RESUMEN
BACKGROUND: Diagnosing non-gestational uterine choriocarcinoma in children is challenging because of its rarity and nonspecific imaging findings. Herein, we report a case of non-gestational uterine choriocarcinoma in a child, which was unexpectedly found during exploratory laparotomy and confirmed by histopathological findings. However, the tumor did not respond to chemotherapy. CASE PRESENTATION: A 4-year-old Indonesian female patient was brought into the emergency unit with chief complaint of vaginal bleeding. She had suffered from vaginal spotting 4 months before being admitted to the hospital. Physical examination revealed a distended abdomen in the left lumbar region and a palpable fixed mass with a smooth surface. Abdominal computed tomography scans revealed a large mass (10 × 6 × 12 cm) with fluid density and calcification. Thus, we suspected left ovarian teratoma. The patient's luteinizing hormone, follicle-stimulating hormone, and lactate dehydrogenase levels were 25.2 mIU/ml, 0.1 mIU/ml, and 406 U/l, respectively. According to the clinical and radiological findings, we decided to perform an exploratory laparotomy and found a tumor originating from the uterus, not the ovarium. We did not observe liver nodules and any enlargement of abdominal lymph nodes. Subsequently, we performed hysterectomy. The histopathological findings supported the diagnosis of choriocarcinoma. The patient was discharged uneventfully on postoperative day 5. Thereafter, the patient underwent nine cycles of chemotherapy, including carboplatin (600 mg/m2 IV), etoposide (120 mg/m2 IV), and bleomycin (15 mg/m2 IV). However, on the basis of the clinical findings of a palpable mass and partial intestinal obstruction, the tumor relapsed soon after the ninth cycle of chemotherapy. Currently, the patient is undergoing chemotherapy again. CONCLUSIONS: Although pure non-gestational uterine choriocarcinoma is rare, it should be considered as one of the differential diagnoses for intraabdominal tumors in a child, so as to better guide and counsel families regarding the surgical plan and prognosis, respectively. In the present case, the patient's response to chemotherapy was poor, implying that the treatment of non-gestational choriocarcinoma is still challenging, particularly in the pediatric population.
Asunto(s)
Coriocarcinoma no Gestacional , Histerectomía , Neoplasias Uterinas , Humanos , Femenino , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/cirugía , Neoplasias Uterinas/patología , Neoplasias Uterinas/tratamiento farmacológico , Neoplasias Uterinas/terapia , Preescolar , Coriocarcinoma no Gestacional/diagnóstico , Coriocarcinoma no Gestacional/patología , Coriocarcinoma no Gestacional/tratamiento farmacológico , Coriocarcinoma no Gestacional/terapia , Tomografía Computarizada por Rayos X , Diagnóstico Diferencial , Laparotomía , Hemorragia Uterina/etiología , Etopósido/uso terapéutico , Etopósido/administración & dosificaciónRESUMEN
INTRODUCTION: Trophoblastic neoplasms are often associated with pregnancy, and nongestational trophoblastic neoplasms are extremely rare. Nongestational ovarian choriocarcinoma (NGCO) is a highly aggressive germ cell-derived tumor frequently presenting with early hematogenous metastasis. PATIENT CONCERNS: Herein, we report a case of a 28-year-old unmarried woman with regular menstruation who experienced vaginal bleeding 1 week after her last menstrual cycle. Doppler ultrasound revealed bilateral adnexal masses and elevated serum human chorionic gonadotropin (hCG) levels. The patient was initially misdiagnosed as presenting an ectopic pregnancy. DIAGNOSIS: The final pathology confirmed an International Federation of Gynecology and Obstetrics stage IA NGCO with bilateral mature teratoma of the ovary. This is an extraordinary instance of ovarian choriocarcinoma which emerged without any prior gestation, and the patient's lack of a history of pregnancy made the diagnosis ignored. INTERVENTIONS: After initial surgery and 1 cycle of bleomycin, etoposide, and cisplatin (BEP) chemotherapy, a laparoscopic fertility-preserving comprehensive staging surgery was performed. Two cycles of chemotherapy with BEP were administered as supplemental therapy postsurgery, and leuprorelin was administered to protect ovarian function. OUTCOMES: Menstruation resumed 4 months after chemotherapy completion, and tumor indicators were within the normal range. No signs of recurrence were observed at the 36-month follow-up. CONCLUSION: NGCO should be considered if a female patient exhibits irregular vaginal bleeding and masses in the adnexal area. The present case and our literature review also highlighted that fertility-sparing surgery and multidrug chemotherapy are effective methods for treating NGCO.
