABSTRACT

Endodermal sinus tumor is a type of germ cell tumor that is relatively common in children. An important problem concerns secondary neoplasms after treatment. We report a case of osteosarcoma that developed five years after treatment of intrapelvic endodermal sinus tumor. The patient was a seven-year-old girl who presented with right thigh and knee joint pain. The patient had been diagnosed with endodermal sinus tumor five years previously and treated with a regimen consisting of high dose cisplatin, etoposide, bleomycin and surgery but without radiotherapy. We detected a mass shadow on the right distal femur that proved to be osteoblastic osteosarcoma by incisional biopsy. The patient received surgical treatment after chemotherapy that included high dose methotrexate. The follow-up bone scan revealed no abnormal uptakes. There has been no evidence of recurrence eighteen months of follow-up after chemotherapy.


Subject(s)
Child , Female , Humans , Biopsy , Bleomycin , Cisplatin , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Femur , Follow-Up Studies , Knee Joint , Methotrexate , Neoplasms, Germ Cell and Embryonal , Neoplasms, Second Primary , Osteoblasts , Osteosarcoma , Radiotherapy , Recurrence , Thigh
14.
Journal of the Korean Association of Pediatric Surgeons ; : 25-31, 2013.
Article in English | WPRIM | ID: wpr-88304
15.
Arch. méd. Camaguey ; 16(2): 199-204, mar.-abr. 2012.
Article in Spanish | LILACS | ID: lil-628126

ABSTRACT

Fundamento: los tumores de células germinales del ovario son poco comunes y se observan con mayor frecuencia en adolescentes. Dentro de la gama de tumores de las células germinales, el tumor de seno endodérmico ocupa el 2 %. Objetivo: describir un nuevo caso de tumor del seno endodérmico dentro de los existentes en la literatura. Caso clínico: paciente femenina de 17 años de edad, adolescente que debutó con trastornos menstruales, en el estudio ecográfico abdominal se halló la presencia de una tumoración ovárica. Lo inusual fue que se observó un rápido crecimiento del tumor en corto tiempo. La paciente fue operada y seguida por los oncólogos, recibió tratamiento con poliquimioterapia y no presentó recidivas. Conclusiones: el tumor de seno endodérmico en un número de casos se presentan de forma silenciosa, detectados ocasionalmente por ecografía, aunque en la mayoría, una forma de presentación frecuente es dolor abdominal, que puede ser intermitente o agudo, asociado a una masa abdominal o pélvica palpable, datos que no se recogieron en la paciente. En un 10 % (formas agudas) ocurre torsión, ruptura y/o hemorragia, por lo que es importante tener presente esta enfermedad cuando se presenta en niñas prepuberales y adolescentes.


Background: germ cell tumors of ovary are uncommon and occur most frequently in adolescents. Within the range of germ cell tumors, endodermal sinus tumor occupies the 2 %. Objective: to describe a new case of endodermal sinus tumor among the existing literature. Clinical case: a 17-year-old female patient was presented with menstrual disorders; in the abdominal ultrasound was found the presence of an ovarian tumor. The unusual of this case was the quick growth of the tumor in a short time. The patient was operated and followed by oncologists, polychemotherapy treatment was performed and no recidivation was presented. Conclusion: endodermal sinus tumor in a number of cases occur silently, occasionally detected by ultrasound examination, although most of them, a frequent presentation is abdominal pain which may be intermittent or acute pain, associated with a palpable abdominal or pelvic mass, data that were not collected in this patient. In a 10 % of the acute forms occurs torsion, rupture with or without bleeding, that is why it is important to take into account this disease because it may appears in prepuberal girls and adolescents.

16.
Chinese Journal of Medical Ultrasound (Electronic Edition) ; (12): 535-538, 2012.
Article in Chinese | WPRIM | ID: wpr-635771

ABSTRACT

Objective To investigate ultrasonographic features of primary and metastatic ovarian yolk sac tumors.Methods Ultrasonographic features of 19 primary lesions and 33 metastatic lesions in 35 patients were retrospectively analyzed.Results Primary tumors were sized (14.6±3.6)cm in maximum diameter,manifesting as cysti-solid masses.Solid components of primary tumors were mainly hypoechoic or isoechoic(16/19)with rich blood supplies.Thirty-three metastatic lesions were located in pelvoceliac cavity(26/33) and liver parenchyma(7/33),sized (9.4±4.5)cm,(9.2±4.9)cm and (5.6±1.6)cm in maximum diameter respectively.Metastatic lesions in pelvoceliac lesions mainly demonstrated as hypoechoic masses(21/26), however lesions in the liver were mainly hyperechoic(5/7).Anechoic regions could be found in 9/26 of the pelvoceliac lesions.Blood supply was found less rich in metastatic masses than that in primary ones.Elevated serum level of alpha fetoprotein (AFP) was observed in all patients; ranging from 217 to 211 682 μg/L.Conclusions Primary and metastatic lesions of ovarian yolk sac tumor have obvious ultrasonographic characteristics.Combined with serum AFP level,the accuracy of diagnosis could be improved.

18.
Indian J Pathol Microbiol ; 2009 Jul-Sept; 52(3): 403-404
Article in English | IMSEAR | ID: sea-141495

ABSTRACT

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.

19.
Chinese Journal of Urology ; (12): 498-500, 2009.
Article in Chinese | WPRIM | ID: wpr-393859

ABSTRACT

Objective To investigate the diagnosis and treatment outcomes of testieulax endodermal sinus tumor. Methods Twenty-four cases diagnosed with testieular endodermal sinus tumors from November 1996 to April 2007 were retruspeetively reviewed. Eighteen patients presented with stage Ⅰ disease, 4 presented with stage Ⅰ , and 2 presented with stage Ⅲ. Inguinal radical or-chieetomy were performed in all patients. Retroperitoneal lymph node dissection was performed in 8 ea-ses. Results The histological structures were rather complicated in 24 tumors. Twenty-three eases (96%) were found with reticular pattern, 22(92%) with hyaline body, 20(83%) with glandlike structure, 16(67%) with Shiller-Dural body, and 13(54%) with solid formation. The former 3 kinds were regarded as the main diagnostic criteria. Twenty-one eases were followed up for 20 months to 12 years. During the follow-up, 2 patients died of the disease. Condesions Early diagnosis and combi-nation therapy, including radical orehieetomy and chemotherapy, are the keys to improve the curative effect of testieular endodermal sinus tumors. Active surveillance of AFP is critical for monitoring the recurrence and metastasis of this tumor.

20.
Korean Journal of Pathology ; : 570-574, 2009.
Article in English | WPRIM | ID: wpr-28152

ABSTRACT

Ovarian endometrioid adenocarcinoma (EAC) with a yolk sac tumor (YST) component is extremely rare. Only twelve cases have been reported in the English literature. We report here two additional cases of this rare tumor. The YST component showed classic microscopic features, and immunohistochemically stained positive for alpha-fetoprotein (AFP), but negative for cytokeratin 7 (CK7), epithelial membrane antigen (EMA), estrogen receptor (ER) and progesterone receptor (PR). The EAC appeared to blend into the YST in several areas and immunohistochemically stained positive for CK7, EMA, ER, and PR, but negative for AFP.


Subject(s)
Female , alpha-Fetoproteins , Aminocaproates , Carcinoma, Endometrioid , Endodermal Sinus Tumor , Estrogens , Keratin-7 , Mucin-1 , Ovary , Receptors, Progesterone , Yolk Sac
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