Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency

Shaw, Rachel; Issekutz, Andrew C.; Fraser, Robert; Schmit, Pierre; Morash, Barb; Monaco-Shawver, Linda; Orange, Jordan S.; Fernandez, Conrad V.

doi:10.1182/blood-2011-10-385377

Cited by 53 publications

(61 citation statements)

References 18 publications

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“…[1][2][3] NK cell function is regulated by integrating activating and inhibitory signals from engaged NK cell receptors. 4 The NK cell receptor repertoire in mice includes the Ly49, NKG2D, CD94/ NKG2, and NKR-P1 families of receptors, all of which are encoded by genes in the NK gene complex (NKC) on chromosome 6.…”

Section: Introductionmentioning

confidence: 99%

The mouse NKR-P1B:Clr-b recognition system is a negative regulator of innate immune responses

Rahim

Chen

Mottashed

et al. 2015

Blood

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Section: Introductionmentioning

confidence: 99%

The mouse NKR-P1B:Clr-b recognition system is a negative regulator of innate immune responses

Rahim

Chen

Mottashed

et al. 2015

Blood

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“…EBV-positive smooth muscle tumours (SMT) are not specific for transplant patients but are associated with any patients on long-term immunosuppression. Similar tumours can manifest in patients who suffer from human immunodeficiency virus (HIV) infection (HIV-SMT) or congenital immunodeficiency syndromes (CI-SMT) [3,4,5,6,7,8,9,10,11]. Among HIV patients and patients with rare congenital defects, the exact frequency of tumour manifestation is not known, but is most likely <5%.…”

Section: Pathogenesis Risk Factors and Molecular Pathologymentioning

confidence: 99%

“…There is no gender-specific risk increase regarding any of the three tumour types [2,3,4,5,6,7,8,9,10,11]. In transplant patients, the type of immunosuppressive drug, the transplant organ and the manifestation of PTLD are not associated with PTSMT manifestation [2].…”

Section: Pathogenesis Risk Factors and Molecular Pathologymentioning

confidence: 99%

“…Only a few CI-SMT cases have been described (all children) and in these cases at least, no association with a particular type of immune defect was evident, i.e. immunodeficiencies predisposing to severe EBV-associated complications like X-linked lymphoproliferative disease or CD27 deficiency do not seem to have an impact on CI-SMT development [6,7,8,9,10,11]. Furthermore, some patients with congenital immunodeficiency syndromes have undergone bone marrow transplantation and have then developed EBV-positive SMT (overlap between PTSMT and CI-SMT) [2,12,13].…”

Section: Pathogenesis Risk Factors and Molecular Pathologymentioning

confidence: 99%

“…in the cerebral sinus, the tumour founder cell is thought to be derived from an aberrant myogenous venous wall cell [2,14]. Almost all PTSMT and CI-SMT are EBV-positive while up to 30% of HIV-SMT are EBV-negative (therefore, in these latter HIV-SMT cases, the proliferation cannot be linked to EBV) [2,3,4,5,6,7,8,9,10,11]. In lymphoid diseases, EBV infects B cells by using C3d/EBV receptor CD21 [1].…”

Section: Pathogenesis Risk Factors and Molecular Pathologymentioning

confidence: 99%

See 2 more Smart Citations

Epstein-Barr Virus-Associated Smooth Muscle Tumours after Transplantation, Infection with Human Immunodeficiency Virus and Congenital Immunodeficiency Syndromes

Hussein¹,

Maecker‐Kolhoff

Donnerstag

et al. 2013

Pathobiology

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Smooth muscle tumours (SMT) after transplantation (PTSMT) or associated with congenital immunodeficiency syndromes (CI-SMT) and human immunodeficiency virus (HIV-SMT) are rare. The majority of PTSMT and CI-SMT are associated with Epstein-Barr virus (EBV), while some HIV-SMT can be EBV-negative. SMT in immunodeficient states may present with unspecific symptoms which are mainly related to tumour localisation. In PTSMT, >50% of tumours manifest in the liver/transplant liver, but in general PTSMT, HIV-SMT and CI-SMT can occur at any site as single or multiple tumours. Multiple tumour manifestations do not define metastatic disease as PTSMT can occur synchronously and/or metachronously. PTSMT can originate from the recipient as well as from the donor. Morphologically, most tumours, in particular PTSMT, lack marked histological atypia or tumour necrosis, while some HIV-SMT and CI-SMT can present as sarcoma-like variants, but histomorphology does not predict clinical aggressiveness or tumourbiological behaviour. In PTSMT, surgery and reduced immunosuppression show comparable overall survival rates, while poor prognosis is mainly associated with intracranial manifestation and non-resectable tumours. In HIV-SMT and CI-SMT, surgery should be performed. In all 3 tumour types, adverse prognosis is mainly related to comorbidities associated with immunosuppression but not with the extent of histological atypia or tumour size.

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Imaging findings in children with proliferative disorders following multivisceral transplantation

et al. 2015

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Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency

Cited by 53 publications

References 18 publications

The mouse NKR-P1B:Clr-b recognition system is a negative regulator of innate immune responses

The mouse NKR-P1B:Clr-b recognition system is a negative regulator of innate immune responses

Epstein-Barr Virus-Associated Smooth Muscle Tumours after Transplantation, Infection with Human Immunodeficiency Virus and Congenital Immunodeficiency Syndromes

Imaging findings in children with proliferative disorders following multivisceral transplantation

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