Asunto(s)
Coriocarcinoma no Gestacional , Neoplasias Ováricas , Teratoma , Humanos , Femenino , Adulto , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/patología , Teratoma/diagnóstico , Teratoma/patología , Coriocarcinoma no Gestacional/diagnóstico , Coriocarcinoma no Gestacional/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Etopósido/uso terapéutico , Etopósido/administración & dosificación , Embarazo , Bleomicina/administración & dosificación , Bleomicina/uso terapéuticoRESUMEN
Choriocarcinoma is a highly vascular and invasive tumor of anaplastic trophoblast, predominantly made up of cytotrophoblasts and syncytiotrophoblasts without villi. Based on its origin, choriocarcinoma can be either gestational or non-gestational. Non-gestational choriocarcinoma can be of germ cell origin, or can be seen in association with a somatic high-grade malignancy. It is difficult to differentiate gestational from non-gestational choriocarcinoma, especially in the reproductive age group. It is important to distinguish between the two, for accurate staging and prognostication, deciding the primary treatment modality, (ie, surgery or chemotherapy), and tailoring follow-up timeframes after diagnosis. An extensive literature search was performed regarding all cases of non-gestational choriocarcinoma, published before March 2023. A note was made of whether the origin of choriocarcinoma was ascertained and how gestational choriocarcinoma was differentiated from non-gestational choriocarcinoma. The keywords used for literature search were "non-gestational choriocarcinoma", "primary choriocarcinoma", "ovarian choriocarcinoma", "ovarian germ cell tumors", or "choriocarcinomatous differentiation". This review aims to summarize the similarities and differences in the epidemiology, pathogenesis, clinical presentation, and management guidelines between gestational and non-gestational choriocarcinoma, which can form an important educational resource for clinicians and laboratory physicians dealing with such cases.
Asunto(s)
Coriocarcinoma no Gestacional , Humanos , Femenino , Embarazo , Coriocarcinoma no Gestacional/diagnóstico , Coriocarcinoma no Gestacional/patología , Coriocarcinoma no Gestacional/terapia , Coriocarcinoma/diagnóstico , Coriocarcinoma/patología , Coriocarcinoma/terapia , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/patología , Neoplasias Ováricas/terapia , Neoplasias Uterinas/patología , Neoplasias Uterinas/diagnósticoRESUMEN
BACKGROUND: Non-gestational choriocarcinoma (NGC) is a rare subtype of malignant germ cell tumour and there is no consensus on its treatment. The lack of suitable preclinical models for NGC is a challenge in drug discovery research. Patient-derived xenograft (PDX) models recapitulate the tumour microenvironment of the original cancer tissue. Therefore, they have received considerable attention for studies on rare cancer. Here, we aimed to establish a PDX model from a patient with recurrent NGC. METHODS: Fresh NGC tumour tissue was immediately transplanted into a severely immune-deficient mouse (NOD.Cg-Prkdcscid1l2rgtm1Wjl/SzJ) and maintained for more than three in vivo passages. Subsequently, we evaluated the molecular characteristics of the PDX model using immunohistochemistry, polymerase chain reaction, and RNA sequencing. Moreover, the PDX tumours were transplanted into BALB/c nude mice, and we evaluated their sensitivity for cisplatin and methotrexate. RESULTS: The PDX tumour maintained the morphological features of NGC. Moreover, Immunohistochemistry revealed that the human chorionic gonadotropin, cytokeratin 7, and EpCAM expression levels were similar to those in the primary tumour. Furthermore, serum human chorionic gonadotropin levels were elevated in both the primary tumour and the PDX models. Additionally, using PCR analysis with species-specific primers, we confirmed that the PDX tumour contained human genes and was derived from human tissue. Moreover, the gene expression profile of the NGC was compared with that of epithelial ovarian cancer samples and cell lines, and 568 dysregulated genes in the NGC were extracted. The expression of the dysregulated genes in PDX was significantly correlated with that in the primary tumour (R2 = 0.873, P < 0.001). Finally, we demonstrated that the PDX tumour was sensitive to cisplatin and methotrexate; therefore, its clinical response to the agents was similar to that of the primary tumour. CONCLUSIONS: We successfully established a PDX model of NGC, to the best of our knowledge, for the first time. The established PDX retained the molecular and transcriptome characteristics of the primary tumour and can be used to predict drug effects. It may facilitate further research and the development of novel therapeutic agents for NGC.
