Esophagus
Carcinoma
Spindle cell (squamous) carcinoma

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 24 January 2018, last major update January 2014

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

PubMed Search: Spindle cell squamous carcinoma esophagus

Cite this page: Weisenberg, E. Spindle cell (squamous) carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagussarcomatoid.html. Accessed May 27th, 2018.
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

Images hosted on PathOut server:

Images contributed by Dr. Mark R. Wick:

Rhabdomyo-sarcomatous 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

Images hosted on PathOut server:

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