ORAL CAVITY TUMORS (1-7)

 

Epulis (8)

Benign granular cell tumor which is solid. It arises from the alveolar ridge. Color and power Doppler usually demonstrates marked blood flow in the tumor. It is a self limiting lesion and responds to conservative excision.

Foregut duplication cyst (10)

Enteric duplication cyst may occur in the floor of the mouth. It is cystic in nature and may closely mimic a ranula.

Epignathus (11,12)

Oropharyngeal teratoma mostly arising from the palate. It appears as solid-cystic tumor with mixed areas of hypo and hyperechogenicity, may have calcifications and is found in association with polyhydramnios. It can cause significant morbidity and mortality

Median palatal mucosal cyst [Epstein’s pearl]

Benign and self resolving

Vascular hamartomas (4,5)

Hemangiomas or lymphangiomas [cystic hygromas] and are usually located on the tongue. These tumors may appear as solid- cystic masses on ultrasound and require surgical excision.

Other tumors of the tongue (1-16)

• Thyroglossal duct cyst
• Lingual thyroid
• Dermoid cyst
• Granular cell myoblastoma and
• Heterotopic gastric mucosal cyst.

 

RANULA

- RETENTION CYST, MUCOCELE, ORAL PSEUDOCYST

 

A congenital ranula is a cystic malformation seen in the oral cavity that usually results from the obstruction of the sublingual or minor salivary glands. These pseudocysts are normally located in the sub-lingual space between the mylohyoid muscle and the lingual mucosa.

 

PREVALENCE

 

The incidence of a congenital ranula is estimated to be 0.74%.

 

ETIOLOGY

 

A ranula is a fluid collection that occurs either due to:
1. Disruption of minor salivary ducts leading to extravasation of mucous structures into adjacent structure and resulting in a mucous extravasation cyst. These are more common in children and young adults and rarely occur in neonates. The ranula is not lined by an epithelium in this case.
2. A blocked duct causing proximal expansion and resulting in a mucous retention cyst, seen in neonates and the fluid collection is lined by salivary duct epithelium.

Types: Ranulas can be classified according to their site of location. They can be

• A simple ranula – located in the floor of the mouth,
• A cervical ranula – located in the paracervical region,
• And a plunging ranula – located near the upper airway and extending into the floor of the mouth. [plunging ranulas exhibit a so called ‘tail sign’ on MRI].

 

ULTRASOUND

 

• A hypoechoic cystic mass in the floor of the mouth, with no solid components.
• If very large, the mass may displace the tongue upwards.
• No vascularity can be seen within this cystic structure.

If the mass becomes too large, it may interfere with swallowing resulting in polyhydramnios.

 

REFERENCES


1. Shipp TD, Bromley B, Benacerraf B. The ultrasonographic appearance and outcome for fetuses with masses distorting the fetal face. J Ultrasound Med. 1995 Sep; 14(9):673-8.
2. Fernandez Moya JM, Cifuentes Sulzberger S, Diaz Recasens J, et al. Antenatal diagnosis and management of a ranula. Ultrasound Obstet Gynecol. 1998 Feb; 11(2):147-8.
3. Onderoglu L, Saygan-Karamursel B, Deren O, et al. Prenatal diagnosis of ranula at 21 weeks of gestation. Ultrasound Obstet Gynecol. 2003 Oct; 22(4):399-401.
4. Rousseau T, Couvreur S, et al. Prenatal diagnosis of enteric duplication cyst of the tongue. Prenat Diagn. 2004 Feb; 24(2):98-100.
5. Akyol MU, Orhan D. Lingual tumors in infants: a case report and review of the literature. Int J Pediatr Otorhinolaryngol. 2004 Jan; 68(1):111-5.
6. Jorgenson RJ, Shapiro SD, Salinas CF, Levin LS. Intraoral findings and anomalies in neonates. Pediatrics. 1982 May; 69(5):577-82.
7. Polak P, Santavy J, Micanik B, et al. An unusual tumor of the oral cavity in a fetus and prenatal ultrasonography--case report. Ceska Gynekol. 2002 May; 67(3):163-7.
8. Nakata M, Anno K, Matsumori LT, et al. Prenatal diagnosis of congenital epulis: a case report. Ultrasound Obstet Gynecol. 2002 Dec; 20(6):627-9.
9. Saheeb BD. Recurrent congenital bilateral ranula: a case report.
SADJ. 2001 Aug; 56(8):366-8.
10. Kong K, Walker P, Cassey J, O"Callaghan S. Foregut duplication cyst arising in the floor of mouth. Int J Pediatr Otorhinolaryngol. 2004 Jun; 68(6):827-30.
11. Morof D, Levine D, Grable I, et al. Oropharyngeal Teratoma: Prenatal Diagnosis and Assessment Using Sonography, MRI, and CT with Management by Ex Utero Intrapartum Treatment Procedure. AJR Am J Roentgenol. 2004 Aug; 183(2):493-6.
12. Gaucherand P, Rudigoz RC, Chappuis JP. Epignathus: clinical and sonographic observations of two cases.
Ultrasound Obstet Gynecol. 1994 May 1;4(3):241-4
13. Ikemura K, Kakinoki Y, Nishio K, Suenaga Y. Cysts of the oral mucosa in newborns: a clinical observation. J UOEH. 1983 Jun 1; 5(2):163-8.
14. Lalwani AK, Engel TL. Teratoma of the tongue: a case report and review of the literature. Int J Pediatr Otorhinolaryngol. 1992 Nov; 24(3):261-8.
15. Stevens GH, Schoot BC, Smets MJ, et al. The ex utero intrapartum treatment (EXIT) procedure in fetal neck masses: a case report and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2002 Jan 10; 100(2):246-50.
16. Haberal I, Gocmen H, Samim E. Surgical management of pediatric ranula. Int J Pediatr Otorhinolaryngol. 2004 Feb; 68(2):161-3.

