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Relapsing polychondritis associated with bilateral stapes footplate fixation: a case report
Authors:Yohanna M Takwoingi
Institution:1. Department of Neurosciences - Section of Neurosurgery, University of Bari Medical School, Bari, Italy
2. Department of Biological and Environmental Sciences and Technologies (DiSTeBA), Ecotekne, Via Prov.le per Monteroni, 73100, Lecce, Italy
3. CNR-IFC Lecce section, Ecotekne, Via Prov.le per Monteroni, 73100, Lecce, Italy
4. Department of Pathology - Section of Neuropathology, University of Bari Medical School, Bari, Italy
5. Department of Clinical Immunology and Allergology, Sant'Anrea Hospital, Rome, Italy
6. Neurosurgery Operative Unit, V. Fazzi Hospital, 73100, Lecce, Italy
Abstract:

Introduction

Two types of gliomatosis cerebri exist: Type I and Type II. We report the results of a histological and genetic study of two cases of gliomatosis cerebri Type II, correlating these results with therapy and prognosis.

Case presentation

Two patients, a 52-year-old man (Patient 1) and a 76-year-old man (Patient 2) with gliomatosis cerebri II were admitted to our institution; they underwent surgical treatment and received radiotherapy and chemotherapy. At the 24-month follow-up, Patient 1 was still alive, while Patient 2 had died. The poor prognosis of Patient 2 was underlined by molecular analysis which showed that the angiogenesis related genes VCAM1 and VEGF were overexpressed, reflecting the high degree of neovascularization.

Conclusion

Genes involved in drug resistance and metallothioneins were highly expressed in Patient 2 and this, associated with unmethylated O6-methylguanine methyltransferase, can explain the lack of response to chemotherapy.
Keywords:
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