Papadopoulou Erofili, Pettas Efstathios, Gkoutzanis Lampros, Katoumas Konstantinos, Georgaki Maria, Vardas Emmanouil, Piperi Evangelia, Nikitakis Nikolaos G
Department of Oral Medicine & Pathology and Hospital Dentistry, School of Dentistry, National and Kapodistrian University of Athens (NKUA), AthensGreece.
Department of Oral and Maxillofacial Surgery, School of Dentistry, National and Kapodistrian University of Athens (NKUA), AthensGreece.
J Oral Maxillofac Res. 2023 Mar 31;14(1):e5. doi: 10.5037/jomr.2023.14105. eCollection 2023 Jan-Mar.
Congenital cystic swellings involving the floor of the mouth include various lesions such as developmental cysts (e.g., dermoid and epidermoid cysts), ranulas, vascular malformations etc. However, coexistence of such conditions, possibly with a cause-and-effect- relationship, is rare. The purpose of this case report is to present a rare case of a congenital epidermoid cyst associated with a mucous retention cyst in a newborn.
A 6-month-old female infant was referred to an Oral Medicine Clinic in Athens, Greece on October 2019 for evaluation of a swelling at the floor of the mouth, first noticed by her paediatrician just after birth. Clinically, a yellowish "pearly" nodule in close association with the orifice of the left submandibular duct, posteriorly transitioning to a diffuse bluish cystic swelling of the left floor of the mouth was observed. With a provisional diagnosis of a dermoid cyst and/or ranula, a surgical excision was performed under general anaesthesia.
Histopathologically, a well-defined, keratin-filled, cystic cavity lined by orthokeratinized stratified squamous epithelium was observed in the anterior aspect while posteriorly and in close proximity, a dilated salivary duct lined by cylindrical, cuboidal or pseudostratified epithelium was noted. A final diagnosis of an epidermoid cyst intimately associated with a mucus retention cyst (ranula) of the submandibular duct was rendered.
The coexistence of two cystic lesions in the floor of the mouth with features of epidermoid and mucous retention cyst, respectively, is rare and its pathogenesis intriguing, especially in a newborn.
累及口腔底部的先天性囊性肿物包括多种病变，如发育性囊肿（如皮样囊肿和表皮样囊肿）、舌下囊肿、血管畸形等。然而，这些情况同时存在，且可能存在因果关系的情况很少见。本病例报告的目的是介绍一例新生儿罕见的先天性表皮样囊肿合并黏液潴留囊肿的病例。
2019年10月，一名6个月大的女婴因口腔底部肿物被转诊至希腊雅典的口腔内科诊所，该肿物在出生后不久就被其儿科医生发现。临床上，观察到一个淡黄色的“珍珠样”结节与左下颌下腺导管口紧密相连，向后转变为左口腔底部弥漫性的蓝色囊性肿物。初步诊断为皮样囊肿和/或舌下囊肿，在全身麻醉下进行了手术切除。
组织病理学检查发现，前部有一个边界清晰、充满角蛋白的囊性腔隙，内衬正角化复层鳞状上皮，而后部且与之紧邻处，可见一个由柱状、立方状或假复层上皮衬里的扩张涎腺导管。最终诊断为与下颌下腺导管黏液潴留囊肿（舌下囊肿）紧密相关的表皮样囊肿。
口腔底部同时存在分别具有表皮样囊肿和黏液潴留囊肿特征的两种囊性病变的情况罕见，其发病机制引人关注，尤其是在新生儿中。
