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Heterotopic gastrointestinal cyst partially lined with dermoid cyst epithelium
Heterotopic gastrointestinal cyst partially lined with dermoid cyst epithelium Marcelo Macedo Crivelini, DDS, PhD,a Ana Maria Pires Soubhia, DDS, PhD,a Éder Ricardo Biazolla, DDS, PhD,b and Sebastião Conrado Neto, MM,c São Paulo, Brazil STATE UNIVERSITY, ARAÇATUBA
We report a rare heterotopic gastrointestinal cyst located in the right submandibular/submental area with histopathologic features that included portions resembling a dermoid cyst. Some theories of pathogenesis are discussed, and an origin of this lesion in entrapped undifferentiated endodermal cells is suggested. (Oral Surg Oral Med Oral Pathol Oral Radiol
Endod 2001;91:686-8)
Heterotopic gastrointestinal cysts of the oral cavity are rare lesions encountered most frequently within the anterior 2/3 of the tongue; the patients are diagnosed in infancy or early childhood.1-9 As of 1996, approximately 32 cases were reported in the English-language literature and a male predilection was noted.9 The lining of these lesions resembles gastric or intestinal mucosa, and stratified squamous, simple columnar, or ciliated columnar epithelium may be seen. Simultaneous occurrence with a dermoid cyst in the floor of the mouth has been reported.10,11 To our knowledge, a dermoid cyst–type zone as a prominent histopathologic feature of a heterotopic gastrointestinal cyst has not been previously reported. We describe such a lesion and discuss its pathogenesis. CASE REPORT A 13-year-old boy had a firm, painless 4.5-cm round swelling in the right submandibular/submental area (Fig 1) involving the floor of the mouth with slight intraoral swelling. The lesion was excised with the patient under general anesthesia (Fig 2), and no recurrence was observed after 1 year. The gross specimen measured 4.6 × 4.0 × 2.0 cm and consisted of a unilocular cystic lesion with focal gummy contents. Microscopically, the cavity was partly lined with gastric mucosa having tall columnar mucous cells on the surface and numerous closed short crypts lined with goblet cells that resembled fundus glands. Stratified squaPresented at VI Congresso e XXIV Jornada Brasileira de Estomatologia, Diamantina city–MG, 1998. aAssistant Professor, Department of Oral Pathology, School of Dentistry, State University, Araçatuba, São Paulo. bAssociate Professor, Department of Oral Pathology, School of Dentistry, State University, Araçatuba, São Paulo. cPhysician, Oral Oncology Center, School of Dentistry, State University, Araçatuba, São Paulo. Received for publication May 25, 2000; returned for revision Jul 12, 2000; accepted for publication Jan 10, 2001. Copyright © 2001 by Mosby, Inc. 1079-2104/2001/$35.00 + 0 7/14/114156 doi:10.1067/moe.2001.114156
686
Fig 1. Swelling of right submandibular/submental area.
Fig 2. View of cystic lesion during extraoral excision. mous, simple columnar, and ciliated columnar epithelium were observed in other areas (Figs 3 and 4). A small part of the lesion had features consistent with a dermoid cyst— namely, keratinized stratified squamous epithelium with
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 91, Number 6
Crivelini et al 687
Fig 3. Heterotopic gastrointestinal cyst lined with different types of epithelium: ciliated columnar (A), stratified squamous (B), and gastric mucosa-like (C) (hematoxylin-eosin stain, original magnification ×100).
Fig 5. Keratinized stratified squamous epithelium with sebaceous glands characteristic of dermoid cyst–lined part of heterotopic gastrointestinal cyst (hematoxylin-eosin stain, original magnification ×400).
Fig 4. Crypts lined with goblet cells resemble glands of gastric fundus (hematoxylin-eosin stain, original magnification ×400). dermal appendages (hair follicles and sebaceous glands) in the cyst wall (Fig 5).
