ESPE Abstracts (2019) 92 T3

Hypothalamus and Pituitary Gland Antibodies in Childhood-Onset Brain Tumors and Pituitary Dysfunction

Giuseppa Patti1, Erika Calandra1, Annamaria De Bellis2, Annalisa Gallizia1, Flavia Napoli1, Marco Crocco1, Giuseppe Bellastella2, Maria Ida Maiorino2, Maria Luisa Garrè3, Stefano Parodi4, Mohamad Maghnie1, Natascia Di Iorgi1


1Department of Pediatrics, IRCCS Istituto Giannina Gaslini, University of Genova, Genova, Italy. 2University of Campania Luigi Vanvitelli, Napoli, Italy. 3Department of Neuroncology, IRCCS Istituto Giannina Gaslini, Genova, Italy. 4Department of Biostatistics, IRCCS Istituto Giannina Gaslini, Genova, Italy


Background: Antipituitary (APA) and antihypothalamus antibodies (AHA) have not been investigated in children and adolescents with brain tumors.

Patients and Methods: Sixty-three patients with craniopharyngioma, glioma and germinoma treated with surgery and/or chemotherapy and/or radiotherapy were evaluated at a median age of 13 years. Forty-one had MPHD, 6 had a single defect being GH the most common (65.1%), followed by AVP (61.9%), TSH (57.1%), ACTH (49.2%) and gonadotropin (38.1%). APA and AHA were evaluated by indirect immunofluorescence in patients and in fifty controls.

Aim: To detect the presence and role of APA and AHA in patients with craniopharyngioma, glioma or germinoma treated with different modalities.

Results: APA+ and/or AHA+ were detected in half of the patients but not in the controls (P<0.001); 25 were APA+ (P=0.001), 26 were AHA+ and 20 were both APA+ and AHA+ (P<0.001), mostly with germinoma. APA+ (P<0.001) and their titers (P= 0.008) were significantly associated with the number of pituitary defects, with a 25% risk of developing an additional pituitary defect at each increase in antibody titers from one level to a higher one; this risk was confirmed also after correction for tumor type (18.4%, P=0.002). A similar relation was found for AHA+ (P=0.028). There was a significant association between the presence of APA+ and radiotherapy (P=0.03).

Conclusion: Patients with germinoma are prone to develop an autoimmune process involving the pituitary gland and the hypothalamus that contributes to endocrine dysfunction. Attention should be paid to avoid misdiagnosis of germinoma masked by an autoimmune pituitary condition.