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Yolk Sac Tumors of the Head and Neck in Aicardi Syndrome.
- Source :
-
The Annals of otology, rhinology, and laryngology [Ann Otol Rhinol Laryngol] 2020 Mar; Vol. 129 (3), pp. 301-305. Date of Electronic Publication: 2019 Oct 22. - Publication Year :
- 2020
-
Abstract
- Objectives: To understand that yolk sac tumors (YSTs) of the head and neck (H&N) are exceedingly rare and typically carry a poor prognosis. To acknowledge the possibility of increased incidence in patients with Aicardi Syndrome and the ramifications this has on early diagnosis and treatment in this population.<br />Methods: To date, four germ cells tumors of the H&N have been reported in patients with Aicardi Syndrome. This report presents the second known case of a H&N YST in a patient with Aicardi syndrome. In both cases, the patient was initially misdiagnosed given unconvincing radiologic evidence. However, tissue diagnosis and elevated alpha-fetoprotein (AFP) levels were suggestive of a YST.<br />Results: In contrast to the poor prognosis previously described, both patients with Aicardi syndrome had an excellent chemotherapeutic response exhibited by normalization of AFP levels and imaging.<br />Conclusions: Rare germ cell tumors of the H&N, such as YSTs, have now been documented in several patients with Aicardi syndrome, indicating a possible association given the rarity of these tumors in the population. YSTs should be considered in the differential diagnosis of H&N masses in these patients, with emphasis on early tissue diagnosis and treatment.
- Subjects :
- Antineoplastic Combined Chemotherapy Protocols therapeutic use
Endodermal Sinus Tumor drug therapy
Female
Head and Neck Neoplasms drug therapy
Humans
Infant
Magnetic Resonance Imaging
Tomography, X-Ray Computed
alpha-Fetoproteins analysis
Aicardi Syndrome complications
Endodermal Sinus Tumor diagnostic imaging
Head and Neck Neoplasms diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1943-572X
- Volume :
- 129
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The Annals of otology, rhinology, and laryngology
- Publication Type :
- Academic Journal
- Accession number :
- 31640397
- Full Text :
- https://doi.org/10.1177/0003489419883660