Spontaneous Remission of Phaeochromocytoma | Spontaneous Remission Database

Abstract

Adrenal cortical carcinoma is an uncommon neoplasm in children. Only a handful of congenital adrenal cortical carcinoma cases have been described. A newborn who had metastatic adrenal cortical carcinoma (skin metastases and cerebral lesions) is described. This patient underwent surgical resection of the right adrenal primary, but no further treatment was given. Hemihypertrophy developed in this patient by 2 months of age, and at 4 months of age spontaneous regression of all skin nodules and central nervous system (CNS) lesions was observed. Follow-up at 1 year shows the patient to be alive, well, and disease-free. Evaluation of the tumor included DNA ploidy analysis that showed the tumor to be polyploid, a pattern recently associated with nonmetastasizing adrenal cortical neoplasm. The observation of apparent metastatic disease that regressed spontaneously highlights the prognostic value of DNA ploidy analysis and raises the possibility of an adrenal tumor with properties similar to those of Stage IV-S neuroblastoma.

Case Details

Personal Characteristics

A 20-year-old maori woman

Clinical Characteristics

2-year history of personality change with sudden episodes of anger between periods of normal temperament, exophthalmos, a blood pressure of 170/120 mmhg and papilloedema

Remission Characteristics

Reversal of cardiac failure, return of heart size to normal, return of the catecholamines to normal, the blood pressure to normal and the calcification of the adrenal lesion on CT scan

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Treatment was initiated with atenolol and prazosin with a good response in her blood pressure

Additional Notes

The patient was pregnant during the course of the disease. She refused surgical treatment. Postpartum mild hypertension developed, this settling spontaneously during observation as an outpatient to 130/90 mmHg. Her most recent urinary catecholamines are normal, and a CT scan shows a calcified lesion of 1.6 x 1.9 centimeters within the left adrenal.