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.

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.

Unusual anatomic location of a primary intracranial yolk sac tumor

A nongerminomatous germ cell tumor occurring in the brain parenchyma is extremely rare. A 2-year-old boy presented with symptoms of abnormal movements in the hand and mouth. MRI scanning revealed a lesion occupying the left temporoparietal region. Craniotomy was performed and the tumor was removed by en bloc resection. Histological examination revealed that the tumor was arranged in a reticular pattern, and Schiller-Duval bodies were evident at the center of the tumor. Immunohistochemical study showed that the tumor cells were positive for alpha-fetoprotein and vimentin, but negative for glial fibril-lary acidic protein. The histological diagnosis was pure yolk sac tumor

A case of ovarian endodermal sinus tumor diagnosed during pregnancy.

Pregnancy complicated by endodermal sinus tumor of the ovary is extremely rare. The authors present a case report of a pregnant woman with persistent left adnexal mass and subsequently found to have a primary endodermal sinus tumor of the ovary that was diagnosed at 19 weeks of gestation. After left salpingo-oophorectomy had been performed, the patient chose to terminate the pregnancy before the initiation of combination chemotherapy with bleomycin, etoposide, and cisplatin. The response to chemotherapy was not satisfactory. The patient expired after seven cycles of treatment had been completed because of pulmonary fibrosis and the drug toxicity of bleomycin.

FNAC diagnosis of yolk sac tumor: a case report.

Yolk sac tumor is the second most common germ cell tumor of the ovary. A nine year old female child presenting with a lower abdominal mass diagnosed as a yolk sac tumor on fine needle aspiration cytology (FNAC) is described. Ultrasonographically (USG) guided FNAC of the lesion revealed tight clusters and papillary fronds of cells associated with homogeneous acellular eosinophilic bodies. A preoperative diagnosis of this tumor is helpful in planning further diagnostic and therapeutic steps.

Vaginal endodermal sinus tumor.

Malignant germ cell tumors are rare tumors of childhood accounting less than 3% of pediatric malignancies, and endodermal sinus tumor (EST) is the most common histological subtype. The vagina is an extremely rare site for germ cell tumors (GCT). A one-year female was admitted with history of bleeding per vagina. She had pallor and a mass was palpable anteriorly on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor mass at the junction of cervix and vagina and biopsy was suggestive of a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She underwent vaginohysterectomy. The tumor was excised in toto and patient received four courses of cisplatin, etoposide and bleomycin. At one year of follow up, patient was symptom free and serum AFP remained at normal level. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. It is both locally aggressive and capable of metastasis. Even though more conservative surgery is advised to maintain sexual and reproductive function, at times, radical surgery is mandatory depending on the infiltration of the tumor to the surrounding structures. Simple tumor excision is not sufficient, as residual cells induce recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.

Tumor del seno endodermico primario de hígado: presentación de un caso y revisión de la literatura / Endodermal sinus tumor primary of liver: presentation of a case and review of the literature

El tumor del seno endodérmico es una neoplasia maligna poco frecuente, con localización extragonadal extremadamente rara. Presentamos un caso de un paciente femenino de 58 años de edad, con diagnóstico clínico de absceso hepático. Los estudios imagenológicos mostraron una lesión neoplásica en los segmentos VI-VII del lóbulo hepático derecho. Además presentaba niveles elevados de alfa-fetoproteína (6616 ng/mL). Se le realizó hepatectomía derecha y drenaje toráxico. El estudio histopatológico e inmunohistoquímico concluyó que se trataba de un tumor del seno endodérmico. No se demostró la presencia de tumor primario extrahepático

Tumores germinales extragonadales de mediastino / Germ cell tumors of the mediastinum

