Adult atypical sellar teratoid tumor presenting as diabetes insipidus

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Conference Proceeding

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Endocr Pract


Objective: Atypical teratoid /rhabdoid tumor (ATRT) is a rare and aggressive central nervous system tumor that usually occurs in childhood but has rarely been reported in adults. We were able to identify 50 reported adult cases, 12 of which were sellar. We report a case of adult sellar ATRT who died within 2 months of presentation with headaches, visual deficits, polyuria, and polydipsia. Our case aims to highlight the importance of considering ATRT in the differential of sellar masses especially in adults presenting with rapid progression of symptoms. Case Presentation: A 62-year-old female was transferred to our institution for management of a pituitary mass. Her symptoms developed over 2 months, starting with daily headaches, nausea, followed by polyuria, polydipsia and finally double vision. She had left 6th nerve palsy on admission. She was diagnosed with central hypothyroidism and diabetes insipidus (DI). Magnetic resonance imaging (MRI) revealed a sellar mass with extension into superior sellar margin, an adjacent mass within the anterior third ventricle, with minimal connection between these adjacent masses with intraventricular and subarachnoid hemorrhage. She rapidly deteriorated, developed seizures, head CT demonstrated pituitary apoplexy and worsening intraventricular hemorrhage. She underwent emergent craniotomy, a large pituitary mass was found and decompressed, and third ventricular clot was removed. Histopathological study revealed malignant epithelioid neoplasm, consistent with ATRT infiltrating the pituitary gland and fibrocollagenous stroma. The neoplastic cells showed divergent phenotypes based on immunophenotypes - cytokeratin+, EMA +, SMA+, CD 34+ and loss of SMARCB1/ INI1 protein. Radiation therapy was started but her condition continued to deteriorate. She underwent left frontal ventriculoperitoneal shunt with external ventricular drain placement for progressive hydrocephalus, without significant improvement. MRI brain demonstrated leptomeningeal spread. She expired within 2 months of diagnosis. Conclusion: ATRT is very rare in adults and has a very poor prognosis. The more common presenting symptoms of sellar ATRT are headaches and visual symptoms. DI has not been previously reported and increased the suspicion for a non-adenoma etiology in our patient. Definite diagnosis is based on immunohistochemical features. Treatment modalities in adults include surgery, chemotherapy and radiotherapy. ATRT should be considered in the differential for patients with sellar masses presenting with atypical features or rapid progression. Early recognition and aggressive combination therapy is reported to be associated with improved outcomes.



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