TY - JOUR
T1 - Abdominal lymphangioleiomyomatosis in a man with Klinefelter syndrome
T2 - The first reported case
AU - Fiore, Maria Grazia
AU - Sanguedolce, Francesca
AU - Lolli, Ivan
AU - Piscitelli, Domenico
AU - Ricco, Rosalia
PY - 2005/4
Y1 - 2005/4
N2 - Lymphangioleiomyomatosis (LAM) is an uncommon progressive disease characterized by a hamartomatous tumor-like proliferation of smooth muscle cells that occurs most often in women. This disease commonly involves the lymph nodes, lungs, and mediastinum, and rarely the abdominal sites. We report a case of mesenteric LAM occurring in a 37-year-old man affected by Klinefelter syndrome with a 17-year history of androgen replacement therapy. Histology revealed a hamartomatous proliferation of spindle cells surrounding multiple ectatic lymphatic spaces intermixed with lymphatic follicles. When subjected to immunohistochemical studies, the tumor cells stained positive for muscular markers and negative for estrogen, progesterone, and androgen receptors. The occurrence of LAM in association with Klinefelter syndrome has never been reported in the literature and could represent a further clue in the still unclear pathogenesis of this disease.
AB - Lymphangioleiomyomatosis (LAM) is an uncommon progressive disease characterized by a hamartomatous tumor-like proliferation of smooth muscle cells that occurs most often in women. This disease commonly involves the lymph nodes, lungs, and mediastinum, and rarely the abdominal sites. We report a case of mesenteric LAM occurring in a 37-year-old man affected by Klinefelter syndrome with a 17-year history of androgen replacement therapy. Histology revealed a hamartomatous proliferation of spindle cells surrounding multiple ectatic lymphatic spaces intermixed with lymphatic follicles. When subjected to immunohistochemical studies, the tumor cells stained positive for muscular markers and negative for estrogen, progesterone, and androgen receptors. The occurrence of LAM in association with Klinefelter syndrome has never been reported in the literature and could represent a further clue in the still unclear pathogenesis of this disease.
KW - Androgen therapy
KW - Klinefelter syndrome
KW - Mesenteric lymphangioleiomyomatosis
KW - Sex hormones
UR - http://www.scopus.com/inward/record.url?scp=16244379804&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=16244379804&partnerID=8YFLogxK
U2 - 10.1016/j.anndiagpath.2004.12.007
DO - 10.1016/j.anndiagpath.2004.12.007
M3 - Article
C2 - 15806517
AN - SCOPUS:16244379804
VL - 9
SP - 96
EP - 100
JO - Annals of Diagnostic Pathology
JF - Annals of Diagnostic Pathology
SN - 1092-9134
IS - 2
ER -