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Well-Differentiated/Dedifferentiated Liposarcoma Arising in the Upper Aerodigestive Tract: 8 Cases Mimicking Non-adipocytic Lesions
- Source :
- Head Neck Pathol
- Publication Year :
- 2020
-
Abstract
- Well-differentiated (WDL) and dedifferentiated liposarcomas (DL) of the pharynx, larynx and oral cavity are rare, often mimicking benign lipomatous neoplasms or non-lipogenic mesenchymal tumors. Cases of WDL/DL arising in the upper aerodigestive tract, exclusive of the cervical esophagus, were reviewed. Morphologic features, ancillary studies, including fluorescence in situ hybridization (FISH) studies for CPM/MDM2, and clinical data was catalogued. Eight WDL/DL (4 WDL, 4 DL); were identified in patients ranging from 32 to 77 years (median 52.5 years; 6 males, 2 females) with sites of origin including hypopharynx (5 cases), larynx (2 cases) and oral cavity (1 case). Six of the 8 cases were received for expert consultation, and the remaining 2 cases were initially misdiagnosed as benign lymphangiomatous or fibroepithelial polyps. Morphologically, 4 tumors had areas mimicking various non-lipomatous soft tissue tumors including nodular fasciitis, mammary-type myofibroblastoma, low-grade myofibroblastic sarcoma and undifferentiated pleomorphic sarcoma, 2 cases simulated benign hypopharyngeal polyps, and 1 lesion was notable for a dense lymphoplasmacytic infiltrate suggestive of hematolymphoid neoplasm or IgG4-related sclerosing disease. FISH showed amplification of CPM/MDM2 (8/8 cases). All cases (4/4) with longer than 1-year of follow-up recurred (45-118 months) with 1 tumor showing progression to DL. WDL/DL presenting in the upper aerodigestive tract are rare and diagnostically challenging. Awareness of the morphologic spectrum of WDL/DL coupled with appropriate use of MDM2 FISH is essential for accurate classification and management, as these tumors appear to have a high risk for local recurrence and eventual dedifferentiation in these anatomical locations.
- Subjects :
- 0301 basic medicine
Larynx
Adult
Male
Pathology
medicine.medical_specialty
Nodular fasciitis
Liposarcoma
Undifferentiated Pleomorphic Sarcoma
Pathology and Forensic Medicine
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
Medicine
Humans
Laryngeal Neoplasms
Aged
Original Paper
business.industry
Pharynx
Pharyngeal Neoplasms
Middle Aged
medicine.disease
030104 developmental biology
medicine.anatomical_structure
Oncology
Otorhinolaryngology
030220 oncology & carcinogenesis
Female
Mouth Neoplasms
Lipomatous Neoplasm
Sarcoma
business
Myofibroblastoma
Subjects
Details
- ISSN :
- 19360568
- Volume :
- 14
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Head and neck pathology
- Accession number :
- edsair.doi.dedup.....fd406a93fbac65f755ae0d7994fb0e5b