CLINICAL, RADIOLOGICAL AND PATHOLOGICAL FEATURES OF PATIENTS WITH RATHKES CLEFT CYSTS - TUMORS THAT MAY RECUR

Rathke's cleft cysts are cystic sellar and suprasellar lesions, charac teristically lined by a single layer of ciliated cuboidal or columnar epithelium. In contrast, craniopharyngiomas, which are also cystic sel lar and suprasellar lesions, are characteristically lined by stratifie d squamous epithelium with keratinization on a layer of connective tis sue. The usual management recommended for Rathke's cleft cysts is simp le surgical drainage with partial excision of the cyst wall. Recurrenc es of these cysts reportedly have been very rare. This retrospective s tudy presents the details of 12 patients (6 females; median age 30 yr, range 21-58 yr) with Rathke's cleft cyst, referred to our department over a 15-yr period (1981-1996), an unusual feature being the recurren ce of 4 (33%) of these lesions. Clinical, endocrine, radiological, sur gical (10 transsphenoidal; 2 transcranial), and pathological details w ere recorded. Nine out of 12 patients (75%) were symptomatic; visual s ymptoms were the commonest, and 8 had visual field defects. The median duration of symptoms was 12 months (range 3-24 months). Three patient s (25%) had panhypopituitarism, 2 of whom also had diabetes insipidus (17%). The cysts varied in size from 6 mm to 50 mm, 1 being entirely s uprasellar. There were no pathognomonic clinical or radiological featu res to differentiate them from other pituitary lesions, although the p resence of diabetes insipidus in 2 patients suggested that the lesion was not a pituitary adenoma. A definite histological diagnosis was pos sible in 8 patients; in 4, the diagnosis was presumptive. The median d uration of follow-up was 30 months (1-168 months). Four patients (33%) showed reexpansion at 3, 6, 48, and 48 months after initial surgery, 3 of whom were symptomatic and required repeat surgery. Two of these p atients were given postoperative external beam pituitary radiotherapy. Apparent recurrence of Rathke's cleft cysts after initially successfu l surgery in our series was higher than suggested by previous reports, and thus, long-term follow-up with pituitary imaging and neuroophthal mological assessment is essential. There are no specific characteristi cs of the cyst that predict recurrence. Ideal management of these cyst s is unclear, but aspiration, followed by extensive excision of the cy st wall when possible, seems to be the best initial option. For recurr ent symptomatic tumors, surgical resection is the treatment of choice. Considering the high recurrence rate with residual structural and fun ctional dysfunction, the role of radiotherapy in preventing recurrence of these cysts needs careful evaluation with a larger study with a lo nger follow-up period.