Unusually long survival of an adult patient with atypical teratoid/rhabdoid tumor of the sellar region: A follow-up report

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.

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Rare intraparenchymal choroid plexus carcinoma resembling atypical teratoid/rhabdoid tumor diagnosed by immunostaining for INI1 protein

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.5.peds0955 ◽

2009 ◽

Vol 4 (4) ◽

pp. 368-371 ◽

Cited By ~ 6

Author(s):

E. Andrew Stevens ◽

Constance A. Stanton ◽

Kyle Nichols ◽

Thomas L. Ellis

Keyword(s):

Choroid Plexus ◽

Rhabdoid Tumor ◽

Choroid Plexus Carcinoma ◽

Nuclear Staining ◽

Atypical Teratoid Rhabdoid Tumor ◽

Imaging Studies ◽

Atypical Teratoid ◽

History Of ◽

The Right

The authors present the case of a rare extraventricular, intraparenchymal choroid plexus carcinoma (CPC). This 6-year-old girl presented to the emergency department with a 1-week history of headaches, nausea, and vomiting. Imaging studies revealed an intraaxial cystic and solid mass located in the right frontal lobe with central nodular enhancement and minimally enhancing cyst walls. Gross-total resection was accomplished via craniotomy without complications. The initial pathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT); however, immunostaining for INI1 protein (using the BAF47/SNF5 antibody) showed retention of nuclear staining in the tumor cells, resulting in a change in the diagnosis to CPC. There was no evidence of recurrence at the last follow-up 2.5 years after treatment, which supports the diagnosis of CPC over AT/RT. This case emphasizes the importance of immunostaining for INI1 protein for distinguishing CPC from AT/RT in cases with atypical or indeterminate features.

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Laser interstitial thermal therapy for pediatric atypical teratoid/rhabdoid tumor: case report

Neurosurgical FOCUS ◽

10.3171/2019.10.focus19746 ◽

2020 ◽

Vol 48 (1) ◽

pp. E11

Author(s):

Abdullah H. Feroze ◽

Margaret McGrath ◽

John R. Williams ◽

Christopher C. Young ◽

Chibawanye I. Ene ◽

...

Keyword(s):

Rhabdoid Tumor ◽

Thermal Therapy ◽

Atypical Teratoid Rhabdoid Tumor ◽

Laser Interstitial Thermal Therapy ◽

Published Report ◽

Atypical Teratoid ◽

Left Caudate ◽

The Right ◽

Radiographic Improvement

Herein, the authors describe the successful use of laser interstitial thermal therapy (LITT) for management of metastatic craniospinal disease for biopsy-proven atypical teratoid/rhabdoid tumor in a 16-month-old boy presenting to their care. Specifically, LITT was administered to lesions of the right insula and left caudate. The patient tolerated 2 stages of LITT to the aforementioned lesions without complication and with evidence of radiographic improvement of lesions at the 2- and 6-month follow-up appointments. To the authors’ knowledge, this represents the first such published report of LITT for management of atypical teratoid/rhabdoid tumor.

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Central Nervous System Atypical Teratoid/Rhabdoid Tumor: Results of Therapy in Children Enrolled in a Registry

Journal of Clinical Oncology ◽

10.1200/jco.2004.07.073 ◽

2004 ◽

Vol 22 (14) ◽

pp. 2877-2884 ◽

Cited By ~ 282

Author(s):

Joanne M. Hilden ◽

Sharon Meerbaum ◽

Peter Burger ◽

Jonathan Finlay ◽

Anna Janss ◽

...

Keyword(s):

Treatment Guidelines ◽

Tumor Therapy ◽

Intrathecal Chemotherapy ◽

Rhabdoid Tumor ◽

Primary Therapy ◽

Atypical Teratoid Rhabdoid Tumor ◽

Stem Cell Rescue ◽

Atypical Teratoid ◽

Disseminated Disease

Purpose Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor. Materials and Methods A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. Follow-up information was sought twice yearly. Results Information was complete for 42 patients. Median age at diagnosis was 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen tumors were infratentorial; 26 were supratentorial. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (3 to 62 months from diagnosis) and one patient died of toxicity. Fourteen patients (33%) show no evidence of disease (9.5 to 96 months from diagnosis). The median survival is 16.75 months and the median event-free survival is 10 months. Conclusion Aggressive therapy has prolonged the natural history in a subset of children. Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Enrollment onto the AT/RT registry should be continued.

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Atypical teratoid/rhabdoid tumor arising from the trigeminal nerve in an adult

The Neuroradiology Journal ◽

10.1177/1971400916654319 ◽

2016 ◽

Vol 29 (6) ◽

pp. 447-449 ◽

Cited By ~ 4

Author(s):

Fang Yu ◽

Florence Chiang ◽

Carlos Bazan

Keyword(s):

Trigeminal Nerve ◽

Diagnostic Testing ◽

Rhabdoid Tumor ◽

Imaging Features ◽

Atypical Teratoid Rhabdoid Tumor ◽

Immunohistochemistry Staining ◽

Bony Destruction ◽

Atypical Teratoid ◽

Appropriate Therapy

A 30-year-old male presented with left facial pain and numbness. Initial MRI demonstrated an enhancing mass involving the left trigeminal nerve. Follow-up imaging showed interval growth with erosion of the sphenoid body. Surgical resection was performed and immunohistochemistry staining was consistent with an atypical teratoid/rhabdoid tumor. Awareness of this entity and its imaging features such as diffusion restriction, intratumoral hemorrhage, and bony destruction, can help guide confirmatory diagnostic testing and appropriate therapy.

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