Inflammatory myofibroblastic tumour arising in the adrenal gland: a case report
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Inflammatory myofibroblastic tumour arising in the adrenal gland is exceptional. As far as we are aware, there have been only three previous reports in the literature. We report a fourth case.
A 29-year-old Caucasian man presented with upper quadrant pain due to a 15cm heterogenous adrenal mass that displaced his liver. He underwent an open right adrenalectomy. Histopathological examination showed the mass to be an inflammatory myofibroblastic tumour, a histologically distinctive lesion composed of myofibroblasts, plasma cells, lymphocytes and histiocytes. Ten months later he is well with no sign of recurrence.
The lesion was indistinguishable on imaging from an adrenal cortical tumour. Surgical treatment is the same but inflammatory myofibroblastic tumour carries a favourable prognosis.
KeywordsAdrenal gland Histology Immunohistochemistry Inflammatory myofibroblastic tumour
Anaplastic lymphoma kinase
Inflammatory myofibroblastic tumour
Inflammatory myofibroblastic tumour (IMT) is an uncommon spindle-cell proliferation that occurs at various sites including the abdomen, lungs, orbit, gastrointestinal and genitourinary tracts, usually of children and young adults ; however, the overall age range and anatomical distribution is wide and IMTs may arise in almost any location. The adrenal gland is a rare primary site, with only three reports in the world literature [2, 3, 4]. We report a fourth case and describe the radiological and immunohistochemical features.
The term ‘inflammatory myofibroblastic tumour’ was introduced in 1986  to describe a lesion previously known as plasma cell granuloma  or inflammatory pseudotumour . The advent of immunohistochemistry brought increasing awareness of its distinctive features and potential for local recurrence . In recent years, cytogenetic and molecular genetic analysis has confirmed the neoplastic nature of these lesions .
On histological examination, IMTs are characterized by a proliferation of plump fibroblasts or myofibroblasts accompanied by a prominent infiltrate of chronic inflammatory cells, in particular plasma cells. This feature best distinguishes IMT from fibromatosis and fasciitis, with which it overlaps. A rare epithelioid variant of IMT is associated with an abdominal location and an aggressive clinical course .
On immunohistochemical examination, IMTs usually express actin and may also show staining for desmin and keratin. ALK gene rearrangements, resulting in overexpression of ALK protein, are present in 30 to 40% of cases, mainly in the paediatric age group. Immunostaining for ALK is therefore seen only in a minority of cases. Of interest, the IMTs that have metastasized are most often ALK negative .
IMT in the adrenal gland is rare but must be considered in the differential diagnosis of radiologically suspicious adrenal masses. Recurrence or metastasis of adrenal IMT has not been reported but in view of the lesion’s occasional metastatic potential at other sites careful postoperative follow up including repeat imaging at 6 months to exclude local recurrence is advised.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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