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Case Reports
. 2020 Apr;42(3):218-219.
doi: 10.1097/MPH.0000000000001721.

A Nonchordomatous-looking Chordoma: When INI-1 and Radiology Came to the Rescue!!!

Angad Singh  1 , Anirban Das  2 , Argha Chatterjee  3 , Rimpa B Achari  4 , Reghu K S  2 , Paromita Roy  1
Affiliations
Case Reports

A Nonchordomatous-looking Chordoma: When INI-1 and Radiology Came to the Rescue!!!

Angad Singh et al. J Pediatr Hematol Oncol. 2020 Apr.

Abstract

SMARCB1/integrase interactor (INI)-1 is one of the core subunit proteins of the ATP-dependent SWI/SNF chromatin remodeling complex and acts as a tumor suppressor. INI-1 loss can be easily assessed using immunohistochemistry and is an important diagnostic clue for a histopathologist. Chordoma is a malignant tumor commonly occurring in the sacrococcygeal spine of adults and is characterized by nuclear expression of brachyury. Poorly differentiated chordoma, a morphologically and molecularly distinct entity, also shows nuclear brachyury positivity along with INI-1 loss. It usually occurs in children and has a predilection to involve the base of the skull. We describe a case of a poorly differentiated chordoma in a 5-year-old girl and discuss its unusual histomorphologic and immunohistochemical features.

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References

    1. Kohashi K, Oda Y. Oncogenic roles of SMARCB 1/INI 1 and its deficient tumors. Cancer Sci. 2017;108:547–552.
    1. Hasselblatt M, Thomas C, Hovestadt V, et al. Poorly differentiated chordoma with SMARCB1/INI1 loss: a distinct molecular entity with dismal prognosis. Acta Neuropathol. 2016;132:149–151.
    1. Mobley BC, McKenney JK, Bangs CD, et al. Loss of SMARCB1/INI1 expression in poorly differentiated chordomas. Acta Neuropathol. 2010;120:745–753.
    1. Shih AR, Cote GM, Chebib I, et al. Clinicopathologic characteristics of poorly differentiated chordoma. Mod Pathol. 2018;31:1237–1245.
    1. Kalimuthu SN, Chetty R. Gene of the month: SMARCB1. J Clin Pathol. 2016;69:484–489.

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