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Endocrine Abstracts (2018) 59 CC6 | DOI: 10.1530/endoabs.59.CC6

SFEBES2018 Featured Clinical Cases Featured Clinical Cases (10 abstracts)

What lies beneath: cutaneous Kaposi’s sarcoma as a first manifestation of ectopic ACTH-dependent Cushing’s syndrome

Alberto S Tresoldi 1, , Yasir S Elhassan 1, , Miriam Asia 2 , Mona Elshafie 4 , Peter Lane 5 , Konstantinos N Manolopoulos 1, , Shireen S Velangi 6 , Steven Watkins 7 , Wiebke Arlt 1, & Michael W O’Reilly 1,


1Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK; 2Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK; 3Department of Clinical Sciences and Community Health, Milan, Italy; 4Department of Histopathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK; 5Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK; 6Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK; 7Department of Oncology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.

Introduction: Immune dysregulation is a feature of Cushing’s syndrome (CS). We report a case of CS that presented with rapidly developing cutaneous Kaposi’s sarcoma (KS).

Case description: A previously well 59-year-old heterosexual man presented with a two-month history of proximal muscle weakness, recurrent mouth ulcers, and purplish skin lesions. He had a background history of hypertension. Skin biopsies were compatible with KS. History of past residence in human herpesvirus 8 (HHV-8) endemic countries was confirmed. Blood tests revealed T cell lymphopenia with low CD4+ and CD8+ lymphocytes and normal natural killer and B cells. He underwent investigations in four hospital departments (neurology, dermatology, oncology and immunology) but no underlying predisposing factor was identified. Four months later, centripetal obesity, facial plethora and dorsocervical fat pad were noted, associated with hypokalaemia, which triggered a referral to the endocrine department. Work-up showed a significantly elevated serum cortisol with non-suppressibility after 1 mg overnight dexamethasone (794 nmol/l) and very high level of urinary free cortisol (>3050 nmol/24 h). ACTH dependency was confirmed by increased ACTH (100.3 pg/ml, reference range 7–63), alongside elevated androstenedione and DHEAS. A gadolinium-enhanced pituitary MRI scan showed a 5 mm focal hypointensity, possibly compatible with a pituitary microadenoma. However, there was no ACTH or cortisol response to CRH and also no central:peripheral ACTH gradient before and after CRH on inferior petrosal sinus sampling, consistent with an ectopic ACTH source. Cross-sectional imaging, FDG-PET and 68Ga-DOTATE PET did not reveal any radiological evidence of a putative ACTH-producing lesion. Metyrapone treatment was commenced in advance of a scheduled bilateral adrenalectomy.

Conclusion: KS is an angioproliferative disorder related to HHV-8 infection. Severe immunodeficiency, such as with AIDS or the use of immunosuppressants, predisposes to KS. We propose that CS should be included in the differential diagnosis of immunodeficiency.

Volume 59

Society for Endocrinology BES 2018

Glasgow, UK
19 Nov 2018 - 21 Nov 2018

Society for Endocrinology 

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