Session: SUN 163-194-Pituitary Disorders & Case Reports
Poster Board SUN-180
Clinical Case: A 46-year old woman presented with CD. UFC was 689ug/24h (nl<45ug) and ACTH 99pg/ml. MRI revealed infiltration of the clivus and sphenoid with extension into the tuberculum sella. IPSS confirmed CD and transsphenoidal (TSS) surgery was performed. A radical but incomplete resection was achieved due to invasion of the skull base and UFC remained elevated at 90ug/24h. Histological examination showed an atypical pituitary adenoma with ACTH and p53 immunoreactivity. RT was recommended but refused. SOM230 was given for 4 weeks, but the patient developed bilateral 9th and 12th cranial nerve (CN) palsies requiring intubation and tracheostomy secondary to tumor progression including infiltration of the hypoglossal canal. RT (4200 cGY) was administered, resulting in a dramatic reversal of the neurologic symptoms, normalization of cortisol levels and tumor regression on MRI within 3 months. 1 year later CD recurred and MRI showed a 2.1cm sellar mass abutting the optic chiasm, with persistent clival infiltration. Repeat TSS was performed and histology demonstrated a distinctly higher Ki67 index (15-20%). CD persisted despite maximal medical therapy. The patient developed recurrent tongue weakness and progression of the skull based lesion. Salvage chemotherapy with CAPTEM, a novel oral chemotherapy regimen that is synergistic for induction of apoptosis in neuroendocrine cell lines, was administered. 6 months after CAPTEM initiation the CN symptoms resolved, ACTH and cortisol fell to 5pg/ml and 0.5ug/dL respectively, and MRI revealed a 50% decrease in tumor size. After 12 months, MRI showed tumor resolution. After 23 cycles of CAPTEM, the patient has had 29 months of progression free survival and her CD remains in clinical remission.
Conclusion: Temozolomide alone is reported to be effective in the treatment of some aggressive pituitary adenomas. Our experience suggests combination chemotherapy with CAPTEM may be superior. This is the first report of complete radiologic remission, complete biochemical remission, and reversal of life threatening complications of CD, following the administration of CAPTEM chemotherapy, highlighting the potential efficacy of this regimen for the treatment of aggressive corticotrophic adenomas.
Nothing to Disclose: GP, SLW, PUF, JNB, RLF
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