Adult Sellar Region Atypical Teratoid/Rhabdoid Tumor: A Retrospective Study and Literature Review

Abstract

Background: Adult sellar region atypical teratoid/rhabdoid tumor (AT/RT) is a rare lesion. We aimed to elucidate clinical, radiologic, and pathological characteristics, treatment strategies, and outcomes of this disease.Methods: Five adult sellar AT/RT patients were retrospectively analyzed between January 2015 and December 2018. In addition, we performed a review of the reported data on adult sellar AT/RT.Results: Patients (n = 5) were female with a median age of 50 years. The mean duration of symptoms, of which headache was the most frequent, was 1.6 months (range, 2 weeks−8 months). The average tumor size was 2.82 cm (range, 1.9–4.5 cm). All lesions were irregularly shaped. MRI showed heterogeneous enhancement in three of five lesions. Four of five patients underwent subtotal resection (STR) and one gross total resection (GTR). Whereas, one patient received post-operative adjuvant radiotherapy, one patient received post-operative combination of radio- and chemotherapy. The review of the reported data showed that 39 cases of adult sellar AT/RT had been reported. The estimated median overall survival (OS) was 23 months with a 1-year survival estimate of 59.7%. The median OS for patients with GTR was 28 months and 17 months for patients with STR. Kaplan–Meier analysis showed that patients with high (≥35%) MIB-1/Ki67 index value had a significantly shorter OS compared with those with low (<35%) index value (p = 0.033), and that patients who received post-operative combination radio- and chemotherapy had longer OS than that of those who did not (p < 0.001).Conclusion: Adult sellar region AT/RT is a rapidly growing tumor with a poor prognosis. High levels of MIB1/Ki-67 on histology may indicate aggressive feature of the tumor. Maximal safe resection followed by adjuvant radiotherapy combined with chemotherapy may be the optimal therapeutic strategy for adult sellar region AT/RT.

Atypical Teratoid Rhabdoid Tumor: A Possible Oriented Female Pathology?

“…On the base of a literature review, we found 50 cases of ATRTs reported before (12,(15)(16)(17)(18)(19)(20); we have considered Articles that describe primitive sellar tumor cases in adults and were resulted ATRT with loss of INI, from 2000 to 2021 (Table 2).…”

Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors

“…Besides, most adult AT/RTs found to belong to the ATRT‐MYC subgroup and further clinical and molecular heterogeneity in ATRT‐MYC may be revealed. 39 , 40 Using both in vitro and in vivo data, Alimova et al. 41 showed that the c‐MYC oncogene is a critical regulator of malignant behavior in SMARCB1‐deficient AT/RTs.…”

“…The median age is significantly higher among patients with ATRT‐MYC than among patients with the other subtypes of AT/RTs. Besides, most adult AT/RTs found to belong to the ATRT‐MYC subgroup and further clinical and molecular heterogeneity in ATRT‐MYC may be revealed 39,40 . Using both in vitro and in vivo data, Alimova et al 41 .…”

“…Besides, most adult AT/RTs found to belong to the ATRT-MYC subgroup and further clinical and molecular heterogeneity in ATRT-MYC may be revealed. 39,40 Using both in vitro and in vivo data, Alimova et al 41 showed that the c-MYC oncogene is a critical regulator of malignant behavior in SMARCB1-deficient AT/RTs. A recent study demonstrated that ATRT-MYC is susceptible to glutamine metabolic inhibition with 6-diazo-5-oxo-norleucine (DON) therapy; this approach inhibits glutamine-dependent synthesis of glutathione and synergizes with carboplatin to extend survival in orthotopic mouse models with ATRT-MYC.…”

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult Male: A Case Report

“…Nonetheless, there are around 50 cases reported of adults; the average age was 36.69 (SD = 15.19) years with a range from 18 to 59 years [ 4 ]. Thirty-nine of these cases are sellar AT/RT in adults to date [ 5 ]. AT/RT can occur anywhere in CNS.…”