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Carcinomatous transformation of a sublingual dermoid cyst
Int. J. Oral Maxillofac. Surg. 2000; 29:126-127 Printed in Denmark. All rights reserved
Copyright©Munksgaard2000 lntemationa]]oumalof
• Ord Maxillofacial Surgery ISSN 0901-5027
Carcinomatous transformation of a sublingual dermoid cyst
J. C. Devine, D. C. Jones Regional Centre for Maxillofacial Surgery, University Hospital Aintree, Liverpool, UK
A case report J. C. Devine, D. C. Jones: Carcinomatous transformation o f a sublingual dermoid cyst. A case report. Int. J. Oral Maxillofac. Surg. 2000; 29: 126-127. © Munksgaard, 2000 Abstract. Sublingual d e r m o i d cysts are rare lesions. Typically they present as slow-growing masses that may cause elevation of the tongue, interference with speech or the appearance of a double chin. We report the first case of malignant transformation to squamous cell carcinoma of a long-standing sublingual dermoid cyst.
Case report A 56-year-old man was referred by his general surgeon to the Regional Maxillofacial Unit, complaining of rapid, recent enlargement of a midline upper cervical swelling that had previously been asymptomatic for over 20 years. The patient reported that he had experienced progressive problems with his speech due to the enlarging sublingual mass. Examination revealed a large, non-fluctuant, anterior, cervical swelling that extended to the floor of the patient's mouth causing gross protrusion of the tongue. MRI scan confirmed the full extent of the mass and a provisional diagnosis of a dermoid cyst was made (Fig. 1). Histological examination showed that the lesion was a dermoid cyst that had undergone malignant transformation to squamous cell carcinoma (Figs. 2, 3). The presence of folliculo-sebaceous and sudoriferous elements confirmed the lesion as a dermoid rather than an epidermoid cyst. Following complete excision of the tumour, the patient received radiotherapy to the neck with a total of 5000 cGy over 25 treatments and showed no sign of recurrence two years later.
which enlarge by the desquamation of lining cells into a central cavity. The classically described ovarian dermoid is actually a teratoma derived from germ cells. This differs from the peripheral d e r m o i d cyst, as seen in the head and neck, which is derived from enclosed n o n - g e r m cells situated along lines of closure of junctions of bone, soft tissue
Key words: dermoid cyst; malignant transformation. Accepted for publication 29 September 1999
and embryonic membranes. Such derm o i d cysts contain ectodermal derivatives only 2. Therefore, they are clearly different from craniofacial teratomas which contain a mixture of heterogeneous tissues reflecting more than one of the three embryonic layers. D e r m o i d cysts in the floor of the m o u t h are relatively rare, accounting for
Discussion There is m u c h confusion over the use of the term " d e r m o i d " . D e r m o i d cysts are generally regarded as benign entities
Fig. 1. Magnetic resonance scan showing the dermoid cyst extending from the neck and displacing the tongue cephalad.
Carcinomatous transformation o f sublingual dermoid cyst
127
Fig. 2. The developing carcinoma at scanning magnification. Islands
Fig. 3. Higher magnification of the carcinoma. The lower border of
of malignant keratocytes with variable keratinization and necrosis can be seen infiltrating the cyst wall. The origin of the neoplasm from the cyst epithelial lining can be clearly seen (arrowhead). Haematoxylin and Eosin stain, ×25 magnification.
the epithelial lining is irregular, with islands of malignant keratinocytes protruding into the underlying cyst wall. Haematoxylin and Eosin stain, x 100 magnification.
1.8% of all dermoids 6. Typically they are slow-growing, presenting in early adult life as an asymptomatic swelling that may occasionally cause protrusion of the tongue and give the appearance of a double chin. Attempts have been m a d e to classify them according to site or histological characteristics 1'9. Sub-lingual d e r m o i d cysts may occur superficial or deep to the geniohyoid muscle and they may penetrate through the mylohyoid muscle. Surgically they can be approached either extra-orally or intraorally 4. A midline lip-splitting incision followed by symphyseal osteotomy has been advocated as providing superior access to the sub-lingual d e r m o i d in difficult cases 8. Malignant transformation o f a derm o i d cyst to squamous cell carcinoma is exceptionally rare and has previously been described in ovarian 3, intracranial 7,1° and lumbar 5 d e r m o i d cysts, but until now there have been no reported cases of such change in the head and neck. It is unlikely that the lesion was metastatic in origin, since the neoplastic change was only seen in squamous epithelial elements o f the cyst lining and there was no evidence of dysplasia of the other cellular elements. The prognosis following complete excision of this lesion is uncertain. However, the prognosis of malignant
change in dermoid cysts at other sites is reported as being very p o o r s,7,11, resulting in patient death in several cases. In view of this, prophylactic external b e a m radiotherapy to the wound bed was thought to be justified. This case also highlights the need for surgical intervention in cases of unexpected change in size or new symptoms attributable to a long-standing lesion.
Acknowledgments. We would like to thank Mr O. A. Pospisil, Consultant Maxillofacial Surgeon, for allowing us to present this case history and Dr A. Triantafyllou, Oral Pathologist, for reporting the histopathology.
