Section: UGI Sub-section: Oesophagus Curriculum: Curriculum, page 94
Overview
- Less common than oesophageal cancer
- More commonly submucosa and muscularis than mucosal
- Most asymptomatic and incidental
- Mucosal Tumours
- Submucosal Tumours
- Granular Cell Tumours
- Lipomas
- Haemangiomas
- Neural Tumours
- DDx Leiomyosarcoma, GIST
- Tumours of Muscularis Propria
- Duplication Cyst
Mucosal Tumours
- Fibrovascular Polyps
- Squamous Papillomas
Fibrovascular Polyps
- Heterogenous group of soft tissue tumours
- Fibromas, fibrolipomas, myomas, and lipomas
- Contain a mixture of fibrous, vascular, and adipose tissue covered by squamous epithelium
- Risk
- Men 75%
- 60-70’s
- Clinical
- Location
- Cylindrical or elongated with stalk
- 85% found cervical oesophagus or by cricopharyngeus
- Presentation
- Mostly asymptomatic until very large
- Giant ones can cause severe dysphagia
- Can also prolapse into hypopharynx (causing airway obstruction!)
- Location
- Management
- Most can be resected endoscopically
- EUS prior to resection ?feeding vessel
- Endoscopic resection can be technically difficult when the stalk is attached to the proximal esophagus. Surgical resection may be required if a large feeding vessel is present or if the base of the stalk is endoscopically inaccessible.
Oesophageal squamous papillomas
- Most frequent benign epithelial tumours of the oesophagus
- Usually associated with some underlying inflammation
- Distinctive endoscopic appearance
- Most often middle or lower 1/3
- Colourless, exophytic lesions
- Can be sessile or partly pedunculated
- Well demarcated
- Usually < 5mm
- Very rarely become large and symptomatic
- Excision biopsy warranted to exclude malignancy
Submucosal Tumours
- Granular Cell Tumours
- Lipomas
- Haemangiomas
- Neural Tumours
Granular Cell Tumours
- Found in skin, respiratory tract, GI tract, breast, tongue
- Distal 1/3 oesophagus most common location
- Bulging lesion with overlying normal mucosa
- Standard biopsies may be non-diagnostic
- Tunnelled biopsy shows eosinophilic granules
- Stain positive for S100
- EUS shows regular bordered lesion in 1st or 2nd sonographic layer
- Mostly benign, 1-2% malignant
- If symptomatic, atypical EUS, size > 2cm consider resection
- EMR for biopsy and treatment if feasible
Other Submucosal Tumours
Lipomas
- Characteristic, homogeneous, hyperechoic, smooth appearance on EUS
- Symptoms are rare even with large tumours
- Resection is seldom warranted
Haemangiomas
- Typically purple or reddish nodule
- Smooth, hypoechoic, submucosal mass on EUS
- Most asymptomatic
- If dysphagia or bleeding – treat endoscopically
Neural tumours
- Neurofibromas and schwannomas
- Rare in the oesophagus
- Majority benign
- Handful of case reports on malignant oesophageal schwannoma
- Symptomatic tumours
- Resected by enucleation
- Large tumours may require oesophagectomy
Muscularis Propria
Leiomyoma
- Most common benign Mesenchymal tumours of oesophagus
- M > F 2:1
- Typically distal or middle 1/3
- Arise in muscularis propria
- Most < 3cm
- Most asymptomatic
- If larger can cause dysphagia
- Form hard white/greyish mass
- Shows smooth filling defect on barium swallow
- Similar round, smooth defect with overlying normal mucosa on OGD
- EUS shows regular bordered, hypoechoic lesions arising from muscularis
- Can manage small (< 2cm), classically appearing lesions conservatively unless symptomatic
- Enucleate if uncertain or symptomatic
- NB: Biopsy = Harder to enucleate!
- Enucleate if uncertain or symptomatic
- Immunostains for Desmin and Smooth Muscle Actin
- Differentiates from GIST
- Stains for KIT and DOG1
- Differentiates from GIST
- DDX Leiomyosarcoma, GIST
Duplication Cyst
- Congenital anomalies, arising in early embryonic development
- Most commonly small intestine
- Can be oesophagus, stomach, colon
- Oesophagus seen in 1:8000 live births
- 3 criteria
- Lay in wall of oesophagus
- Covered by two muscle layers
- Contain squamous epithelium
- Or lining compatible with tissue found in oesophagus
- Up to 1/3 contain heterotrophic gastric mucosa
- Can also have pancreatic mucosa or mucosa consistent with Peyer’s Patches
- 80% do not communicate with oesophageal lumen
- Others run in parallel and do communicate
- Most commonly on the right lateral portion of oesophagus
- Frequently cause symptoms (unlike in other parts of the GI tract)
- Because of this, most diagnosed before the age of 2
- Symptoms from compression
- Dysphagia
- Epigastric or retrosternal pain
- Respiratory symptoms (cough, stridor, wheeze)
- Surgically resect symptomatic cysts
- Malignancy rare but has been reported