Kh. Vanhoeven et al., VISCERAL MYOGENIC TUMORS - A MANIFESTATION OF HIV-INFECTION IN CHILDREN, The American journal of surgical pathology, 17(11), 1993, pp. 1176-1181
We report a primary smooth-muscle tumor of undetermined malignant pote
ntial of the liver in a child with acquired immune deficiency syndrome
(AIDS). This patient represents the eighth child infected with the hu
man immunodeficiency virus who developed a mesenchymal tumor other tha
n Kaposi's sarcoma. All these children were younger than 10 years of a
ge. These tumors often were histologically or clinically malignant and
all but one were smooth-muscle tumors. These tumors arose exclusively
in visceral organs, and the hepatobiliary, gastrointestinal, and trac
heopulmonary systems were involved. Transmission of the virus occurred
both vertically (in six children) and via blood transfusion (in two).
Given the rarity of smooth-muscle tumors in uninfected children, the
unusual frequency of these tumors suggests that immunosuppression indu
ced by the virus permits the unregulated proliferation of a primitive
mesenchymal cell disposed to myogeneous differentiation, a situation n
ot unlike that observed in the development of AIDS-related Kaposi's sa
rcoma in adults.