International Journal of Pediatric Otorhinolaryngology, 24 (1992) 261-268 0 1992 Elsevier Science Publishers B.V. All rightsreserved 0165-5876/92/$05.00

PEDOT

261

00815

Teratoma of the tongue: a case report and review of the literature Anil K. Lalwani

and Thomas

L. Engel

Department of Otolaryngology - Head & Neck Surgery, University qf California, San Francisco, CA (USA) (Received 3 October 1991) (Revised version received 9 December (Accepted 15 December 1991)

Key words; Teratoma

of the tongue;

Dermoid

cyst; CO,

1991)

laser

Abstract

Teratoma of the tongue is a rare tumor of the oral cavity with only six reported cases in the literature. Teratomas are composed of ectoderm, mesoderm and endoderm with differentiation to identifiable tissues and organs. Embryologically, they are thought to occur as a result of displacement of cells from normal tissue during fetal life. In the tongue, the teratoma may result from misplaced cells from the tuberculum impar. Differential diagnosis of tongue lesions includes thyroglossal duct cyst, lingual thyroid, lymphangioma, hemangioma, dermoid cyst, granular cell myoblastoma and heterotopic gastric mucosal cyst. Computerized tomography is useful in differential diagnosis and defining extent of disease. Surgical excision with laser is curative and recurrence is rare.

Introduction

Teratomas of the head and neck are common, but rarely occur in the oral cavity. The usual sites of involvement in the head and neck are the nasopharynx and the cervical region which frequently present with airway obstruction. Teratoma of the tongue is a rare tumor of the oral cavity with only six reported cases in the literature [1,3,7,11,13,14]. We present a case of benign cystic teratoma of the tongue and review the literature. Correspondence to: Dr. Anil K. Lalwani, 350 Parnassus, Suite 210, Department of Otolaryngology-Head & Neck Surgery, University of California at San Francisco, San Francisco, CA 94117-0958, USA.

262

Case report

A 2870 g baby girl was born to a 34-year-old woman (gravida 3, para 2) at term with an Apgar of 8/9 at 1 and 5 minutes. The prenatal and perinatal courses were uncomplicated. At birth, the baby was noted to have a large cystic mass protruding through the mouth that prevented oral feedings. There was no respiratory compromise. Examination revealed a broadly based, sessile, 4 x 5 cm multicystic mass arising from the right anterior dorsal tongue and extending to the base of the tongue (Fig. 1). The mass appeared to infiltrate the tongue musculature. Computerized tomography (CT) of the head and neck demonstrated a large ovoid mass arising from and contiguous with the tongue, primarily on the right side; no other pharyngeal or skeletal abnormalities were noted (Fig. 2). The lesion was completely excised, with a 5 mm margin of normal tongue tissue, using a hand held Cooper CO, laser (wavelength 10.6 micrometers, spot size 0.18 mm, power setting 8 watt, focal length of 100 mm) on continuous mode as the cutting instrument and primary closure of the tongue defect was performed (Fig. 3). Intraoperative blood loss was insignificant and limited to less than 2 ml. Microscopic examination revealed a mass lined by nonkeratinizing squamous epithelium, with numerous cystic spaces of variable histology and intertwined disorganized skeletal muscle. The cystic spaces contained branching papillary structures consistent with the choroid plexus, ciliated columnar respiratory epithelium, squamous epithelium and gastrointestinal epithelium (Figs. 4 and 5). Well differentiated neural tissue was identified throughout the mass (Figs. 4 and 5). Thus, the presence of ectodermal, mesodermal and endodermal tissue histologically combined with the clinical presentation confirmed the diagnosis of benign congenital cystic teratoma. The postoperative course was uncomplicated and oral feedings were begun on the second postoperative day. At eighteen months, there was no evidence of recurrence.

Discussion

Dysontogenetic cysts of the oral cavity include the epidermoid, the dermoid, and the teratoid type and have commonly been grouped together as dermoid cysts. Cystic teratoma is the least common subtype of these congenital dermoid cysts. Teratomas commonly occur in the sacrococcygeal region, ovary, testicle, retroperitoneum, mediastinum, and the orbit. Teratomas of the head and neck comprise 2-9% of all teratomas, but rarely occur in the oral cavity [19]. Teratomas are composed of ectodermal, mesodermal and endodermal derivatives with differentiation to identifiable tissues and organs. These lesions should be differentiated from the following: an epidermoid cyst composed of epidermal cells; a dermoid cyst composed of ectodermal and mesodermal derivatives; a teratoid cyst, which is a poorly differentiated tumor derived from all three germ layers; and an epignathus with presence of developmental fetal organs and limbs. Teratomas

Fig. 1. Photomicrograph of the neonate demonstrating a broadly based, sessile, 4 x 5 cm multicystic mass arising from the right anterior dorsal tongue and extending to the base of the tongue. Fig. 2. Computerized tomography of the head and neck demonstrates a large ovoid mass contiguous with the tongue and extending to the base of the tongue (arrows). Fig. 3. Postoperative photograph mal tongue without residual

shovving tumor.

nor-

Fig. 4. Photomicrograph shows cystic spaces lined by striated respiratory epithelium (A), squamous epithelium (A), and gastrointestinal mucosa (B) and neural tissue (N). (Hematoxylin-eosin stain.) Fig. 5. Photomicrograph shows cystic space containing branching papillary structures consistent with the choroid plexus (P) and adjacent neural tissue (N). (Hematoxylin-eosin stain.)

