Bilateral smooth-muscle tumors of the adrenals in a child with AIDS

Abstract

We report a case of bilateral smooth muscle tumors (SMT) involving the adrenal glands in an 11-year-old female with acquired immunodeficiency syndrome (AIDS). The SMT of the right adrenal gland extended into the inferior vena cava, producing a tumor thrombus.

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

“…Bilateral adrenal involvement has only been reported in one case of an 11-year-old girl with HIV. 6 In light of the present observations, it is worth noting that NK cell functional defects are known in HIV/AIDS, and it is possible that a unifying theme in susceptibility to EBV-SMT can be impaired NK cell defense. 17 Treatment of EBV-SMTs in patients with HIV/AIDS has included tumor resection, chemotherapy, and radiation therapy, although the optimal management is unknown.…”

“…We speculate that the severe deficiency of NK cells in our patient, despite normal cytotoxic CD8 ϩ T cells, was sufficient to compromise the initial immune response, therefore contributing to the development of an EBV-SMT as seen in HIV, organ transplant, and congenital T-cell deficiency patients. [3][4][5][6][7][8]12 The NK cell deficiency appeared to be quantitative because the few cells present were phenotypically normal and low-level activity could be detected after IL-2 stimulation. It has been shown that EBV transformation can increase levels of indoleamine 2,3-dioxygenase in some cells, leading to down-modulation of NKG2D from NK cells.…”

“…Adrenal gland involvement in EBV-SMTs is surprising given the limited smooth muscle present. 6 Nevertheless, 8 cases of unilateral adrenal EBV-SMTs in patients with HIV or AIDS have been documented since 1995, 7,15,16 although only 2 involved children. Bilateral adrenal involvement has only been reported in one case of an 11-year-old girl with HIV.…”

“…EBV has never been linked to an isolated NK cell deficiency. [3][4][5][6] Classic NK deficiency is defined as a deficiency in NK cell numbers and function with normal NKT cell (CD56 ϩ /CD3 ϩ ) numbers in the presence of otherwise intact immunity. 1 We report, to our knowledge, the first case of classic NK deficiency discovered in the presence of bilateral EBV ϩ adrenal SMTs.…”

Adrenal Leiomyoma Mimicking Adrenal Malignancy: Diagnostic Challenges and Review of Literature

“…Leiomyoma arising from adrenals are extremely rare with less than 20 cases reported in medical literature, to the best of our knowledge [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Adrenal leiomyomas are clinically asymptomatic, hence they usually present as incidentalomas [3,16,17].…”