 

EPIGNATHUS

 - TERATOMA OF ORAL CAVITY OR PHARYNX 

Epignathus is a congenital teratoma of the hard palate in the region of Rathke's pouch.

Incidence: 1:35,000 to 1:200000 live births.
 

PATHOLOGY (1,2)
  • Consist of tissue derived from any of the three germinal layers.
  • Most contain adipose tissue, cartilage, bone and nervous tissue.
  • Most are benign.
  • Most are thought to arise from pluripotent cells in Rathke’s pouch that grow in a disorganized manner.
  • Some arise from hard and soft palate, pharynx, tongue or jaw.
  • They grow into the oral cavity, nasal cavity or intracranially from their sites of origin.
  • They can completely occupy the mouth and airways and lead to rapid neonatal asphyxia unless they are recognized antenatally.

 

ULTRASOUND (2-5)



  1. Solid tumor occupying the oral cavity and extending a variable distance out of the oral cavity.
  2. ± Calcifications.
  3. ± Cystic components.
  4. Polyhydramnios due to obstruction to fetal swallowing (poor prognosis).
  5. The tumor is usually unidirectional and extends into the oral cavity. Bidirectional epignathus has been reported (8) involving both the oral cavity and intracranial structures.
  6. Maternal serum alpha-fetoprotein may be elevated.
  7. Earliest diagnosis – 15 weeks (8). Most are diagnosed after 20 weeks.

 

CT Scan on Day 1

 

 

ASSOCIATED ANOMALIES (2,6, 7)

6% of fetuses have associated anomalies:

  • Multiple facial hemangiomas.
  • Cleft palate.
  • Bifid tongue or nose.
  • Branchial cysts.
  • Hypertelorism.
  • Umbilical hernia.
  • Congenital heart defects.
  • Chromosomal aberrations that have been associated include (10-12): 45,Xx/46,r(X) mosaicism; trisomy 13; duplication of 1q and 19 p; inverted proximal 1q duplication.

 

DIFFERENTIAL DIAGNOSIS
  1. Teratomas of the neck.
  2. Cephaloceles.
  3. Conjoined twins.
  4. Other facial tumors (hemangioma, lymphangioma, neurofibroma and granular myoblastoma) (9).

 

 

REFERENCES
  1. Carney JA, Thompson DP, Johnson CL et.al. Teratomas in children: Clinical and pathologic aspects. J Pediatr Surg 1972;7:271.
  2. Shah BL, Vasan U, Raye JR. Teratoma of the tonsil in a premature infant. Case report and review of the literature. Am J Dis CHILD 1979;133:79.
  3. Chervanek FA, Tortora M, Moya FR et.al. Antenatal sonographic diagnosis of epignathus. J Ultrasound Med 1984;3:235.
  4. Hawkins DB, Park R. Teratoma of the pharynx and neck. Ann Otol Rhinol Laryngol 1972;81:848.
  5. Kang KW, Hissong SL, Langer A. Prenatal ultrasonic diagnosis of epignathus. J Clin Ultrasound 1978;6:330.
  6. Fraumeni JF Jr, Li FP, Dalager N. Teratomas in children: Epidemiologic features. J Natl Cancer Inst 1973;51:1425.
  7. Wilson JW, Gehweiler JA. Teratoma of the face associated with a patent canal extending into the cranial cavity (Rathke's pouch) in a three week old child. J Pediatr Surg 1970;5:349.
  8. Gull I, Wolman I, Har-Toov J et.al. Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy. Ultrasound Obstet Gynecol 1999;13:271-273.
  9. Meizner I, Shalev J, Mashiach R et.al. Prenatal ultrasonographic diagnosis of oral granular cell myoblastoma. J Ultrasound Med 2000;19:337-339.
  10. Witters I, Moerman P, Louwagie D et.al. Second trimester prenatal diagnosis of epignathus teratoma in ring X chromosome mosaicism with inactive ring X chromosome. Ann Genet 2001;44:179-182.
  11. Yapar EG, Ekici E, Gokmen O. Sonographic diagnosis of eopignathus, prosencephaly, meromelia and ligohydramnios in a fetus with trisomy 13. Clin Dysmorphol 1995;4:266-271
  12. Schwartz S, Raffel LJ, Sun CC et.al. An unusual mosaic karyotype detected through prenatal diagnosis with duplication of 1q and 19 p and associated teratoma development. Teratology 1992;46:399-404.