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Adrenal insufficiency in a child following unilateral excision of a dual-hormone secreting phaeochromocytoma.

Sjoeholm A, Li C, Leem C, Lee A, Stack MP, Hofman PL, Wheeler BJ - Endocrinol Diabetes Metab Case Rep (2015)

Bottom Line: ACTH is the most common hormone co-produced, and is potentially lethal if not diagnosed.Post-operatively the child experienced severe fatigue and was subsequently confirmed to have adrenal insufficiency.He improved markedly with hydrocortisone replacement therapy, which is ongoing 6 months post-operatively.

View Article: PubMed Central - PubMed

Affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.

ABSTRACT

Unlabelled: Phaeochromocytomas are a rare clinical entity, with dual hormone-secreting lesions particularly uncommon, seen in <1%. ACTH is the most common hormone co-produced, and is potentially lethal if not diagnosed. We present the case of a previously well 10-year-old boy, who presented acutely with a hypertensive crisis and was found to have a unilateral, non-syndromic phaeochromocytoma. Medical stabilization of his hypertension was challenging, and took 3 weeks to achieve, before proceeding to unilateral adrenalectomy. Post-operatively the child experienced severe fatigue and was subsequently confirmed to have adrenal insufficiency. He improved markedly with hydrocortisone replacement therapy, which is ongoing 6 months post-operatively. In retrospect this likely represents unrecognized, sub-clinical ACTH-dependent Cushing's syndrome secondary to an ACTH/or precursor dual-hormone secreting phaeochromocytoma. At follow-up, his hypertension had resolved, there was no biochemical evidence of recurrence of the phaeochromocytoma, and genetic analysis was indicative of a sporadic lesion.

Learning points: Dual hormone secreting phaeochromocytomas with ACTH/or a precursor may cause secondary adrenal insufficiency following surgical removal.The concurrent features of Cushing's syndrome can be mild and easily overlooked presenting diagnostic and management pitfalls.As concomitant syndromes of hormone excess are rare in phaeochromocytomas; the diagnosis requires a high index of suspicion.Serial/diurnal cortisol levels, ACTH measurement +/- low dose dexamethasone suppression (when clinically stable, appropriate adrenergic blockade in place, and well supervised), can all be considered as needed.

No MeSH data available.


Related in: MedlinePlus

Coronal T2 weighted MRI of phaeochromocytoma. Arrow indicates tumour.
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fig1: Coronal T2 weighted MRI of phaeochromocytoma. Arrow indicates tumour.

Mentions: An MRI of the abdomen confirmed a 21 mm transverse×19 mm anteroposterior×24 mm cranio-caudal right-sided suprarenal lesion inseparable from the right adrenal gland. The appearances were compatible with a phaeochromocytoma. Nil extension or other intra-abdominal pathology was identified (Fig. 1). Analyses of urine and plasma catecholamines and metanephrines were undertaken. Elevated levels confirming the diagnosis of phaeochromocytoma are as follows (all others not mentioned were within normal ranges): plasma normetanephrine 4744 pmol/l (<900), urine noradrenaline 5100 nmol/day (0–470), urine normetanephrine 11 400 nmol/day (0–2300), and urine vanillyl mandelic acid (VMA) 56 μmol/day (0–32).


Adrenal insufficiency in a child following unilateral excision of a dual-hormone secreting phaeochromocytoma.

Sjoeholm A, Li C, Leem C, Lee A, Stack MP, Hofman PL, Wheeler BJ - Endocrinol Diabetes Metab Case Rep (2015)

Coronal T2 weighted MRI of phaeochromocytoma. Arrow indicates tumour.
© Copyright Policy - license
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4477238&req=5

fig1: Coronal T2 weighted MRI of phaeochromocytoma. Arrow indicates tumour.
Mentions: An MRI of the abdomen confirmed a 21 mm transverse×19 mm anteroposterior×24 mm cranio-caudal right-sided suprarenal lesion inseparable from the right adrenal gland. The appearances were compatible with a phaeochromocytoma. Nil extension or other intra-abdominal pathology was identified (Fig. 1). Analyses of urine and plasma catecholamines and metanephrines were undertaken. Elevated levels confirming the diagnosis of phaeochromocytoma are as follows (all others not mentioned were within normal ranges): plasma normetanephrine 4744 pmol/l (<900), urine noradrenaline 5100 nmol/day (0–470), urine normetanephrine 11 400 nmol/day (0–2300), and urine vanillyl mandelic acid (VMA) 56 μmol/day (0–32).

Bottom Line: ACTH is the most common hormone co-produced, and is potentially lethal if not diagnosed.Post-operatively the child experienced severe fatigue and was subsequently confirmed to have adrenal insufficiency.He improved markedly with hydrocortisone replacement therapy, which is ongoing 6 months post-operatively.

View Article: PubMed Central - PubMed

Affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.

ABSTRACT

Unlabelled: Phaeochromocytomas are a rare clinical entity, with dual hormone-secreting lesions particularly uncommon, seen in <1%. ACTH is the most common hormone co-produced, and is potentially lethal if not diagnosed. We present the case of a previously well 10-year-old boy, who presented acutely with a hypertensive crisis and was found to have a unilateral, non-syndromic phaeochromocytoma. Medical stabilization of his hypertension was challenging, and took 3 weeks to achieve, before proceeding to unilateral adrenalectomy. Post-operatively the child experienced severe fatigue and was subsequently confirmed to have adrenal insufficiency. He improved markedly with hydrocortisone replacement therapy, which is ongoing 6 months post-operatively. In retrospect this likely represents unrecognized, sub-clinical ACTH-dependent Cushing's syndrome secondary to an ACTH/or precursor dual-hormone secreting phaeochromocytoma. At follow-up, his hypertension had resolved, there was no biochemical evidence of recurrence of the phaeochromocytoma, and genetic analysis was indicative of a sporadic lesion.

Learning points: Dual hormone secreting phaeochromocytomas with ACTH/or a precursor may cause secondary adrenal insufficiency following surgical removal.The concurrent features of Cushing's syndrome can be mild and easily overlooked presenting diagnostic and management pitfalls.As concomitant syndromes of hormone excess are rare in phaeochromocytomas; the diagnosis requires a high index of suspicion.Serial/diurnal cortisol levels, ACTH measurement +/- low dose dexamethasone suppression (when clinically stable, appropriate adrenergic blockade in place, and well supervised), can all be considered as needed.

No MeSH data available.


Related in: MedlinePlus