PMC6712354_01
Cyst volume (cm3) in response to treatment course for patient 1. (A) Diagnostic MRis. (B) Early response to interferon. (C) Response at end of bleomycin. (D) Best response to tocilizumab alone. (E) Best response to tocilizumab and bevacizumab. (F) Six months off all therapy. (G) Most recent scan. (H) Cyst volume (cm3) in response to treatment course.
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F1
MRI (Figure 1A) confirmed the findings, which were most consistent with the diagnosis of craniopharyngioma. TeraRecon, Forest City, CA) was used to measure tumor volumes showing primary cystic disease with minimal solid tumor component (Figure 1, graph). The patient's medical history included premature birth at 34 weeks. Overall cyst volume initially decreased (Figure 1B) however, after 4 months of therapy, the cystic component began to increase in size. MRI demonstrated new edema involving the left pons, middle cerebellar peduncle, and anterior right cerebellar hemisphere, in continuity with one wall of the cyst receiving bleomycin (Figure 1C). During the initial 7 months, there was radiographic improvement of the cystic portions of his lesion however, imaging at 14 months off therapy demonstrated progression of his cystic disease (Figure 1, graph). IV q2 weeks) on a compassionate use basis, which he received for 6 months and tolerated well. Imaging 6 months following the start of therapy demonstrated a decrease in volume of some cysts and an apparent slowing of expansion of others (Figure 1D). Following 8 months of therapy, MRI showed evidence of progression of the cystic component of his ACP (Figure 1, graph). In total, he received 14 months of combination therapy over 28 months with MRIs showing a significant decrease in cystic disease (Figure 1E). At this time, an MRI demonstrated an overall increase in his cystic mass (Figure 1F). The patient has remained on combination therapy for 13 months (Figure 1G), to date, and his seizures are controlled with levetiracetam.
PMC6712354_02
Cyst volume (cm3) in response to treatment course for patient 2. (A) Diagnostic MRis. (B) Scan immediately before ommaya reservoir replacement. (C) Scan immediately stopping bleomycin for right frontal lobe edema. (D) Scan while on Tocilizumab. (E) Patient two cyst volume (cm3) in response to treatment course.
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F2
MRI brain demonstrated a large cystic sellar/suprasellar mass, most consistent with craniopharyngioma (Figure 2A). MRI volume measures as with Case 1 demonstrated primary cystic disease with minimal solid tumor component (Figure 2, graph). Three months after catheter placement, MRI demonstrated an increase in cyst size (Figure 2B), and repositioning of the catheter was undertaken. Resumption of scheduled cyst aspiration again led to a decrease in the cyst size (Figure 2, graph). However, after 1 month, the patient developed new onset lethargy, and imaging demonstrated right frontal lobe edema adjacent to the cyst wall (Figure 2C). After 6 months of therapy, the cyst exhibited a partial response with measurements improved from 26 x 28 x 27 mm (AP x TRV x CC) to 18 x 24 x 18 mm (AP x TRV x CC) (Figure 2D).