Esophagus
Carcinoma
Spindle cell (squamous) carcinoma


Topic Completed: 1 January 2014

Revised: 27 February 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Spindle cell squamous carcinoma esophagus


Elliot Weisenberg, M.D.
Page views in 2018: 1,040
Page views in 2019 to date: 1,129
Cite this page: Weisenberg E. Spindle cell (squamous) carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagussarcomatoid.html. Accessed December 14th, 2019.
Definition / general
  • Squamous cell carcinoma with a variable spindle cell component (WHO definition)
Terminology
  • WHO uses the term "spindle cell (squamous) carcinoma"
  • Spindle cell carcinoma (Odze) and sarcomatoid carcinoma (Rosai) are also commonly used
  • Synonyms include carcinosarcoma, pseudosarcomatous squamous cell carcinoma, polypoid carcinoma, metaplastic carcinoma, squamous cell carcinoma with a spindle cell component, Lane tumor, carcinoma with mesenchymal stroma and pseudosarcoma
Epidemiology
  • Uncommon, usually middle aged to elderly men
Sites
  • Typically in mid esophagus
Pathophysiology
  • In the vast majority of cases the epithelial component is squamous and the lesion is generally regarded as a variant of squamous cell carcinoma
  • Very rare examples of spindle cell carcinoma associated with adenocarcinoma are known
  • Spindle cell component dominates, has growth advantage over epithelial component, although both evolve from same clone
Clinical features
  • Patients generally present with short history of dysphagia, weight loss and pain
Diagnosis
  • Endoscopic biopsy or resection
Radiology description
  • Large polypoid lesion, typically with narrow pedicle leads to characteristic "cupola" sign
Prognostic factors
  • Better survival than typical squamous cell carcinoma, overall survival about 50%
  • Tends to present at low stage with intraluminal instead of intramural growth
  • 30% have nodal metastases at presentation (usually epithelial or both components)
  • Sarcomatous component thought to represent sarcomatous metaplasia of malignant epithelial cells; has malignant potential
Case reports
Gross description
  • Bulky, exophytic and polypoid mass that grows into lumina, often with short pedicle, mean 6 cm
  • Surrounding mucosa is grossly normal
Gross images

Contributed by Dr. Mark R. Wick

Rhabdomyosarcomatous differentiation

Microscopic (histologic) description
  • Biphasic carcinomatous and spindle cell components
  • Spindle cells predominate, are elongated with blunt nuclei and atypia resembling undifferentiated pleomorphic sarcoma (MFH)
  • May have bizarre giant cells, bone, cartilage, strap cells
  • Stroma is edematous with scattered or abundant collagen and mucopolysaccharides
  • Epithelial component usually is squamous or basaloid, rarely adenocarcinoma and often is limited with only superficial invasion or in situ disease
  • Prominent mitotic activity
  • May have neuroendocrine differentiation
  • Metastasis may have one or both components
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick

With rhabdomyosarcomatous differentiation



AFIP images

Pseudosarcomatous squamous cell carcinoma

Positive stains
Negative stains
Electron microscopy description
  • Dilated cisternae of rough endoplasmic reticulum, peripheral cytoplasmic intermediate filaments
  • Also some intermediate type junctions and subplasmalemmal linear densities
  • Sarcomatous cells may retain epithelial features including tonofibril bundles or desmosomes or lack epithelial elements and have fibrohistiocytic differentiation (Hum Pathol 1987;18:692)
  • Spindle cells may resemble myofibroblasts
Molecular / cytogenetics description
Differential diagnosis
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