Esophagus
Other malignancies
Leiomyosarcoma

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

Revised: 30 January 2018, last major update October 2013

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

PubMed Search: Leiomyosarcoma[TI] esophagus

Cite this page: Bhaijee, F. Leiomyosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusLMS.html. Accessed October 16th, 2018.
Definition / general
  • Malignant smooth muscle tumor of the esophagus
    • Benign counterpart is leiomyoma
Epidemiology
  • Rare: < 1% of all esophageal cancers but most common esophageal sarcoma
  • Wide age range (26 - 76 years), median age 58 years
  • No gender predilection
Sites
  • Cervical, mid or lower esophagus
  • Likely arises from muscularis mucosae / propria
Clinical features
  • Dysphagia, odynophagia, pain (epigastric, retrosternal, back), weight loss, upper GI hemorrhage
  • Slow growth and late metastases (Dis Esophagus 2014;27:547)
Diagnosis
  • Histopathologic evaluation with IHC confirmation
Radiology description
  • Chest Xray:
  • Barium studies:
    • Intramural lesions with large exophytic components, variable ulceration / tracking
    • Intraluminal lesions: expansile masses, may be polypoid
    • Infiltrative lesions
    • With esophageal stricture
    • Arising in an esophageal remnant following esophagectomy / radiotherapy for SCC (Jpn J Clin Oncol 2001;31:517)
  • CT:
    • Heterogeneous lesions with large exophytic components and central low density areas
  • MRI:
    • Large masses, isointense with skeletal muscle on T1 weighted images, hyperintense on T2 weighted images
  • Radial EUS:
    • Intramural, hypoechoic, heterogeneous mass with transmural involvement
Radiology images

Images hosted on other servers:

Dilated thoracic esophagus

Lower esophageal wall thickening

Round, smooth mass in upper esophagus

Large, homogeneously enhancing soft tissue mass

Intense segmental fluorodeoxyglucose uptake

Prognostic factors
  • Factors affecting survival: (Ann Thorac Surg 1998;66:894)
    • Completeness of resection
    • Growth pattern (infiltrative pattern has worse prognosis than polypoid / intramural tumors)
    • Postsurgical stage
    • Tumor grade
    • Tumor location (cervical location confers worse prognosis than thoracic location)
Case reports
Treatment
  • Radical esophagectomy
Clinical images

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Intraluminal polypoid lesion

Intraluminal polypoid mass

Gross description
  • Polypoid / pedunculated, infiltrative or intramural mass
  • Variable hemorrhage and necrosis
Gross images

Images hosted on PathOut server:

Sarcoma



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Resected specimen

Nodular tumor

Microscopic (histologic) description
  • Highly cellular intersecting fascicles of spindled tumor cells
  • Eosinophilic fibrillary cytoplasm; elongated, cigar shaped nuclei with occasional paranuclear vacuoles
  • Abundant pleomorphism (cytologic atypia), mitotic activity and necrosis
Microscopic (histologic) images

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Leiomyosarcoma

Moderately pleomorphic, mitotically active nuclei

Pleomorphic spindle cells

Arising in remnant esophagus

Cytology description
  • Cellular fragments of spindle cells in fascicles
  • Cigar shaped nuclei with blunted ends, cellular atypia, increased mitotic activity
Cytology images

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Case from Papanicolaou Society

Positive stains
Negative stains
Electron microscopy description
  • Features of smooth muscle differentiation: thin filaments, pinocytic vesicles, attachment plaques, interrupted external lamina
Molecular / cytogenetics description
  • No recurrent molecular aberrations reported
Differential diagnosis
  • Other mesenchymal tumors
    • GIST: CD117+, DOG1+, CD34+; focal SMA+, focal desmin+, focal S100+
    • Glomus tumor: round to polygonal cells with scant cytoplasm and marked cellularity uniformity; SMA+, desmin-, S100-
    • Granular cell tumor: large tumor cells with abundant granular cytoplasm, S100+, SMA-, desmin-
    • Leiomyoma: less cellularity, no / rare atypia, mitotic activity or necrosis; smooth muscle markers+ but IMP3-
    • Schwannoma: S100+, SMA-, desmin-