RARE-02. SYMPTOMATIC SALIVARY GLAND-LIKE TUMORS OF THE SELLAR REGION: ADDITIONAL FEATURES AND REVIEW OF THE LITERATURE

Abstract

Abstract INTRODUCTION Sellar symptomatic salivary gland-like tumors (SSGTs) are exceedingly rare, with less than a dozen case reports in the literature. They usually consist of amorphous colloid identical to Rathke cleft cyst (RCC) contents, but additionally the cyst wall contains a large, cohesive collection of benign salivary glands. We report two new examples, one of which represents the first instance of recurrence of symptoms 2 years after first surgery, the second time with cyst confined to infundibular stalk and causing severe gliosis, indicative of mass effect. The second example underscores that SSGT remains poorly recognized, even today amongst experts. METHODS One case each was retrieved from the files of University of Colorado Denver (UCD) and Memorial Sloan Kettering (MSK); the UCD case represents the sole example in 2000+ sellar region masses seen at our institution since 1997. The MSK case had been recently received in consultation. RESULTS Case 1 is a 20-year-old female who presented with 3 weeks of symptoms referable to obstructive hydrocephalus; neuroimaging disclosed a sellar/suprasellar 2.4 x 2.2 x 2.4 cm mass. Following surgical resection, SSGT was diagnosed. She received no adjuvant therapy, but recurrence of headaches and blurred vision 2 years later prompted her return to medical attention. MRI revealed a recurrent cyst confined to the center of a thickened, bulbous infundibular stalk. Second resection yielded amorphous mucin within the cystic and severely-gliotic infundibular stalk, but no salivary-type glands. She has been followed for the last 6 years without tumor recurrence. Case 2 is a 29-year-old female with headache. The lesion was seen initially by a neuropathologist at another institution before it was sent to MSK for consultation and SSGT was diagnosed. CONCLUSION Few pathologists or neuropathologists have encountered SSGT in their practices; case 1 produced recurrence and significant local stalk damage, features not previously reported.