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Coriocarcinoma no Gestacional , Cisplatino , Femenino , Humanos , Ratones , Animales , Ensayos Antitumor por Modelo de Xenoinjerto , Metotrexato , Xenoinjertos , Ratones Desnudos , Ratones Endogámicos NOD , Modelos Animales de Enfermedad , Gonadotropina Coriónica , Ratones SCID , Microambiente TumoralAsunto(s)
Pared Abdominal , Coriocarcinoma no Gestacional , Coriocarcinoma , Embarazo , Femenino , Humanos , Coriocarcinoma/patologíaAsunto(s)
Adenocarcinoma de Células Claras , Coriocarcinoma no Gestacional , Coriocarcinoma , Neoplasias Uterinas , Embarazo , Femenino , Humanos , Coriocarcinoma no Gestacional/patología , Neoplasias Uterinas/patología , Coriocarcinoma/tratamiento farmacológico , Coriocarcinoma/patología , Adenocarcinoma de Células Claras/tratamiento farmacológicoRESUMEN
O coriocarcinoma ovariano não gestacional é uma apresentação rara de câncer de ovário, acometendo principalmente mulheres pré-púberes. É considerada uma neoplasia agressiva, sendo comum a ocorrência de expansão para o pulmão em cerca de 80% dos pacientes, como no caso a seguir. Relato do caso: Sexo feminino, 12 anos de idade, com sangramento vaginal e distensão abdominal prolongados. A tomografia computadorizada mostrou volumosa massa heterogênea predominantemente cística e múltiplos septos grosseiros de permeio. Dosagem do beta-HCG de 49.929,81 mUI/ml. Foi submetida à laparotomia mediana para estadiamento, com anexectomia esquerda mais ressecção do tumor retroperitoneal e do omento, identificando-se estádio IV. O exame histopatológico concluiu ser um tumor de células germinativas do ovário constituído por coriocarcinoma não gestacional. Após alta hospitalar, foi submetida a sessões de quimioterapia. Posteriormente, apresentou em exames de imagem nódulos em ambos os pulmões, além de formações expansivas distribuídas no parênquima hepático. Nesse contexto, foi realizada metastectomia pulmonar meses depois. Após isso, novos exames de imagem foram realizados para o reestadiamento da doença. Foram encontrados alguns nódulos pulmonares residuais e, na ressonância magnética de crânio, sinais de hemorragia crônica. A evolução da paciente não foi favorável, havendo agravamento do estado geral e óbito um ano após o diagnóstico. Conclusão: Compreende-se, desse modo, a agressividade dessa doença, em especial na faixa pediátrica feminina, uma vez que a metástase precoce ocorre em uma porcentagem significativa dos casos, levando a um prognóstico desfavorável
Non-gestational ovarian choriocarcinoma is a rare form of ovarian cancer, mainly affecting prepubertal women. It is considered an aggressive neoplasm and expansion to the lung is common in around 80% of patients, as in the following case. Case report: Female, 12 years old, with prolonged vaginal bleeding and abdominal distension. Computed tomography showed a large heterogeneous mass, predominantly cystic, with multiple coarse septa. The beta HCG level was 49,929.81 mUI/ml. She underwent median laparotomy for staging, with left adnexectomy plus resection of the retroperitoneal tumor and omentum, identifying stage IV. The histopathological examination concluded that it was a germ cell tumor of the ovary consisting of non-gestational choriocarcinoma. After being discharged from hospital, she underwent chemotherapy sessions. Subsequently, imaging showed nodules in both lungs, as well as expansive formations distributed in the liver parenchyma. In this context, pulmonary metastasectomy was performed months later. After this, new imaging tests were carried out to restage the disease and the following findings were seen: some residual pulmonary nodules and on the MRI of the skull, a sign of chronic hemorrhage. The patient's evolution was not favorable, her general condition worsened and she died one year after diagnosis. Conclusion: The aggressiveness of this disease is clear, especially in female pediatric patients, since early metastasis occurs in a significant percentage of cases, leading to an unfavorable prognosis.
