Medical Care
The National Comprehensive Cancer Network (NCCN) workup for intra-abdominal sarcomas includes evaluation and management, prior to initiation of therapy, by a multidisciplinary team with expertise and experience in sarcoma. A pretreatment chest/abdominal/pelvic computed tomography (CT) scan with contrast, with or without magnetic resonance imaging (MRI), is also indicated.
Preresection biopsy is not necessarily required, based on the degree of suspicion of other malignancies. Biopsy is required for patients receiving preoperative radiation therapy (RT) or chemotherapy. For resectable disease, surgery with or without intraoperative RT or preoperative therapy with RT and chemotherapy are options.
Chemotherapy and radiation have shown only limited benefit in the treatment of leiomyosarcomas. Response rates to various chemotherapeutic regiments generally have been below 40%.
Surgical Care
Resection of the tumor is the only hope for cure. Remove the associated lymph nodes, but extended lymphadenectomy is not necessary because these tumors rarely metastasize to lymph nodes (see Pathophysiology). [15]
Per the National Comprehensive Cancer Network (NCCN), for resectable disease, surgery with or without intraoperative radiation therapy (RT) or preoperative therapy with RT and chemotherapy are options. For unresectable disease, attempt downstaging of the tumor with chemotherapy or RT and then resect. If it is still unresectable or progressive disease, consider palliative chemotherapy, palliative RT, palliative surgery for symptom control, observation if asymptomatic, and always consider resection of resectable metastatic disease if the primary tumor can be controlled.
Postoperative treatment with RT or reresection, if technically feasible, may be options depending on the surgical outcomes. With R0 disease (negative margins), consider postoperative RT in highly selected patients. With R1 disease (positive margins), consider postoperative RT if no preoperative RT was given or consider a boost (10-16 Gy) if preoperative RT was given. For R2 disease (macroscopic incomplete resection), consider reresection if technically feasible or follow the course for unresectable disease as described above.
Consultations
A gastroenterologist will likely be involved in most cases of leiomyosarcoma, because affected patients generally present with gastrointestinal (GI) bleeding. Endoscopic ultrasonography may be of some benefit for diagnosing the more proximal tumors. Also, if ulceration occurs, performing a biopsy may be possible.
A surgeon must be involved to provide the definitive treatment.
A hematologist/oncologist may be able to provide insights into the prognosis, define the grade of the tumor and offer possible chemotherapy.
A radiation oncologist may be able to provide insight into possible preoperative, intraoperative, and/or postoperative RT.
Long-Term Monitoring
Local and systemic recurrence is a real possibility, and even a probability, in many cases of leiomyosarcoma. Closely monitor the patient for such a recurrence, but specific guidelines for follow-up are lacking because of the relatively rare nature of this tumor.
Perform regularly scheduled computed tomography (CT) scans and, as appropriate, endoscopic examinations together with blood work (blood counts and liver profile). The authors suggest a follow-up CT scan of the abdomen and endoscopy, if possible, at 3 and 6 months after surgery. This is followed with yearly screening.
The National Comprehensive Cancer Network (NCCN) recommends physical examination with imaging (abdomen/pelvic CT) every 3-6 months for 2-3 years, then every 6 months for the next 2 years, and then annually.
Stool should be screened for occult blood with the same frequency.
Any abdominal complaint should be evaluated aggressively.
A chest radiograph should be performed with each screening, together with a blood count.
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Colonic mucosa with gastrointestinal stromal tumor (GIST) involving the adjacent submucosa (hematoxylin and eosin [H&E] stain, medium power).
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Clusters of tumor cells separated by a hyaline and mucin-rich stroma (hematoxylin and eosin [H&E] stain, medium power).
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Oval- to spindle-shaped cells forming a fascicle (hematoxylin and eosin [H&E] stain, high power).
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CD-34 stain showing a tumor (medium power). CD-34 is a myeloid progenitor cell antigen.
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High-power magnification with CD-34 antigen immunohistochemical stain showing membrane positivity of the tumor cells.