265

tend to be midline lesions, although they most often present clinically as more prominent on one side. Gonadal, sacrococcygeal, oropharyngeal and nasopharyngeal teratomas preponderate in females; however, in the cervical region and at other sites, the sex incidence is equal [4,19]. No racial predilection has been proven 1191. In 1966, Miller and Owens [14] reported the first case of teratoma of the tongue occurring on the lateral aspect of the dorsal tongue of a newborn. The tumor was successfully excised at birth without recurrence at five years. Subsequently, five additional cases of teratoma of the tongue have been reported in the literature (Table I> [1,3,7,11,13]. Rodin and Singla [161 reported a case of teratoma of the tongue which had been previously reported by Ashley and Shafer [3]. Of the seven total reported cases, three patients have been male, three have been female, and one was unspecified. These statistics suggest an absence of sexual predilection. which is in contrast to the 6: 1 female predominance in nasopharyngeal teratomas [8]. All tumors presented at birth. The size of the tumor varied from 1 x 2 cm to 10 x 12 cm. Feeding difficulty was common and respiratory distress was unusual, even with large tumors. This observation is not surprising, since neonates are obligate nasal breathers. While not reported with teratoma of the tongue, teratomas of the oral cavity may be associated with polyhydraminos in utero secondary to fetal pharyngeal obstruction [I 81. All tumors were surgically excised at birth. Associated congenital anomalies in the seven patients included inguinal hernia in two patients, umbilical hernia (one patient), hydrocele (one patient), and cleft palate (one patient). One patient presented with teratoma of the floor of mouth 5 months after excision of a tongue teratoma. The significance of associated congenital anomalies and multiple teratomas is unclear. Berry et al. [6] reported an increased incidence of pelvic organ and tissue anomalies in patients with sacrococcygeal teratomas that could possibly be secondary to local growth effects due to the presence of the teratoma. Histopathologically, all tumors were benign, with no evidence of malignancy. This is in striking contrast to sacrococcygeal, gonadal, mediastinal and retroperitoneal teratomas, where a malignant component may be present in 10% to SO% of patients [13]. In ovarian teratomas, Norris et al. [15] found that the malignant potential is reflected by the immaturity of tissue, the amount of neuroepithelium, and the size of the teratoma. In sacrococcygeal teratomas, an abundance of neuroectodermal tissue signified a poorer prognosis [lo]. Mahour et al. [131 noted a higher malignancy rate in childhood teratomas presenting beyond the newborn period. In the head and neck, however, the presence of immature or differentiating neural cells has little prognostic significance [.51.The malignant potential of tongue teratomas is unknown due to paucity of cases and limited follow-up. Embryologically, teratomas are thought to arise from primordial germ cells in their course of migration during embryogenesis. Alternatively, they may occur as a result of displacement of specific or totipotential cells from normal tissue during fetal life [9]. Ashley [2] has suggested that extragonadal midline teratomas, such as the case reported, may represent incomplete conjoint twins. In the tongue, the cyst

Tongue

M

?

F

M

M

F

2

3

4

5

6

Our

Benign

Birth

Benign Benign

Birth

Birth

Benign

4x3x1.5

Benign

Birth

Birth

6.5~4.5~2

Benign

Birth

4x5

3.3 x 3.0

10x12

1x2

7x3.5x3

Benign

Birth

Size(cm)

Histology

Age at diagnosis

(-)RD (?)FD (-)RD (+)FD

f-)RD (+)FD

f+)FD ulcerated NA

NA

(+)FD

f-)RD

Symptoms

Surgery

Surgery

Surgery

Surgery

Surgery

Surgery

Surgery

Therapy

RD, respiratory distress; FD, feeding difficulty; NA, not available; F, female; M, male; FOM, floor of mouth.

