Hepatoid adenocarcinoma of the lung metastasizing to the tonsil

Abstract

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 α-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 α-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 ...

<p>Anti-PD-1 Therapy Achieved Disease Control After Multiline Chemotherapy in Unresectable <em>KRAS</em>-Positive Hepatoid Lung Adenocarcinoma: A Case Report and Literature Review</p>

“…Neoadjuvant chemotherapy plus surgical resection could be used to treat non-advanced HAL, whereas the regimens for advanced-stage patients have not been established. [1][2][3][11][12][13][14][15][16][17][18][19][20][21][22][23][24] We searched all known cases of HAL and included a total of 22 patients who were treated with chemotherapy and immunotherapy in the literature review (Table 2). Interestingly, most of the patients were male (20/22) and only 2 female cases (including the present case) were reported which indicated a sex difference of HAL.…”

Hepatoid adenocarcinoma of the lung mimicking metastatic hepatocellular carcinoma

“…The second is that neither a high serum AFP level nor the tumor AFP expression directly results in a poor prognosis. 5,6,17 In the current case, the cancer was aggressive, and the patient died six months after the initial diagnosis regardless of the tumor's negative expression of AFP. Therefore, hepatoid morphology is the most important factor for diagnosing hepatoid adenocarcinoma of the lung.…”

“…Hepatoid adenocarcinoma of the lung is a rare subtype of lung carcinoma, and only 22 cases have been reported in the full-text English literature. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Ishikura et al, 20 first reported a hepatoid adenocarcinoma of the lung and proposed the following two diagnostic criteria: (i) presence of typical acinar or papillary adenocarcinoma, and (ii) presence of a carcinoma component which resembles hepatocellular carcinoma and produces AFP. However, only 12 out of 23 (52.2%) hitherto described cases exhibited high serum AFP levels (>10 ng/ml).…”

“…Out of 19 cases in which the tumor expression of Hep Par-1 was tested, 17 (89.5%) cases, including the current case, were found to be positive for Hep Par-1. [2][3][4][5][6][7][8][9][10][14][15][16][17][18] Although Hep Par-1 is not a specific marker of hepatoid differentiation, 21 a combination of the Hep Par-1-positive immunophenotype and hepatoid morphology should be considered to be supportive of the diagnosis of hepatoid adenocarcinoma.…”

<p>Radiotherapy of Pulmonary Hepatoid Adenocarcinoma with Intrahepatic Hemangioma: A Case Report</p>

“…According to the reported cases, the clinical features, treatments and prognosis of the pulmonary HAC patients are shown in Table 1 (palliative radiotherapy to bone or other metastatic sites is not included in the table). 1,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] The incidence of pulmonary HAC is higher in men, which is similar to the incidence of other pathological types of lung cancer. The age of the patients ranges from 47 to 71, with an average age of 58.91 years.…”