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)

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.

Bleomycin induced hyperpigmentation with yolk sac tumor.

Hyperpigmentation is one of the cutaneous side effects of chemotherapautic agents, but it is usually accepted as a cosmetic problem. We report a child with yolk sac tumor who developed localized pigmentation after the first course of chemotherapy regimen that included cisplatin, etoposide and bleomycin. The hyperpigmentation was diffuse scattered, flagellate like and linear streaking which was thought to be mainly related to the skin toxicity of bleomycin.

Transient cortical blindness during chemotherapy (PVB) for ovarian germ cell tumor.

A 17 year-old female with stage IIIc endodermal sinus tumor of the ovary developed transient cortical blindness and severe hypertension after 5 cycles of PVB regimen consisting of cisplatin, vinblastine and bleomycin. Clinical and radiological findings were compatible with Posterior LeukoEncephalopathy (PLE). Her visual acuity and blood pressure completely recovered within a few days after supportive treatment with antihypertensive drug. This condition is unpredictable but it can be reversible without long term sequelae. Most reports suggested that this rare toxicity was from cisplatin therapy. However, the exact pathophysiogenesis of this condition was not known precisely. Prompt reduction in blood pressure and withdrawal of immunosuppressive agents might lead to rapid reversal of this syndrome and prevent permanent brain damage.

An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report.

Yolk sac tumours are rare conditions among the germ cell tumours. Intracerebral germ cell tumours are exceedingly rare. A 15 year old girl presenting with a one week history of raised intracranial pressure is described. She had bilateral papilloedema and a right 6th nerve palsy. CT scan showed an intra-parenchymatous right frontal ring enhancing lesion of 2 cms diameter. The patient underwent microsurgical total excision of the tumour, followed by chemotherapy. She was asymptomatic at three years following surgery.

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...

Tumor do saco vitelino primário em retroperitônio / Primary retroperitoneal yolk sac tumor

A case of primary extragonadal yolk sac tumor in the retroperitoneum of a young adult male is reported. The symptoms were melena and weakness for two months. Radiologic studies suggested a retroperitoneal tumor infiltrating the duodenum, artery aorta and vein cava, was found. Partial resection was performed, remaining tumor around the vessels. Microscopic examination disclosed a yolk sac tumor infiltrating the duodenum. The patient was managed unsuccessfully with radiotherapy, but good results were actived with chemotherapy. Few cases like that were reported in the literature