An Case of Malignant Phaeochromocytoma with Negtive Urinary Catecholamines at Presentation

Presentation Number: SAT 389
Date of Presentation: April 1st, 2017

Recie Davern*1 and Siobhán Estelle McQuaid2
1Mater Miscericordiae University Hospital, Dublin, IRELAND, 2Mater Miscericordiae University Hospital, Drumree, Ireland

Abstract

Patient Characteristics: 63 year old female with ten years follow up of 18 cm malignant phaeochromocytoma with urinary catecholamine and genetic screening to date all negative. Therefore a case of a non-functioning malignant phaeochromocytoma.

History of presenting illness: At the age of 53 years old, she presented to the gynaecological outpatient clinic with two weeks of left upper abdomen fullness, fatigue and weight loss. No reported history of headache, hypertension or sweating.

Past Medical/Surgical History: Right lobe thyroidectomy in 1983 for cosmetic reasons. Dilation and curettage procedure for investigation of infertility

Social/Family History: She was a non-smoker and had minimal C2H5OH intake. There was no family history of neuroendocrine tumours.

Medications (+ allergies): Eltroxin 100mcg OD and no drug allergies

Physical Examination: She was not tachycardic or hypertensive. Only positive findings were previous partial thyroidectomy scar and splenomegaly.

Report of laboratory and imaging studies: 

Summary of Relevant investigations

  • CT TAP - 18cm adrenal mass with calcification at the centre appearing to arise from the left adrenal gland. No evidence of lymphovascular invasion or distal metastatis
  • MRI Abdomen - 18cm left adrenal mass with left kidney pushed inferiomedially by the mass. Lesion encapsulated with no local extension. Suggestive of adrenal carcinoma
  • 24 hour urinary catecholamine collection - adrenalin 24 nmol/24 hours (0-230nmol/24housr), noradrenalin 160nmol/24 hours (0-290nmol/24 hours) and VMA 9.6 umol/24 hours (2.5-40 umol/24 hours)

Interventions and outcome: Left adrenalectomy with nephrectomy 2006. No intra-operative hypertension or complications. Histology confirmed phaeochromocytoma with malignant potential. It showed tumour composed of epithelioid appearing cells with abundant pink cytoplasm. Individual cells showed severe nuclear pleomorphism including numerous giant forms with prominent nucleoli. Occasional mitotic figures are identified. In addition, there is extensive necrosis. There is evidence of definite lymphovascular space invasion and capsule invasion. A rim of normal residual adrenal gland is identified. These appearances favor a malignant pheochromocytoma.The submitted kidney unremarkable. Immunochemistry testing confirmed neuroendocrine histogenesis/pheochromocytoma. MIB 1 proliferation index of 30-40%. is extremely high and strongly supports a diagnosis of malignancy Subsequent follow up over ten years has revealed negative MIBG scans, CT TAPs, urinary and plasma catecholamines and genetic screening tests. This case raises the issues of management and radiological and biochemical follow up of non functioning and malignant phaeochromocytomas and the role of genetic screening for such tumours. It also highlights the advances in diagnostics that have occurred over the last decade.

 

Nothing to Disclose: RD, SEM