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Periadrenal inflammatory myofibroblastic tumour: half a decade before cure
  1. Rohana Abdul Ghani1,
  2. Fatimah Zaherah Mohamed Shah1,
  3. Mohammad Hanafiah2 and
  4. Mardiana Abdul Aziz3
  1. 1 Endocrine Unit, Department of Medicine, Universiti Teknologi MARA Sungai Buloh Campus, Selangor, Malaysia
  2. 2 Medical Imaging Unit, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh, Malaysia
  3. 3 Anatomic Pathology Unit, Department of Pathology, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh, Malaysia
  1. Correspondence to Dr Rohana Abdul Ghani, agrohana{at}


A 30-year-old ex-smoker with a background history of childhood asthma presented with worsening shortness of breath despite receiving high doses of oral corticosteroid for pemphigus vulgaris which was diagnosed 5 years earlier. A high-resolution CT examination of the thorax reported non-specific bronchiectatic changes and revealed an incidental suprarenal mass. A subsequent CT scan confirmed a large adrenal mass with areas of necrosis and calcification. Serum renin and aldosterone, urinary catecholamine and 5-hydroxyindoleacetic acid were within normal limits. Surgical intervention was delayed due to difficulty in optimising preoperative respiratory functions. He finally underwent a midline laparotomy for removal of the tumour. Histopathological examinations revealed extrapulmonary inflammatory myofibroblastic tumour arising from the periadrenal soft-tissue, with presence of normal adrenal gland. He showed immediate improvements of his asthmatic symptoms and pemphigus vulgaris following the surgery. His oral steroid was rapidly reduced and he achieved complete remission 2 months later.

  • adrenal disorders
  • respiratory system
  • skin
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  • Contributors The authors have provided substantial contributions to the conception or design of this work. RAG and FZMS planned, obtained relevant information and drafted the clinical case write up. MH elaborated on the radiological input including discussion on the role of imaging in the diagnosis. MAA provided the histopathology report and provided the required discussions. All authors revised, criticised and approved the final content. All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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