DISCUSSION We report a rare case of a heterotopic gastrointestinal cyst focally lined by epithelium that resembled a dermoid cyst. The more accepted theories of pathogenesis propose that the origin of these lesions is misplaced embryonic gastric remnants entrapped in the midline of the tongue, in the tuberculum impar region.12 Although this theory does not explain the rare occurrence of the lesion at other locations, such as the lips and oral mucosa, it could be accepted in the present case because the lesion developed
in the submandibular/submental area adjacent to the tongue, extending to the midline and hyoid bone area. However, it is more difficult to explain the development within this lesion of dermoid cyst–like areas from embryonic gastric remnants. Another theory proposes that the lesion arises from islands of endoderm that lined the primitive stomodeum and became entrapped during fusion of the embryonic processes. Differentiation into gastrointestinal epithelium may be related to different environmental and inductive influences.13 As discussed previously, the finding of a focal dermoid cyst–like area in our case did not permit us to accept this theory, because endoderm from the primitive stomodeum should be unable to differentiate into epidermis-like epithelium. Instead, we agree with the suggestion of Woolgar and Smith14 that entrapped undifferentiated endodermal cells give rise to these cysts. The aberrant differentiation would also occur under the action of environmental and inductive influences. Khunamornpong et al8 reported a case with associated pancreatic tissue and supported this concept. Several other hypothetical pathogenetic mechanisms were proposed, including origin in the thyroglossal duct and salivary retention cysts.15,16 Conservative surgical excision is the treatment of choice, with long-term follow-up recommended.1-3,5,7,8 Recurrence is uncommon17 but can occur many years later, probably because of incomplete excision.13
688 Crivelini et al
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY June 2001
REFERENCES
10. Eppley BL, Bell MJ, Sclaroff A. Simultaneous occurrence of dermoid and heterotopic intestinal cysts in the floor of the mouth of a newborn. J Oral Maxillofac Surg 1985;43:880-3. 11. Arcand P, Granger J, Brochu P. Congenital dermoid cyst of the oral cavity with gastric choristoma. J Otolaryngol 1988;17:21922. 12. Gorlin RJ, Jirasek JE. Oral cysts containing gastric or intestinal mucosa. An unusual embryological accident or heterotopia. Arch Otolaryngol 1970;91:594-7. 13. Daley TD, Wysocki GP, Lovas GL, Smout MS. Heterotopic gastric cyst of the oral cavity. Head Neck Surg 1984;7:168-71. 14. Woolgar JA, Smith AJ. Heterotopic gastrointestinal cyst of oral cavity: a developmental lesion? Oral Surg Oral Med Oral Pathol 1988;66:223-5. 15. Willis RA. Some unusual heterotopies. Br Med J 1968;3:267-72. 16. Gruskin P, Landolfe FR. Heterotopic gastric mucosa of the tongue. Arch Pathol 1972;94:1846. 17. Lalwani AK, Lalwani RB, Bartlett PC. Heterotopic gastric mucosal cyst of the tongue. Otolaryngol Head Neck Surg 1993; 108:204-5.
1. Chou L, Hansen LS, Daniels TE. Choristomas of the oral cavity: a review. Oral Surg Oral Med Oral Pathol 1991;72:584-93. 2. Martone CH, Wolf SM, Wesley RK. Heterotopic gastrointestinal cyst of the oral cavity. J Oral Maxillofac Surg 1992;50:1340-2. 3. Bite U, Cramer HM. Mixed heterotopic gastrointestinal and respiratory cyst of the lip: case report and review of the literature. Plast Reconstr Surg 1992;90:1068-72. 4. Lipsett J, Sparnon AL, Byard RW. Embryogenesis of enterocystomas-enteric duplication cysts of the tongue. Oral Surg Oral Med Oral Pathol 1993;75:626-30. 5. Surana R, Losty P, Fitzgerald RJ. Heterotopic gastric cyst of the tongue in a newborn. Eur J Pediatr Surg 1993;3:110-1. 6. Kinoshita Y, Honma Y, Otuka T, Shimura K. Gastrointestinal mucosal cyst of the oral cavity: report of case and review of literature. J Oral Maxillofac Surg 1994;52:1203-5. 7. Fernandes M, Miziara ID, Chung S, Mniti A, Iriya K. Choristoma de la muqueuse gastrique: description d’un cas. Rev Laryngol Otol Rhinol 1995;116:347-9. 8. Khunamornpong S, Yousukh A, Tananuvat R. Heterotopic gastrointestinal and pancreatic tissue of the tongue. A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;81:576-9. 9. Morgan WE, Jones JK, Flaitz CM, Hicks MJ. Congenital heterotopic gastrointestinal cyst of the oral cavity in a neonate: case report and review of literature. Int J Pediatr Otorhinolaryngol 1996;36:69-77.
