Laryngopharyngeal Liposarcoma: A (Somewhat Docile) Wolf in Sheep’s Clothing
摘要
Adipocytic tumors of the head and neck are mostly benign, but laryngopharyngeal tumors are uncommon and poorly characterized. We compared the clinicopathologic profile of laryngopharyngeal liposarcomas (LP-LPS) and lipomas (LP-L) across two institutions.
DesignHead and neck adipocytic tumors were queried for LP-LPS and LP-L. Demographic, pathologic, immunophenotypic features (including MDM2 amplification status), treatment and outcomes were reviewed.
ResultsLPS constituted 1.4% (n = 22/1584) of head and neck adipocytic lesions with a disproportionate representation at laryngopharyngeal sites in both cohorts (8/19, 42.1%; 2/3, 66.7%). LPS comprised a higher proportion of laryngopharyngeal tumors as compared to other site groups (8/18, 44.4%; 2/2, 100%). Adding personal consult cases yielded 12 LP-LPS and 10 LP-L. Only atypical hyperchromatic cells, and MDM2 status differed significantly between groups (adjusted p = 0.037 and < 0.001 respectively). Recurrences were more frequent in LP-LPS (6/11, 54.5%) than LP-L (1/8, 12.5%) with a median disease-free survival of 83.5 months for LP-LPS. Most LP-LPS (10/12, 83.3%) were well differentiated liposarcomas (WDLPS) with two being dedifferentiated liposarcomas (DDLPS). Six of 10 LP-L were conventional while 4 were spindle cell lipomas (SCL). Only 2/10 (20%) of the WDLPS were initially diagnosed as such, with SCL being the most common initial diagnosis (3/10, 30%). Two cases (20%) had prominent angiomatous change. The sclerosing subtype predominated (n = 8/12, 66.7%) often SCL-like. One DDLPS had a pleomorphic rhabdomyosarcoma component while the other was an undifferentiated pleomorphic/spindle cell sarcoma.
ConclusionHead and neck LPS are rare but disproportionately represented at laryngopharyngeal sites both as a proportion of head and neck liposarcomas and proportion of adipocytic tumors by site. LP-LPS share similarities to LP-L, and are easily mistaken for SCL, inflammatory or vascular lesions. While atypical hyperchromatic cells are discriminatory between LP-LPS and LP-L, MDM2 status is more robust and strongly recommended when encountering an adipocytic (particularly SCL-like) or otherwise unclassifiable fibroinflammatory/vascular lesion in laryngopharyngeal sites. LP-LPS tumors often recur locally but are more indolent and somewhat less prone to dedifferentiation than their retroperitoneal counterparts.