Imaging in gynecological disease (22): clinical and ultrasound characteristics of ovarian embryonal carcinomas, non-gestational choriocarcinomas and malignant mixed germ cell tumors.

OBJECTIVE: To describe the clinical and ultrasound characteristics of three types of rare malignant ovarian germ cell tumor: embryonal carcinoma, non-gestational choriocarcinoma and malignant mixed germ cell tumor. METHODS: This was a retrospective multicenter study. From the International Ovarian Tumor Analysis (IOTA) database, we identified patients with a histological diagnosis of ovarian embryonal carcinoma, non-gestational choriocarcinoma or malignant mixed germ cell tumor, who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 2000 and 2020. Additional patients with the same histology were identified from the databases of the departments of gynecological oncology in the participating centers. All tumors were described using IOTA terminology. Three examiners reviewed all available ultrasound images and described them using pattern recognition. RESULTS: One patient with embryonal carcinoma, five patients with non-gestational ovarian choriocarcinoma and seven patients with ovarian malignant mixed germ cell tumor (six primary tumors and one recurrence) were identified. Seven patients were included in the IOTA studies and six patients were examined outside of the IOTA studies. The median age at diagnosis was 26 (range, 14-77) years. Beta-human chorionic gonadotropin levels were highest in non-gestational choriocarcinomas and alpha-fetoprotein levels were highest in malignant mixed germ cell tumors. Most tumors were International Federation of Gynecology and Obstetrics (FIGO) Stage I (9/12 (75.0%)). All tumors were unilateral, and the median largest diameter was 129 (range, 38-216) mm. Of the tumors, 11/13 (84.6%) were solid and 2/13 (15.4%) were multilocular-solid; 9/13 (69.2%) manifested abundant vascularization on color Doppler examination. Using pattern recognition, the typical ultrasound appearance was a large solid tumor with inhomogeneous echogenicity of the solid tissue and often dispersed cysts which, in most cases, were small and irregular. Some tumors had smooth contours while others had irregular contours. CONCLUSIONS: A unilateral, large solid tumor with inhomogeneous echogenicity of the solid tissue and with dispersed small cystic areas in a young woman should raise the suspicion of a rare malignant germ cell tumor. This suspicion can guide the clinician to test tumor markers specific for malignant germ cell tumors. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Carcinoma Embrionário de Mediastino Posterior com Metástase Pulmonar: Relato de Caso / Carcinoma Embrionario de Mediastino Posterior con Metástasis Pulmonar: Relato de Caso / Posterior Mediastinum Embryonal Carcinoma with Pulmonary Metastasis: Case Report

Introdução: Os carcinomas embrionários são os mais raros e, geralmente, se apresentam quando já estão associados com outros componentes de células germinativas. Possuem características clínicas e radiológicas similares aos tumores de saco vitelino. Relato do caso: Paciente G.A.S.L, sexo masculino, 30 anos, ex-tabagista e etilista. Iniciou com quadro de dores no hemitórax esquerdo em fevereiro de 2018 com extensão dos sintomas para a região da coluna torácica e lombar. Realizou tomografia de abdômen total e tórax, com resultado sugestivo de tumor de mediastino, nódulos pulmonares, material tecidual com densidade de partes moles no mediastino posterior e numerosas linfonodomegalias retroperitoneais, evoluiu com paraplegia de membros inferiores por compressão nervosa e com hipoestesia. Realizou biópsia de tumor de mediastino posterior à esquerda com resultado de neoplasia maligna epitelial e diagnóstico de carcinoma embrionário extragonodal pouco diferenciado. O paciente apresentou metástase pulmonar, confirmando que esses tumores frequentemente se infiltram nos órgãos adjacentes. A quimioterapia baseada em cisplatina é o tratamento padrão, levando à melhora da sobrevida em pacientes com esse tipo de tumor. Após a quimioterapia, houve diminuição do volume tumoral, porém, seguiu com a paraplegia de membros inferiores em razão da compressão nervosa. Conclusão: Este estudo relata o caso de um paciente jovem, com tumor raro de células germinativas e metástase pulmonar, que evoluiu clinicamente estável após tratamento específico com quimioterápicos. Por ainda haver uma escassa literatura acerca do tema, este estudo traz novas evidências e achados.

