Abstract
Rationale:Primary Extranodal Natural Killer/T-Cell Nasal-Type Lymphoma (ENKTCL) of spine is rarely reported. This case study presents a rare case of ENKTCL originating from the sixth thoracic vertebra.Patient concerns:Here, we present a case of 49-year-old Asian male with chest and back pain. Physical examination revealed the myodynamia of both lower limbs decreased from IV degree to 0 degree, reflexes of both lower limbs and a large area of cutaneous sensation below xiphoid process disappeared in 3 days.Diagnoses:In immunophenotype analysis, CD2, CD3, CD7, and CD68 were positive and CD56 was suspiciously positive. Granzyme B and T-cell intracellular antigen (TIA-1) were also positive and in situ hybridization was positive for Epstein–Barr virus–encoded mRNA (EBER). Ki-67 was 60%+. Nuclide bone scan showed that the nuclide was unusually concentrated in the sixth thoracic vertebra which considered extremely active and slightly concentrated in the right sacrolilac joint. Magnetic resonance imaging detected an abnormal signal in the sixth thoracic vertebra with corresponding paravertebral and intraspinal occupying lesion. Based on the above features, a diagnosis of ENKTCL was made.Interventions:This patient was treated with surgery and symptomatic supportive treatment.Outcomes:The myodynamia of patient's both lower limbs were elevated to I degree after the operation with chest and back pain partly relieved. However, the patient died about 3 months later.Lessons:ENKTCL could originate from spine. Clinicians should be alert for early stage diagnose and distinguish it from some common spinal tumor such as neurofibroma and hemangioma.
Highlights
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL), previously known as lethal midline granuloma is a distinct clinic-pathological entity associated with Epstein–Barr virus that typically causes destruction of the midface, palatal, and orbital walls
Nasal type ENKTCL seems to be caused by EBV, and Epstein–Barr virus– encoded mRNA (EBER) in situ hybridization is the most reliable way to demonstrate the www.md-journal.com presence of EBV, which can be achieved from paraffin-embedded tissues.[3]
This type of lymphoma arises as a consequence of malignant transformation of NK-cells (NKCs) which express markers CD56+, T-cell intracellular antigen-1 (TIA-1), and lack T-cell receptor (TCR) gene rearrangements, the tumor cells typically have the immunophenotype of CD56+, CD2+, and cytoplasmic CD3+.[4,5,6]
Summary
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL), previously known as lethal midline granuloma is a distinct clinic-pathological entity associated with Epstein–Barr virus that typically causes destruction of the midface, palatal, and orbital walls. ENKTCL can involve the skin, soft tissue, testes, gastrointestinal, and upper respiratory tract.[1] To our limited knowledge, ENKTCL arising primarily from the spine without nasal involvement is distinctly rare with only 1 patient clearly documented in the literature.[2]. Diagnosis of such cases may be challenging due to the lower prevalence. Because this kind of non-Hodgkin lymphoma is aggressive, early diagnosis and treatment is crucial. We report a very rare case of ENKTCL that have a primary origin from the sixth thoracic vertebra and describe the details of diagnosis and treatment
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