Divergent myoid, neuroendocrine, and perineural differentiation in a nasal tumor of a patient with Carney complex Journal Article


Authors: Dao, L. N.; Scheithauer, B. W.; Erlandson, R. A.; Young, W. F. Jr; Carney, J. A.
Article Title: Divergent myoid, neuroendocrine, and perineural differentiation in a nasal tumor of a patient with Carney complex
Abstract: A 39-year-old woman with Carney complex presented with a stroke of undetermined etiology. Computed tomography showed bilateral thalamic infarctions and also an unsuspected multicompartmental cystic neoplasm that had eroded the anterior clivus and extended forward into the nasopharynx. Histologically, the mass appeared benign and was composed of spindle cells and multiple foci of striated muscle. Immunohistochemically, the spindle cells were strongly reactive for S-100 protein and to a lesser extent for CD57, collagen IV, neuron-specific enolase, smooth muscle actin, epithelial membrane antigen, and glut-1. The striated muscle cells were positive for desmin and myogenin. The MIB-1 labeling index was 0.5%. Ultrastructural examination was necessary to reveal the full extent of divergent differentiation. Ultrastructurally, the spindle cells showed divergent differentiation along several cell lines, including smooth muscle, neuroendocrine, hybrid smooth muscle-neuroendocrine, perineural-like cells, and striated muscle. The occurrence of this unique lesion in a patient with the Carney complex raises the possibility that it may be a rare component of the syndrome. © 2008 Lippincott Williams & Wilkins, Inc.
Keywords: immunohistochemistry; adolescent; adult; human tissue; middle aged; clinical feature; histopathology; case report; ki 67 antigen; carney complex; disease association; smooth muscle fiber; tumor localization; cell differentiation; stroke; spindle cell; protein s 100; syndrome delineation; perineurium; tumor; nerve cell; divergent differentiation; nose tumor; nose neoplasms; desmin; myogenin; glucose transporter 1; epithelial membrane antigen; perineum; collagen type 4; neuron specific enolase; smooth muscle actin; cd57 antigen; neuroendocrine; microscopy, electron, transmission; neoplasms, muscle tissue; neurosecretory cell; neoplastic syndromes, hereditary; cell ultrastructure; perineural
Journal Title: American Journal of Surgical Pathology
Volume: 32
Issue: 1
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2008-01-01
Start Page: 167
End Page: 171
Language: English
DOI: 10.1097/PAS.0b013e31813c0e11
PUBMED: 18162785
PROVIDER: scopus
DOI/URL:
Notes: --- - "Export Date: 17 November 2011" - "CODEN: AJSPD" - "Source: Scopus"
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  1. Robert A Erlandson
    125 Erlandson