CLMSs are extremely rare neoplasms, and most of them have been described as case reports. In the past, LMS of the colon’s prognosis has been generally considered to be a benign tumor that displayed optimism with a low propensity for recurrence and distant metastasis. [1, 28] Later on, literature reported that frequent recurrences and distant metastasis have been observed in the CLMS.[29] Due to the paucity of data about CLMS, the information regarding its clinical characteristics and specific treatment was still unclear. Based on prior studies, we identified influencing risk factors for PFS and OS after surgical treatment, and aimed to increase the better understanding of this type of tumor.
LMS of the colon is slightly more frequent in females. Rao BK et al. reviewed 42 cases with CLMS that female dominance was found in his study. [11] The mean age in this study at the time of diagnosis was 59 years old, which is older than that in a literature review that reported a mean age at diagnosis of 50 years old.[13] Meanwhile, we found that older people had a decreased OS.
Based on the only complains and physical examinations, it was difficult to make an identified diagnosis because preoperative symptoms, such as pain, diarrhea and constipation, are insufficient evidence to make a diagnose of CLMS.[12] LMS could be exactly confirmed by the expression of smooth muscle actin and lack of CD117.[30]
Warkel et al. reported that survival of patients with the CLMS was not associated with the tumor size, but with mitotic activity.[29] In contrast, our study indicated that larger tumor size was associated with worsened PFS. One previous study consistent with our study advocated significant association between large tumor size and poor survival was existed. [31] Unfortunately, with not available and incomplete data regarding mitotic activity, we failed to identify the relationship between mitotic activity and survival.
Surgery had been generally considered as the first line for patients with CLMS. EH Ng et al.[32] published a review of 191 patients treated with surgery, and those who underwent complete resection has 25 months longer OS (the median time) than those with incomplete resection. In our series, due to the lack of details about the type of resection in the operative report of resection, we were unable to find significant difference in PFS or OS rates among patients who received different surgical treatments.
One finding of concerns in this study was the extremely high metastasis rate, and the most frequent sites of distant locations mainly in the liver, lung, peritoneum, humerus, and viscera. Even, many reviewers reported that CLMS with an aggressive clinical behavior that tends to high recurrence and metastasis after radical surgery. [31, 33] Seven patients developed distant metastasis in this current review study, in addition, many patients have developed metastasis before they underwent surgery. Patients with a distant metastasis in general had a poor survival. Therefore, adjuvant treatment after surgery might be recommended in patients with malignant tumors. To the Best of Our Knowledge, however, no postoperative radiotherapy for LMS of the colon has been reported yet. Adjuvant chemotherapy has been described in only two studies, with mixed results. Yaren A et al. [20] reported that a 66-year-old female with CLMS underwent adjuvant chemotherapy with ifosfamide plus doxorubicin after surgery, and no evidence of disease was observed during his follow-up time. Kiran P et al. [23] reported that a 54-year old male with LMS of the colon received postoperative chemotherapy with ifosfamide and doxorubicin for six cycles, but then he developed a recurrence after a disease-free period of half a year. After a surgery for recurrence, he was still alive well without disease. With the two better results described, adjuvant chemotherapy following surgery might be optimal for patients with large LMS of the colon. However, we could not definitely confirm the role of it because of inadequate follow-up time and limited cases. Longer follow-up could be performed to identify the effect of adjuvant treatment.