J Neurol Surg B Skull Base 2022; 83(S 01): S1-S270
DOI: 10.1055/s-0042-1743723
Presentation Abstracts
Podium Abstracts

Adenoid Cystic Carcinoma Infiltrating the Skull Base: A Systematic Review

Othman Bin Alamer
1   College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
,
Ali S. Haider
2   Texas A&M University College of Medicine, Houston, Texas, United States
,
Adhiraj Chaudhary
3   Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Kishore Balasubramanian
2   Texas A&M University College of Medicine, Houston, Texas, United States
,
Tessa Breeding
4   Dr. Kiran C. Patel College of Allopathic Medicine, Nova Southeastern University, Fort Lauderdale, Florida, United States
,
Paolo Palmisciano
5   Department of Neurosurgery, Trauma Center, Gamma Knife Center, Cannizzaro Hospital, Catania, Italy
,
Maryam Haider
6   Department of Radiology, Baylor College of Medicine, Houston, Texas, United States
,
Aaron A. Cohen-Gadol
7   Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, Indiana, United States
,
Tarek Y. El Ahmadieh
1   College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
,
Kenny Yu
8   Department of Neurosurgery, Memorial Sloan Kettering Cancer Center, New York, New York, United States
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Introduction: Adenoid cystic carcinomas (ACCs) are rare tumors mostly arising from the salivary glands. They may show an aggressive behavior, extending to the anterior fossa through perineural invasion and posing some challenges to skull base surgery. Currently available data are scarce and controversial, preventing the delineation of standard management strategies.

Objective: To systematically review the clinical characteristics and treatment strategies of patients with ACC infiltrating the skull base.

Methods: PubMed, Scopus, and Cochrane were searched using the preferred reporting items for systematic reviews and meta-analyses guidelines to retrieve studies reporting management strategies and survival of patients with skull base ACCs. Clinical characteristics, management protocols, and treatment outcomes were analyzed.

Results: We included 14 studies involving 154 patients. Median age was 49 years (range, 24–65), and a total of 62 females were identified. ACCs mostly infiltrated Meckel's cave (5.8%), the pterygopalatine fossa (5.2%), the nasopharynx (4.5%), the cavernous sinuses (3.9%), and the ethmoid and sphenoid sinuses (3.9%). Perineural invasion was also described in 7.8% of cases. Symptoms were reported in 12.3% of patients, with the most frequent being headache, ataxia, and cranial nerve neuropathies. Surgical resection (44%) was preferred over biopsy (15%). After tumor resection, the surgical reconstruction of the bony defect was performed using abdominal and anterior tight muscle grafts in 2% of cases each. For adjuvant treatments, 16% of patients received radiotherapy and 8% had chemotherapy, mostly with paclitaxel (30%) and/or cisplatin (23%). Recurrence of skull base ACCs occurred in 11% cases. Median overall survival was 99 months (range, 20–99), and 77% of patients died due to tumor progression.

Conclusion: Skull base ACCs represent challenging surgical lesions mostly due to their aggressive nature and proximity to critical neurovascular structures. The high mortality rate and scarce data on nonsurgical protocols suggest the need to further evaluate the role of innovative therapies aimed at improving patients’ quality-of-life and survival.


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No conflict of interest has been declared by the author(s).


Publication History

Article published online:
15 February 2022

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