ECE2022 Poster Presentations Pituitary and Neuroendocrinology (127 abstracts)
1Sapienza University of Rome, Department of Experimental Medicine, Section of Medical Pathophysiology, Food Science and Endocrinology, Roma, Italy; 2Sapienza University of Rome, Department of Molecular Medicine, Roma, Italy
Case Summary: In May 2018, a 22-year-old man was admitted to the Emergency Room of Polyclinic Umberto I Hospital of Rome, because of progressive headaches and binocular diplopia. Brain MRI revealed a heterogeneous T1WI-hypointense and T2WI-hyperintense mass of 40x28x25 mm (CC, LL, AP) including calcification areas in the left paramedian region of the clivus invading the homolateral cavernous sinus. The patient underwent partial tumour resection via infratemporal approach and a diagnosis of intermediate grade myxoid chondrosarcoma was confirmed on histopathology. The patient was discharged with no neurological deficit. Two months later, the patient presented with newly onset of hypoesthesia of the face, ophthalmoplegia, and dizziness. A new MRI showed a heterogeneous calcified mass of 36x25x25 mm (CC, LL, AP) arising from the left cavernous sinus, expanding into the left sphenoid wing and impinging the pituitary gland, which was attributable to relapse of chondrosarcoma. Therefore, a second surgical resection was performed and after three months the patient was treated with high-dose (70 Gy/35 fractions) intensity-modulated proton therapy (IMPT). The patient was discharged without replacement therapy. The treatment resulted in a marked volumetric reduction of the pathological tissue which was stably maintained in the following three years of follow-up. Pituitary function evaluation tested normal except for mild hyperprolactinemia likely due to deviation of the pituitary stalk (Table 1).
Patients characteristics | 36-months follow-up | ||
Age (years) | 22 | ACTH | 17.60 pg/ml [5.00 - 60.00] |
Gender | Male | Cortisol | 10.90 mg/dl [3.70 - 19.40] |
Symptoms | headache, diplopia, cranial nerve injury | PRL | 34.03 ng/ml [3.46 - 19.4] |
Tumor site | Skull base | TSH | 2.00 μUI/ml [0.35 - 4.94] |
Histology | Myxoid Chondrosarcoma | FT4 | 0.99 ng/dl [0.70 - 1.48] |
Differentiation | G2 | FT3 | 2.89 pg/ml [1.71 - 3.71] |
KPS scores | 100 | FSH | 5.53 mUI/ml [0.95 - 11.95] |
Brainstem compression | No | LH | 2.16 mIU/ml [0.57 - 12.07] |
Optic apparatus compression | No | Estradiol | 28.00 pg/ml [11-44] |
Recurrent disease | Yes | Total Testosterone | 389.04 ng/dl [240.24 -870.68] |
Radiotherapy (IMPT) | 70Gy/35 fractions | GH | 0.31 ng/ml |
Metastases | No | IGF1 | 124.00 ng/ml [98.70 - 289.00] |
Any compression | Cavernous sinus, pituitary gland, | Serum sodium | 136 mEq/l [136 - 145] |
temporo-mesial parenchyma | Serum potassium | 4.10 mEq/l [3.50 - 5.10] |
Conclusion: The treatment of intracranial chondrosarcomas still remains a major challenge, as brain surgery and IMPT can have a detrimental effect on healthy cerebral areas located proximally to the lesion. This case confirms that IMPT can prevent tumour enlargement while preserving normal pituitary function.