Rare Diseases Symptoms Automatic Extraction

Cervical dedifferentiated liposarcoma with meningothelial-like whorling.

[dedifferentiated liposarcoma]

Liposarcomas are the most common soft-tissue sarcoma of adults, but rare in the head and neck. Recently, a subtype of dedifferentiated liposarcoma with meningiothelial-like whorls was reported and we present the first description of such a tumor in the head and neck. A 65 year old male underwent a resection of a calcified retroesophageal mass that was in close relation to the left hemithyroid and recurrent laryngeal nerve. It was resected en bloc with the left thyroid lobe. Initial pathologic evaluation suggested the mass was a schwanomma of the recurrent laryngeal nerve, but positive staining for MDM2 and CDK4 indicated the tumor was a dedifferentiated liposarcoma. Further evaluation elucidated the unique meningothelial-like whorls within the tumor. This case demonstrates dedifferentiated liposarcomas do appear in the head and neck. Furthermore, this is the first report in the head and neck of the mengiothelial-like whorling pattern type of dedifferentiated liposarcoma.

Diseases presenting "first description" symptom

  • cystinuria
  • dedifferentiated liposarcoma
  • erythropoietic protoporphyria
  • kabuki syndrome
  • monosomy 21
  • oral submucous fibrosis
  • phenylketonuria
  • systemic capillary leak syndrome

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