Hazardous giant pharyngoesophageal liposarcoma

Background

Benign fibrovascular polyps (FVP) of the upper aerodigestive tract are uncommon and most commonly occur in the oesophagus, with only a few cases occurring in the hypopharynx. Malignant transformation of these polyps is extremely rare. A rare liposarcoma that mimics a benign polyp occasionally occurs in the hypopharynx. Only 40 cases of hypopharyngeal liposarcoma have been documented in the literature. Because the clinical and radiological features are almost identical to the benign counterpart, diagnosing the lesion during the preoperative period is difficult.1–3

Case presentation

A man in the early 80s presented to the outpatient department with progressively worsening dysphagia to solids, resulting in a 10 kg weight loss over months. He also described a recent episode of breathing difficulty and a very brief expulsion of a solid mass from the mouth. To alleviate his respiratory symptoms, he used to reduce the mass manually.

Physical examination revealed that the oral cavity and oropharyngeal structures were normal. There was no cervical lymphadenopathy, and the neck was soft, with absent crepitus. Flexible endoscopy revealed a yellowish polypoidal lesion with multiple elongations in the hypopharynx that was likely to have originated from the posterior pharyngeal wall on the right side, across from the arytenoid cartilage. This lesion extended inferiorly into the lumen of the oesophagus without infiltration on the walls. The mass was encroaching on the posterior glottic region and blocking the airway. The larynx and oropharynx appeared normal, and the vocal cords were bilaterally mobile (figure 1). Upper gastrointestinal endoscopy revealed an intraoesophageal tumour that extended 30 cm from the dental arch.

Figure 1

Endoscopic view of a lipomatous tumour with attachment (black dot) prolapsing into the laryngeal lumen (yellow arrow).

Investigations

A barium contrast study revealed oesophageal dilatation in the proximal two-thirds, indicating an intraluminal tumour rather than extrinsic compression (figure 2).

Figure 2

Barium swallow showing the dilated oesophagus with intraluminal tumour (black arrow) in lateral neck view and (yellow arrow) in chest view.

Contrast-enhanced neck and chest CT revealed a 16 cmlong well-defined intramural round lesion with fat attenuation filling the oesophageal lumen, consistent with the diagnosis of benign lipoma lesions (figure 3).

Figure 3

CECT showing the well-defined intramural round lesion with fat attenuation filling the oesophageal lumen. CECT, Contrast-enhanced computed tomography.

Differential diagnosis

Based on the clinical appearance of an endoscopically evident fatty lesion and a thick stalk on the posterior pharyngeal wall with an elongated mass, we suspected benign FVP of the hypopharynx. Furthermore, the appearance of a lipomatous lesion on CT supports the diagnosis of FVP. Fibrolipomas, fibromyomas, hamartomas, fibromas and lipomas are other lesions that may look like FVP. We planned an endoscopic tumour excision because all these tumours are benign and require surgical excision to confirm the diagnosis.

Treatment

The polyp was sectioned at the base of its pedicle using a thunder beat vessel sealing device (OLYMPUS MEDICAL SYSTEM TB 5 mm, 35 cm) and the Wierda laryngoscope. Both the hypopharyngeal and oesophageal components were found pedunculated on a broad base that originated in the posterior pharyngeal wall areas of the hypopharynx. The tumour had the macroscopic appearance of a polyp with a smooth surface, measuring 16×5×4 cm, and consisted mainly of a 4 cm long pedicle (figure 4). After 72 hours of nasogastric tube feeding, the patient’s oral diet was restarted. There were no postoperative complications; thus, the patient was discharged. Contrary to our preoperative diagnosis, the histopathological examination demonstrated a well-differentiated liposarcoma with mature adipocytes and the tumour cells showing immunopositivity for S-100 and CD34.

Figure 4

Tumour specimen that was excised.

We advised him to have revision surgery to avoid recurrence after a tumour board discussion because we had previously performed surgery assuming he had a benign tumour. He has been informed about the disease’s nature and prognosis. Due to his age, surgical morbidities, the slow growth of the disease and the low likelihood of the disease spreading over a long distance, he and his family were hesitant to undergo revision surgery. They agreed to return regularly for active surveillance.

After reviewing the literature, we decided not to use any adjuvant therapy, such as radiation or chemotherapy.

Outcome and follow-up

On follow-up till 2 months, he had normal swallowing and breathing. We planned for an upper gastrointestinal endoscopy and an MRI 6-month postsurgery. Unfortunately, he succumbed to COVID-19 infection. His relatives consented to publication over the phone, which was documented. The anonymity of the patient is retained in this article.