A 66-year-old man presented with a 2-month history of sustained hoarseness, sore throat, and dysphagia. The syndromes progressed 1 month later, and a left neck mass was found accidentally. His past medical history included 20 years of hypertension that was controlled by oral irbesartan (one tablet per day) and 20 years of atrial fibrillation and coronary artery disease (one tablet of metoprolol and warfarin once a day, respectively, and half a tablet of digoxin once a day). He also suffered from pulmonary tuberculosis 40 years ago, which was cured (there were no active tuberculosis lesions on a lung CT, and blood test and sputum cultures were negative).
On physical examination, a tender 3x4 cm left cervical mass with an unclear boundary was found at the level III. A strobolaryngoscope revealed a large mass arising from the posterior hypopharynx, and movements of both the glottis and vocal cords were invisible (Figure 1). MRI revealed a 28x14 mm mass located in the left hypopharyngeal region, and multiple lymph nodes of the left neck were integrated. T1-weighted images were hypointense, T2-weighted images were hyperintense, and diffusion-weighted imaging was hyperintense. The lesion underwent heterogeneous enhancement following administration of gadolinium-DTPA; the mass was strongly enhanced and the integrated lymph node was peripherally enhanced (Figure 2A and B). PET/CT showed high standardized uptake values (SUVs) of FDG in the left hypopharyngeal regions (SUVmax=8.9) and left cervical lymph node (SUVmax=9.5), but there were no high FDG lesions in other parts of the body (Figure 3A and B).
Our head and neck oncological multidisciplinary team (MDT; Department of Otolaryngology Head-Neck Surgery, Maxillofacial Surgery, Radiology, Radiotherapy, Microsurgery, Thoracic Surgery, and Vascular Surgery) discussed the preoperative possible diagnosis, therapeutic modalities, and whether further postoperative treatment was needed if surgery was performed. The MDT reached a consensus that the diagnosis was hypopharyngeal carcinoma with cervical lymph node metastasis (T2N2M0) and that the treatment would be determined according to frozen section results. If the frozen sections showed small cell carcinoma/malignant tumor or poorly differentiated carcinoma, the patient would first receive concurrent chemoradiotherapy (CCRT). If the frozen section results suggested other type carcinomas, treatment would be partial laryngopharyngectomy plus bilateral neck dissection (II-VI) with postoperative chemo-radiotherapy according to NCCN guidelines regarding hypo-pharyngeal carcinoma. Under general anesthesia, a biopsy sample was obtained via suspension laryngoscopy. Analysis of the frozen section suggested the presence of a small cell malignant tumor (most likely small cell carcinoma).
According to the preoperative plan developed by the MDT, CCRT was performed. Routine pathology (Figure 4) revealed that the small tumor cells were uniform in size, exhibited marked heteromorphy, and were arranged into nests and cords with sparse cytoplasm. Immunohistochemical staining revealed that the primary tumor was positive for cytokeratins (Figure 5A), CD56 (Figure 5B), chromogranin A (Figure 5C), and synaptophysin (Syn; Figure 5D) and negative for p63, CK7, and CD45. The Ki-67 (Figure 5E) mitotic index approached 80%. These histopathological features confirmed the presence of hypopharyngeal PDNC. The patient received six cycles of chemotherapy.
The patient received regular follow-ups. On March 14, 2017, CT revealed that the lesion in the hypopharynx had shrunk markedly and the metastatic left lymph nodes had disappeared (Figure 6). On March 21, 2017, CT revealed the presence of irregular soft tissue in the left side of the anterior chest wall, sized ~2.7x4.7 cm, invading the corresponding ribs, and multiple high-density nodules in the bilateral lungs (Figure 7). Ultrasonography revealed a hypoechoic lesion in the left liver (Figure 8). Whole body bone imaging revealed tracer concentrated in the right skull, left rib 4, left rib 8, rib 9, right scapula, the vertebral body of lumbar 5, the left superior iliac spine, and right sacroiliac joint (Figure 9). Then, a left chest nodule was biopsied. Immunohistochemical staining revealed that the nodule was positive for chromogranin A, CD56, and Syn. The Ki-67 mitotic index approached 80%. These findings confirmed metastatic PDNC. On April 8, 2017, CT revealed multiple liver metastatic lesions, and swollen metastatic lymph nodes appeared in the hepatogastric gap and porta (Figure 10). On June 14, 2017, CT revealed no invisible swollen lymph nodes bilaterally (Figure 11). No further treatments were given, and the patient died of multiple metastases on August 2017.
CT revealed the presence of irregular soft tissue in the left side of the anterior chest wall (red arrow), sized ~2.7x4.7 cm, invading the corresponding ribs, and multiple high-density nodules (blue arrow) in the bilateral lungs. . Abbreviation: CT, computed tomography.