Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Clinical features | Diagnosis | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Nakanishi Y. Giant fibrovascular polyp / well differentiated liposarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophagusfibrovascularpolyp.html. Accessed January 20th, 2025.
Definition / general
- Giant fibrovascular polyp used to be considered a nonneoplastic polypoid lesion of the esophagus
- The majority of giant fibrovascular polyps of the esophagus now appear to represent well differentiated and dedifferentiated liposarcoma, based on the result of MDM2 FISH (fluorescence in situ hybridization) testing (Mod Pathol 2018;31:337)
Essential features
- Pedunculated polypoid (sausage shaped) mass, up to 23 cm in length (average 13 cm) (World J Gastrointest Oncol 2016;8:835)
- Consists of fibroadipose tissue with slightly enlarged, hyperchromatic stromal cells (Mod Pathol 2018;31:337)
- MDM2 and CDK4 overexpression by immunohistochemistry and amplification by FISH, indicating diagnosis of liposarcoma (Mod Pathol 2018;31:337)
Terminology
- Giant fibrovascular polyp, fibrovascular polyp, well differentiated liposarcoma, atypical lipomatous tumor
Epidemiology
- Approximately 0.5% of all esophageal neoplasms (World J Gastrointest Oncol 2016;8:835)
- Middle aged to elderly patients with male predominance
Sites
- Proximal / cervical esophagus (Int J Surg Case Rep 2019;64:113)
Clinical features
- Middle aged to elderly patients with male predominance (Int J Surg Case Rep 2019;64:113)
- Slowly worsening dysphagia, weight loss, odynophagia and oral regurgitation of polyp
Diagnosis
- Characteristic radiological and endoscopic appearances, plus characteristic histologic findings with immunohistochemical / molecular confirmation
Prognostic factors
- Dedifferentiated type tends to recur and metastasize (Mod Pathol 2018;31:337)
Case reports
- 36 year old woman with an 11 cm giant fibrovascular polyp (Gastrointest Endosc 2020;91;442)
- 42 year old man with a 14 cm giant fibrovascular polyp (Acta Gastroenterol Belg 2019;82:437)
- 42 year old man with an 8 cm giant fibrovascular polyp (ACG Case Rep J 2019;6:e00126)
- 54 year old man with a 17.5 cm giant pedunculated liposarcoma (Int J Surg Case Rep 2019;64:113)
- 75 year old man with a 6.5 cm pedunculated, well differentiated liposarcoma (Ann R Coll Surg Engl 2017;99:e209)
Treatment
- Endoscopic or surgical resection
Gross description
- Pedunculated polypoid (sausage shaped) mass up to 23 cm in length (average 13 cm) (World J Gastrointest Oncol 2016;8:835)
Microscopic (histologic) description
- Fibroadipose tissue with slightly enlarged, hyperchromatic stromal cells, covered with intact squamous mucosa (Mod Pathol 2018;31:337)
- Mostly well differentiated type / atypical lipomatous tumor (Mod Pathol 2018;31:337)
- Occasionally dedifferentiated type (Mod Pathol 2018;31:337)
Microscopic (histologic) images
Molecular / cytogenetics description
- MDM2 and CDK4 overexpression by immunohistochemistry (World J Gastrointest Oncol 2016;8:835)
- MDM2 and CDK4 amplification by FISH (Mod Pathol 2018;31:337)
- Ring or marker chromosomes with regional amplification of chromosome 12q13-21 (Hum Pathol 2012;43:293)
Sample pathology report
- Esophagus, mass, endoscopic resection:
- Well differentiated liposarcoma / giant fibrovascular polyp (see comment)
- Resection margin negative for liposarcoma
- Comment: The slightly atypical stromal cells are positive for MDM2 and CDK4 by immunohistochemistry with appropriate controls. The results support the above diagnosis.
Differential diagnosis
- Lipoma:
- Carcinosarcoma:
- Pedunculated tumor
- Biphasic tumor with carcinomatous and sarcomatous components
Board review style question #1
Which of the following is true about this mass shown in the picture?
- Despite large size of the tumor, patients never present with dysphagia
- Gross features suggest an aggressive tumor
- Patients need an aggressive treatment, including chemotherapy and radiation
- This polypoid tumor is usually seen in the proximal / cervical esophagus
Board review style answer #1
D. Giant fibrovascular polyp / well differentiated liposarcoma is usually seen in the proximal / cervical esophagus
Comment Here
Reference: Giant fibrovascular polyp / well differentiated liposarcoma
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Reference: Giant fibrovascular polyp / well differentiated liposarcoma
Board review style question #2
Which of the following is true about giant fibrovascular polyp of the esophagus?
- Most show MDM2 amplification
- They represent a reactive / nonneoplastic mass of the esophagus
- They usually are composed of markedly pleomorphic, hyperchromatic stromal cells
- They usually are seen in young female patients
Board review style answer #2
A. Most giant fibrovascular polyps show MDM2 amplification and CDK4 amplification supporting the diagnosis of well differentiated liposarcoma
Comment Here
Reference: Giant fibrovascular polyp / well differentiated liposarcoma
Comment Here
Reference: Giant fibrovascular polyp / well differentiated liposarcoma