Introduction: This study aims to describe the outcome of adrenocortical cancer in children through observation. Observation: A 10-year-old girl with no previous pathological history. She presented headaches and severe...Introduction: This study aims to describe the outcome of adrenocortical cancer in children through observation. Observation: A 10-year-old girl with no previous pathological history. She presented headaches and severe hypertension with clinical and biological signs of hyperandrogenism for 6 months. An abdominal CT scan showed an encapsulated left adrenal mass without local or regional invasion or secondary location. A pheochromocytoma or adrenal neuroblastoma was first suspected. The blood pressure was stable at 130/65 mmHg under antihypertensive drugs. She underwent a complete tumor resection without any intraoperative incident. The pathologic study confirmed the adrenocortical carcinoma scored Weiss 7. The severe high blood pressure reappeared 2-year later despite antihypertensive drugs. The thoracic abdominal and pelvic CT scan showed a locally advanced tumor recurrence in the left adrenal gland with parenchymal nodes in the lungs and liver. The outcome was fatal despite medical and surgical management. Conclusion: Adrenocortical cancer is a rare tumor. It is important to hormonal testing in the presence of Cushing’s syndrome in children. It can give a strong indication of the diagnostic possibilities. Histology confirms the diagnosis. The evolution is covered by complications, in particular recurrence with life-threatening metastases.展开更多
文摘Introduction: This study aims to describe the outcome of adrenocortical cancer in children through observation. Observation: A 10-year-old girl with no previous pathological history. She presented headaches and severe hypertension with clinical and biological signs of hyperandrogenism for 6 months. An abdominal CT scan showed an encapsulated left adrenal mass without local or regional invasion or secondary location. A pheochromocytoma or adrenal neuroblastoma was first suspected. The blood pressure was stable at 130/65 mmHg under antihypertensive drugs. She underwent a complete tumor resection without any intraoperative incident. The pathologic study confirmed the adrenocortical carcinoma scored Weiss 7. The severe high blood pressure reappeared 2-year later despite antihypertensive drugs. The thoracic abdominal and pelvic CT scan showed a locally advanced tumor recurrence in the left adrenal gland with parenchymal nodes in the lungs and liver. The outcome was fatal despite medical and surgical management. Conclusion: Adrenocortical cancer is a rare tumor. It is important to hormonal testing in the presence of Cushing’s syndrome in children. It can give a strong indication of the diagnostic possibilities. Histology confirms the diagnosis. The evolution is covered by complications, in particular recurrence with life-threatening metastases.