Introduction: Embryonal carcinomas are the rarest, and usually present when they are already associated with other components of germ cells. They have clinical and radiological features similar to yolk sac tumors. Case report: Patient G.A.S.L, male, 30 years old, former smoker and alcoholic. Initially, the patient reported pain in the left hemithorax in February 2018 with extension of the symptoms to the region of the thoracic and lumbar spine. A tomography of the total abdomen and chest was performed, with result suggestive of mediastinal tumor, pulmonary nodules, tissue material with soft tissue density in the posterior mediastinum and numerous retroperitoneal lymph node enlargement, which evolved with paraplegia of the lower limbs by nerve compression and hypoesthesia. It was conducted a biopsy of a mediastinal tumor posterior to the left with result of malignant epithelial neoplasia and diagnosis of extragonadal embryonic carcinoma very little differentiated. The patient presented pulmonary metastasis confirming that these tumors frequently infiltrate into the adjacent organs. Cisplatin-based chemotherapy is the standard treatment, leading to improved survival in patients with this type of tumor. After chemotherapy, the tumor volume decreased, but the patient continued with paraplegia of lower limbs due to nerve compression. Conclusion: This study reports the case of a young patient with a rare germ cell tumor and pulmonary metastasis who evolved clinically stable after specific chemotherapy treatment. Because there is still scarce literature on the subject, this study brings new evidences and findings.

Introducción: Los carcinomas embrionarios son los más raros y generalmente se presentan cuando ya están asociados con otros componentes de células germinativas. Se presentan características clínicas y radiológicas similares a los tumores de saco vitelino. Relato del caso: Paciente G.A.S.L, sexo masculino, 30 años, ex tabaquista y etilista. Se inició con cuadro de dolores en el hemitórax izquierdo en febrero de 2018 con extensión de los síntomas para la región de la columna torácica y lumbar. Se realizó una tomografía de abdomen total y tórax, con resultado sugestivo de tumor de mediastino, nódulos pulmonares, material tisular con densidad de partes blandas en el mediastino posterior y numerosas linfonodomegalias retroperitoneales, evolucionó con paraplejia de miembros inferiores por compresión nerviosa y con hipoestesia. Se realizó biopsia de tumor de mediastino posterior a la izquierda con resultado de neoplasia maligna epitelial y diagnóstico de carcinoma embrionario extra gonodal poco diferenciado. El paciente presentó metástasis pulmonar confirmando que estos tumores frecuentemente se infiltran en los órganos adyacentes. La quimioterapia basada en cisplatino es el tratamiento estándar, llevando a la mejora de la supervivencia en pacientes con este tipo de tumor. Después de la quimioterapia hubo la disminución del volumen tumoral, sin embargo, siguió con la paraplejia de miembros inferiores debido a la compresión nerviosa. Conclusión: Este estudio informa el caso de un paciente joven con un tumor raro de células germinales y metástasis pulmonar que evolucionó clínicamente estable después de un tratamiento de quimioterapia específico. Debido a que todavía hay poca literatura sobre el tema, este estudio aporta nuevas pruebas y hallazgos.

Metastatic cardiac tumors: literature review and own observation of testicular tumor metastasis in the right ventricle of the heart.

BACKGROUND: Tumors of the heart are uncommon and usually benign (in 93% cases myxomas are observed). More often secondary, metastatic tumors are detected in the heart, as a rule, at pronounced progression of the malignant neoplasm with multiple lesions of other internal organs (lung, pleura, liver, etc.). Literature review on cardiac metastases of different tumors is given. CASE REPORT: Own observation of a young man with rare single metastasis of malignant testicular germ cell tumor with predominance of embryonic carcinoma in the right ventricle of the heart is presented; the primary tumor was detected after metastasis revealing. The diagnostic algorithm using routine histological study supplemented with immunohistochemistry, including detection of cytokeratin pan, cytokeratin 5/6, cytokeratin 7, CD30, OCT4, TTF-1, hCG, and AFP markers expression, is described.

