title: Cardiac presentation of ALK positive anaplastic large cell lymphoma.
abstract: Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We report the case of an immunocompetent 29-year-old male who presented with syncope and arrythmias secondary to a ventricular cardiac mass. Transcutaneous cardiac biopsy was non-diagnostic, therefore an open cardiac biopsy was performed from which a provisional diagnosis of a cardiac inflammatory pseudotumour was made. Six months after presentation, he developed several subcutaneous lesions with systemic symptoms. Histological and immunophenotypic review of the initial cardiac biopsy revealed features consistent with a diagnosis of CD30, ALK1 positive anaplastic large cell lymphoma (ALCL). Despite intensive treatment with combination chemotherapy, there was significant progression of disease, and he died 11 months after diagnosis. The overall prognosis of cardiac lymphoma remains poor, which may be due to the often late presentation of the tumour. To our knowledge, this is the first reported case of a cardiac ALK positive ALCL. Although rare, cardiac presentation of ALCL should be added to the list of differential diagnoses of cardiac lymphomas.
authors.affiliation: Department of Haematological Medicine, Kings College Hospital, London, UK. ziyi.lim@kingsch.nhs.uk
authors.forename: Z Y
authors.initials: ZY
authors.lastname: Lim
authors.forename: R
authors.initials: R
authors.lastname: Grace
authors.forename: J R
authors.initials: JR
authors.lastname: Salisbury
authors.forename: D
authors.initials: D
authors.lastname: Creamer
authors.forename: A
authors.initials: A
authors.lastname: Jayaprakasam
authors.forename: A Y L
authors.initials: AY
authors.lastname: Ho
authors.forename: S
authors.initials: S
authors.lastname: Devereux
authors.forename: G J
authors.initials: GJ
authors.lastname: Mufti
authors.forename: A
authors.initials: A
authors.lastname: Pagliuca
mesh_terms: D030201:Activin Receptors, Type I / Q000378:metabolism; D030301:Activin Receptors, Type II; D000328:Adult; D001706:Biopsy / Q000379:methods; D006338:Heart Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000556:secondary; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D017728:Lymphoma, Large-Cell, Anaplastic / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D008297:Male
chemical_list: C425364:ACVRL1 protein, human; D030201:Activin Receptors, Type I; D030301:Activin Receptors, Type II