Literature DB >> 18673443

Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex.

Hidehiro Takei1, Adekunle M Adesina, Suzanne Z Powell.   

Abstract

Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare.

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Year:  2008        PMID: 18673443     DOI: 10.1111/j.1440-1789.2008.00941.x

Source DB:  PubMed          Journal:  Neuropathology        ISSN: 0919-6544            Impact factor:   1.906


  4 in total

1.  Intraoperative Squash Cytologic Features of Subependymal Giant Cell Astrocytoma.

Authors:  Jitendra Nasit; Viren Vaghsiya; Srilaxmi Hiryur; Smita Patel
Journal:  J Lab Physicians       Date:  2016 Jan-Jun

Review 2.  A case of subependymal giant cell astrocytoma without tuberous sclerosis complex and review of the literature.

Authors:  Michael O'Rawe; Arjun S Chandran; Stuti Joshi; Alexandre Simonin; Jason M Dyke; Sharon Lee
Journal:  Childs Nerv Syst       Date:  2020-08-17       Impact factor: 1.475

3.  Identification of TSC1 or TSC2 mutation limited to the tumor in three cases of solitary subependymal giant cell astrocytoma using next-generation sequencing technology.

Authors:  Martine Fohlen; Ines Harzallah; Marc Polivka; Fabienne Giuliano; Linda Pons; Nathalie Streichenberger; Georg Dorfmüller; Renaud Touraine
Journal:  Childs Nerv Syst       Date:  2020-02-26       Impact factor: 1.475

4.  Subependymal giant cell astrocytomas in Tuberous Sclerosis Complex have consistent TSC1/TSC2 biallelic inactivation, and no BRAF mutations.

Authors:  Anika Bongaarts; Krinio Giannikou; Roy J Reinten; Jasper J Anink; James D Mills; Floor E Jansen; G M Wim Spliet; Willfred F A den Dunnen; Roland Coras; Ingmar Blümcke; Werner Paulus; Theresa Scholl; Martha Feucht; Katarzyna Kotulska; Sergiusz Jozwiak; Anna Maria Buccoliero; Chiara Caporalini; Flavio Giordano; Lorenzo Genitori; Figen Söylemezoğlu; José Pimentel; Mark Nellist; Antoinette Y N Schouten-van Meeteren; Anwesha Nag; Angelika Mühlebner; David J Kwiatkowski; Eleonora Aronica
Journal:  Oncotarget       Date:  2017-09-08
  4 in total

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