Epstein–Barr virus‐associated smooth muscle tumors in children following solid organ transplantation: A review

Jossen, Jacqueline; Chu, Jaime; Hotchkiss, Hilary; Wistinghausen, Birte; Iyer, Kishore; Magid, Margret S.; Kamath, Amita; Roayaie, Sasan; Arnon, Ronen

doi:10.1111/petr.12426

Cited by 41 publications

(67 citation statements)

References 38 publications

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“…6 A few cases occurring in the adrenal glands have been reported. 7 In contrast, PT-SMT occurs preferentially in the liver, 8 lungs/ larynx/pharynx, gut, spleen, and kidneys, sometimes in the brain, and rarely in the adrenal gland and the iris of the eye. 9 Very few cases of congenital immunodeficiency disorderassociated SMT have been reported, and lungs/larynx are most frequently affected, followed by intracranial tumors, liver, adrenal, and spleen.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…However, unlike in somatic SMT, where the malignant behavior and clinical outcome are predicted by the histologic features, the behavior of EBV-SMTs does not correlate well with their histologic features and apparent grade of lesion. 6,8 Some of the well-differentiated tumors devoid of mitoses and atypia have proven to be lethal. 3 Thus, it has been proposed that these tumors be designated as SMT of uncertain potential.…”

Section: Histopathologic Featuresmentioning

confidence: 99%

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Epstein-Barr Virus–Associated Smooth Muscle Tumor

Dekate

Chetty²

2016

Archives of Pathology &Amp; Laboratory Medicine

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Immunodeficient individuals are prone to develop a number of opportunistic infections and unique neoplasms. Epstein-Barr virus-associated smooth muscle tumor is an uncommon neoplasm associated with immunodeficiency. It has been described in patients infected with human immunodeficiency virus, in the posttransplant setting, and in those with congenital immunodeficiency. Different anatomic sites can be involved by Epstein-Barr virus-associated smooth muscle tumor, and even multiple locations can contain these unique lesions within the same patient. The presence of variable numbers of intratumoral lymphocytes and primitive round cell areas are the unique defining features for this tumor. Histopathologic features may vary considerably in terms of cellular atypia, mitotic activity, and necrosis, with no correlation to the clinical behavior. Demonstration of Epstein-Barr virus infection by in situ hybridization within tumor cell remains critical for the diagnosis. The mechanism for Epstein-Barr virus infection of progenitor cells and neoplastic transformation has been an area of interest and conjecture. Different treatment strategies are proposed according to underlying disease status. This paper reviews the clinicopathologic features of this uncommon neoplasm with detailed discussion of the role of Epstein-Barr virus in the pathogenesis.

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Section: Clinical Featuresmentioning

confidence: 99%

Section: Histopathologic Featuresmentioning

confidence: 99%

Epstein-Barr Virus–Associated Smooth Muscle Tumor

Dekate

Chetty²

2016

Archives of Pathology &Amp; Laboratory Medicine

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“…EBV involvement using in situ hybridization for EBV-encoded small RNA (EBER) confirmed the diagnosis of EBV-SMT in all reports. Association with PTLD is common, ranging from 38-82% (18,19). Although PTLD was the working diagnosis at the time of appendiceal surgery, to date no PTLD has been identified in our patient.…”

Section: Discussionmentioning

confidence: 84%

“…The lack of prior EBV immunity may additionally contribute to the higher mortality rate in children (18). A review by Jossen et al found a female predominance of EBV-SMTs following solid organ transplantation (21 female patients and only 8 male patients) (19). Most EBV-SMTs are multifocal at the time of diagnosis, with some occurring in sites with little smooth muscle, such as the dura, spleen, tonsils, and glottis (6,20).…”

Section: Discussionmentioning

confidence: 99%

EBV-associated hepatic smooth muscle tumor of uncertain biologic behavior after heart transplantation in a pediatric patient: case report

Liu

Chintalapati

Dietz

et al. 2017

J. Gastrointest. Oncol.

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Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is a rare neoplasm recognized in immunocompromised patients. There are less than 30 cases of EBV-SMT reported in pediatric population following solid organ transplantation. Herein, we report a case of an 8-year-old female who was incidentally noted to have multiple lesions in the liver 8 years after heart transplantation. The tumor was composed of a cellular proliferation of spindle-shaped cells with low mitotic activity. The diagnosis of EBV-SMT was confirmed by in situ hybridization for EBV-encoded small RNA (EBER) transcripts. Multiple additional lesions were detected by whole body positron emission tomography-computed tomography (PET-CT) scan 4 months after the initial finding of the hepatic lesions. Immunosuppression was switched to a mechanistic target of rapamycin (mTOR) inhibitor. We conclude that EBV-SMT should be included in the differential diagnoses in post-transplantation patients and further investigations should be performed to evaluate additional lesions. transplantation. Serial testing for EBV DNA by quantitative polymerase chain reaction (PCR) was performed on peripheral blood starting 3 years after the heart transplant (she presented with cough and fever), which showed amplification with up to 131,600 copies/mL. Her EBV viral load has been consistent since then. She developed acute abdominal pain and an appendiceal perforation was diagnosed 8 years post-transplant. Two liver masses were incidentally identified in the left and right hepatic lobes by abdominal computed tomography (CT) scan. Both appeared hypoechoic and individually measured up to 2.5 cm. Whole body positron emission tomography (PET)-CT scan was performed thereafter with no evidence of lymphadenopathy or other lesions. The mass in the right lobe was excised and found to be well circumscribed, white, and firm on gross examination. The mass in the left lobe was not removed due to its deep location. KeywordsHistologically, the hepatic lesion was well demarcated from the adjacent liver parenchyma, and exhibited interlacing fascicles of spindle cells with eosinophilic cytoplasm and elongated, blunt-ended nuclei with stippled chromatin (Figure 1). Rare (less than 5%) primitive round cell component was identified. There was no evidence of necrosis or hemorrhage. Few small blood vessels were interspersed within the fascicles of spindle cells. Mitotic activity was less than 1 per 10 high power fields (HPF). Occasional lymphoid cells were seen at the junction between the tumor and adjacent liver parenchyma (Figure 2). Immunohistochemical (IHC) stains were performed to categorize the lesion. Smooth muscle actin ( Figure 3A) and HHF 35 ( Figure 3B) were positive, and desmin showed focal and weak equivocal staining consistent with smooth muscle origin. Ki67 showed only 1-2% of proliferative activity. EBV in situ hybridization was positive with a diffuse staining pattern (Figure 4), diagnostic of EBV infection. All these features were consistent with a diagnosis of EBV-SMT.W...

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“…Pediatric transplant recipients, who are usually EBV-naïve and immunosuppressed, are at much higher risk of developing PTLD following an acute EBV infection. However, transplant recipients can also develop other, rarer forms of EBV-associated malignancies, such as smooth muscle tumors (1), which are poorly described in the literature. Here, we present the case of a pediatric liver recipient who developed an acute illness that was initially suspicious for PTLD, until the surprising diagnosis of lymphoepithelial carcinoma (LEC) was made.…”

Section: To the Editorsmentioning

confidence: 99%