Received 12 November 2009; received in revised form 18 December 2009; accepted 29 December 2009. published online 22 January 2010. Corrected Proof
Abstract
A mature teratoma is a neoplasm which rarely occurs in the Eustachian tube. A 10-month-old girl presented with persistent otorrhea and an increasing nasal obstruction was finally diagnosed as a Eustachian tube mature teratoma. The neoplasm was successfully resected transorally using an endoscope.
Mature teratoma is a true neoplasm composed of all three basic germ cell layers. It usually arises from the saccrococygeal region with a female predominance of 6:1 [1]. It rarely occurs in the head and neck region, especially in the Eustachian tube [1]. We present a case of mature teratoma of the Eustachian tube in a 10-month-old girl with a 4-month history of refractory otitis media and 3-month history of increasing nasal obstruction.
2. Case report
A 10-month-old girl was referred to the Beijing Children's Hospital for refractory otitis media and a nasopharyngeal mass. She was born full-term by cesarean section with normal apgar scores and a birth weight of 2850g. She had no history of feeding difficulties or aspiration. Her mother had an upper respiratory infection during the pregnancy and used some Chinese herbal medicine. At 6 months of age she had left otorrhea without an obvious etiology. She was diagnosed with otitis media and treated with oral and IV antibiotics plus otic drops at another hospital, but the otorrhea persisted. At the same time, she had an increasing nasal obstruction, first on the left, then both sides and progressed to obstructive sleep apnea. A magnetic resonance scanning (MRI) showed a mass in the nasopharynx with no intracranial extension (Fig. 1a). A nasopharyngeal biopsy was performed and showed lymphatic tissue with chronic infection. She was then referred to our department.
Fig. 1. (a) MRI shows a mass in the posterior of the nasopharynx extending down to the oropharynx, and with no intracranial extension. (b) CT 3-dimension reconstruction showed that lesion with a margin enhancing had core-like structure in the posterior nasopharynx and a pedicle into the significantly widened left Eustachian tube orifice up to the hypotympanum. (c) An air-containing middle ear with a left dilated Eustachian tube. (d) Under light microscopy, the lesion is covered with stratified squamous epithelium and some respiratory epithelium, but contained skin appendages (hair follicles, sebaceous glands and sweat glands), well-differentiated fibroadipose tissue, cartilage and dilated small blood vessels and lymphatic vessels.
On physical examination, a left unilateral middle ear effusion was found. After the muco-purulent effusion was aspirated, the anterior–inferior part of the tympanic membrane was bulging with a mass, but no obvious perforation was found. There was a white mass seen in the nasopharynx. Endoscopic examination revealed that the mass seemed to be originated from the left lateral wall of the nasopharynx and its surface had “hair” on it (Fig. 2A). The hearing was evaluated by air-conducted Click ABR. The hearing threshold of the left ear was 70dB, and 60dB on the right.
Fig. 2. (A) Endoscopic examination revealed “hairs” on the surface of the nasopharyngeal mass. (B) Postoperatively the orifice of the left Eustachian tube is intact, but larger than the right side. (C) On gross examination, the lesion was rounded with hair at the nasopharynx end and a pedicle from the Eustachian tube. (D) There was a perforation in the anterior–inferior part of the tympanic membrane after surgery.
CT images demonstrated a 3.2cm×1.8cm×1.4cm heterogeneous soft tissue cystic mass hanging in the nasopharynx extending down to the oropharynx. The lesion had a margin-enhancing core-like structure in the posterior nasopharynx and a pedicle in the significantly widened left Eustachian tube extending up to the hypotympanum. It confirmed the findings of MRI (Fig. 1b). At the same time the CT also showed opacification of the middle ear and mastoid cells on both sides.
In view of the increasing nasal obstruction and sleep apnea and with the risk of a possible facial nerve palsy if a lateral neck surgical was used, we elected to do a transoral resection of the mass.
Under general anesthesia, a Crowe-Davis mouth gag was inserted. Using a transnasal 0̊ Storz endoscope with 3mm diameter, the attachment site of the mass could not be exposed clearly by elevation of the soft palate with a catheter, so a soft palate incision was made. This exposed the attachment of the mass which was defined and a ligature was used with traction on the mass to remove it completely. After closing the palatal incision the operation was completed. We could see the orifice of the left Eustachian tube which remained intact, but larger than the right side (Fig. 2B). A perforation of the anterior–inferior tympanic membrane was seen and the mucous membrane of the promontory had a small amount of bleeding.
