AIDS-Related EBV-Associated Smooth Muscle Tumors:  A Review of 64 Published Cases

Purgina, Bibianna; Rao, Uma; Miettinen, Markku; Pantanowitz, Liron

doi:10.4061/2011/561548

Cited by 98 publications

(161 citation statements)

References 55 publications

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“…82 Although these tumors were originally divided into EBV-associated leiomyomas and leiomyosarcomas, current classification holds them all collectively as smooth muscle tumors as they are usually indolent although somewhat unpredictable in behavior. True metastatic behavior is rare, and was observed only in 4 of 51 AIDS-associated (8%) 77 and 1 of 19 cases (5%) in a mixed AIDS and transplantation-associated series. 85 Based on a large survey of AIDS-associated cases, mitotic rate does not seem to distinguish cases with malignant outcome.…”

Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 98%

“…The smooth muscle tumors can occur in peripheral soft tissue, intracranial space, or visceral sites, and tumor multiplicity is common. [76][77][78][79][80][81][82][83][84][85] Molecular genetic analysis has shown that multiple tumor represent independent clones rather than dissemination of a single neoplastic clone. 85 In multiple tumor cases, tumors arising in the transplant are of donor cell origin, whereas those at the other sites arise from recipient cells, which also points to polyclonal origin of multiple tumors.…”

Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 99%

“…85 Based on a large survey of AIDS-associated cases, mitotic rate does not seem to distinguish cases with malignant outcome. 77 Histologically, the EBV-associated smooth muscle tumors have a spectrum from a well-differentiated conventional-appearing spindle cell smooth muscle tumors to those composed of ovoid cells with incomplete smooth muscle differentiation ( Figure 12). Intratumoral T lymphocytes may be present and even be prominent, but this is not a uniform feature.…”

Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 99%

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Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis

Miettinen

2014

Modern Pathology

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Smooth muscle tumors are here considered an essentially dichotomous group composed of benign leiomyomas and malignant leiomyosarcomas. Soft tissue smooth muscle tumors with both atypia and mitotic activity are generally diagnosed leiomyosarcomas acknowledging potential for metastasis. However, lesions exist that cannot be comfortably placed in either category, and in such cases the designation 'smooth muscle tumor of uncertain biologic potential' is appropriate. The use of this category is often necessary with limited sampling, such as needle core biopsies. Benign smooth muscle tumors include smooth muscle hamartoma and angioleiomyoma. A specific category of leiomyomas are estrogen-receptor positive ones in women. These are similar to uterine leiomyomas and can occur anywhere in the abdomen and abdominal wall. Leiomyosarcomas can occur at any site, although are more frequent in the retroperitoneum and proximal extremities. They are recognized by likeness to smooth muscle cells but can undergo pleomorphic evolution ('dedifferentiation'). Presence of smooth muscle actin is nearly uniform and desmin-positivity usual. This and the lack of KIT expression separate leiomyosarcoma from GIST, an important problem in abdominal soft tissues. EBVassociated smooth muscle tumors are a specific subcategory occurring in AIDS or post-transplant patients. These tumors can have incomplete smooth muscle differentiation but show nuclear EBER as a diagnostic feature. In contrast to many other soft tissue tumors, genetics of smooth muscle tumors are poorly understood and such diagnostic testing is not yet generally applicable in this histogenetic group. Leiomyosarcomas are known to be genetically complex, often showing 'chaotic' karyotypes including aneuploidy or polyploidy, and no recurrent tumor-specific translocations have been detected.

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Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 98%

Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 99%

Section: Ebv-associated Smooth Muscle Tumorsmentioning

confidence: 99%

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Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis

Miettinen

2014

Modern Pathology

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“…A similar mechanism was proposed for EBV-SMT, except CD21 receptors are not detected on smooth muscle cells. Fusion of smooth muscle cells with an infected B-lymphocyte prior to tumor proliferation has therefore been postulated (12). EBV has two strains (types 1 and 2) with different biological behavior, which are distinguished by polymorphisms in EBNA genes.…”

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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“…Recientemente se ha descrito el rol de este virus en la etiología de múltiples tumores de musculo liso que ocurren en pacientes jóvenes con SIDA, en varias localizaciones del cuerpo (23) . El comportamiento de estos tumores no es siempre agresivo, aunque pueden llegar a ser muy sintomáticos dependiendo de su localización, en especial cuando afectan el sistema nervioso central.…”

Section: Virus Epstein Barrunclassified

Virus como inductores de neoplasias cutáneas

Puccio

2014

Rev Peru Med Exp Salud Publica

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El rol oncogénico de los virus en las neoplasias cutáneas es conocido por el hombre desde hace más de un siglo, cuando se atribuía el origen de la verruga vulgar al virus papiloma humano (VPH). En la actualidad, las neoplasias inducidas por virus pueden agruparse en tumores sólidos y procesos linfoproliferativos. Destacan entre los primeros el VPH, del cual ahora conocemos numerosos serotipos, cada uno vinculado a una neoplasia específica, el herpesvirus humano tipo 8 que produce el sarcoma de Kaposi y el poliomavirus vinculado al carcinoma de Merkel. Entre los procesos linfoproliferativos debemos mencionar al virus linfotrópico de células T humanas tipo 1 (HTLV-1) responsable de los linfomas de células T, en los cuales el compromiso cutáneo es inespecífico, con un amplio espectro de presentaciones clínicas y, que por consiguiente, plantean un reto para el diagnóstico diferencial. En este grupo también se encuentra el virus Epstein Barr vinculado a los linfomas nasales de Células NK/T y a los linfomas tipo Hidroa, de reciente descripción. En esta era en la que lo genético y lo molecular priman en las investigaciones en cáncer, no podemos dejar de lado el concepto de neoplasia como resultado de la infección por un agente viral, lo que abre una nueva veta de posibilidades de tratamiento anticanceroso basado en medicamentos antivirales. Palabras clave: Papillomaviridae; Poliomavirus; Carcinoma de células escamosas; Carcinoma de células de Merkel; Virus linfotrópico de células T humanas tipo 1; Linfoma extranodal de células NK-T (fuente: DeCS BIREME). VIRUSES AS AGENTS INDUCING CUTANEOUS NEOPLASMS ABSTRACTThe oncogenic role of viruses in cutaneous neoplasms has been known by humankind for more than a century, when the origin of the common wart, or verruca vulgaris, was attributed to the human papilloma virus (HPV). Currently, virusinduced cutaneous neoplasms may be grouped into solid tumors and lymphoproliferative disorders. HPV, from which various serotypes are now known, each being linked to a specific neoplasm, the human herpes virus type 8 producing Kaposi sarcoma, and the Merkel cell polyomavirus, highlight among the first group. Regarding the lymphoproliferative disorders, we should mention the human T-lymphotropic virus type I (HTLV-1), which is responsible for the T-cell lymphomas, in which the cutaneous manifestations are non-specific and have a wide spectrum, thus posing a challenge for differential diagnosis. The Epstein Barr virus, linked to nasal lymphomas of NK/T-cells and Hydroa-like cutaneous lymphomas, is also part of this group. In an era in which the genetic and molecular aspects of cancer research prevail, we may not leave behind the concept of neoplasms as a result an infection with a viral agent, which opens a wide array of new possibilities for cancer treatment based on antiviral drugs.

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AIDS-Related EBV-Associated Smooth Muscle Tumors: A Review of 64 Published Cases

Cited by 98 publications

References 55 publications

Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis

Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis

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

Virus como inductores de neoplasias cutáneas

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