El coriocarcinoma ovárico no gestacional es una presentación poco frecuente del cáncer de ovario, que afecta principalmente a mujeres prepúberes. Se considera una neoplasia agresiva y la expansión al pulmón es frecuente en alrededor del 80% de las pacientes, como en el caso siguiente. Informe del caso: Mujer de 12 años con hemorragia vaginal prolongada y distensión abdominal. La tomografía computarizada mostró una gran masa heterogénea, predominantemente quística, con múltiples septos gruesos. El nivel de beta HCG era de 49 929,81 mUI/ml. Se le practicó una laparotomía media para la estadificación, con anexectomía izquierda más resección del tumor retroperitoneal y del epiplón, identificándose un estadio IV. El examen histopatológico concluyó que se trataba de un tumor germinal de ovario consistente en un coriocarcinoma no gestacional. Tras el alta hospitalaria, se sometió a sesiones de quimioterapia. Posteriormente, el diagnóstico por imagen mostró nódulos en ambos pulmones, así como formaciones expansivas distribuidas en el parénquima hepático. En este contexto, meses más tarde se le practicó una metastasectomía pulmonar. Tras ésta, se realizaron nuevas pruebas de imagen para reestadificar la enfermedad y se observaron los siguientes hallazgos: algunos nódulos pulmonares residuales y, en la resonancia magnética del cráneo, una señal de hemorragia crónica. La evolución de la paciente no fue favorable, su estado general empeoró y falleció un año después del diagnóstico.Conclusión: Por lo tanto, es comprensible la agresividad de esta enfermedad, especialmente en las mujeres pediátricas, ya que en un porcentaje significativo de casos se producen metástasis tempranas, lo que conlleva un pronóstico desfavorable
Asunto(s)
Coriocarcinoma no Gestacional , Pediatría , NeoplasiasRESUMEN
OBJECTIVE: To report the rare case of gestational primary ovarian choriocarcinoma coexistent with intrauterine pregnancy, successfully treated with surgery and systemic chemotherapy. We also describe the utility of short tandem repeat (STR) genotyping in the diagnosis of choriocarcinoma. CASE REPORT: A 38-year-old woman at 17 gestational weeks presented with an ovarian tumor rupture in the left ovary. Left salpingo-oophorectomy was performed and the patient was diagnosed with gestational ovarian choriocarcinoma via histopathology and STR genotyping. After artificial abortion, the patient underwent 8 cycles of chemotherapy. Abdominal hysterectomy was performed because of the presence of low levels of human chorionic gonadotropin and the tumor that developed behind the uterus. However, no viable choriocarcinoma cells were found in the residual tumor, suggesting that the patient achieved full remission. CONCLUSIONS: Early detection is crucial in treating choriocarcinomas; thus, clinicians should consider the possibility of choriocarcinoma at the presence of an ovarian tumor during pregnancy. Gestational and non-gestational choriocarcinomas differ in prognosis and sensitivity to chemotherapy due to their different etiologies. Therefore, STR genotyping may be beneficial in predicting the patient's prognosis or selecting the appropriate regimen.
Asunto(s)
Coriocarcinoma no Gestacional , Coriocarcinoma , Neoplasias Ováricas , Adulto , Coriocarcinoma/complicaciones , Coriocarcinoma/diagnóstico , Coriocarcinoma/terapia , Coriocarcinoma no Gestacional/complicaciones , Coriocarcinoma no Gestacional/diagnóstico , Coriocarcinoma no Gestacional/genética , Femenino , Humanos , Neoplasias de Células Germinales y Embrionarias , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/terapia , Embarazo , PronósticoAsunto(s)
Coriocarcinoma no Gestacional , Coriocarcinoma , Neoplasias Uterinas , Coriocarcinoma/diagnóstico , Coriocarcinoma no Gestacional/diagnóstico , Coriocarcinoma no Gestacional/patología , Femenino , Humanos , Embarazo , Neoplasias Uterinas/diagnóstico por imagen , Neoplasias Uterinas/patologíaRESUMEN
Choriocarcinoma is a highly malignant tumour emerging from the syncytiotrophoblast divided into gestational and non-gestational presentations. Primary choriocarcinoma of the mediastinum is rare. Metastases to the brain often occur; however, brainstem involvement has not been reported for non-gestational choriocarcinoma. We described a middle-aged man who developed a complete left oculomotor nerve paralysis secondary to a brainstem tumour at the midbrain. The workup for the primary source of the brainstem tumour included a chest CT scan, which revealed a mediastinal mass. A mediastinal mass needle biopsy confirmed the diagnosis of primary mediastinal choriocarcinoma. Despite aggressive chemotherapy, the patient died 6 months after the initial presentation from neurological complications and multiorgan failure.
