Other Esophageal Tumors Flashcards
Percent of malignant esophagal tumors that are NOT either adenocarcinoma or SCC
2%
(Adenocarcinoma + SCC = 98% of primary malignant esophageal tumors)
Other non-adenocarcinoma/SCC malignant esophageal tumors include:
- Small cell (neuroendocrine) tumors
- Carcinosarcomas
- Melanomas
- Leiomyosarcomas
- GIST tumors
Benign esophageal tumors include:
- Leiomyoma
- Esophageal cysts
- Fibrovacsular polyp
- Squamous cell papilloma
- Granular cell tumors
- Hemangioma
- Inflammatory pseudotumor
- Adenoma
Characteristics of small cell (neuroendocrine) tumors of esophagus
- MC location: distal 1/3 of esophagus
- Prognosis poor
- Tx: esophagectomy + adjuvant chemotherapy (if confined to esophagus)
- some advocate XRT rather than esophagectomy due to poor prognosis
Polypoid lesion in lower 2/3 of esophagus
Carcinosarcoma
- carcinomatous + sarcomatous elements
Polypoid, ulcerated mass in lower 2/3 of esophagus
Melanoma
- Often with metastatic disease at time of presentation
MC location for leiomyosarcoma
Throughout esophagus
- Often difficult to differentiate from leiomyoma
- Tx: Resection (low grade)
Mesenchymal submucosal malignant esophageal tumor
GI stromal tumor (GIST)
- c-kit mutations (tyrosine kinase receptor)
- Prognosis dependent on: size + grade (# mitoses/hpf)
- High-risk for recurrence:
- Size > 5 cm
- > 5 mitoses/hpf
- High-risk for recurrence:
- Tx: Resection +/- adjuvant imatinib (blocks tyrosine kinase receptor)
- Intermediate - high risk: adjuvant imatinib
- Bulky, resectable disease: neoadjuvant imatinib
- Advanced, metastatic disease: imatinib +/- tumor debulking
MC benign esophageal tumor:
Leiomyoma
- followed by: esophageal cysts and polyps
MC presentation of benign esophageal tumors
Assymptomatic
- Obstruction when tumor ~ 5 cm
Diagnostic w/u algorithm for esophageal tumor
- Barium esophagram
- Endoscopy
- EUS
- CT
T/F
Intramural esophageal tumors should not be biopsied endoscopically
True
MC location of esophageal leiomyoma
distal 1/3 of esophagus
- originates from the muscularis propria of esophagus
- TOC: enucleation
Histologic features of esophageal leiomyoma
whorls of spindle cells with esosinophilic neoplasm within connective tissue
Indications for surgical treatment of esophageal leiomyoma
- Symptomatic (e.g. obstruction)
- Increasing size
- Diagnosis in doubt
- TOC: enucleation
Characteristics of esophageal cysts
- develop from persistent vaculoes in wall of foregut
- occur in close proximity to or within wall of esophagus
- MC in children (sx of esophageal or airway obstruction)
- TOC: enucleation
- even if asymptomatic (prevent growth and obstruction
MC intraluminal benign esophageal tumor
Fibrovacsular polyp
- upper 1/3 of esophagus
- usually very large
TOC and indications for surgical resection for esophageal fibrovascular polyp
- TOC: resection
- small tumors: endoscopic resection
- larger tumors: excision via esophagotomy
- Indication: resection when diagnosed to avoid growth and obstructive sx
Very rare benign esophageal tumor associated with HPV
Squamous cell papilloma
- older patients
- distal 1/3 of esophagus
- TOC: resection
- obstruction
- inability to distinguish from SCC
Benign submucosal tumor in distal 1/3 of esophagus that originates from Schwann cells
Grancular cell tumor
- Resection for large, symptomatic tumors (malignancy can not be excluded)
Blue appearing, beign submucosal esophageal tumor
Hemangioma
- Distal 1/3 of esophagus
- Association with Osler-Weber-Rendu syndrome
- MC presentation: asymptomatic
- May present with bleeding and dysphagia
Diagnostic evaluation for suspected esophageal hemangioma
CT and radionuclide angiography
- Do not biopsy (risk of bleeding)
TOC for esophageal hemangioma
Expectant managmen for asymptomatic lesions
Resection for symptomatic (or bleeding) tumors
Polyps arising from cloumnar epithelium of esophagus
Adenoma
- Sampling required to rule out dysplasia
Characteristics of inflammatory pseudotumor of esophagus
- MC in lower 1/3 of esophagus
- Difficult to distingish from malignant tumor
- Multiple biopsies required
- No specific treatment for histologically confirmed psueudotumors
