Abstract
To the Editor:
Epstein-Barr virus (EBV), a member of the herpes virus family, is one of the most prevalent viruses globally that is associated with nasopharyngeal carcinoma, lymphoid malignancies, and gastric carcinomas [1]. Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is rare and reported in immunosuppressed patients such as post-transplant patients, patients with acquired immunodeficiency syndrome (AIDS), and patients with congenital immunodeficiencies [2]. Epstein-Barr virus-associated smooth muscle tumor predominantly occurs in young adult males and can affect many organ systems, such as the central nervous systems (CNS), liver, lung, and genitourinary systems [3]. The disease can be multifocal. We report a young male patient with AIDS managed with radiofrequency ablation (RFA) for EBV-SMT of the liver.
A 27-year-old homosexual male presented with a five month history of watery diarrhea, dysphagia, and weight loss. He was diagnosed with human immunodeficiency virus (HIV) infection, fulfilling the criteria for AIDS with a CD4 count <20 per milliliter and an HIV viral load of 398,000 copies/mL. Combination antiretroviral treatment (abacavir, dolutegravir, lamivudine) was started. He developed opportunistic infections, including cytomegalovirus retinitis and uveitis with substantial visual impairment, ocular syphilis, oral and esophageal candidiasis, and chlamydial disease. He was treated with antivirals, anti-fungal, and antibiotic agents with a good response. A computed tomography (CT) scan of the neck and thorax was performed for neck abscess. On the CT scan, an incidental liver lesion was identified, and subsequent liver magnetic resonance imaging (MRI) scan showed a 24-mm solitary segment 6 liver lesion with arterial phase hyperenhancement and delayed phase washout. Hepatitis B and C screening were negative. The liver function test and alpha-fetoprotein were normal.
A multidisciplinary team (MDT) suggested a liver biopsy with the intent to rule out infectious etiology. The histology revealed EBV-SMT. The patient was counseled for surgical resection or RFA as possible therapeutic options, and he chose RFA. The RFA of the segment 6 liver lesion was uneventful, and he is well at six months of follow-up.
Epstein-Barr virus-associated smooth muscle tumors are rare neoplasms, with a prevalence of 0.6%–5%, and are usually found in immunosuppressed individuals such as patients with AIDS and post-transplant patients [4]. Epstein-Barr virus triggers the smooth muscle transformation by activating the Akt/mammalian target of the rapamycin (mTOR) signaling pathway [5]. Notably, all the case reports of EBV-SMT include young male patients, consistent with our report. Patients with long-standing HIV-associated immunosuppression may be at risk of EBV-SMT. However, our patient was recently diagnosed with HIV. This could be because of a latent period from HIV infection to the development of symptoms and eventual diagnosis. The clinical presentation of EBV-SMT of the liver is variable, ranging from asymptomatic (similar to our patient) to localized or systemic symptoms. In our patient, it was crucial to exclude primary neoplasms of the liver while considering AIDS-defining cancers, including Kaposi sarcoma and non-Hodgkin lymphoma, thus histology confirmation was advocated by MDT. For liver lesions <3 cm, RFA is curative and avoids surgical risks or the need for organ donation. Because of the paucity of evidence and the rarity of EBV-SMT in the liver, a clinician must report any cases.