Sella Turcica Atypical Teratoid/Rhabdoid Tumor Complicated with Lung Metastasis in an Adult Female

Abstract

Here we present the case of a 60-year-old woman with a rare sellar region atypical teratoid/rhabdoid tumor (AT/RT), complicated by lung metastasis and treated with neurosurgery, radiotherapy, and chemotherapy. The patient had recurrent headache associated with left cavernous sinus syndrome after a previous endonasal transsphenoidal resection for a presumptive pituitary macroadenoma. Pituitary magnetic resonance imaging showed a tumor regrowth in the original location with a haemorrhagic component involving the left cavernous sinus. A near complete transsphenoidal resection of the sellar mass was performed followed by 3 months of stereotactic radiotherapy. Because of a worsening of the general clinical conditions, respiratory failure, and asthenia, the patient underwent a contrast enhanced computer tomography of the whole body which showed the presence of lung metastasis. The histopathological diagnosis on samples from pituitary and lung tissues was AT/RT. The patient survived 30 months after diagnosis regardless chemotherapy. In the adult, the AT/RT should be considered as a possible rare, aggressive, and malignant neoplasm localized in the sella turcica.

Sellar Atypical Teratoid/Rhabdoid Tumor: Any Preoperative Diagnostic Clues?

“…It is thus clinically relevant for endocrinologists, who are usually the primary physicians caring for patients with sellar masses, to identify preoperative diagnostic clues that may lead to the suspicion of sellar AT/RT. Imaging characteristics of AT/RTs and pituitary adenomas are remarkably similar; on MRI, both are isointense with brain parenchyma on T1-weighted imaging and enhance following gadolinium administration (8)(9)(10)(11)(12)(13)(14). Indeed, in all cases of sellar AT/ RT, including the one reported here, the presenting sellar masses are large (~2-3 cm), but MRI usually did not reveal any suspicious features that would suggest tumors other than pituitary adenomas.…”

“…There is no specific treatment for sellar AT/RT. Most patients undergo surgical resection followed by radiation therapy and/or chemotherapy, but there is no consensus on the best strategy (8)(9)(10)(11)(12)(13)(14). Stereotactic radiosurgery and external beam radiation are both used, but their efficacies are unclear without a control group (the present case and 9,13).…”

“…For example, the absence of immunohistochemical markers S-100 and HMB-45 rules RT can also occur in adults, and its most common primary site is supratentorial (6,7). There have been several reports of primary sellar AT/RT in adults, but they are mostly in the neurosurgical literature and not familiar to endocrinologists (Table 1) (8)(9)(10)(11)(12)(13)(14). There is no specific treatment for sellar AT/RT.…”

“…Sellar AR/RT carries a poor prognosis; most patients die within a few years of presentation (Table 1) (8)(9)(10)(11)(12)(13)(14). The grave prognosis of sellar AT/RT is in stark contrast to the benign clinical course of most pituitary adenomas.…”

“…The patient was admitted for further evaluation. Her endocrine laboratory values were as follows: prolactin 50.2 ng/mL (normal 3.8-23.2), luteinizing hormone <0.5 mIU/mL (0.6-19), follicle-stimulating hormone 2.9 mIU/ mL (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), estradiol <20 pg/mL (37-298), thyrotropin 0.60 mIU/mL (0.39-4.60), "low" free thyroxine, morning cortisol 5.4 mcg/dL (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and insulin-like growth factor-1 107 ng/mL (114-492). Pituitary magnetic resonance imaging (MRI) confirmed a large sellar mass measuring 3.3 × 3.2 × 2.3 cm with compression of the optic chiasm and invasion into the left cavernous sinus ( Fig.…”