Hematoma retroperitoneal como presentación de cáncer testicular / Retroperitoneal hematoma debut as testicular cancer

Resumen Introducción: El hematoma retroperitoneal (HR) es una enfermedad infrecuente con una elevada morbimortalidad, siendo complicado cuando se presenta con dolor y shock hipovolémico. Presentación del caso: Paciente del sexo masculino, de 20 años de edad, sin antecedentes mórbidos. Ingresa en Urgencias por dolor abdominal en el flanco izquierdo, irradiado a dorso y testículo ipsilateral, de 6 h de evolución, de inicio súbito e intensidad severa; el paciente está pálido, hemodinámicamente estable, sin signos de irritación peritoneal. Se solicita pielo-TC por sospecha de litiasis ureteral, que muestra un extenso HR, probable aneurisma aórtico roto. Una angio-TC informa HR adyacente y anterior a psoasilíaco izquierdo, de20 × 11 × 8,5 cm, volumen 972 cc, adenopatías retroperitoneales paraaórticas bilaterales sangrantes y múltiples nódulos pulmonares bilaterales indicativos de diseminación secundaria. Se constata testículo derecho duro, de tamaño normal, eco testicular con masa sólida quística, que indica de lesión orgánica. Discusión: Trauma y enfermedad tumoral son las principales causas de HR. El cáncer testicular suele presentarse en pacientes jóvenes, requiriendo una pronta derivación y estudio debido a su rápida progresión. En nuestro caso, el HR fue un hallazgo imagenológico, destacando que el sangrado de un conglomerado de adenopatías es anecdótico.

Abstract Introduction: Retroperitoneal hematoma (RH) is a rare disease with high morbidity, being complicated when presented with pain and hypovolemic shock. Case report: Male, 20 years old, no morbid history. Arrive to Emergency Service for abdominal pain in the left flank radiating to the back and ipsilateral testis, 6 h of evolution, sudden onset, high intensity; pacient pale, hemodynamically stable without signs of peritoneal irritation. Pielo-TC is requested on suspicion of ureteral stones showing extensive RH, likely ruptured aortic aneurysm. CT angiography reports RH and adjacent preceding left iliopsoas, 20 × 11 × 8.5 cm, volume 972 cc, retroperitoneal bleeding bilateral para-aortic lymphadenopathy and multiple bilateral pulmonary nodules suggestive of secondary spread. Hard right testicle with normal size, testicular ultrasound pointing solid cystic mass, suggestive of organic lesion. Discussion: Trauma and tumor pathology are the main causes of RH. Testicular cancer usually occurs in young patients, requiring early referal and study because of its rapid progression. In our case, the HR was an imaging finding, highlighting that the bleeding of a cluster of lymph nodes is anecdotal.

Embryonal carcinoma presenting as a calcified solitary testicular mass on ultrasound.

A 24-year-old man presented with a 2-week history of a painless right testicular mass; ultrasound demonstrated a dense, solitary calcified mass. The patient elected observation after further workup showed no evidence of metastasis.A repeat ultrasound 3 months later showed interval growth and the patient underwent right radical orchiectomy. Pathology was consistent with pure embryonal carcinoma of the testis. Calcified testicular masses are typically benign but do carry a differential of spermatic granuloma, large-cell calcifying Sertoli cell tumour, trauma, tuberculosis, filariasis, calcified Leydig cell tumour and burned-out testicular tumour.To our knowledge, this is the first case report of pure embryonal carcinoma presenting as a solitary calcified testicular mass.

Clinical and Radiologic Features of Pediatric Basal Ganglia Germ Cell Tumors.

