Some cases of AFP-producing lung carcinoma were reported in the 1980s. Ishikura et al. first reviewed seven cases of AFP-producing lung carcinoma in 1990 and diagnosed five of the seven cases with HAC . Since that time, 18 cases of HAC of the lung have been reported [1–10], clarifying the clinicopathological and immunohistochemical features. According to those reports, HAC is associated with large tumor, AFP production, poor prognosis, a predilection for older patients, regional lymphadenopathies, and distant metastases [2, 6]. Some studies have described long-term survival of 2–7 years [6–9]. These cases were all in the early pathological stage, with two cases in stage IIB, one in stage IIA, and one in stage IB. Our case was likewise in stage IB and has so far survived 51 months postoperatively. From the above findings, curative resection at an early stage could contribute to long-term survival.
HAC is an extremely aggressive tumor with poor prognosis, and effective chemotherapies have yet to be established. On the other hand, survival for 9 years and for 37 months has been reported in patients with clinical stage IV HAC in the lung . Those patients received multimodal therapy comprising surgery/tumor debulking and chemoradiotherapy, but no description of the specific regimen was provided. The results, however, suggest that intensive treatment can improve patient survival. We were conducting a clinical trial of cisplatin (CDDP) and gemcitabine (GEM) as adjuvant chemotherapy for patients with completely resected pathological stage IB-IIIA non-small cell lung carcinoma (UMIN000005256) at the time we encountered the present patient and enrolled him in the trial. A previous study suggested a survival advantage of adjuvant chemotherapy for stage IB patients who had tumors ≥4 cm in diameter , which was why we designed this study cohort to include stage IB tumors.
The possibility of pulmonary metastasis from hepatocellular carcinoma was excluded for the lung tumor in this case, because no liver tumors were apparent on preoperative contrast-enhanced CT, FDG-PET, or postoperative ultrasonography. Metastasis from hepatocellular carcinoma of the stomach or other organs was also ruled out. HAC of the lung was therefore diagnosed.
In this case, serum AFP levels decreased from 4497 ng/ml at 44 days preoperatively to 125.5 ng/ml at 25 days after surgery and within normal range by 82 days after surgery. From that time, serum AFP level has remained within normal limits and no recurrence has been identified. Serum AFP level could offer a useful marker of disease progression for HAC.
In our case, EBUS-TBNA was useful for accurate staging. As the absence of lymph node metastases was confirmed by EBUS-TBNA, the patient was able to undergo surgery while the tumor was still in the early stage.