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Diagnosis and Treatment > Signs and Symptoms

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Stephanie Teasdale
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Elham Reda Endocrinology, Gold Coast University Hospital, Southport, Queensland, Australia

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Summary

We present two cases of adrenal phaeochromocytoma in patients with a previous diagnosis of neurofibromatosis type 1 (NF1). One had an adrenergic phenotype. The other had a more noradrenergic phenotype. Both had large primary tumours, which increases the likelihood of malignancy. Both also had elevated plasma-free methoxytyramine, which has been linked with malignancy even in non-SDHB phaeochromocytomas.

Learning points

  • Phaeochromocytoma can have varied clinical presentations.

  • Methoxytyramine can be useful in the biochemical work-up of both SDHB-positive and SDHB-negative phaeochromocytoma.

  • The utility of methoxytyramine as a marker of malignancy in NF1-related phaeochromocytoma is unclear, and cases with elevated titres warrant longer follow-up.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Tumours and neoplasia, Condition/ Syndrome, Ischaemic heart disease, Neurofibromatosis, Phaeochromocytoma, Takotsubo cardiomyopathy, Patient Demographics, Age, Adult, Gender, Female, Male, Ethnicity, White, Country of Treatment, Australia, Diagnosis and Treatment, Signs and Symptoms, Abdominal lump, Cardiomyopathy, Chest pain, Dizziness, Headache, Hypertension, Neurofibromas, Palpitations, Sweating, Tachycardia, Weight loss, Investigation, Catecholamines (plasma), CT scan, Dopamine, Echocardiogram, Electrocardiogram, Histopathology, Liver function, Metanephrines (plasma), Methoxytyramine, MIBG scan, MRI, Troponin, Ultrasound scan, Medication, Alpha-blockers, Beta-blockers, Digoxin, Diltiazem, Labetalol, Metoprolol, Phenoxybenzamine, Prazosin, Related Disciplines, Cardiology, Surgery, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-15-0059
Volume/Issue:
Volume 2015: Issue 1
Open access
Durgesh Gowda Departments of Diabetes and Endocrinology

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Vasant Shenoy Departments of Diabetes and Endocrinology

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Usman Malabu Departments of Diabetes and Endocrinology

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Donald Cameron Surgery, The Townsville Hospital, Townsville, Queensland, Australia

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Kunwarjit Sangla Departments of Diabetes and Endocrinology

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Summary

Our patient had drainage of a large amoebic liver abscess. This got complicated by a severe degree of hypotension, which required aggressive fluid resuscitation and hydrocortisone support. Computerised tomography (CT) of the abdomen revealed bilateral adrenal gland haemorrhage (BAH) resulting in primary adrenal gland failure, which was the cause for hypotension. Patient was on long-term warfarin for provoked deep vein thrombosis of lower limb, which was discontinued before the procedure. Thrombophilia profile indicated the presence of lupus anticoagulant factor with prolonged activated partial thromboplastin time (aPTT). Patient was discharged on lifelong warfarin. This case emphasises the need for strong clinical suspicion for diagnosing BAH, rare but life-threatening condition, and its association with amoebic liver abscess and anti-phospholipid antibody syndrome (APLS).

Learning points

  • Recognition of BAH as a rare complication of sepsis.

  • APLS can rarely cause BAH.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Condition/ Syndrome, Abscess, Anti-phospholipid antibody syndrome, Waterhouse-Friderichsen syndrome, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, White, Country of Treatment, Australia, Diagnosis and Treatment, Signs and Symptoms, Abdominal pain, Chills, Diarrhoea, Hyponatraemia, Hypotension, Pyrexia, Sweating, Tachycardia, Weight loss, Investigation, Activated partial thromboplastin time, Aldosterone (blood), Anticardiolipin antibody, Apolipoprotein H, Cortisol (serum), CT scan, Intervention, Abscess drainage, Medication, Fludrocortisone, Glucocorticoids, Hydrocortisone, Mineralocorticoids, Warfarin, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-14-0058
Volume/Issue:
Volume 2014: Issue 1
Open access