BACKGROUND AND OBJECTIVE: Pediatric basal ganglia germ cell tumors (GCTs) represent a rare subset of tumors about which little is known. We aimed to summarize the clinical features and radiological findings of this special subgroup of GCTs. METHODS: From January 2010 to January 2015, 12 pediatric patients with basal ganglia GCTs were treated in our hospital. The clinical features, radiologic findings, diagnosis, treatment, and outcome of these patients were analyzed retrospectively. Our institutional diagnostic principle and treatment strategy of this disease were discussed. RESULTS: GCTs accounted for 25.5% of all the pediatric basal ganglia tumors treated in our hospital. There were 9 male and 3 female patients with a mean age of 11.5 ± 2.1 years. The most common symptom was progressive hemiparesis (n = 9, 75%). The radiologic findings showed that the lesions predominately located in caput of caudate nucleus (n = 9, 75.0%), followed by lenticular nucleus (n = 3, 25.0%). Hemiatrophy was commonly observed (n = 8, 66.7%). Eight patients were diagnosed as having germinomas, and 4 patients as having nongerminomatous germ cell tumors. During the follow-up period, preoperative neurologic dysfunctions improved in 7 patients and remained stable in 3. Two patients developed new onset of neurologic dysfunction after the treatment. Two patients suffered from tumor recurrence. CONCLUSIONS: GCTs are not as rare as considered in pediatric basal ganglia tumors. They bear some distinctive clinical and radiologic features, which can help with the accurate diagnosis and successful management of such tumors.

Ovarian malignant mixed germ cell tumor with clear cell carcinoma in a postmenopausal woman.

Malignant germ cell tumors of the ovary are very rare and account for about 2-5% of all ovarian tumors of germ origin. Most patients are adolescent and young women, approximately two-thirds of them are under 20 years of age, occasionally in postmenopausal women. But clear cell carcinoma usually occurs in older patients (median age: 57-year old), and closely related with endometriosis. Here we report a case of a 55-year old woman with right ovarian mass that discovered by B ultrasonic. Her serum levels of human chorionic gonadotropin (hCG) and α-fetoprotein (AFP) were elevated. Pathological examination revealed the tumor to be a mixed germ cell tumor (yolk sac tumor, embryonal carcinoma and mature teratoma) with clear cell carcinoma in a background of endometriosis. Immunohistochemical staining showed SALL4 and PLAP were positive in germ cell tumor area, hCG, CD30 and OCT4 were positive in epithelial-like cells and giant synctiotrophoblastic cells, AFP, AAT, CD117 and Glyp3 were positive in yolk sac component, EMA and CK7 were positive in clear cell carcinoma, CD10 was positive in endometrial cells of endometriotic area. She was treated with surgery followed by seven courses of chemotherapy. She is well and serum levels of hCG and AFP have been decreased to normal levels.

Echocardiography in imaging an extremely rare cause of extrinsic pulmonary stenosis: rapidly progressive primary mediastinal embryonal carcinoma.

We report a case of a young man with a very rare cause of atypical chest pain in whom primary mediastinal embryonal carcinoma causing extrinsic pulmonary stenosis was diagnosed with echocardiography and computed tomography. This patient illustrates an unusual presentation of the very rapid progression of the tumor in as little as 6 months. The patient underwent surgical resection and was successfully treated with adjuvant chemotherapy.

Undifferentiated embryonal sarcoma: a case report.

BACKGROUND: Undifferentiated embryonal sarcoma (UES) is the fourth most common hepatic malignancy diagnosed in children, but is extremely rare in older patients. Classification of the tumor is difficult because of its unknown etiology. PURPOSE: The purpose of this study is to describe a case of UES in a 19-year-old pregnant woman with UES who delivered a stillborn fetus and passed away because of UES, and how medical imaging can be used to help differentially diagnose UES. CONCLUSION: A multiphase liver computed tomography protocol best demonstrates all forms of liver cancers and lesions; therefore, it is the scan of choice when a liver abnormality is suspected but the type of lesion is unknown.

Non-palpable testicular embryonal carcinoma diagnosed by ultrasound: a case report.

