title: Primary orbital precursor T-cell lymphoblastic lymphoma: Report of a unique case.
abstract: Primary T-cell lymphoblastic lymphoma (T-LBL) in the eye region is very rare. The present study described a unique case of T-LBL involving the extraocular muscles. A 22-year-old male patient presented with a 3-week history of headache, reduced visual acuity and edema of the left eye. Clinical examination revealed left-sided exophthalmus, periorbital edema, chemosis, and reduced motility of the left eye. A magnetic resonance imaging scan revealed thickening of the left orbital muscles and a positron emission tomography-computed tomography scan also demonstrated activity in a subclavicular lymph node. Histopathological analysis of both lesions revealed infiltration by medium-sized neoplastic lymphoid cells with a high nuclear-cytoplasmic ratio and a high mitotic index. Immunostaining revealed positivity for CD2, CD3, CD99, Tia-1, and GranzymB, and variable positivity for CD4. There was no involvement of the bone marrow. Based on the clinical and histopathological findings, a diagnosis of T-LBL was made. There was no evidence ofNOTCH1mutation or rearrangements of theETV6andMLLgenes and high-resolution array-based comparative genomic hybridization (arrayCGH) analysis revealed a normal genomic profile. The patient received chemotherapy according to the high-risk NOPHO protocol, followed by myeloablative allogenic bone marrow transplantation. At 35 months after diagnosis, the patient remained in complete first remission, but without light perception on his left eye. To the best of our knowledge, this is the first report of a case of T-LBL involving the extraocular muscles. Although primary T-LBL in the eye region is very rare, our findings demonstrate that lymphoma should be considered in the differential diagnosis of patients with similar symptoms.
authors.affiliation: Department of Ophthalmology, Rigshospitalet, University of Copenhagen, DK-2100 Copenhagen, Denmark.
authors.forename: Lisa
authors.initials: L
authors.lastname: Stenman
authors.affiliation: Department of Pathology and Genetics, Sahlgrenska Cancer Center, University of Gothenburg, SE-40530 Gothenburg, Sweden.
authors.forename: Marta
authors.initials: M
authors.lastname: Persson
authors.affiliation: Department of Pathology and Genetics, Sahlgrenska University Hospital, SE-41345 Gothenburg, Sweden.
authors.forename: Fredrik
authors.initials: F
authors.lastname: Enlund
authors.affiliation: Department of Pathology, Rigshospitalet, University of Copenhagen, DK-2100 Copenhagen, Denmark.
authors.forename: Erik
authors.initials: E
authors.lastname: Clasen-Linde
authors.affiliation: Department of Pathology and Genetics, Sahlgrenska Cancer Center, University of Gothenburg, SE-40530 Gothenburg, Sweden.
authors.forename: Göran
authors.initials: G
authors.lastname: Stenman
authors.affiliation: Department of Ophthalmology, Rigshospitalet, University of Copenhagen, DK-2100 Copenhagen, Denmark; Department of Pathology, Rigshospitalet, University of Copenhagen, DK-2100 Copenhagen, Denmark.
authors.forename: Steffen
authors.initials: S
authors.lastname: Heegaard
keywords: T-cell lymphoblastic lymphoma; array-based comparative genomic hybridization analysis; orbital manifestation