Esophagus
Other tumors
Leiomyosarcoma
Authors: Feriyl Bhaijee, M.D., Israh Akhtar, M.D.
Topic Completed: 1 October 2013
Minor changes: 18 December 2020
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Leiomyosarcoma[TI] esophagus
Table of Contents
Definition / general | Epidemiology | Sites | Clinical features | Diagnosis | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Bhaijee F, Akhtar I. Leiomyosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophagusLMS.html. Accessed January 24th, 2021.
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:
- Mediastinal mass (AJR Am J Roentgenol 1996;167:27)
- 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
- Leiomyosarcoma with overlying squamous cell carcinoma (World J Gastroenterol 2013;19:5385)
- 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
- 52 year old man with leiomyosarcoma arising in a remnant esophagus after esophagectomy (Jpn J Clin Oncol 2001;31:517)
- 56 year old woman with leiomyosarcoma metastatic to eyelid (Ophthal Plast Reconstr Surg 2002;18:159)
- 61 year old woman with leiomyosarcoma and chagasic megaesophagus (Am J Trop Med Hyg 1999;60:879)
- 72 year old man with rapidly progressing leiomyosarcoma combined with squamous cell carcinoma (World J Gastroenterol 2013;19:5385)
- 75 year old man with concomitant leiomyoma and leiomyosarcoma (J Exp Clin Cancer Res 2005;24:487)
Treatment
- Radical esophagectomy
Clinical images
Images hosted on other servers:
Intraluminal polypoid lesion
Intraluminal polypoid mass
Gross description
- Polypoid / pedunculated, infiltrative or intramural mass
- Variable hemorrhage and necrosis
Gross images
AFIP images
Sarcoma
Images hosted on other servers:
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
Images hosted on other servers:
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
Positive stains
- Smooth muscle markers: smooth muscle actin, desmin, calponin, caldesmon
- IMP3 (50%, Human Pathology 2012;43:1567)
- Focal cytokeratin (rarely)
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-
