Primary sellar atypical teratoid/rhabdoid tumor (AT/RT) is the most aggressive sellar mass. Although rare, sellar AT/RT exhibits a very relentless clinical course and usually results in death within months to a few years after diagnosis. The best clinical evidence suggests that surgical debulking and timely adjuvant chemoradiation are most effective in prolonging survival. A preoperative radiological diagnosis of sellar AT/RT thus is crucial in informing patients and physicians about this devastating disease. This minireview summaries the imaging features of sellar AT/RT. magnetic resonance imaging features of sellar AT/RT and the much more common sellar mass, pituitary macroadenoma, are similar in most aspects: They are both isointense to brain gray matter on T1 and T2 imaging and enhance upon gadolinium administration. Suprasellar extension and cavernous sinus invasion are present in practically all cases of sellar AT/RT, but are also present in 50%-75% of pituitary macroadenomas, especially in large ones, suggesting that suprasellar extension and cavernous sinus invasion disproportionate to the tumor size may favor sellar AT/RT diagnosis. Since sellar AT/RT grows very rapidly and does not allow significant remodeling of perisellar structures, the imaging features of perisellar structures such as optic chiasm and cavernous sinus may be key for imaging diagnosis of sellar AT/RT although they have not been well described in sellar AT/RT. In limited cases of sellar AT/RT, optic chiasm degeneration and thinning, which are very common in pituitary macroadenoma, are not present, giving hope for using features of perisellar structures to diagnose sellar AT/RT by imaging.

• Primary sellar atypical teratoid/rhabdoid tumor
• Sellar imaging
• Sellar mass
• Pituitary macroadenoma
• Optic chiasm
• Cavernous sinus

The predominance of pituitary adenoma in the etiology of sellar masses often leads to the diagnostic fallacy of “availability bias” so that pituitary adenoma is almost always considered the most likely diagnosis of all sellar masses, even when clinical evidence suggests otherwise. Primary sellar atypical teratoid/ rhabdoid tumor (AT/RT) is the most aggressive sellar tumor. Most patients with sellar AT/RT are initially misdiagnosed with pituitary macroadenoma. Early diagnosis of sellar AT/RT is of paramount importance to counsel patients and family on the grave prognosis and to avoid futile surgical procedures. Since there are no discerning imaging features to differentiate AT/RT from other sellar tumors, the acuity of sellar compression symptoms characteristic of AT/RT is the only evidence indicative of the AT/RT diagnosis. Based on the biological and anatomical properties of the sella turcica and its surrounding structures, the nature, order of manifestation, and acuity of the sellar compression symptoms in response to sellar content expansion are mostly predictable. It is concluded that rapidly progressive headache and subsequent similarly rapidly progressive visual symptoms in a female with a large sellar mass are pathognomonic of sellar AT/RT (the “Yu rule”).

• Sellar atypical teratoid/rhabdoid tumor
• Sellar compression symptoms
• Headache
• Visual symptoms
• Symptom acuity
• Clinical diagnosis

• atypical teratoid/rhabdoid tumor
• nomogram
• prognosis
• risk grouping system
• treatment

• Humans
• Rhabdoid Tumor/mortality
• Rhabdoid Tumor/diagnosis
• Rhabdoid Tumor/therapy
• Nomograms
• Female
• Male
• Teratoma/mortality
• Teratoma/diagnosis
• Teratoma/therapy
• Infant
• Child, Preschool
• Prognosis
• Child
• Proportional Hazards Models

• ATRT
• Adult
• Lung metastasis
• Sellar

• Atypical teratoid rhabdoid tumor
• Brain tumor
• Children
• Radiotherapy

• Humans
• Rhabdoid Tumor/radiotherapy
• Rhabdoid Tumor/therapy
• Teratoma/radiotherapy
• Radiotherapy Dosage
• Child, Preschool
• Infant

• COSMIC
• Genomic Landscape
• LASSO
• Random Forest
• SEER
• atypical teratoid/rhabdoid tumor
• risk prognostic model

• R1047 Chongqing Medical University Professor Scientific Research Start-up Fund

• Atypical teratoid/rhabdoid tumors
• Nomogram
• Overall survival
• Postoperative radiotherapy
• SEER

• Phelan McDermid syndrome
• SHANK3 deficiency
• atypical teratoid rhabdoid tumor
• chromosomal aberrations
• radiation sensitivity

• Child
• Humans
• Autism Spectrum Disorder/genetics
• In Situ Hybridization, Fluorescence
• Pilot Projects
• Syndrome
• Neoplasms

• ATRT
• Posterior fossa tumour
• Survival analysis

Introduction: Atypical Teratoid/Rhabdoid tumors are rare, highly malignant tumors in adults, with a median survival of 20 months. We report a case of a sellar atypical teratoid/rhabdoid tumor in a 70-year-old female treated with intraventricular chemotherapy, followed by a systematic review of the current management of sellar AT/RTs.

