Abstract. Hepatoid adenocarcinoma (HAC) is a rare tumor that typically originates in gastrointestinal tissue, such as the stomach, but can also occasionally originate in the lung. The majority of HACs express 伪-fetoprotein (AFP) on tumor cells, and serum AFP can be used as a marker of response to treatment or disease progression. HAC has a poor prognosis, although early diagnosis and aggressive treatment can result in long-term survival. To the best of our knowledge, this is the first reported case of HAC metastasizing to the tonsil. Irradiation using intensity-modulated radiation therapy (IMRT) to 30 Gray (Gy) achieved a significant, and durable tumor response. IMRT can be considered for local control of HAC at other metastatic sites of disease.
IntroductionHepatoid adenocarcinoma (HAC) is a rare 伪-fetoprotein (AFP)-producing tumor that morphologically resembles hepatocellular carcinoma. Tumor cells have eosinophilic cytoplasm, centrally located nuclei proliferating in a trabecular pattern, and express AFP, HepPar1, HEA125 and MOC31 (1,2). The lung is the primary site in approximately 5% of cases of HAC (1). HAC metastasis to the brain, liver, adrenal gland, lymph nodes and bones has been previously described (1). The prognosis in patients with unresectable disease is generally poor with overall survival of 6 to 11 months (3).In the present study, we report, to the best of our knowledge, the first case of primary lung HAC metastasizing to the tonsil.Wide surgical excision, chemotherapy, and radiation therapy (RT) have been used to treat oropharyngeal metastases from solid tumors (4). However, the role of RT for tonsillar metastases has not been previously described. The potential benefits of palliative RT in patients with lung cancer metastasis to the tonsils include mitigation of asphyxiation risk, durable remission, and an improvement in survival (5-7). Thus, RT should be considered a potentially effective treatment for tonsillar metastasis albeit tonsillar metastases portend a poor prognosis with a high likelihood of disease at other sites (5).
Case reportA 61-year-old male presenting with left-sided chest pain due to malignant pleural effusion was diagnosed with stage IVA HAC of the lung (Fig. 1). Despite front-line chemotherapy with cisplatin and pemetrexed, he experienced multiple recurrences with metastasis to the lungs, liver, adrenal gland, and spine and received four additional lines of chemotherapy. Forty-five months after diagnosis, the patient complained of a foreign-body sensation in his throat that caused intermittent gagging and a mass was identified in the left palatine tonsillar fossa ( Fig. 2A). He had no systemic complaints except intermittent fatigue and mid-back pain from a metastatic thoracic spine lesion previously treated with RT. Magnetic resonance imaging (MRI) of the neck showed asymmetry of the tonsils (left larger than right), no direct invasion of the tonsillar mass into deeper structures and no cervical lymphadenopathy (Fig. 2B). A fluorodeoxyglucose positron emission tomography ...