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Coriocarcinoma no Gestacional , Coriocarcinoma , Neoplasias del Mediastino , Tronco Encefálico/patología , Coriocarcinoma/tratamiento farmacológico , Coriocarcinoma no Gestacional/tratamiento farmacológico , Coriocarcinoma no Gestacional/secundario , Femenino , Humanos , Masculino , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/tratamiento farmacológico , Mediastino/patología , Persona de Mediana Edad , EmbarazoAsunto(s)
Coriocarcinoma no Gestacional/secundario , Melena/etiología , Neoplasias Gástricas/secundario , Neoplasias Testiculares/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Coriocarcinoma no Gestacional/complicaciones , Coriocarcinoma no Gestacional/terapia , Endoscopía Gastrointestinal , Humanos , Masculino , Orquiectomía , Neoplasias Gástricas/complicaciones , Neoplasias Gástricas/terapia , Neoplasias Testiculares/terapia , Resultado del Tratamiento , Adulto JovenRESUMEN
Extragonadal non-gestational choriocarcinoma is a rare but well-described phenomenon occurring in patients with midline germ cell tumors. Choriocarcinoma (ChC) is an aggressive neoplasm usually developing in women as a rare complication of pregnancy. In male patients ChC occurs in the testes, usually as a component of mixed germ cell tumors. Very few patients develop extragonadal choriocarcinoma with the tumor occurring in midline locations, such as the mediastinum, retroperitoneum, and central nervous system (mostly pineal gland). Non-midline choriocarcinoma can occur in the lung, gastrointestinal tract, and breast, sometimes blended with another primary malignancy. A midline choriocarcinoma manifesting as a head and neck malignancy is exceptional. During an evaluation of multiple enlarged cervical lymph nodes suspected to be lymphoma in a 72-year-old man, a core biopsy was taken from one of the left neck lymph nodes which histologically showed a necrotic malignancy with strong diffuse pancytokeratin staining. After an initial interpretation of metastatic carcinoma, further samples were taken from both tonsils and from a right level 5 neck lymph node. Histologically, all samples contained the same tumor, showing profound pleomorphism and multinucleated syncytial-type giant cells. A panel of immunohistochemistry studies were performed, including ß-human chorionic gonadotropin, with positive findings leading to a diagnosis of extragonadal non-gestational choriocarcinoma.
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Coriocarcinoma no Gestacional/patología , Neoplasias Tonsilares/patología , Anciano , Humanos , MasculinoRESUMEN
Cutaneous metastases of choriocarcinoma are rare. They may indicate poor prognosis and resistance to chemotherapy. In this report, we present a case of a 25-year-old man who presented with central pleuritic chest pain and right upper arm mass for about a week. The patient also had significant weight loss during the last 5 months along with an episode of generalized seizure. Chest computed tomography scan revealed an 8 cm anterior mediastinal mass. A skin punch biopsy from the right upper arm mass revealed a malignant neoplasm with morphology consistent with metastatic choriocarcinoma. Further work-up revealed multiple lung and brain lesions. Ultrasound of the testes revealed no abnormalities. Several chemotherapy regimens were tried; however, there was no response and the disease showed progression. The patient died 6 months after initial presentation.
