Yolk Sac Tumors of the Head and Neck in Aicardi Syndrome.

Autor:	Epperson MV; University of Cincinnati College of Medicine, Cincinnati, OH, USA., Born HL; Department of Otolaryngology Head & Neck Surgery, University of Cincinnati Medical Center, Cincinnati, OH, USA., Wang D; Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA., Myer CM 4th; University of Cincinnati College of Medicine, Cincinnati, OH, USA.; Department of Otolaryngology Head & Neck Surgery, University of Cincinnati Medical Center, Cincinnati, OH, USA.; Division of Pediatric Otolaryngology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Jazyk:	angličtina
Zdroj:	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.
DOI:	10.1177/0003489419883660
Abstrakt:	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. 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. 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. 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.
Databáze:	MEDLINE
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