Synchronous Laryngeal Squamous Cell Carcinoma and Intrahepatic Cholangiocarcinoma Present in an Obese Male with Poor Prognosis

Discussion

Laryngeal squamous cell carcinoma is the second most common respiratory tract cancer, only after lung cancer (7). It is often tobacco and alcohol related. HPV 16 and 18 are also frequently implicated in the development of squamous cell laryngeal carcinoma. The most common early symptoms are hoarseness, dyspnea, and stridor (7). There are also geographical differences in topographical distribution of laryngeal SCC. Diagnosis of laryngeal SCC can often be made via biopsy with hematoxylin and eosin (H&E) staining. In this case, the patient presented to the emergency department with hoarseness and difficulty swallowing and a 30-pack-year smoking history. The tumor was found mainly in supraglottis with extension to the left true vocal cord, verified by both CT/MRI images and laryngoscopy. H&E stain of the biopsy revealed an exophytic well–differentiated squamous cell carcinoma. Neck lymph node enlargement was also present. A total laryngectomy with bilateral neck lymph nodes dissection was initially considered. However, the patient was found to have a 4.6-cm liver mass suspicious for metastatic laryngeal carcinoma in the pre-operative work-up.

This is a rare case of synchronous primary malignancies with both larynx cancer and intrahepatic cholangiocarcinoma. There was concern for metastasis when the liver lesion was initially identified on CT. MRI was helpful to rule out the hepatocellular carcinoma based on the imaging characteristics but metastatic squamous cell carcinoma remained on the differential. The liver mass was not confirmed as a metastatic SCC. Instead, it was diagnosed as a moderately differentiated adenocarcinoma. Diagnosis of cholangiocarcinoma is typically considered as a diagnosis of exclusion (8). The tumor cells were strongly and diffusely positive for CK7 and CK19, weakly positive for CDX2, negative for CD20, PSA and TTF1. Based on clinical information, imaging findings and immunoprofile, the final diagnosis was synchronous intrahepatic cholangiocarcinoma.

The incidence of cholangiocarcinoma, particularly ICC, has increased globally over the past few decades. Surgical resection remains the mainstay of potentially curative treatment with median disease-free survival (DFS) durations of 12-36 months reported in various patient series (9, 10). For patients with advanced or unresectable disease, the prognosis is poor. Locoregional and systemic chemotherapies are the preferred treatment options (11). Our patient had a large cholangiocarcinoma in the segment 5/8 with capsule retraction. While intrahepatic cholangiocarcinoma alone is usually treated by hepatic resection aided by adjuvant therapy (12), benefit of surgery in this patient was not certain due to the presence of the second cancer. In addition, the baseline health condition of the patient did not render him a good surgical candidate either. Medical treatment of synchronous laryngeal carcinoma and cholangiocarcinoma also appeared to be elusive with the concern over efficacy and added toxicity since no similar cases could be found in the literature. The patient deteriorated rapidly and unfortunately deceased soon after the diagnoses were made.

In conclusion, we present the first documented case of synchronous laryngeal squamous cell carcinoma and intrahepatic cholangiocarcinoma. Diagnosis and management of multiple primary malignancies are often difficult because of the lack of relevant literature. Due to the poor prognosis and significant morbidity and mortality of these cancers it is important to understand the natural progression, imaging, pathologic presentation, and potential treatment options.