Dados do Trabalho

Título

PRIMARY MEDIASTINAL CORIOCARCINOMA IN A MALE CHILD: CASE REPORT

Descrição sucinta do(s) objetivo(s)

To review the clinical history, radiological images and evolution of a case of primary mediastinal choriocarcinoma.
Discussion of mediastinal germ cell tumors: classification and main features; with emphasis on primary mediastinal choriocarcinoma.

História clínica

10-year-old male, who was under study since 7 for pubic hair growth, change in voice tone and gynecomastia. mother reports that 11 days ago he started with kneeling type chest pain and intermittent fever. one week later he has presented a dry cough, tiredness, daily fever, starts to require pillows to sleep and difficulty in speech. goes to emergency where is intubed.

Discussão e diagnóstico

Mediastinal choriocarcinoma without a detectable primary in the gonads or metastasic desease in the retroperitoneal lymph nodes.
Extremely rare, less than 50 cases reported to date. Nearly exclusive of young men.
Associated with Klinefelter Syndrome (10 times more frecuent). Usually asintomatic, in advanced cases presents with the triad of dyspnea, cough and gynecomasti, this last one is related to the production of chorionic gonadotropin; which stimulates Leydig cells to produce testosterone and estrogen. The alpha chain of HCG, which shares an almost identical sequence with TSH, causes hyperplasia of thyroid follicles. The beta chain is a diagnostic biomarker, values ​​above 1000 are consistent with choriocarcinoma.
Mediastinal choriocarcinomas are usually large at the time of diagnosis, with a soft and friable consistency, extensive areas of hemorrhage, and focal necrotic areas.
The images show a large solid mediastinal mass mainly in the prevascular mediastinum and in the posterior mediastinum, with poorly defined margins and poor contrast enhancement. very invasive locally, affecting large vessels, pleura and pericardium. It is usually metastatic at the time of diagnosis, mainly to the lung with the classic cannoball metastasis, liver, kidney, lymph nodes and brain.

Conclusões

Germ cell tumors and most importantly primary mediastinal choriocarcinoma is an infrequent entity, which leads to a late diagnosis and a lack of established treatment.
This, together with its aggressive behavior and frequent presence of metastasis at diagnosis, makes it a highly lethal condition.
Knowledge of their characteristics and suspicion in pediatric patients with hormonal alterations play a fundamental role for a timely treatment and a better prognosis.

Palavras Chave

PRIMARY MEDIASTINAL CORIOCARCINOMA

Arquivos

Área

Pediatria