Antecedentes: los tumores germinales del mediastino son lesiones de infrecuente presentación. Existe cierta tendencia a explorar los tumores mediastinales operables sin tener en cuenta que determinadas entidades patológicas, entre ellas algunos tumores germinales, tienen indicación inicial de otro tratamiento diferente. Objetivo: relatar la experiencia de los autores en una sola institución asistencial. Comunicar un algoritmo para el estudio y el tratamiento de los tumores germinales primarios de mediastino. Lugar de aplicación: Centro oncológico universitario de asistencia, docencia e investigación. Diseño: retrospectivo. Población: 9 casos. 1 mujer portadora de teratoma maduro, 8 hombres con tumores germinales malignos. Edades entre 18 y 39 años. Se diagnosticaron 3 seminomas, 2 carcinomas embrionarios, 1 tumor se seno endodérmico, 1 teratoma+carcinoma embrionario, un tumor maligno mixto (seminoma+carcinoma embrionario+teratoma inmaduro) y 1 teratoma maduro con esbozos de órganos. La forma de presentación fue dolor esternal en 4 (uno de ellos con taponamiento cardíaco por derrame pericárdico), disnea en 2, disfagia en 1 y adenopatías cervicales asintomáticas en 2. La manifestación radiológica fue tumor circunscripto en el mediastino. El teratoma maduro presentaba calcificaciones. Se operaron 5 enfermos (4 toracotomías y una videotoracoscopía). En 4 se pudo realizar exéresis completa; 1 fue irresecable por invasión de estructuras nobles contiguas. Resultados: la paciente operada por teratoma maduro, se encuentra curada. De los 8 malignos restantes 2 fueron operados inicialmente, sólo 1 fue resecado. Ambos recibieron Qt a pesar de lo cual tuvieron progresión o recaída. 6 tuvieron diagnóstico histológico antes de programar el tratamiento y recibieron quimioterapia (Qt). De éstos últimos, 2 fueron intervenidos con criterio de rescate (CxR) por persistencia de imagen tumoral luego de Qt. La paciente operada por teratoma maduro, se encuentra curada. De los 8 malignos restantes, los 2 operados inicialmente recibieron Qt: 1 de ellos se curó y el otro tuvo progresión lesional. Por efecto de la Qt exclusiva (4 enfermos), 2 se curaron y llevan más de 10 años, 2 progresaron y fallecieron. Los 2 restantes fueron sometidos a cirugía de rescate post Qt, de los cuales uno lleva 5 meses libres de enfermedad y otro falleció diseminado...

Infantile mediastinal primary endodermal sinus tumour.

Primary extragonadal mediastinal endodermal sinus tumour is rare, and to date very few cases have been reported in the literature. We present here a case of rare extragonadal highly malignant commonest germ cell tumour in an infant who presented with a rapidly progressive mediastinal mass with dry non-productive cough, tachypnea without significant respiratory distress or toxicity.

Primary germ cell tumours of the mediastinum.

Twenty nine cases of primary mediastinal germ cell tumours (MGCT) were seen at the Tata Memorial Hospital over a 16--year period (1974-1989). There were 5 benign MGCT occurring predominantly in females (80%), with these patients having an excellent result after surgery with all patients disease free at an median follow-up of 27 months. Malignant MGCT occurred only in males and demonstrated wide variation in response to treatment depending upon whether the tumour was seminomatous or non-seminomatous. There were 11 Seminomas, 5 Embryonal carcinomas, 5 Endodermal sinus tumours and 3 Teratocarcinomas. The diagnosis was established by surgical exploration or by biopsy of a lymph node or chest wall nodule in 20 patients. Four patients had needle biopsy. Seminomatous MGCT received radiotherapy as their main treatment modality and did well with 75% of the patients alive without disease at an average follow up of 33 months. The non-seminomatous MGCT could be divided into two groups. The mean survival for patients receiving cisplatinum based chemotherapy was 14 months as compared to the group not receiving such therapy where the survival was only 5.3 months. However, because of the advanced disease at presentation even in the group receiving cisplatinum chemotherapy, a long term complete response rate of only 20% could be achieved.

Tumor de seio endodérmico de ovário: relato de caso / Endodermal sinus tumor of the ovary: case report

Os tumores de seio endodérmico de ovário säo neoplasias raras e extremamente malignas. Geralmente ocorrem em adolescentes e mulheres jovens. Níveis séricos de alfafetoproteína têm sido demonstrados em pacientes portadoras desta neoplasia e servem como marcador tumoral e também como monitor do progresso terapêutico e recorrência tumoral. O prognóstico é péssimo, como demonstrado em nosso caso. Relatamos aqui um caso de tumor de seio endodérmico.