Methods: A comprehensive systematic literature search was conducted on Web of Science, Scopus, and PubMed Central using the key terms “sellar” and “atypical teratoid/rhabdoid tumors”, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data, including patient demographics, histology, treatments, and overall survival were extracted and analyzed. Kaplan-Meier survival curves and log-rank analysis were used to compare survival outcomes between different treatment regimens.

Results: Our literature search disclosed 123 publications. After prespecified exclusions, 41 patients with sellar AT/RT from 30 manuscripts were identified, and 38 were included in the final analysis. Including our patient, the median age was 44 (range: 20-70) with a substantial female predominance (94.7%). Collectively, patients who received combined chemoradiation therapy had a significantly increased overall survival compared to those who received single modality or no adjuvant therapies (median OS 27 vs. 1.25 months; p=0.0052).

Conclusion: Atypical teratoid/rhabdoid tumor in the sellar region carries a poor prognosis. Adjuvant chemotherapy and radiation therapy were associated with significantly increased overall survival. Early consideration of neuro-oncology and radiation-oncology referral and management is likely beneficial in this patient population. Intrathecal chemotherapy is a treatment modality that requires further exploration given the limited options and current dismal prognosis of adult sellar AT/RT.

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.

• adult female
• atypical teratoid/rhabdoid tumor
• clinicopathologic and radiologic features
• sella region
• treatment

• Adult
• Atypical teratoid rhabdoid tumor
• Pineal gland
• Tumor

Atypical teratoid/rhabdoid tumors (AT/RTs) of infancy are highly malignant central nervous system neoplasms that are most commonly seen during the first 2 years of life with limited therapeutic options. To date, only two cases have been described in the lateral ventricle. A 4-year-old boy presented with a 4-month history of increased intracranial pressure. Cerebral magnetic resonance imaging (MRI) revealed a huge intraventricular tumor, occupying the entire temporal horn and the body of the left lateral ventricle. The boy was operated through a left temporal transventricular approach with gross total removal of the lesion. The histopathological diagnosis was an AT/RT. The infant underwent adjuvant chemotherapy and radiation therapy. The 1-year MRI of control showed a local recurrence of the tumor. Then after, Gamma Knife radiosurgery was performed because of the small volume and the deep location of the lesion. At the 3-month follow-up, the MRI showed a significant growth of the tumor volume, and the child was given additional adjuvant chemotherapy. Unfortunately, he died 9 months later. AT/RT of the lateral ventricle is a very rare tumor in children, associated with a poor prognosis in spite of multimodal treatment. Gamma knife surgery (GKS) was rarely reported as a treatment modality of AT/RT. The aim of this work is to discuss about the rarity of this tumor and the best treatment strategy to improve prognosis.

• Atypical teratoid/rhabdoid tumor
• children
• lateral ventricle
• radiosurgery
• surgery

• adjuvant chemotherapy
• atypical teratoid rhabdoid tumor
• chemoradiotherapy
• cohort studies
• combined (triple) modality therapy
• primary brain tumor
• propensity score

• CNS tumors
• Children
• Lymphoma
• Pediatric cancer
• Proton therapy
• Sarcoma

Rhabdoid tumors, which tend to occur prior to the age of 2 years, are one of the most aggressive malignancies and have a poor prognosis due to the frequency of metastasis. Silibinin, a natural extract, has been approved as a potential tumor suppressor in various studies, however, whether or not it also exerts its antitumor capacity in rhabdoid tumors, particularly with regards to tumor migration and invasion, is unclear. The rhabdoid tumor G401 cell line was used in the present in vitro study. An MTT assay was used to assess the cytotoxicity of silibinin on G401 cells, cell migration was studied using a wound healing assay and a Transwell migration assay, and cell invasion was determined using a Transwell invasion assay. The underlying mechanism in silibinin inhibited cell migration and invasion was investigated by western blot analysis and further confirmed using a specific inhibitor. Experimental results demonstrated that high doses of silibinin suppressed cell viability, and that low doses of silibinin inhibited cell migration and invasion without affecting cell proliferation. The phosphatidylinositol 3-kinase/protein kinase B (PI3K/Akt) signaling pathway was involved in the silibinin-induced inhibition of metastasis. Silibinin inactivated the PI3K/Akt pathway, and inhibited cell migration and invasion, an effect that was further enhanced when LY294002, a classic PI3K inhibitor, was used concurrently. In general, silibinin inhibits migration and invasion of the rhabdoid tumor G401 cell line via inactivation of the PI3K/Akt signaling pathway and may be a potential chemotherapeutic drug to combat rhabdoid tumors in the future.

• invasion
• migration
• phosphatidylinositol 3-kinase/Akt
• rhabdoid tumor
• silibinin