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Malignant pituitary chromophobe adenoma in an infant accompanying diabetes insipidus
Insights
This report details a rare case of malignant pituitary chromophobe adenoma in a 1.5-year-old infant, presenting with diabetes insipidus and viral pneumonia. This youngest reported case highlights unusual tumor behavior and endocrine dysfunction.
Area of Science:
- Pediatric Endocrinology
- Neuro-oncology
- Pathology
Background:
- Malignant pituitary chromophobe adenomas are rare, particularly in infants.
- Diabetes insipidus is an uncommon manifestation of chromophobe adenomas.
Observation:
- A 1.5-year-old male infant presented with symptoms of polydipsia, polyuria, viral pneumonia, and seizures.
- Autopsy revealed a malignant pituitary chromophobe adenoma with extensive invasion.
- The tumor infiltrated the third ventricle, surrounding brain areas, optic nerve vicinity, and subarachnoid space.
Findings:
- This represents the youngest reported case of malignant chromophobe adenoma.
- The tumor's extensive invasion and association with diabetes insipidus are notable.
- The proposed etiology involves malignant transformation of primitive chromophobe cells in the posterior pituitary.
Implications:
- This case expands the understanding of rare pediatric pituitary tumors.
- It underscores the potential for unusual endocrine and neurological manifestations in pediatric oncology.
- Highlights the importance of early diagnosis and comprehensive evaluation in infant tumor cases.
Abstract:
This report deals with a 1.5-year-old male infant terminating in viral pneumonia and "diabetes insipidus". The autopsy revealed malignant pituitary chromophobe adenoma, invading the wall of the third ventricle, extending to the periventricular-, dorsomedial- and ventromedial area, skipping to the lateral area adjacent to the optic nerve, and permeating into the subarachnoid space of the cerebrum. At the onset of disease polydypsia and polyuria were marked followed by interstitial pneumonia with high fever, and later generalized tonic convulsion terminated in death. This case is peculiar in at least two respects. Firstly, this is the youngest reported case of malignant chromophobe adenoma. Secondly, the manifestation of diabetes insipidus is rare in chromophobe adenoma. The malignant adenoma probably occurred from the primitive gland rest of chromophobe cells in the posterior lobe becoming malignant during the course of growth development and destroyed the cells of the posterior lobe, resulting in diabetes insipidus.