<b>Uterine Leiomyosarcoma & Inherited Susceptibility</b> 

<b>Two medical journal abstracts.</b> 


compiled by doctordee

Proc Natl Acad Sci U S A 2001 Mar 13;98(6):3387-3392

<b>Inherited susceptibility to uterine leiomyomas and renal cell cancer.</b> Launonen V, Vierimaa O, Kiuru M, Isola J, Roth S, Pukkala E, Sistonen P, Herva R Aaltonen LA.

Department of Medical Genetics, Haartman Institute, University of Helsinki, P. O. Box 21, FIN-00014, Helsinki, Finland; Department of Clinical Genetics, Oulu University Hospital, Kajaanintie 50, FIN-90220, Oulu, Finland; Institute of Medical Technology, University of Tampere, FIN-33014, Tampere, Finland; Finnish Cancer Registry, Institute for Statistical and Epidemiological Cancer Research, Finnish Cancer Registry, FIN-00170, Helsinki, Finland; Finnish Red Cross Blood Transfusion Service, Kivihaantie 7, FIN-00310, Helsinki, Finland; and Department of Pathology, Oulu University Hospital, Kajaanintie 52, FIN-90220, Oulu, Finland.

Herein we report the clinical, histopathological, and molecular features of a cancer syndrome with predisposition to uterine leiomyomas and papillary renal cell carcinoma. The studied kindred included 11 family members with uterine leiomyomas and <b>two with uterine leiomyosarcoma.</b> Seven individuals had a history of cutaneous nodules, two of which were confirmed to be cutaneous leiomyomatosis. The four kidney cancer cases occurred in young (33- to 48-year-old) females and displayed a unique natural history. All these kidney cancers displayed a distinct papillary histology and presented as unilateral solitary lesions that had metastasized at the time of diagnosis. Genetic-marker analysis mapped the predisposition gene to chromosome 1q. Losses of the normal chromosome 1q were observed in tumors that had occurred in the kindred, including a uterine leiomyoma. Moreover, the observed histological features were used as a tool to diagnose a second kindred displaying the phenotype. <b>We have shown that predisposition to uterine leiomyomas and papillary renal cell cancercan be inherited dominantly through the hereditary leiomyomatosis and renal cell cancer (HLRCC) gene.</b> The HLRCC gene maps to chromosome 1q and is likely to be a tumor suppressor. Clinical, histopathological, and molecular tools are now available for accurate detection and diagnosis of this cancer syndrome. 
&&url PMID: 11248088



Int J Gynaecol Obstet 1988 Oct;27(2):289-91

<b>Recurrent uterine myomata in three sisters--an uncommon occurrence.</b> Kulenthran A, Sivanesaratnam V. Department Obstetrics and Gynaecology, University Hospital, Kuala Lumpur,Malaysia.
Three sisters who developed recurrent uterine myomata from a very young age are presented. Despite repeated attempts at myomectomy, all three cases had hysterectomies ultimately. Complications encountered during surgery were severe hemorrhage, inadvertent injury to bladder and bowel in two patients and a rare complication of colonic-uteric-cutaneous fistula occurring post-operatively in one patient. <b>Histology of the final hysterectomy specimens in two cases showed low grade leiomyosarcoma and cell ular myoma, respectively.</b> 
&&url PMID: 2903099
