A 64-year-old female with a germline VHL missense mutation (R167W), a known large suprasellar hemangioblastoma, and history of right eye enucleation presented with worsening vision of her remaining left eye. The patient had a past history of severe VHL disease with bilateral clear cell renal cell carcinomas, bilateral pheochromocytomas, pancreatic cysts, retinal HBs, and multiple CNS HBs. Neurological exam was significant for Romberg sign and mild dysmetria of bilateral upper extremities. Left eye extraocular movements and pupillary reaction intact. A visual field test of the left eye revealed new onset inferior temporal quadrantanopsia. Magnetic resonance imaging confirmed a slowly growing (3.45 cm3) suprasellar hemangioblastoma within the circle of Willis with increased optic tract edema and compression of the optic chiasm (Fig. 3A).Open in a separate windowFigure 3Symptomatic, surgically unresectable VHL-HB treated with octreotide.(A) T1 magnetic resonance image with contrast of 64 year-old female patient with germline VHL missense mutation presents with a 3.45 cm3 suprasellar HB. (B) 68GA-DOTATATE PET/CT imaging of suprasellar HB showing avid 68GA-DOTATATE uptake. (C) 68Ga-DOTATATE-PET imaging at presentation and 6 months after octreotide treatment (D). The pituitary stalk HB exhibits diminished uptake of 12.0 SUV compared to 22.6 SUV in initial study. Central photopenia also noted, indicative of necrotic process. (E) Tumor volume was measured using Osirix® software on FLAIR images. Seven images were analyzed, with the first image acquired just prior to initiation of Octreotide treatment. Measurements in cm3. (F) Visual field analysis of patient’s left visual field prior to starting octreotide treatment (left) and 6 months after beginning treatment.The newly symptomatic suprasellar mass was deemed inoperable due to the location (suprasellar, within the circle of Willis), her extensive co-morbidities, and the risk of loss of vision in her remaining eye with an attempted surgical resection. Whole body 68Ga-DOTATATE-PET revealed that the suprasellar mass was avid for DOTATATE (maximum standard uptake value (SUVMax) = 22.6) (Fig. 3B and C). As such, octreotide was initiated off label with a trial dosing of 20 μg daily subcutaneously for two weeks. Following this, she was prescribed long-acting Octreotide-LAR 30 mg by intramuscular injection, once a month, for 9 months. On Octreotide-LAR, she reported diarrhea managed with increased pancrealipase dosing and an increased insulin requirement. Follow up 68Ga-DOTATATE-PET imaging was done 6 months after primary scans and more than one month after her most recent octreotide dose. These images revealed maximum SUVMax of 12.0, compared to the initial SUVMax of 22.6 prior to starting treatment. Moreover, the 6 month follow up scan revealed central tumor photopenia, indicative of necrosis (Fig. 3D). As a reference, the pituitary uptake initially measured 24.9 SUV and 6 months after treatment measured 50.3 SUV. Serial FLAIR MRI images were acquired periodically throughout octreotide treatment and revealed a steady decline in the volume of the suprasellar mass from 3.45 cm3 to 2.54 cm3 with 9 months of Octreotide-LAR therapy (Fig. 3E). Furthermore, visual field analysis remained stable with continuous therapy (Fig. 3F).