title: Malignant extra-renal rhabdoid tumor with unusual presentation: A report of two cases.
abstract: Malignant extra-renal rhabdoid tumor (MERT) is a rare highly aggressive tumor that occurs in young children with the very poor clinical outcome. The tumor is characterized by a diffuse proliferation of "rhabdoid cells," which are round or polygonal with eccentric nuclei, prominent nucleoli, and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies. However, rhabdoid cells are also seen in certain other soft tissue sarcomas such as proximal-type epithelioid sarcoma, rarely synovial sarcoma, and extra-skeletal myxoid chondrosarcoma. Because of its poor prognosis and histomorphological similarities with other soft tissue tumors, an accurate diagnosis is required using a wide immunohistochemical panel. Very few cases of MERT have been reported in the literature and to our knowledge none in the supra-glottis area. Due to the rarity and poor outcome of this tumor, we are reporting two cases of MERT.
authors.forename: Rakesh Kumar
authors.initials: RK
authors.lastname: Gupta
authors.affiliation: Department of Pathology, G B Pant Hospital, New Delhi, India.
authors.forename: Vineeta V
authors.initials: VV
authors.lastname: Batra
authors.forename: Mridul Chandra
authors.initials: MC
authors.lastname: Das
authors.forename: Ajay
authors.initials: A
authors.lastname: Sharma
authors.forename: Poonam
authors.initials: P
authors.lastname: Narang
mesh_terms: D002675:Child, Preschool; D005260:Female; D005931:Glottis / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D018335:Rhabdoid Tumor / Q000150:complications / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000150:complications / Q000473:pathology*; D013125:Spinal Neoplasms / Q000150:complications / Q000473:pathology*