摘要
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.
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.
作者
Thierry Mukenge Wa Mukengeshay
Fatoumata Binta Balde
Zineb Benmassaoud
Priscilla Kinsala
Othmane Allaoui
Abdelhalim Mahmoudi
Khalid Khattala
Sara Benmilloud
Youssef Bouabdallah
Thierry Mukenge Wa Mukengeshay;Fatoumata Binta Balde;Zineb Benmassaoud;Priscilla Kinsala;Othmane Allaoui;Abdelhalim Mahmoudi;Khalid Khattala;Sara Benmilloud;Youssef Bouabdallah(Service de Chirurgie Pédiatrique, Centre Hospitalier et Universitaire Hassan II de Fès, Fès, Morocco;Service de Pédiatrie, Unité d’Oncologie, Centre Hospitalier et Universitaire Hassan II de Fès, Fès, Morocco;Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdallah de Fès, Fès, Morocco)