On gross examination, the lesion was white and enlarged like a “ball” at the nasopharynx end, with a smooth and intact surface, except at the middle ear portion. There was some “hair” on the nasopharynx end (Fig. 2C). Microscopically, the lesion was covered with stratified squamous epithelium and some respiratory epithelium. It also contained skin appendages (hair follicles, sebaceous glands and, sweat glands), well-differentiated fibroadipose tissue, cartilage and dilated small blood vessels and lymphatic vessels (Fig. 1c). A mature teratoma was the final microscopic diagnosis.
Immediately after the operation, the ear discharge and sleep apnea cleared, but the girl's mother complained of hearing air coming from her left ear. The hearing was reevaluated using the ABR. The threshold of the right ear was 20dB, and the left was 50dB. The tympangram showed a type A on the right. One week later, there was a persistent perforation in the left tympanic membrane (Fig. 2D). A postoperative CT demonstrated an air-containing middle ear space with a dilated Eustachian tube on the left side. (Fig. 1c).
3. Discussion
We used PUBMED to search the literature using the keywords, mature teratoma and Eustachian tube. We found only 3 papers reported in the English literature. The Eustachian tube is a rare site for a mature teratoma. One author noted a middle ear teratoma arising from or involving the Eustachian tube [2]. The present case is a mature teratoma of the Eustachian tube presenting with refractory otitis media and nasal obstruction causing sleep apnea. Compared with cases reported in 3 papers, otorrhea and nasal blockage symptoms emerged early, and the mass involved the middle ear and nasopharynx was noticed before surgery. There were three reasons for considering the Eustachian tube as the site of origin: (1) symptoms of otorrhea, nasal obstruction, sleep apnea, with a mass located in the middle ear and nasopharynx. (2) Discoherent mucous membrane could only be seen at the peduncalated end of the resected mass. (3) A white bulging mass could be observed in the anterior–inferior part of tympanic membrane preoperatively. Except for the promontory surface, no bleeding happened postoperatively from the Eustachian tube orifice or nasopharynx.
Otorrhea and nasal obstruction were the main complaints due to the mechanical obstruction by the mass and were not specific for the diagnosis of a teratoma. We diagnosed this case as a first branchial cleft cyst preoperatively based mainly on radiological findings. However, all 3-germ-layer cells differentiated well, which indicate a mature teratoma, were found on microscopic examination of the pathological specimen. A mature teratoma is composed of all 3 germinal layers: ectoderm, mesoderm and endoderm. This differentiates it histologically from hairy polyps, dermoid cysts and hamartomas. Hairy polyps contain just two germinal layers of mesoderm and ectoderm foreign to the region [3]. “Dermoid cysts contain desquamated epithelial products and developed from the incomplete breakdown of epithelium at embryological fusion lines or from traumatic implantation of epithelium” [3]. Hamartomas refer to an excessive, but focal overgrowth of cells and tissue native to the organ where they occur [4].
In regards to the treatment of a mature teratoma, resection of the mass is thought to be curative. In our case, the teratoma was successfully resected and removed from the Eustachian tube with traction. As reported by Forrest et al. [5], the pedicle was attached loosely to the Eustachian tube, which allow the mass slip down and obstruct the larynx. The transnasal and transoral approach should be considered first to avoid serious damage to other structures in the area. Our patient had been followed for 2 months by telephone, and she got a cold and left otitis media occurred again 1 month later after surgery. Otorrhea stopped soon after ear drops and oral antibiotics used. We scheduled to follow up her until the tympanic membrane perforation cured.
[4]. [4]Wenig RM, Heffner DK. Respiratory epithelial adenomatoid hamartomas of the sinonasal tract and nasopharynx: a clinicopathologic study of 31 cases. Ann. Otol. Rhiniol. Laryngol.1995;104:639–645.
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g. She had no history of feeding difficulties or aspiration. Her mother had an upper respiratory infection during the pregnancy and used some Chinese herbal medicine. At 6 months of age she had left otorrhea without an obvious etiology. She was diagnosed with otitis media and treated with oral and IV antibiotics plus otic drops at another hospital, but the otorrhea persisted. At the same time, she had an increasing nasal obstruction, first on the left, then both sides and progressed to obstructive sleep apnea. A magnetic resonance scanning (MRI) showed a mass in the nasopharynx with no intracranial extension (
