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Esophagus Master > Other Esophageal Tumors > Flashcards

Other Esophageal Tumors Flashcards

1
Q

Percent of malignant esophagal tumors that are NOT either adenocarcinoma or SCC

A

2%

(Adenocarcinoma + SCC = 98% of primary malignant esophageal tumors)

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2
Q

Other non-adenocarcinoma/SCC malignant esophageal tumors include:

A
  1. Adenoidcystic carcinoma
  2. Mucoepidermoid
  3. Verrucous
  4. Small cell (neuroendocrine) tumors
  5. Carcinosarcomas
  6. Melanomas
  7. Leiomyosarcomas
  8. GIST tumors
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3
Q

Benign esophageal tumors include:

A
  1. Leiomyoma
  2. Esophageal cysts
  3. Fibrovacsular polyp
  4. Squamous cell papilloma
  5. Granular cell tumors
  6. Hemangioma
  7. Inflammatory pseudotumor
  8. Adenoma
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4
Q

Characteristics of small cell (neuroendocrine) tumors of esophagus

A
  • 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
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5
Q

Polypoid lesion in lower 2/3 of esophagus

A

Carcinosarcoma

  • carcinomatous + sarcomatous elements
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6
Q

Polypoid, ulcerated mass in lower 2/3 of esophagus

A

Melanoma

  • Often with metastatic disease at time of presentation
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7
Q

MC location for leiomyosarcoma

A

Throughout esophagus

  • Often difficult to differentiate from leiomyoma
  • Tx: Resection (low grade)
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8
Q

Mesenchymal submucosal malignant esophageal tumor

A

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
  • Tx: Resection +/- adjuvant imatinib (blocks tyrosine kinase receptor)
    • Intermediate - high risk: adjuvant imatinib
    • Bulky, resectable disease: neoadjuvant imatinib
    • Advanced, metastatic disease: imatinib +/- tumor debulking
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9
Q

MC benign esophageal tumor:

A

Leiomyoma

  • followed by: esophageal cysts and polyps
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10
Q

MC presentation of benign esophageal tumors

A

Assymptomatic

  • Obstruction when tumor ~ 5 cm
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11
Q

Diagnostic w/u algorithm for esophageal tumor

A
  • Barium esophagram
  • Endoscopy
  • EUS
  • CT
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12
Q

T/F

Intramural esophageal tumors should not be biopsied endoscopically

A

True

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13
Q

MC location of esophageal leiomyoma

A

distal 1/3 of esophagus

  • originates from the muscularis propria of esophagus
  • TOC: enucleation
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14
Q

Histologic features of esophageal leiomyoma

A

whorls of spindle cells with esosinophilic neoplasm within connective tissue

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15
Q

Indications for surgical treatment of esophageal leiomyoma

A
  • Symptomatic (e.g. obstruction)
  • Increasing size
  • Diagnosis in doubt
  • TOC: enucleation
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16
Q

Characteristics of esophageal cysts

A
  • 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
17
Q

MC intraluminal benign esophageal tumor

A

Fibrovacsular polyp

  • upper 1/3 of esophagus
  • usually very large
18
Q

TOC and indications for surgical resection for esophageal fibrovascular polyp

A
  • TOC: resection
    • small tumors: endoscopic resection
    • larger tumors: excision via esophagotomy
  • Indication: resection when diagnosed to avoid growth and obstructive sx
19
Q

Very rare benign esophageal tumor associated with HPV

A

Squamous cell papilloma

  • older patients
  • distal 1/3 of esophagus
  • TOC: resection
    • obstruction
    • inability to distinguish from SCC
20
Q

Benign submucosal tumor in distal 1/3 of esophagus that originates from Schwann cells

A

Grancular cell tumor

  • Resection for large, symptomatic tumors (malignancy can not be excluded)
21
Q

Blue appearing, beign submucosal esophageal tumor

A

Hemangioma

  • Distal 1/3 of esophagus
  • Association with Osler-Weber-Rendu syndrome
  • MC presentation: asymptomatic
    • May present with bleeding and dysphagia
22
Q

Diagnostic evaluation for suspected esophageal hemangioma

A

CT and radionuclide angiography

  • Do not biopsy (risk of bleeding)
23
Q

TOC for esophageal hemangioma

A

Expectant managmen for asymptomatic lesions

Resection for symptomatic (or bleeding) tumors

24
Q

Polyps arising from cloumnar epithelium of esophagus

A

Adenoma

  • Sampling required to rule out dysplasia
25
Q

Characteristics of inflammatory pseudotumor of esophagus

A
  • MC in lower 1/3 of esophagus
  • Difficult to distingish from malignant tumor
  • Multiple biopsies required
  • No specific treatment for histologically confirmed psueudotumors
26
Q

Frequency of esophageal NET

A

Very rare, most of the knowledge on this topic comes from case reports.

27
Q

Most common histologic subtype of Esophageal NET ?

A

Small cell

28
Q

% of Esophageal NET tumors that are metastatic at diagnosis ?

A

30-90%

29
Q

Subtypes of NET in the esophagus

A
  1. Small cell
  2. Large Cell
  3. atypical carcinoid tumor
  4. typical carcinoid tumor
  5. combined endocrine tumor and adenocarcinoma
30
Q

General Definition of Adenocystic carcinoma

A

cylindroma

  • Form of adenocarcinoma that arises from secretory gland.
  • Frequently found in salivary glands
31
Q

Types of Adenocystic carcinoma of the esophagus

A
  1. Cribriform: nest of cells with cylindromatous microcystic spaces.
  2. Tubular: well-formed ducts and tubules lined with inner epithelial cells and outer myoepithelial cells.
  3. Solid: form is characterized by uniform sheets of basaloid cells lacking tubular or microcystic formation.
32
Q

Typical presentation of Adenocystic Ca of the esophagus

A

Epidemiology and Clinical presentation:

Typical presentation:

  • progressive dysphagia
  • average age: 65 years
  • Male: Female: 3.4:1
33
Q

Varriants of Squamous cell Carcinoma

A
  1. Verrucous Carcinoma
  2. Polypoid Carcinoma
34
Q

NET Tumors of the esophagus

A
  1. Small cell carcinoma
  2. Carcinoid
  3. Melanoma
35
Q

Mesenchymal varriants of esophageal

A
  • Leiomyosarcoma
  • Rhabdomyosarcoma
  • Fibrosarcoma
  • Chondrosarcoma

Esophagus Master (11 decks)

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