Extra-uterine myoid tumours in patients with acquired immunodeficiency syndrome: a clinicopathological reappraisal

Abstract

While the reappraised spectrum of AIDS-MTs does not demonstrate divergent subtype-determined clinical behaviour, heightened awareness/recognition of this expanded spectrum will not only promote improved diagnosis of pleomorphic and myopericytic variants, which may be the sentinel clue to AIDS and its comorbidity, but will also facilitate distinction from histopathological mimics in specific anatomic locations.

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

“…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%.…”

“…Among PTSMT patients, 38% are children and the tumour occurs significantly earlier in juvenile patients [2]. In HIV-SMT, <40% of patients are children [3,4,5]. In these juvenile cases, maternofetal HIV infection, infection after birth or duration of HIV infection cannot be linked to tumour manifestation [3,4,5].…”

“…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].…”

“…pain and organ dysfunction) and depends on the localisation and size of the tumour mass [2,3,4,5,6,7,8,9,10,11,16]. Most PTSMT, HIV-SMT and CI-SMT are <5 cm diameter but can be as big as 15 cm [18], 14 cm [5] or 21 cm [11], respectively.…”

“…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].…”