Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2014) 34 P185 | DOI: 10.1530/endoabs.34.P185

SFEBES2014 Poster Presentations Neoplasia, cancer and late effects (25 abstracts)

A rare adrenal lieomyosarcome in a subject with lupus and the antiphospholipid syndrome

I N Khan 1 , A Guha 1 , M A Adlan 1 , M Stetchman 2 & L D Premawardhana 1,


1Section of Endocrinology, Ysbyty Ystrad Fawr Hospital, Hengeod, Wales CF82 7EP, UK; 2Section of Endocrine Surgery University Hospital of wales Cardiff, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK; 3Section of Endocrinology, University Hospital of Wales, Cardiff, Wales, UK.


Introduction: Adrenal incidentalomas (AI) are mostly benign and non secretory, but some are malignant. Current diagnostic and management algorithms are not fail safe when assessing malignant potential, although functional status is much easier to assess. We present a subject with multiple comorbidities whose AI was a very rare adrenal leiomyosarcoma (AL)

Case presentation: A 40-year-old woman with SLE and the antiphospholid syndrome on lifelong warfarin therapy, presented with left sided loin pain. She was normotensive and did not have signs of endocrinopathy. Ultrasound scans showed scarring or an angiomyolipoma of the left kidney, but subsequent CT scans showed a 2.7×3.9 cm abnormality in the left adrenal. MRI scans several months later confirmed this lesion, without significant fat and no interval growth. Further investigations confirmed cortisol after 1 mg dexamethasone, urinary free cortisol, urinary catecholamines and metanephrines, aldosterone:renin ratio, random 17-hydroxyprogesterone and chromogranin A and B were all within the reference range. Although, an adrenal bleed was thought likely initially in view of her warfarin therapy and acute presentation, she underwent adrenalectomy because of an increase in size of the lesion between scans. Histology confirmed the typical appearances of a leiomyosarcoma. She had subsequent radiotherapy for localised disease.

Discussion: Our subject presented with a rare AL. We like to highlight the following about her diagnosis and management – i) the initial diagnosis of an adrenal bleed in this warfarinised woman, had to be revised because of interval growth of the mass; ii) the tumour was relatively small compared to typical ALs described in the literature – average size of ~11 cm; and iii) this subject has survived for well over 48 months despite a malignant AL perhaps because of early detection and small localised tumour, resulting from her acute presentation.

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