Hypopharyngo-oesophageal leiomyosarcoma

Case contributed by Dr Gilcrease-Garcia


65 year old male presenting with several months of progressive dysphagia, bloating and difficulty belching. Endoscopic evaluation discovered an oesophageal mass. A biopsy was performed and initial histopathologic evaluation showed hyperplastic squamous epithelium and inflammation with no evidence of malignancy.

Patient Data

Age: 65
Gender: Male

Large, multilobulated mass of the proximal/mid thoracic oesophagus. Additional filling defects superior to the mass may represent retained food. Contrast passed normally distal to the mass. The oesophageal motility is normal. There is passage of contrast through a normal gastro-oesophageal junction.

An oesophageal mass is centred at the proximal esophagus at the level of the thoracic inlet, and extends down to the distal thoracic oesophagus. The mass appears intralumenal. It has heterogenous, predominantly soft tissue attenuation, and is multilobulated in configuration. There is no invasion or compression of adjacent thoracic structures.  There is no mediastinal lymphadenopathy.

Case Discussion

The patient was sent to a tertiary care center for further evaluation.  

Repeat endoscopic biopsy of the oesophageal mass again showed primarily squamous epithelium, although a single fragment of tissue showed monotonous spindle cells. Based on the specimen, the differential diagnosis included oesophageal gastrointestinal stromal tumor, leiomyoma, or less likely a schwannoma. There was not enough tissue to run immunohistochemistry analysis.

Due to progressive symptoms and indeterminate etiology of the mass, it was decided to resect the mass. An initial attempt was made at endoscopic excision; however, the mass was too large to be encircled by cautery snare, and so resection was achieved by transcervical approach.

Intraoperatively, it was discovered that the mass originated from the hypopharynx (above the level of the cricopharyngeus), and hung from a pedicle down into the lower esophagus.  The surgical pathology showed a 11 cm submucosal mass consistent with leiomyosarcoma.

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Case information

rID: 48658
Case created: 19th Oct 2016
Last edited: 21st Dec 2017
Inclusion in quiz mode: Excluded

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