Asunto(s)
Coriocarcinoma no Gestacional/secundario , Neoplasias del Mediastino/patología , Neoplasias Cutáneas/secundario , Adulto , Resistencia a Antineoplásicos , Resultado Fatal , Humanos , MasculinoRESUMEN
BACKGROUND: Nongestational choriocarcinoma is a rare ovarian malignancy with a prognosis worse than that of gestational choriocarcinoma. Debulking surgery is the primary treatment for ovarian carcinoma. However, fertility preservation is important in young women. CASE: A 15-year-old girl with no sexual experience was admitted for abnormal uterine bleeding. Ultrasonography showed a solid mass in the right ovary and her serum ß-human chorionic gonadotrophin levels were markedly elevated. We performed right oophorectomy, omentectomy, and peritoneal washing cytology. The uterus and left adnexa were preserved. She was diagnosed with nongestational choriocarcinoma, stage IIA. She received adjuvant chemotherapy (etoposide, methotrexate, actinomycin, cyclophosphamide, and oncovin regimen) and has been disease-free for more than 5 years. SUMMARY AND CONCLUSION: Fertility-sparing surgery combined with chemotherapy is an acceptable treatment option for young patients with locally advanced nongestational choriocarcinoma.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Coriocarcinoma no Gestacional/terapia , Procedimientos Quirúrgicos de Citorreducción/métodos , Neoplasias Ováricas/terapia , Adolescente , Quimioterapia Adyuvante , Coriocarcinoma no Gestacional/patología , Femenino , Preservación de la Fertilidad , Humanos , Neoplasias Ováricas/patología , Embarazo , Pronóstico , Resultado del TratamientoRESUMEN
PURPOSE: The objective of this study was to describe and analyze the clinicopathological features of primary choriocarcinoma (PCC) observed in male patients treated at the Samsung Medical Center between 1996 and 2020. MATERIALS AND METHODS: We reviewed the clinical records of 14 male patients with PCC retrospectively to assess their demographic, histological, and clinical characteristics at the time of diagnosis as well as identify the treatment outcomes. RESULTS: The median age of the patients was 33 years. The primary tumor site was the testicles in seven cases (50%), the mediastinum in six cases (43%), and the brain in one case (7%). The most common metastatic site was the lungs (79%), followed by the brain (43%). All patients with PCC received cytotoxic chemotherapy. Twelve patients had records of their response to cytotoxic chemotherapy; of these 12 patients, eight (8/12, 67%) achieved an objective response, and four (4/12, 33%) achieved stable disease response as the best response during chemotherapy. CONCLUSION: It is known that most male PCC patients eventually develop resistance to cytotoxic chemotherapy and die. Factors such as poor response to chemotherapy, high disease burden, brain metastasis, and hemoptysis at the time of diagnosis are associated with shorter survival time in male PCC patients. Programmed death-1/programmed death-ligand 1 blockade therapy can be a salvage treatment for chemotherapy-resistant male PCC patients.
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Neoplasias Encefálicas/diagnóstico , Coriocarcinoma no Gestacional/diagnóstico , Neoplasias Pulmonares/diagnóstico , Neoplasias del Mediastino/diagnóstico , Neoplasias Testiculares/diagnóstico , Adulto , Antígeno B7-H1/antagonistas & inhibidores , Biomarcadores de Tumor/análisis , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Coriocarcinoma no Gestacional/tratamiento farmacológico , Coriocarcinoma no Gestacional/mortalidad , Coriocarcinoma no Gestacional/secundario , Resistencia a Antineoplásicos , Humanos , Inhibidores de Puntos de Control Inmunológico/farmacología , Inhibidores de Puntos de Control Inmunológico/uso terapéutico , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/secundario , Masculino , Neoplasias del Mediastino/tratamiento farmacológico , Neoplasias del Mediastino/mortalidad , Neoplasias del Mediastino/patología , Persona de Mediana Edad , República de Corea/epidemiología , Estudios Retrospectivos , Terapia Recuperativa/métodos , Neoplasias Testiculares/tratamiento farmacológico , Neoplasias Testiculares/mortalidad , Neoplasias Testiculares/patología , Resultado del Tratamiento , Adulto JovenRESUMEN
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.