Reprint requests: M. M. Crivelini Disciplina de Patologia Bucal Faculdade de Odontologia de Araçatuba–UNESP Caixa Postal 341–Araçatuba-SP–Brazil CEP 16015-050
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We report a rare heterotopic gastrointestinal cyst located in the right submandibular/submental area with histopathologic features that included portions resembling a dermoid cyst. Some theories of pathogenesis are discussed, and an origin of this lesion in entrapped undifferentiated endodermal cells is suggested. (Oral Surg Oral Med Oral Pathol Oral Radiol
Endod 2001;91:686-8)
Heterotopic gastrointestinal cysts of the oral cavity are rare lesions encountered most frequently within the anterior 2/3 of the tongue; the patients are diagnosed in infancy or early childhood.1-9 As of 1996, approximately 32 cases were reported in the English-language literature and a male predilection was noted.9 The lining of these lesions resembles gastric or intestinal mucosa, and stratified squamous, simple columnar, or ciliated columnar epithelium may be seen. Simultaneous occurrence with a dermoid cyst in the floor of the mouth has been reported.10,11 To our knowledge, a dermoid cyst–type zone as a prominent histopathologic feature of a heterotopic gastrointestinal cyst has not been previously reported. We describe such a lesion and discuss its pathogenesis. CASE REPORT A 13-year-old boy had a firm, painless 4.5-cm round swelling in the right submandibular/submental area (Fig 1) involving the floor of the mouth with slight intraoral swelling. The lesion was excised with the patient under general anesthesia (Fig 2), and no recurrence was observed after 1 year. The gross specimen measured 4.6 × 4.0 × 2.0 cm and consisted of a unilocular cystic lesion with focal gummy contents. Microscopically, the cavity was partly lined with gastric mucosa having tall columnar mucous cells on the surface and numerous closed short crypts lined with goblet cells that resembled fundus glands. Stratified squaPresented at VI Congresso e XXIV Jornada Brasileira de Estomatologia, Diamantina city–MG, 1998. aAssistant Professor, Department of Oral Pathology, School of Dentistry, State University, Araçatuba, São Paulo. bAssociate Professor, Department of Oral Pathology, School of Dentistry, State University, Araçatuba, São Paulo. cPhysician, Oral Oncology Center, School of Dentistry, State University, Araçatuba, São Paulo. Received for publication May 25, 2000; returned for revision Jul 12, 2000; accepted for publication Jan 10, 2001. Copyright © 2001 by Mosby, Inc. 1079-2104/2001/$35.00 + 0 7/14/114156 doi:10.1067/moe.2001.114156
686
Fig 1. Swelling of right submandibular/submental area.
Fig 2. View of cystic lesion during extraoral excision. mous, simple columnar, and ciliated columnar epithelium were observed in other areas (Figs 3 and 4). A small part of the lesion had features consistent with a dermoid cyst— namely, keratinized stratified squamous epithelium with
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 91, Number 6
Crivelini et al 687
Fig 3. Heterotopic gastrointestinal cyst lined with different types of epithelium: ciliated columnar (A), stratified squamous (B), and gastric mucosa-like (C) (hematoxylin-eosin stain, original magnification ×100).
Fig 5. Keratinized stratified squamous epithelium with sebaceous glands characteristic of dermoid cyst–lined part of heterotopic gastrointestinal cyst (hematoxylin-eosin stain, original magnification ×400).
Fig 4. Crypts lined with goblet cells resemble glands of gastric fundus (hematoxylin-eosin stain, original magnification ×400). dermal appendages (hair follicles and sebaceous glands) in the cyst wall (Fig 5).
DISCUSSION We report a rare case of a heterotopic gastrointestinal cyst focally lined by epithelium that resembled a dermoid cyst. The more accepted theories of pathogenesis propose that the origin of these lesions is misplaced embryonic gastric remnants entrapped in the midline of the tongue, in the tuberculum impar region.12 Although this theory does not explain the rare occurrence of the lesion at other locations, such as the lips and oral mucosa, it could be accepted in the present case because the lesion developed