Foramen cecum Right anterodorsal tongue

Anterior 2/3 tongue

Right anterolateral tongue base of tongue Left tongue

F

1

Location

Sex

Ref

Teratoma of the tongue

TABLE I

1.5 years

None

2 years

None

Submandibular gland teratoma Inguinal hernia umbilical hernia hydrocele None

None

None

Cleft palate FOM teratoma None

None

Other

None

5 years

Follow-up

may result from misplaced cells from the tuberculum impar at the fourth week of gestation [14]. Differential diagnosis of tongue lesions includes, among others, thyroglossal duct cyst, lingual thyroid, lymphangioma, hemangioma, hamartomas, gliomas, dermoid cyst, heterotopic gastric mucosal cyst, retention cyst, squamous cell carcinoma, rhabdomyosarcoma and granular cell myoblastoma. Not infrequently, the definitive diagnosis is uncertain and is established postoperatively after surgical intervention. In a patient with an oral cavity mass, CT is useful in evaluating oropharyngeal, nasopharyngeal, hypopharyngeal, laryngeal and tracheal airways. Likewise, associated skeletal, vertebral, cranial and central nervous system anomalies can be studied. In our case, preoperative CT was useful in formulating a differential diagnosis, defining the tumor origin and determining the anatomic extent of disease. In previous case reports of teratomas of the tongue the authors did not utilize CT scan in their evaluations. While not present in the CT scan of our patient, calcifications and cysts within a mass may be seen, and are typical of teratomas of the head, neck, and other body sites [17]. The role of magnetic resonance scanning remains to be delineated. Routine or diagnostic prenatal ultrasound may detect an incidental tongue mass suggestive of teratoma. Mixed echogenic signals suggestive of semisolid and semicystic components are typical findings on ultrasound. In the presence of polyhydroaminos, Holinger and Birnholz [12] recommend in utero ultrasound evaluation of the airway to exclude oropharyngeal teratoma. Definitive treatment of teratoma of the tongue is complete surgical excision. Unlike cervical or nasopharyngeal teratomas, feeding difficulty rather than respiratory distress is the primary indication for surgical intervention. Surgery is curative and the prognosis is excellent. The CO, laser is helpful in obtaining exact surgical margins while maintaining normal tissue. Additionally, CO, laser aids in hemostasis and minimizing blood loss which is paramount in the neonate. Recurrence has not been reported.

References 1 Antoine, F.A., White, J.D. Jr., Heffner, D.K. and Case, C., Teratoma of the tongue: a case report and literature review. Laryngoscope, 95 (1985) 1262-63. 2 Ashley, D.J.. Origin of teratomas. Cancer 32 (1973) 390-394. 3 Ashley, J.V. and Shafer, A.D., Teratoma of the tongue in a newborn. Cleve. Clin. Q., 50 (1983) 34-36. 4 Bale, P.M., Painter, D.M. and Cohen, D., Teratomas in childhood. Pathology, 7 (1975) 209-218. 5 Batsakis, J.G., Teratomas of the head and neck. In Tumors of the Head and Neck, 2nd Edn.. Williams & Wilkin, Baltimore, 1979, pp. 226-232. 6 Berry, CL., Keeling, 3. and Hilton, C., Coincidence of congenital malformations and embryonic tumors of childhood. Arch. Dis. Child., 45 (1970) 229-231. 7 Bras, G., Butts, D. and Hoyte, D.A., Gliomatous teratoma of the tongue: report of a case. Cancer 24 (1969) 1045-1060.

268 8 Foxwell, P.B. and Kelham, B.H., Teratoid tumors of the nasopharynx. J. Laryngol. Otol., 72 (1958) 647-657. 9 Gonzales-Cruzzi, F., Extragonal teratomas. In Atlas of Tumor Pathology, 2nd series, Fascicle 18. Armed Forces Institute of Pathology, Washington, DC, 1982. 10 Gonzales-Cruzzi, F., Winklen, R.F. and Mirkin, D.L., Sacrococcygeal teratomas in infants and children: relationship of history and prognosis in 40 cases. Arch. Pathol. Lab. Med., 102 (1978) 420-425. 11 Greir, D.A. and MacNerland, R.H., Benign teratoma of the tongue. Ill. Med. J. 132 (1967) 43-45. 12 Holinger, L.D. and Birnholz, J.C., Management of infants with prenatal ultrasound diagnosis of airway obstruction by teratoma. Ann. Otol. Rhinol. Laryngol., 96 (1987) 61-64. 13 Mahour, G.H., Landing, B.H. and Woolley, M.M., Teratomas in children: clinicopathologic studies in 133 patients. Z. Kinderchir., 23 (1978) 365-380. 14 Miller, A.P. and Owens, J.B. Jr., Teratoma of the tongue. Cancer, 19 (1966) 1583-1586. 15 Norris, H.J., Zinkin, H.J. and Benson, W.L., Immature (malignant) teratoma of the ovary: a clinical and pathological study of 58 cases. Cancer 37 (1976) 2359-2372. 16 Rodin, A.D. and Singla, P., Teratomas of the tongue present at birth. Pediatr. Pathol., 3 (1985) 291-298. 17 Senac, M.O. and Segall, H.D., CTdiagnosis of an atypical nasopharyngeal teratoma in a newborn. AJNR 8 (1987) 710-712. 18 Teal, L.N., Angtuaro, T.L., Jimenez, J.F. and Quirck, J.G., Fetal teratomas: antenatal diagnosis and clinical management. J. Clin. Ultrasound 16 (1988) 329-336. 19 Ward, R.F. and April, M., Teratomas of the head and neck. Otolaryngol. Clin. North Am. 22 (1989) 621-629.

Teratoma of the tongue: a case report and review of the literature.

Teratoma of the tongue is a rare tumor of the oral cavity with only six reported cases in the literature. Teratomas are composed of ectoderm, mesoderm...
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