Esophagus
Other malignancies
Liposarcoma

Author: Feriyl Bhaijee, M.D. (see Authors page)
Editor: Israh Akhtar, M.D.

Revised: 1 February 2018, last major update October 2013

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Liposarcoma esophagus

Cite this page: Bhaijee, F. Liposarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusliposarcoma.html. Accessed August 19th, 2018.
Definition / general
Epidemiology
  • Common soft tissue sarcoma in adults but rare in GI tract
  • Extremely rare in the esophagus: < 25 cases reported; < 1.5% of all GI liposarcomas
  • Slight male predominance
  • Wide age range: 38 - 73 years
Sites
  • Usually upper (cervical) esophagus (80%)
  • Likely arises in submucosa
Clinical features
  • Dysphagia, weight loss, vomiting
  • 5 year survival: 75 - 100%
Radiology description
  • CT / MRI: low density adipocytic components, often with inhomogenous enhancement (Dysphagia 2008;23:327)
  • Barium studies: show intraluminal polypoid mass / filling defects
  • Endoscopic guided ultrasound: lipomatous differentiation and subtle inhomogeneity
Radiology images

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Tumor protruding into lumen

Tumor mass occupying dilated esophaugs

Low density discrete mass herniating

Lobulated liposarcoma

Long tumor mass filling thoracic esophagus


Narrowing of lumen

Well circumscribed, heterogeneous mass

Liposarcoma with rhabdomyomatous

Luminal distension in upper esophagus

Prognostic factors
  • Grade
  • Histologic subtype (well differentiated liposarcoma has best prognosis)
  • Location
  • Adequacy of surgical resection
Case reports
Treatment
Clinical images

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Large pedunculated tumor mass

Huge mass with stalk

Endoscopic submucosal dissection

Esophageal obturation

Distal transmural liposarcomas

Normal mucosa and space occupying mass

Gross description
  • Usually large, pedunculated, intraluminal mass
  • Wide size variation: 4 - 23 cm
  • May be transmural (Eur J Cardiothorac Surg 2011;40:1253, Eur J Cardiothorac Surg 2011;39:795)
  • Variants:
    • Well differentiated: well circumscribed mass with uniform, yellow cut surfaces
    • Myxoid: well circumscribed gelatinous masses
    • Dedifferentiated: multinodular mass with yellow cut surfaces and firm tan gray areas
Gross images

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Yellow, multilobulated mass divided by fibrous septa

Liposarcoma

Pink tan tissue fragments

Distal esophagus with transected tumor

Liposarcoma

Liposarcoma with rhabdomyomatous differentiation

Microscopic (histologic) description
  • 4 histologic variants
    • Well differentiated: proliferation of mature adipocytes with marked size / shape variation and focal nuclear atypia in adipocytes or stromal cells
      • Subtypes:
        • Sclerosing: hyperchromatic bizarre stromal cells
        • Inflammatory: marked lymphoplasmacytic infiltrate
        • Spindle cell: predominantly spindle cell proliferation with variable number of lipoblasts
    • Myxoid: proliferation of spindle / round cells and monovacuolated lipoblasts in myxoid stroma with a plexiform vascular network
    • Pleomorphic: pleomorphic high grade sarcoma with variable number of lipoblasts
      • Pleomorphic lipoblasts: giant multinucleated lipoblasts with bizarre hyperchromatic nuclei and cytoplasmic lipid vacuoles
    • Dedifferentiated: transition from well differentiated liposarcoma to high grade nonlipogenic sarcoma
      • High grade components: marked pleomorphism, atypia, increased mitotic activity, variable necrosis
  • All variants may have lipoblasts (multivaculoated cells with hyperchromatic scalloped nuclei), which are not essential to the diagnosis
  • Nonlipogenic components in pleomorphic or dedifferentiated tumors may resemble other soft tissue sarcomas, e.g. MFH, epithelioid sarcoma
Microscopic (histologic) images

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Well differentiated components:

Submucosal lesion

Atypical hyperchromatic stromal cells

Bizarre, big nucleus

Nuclear expression of MDM2



Myxoid / round cell delicate vasculature:


Dedifferentiated components:

Atypical spindle cells



Pleomorphic liposarcoma:

Fig 3a: normal squamous
epithelium; fig 3c:
resembles a malignant
fibrous histiocytoma



Liposarcoma with rhabdomyomatous differentiation arising in a giant fibrovascular polyp:
Cytology description
  • Well differentiated variant: mature adipocytes with size variation and nuclear atypia
  • Myxoid / round cell variant: granular myxoid matrix, branching thin walled capillaries, round to oval cells with occasional cytoplasmic vacuoles, mono / multivacuolated lipoblasts
  • Pleomorphic variant: pleomorphic lipoblasts
Cytology images

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Myxoid / round cell variant:
Positive stains
  • S100 in lipogenic zones
  • Well differentiated liposarcoma: MDM2, CDK4
  • Dedifferentiated liposarcoma: focal cytokeratin or CD68 expression
Negative stains
Electron microscopy description
  • Tumor cells may contain nonmembrane bound intracytoplasmic lipid droplets of varying sizes / densities
Molecular / cytogenetics description
Molecular / cytogenetics images

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CPM gene amplification

MDM2 gene amplification

Differential diagnosis
  • Benign lipoma: little adipocytic size / shape variation, minimal cytologic atypia; MDM2-, CDK4-
  • Giant fibroepithelial polyp: may contain stromal adipocytes with minimal atypia; MDM2-, CDK4-