eISSN: 2084-9869
ISSN: 1233-9687
Polish Journal of Pathology
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SCImago Journal & Country Rank
2/2021
vol. 72
 
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abstract:
Case report

Complex grade III astrocytoma with granular cells and PXA features in a patient with tuberous sclerosis complex (Bourneville-Pringle syndrome) – case report and review of literature

Anna Mucha-Małecka
1
,
Krzysztof Małecki
2, 3
,
Michał Kurzyński
4
,
Dariusz Adamek
5
,
Beata Biesaga
6, 7
,
Konrad Urbanek
1
,
Aleksandra Grela-Wojewoda
8
,
Elżbieta Pluta
1
,
Anna Patla
1
,
Maciej Modrzejewski
1
,
Paweł Blecharz
9
,
Natalia Amrogowicz
10

1.
Department of Radiotherapy, Maria Sklodowska-Curie National Research Institute of Oncology, Krakow Branch, Poland
2.
Department of Radiotherapy for Children and Adults, University Children’s Hospital of Krakow, Poland
3.
Jagiellonian University in Cracow, Faculty of Health Sciences, Krakow, Poland
4.
Amethyst Radiotherapy Poland, Krakow, Poland
5.
Department of Pathology, Jagiellonian University Medical College, Krakow, Poland
6.
Center for Translational Research and Molecular Biology of Cancer, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
7.
Department of Tumour Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, Krakow Branch, Poland
8.
Department of Clinical Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Krakow Branch, Poland
9.
Gynecologic Oncology Department, Maria Sklodowska-Curie National Research Institute of Oncology, Krakow Branch, Poland
10.
I Radiation and Clinical Oncology Department, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
Pol J Pathol 2021; 72 (2): 180-184
Online publish date: 2021/09/30
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Tuberous sclerosis complex (Bourneville-Pringle syndrome) is a rare genetic condition included in the group of diseases called phakomatoses. Most of the patients are diagnosed with abnormalities within the central nervous system and tend to develop tumors more frequently, especially gliomas. We present a case of 50-year-old patient suffering from tuberous sclerosis complex, who had been diagnosed with pleomorphic xanthoastrocytoma (PXA). The patient underwent surgery and adjuvant radiotherapy and has remained free from local recurrence for 5 years.
keywords:

pleomorphic xanthoastrocytoma, tuberous sclerosis complex, Bourneville-Pringle syndrome, surgery, radiotherapy

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