Cushing Syndrome in an Infant Due to Cortisol Secreting Adrenal Pheochromocytoma: A Rare Association

Kumar, M. Ravi; Kumar, V. Ravi; Talukdar, B; Mohta, Anup; Khurana, Nita

doi:10.1515/jpem.2010.102

Cited by 22 publications

(11 citation statements)

References 9 publications

(11 reference statements)

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“…There are reports in literature on the concurrent CS and pheochromocytoma [3,4], sarcoidosis [5], pancreatic acinar cell carcinoma [6], pre-eclamptic findings [7], malignant gastrinoma [8], bronchial carcinoid lung tumor [9], pancreatic neuroendocrine tumor [10], Hippel-Lindau disease [10], or mesenteric neuroendocrine carcinoma [11]. Our patients had nothing of these associations.…”

Section: Discussionmentioning

confidence: 63%

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Domi¹

2011

J Endocrinol Metab

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Cushing syndrome is a multietiologic clinical situation, resulting in several features like obesity, hyperglycemia, hypertension, proximal muscle weakness, skin thinning, menstrual irregularities, buffalo hump, and purple striae. In perioperative period the anesthesiologist must deal with difficult ventilation and intubation, hemodynamic disturbances, volume overload and hypokalemia, glucose intolerance and diabetes, maintaining the blood cortisol level and preventing the glucocorticoid deficiency. This syndrome is quite rare and its features make these patients very difficult to the anaesthesiologist.

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Section: Discussionmentioning

confidence: 63%

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Domi¹

2011

J Endocrinol Metab

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“…Suppression of the HPA axis from this ectopic source of ACTH, or ACTH-precursor, likely resulted in the subsequent post-operative presentation of symptomatic adrenal insufficiency. To date, only two paediatric cases have been reported in the English literature with this occurrence, a 15-year-old girl (4) and an 11-month-old boy (5). These cases presented predominantly with symptoms of Cushing's syndrome and were only found to have phaeochromocytomas on histology.…”

Section: Discussionmentioning

confidence: 99%

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

Sjoeholm

Leem

et al. 2015

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryPhaeochromocytomas 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.

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“…These are reported in literature and include conditions like pheochromocytoma,[34] sarcoidosis,[5] pancreatic acinar cell carcinoma,[6] pre-eclamptic findings,[7] malignant gastrinoma,[8] bronchial carcinoid lung tumor,[9] pancreatic neuroendocrinetumor,[10] Hippel-Lindau disease,[10] and mesenteric neuroendocrine carcinoma. [11]…”

Section: Discussionmentioning

confidence: 99%

Cushing syndrome and the anesthesiologist, two case reports

Domi

Sula

2011

Indian J Endocr Metab

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Cushing's syndrome (CS) is associated with reduced life quality and increased mortality, mostly due to cardiovascular disease. The features of this syndrome are central obesity, moon facies, facial plethora, supraclavicular fat pads, buffalo hump, and purple striae. Other complications include hyperglycemia, hypertension, proximal muscle weakness, skin thinning, menstrual irregularities, amenorrhea and osteopenia. These make perioperative and anesthetic management difficult and present a challenge to the operating team, especially the anaesthesiologist. In this paper, we present two such cases of CS, which were treated with adrenalectomy. We aim to highlight the special care and precautions that need to be taken while administering anesthesia, and in the post operatory period. Anaesthesia induction in the two cases of CS was done prior to the adrenalectomy procedure and special pre and post operative care was taken. Continuous intra operative monitoring of vitals and checking for the stability of the haemodynamics was performed. With adequate care and using advanced anesthetic techniques, the patients showed uneventful post operative recovery. Though the anesthetic management of patients with CS is difficult, desired results can be achieved with continuous monitoring and special precautions.

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Cushing Syndrome in an Infant Due to Cortisol Secreting Adrenal Pheochromocytoma: A Rare Association

Cited by 22 publications

References 9 publications

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

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

Cushing syndrome and the anesthesiologist, two case reports

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