Asunto(s)
Neoplasias Ováricas/diagnóstico por imagen , Adolescente , Adulto , Anciano , Carcinoma Embrionario/diagnóstico por imagen , Coriocarcinoma no Gestacional/diagnóstico por imagen , Bases de Datos Factuales , Femenino , Humanos , Italia , Persona de Mediana Edad , Neoplasias de Células Germinales y Embrionarias/diagnóstico por imagen , Estudios Retrospectivos , Ultrasonografía , Servicios de Salud para Mujeres , Adulto JovenRESUMEN
Los vómitos son un motivo frecuente de consulta. La mayoría de las veces se deben a enfermedades benignas; sin embargo, pueden ser el síntoma inicial de patologías graves y complejas, como procesos neoplásicos con afectación del sistema nervioso central, donde son el resultado del aumento de la presión intracraneal. El cáncer de testículo representa aproximadamente el 1 % de todas las neoplasias y constituye la neoplasia maligna más frecuente en hombres de entre 15 y 34 años. El coriocarcinoma supone entre el 0,3 y el 1 % de las neoplasias testiculares; es considerado como el más raro y con peor pronóstico de todas debido a su rápida diseminación. La mayoría de los casos debutan con síntomas en relación con las metástasis. Presentamos el caso de un paciente de 16 años que acude a consulta por mareos y vómitos. Presentaba una auscultación pulmonar patológica, por la que se solicitó una radiografía torácica que mostró neumotórax derecho e imagen en suelta de globos. Al ampliar estudio, se objetiva una B-HCG elevada y lesión nodular en la ecografía testicular. En el TAC cerebral se objetivaron lesiones metastásicas responsable de los vómitos. Se realizó biopsia de una lesión ulcerada del cuero cabelludo que informó metástasis de coriocarcinoma testicular
Vomiting is a frequent complaint. Most times it is caused by benign conditions. However, it can also be the initial symptom of serious and complex illnesses, such as neoplastic processes with central nervous system involvement, where vomiting is the result of increased intracranial pressure. Testicular cancer accounts for 1% of all neoplasms, and is the most frequent malignancy in men between 15 and 34 years. Choriocarcinoma accounts for 0.3-1% of testicular neoplasms, and it is considered the rarest of all and the one with the worst prognosis due to rapid spread. Most cases begin with symptoms related to the metastases. We present the case of a 16-year-old patient with dizziness and vomiting. His pulmonary auscultation was pathological, so a chest x-ray was performed, showing right pneumothorax and multiple pulmonary nodules. Further study showed elevated B-HCG and a nodular lesion in testicular ultrasound. Metastatic lesions responsible for the vomiting were found in the brain CT. A biopsy of an ulcerated lesion of the scalp showed metastasis of testicular choriocarcinoma
Asunto(s)
Humanos , Masculino , Adolescente , Neoplasias Testiculares/patología , Neoplasias Encefálicas/secundario , Neumotórax/diagnóstico por imagen , Coriocarcinoma no Gestacional/diagnóstico por imagen , Vómitos/etiología , Metástasis de la Neoplasia/diagnóstico por imagen , Neoplasias Pulmonares/secundario , Metástasis Linfática/patología , OrquiectomíaRESUMEN
Trophoblastic differentiation (including choriocarcinoma) arising in urothelial carcinoma has been described in numerous case reports, but never in a single series. We present a series of these tumors, describing the morphologic spectrum, applying traditional and novel immunohistochemical stains, and characterizing clinical follow-up. We identified 16 cases, arising predominantly in the bladder (N=14), but also the ureter (N=1) and prostatic urethra (N=1). Six of our cases (38%) contained invasive urothelial carcinoma with admixed syncytiotrophoblasts, 8 cases (50%) consisted of invasive urothelial carcinoma with choriocarcinoma, 1 case (6%) showed urothelial carcinoma in situ with associated choriocarcinoma, and 1 case (6%) consisted of pure choriocarcinoma. Other subtypes of variant morphology were seen in 5 of our cases (31%) and included squamous, glandular, lipoid, chordoid/myxoid, and sarcomatoid features. Given the limited specificity of human chorionic gonadotropin immunohistochemistry, we also studied the expression of a novel specific trophoblastic marker, hydroxyl-δ-5-steroid dehydrogenase, as well as Sal-like protein 4. Human chorionic gonadotropin expression was seen in nearly all cases (93%) but was often not limited to the trophoblastic component, staining the urothelial component also in 85% of the cases. Expression of hydroxyl-δ-5-steroid dehydrogenase was more sensitive and more specific, staining 100% of the cases and limited to trophoblasts in all but 1 case. Sal-like protein 4 expression was variable, staining trophoblast in only 50% of cases and staining the urothelial carcinoma component in 43% of those positive cases. Most of our tumors presented at a high stage and were associated with poor clinical outcomes, with at least muscle-invasive disease (pT2) in 10 of the 14 bladder tumors (71%), periureteric fat invasion in the ureter tumor (pT3), distant metastases in 7 of 16 cases (44%) and death of disease in 3 of the 15 patients with follow-up (20%). Our study describes a series of urothelial carcinomas with trophoblastic differentiation, demonstrating the morphologic spectrum of this entity, its frequent association with other subtypes of variant morphology, its characteristic immunoprofile, and its aggressive clinical behavior.