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Ectopic ACTH syndrome due to thymic carcinoid tumor in a girl
Insights
This case report details an 8-year-old girl with Cushing's syndrome caused by a rare thymic carcinoid tumor. The study highlights the youngest reported patient diagnosed with this condition.
Area of Science:
- Pediatric Endocrinology
- Thoracic Oncology
- Diagnostic Imaging
Background:
- Thymic carcinoid tumors are rare neuroendocrine neoplasms.
- Cushing's syndrome in children can be caused by ectopic hormone production.
- Early diagnosis and surgical intervention are crucial for managing thymic tumors.
Observation:
- An 8-year-old girl presented with a six-month history of cushingoid appearance.
- Hormone studies revealed extremely high serum cortisol and adrenocorticotropic hormone levels.
- Imaging revealed a mediastinal mass, initially undetected by chest X-ray but identified by technetium-99m MIBI scan and MRI.
Findings:
- The patient was diagnosed with a thymic carcinoid tumor.
- Surgical removal of the tumor with partial thymectomy was successful.
- Pathology confirmed the diagnosis of thymic carcinoid.
Implications:
- This case represents the youngest patient reported with a thymic carcinoid tumor.
- Highlights the importance of advanced imaging in diagnosing rare pediatric mediastinal masses.
- Underscores the association between thymic carcinoid tumors and Cushing's syndrome in pediatric populations.
Abstract:
An 8 year-old girl had a cushingoid appearance for six months. Hormone study showed extremely high serum levels of cortisol (> 60 micrograms/dl) and adrenocorticotropic hormone (930 pg/ml). Initial chest X-ray showed nothing unusual, but a technetium-99mm MIBI scan showed an accumulation lesion in the left upper chest cavity. Chest magnetic resonance imaging demonstrated that the mass was in the superior anterior mediastinum. She had complete removal of the tumor with partial thymectomy. The pathology revealed a thymic carcinoid tumor. Carcinoid tumors of the thymus are extremely rare in children and they usually present with Cushing's syndrome. To our knowledge, this is the youngest patient who has ever been reported with this disease.