Esophagus
Other malignancies
Sarcoma - general

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

Revised: 21 December 2015, last major update December 2013

Copyright: (c) 2003-2015, PathologyOutlines.com, Inc.

PubMed Search: Sarcoma [title] esophagus
Definition / General
  • Malignant mesenchymal tumors of the esophagus with variable degrees of differentiation, biologic behavior, and prognosis
  • Examples
    • Angiosarcoma: high-grade malignancy of endothelial cells
    • Ewing sarcoma: highly malignant small round blue cell tumor characterized by recurrent chromosomal translocations [t(11;22) EWSR1-FLI1 or t(21;22) EWSR1-ERG] and membranous MIC2 / CD99 overexpression
    • Fibrosarcoma: spindle cell neoplasm of low grade to intermediate grade malignancy
    • Gastrointestinal stromal tumor (GIST): mesenchymal tumor of digestive tract, likely originating from multipotential progenitors of interstitial cells of Cajal
    • Hemangiopericytoma (solitary fibrous tumor): ubiquitous mesenchymal tumor showing uncertain line of differentiation (not true microvascular pericytes)
    • Kaposi sarcoma: uncommon, low grade, vascular malignancy caused by Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV / HHV-8) infection
    • Leiomyosarcoma: malignant smooth muscle tumor
    • Liposarcoma: malignant adipocytic / lipomatous tumor
    • Malignant peripheral nerve sheath tumor (MPNST): malignant tumor arising from peripheral nerve or a neurofibroma, or in extraneural soft tissue and showing nerve sheath differentiation
    • Osteosarcoma: mesenchymal malignancy characterized by neoplastic cells that produce osteoid matrix
    • Synovial sarcoma (SS): malignant mesenchymal tumor showing epithelial differentiation, either overtly (biphasic SS) or by IHC alone (monophasic SS)
    • Undifferentiated pleomorphic sarcoma: highly malignant pleomorphic neoplasm lacking any specific line of differentiation
Epidemiology
  • Extremely rare
Sites
  • Likely arise from submucosal or intramural elements
Clinical Features
  • Asymptomatic mass
  • Obstructive features: dysphagia, odynophagia, nausea, vomiting
Diagnosis
  • Biopsy or resection with immunohistochemical or molecular ancillary studies
Radiology Description
  • Highly variable
    • Destructive mass with variable hemorrhage or necrosis
    • Strictures or obstruction
Radiology Images

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Dilated thoracic esophagus

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Gastrographin esophagography

Prognostic Factors
  • Poor prognostic factors
    • High histologic grade
      • Cytologic atypia, nuclear pleomorphism
      • Necrosis
      • Mitotic activity
  • Poor degree of differentiation
Treatment
  • Treatment options depend on type of sarcoma and clinical stage
  • Treatment modalities include surgery, chemotherapy, radiotherapy
Clinical Images

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GIST

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Kaposi sarcoma

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Leiomyosarcoma

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Liposarcoma

Gross Description
  • Often large tumor with infiltrative margins and variable hemorrhage, necrosis
Gross Images
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Sarcoma


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GIST

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Liposarcoma

Micro Description
  • Highly variable
    • Round blue cell tumors: Ewing sarcoma, small cell variant of osteosarcoma
    • Spindle cell tumors: angiosarcoma, fibrosarcoma, GIST, hemangiopericytoma, Kaposi sarcoma, leiomyosarcoma, MPNST, synovial sarcoma
    • Adipocytic differentiation: liposarcoma
    • Osteoid production: osteosarcoma
    • Undifferentiated: pleomorphic sarcoma
Micro Images
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Sarcoma, not otherwise specified



Images courtesy of Dr. Zafar Ali - 36 year old man with mass just below upper esophageal sphincter, synovial sarcoma (biphasic):

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CD99

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EMA

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TLE1



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Hemangioperi-
cytoma

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Ewing sarcoma

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Leiomyosarcoma

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Liposarcoma

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Osteosarcoma

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Synovial sarcoma

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Angiosarcoma

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GIST

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Kaposi sarcoma

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MPNST

Positive Stains
Flow Cytometry Description
  • Useful to exclude hematopoietic malignancies
Electron Microscopy Description
  • Angiosarcoma: Weibel-Palade bodies
  • Ewing sarcoma: extensive cytoplasmic glycogen deposits
  • Hemangiopericytoma: undifferentiated spindle cell or fibroblastic features
  • Leiomyosarcoma: features of smooth muscle differentiation (thin filaments, pinocytic vesicles, attachment plaques, interrupted external lamina)
  • Liposarcoma: non-membrane bound intracytoplasmic lipid droplets of varying sizes/densities
  • MPNST: tumor cells with external laminae, indicating schwannian differentiation
  • Osteosarcoma: osteoid matrix comprises non-periodic fibrils, scattered collagen fibers, and hydroxyapatite calcium crystals
Molecular / Cytogenetics Description
  • RT-PCR or FISH
    • Ewing sarcoma: recurrent chromosomal translocations including t(11;22)(q24;q12) EWS-FLI1, t(21;22)(q22;q12) EWS-ERG, t(7;22) EWS-ETV1, t(17;22) EWS-E1AF, t(2;22) EWS-FEV
    • GIST: mutually exclusive mutations of activating KIT (95%) or platelet derived growth factor alpha (PDGFRA) receptor tyrosine kinase (5%)
    • Kaposi sarcoma: HHV8 detected by PCR
    • Leiomyosarcoma: no consistent genetic events reported
    • Well-differentiated liposarcoma: giant marker or supernumerary ring chromosomes with amplification of 12q12-15 region, including MDM2, CDK4, and other genes
    • Myxoid / round cell liposarcoma: t(12;16) DDIT3-TLS or t(12;22) DDIT3-EWS balanced translocations
    • Synovial sarcoma: characteristic t(X;18) SSYT-SSX1/2 translocations
Molecular / Cytogenetics Images

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Well-differentiated liposarcoma

Differential Diagnosis
  • Benign spindle cell proliferations, e.g.
  • Lymphoma: usually CD45 (LCA)+, flow cytometry may be diagnostic
  • Melanoma: melanoma markers+ (S100, HMB45, MART, MITF, MelanA, SOX10, tyrosinase)
  • Other round blue cell tumors, e.g.
    • Alveolar rhabdomyosarcoma: desmin+, myogenin+, myoD1+
    • Desmoplastic small round cell tumor (DSRCT): striking desmoplasia, coexpression of cytokeratin/desmin, WT1+
    • Glomus tumor: round to polygonal cells with scant cytoplasm and marked cellularity uniformity; SMA+, desmin-, S100-
    • Neuroendocrine (small cell) carcinoma: neuroendocrine markers+ (CD56, chromogranin, synaptophysin)
  • Poorly differentiated carcinoma: focal cytokeratin+