Well-Differentiated/Dedifferentiated Liposarcoma Arising in the Upper Aerodigestive Tract: 8 Cases Mimicking Non-adipocytic Lesions

Fritchie, Karen; Ghosh, Toshi; Graham, Rondell P.; Roden, Anja C.; Schembri-Wismayer, David; Folpe, Andrew L.; Rivera, Michael

doi:10.1007/s12105-020-01171-x

Cited by 15 publications

(24 citation statements)

References 52 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Few cases had been described to undergo dedifferentiation after recurrence [5,6,10]. Hypopharyngeal WDL has predilection for middle aged males and uncommonly arise in individuals younger than 40 years [3][4][5][6][7][8][9][10]. In our case, the patient was only 36 years old, which is a rather unusual finding.…”

Section: Discussionmentioning

confidence: 69%

“…Its occurrence in the hypopharynx is exceedingly rare with approximately 40 cases reported in the literature [4][5][6][7][8][9][10]. Complete surgical resection is difficult to achieve at this site, therefore, the clinical course of hypopharyngeal WDL is typically characterized by multiple local recurrences, however, distant metastasis has not been reported [4][5][6][7][8][9][10]. Few cases had been described to undergo dedifferentiation after recurrence [5,6,10].…”

Section: Discussionmentioning

confidence: 99%

“…Complete surgical resection is difficult to achieve at this site, therefore, the clinical course of hypopharyngeal WDL is typically characterized by multiple local recurrences, however, distant metastasis has not been reported [4][5][6][7][8][9][10]. Few cases had been described to undergo dedifferentiation after recurrence [5,6,10]. Hypopharyngeal WDL has predilection for middle aged males and uncommonly arise in individuals younger than 40 years [3][4][5][6][7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

“…ATL is the preferred terminology for tumors that arise at sites amenable to surgical resection such as the extremities and trunk, though WDL is the terminology used for tumors at deep-seated locations such as the retroperitoneum and mediastinum [1][2][3]. Hypopharyngeal/laryngeal liposarcomas are exceedingly rare [4][5][6][7][8][9][10]. Most reported cases are of well-differentiated or de-differentiated types [4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

“…Hypopharyngeal/laryngeal liposarcomas are exceedingly rare [4][5][6][7][8][9][10]. Most reported cases are of well-differentiated or de-differentiated types [4][5][6][7][8][9][10]. Occasionally, some liposarcomas show combined morphologic features of two or more subtypes [1,2].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Well-Differentiated Liposarcoma of the Hypopharynx Exhibiting Myxoid Liposarcoma-like Morphology with MDM2 and DDIT3 Co-Amplification

Murshed

Samra

Ammar

2021

Head and Neck Pathol

View full text Add to dashboard Cite

Well-differentiated liposarcoma (WDL) is one of the most common soft tissue sarcomas in adults. It has a predilection for middle-aged males and arises in deep-seated locations such as retroperitoneum, mediastinum, and spermatic cord. Its occurrence in young individuals at the hypopharyngeal region is an exceedingly rare event. Myxoid liposarcoma (ML)-like changes can seldom occur in some cases of WDL, which makes the diagnosis of WDL more challenging. Amplification of DDIT3 gene in a subset of cases of WDL has shown to be associated with such unique morphology. Herein, we present a case of a 36-year-old gentleman who presented with difficulty in breathing and swallowing for 3 months duration. CT scan of the neck revealed a lesion along the posterior wall of the hypopharynx measuring 3.5 cm. Histopathologic examination revealed a tumor composed of lobules of oval to spindle cells in a prominent myxoid stroma with delicate chicken-wire vasculature. In the vicinity, there were lobules composed of variably sized adipocytes separated by thick fibrous septa that contains atypical hyperchromatic spindle cells. By immunohistochemistry, the tumor cells in both components were immunoreactive for CDK4, but negative for MDM2. Fluorescence in-situ hybridization (FISH) confirmed the presence of MDM2 gene amplification. There was no evidence of FUS-DDIT3 gene rearrangement, however, DDIT3 gene was also amplified. The diagnosis of well-differentiated liposarcoma with prominent myxoid stroma was rendered. This is the first documentation of WDL with ML-like morphology harboring co-amplification of MDM2 and DDIT3 in the hypopharynx.

show abstract