| Objective: To analyze clinical and pathological characteristics and prognosticfactors of extrapulmonary small cell carcinoma (EPSCC).Methods: Retrospective analysis the clinicopathological characteristics andpotential prognostic factors in50cases of extrapulmonary small cell carcinoma whichwere diagnosed and treated at Fujian Union Hospital from February2007to March2011.Results: There were35males and15females With a median age of61.5years old（31-87years). The primary origin of EPSCC included:32cases of gastrointestinal,seven cases of mediastinal, four cases of head and neck, two cases of bladder, twocases of breast, one case of liver, and another two cases of unknown primary tumor.At diagnosis,31patients (62%) had limited disease, and19cases (38%) had extensivedisease. The regional lymph node metastatic rate was66%, and the distant metastaticrate was about30%, involving liver, brain and lung. Liver was the most frequent siteof metastasis. There were only two brain metastases in the group accounting for4%.Thirty-nine cases had the histologic appearance of simple cell type, and11(accounting for22%) had mixed cell type; with local differentiation of squamous cellcarcinoma or adenocarcinoma. Immunohistochemistry markers of Syn, NSE, CD56,TTF-1, CK and CD57had positive rates of97.4%,94.7%,78.6%,100%,70%and60%respectively. There was a median of1.3months (0.3to60.5months) follow-up.Median survival time (MST) was approximately12.0months and the MST withlimited disease was significantly longer than in patients with extensive disease;19.8νs.5.0months (P<0.0001). With the TNM staging system, the advanced stage patientshad a shorter survival time. MST of Stage I-II was21.4months, stage III was19.8months, and for stage IV it was4.4months (P=0.001). The patients with biggertumors had shorter survival time;<5cm16.0months νs.≥5cm11.0months (P=0.032).At the limited disease stage,MST of patients without regional lymph node metastaseswas longer than those with regional lymph node metastases;32.2months vs.17.0months,and the2-year and3-year survival rates were54.0%νs.33.3%and40.5%νs.22.2%respectively(P=0.562).The survival time of the patients with distantmetastases was significantly shorter than those without distant metastases;4.9months νs.17.0months (P<0.0001). The survival time of the patients with brain metastaseswas shorter than those without brain metastases, but the difference between thesegroups was not statistically significant;6.9months νs.12.6months. The MST of thepatients who were untreated (nine cases) was3.4months, while the MST of the11patients with local treatment was17.0months,12patients with chemotherapy alonewas11.0months, and the18patients with comprehensive treatment was16.0months(P=0.020).1-year and2-year survival rates of the patients with LD who had acceptedlocal treatment alone, chemotherapy alone, comprehensive treatment, and wereuntreated were76.2%,60.0%,76.9%and0%for1-year survival, and38.1%,20.0%,51.3%,0%for2-year survival respectively (P<0.0001).Of patients with LD,the survialtime of the ones without operation(nine cases) was shorter than those with operation(22cases);12.6months νs.21.4months; and the1-year and2-year survival rates were42.9%νs.76.6%and14.3%νs.45.9%respectively. Patients with LD who receivedchemotherapy alone (11cases) had shorter MST than those without chemotherapy (20cases),16.3months νs.21.4months, and the1-year and2-year survival rate were58.3%νs.78.9%and35.0%νs.39.5%respectively. At the extensive stage, the sixmonth survival rates of patients untreated (six cases), receiving local treatment (twocases), chemotherapy alone (seven cases), and with comprehensive treatment (fourcases) were50%,0%,34.3%and50%, and the MST was4.4months,5.0months,4.9months, and6.9m respectively (P=0.850). The the patients with operations (threecases) did not have longer MST than those without operation (16cases),1.9months ν6.9months, P=0.181). Patients who accepted chemotherapy (11cases) did not survivelonger than those did not accept chemotherapy (8cases), and the1-year survival rateswere10.1%νs.14.3%and the MST were6.9months νs.5.0months (P=0.514).Survival time of smokers was shorter than that of non-smokers,8.1months νs.21.4months (P=0.006). Multivariate analysis showed that distant metastases (especiallyliver), VALGS stage, TNM stage, and treatment were the prognostic factors ofEPSCC. Conclusions: Digestive tract was the most common primary site of (especiallythe esophagus) EPSCC. There were high metastasis rate of local lymph nodes anddistant metastasis in EPSCC, and liver metastasis was the most common. MST ofpatients with regional lymph node metastases was shorter than those without regionallymph node metastases,and had a worse prognosis.The patients with distant metastasishad a worse prognosis.The survial time of patients with brain metastases wasshorter,and had a worse prognosis,but the brain metastatic rate was low. Staging(VALGS staging, TNM staging), treatment, distant metastasis (especially livermetastasis) were the prognostic factors of EPSCC. The survival prognosis of LD wasbetter than ED. With TNM Staging system the advanced stage had a worse prognosis.Treatment could prolonged survival time and improved prognosis, patients with LDmight benefit from the surgery and chemotherapy. The combined therapy couldprolong the survival time of patients with LD more than local treatment andchemotherapy alone. Surgery couldn’t prolong the survival time of ED-EPSCC. |
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