Case Reports
doi: 10.3389/fped.2021.680113.
eCollection 2021.
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Case Reports
Front Pediatr.
.
Abstract
Herein, we reported a rare case of Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) combined with juvenile idiopathic arthritis (JIA) in a 6-year old girl without HIV, organ transplantation, or congenital immunodeficiency. The patient suffered from pain in the bilateral hip joints, which drastically affected her physical activity. Consequently, she was diagnosed with JIA (September 2019). She was given methotrexate and methylprednisolone pills via oral route and a subcutaneous injection of Recombinant Human Tumor Necrosis Factor-α Receptor II;lgG Fc Fusion Protein for 4 weeks that successfully relieved the pain. In May 2020, the pain reoccurred and was accompanied by occasional headaches. After extensive pathological examination, the patient was diagnosed with EBV-SMT. The imaging examinations after admission showed multiple lesions in the skull, lungs, and vertebral body. Biopsy of the L2 vertebral body was then performed to clarify the diagnosis. Finally, the in-situ hybridization of the tumor of the lumbar vertebrae suggested a non-HIV/transplantation-related EBV-SMT. Consequently, the patient received surgery without chemotherapy and radiotherapy, after which her conditions improved.
Keywords:
children; epstein barr virus; hip joint; juvenile idiopathic arthritis; smooth muscle tumor.
Copyright © 2021 Kang, Xu and Li.
Conflict of interest statement
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Figures
Figure 1
Diagram of the disease progression.
Figure 2
Imaging examination. (A) Plain and enhanced MRI scanning of the knee joints and hip joints showed that the synovium of bilateral knee and hip joints were slightly thickened and enhanced, with a small amount of effusion in joints. (B) Plain CT scanning of lungs showed multiple nodules and stripe-shaped shadows in bilateral lungs, and the size of the largest shadow was 0.63 × 0.74 cm. (C) Plain and enhanced MRI scanning of the skull showed nodular lesions at the parasellar and meninges of the right parietal lobe. (D) Plain and enhanced MRI scanning of the spine showed bone mass destruction of the L2 vertebral body and left vertebral pedicle.
Figure 3
Pathological examination of the biopsy of lesion tissues of the L2 vertebral body. (A) The bone tissue were invaded by tumor cells (HE × 100). (B) The tumor cells was spindle-shaped, with abundant cytoplasm, red stain and mild atypia, while no evident nuclear fission was observed (HE × 200). (C) The actin were expressed in the cytoplasm of tumor cells (IHC × 400). (D) The highest ki-67 positive index was 6% (IHC × 400). (E) The h-Caldesmon were expressed diffusely in the cytoplasm of tumor cells (IHC × 400). (F) SMA were expressed diffusely in the cytoplasm of tumor cells (IHC × 400). (G) Epstein-Barr virus encoded RNA situ hybridization showed diffusely positive (EBER × 400).
References