Sellar Atypical Teratoid Rhabdoid Tumor (ATRT) In An Adult: A Case Report and Review of The Literature
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2018, Vol 12, Issue 1
Abstract
Background: Atypical teratoid rhabdoid tumor (ATRT) is a rare type of central nervous system (CNS) tumor, which is seen mostly in the pediatric population. However, there are a few dozen of cases reported in adults. This entity was first differentiated from a Wilm’s tumor in 1978. Since then much progress has been made in understanding the pathophysiology of this disease, but only slight progression has been done in terms of treatment and prognosis, thus ATRT remains a high mortality-disease with grim prognosis and outcome. Case Description: 54 year-old female, presented with symptoms concurrent of a pituitary macroadenoma (e.g. temporal field vision loss, hyperprolactinemia) which was found out to be a tumor of ATRT characteristics on pathology. Conclusion: ATRT is a rare entity, but should be kept in mind since it has a poor prognosis. Moreover, considering the grim prognosis, and the rarity of the disease, once ATRT diagnosis was made, it should be treated by a multidisciplinary team composed with surgeons, oncologists and radiation oncologists.Atypical teratoid rhabdoid tumor (ATRT) is a rare type of central nervous system (CNS) tumor, composed of rhabdoid cells. It effects mostly children younger than three years of age. The location of the tumor is often supratentorial, but it can be found in all parts of the CNS [1], this tumor is characterized with a high mortality rate [1,2]. It was first described by Beckwith and Palmer as a variant of Wilms tumor in 1978 [3]. There are few reports about ATRT in adults, but it is less common in that population [4-6]. In a review made by Sinha et al. the majority of the tumors in adults were located within the lobes of the cerebrum[6]. The rarity of the entity and its variable presentation make the diagnosis challenging, and even with the use of radiology and pathology ATRT is still misdiagnosed [7]. We report a case of a primary sellar ATRT in a 54 year-old female, which was presented and treated as a pituitary macroadenoma until the pathological diagnosis was made as an ATRT tumor. A 54 year-old right handed female, without any significant past medical history, was admitted with a 3 month history of progressing fatigue, nausea, shivers, and weight loss. Headaches, blurred vision or galactorrhea were absent. On physical examination the patient was fully oriented, with a left side 3rd cranial nerve palsy displaying a left side ptosis and slight left pupil dilatation. Both Pupils were reactive to light with no limits on eye movement. The rest of the physical exam was insignificant. A neuro-opthalmologic examination revealed bilateral visual accuracy of 20/30, left eye temporal field loss and enlargement of the blind spot in the right eye. Laboratory analysis showed hyperprolactinemia (54ng/ml), and low cortisol levels (2.6mcg/dL). Due to these values, a 5 miligram dose of prednisone twice daily was initiated. A synacthen test was preformed which revealed low levels of cortisol and free T4, therefore treatment with daily thyroxine 50mcg was started as well. The clinical findings were suitable with a pituitary adenoma, with stalk effect.
Authors and Affiliations
Daniel Barsky, Uri P Hadelsberg, Lior Gonen, Nevo Margalit
Keywords
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- EP ID EP586959
- DOI 10.26717/BJSTR.2018.12.002227
- Views 161
- Downloads 0
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