Introduction: Rathke cleft cysts (RCC) are common, sellar-based cystic lesions that are usually incidental findings, but occasionally cause significant visual and/or endocrine problems. Despite repeat surgical drainage, a proportion of cysts can recur. The standard treatment for recurrent cysts is attempted removal, but we hypothesize stereotactic radiation therapy can be an effective salvage therapy in these challenging, recurrent cases.
Methods: Case records of patients with multiply recurrent RCC at our institution were reviewed from 2005 until the present. Descriptive statistics were performed.
Results: Out of 130 cyst drainage operations since 2005, six patients had multiply recurrent refractory RCC and underwent salvage radiotherapy. All six patients had immediate improvement in symptoms after their initial surgery but subsequently developed symptomatic early recurrence, necessitating at least one additional surgery. Median number of surgeries prior to radiation was 3 (range 2-4). Four patients required cyst drainage for symptom control immediately prior to radiation therapy. Four patients underwent LINAC-based radiotherapy while the other two had proton radiotherapy (stereotactic radiation; dose: 45 Gy in 1.8 Gy/fraction). Median follow-up after radiation was 47 months (range 14 – 144).
Two patients required repeat drainage after radiation therapy: one patient had only one surgery within one year after radiation and has been recurrence-free for 12 months, while the other had two surgeries immediately after radiation therapy and a third surgery approximately 5 years later. This latter patient has been recurrence-free for approximately 7 years after the last surgery. Only one patient developed endocrine deficits after radiation (hypothyroidism and hypoadrenalism).
Conclusions: Stereotactic radiotherapy is an effective salvage treatment for recurrent, symptomatic RCC refractory to multiple drainages. Rapidly recurring cysts may require drainage after XRT, since radiation effect may be delayed. All patients had durable remission with improvement in pre-operative symptoms. Radiotherapy can be an adjunct or alternative to an attempt at complete cyst removal.
Patient Care: This is the first report of an effective radiotherapy option for salvage treatment of challenging, multiply recurrent Rathke cleft cysts
Learning Objectives: Multiply recurrent rathke cleft cysts pose a clinical dilemma as more aggressive attempts at cyst removal incur additional risk.
Stereotactic radiotherapy at 45 Gray (1.8 Gy/fraction) is an effective salvage treatment for multiply recurrent rathke cleft cysts.