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:: Leiomyosarcoma (LMS) Defined ::

Leiomyosarcoma (Gr. Leio- =smooth, myo- = muscle, sarcoma = fleshy growth) is a type of sarcoma which is a neoplasm of smooth muscle. (When a uterine neoplasm is benign, it is a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. It is therefore possible for leiomyosarcomas to appear at any site in the body. It is however most commonly found in the stomach, small intestine and retroperitoneum.

Leiomyosarcoma is a very rare cancer. It makes up 5-10% of soft tissue sarcomas, which are in themselves rare cancers.

:: About Leiomyosarcoma ::

Leiomyosarcoma is a form of cancer that affects the smooth muscle of the body. It spreads through the blood stream and can affect the lungs, liver, blood vessels, or any other soft tissue in the body.

LMS is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in the uterus, stomach and intestines, walls of all blood vessels, and skin.

Leiomyosarcoma is a very rare cancer. It makes up 7% of soft tissue sarcomas; in all, LMS affects 4 out of 1,000,000 people.

Presently there is no cure. Remission can be attained, but this rare cancer can reappear at any time. Because of its rarity, few doctors know how to treat it and it attracts very little research.

Saturday, August 29, 2009

Circumferential resection of the inferior vena cava for primary and recurrent malignant tumors - Abstract

27 August 2009

University of South Florida, Tampa, Florida, USA.

Circumferential vena caval resection is occasionally performed in patients with advanced malignancy. We explored the oncological effectiveness of inferior vena caval resection, as determined by margin status, cancer recurrence and survival. Also, we addressed the morbidity associated with inferior vena caval obstruction and resection, and determined indications for inferior vena caval reconstruction.

A total of 18 patients underwent attempted inferior vena caval resection from 1999 to 2008. Primary tumor type was renal cell carcinoma in 7 patients, metastatic testicular cancer in 5, leiomyosarcoma in 3, and adrenal cortical carcinoma, primary retroperitoneal germ cell tumor and upper tract transitional cell carcinoma in 1 each. Data reviewed included preoperative and postoperative sequelae of inferior vena caval obstruction, postoperative complications, pathological results, cancer recurrence, graft requirements and functional outcomes.

Mean followup in the entire patient cohort was 24 months. Inferior vena caval resection was completed in 15 of 18 patients, of whom 12 (80%) had negative surgical margins. Of the patients 50% presented with symptoms of venous hypertension, including lower extremity edema with or without venous thrombosis, or abdominal wall varicosity. After inferior vena caval resection symptoms resolved in half of them, likely due to the ongoing formation of collateral vessels. Five asymptomatic patients with incomplete inferior vena caval occlusion underwent reconstruction with inferior vena caval vascular grafts of polytetrafluoroethylene (4) or Dacron (1). The polytetrafluoroethylene grafts remained patent. A total of 12 patients underwent simultaneous nephrectomy and/or left renal vein ligation in the same setting with acceptable alterations in postoperative renal function and no need for permanent dialysis. Cancer recurred locally in 4 of 15 patients who underwent resection. Five of 15 patients in the resection group died of disease or were lost to followup compared to all 3 in whom resection was aborted or macroscopically incomplete (mean followup 19.2 vs 4.3 months).

Local cancer control and potentially increased cancer specific survival can be achieved with successful complete circumferential resection of the inferior vena cava as a component of multimodality care in select patients with locally advanced malignancy. Polytetrafluoroethylene is the preferred prosthetic material when inferior vena caval replacement is indicated. The most common postoperative complications are renal insufficiency and lower extremity edema, which are generally transient.

Written by:
Caso J, Seigne J, Back M, Spiess PE, Pow-Sang J, Sexton WJ. Are you the author?

Reference:
J Urol. 2009 Sep;182(3):887-93.
doi:10.1016/j.juro.2009.05.015

PubMed Abstract
PMID:19616230

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