RONC-21. THE RELATIONSHIP BETWEEN VENTRICULAR SIZE AND WHOLE BRAIN IRRADIATION DOSE IN CENTRAL NERVOUS SYSTEM GERM CELL TUMORS

✓ A retrospective analysis of 21 cases of primary central nervous system (CNS) lymphoma is reported. All patients presented with a solitary mass in the supratentorial region. None had previously received immunosuppressive therapy. Neuroradiological studies included technetium-99m-pertechnetate brain scanning in eight cases, cerebral arteriography in all 21 cases, and computerized tomography (CT) in 14 cases. The characteristic features were increased uptake in brain scans, mass effect in arteriograms, and marked contrast enhancement on CT scans. Abnormal tumor vessels were occasionally seen on arteriography, and subtraction films were usually required to appreciate tumor stain. All patients underwent craniotomy, and histological studies of the tumors showed a diffuse type of lymphoma in all cases. Immunoglobulin testing was performed in 19 cases and a monoclonal spike was verified in 10, suggesting a B cell origin. All patients were followed until their death except one who was still alive 12 months from onset of symptoms. Therapy included subtotal resection in all 21 cases, whole-brain irradiation in six cases, chemotherapy in two cases, and a combination of whole-brain irradiation and chemotherapy in nine cases. Three different forms of chemotherapy were used. The results suggest that chemotherapy is an important addition to subtotal resection and whole-brain irradiation in the treatment of primary CNS lymphoma.

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Is whole-brain irradiation necessary for primary central nervous system lymphoma?

Cancer ◽

10.1002/cncr.11035 ◽

2002 ◽

Vol 97 (1) ◽

pp. 128-133 ◽

Cited By ~ 63

Author(s):

Yuta Shibamoto ◽

Naofumi Hayabuchi ◽

Jun-ichi Hiratsuka ◽

Sunao Tokumaru ◽

Hiroki Shirato ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Whole Brain Irradiation ◽

Whole Brain ◽

Brain Irradiation

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GCT-15. INTEGRATED CLINICAL, HISTOPATHOLOGICAL, AND MOLECULAR DATA ANALYSIS OF 190 CENTRAL NERVOUS SYSTEM GERM CELL TUMORS FROM THE IGCT CONSORTIUM

Neuro-Oncology ◽

10.1093/neuonc/noaa222.235 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii330-iii331

Author(s):

Hirokazu Takami ◽

Koichi Ichimura ◽

Kohei Fukuoka ◽

Akitake Mukasa ◽

Nobuhito Saito ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Basal Ganglia ◽

Pineal Gland ◽

Germ Cell ◽

Prognostic Significance ◽

Germ Cell Tumors ◽

Molecular Data ◽

Molecular Heterogeneity ◽

Intracranial Germ Cell Tumor

Abstract BACKGROUND We integrated clinical, histopathological, and molecular data of central nervous system germ cell tumors to provide insights into their management. METHODS Data from the Intracranial Germ Cell Tumor Genome Analysis Consortium were reviewed. A total of 190 cases were classified as primary GCTs based on central pathological reviews. RESULTS All but one of the cases that were bifocal (neurohypophysis and pineal glands) and cases with multiple lesions including neurohypophysis or pineal gland were germinomas (34 of 35). Age was significantly higher in patients with germinoma than other histologies. Comparison between tumor marker and histopathological diagnoses showed that 18.2% of histopathologically diagnosed germinomas were marker-positive and 6.1% of non-germinomatous GCTs were marker-negative, suggesting a limitation in the utility of markers or histopathology alone using small specimens for diagnosis. Comparison between local and central histopathological diagnoses revealed a discordance of 12.7%. Discordance was significantly less frequent in biopsy cases, implying difficulty in detecting all histopathological components of heterogeneous GCTs. Germinomas at the typical sites (neurohypophysis or pineal gland) showed a better PFS than those at atypical sites (p=0.03). A molecular-clinical association study revealed frequent MAPK pathway mutations in males (51.4 vs 14.3 %, p=0.007), and PI3K/mTOR pathway mutations in basal ganglia cases (p=0.004). Basal ganglia cases also had frequent chromosomal losses. Some chromosomal aberrations (2q, 8q gain, 5q, 9p/q, 13q, 15q loss) showed potential prognostic significance. CONCLUSIONS These in-depth findings of this study regarding the clinical and molecular heterogeneity will increase our understanding of the pathogenesis of this enigmatic tumor.

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Endocrine manifestations of central nervous system germ cell tumors in children

Endocrinología Diabetes y Nutrición (English ed ) ◽

10.1016/j.endien.2019.11.011 ◽

2020 ◽

Vol 67 (8) ◽

pp. 540-544

Author(s):

Emilio García García ◽

Ana Lucía Gómez Gila ◽

Elena Merchante ◽

Mónica Rivero Garvia ◽

Eva Venegas Moreno ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Germ Cell ◽

Germ Cell Tumors

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The Role of Radiation Therapy in the Management of Primary Non-Hodgkin Lymphomas of the Central Nervous System: Clinical Study of 10 Cases

Tumori Journal ◽

10.1177/030089168607200605 ◽

1986 ◽

Vol 72 (6) ◽

pp. 565-573 ◽

Cited By ~ 9

Author(s):

Adriano Di Marco ◽

Luisa Rosta ◽

Franco Campostrini ◽

Andrea Bonetti ◽

Mario Palazzi ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Radiation Therapy ◽

Whole Brain Irradiation ◽

Brain Irradiation ◽

Non Hodgkin Lymphoma ◽

Uncommon Disease ◽

The Central Nervous System ◽

Disseminated Disease ◽

Clinical Conditions

A series of 10 patients with a primary non-Hodgkin lymphoma of the central nervous system were observed and treated by the authors. All were diagnosed after a pathologic examination of the surgical material; the lesions were supratentorial in all cases and unifocal in 9. In every case radiation therapy improved clinical conditions and in most cases even radiologic features of the patients. Four patients were alive and without evidence of disease many months after therapy. Four patients died with a relapse in the central nervous system; 3 of them had disseminated disease at the time of death. Two patients died for an unknown cause. Radiation therapy was performed with 60Co source with 2 opposed portals and the whole brain irradiation technique in all cases but 2. Doses ranged from 35 to 54 Gy. The recent literature on this uncommon disease and the most adequate therapeutic possibilities of radiation therapy and chemotherapy, or both, are discussed.

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