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Adult orbital precursor B‐lymphoblastic lymphoma with involvement of the extraocular muscles
Author(s) -
Mikkelsen L.H.,
Ejstrup R.,
ClasenLinde E.,
Andersen M.K.,
Gjerdrum M.L.R.,
Heegaard S.
Publication year - 2017
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2017.04146
Subject(s) - medicine , ptosis , extraocular muscles , pathology , biopsy , lymphoblastic lymphoma , exophthalmos , anatomy , radiology , surgery , immune system , t cell , immunology
Purpose To report a very rare case of a precursor‐B Lymphoblastic lymphoma (pre‐B‐ LBL ) with periocular muscle involvement in an adult. The clinical, histopathological, and genetic findings will be reported. Methods Case report, Immunohistochemistry, FISH , and Array CGH . Results A 56‐year‐old male experienced sudden onset of left sided ptosis and diplopia. Pain was not prominent. The patient had complete remission of an abdominal pre‐B‐ LBL . On examination, proptosis and a left sided third cranial nerve palsy was found. After a few days, mydriasis without rapid afferent pupillary defect developed. MRI showed thickening of the medial and inferior rectus muscles. A biopsy of the inferior rectus muscle was obtained for histopathological examination. Histologically, an infiltrate of immature lymphocytic tumour cells was seen within the orbital skeletal muscle tissue. The tumour cells were small and presented with fine blastic nuclear chromatin along with small peripheral nucleoli. The tumour cells stained positively for CD 10, CD 79a, PAX 5, and BCL ‐2. The Ki‐67 proliferation index was 90%. These findings were consistent with a TdT‐negative pre‐B‐ LBL relapse. FISH was negative for BCR / ABL , MLL ‐rearrangement, and ETV ‐6‐break. Array CGH was performed. A bone marrow biopsy confirmed systemic involvement. The patient was immediately started on NOPHO High Risk chemotherapy, followed by orbital radiation and awaits allogeneic bone marrow transplantation. The patient has no light perception in his left eye. Conclusions This is the first reported case of a very rare rapid growing orbital pre‐B‐ LBL in an adult.