in the submandibular/submental area adjacent to the tongue, extending to the midline and hyoid bone area. However, it is more difficult to explain the development within this lesion of dermoid cyst–like areas from embryonic gastric remnants. Another theory proposes that the lesion arises from islands of endoderm that lined the primitive stomodeum and became entrapped during fusion of the embryonic processes. Differentiation into gastrointestinal epithelium may be related to different environmental and inductive influences.13 As discussed previously, the finding of a focal dermoid cyst–like area in our case did not permit us to accept this theory, because endoderm from the primitive stomodeum should be unable to differentiate into epidermis-like epithelium. Instead, we agree with the suggestion of Woolgar and Smith14 that entrapped undifferentiated endodermal cells give rise to these cysts. The aberrant differentiation would also occur under the action of environmental and inductive influences. Khunamornpong et al8 reported a case with associated pancreatic tissue and supported this concept. Several other hypothetical pathogenetic mechanisms were proposed, including origin in the thyroglossal duct and salivary retention cysts.15,16 Conservative surgical excision is the treatment of choice, with long-term follow-up recommended.1-3,5,7,8 Recurrence is uncommon17 but can occur many years later, probably because of incomplete excision.13
688 Crivelini et al
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY June 2001
REFERENCES
10. Eppley BL, Bell MJ, Sclaroff A. Simultaneous occurrence of dermoid and heterotopic intestinal cysts in the floor of the mouth of a newborn. J Oral Maxillofac Surg 1985;43:880-3. 11. Arcand P, Granger J, Brochu P. Congenital dermoid cyst of the oral cavity with gastric choristoma. J Otolaryngol 1988;17:21922. 12. Gorlin RJ, Jirasek JE. Oral cysts containing gastric or intestinal mucosa. An unusual embryological accident or heterotopia. Arch Otolaryngol 1970;91:594-7. 13. Daley TD, Wysocki GP, Lovas GL, Smout MS. Heterotopic gastric cyst of the oral cavity. Head Neck Surg 1984;7:168-71. 14. Woolgar JA, Smith AJ. Heterotopic gastrointestinal cyst of oral cavity: a developmental lesion? Oral Surg Oral Med Oral Pathol 1988;66:223-5. 15. Willis RA. Some unusual heterotopies. Br Med J 1968;3:267-72. 16. Gruskin P, Landolfe FR. Heterotopic gastric mucosa of the tongue. Arch Pathol 1972;94:1846. 17. Lalwani AK, Lalwani RB, Bartlett PC. Heterotopic gastric mucosal cyst of the tongue. Otolaryngol Head Neck Surg 1993; 108:204-5.
1. Chou L, Hansen LS, Daniels TE. Choristomas of the oral cavity: a review. Oral Surg Oral Med Oral Pathol 1991;72:584-93. 2. Martone CH, Wolf SM, Wesley RK. Heterotopic gastrointestinal cyst of the oral cavity. J Oral Maxillofac Surg 1992;50:1340-2. 3. Bite U, Cramer HM. Mixed heterotopic gastrointestinal and respiratory cyst of the lip: case report and review of the literature. Plast Reconstr Surg 1992;90:1068-72. 4. Lipsett J, Sparnon AL, Byard RW. Embryogenesis of enterocystomas-enteric duplication cysts of the tongue. Oral Surg Oral Med Oral Pathol 1993;75:626-30. 5. Surana R, Losty P, Fitzgerald RJ. Heterotopic gastric cyst of the tongue in a newborn. Eur J Pediatr Surg 1993;3:110-1. 6. Kinoshita Y, Honma Y, Otuka T, Shimura K. Gastrointestinal mucosal cyst of the oral cavity: report of case and review of literature. J Oral Maxillofac Surg 1994;52:1203-5. 7. Fernandes M, Miziara ID, Chung S, Mniti A, Iriya K. Choristoma de la muqueuse gastrique: description d’un cas. Rev Laryngol Otol Rhinol 1995;116:347-9. 8. Khunamornpong S, Yousukh A, Tananuvat R. Heterotopic gastrointestinal and pancreatic tissue of the tongue. A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;81:576-9. 9. Morgan WE, Jones JK, Flaitz CM, Hicks MJ. Congenital heterotopic gastrointestinal cyst of the oral cavity in a neonate: case report and review of literature. Int J Pediatr Otorhinolaryngol 1996;36:69-77.
Reprint requests: M. M. Crivelini Disciplina de Patologia Bucal Faculdade de Odontologia de Araçatuba–UNESP Caixa Postal 341–Araçatuba-SP–Brazil CEP 16015-050
Access to Journal of Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics (OOOOE) Online is now reserved for print subscribers! Full-text access to OOOOE Online is now available for all print subscribers. To activate your individual online subscription, please visit OOOOE Online, point your browser to http://www.mosby.com/tripleo, follow the prompts to activate your online access, and follow the instructions. To activate your account, you will need your subscriber account number, which you can find on your mailing label (note: the number of digits in your subscriber account number varies from 6 to 10). See the example below in which the subscriber account number has been circled: Sample mailing label
This is your subscription account number
**************************3-DIGIT 001 SJ P1 FEB00 J075 C: 1 1234567-89 U 05/00 Q: 1 J. H. DOE, MD 531 MAIN ST CENTER CITY, NY 10001-001
Personal subscriptions to OOOOE Online are for individual use only and may not be transferred. Use of OOOOE Online is subject to agreement to the terms and conditions as indicated online.