With the extensive use of scrotal ultrasound (US), incidental non-palpable testicular tumors have thus been unexpectedly discovered. This report documents the case of 24-year-old male with a non-palpable testicular tumor that contained non-seminomatous germ cell components detected by US. Radical orchiectomy was performed and histological examinations confirmed a diagnosis of a mixed tumor of seminoma and embryonal carcinoma. Serum alpha-fetoprotein (AFP) rose from 7.8 to 43 ng/ml and CT scan revealed multiple metastases only 1 month after the operation. Systemic chemotherapy was introduced immediately, and the serum level of AFP decreased to the normal range and the metastatic lesions had disappeared after three courses of the chemotherapy. No recurrence was observed at 18 months follow-up after the chemotherapy. This is the first case of non-palpable testicular embryonal carcinoma, which metastasized soon after the resection. The obscurity and implications of such a diagnosis are also discussed.

Early evaluation of the effects of chemotherapy with longitudinal FDG small-animal PET in human testicular cancer xenografts: early flare response does not reflect refractory disease.

AIM: We aimed to evaluate the usefulness of FDG PET in the early prediction of the effects of chemotherapy on human testicular cancer xenografts. MATERIAL AND METHODS: Nude rats bearing subcutaneous human embryonal carcinoma xenografts received either cisplatin (5 mg/kg) or saline serum. Small-animal PET studies were performed on days 0, 2, 4 and 7 and compared to immunochemistry studies, flow cytometry studies and hexokinase assays. RESULTS: Cisplatin treatment resulted in biphasic FDG uptake evolution: a peak was observed on day 2, followed by a marked decrease on day 7 despite an insignificant change in tumour volume. Similarly, a peak in cyclin A immunostaining was observed on days 2 and 4), followed by a significant decrease on day 7. Flow cytometry showed that the cyclin A peak was not related to increased cell proliferation but was due to a transient S and G(2)/M cell cycle arrest. A marked increase in cell apoptosis was observed from day 2 to day 7. GLUT-1 showed a significant decrease on day 7. Macrophagic infiltrate remained stable except for an increase observed on day 7. In control tumours, continuous growth was observed, all immunostaining markers remaining stable over time. Hexokinase activity was significantly lower on day 7 in treated tumours than in controls. CONCLUSION: FDG PET may be useful in the early evaluation of treatment in patients with testicular cancer. In our model, a very early increased [(18)F]-FDG uptake was related to a transient cell cycle arrest and early stage apoptosis but did not reveal refractory disease.

[Positron emission tomography in oncourology].

The authors present the results of the examination of 61 patients with genitourinary space-occupying lesions, using 18F- fluorodeoxyglucose positron emission tomography (PET) in whole body mode. In all cases the diagnosis was verified morphologically. The results demonstrated high diagnostic accuracy of PET, including possibility to determine the extent of oncourological cancer. However, the method displays poor efficacy in cases of hypernephroid cancer due to low level of glycolysis in this type of tumor.

Primary embryonal carcinoma of the orbit in a 10-month-old female: a seven-year follow-up.

PURPOSE: Extra-gonadal germ cell tumors (GCTs) are rare and can be highly aggressive. If correctly identified and treated with multimodality chemotherapy, their prognosis can be significantly improved. We examined a 10 month-old female with primary embryonal carcinoma of the orbit. DESIGN: Case report and literature review. METHODS: Case study with 7-year follow-up and literature review of intracranial and intraorbital GCT cases. RESULTS: The patient presented with progressive proptosis and ophthalmoplegia. CT scan revealed an orbital apex mass and biopsy demonstrated a nongerminomatous GCT--an embryonal carcinoma. The patient is tumor-free 7 years after multimodality chemotherapy. She has mild amblyopia and a right micro esotropia. CONCLUSIONS: Nongonadal GCTs of the orbit can occur and should be considered in the differential diagnosis of a young child with proptosis and ophthalmoplegia. Five-year survival rates improve significantly with accurate identification and treatment.

Testicular recurrence from "primary" retroperitoneal germ cell tumor.

Extragonadal germ cell tumors are a rare clinical entity. We present the case of an extragonadal germ cell tumor followed by testicular recurrence more than 4 years later. This presents an interesting dilemma as to whether this was recurrence of the original tumor or a new, primary tumor. The patient's outcome and surveillance protocol for these tumors are presented.