Esophagus
Carcinoma
Sarcomatoid carcinoma
Author: Elliot Weisenberg, M.D.
Last author update: 1 January 2014
Last staff update: 12 December 2023
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: Spindle cell squamous carcinoma esophagus
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Weisenberg E. Sarcomatoid carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophagussarcomatoid.html. Accessed December 27th, 2024.
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
- 46 year old man whose tumor had extensive osseous differentiation (Indian J Pathol Microbiol 2003;46:49)
- 57 year old man with polypoid tumor (Am J Surg Pathol 1978;2:201)
- 64 year old man with esophageal carcinosarcoma with basaloid squamous carcinoma and rhabdomyosarcoma components with p53 mutation (Pathol Int 2004;54:803)
- Polypoid squamous carcinoma of the esophagus (Am J Surg Pathol 1983;7:495)
- So called pseudosarcoma of the esophagus (Arch Pathol Lab Med 1977;101:604)
- True carcinosarcoma of the esophagus (Dis Esophagus 2006;19:48)
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
- Derived from single clone (Hum Pathol 2004;35:322), sarcoma component is more often aneuploid than carcinoma component (Hum Pathol 1998;29:863)
Differential diagnosis
- Gastrointestinal stromal tumor
- Leiomyosarcoma
- Melanoma
- Sarcoma: must section extensively to rule out presence of epithelial component
