<i>Edited PDQ Statement on Adult Soft Tissue Sarcomas, Health Professional's Edition. [ [ ] indicate compiler's additional comments.]</i>

	<b>[The comments on treatment below, are generalized comments on treatments of SARCOMAS in general, NOT leiomyosarcoma in particular.  This is a VERY important point, because some sarcomas are curable by radiation or chemotherapy, while leiomyosarcoma is usually not cured by these techniques. doctordee] 

	[The treatments for LMS do differ from other sarcomas--For instance--there is NO advantage in survival time, in stage IV leiomyosarcoma, to using adriamycin and dacarbazine together.  They can be used separately and sequentially for the same survival advantage, and MUCH fewer side effects.  Generally, sequential treatment with single chemotherapy agents, used until further tumor growth occurs, can judiciously extend survival time in inoperable stage IV LMS.  MAID is no longer frequently used.  AIM [adriamycin, ifosfamide, mesna] is sometimes used to get control of aggressive cascading disease, or when a quick response is necessary for a life threatening metastasis [eg blocking off a main bronchus in the lung. doctordee]

	[It is for the above reason that it is important to have your treatment overseen by a sarcoma oncologist.  	"Sarcoma oncologists" are not members of a formal sub-subspecialty, but are oncologists with a special interest in sarcomas, often working at a sarcoma center. A sarcoma oncologist will probably see around 100 sarcoma patients a year.  Sarcoma oncologists will make sure that your tumor slides are double-checked by a sarcoma pathologist. Survival times are better at sarcoma centers.  See General Approach to Management Page, and references there. doctordee]
	
	[Even excellent medical facilities and cancer centers may lack a sarcoma oncologist. If you do not live near a sarcoma oncologist, find a local oncologist willing to work closely with the sarcoma oncologist. And sarcoma oncologists can do telephone consultations to local oncologists abroad as well as within the United States. doctordee] </b>

back to the PDQ statement....
 
The prognosis [outcome] for patients with adult soft tissue sarcomas depends on several factors, including the patient's age and the size, histologic grade[determined by the pathologist], and stage of the tumor [determined by tests, surgery, and grade of the tumor]. 

Patients older than 60 years of age, tumors larger than 5 centimeters, or high-grade tumor histology are associated with a poorer prognosis 

While low-grade tumors are usually curable by surgery alone, higher grade sarcomas (as determined by the mitotic index and the presence of hemorrhage and necrosis) are associated with higher local treatment failure rates and increased metastatic potential [tendency]. 

Patients with high-grade tumors (grades 3 or 4) greater than 5 centimeters in diameter have the greatest tendency for disease to metastasize and are eligible for prospective clinical trials of adjuvant chemotherapy. 

With distant metastases (stage IV), surgery with curative intent is possible for patients selected for optimal underlying biologic behavior (i.e., patients with a limited number of metastases, with a long disease-free interval, and with slow clinical growth) with pulmonary metastases who have undergone or are undergoing complete resection of the primary tumor. 

Doxorubicin alone or with dacarbazine is considered the best chemotherapeutic regimen for advanced sarcoma. A randomized trial of 340 patients with advanced sarcoma showed a higher response rate (32% versus 17%, p<.002) and longer time-to-progression (6 versus 4 months, p<.02) for doxorubicin, dacarbazine, ifosfamide, and mesna (MAID) versus doxorubicin and dacarbazine alone. The increased response rate of the MAID regimen may be justified in preoperative management of younger patients with borderline resectability, but the increased toxic effects argue against its use in older patients. 

Complete surgical resection is often difficult for sarcomas of the retroperitoneum due to large size before detection and anatomic location. Prospective randomized trials have not shown improved survival with preoperative or postoperative adjuvant chemotherapy for this subgroup. 

<b>[HOW STAGING IS DONE]</b> 

<b>Cellular Classification</b> 
Soft tissue sarcomas are classified histologically according to the soft tissue cell of origin [e.g. leiomyosarcoma, GIST,] although the cell type is not part of the staging system. Additional studies, including electron microscopy, histochemistry, flow cytometry, cytogenetics, and tissue culture studies, may allow identification of particular subtypes within the major histologic categories. The histologic grade reflects the metastatic potential of these tumors more accurately than the classic cellular classification. The histologic grade is also called the tumor grade. 

<b>[Tumor Grade]</b>
Pathologists assign tumor grade based on the number of mitoses per high-powered field, presence of necrosis, cellular and nuclear morphology, and the degree of cellularity; discordance among expert pathologists can reach 40% on prospective review. [It is recommended that the slides be reviewed by a pathologist skilled in sarcomas.] 

<b>Stage Information</b>
Staging has an important role in determining the most effective treatment of soft tissue sarcomas. The stage is determined by the size of the tumor, the histologic grade, and whether it has spread to lymph nodes or distant sites. 

Intracompartmental or extracompartmental extension of extremity sarcomas is also important for surgical decision making. For complete staging, a thorough physical examination, x-rays, laboratory studies, and careful review of all biopsy specimens (including those from the primary tumor, lymph nodes, or other suspicious lesions) are essential. Computed tomographic scan of the chest is recommended for sarcomas greater than 5 centimeters (T2) or with moderate to poor differentiation (grades 2-4). 

The American Joint Committee on Cancer (AJCC) has designated staging by the four criteria of tumor size, nodal status, grade, and metastasis (TNGM).2 

<b>Grade and TNM definitions</b>