Smooth muscle: Intravenous leiomyomatosis

2003-07-01   Paola Dal Cin 

1.Department of Pathology, Brigham, Womens Hospital, 75 Francis Street, Boston, MA 02115, USA

Clinics and Pathology

Etiology

Intravenous leiomyomatosis (IVL) is a rare smooth muscle proliferation of special interest because of its quasi-malignant behavior.

Clinics

IVL is a smooth muscle proliferation that arises from the uterus with vermiform extensions in some cases into the inferior vena cava and ultimately into the right heart.

Pathology

The neoplastic smooth muscle of intravenous leiomyomatosis resembles that of benign uterine leiomyomata and may include the same histological variants found in the uterus. Furthermore, the neoplastic smooth cells of IVL express estrogen and progesterone receptors and tumor growth appears to respond to hormonal manipulation.

Treatment

Although benign, its hemodynamic effects may require invasive extirpation.

Cytogenetics

Cytogenetics morphological

There are only two cytogenetic reports in IVL and both exhibited a karyotype with a der(14)t(12;14)(q15;q24) and two normal copies of chromosome 12.
Fluorescence in situ hybridization revealed three copies of HMGA2 (a.k.a. HMGIC): two signals were detected on the normal chromosomes 12 at 12q15, as well as another on the der(14) in the breakpoint region, suggesting that the 12q breakpoint occurred 5 (centromeric) to HMGA2, as has been commonly observed and previously described in uterine leiomyoma with a t(12;14). Based on these findings, it has been suggested that the pathogenesis of IVL is more similar to that of typical uterine leiomyoma than it is to leiomyosarcoma.

Bibliography

Pubmed IDLast YearTitleAuthors
32022471988Intravenous leiomyomatosis of the uterus. A clinicopathological analysis of 16 cases with unusual histologic features.Clement PB et al
125082492003Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24).Dal Cin P et al
119043482002Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case.Quade BJ et al
126491982003Fusion transcripts involving HMGA2 are not a common molecular mechanism in uterine leiomyomata with rearrangements in 12q15.Quade BJ et al

External Links

Citation

Paola Dal Cin

Smooth muscle: Intravenous leiomyomatosis

Atlas Genet Cytogenet Oncol Haematol. 2003-07-01

Online version: http://atlasgeneticsoncology.org/solid-tumor/5158/smooth-muscle-intravenous-leiomyomatosis