References 1. BARKERAE. Sebaceous or dermoid cysts of the tongue, removal by submental incision; cure. Trans Clin Soc Lond 1883: 16: 225. 2. BATSAKISJG. Nomenclature of developmental tumors. Acta Otol Rhinol Laryngol 1984: 93:98 9. 3. CHANGCmEN C-C, CrmN L, ENG H-L. Sebaceous carcinoma arising in a benign dermoid cyst of the ovary. Acta Obstet Gynecol Scand 1994: 73:355 8. 4. DI FRANCESCOA, CHIAPASCOM, BIGLIOLI F, ANCONAD. Intraoral approach to large dermoid cysts of the floor of the mouth: a technical note. Int J Oral Maxillofac Surg 1995: 24: 233-5.
5. INO M, NAGASEM, TSUGE K, KAMATA M, UDAGAWAE. Malignant squamous cell carcinoma arising in a dermoid cyst. Int Orthop 1995: 19:185 6. 6. KATZ AD. Midline dermoid tumors of the neck. Arch Surg 1974: 109: 822-3. 7. KOBAYASm T, KucrnwAKI H, INAO S, NAKASmMA N. A squamons cell carcinoma originated from intracranial dermoid cyst. Neurochirurgia 1993: 36: 269. 8. McGREGOR IA. Symphyseal mandibular osteotomy in the approach to sublingual dermoid cysts. Br J Plast Surg 1991: 44: 544-5. 9. M~mR I. Dermoid cysts (dermoids) of the floor of the mouth. Oral Surg Oral Med Oral Pathol 1955: 8: 1149. 10. NrSHIO S, TAKESHITAI, MORIOKAT, FvKtn M. Primary intracranial squamous cell carcinomas: report of two cases. Neurosurgery 1995: 37: 329-32. 11. STEPHENSONGC, IRONSIDEJW. Squamous cell carcinoma arising in a subcutaneous dermoid cyst. Postgrad Med J 1991: 67: 84-6.
Address:
J. C. Devine Regional Centre for Maxillofacial Surgery University Hospital Aintree Longmoor Lane Liverpool L9 7AL UK
Copyright©Munksgaard2000 lntemationa]]oumalof
• Ord Maxillofacial Surgery ISSN 0901-5027
Carcinomatous transformation of a sublingual dermoid cyst
J. C. Devine, D. C. Jones Regional Centre for Maxillofacial Surgery, University Hospital Aintree, Liverpool, UK
A case report J. C. Devine, D. C. Jones: Carcinomatous transformation o f a sublingual dermoid cyst. A case report. Int. J. Oral Maxillofac. Surg. 2000; 29: 126-127. © Munksgaard, 2000 Abstract. Sublingual d e r m o i d cysts are rare lesions. Typically they present as slow-growing masses that may cause elevation of the tongue, interference with speech or the appearance of a double chin. We report the first case of malignant transformation to squamous cell carcinoma of a long-standing sublingual dermoid cyst.
Case report A 56-year-old man was referred by his general surgeon to the Regional Maxillofacial Unit, complaining of rapid, recent enlargement of a midline upper cervical swelling that had previously been asymptomatic for over 20 years. The patient reported that he had experienced progressive problems with his speech due to the enlarging sublingual mass. Examination revealed a large, non-fluctuant, anterior, cervical swelling that extended to the floor of the patient's mouth causing gross protrusion of the tongue. MRI scan confirmed the full extent of the mass and a provisional diagnosis of a dermoid cyst was made (Fig. 1). Histological examination showed that the lesion was a dermoid cyst that had undergone malignant transformation to squamous cell carcinoma (Figs. 2, 3). The presence of folliculo-sebaceous and sudoriferous elements confirmed the lesion as a dermoid rather than an epidermoid cyst. Following complete excision of the tumour, the patient received radiotherapy to the neck with a total of 5000 cGy over 25 treatments and showed no sign of recurrence two years later.
which enlarge by the desquamation of lining cells into a central cavity. The classically described ovarian dermoid is actually a teratoma derived from germ cells. This differs from the peripheral d e r m o i d cyst, as seen in the head and neck, which is derived from enclosed n o n - g e r m cells situated along lines of closure of junctions of bone, soft tissue
Key words: dermoid cyst; malignant transformation. Accepted for publication 29 September 1999
and embryonic membranes. Such derm o i d cysts contain ectodermal derivatives only 2. Therefore, they are clearly different from craniofacial teratomas which contain a mixture of heterogeneous tissues reflecting more than one of the three embryonic layers. D e r m o i d cysts in the floor of the m o u t h are relatively rare, accounting for
Discussion There is m u c h confusion over the use of the term " d e r m o i d " . D e r m o i d cysts are generally regarded as benign entities
Fig. 1. Magnetic resonance scan showing the dermoid cyst extending from the neck and displacing the tongue cephalad.
Carcinomatous transformation o f sublingual dermoid cyst
127
Fig. 2. The developing carcinoma at scanning magnification. Islands
Fig. 3. Higher magnification of the carcinoma. The lower border of
of malignant keratocytes with variable keratinization and necrosis can be seen infiltrating the cyst wall. The origin of the neoplasm from the cyst epithelial lining can be clearly seen (arrowhead). Haematoxylin and Eosin stain, ×25 magnification.
the epithelial lining is irregular, with islands of malignant keratinocytes protruding into the underlying cyst wall. Haematoxylin and Eosin stain, x 100 magnification.
1.8% of all dermoids 6. Typically they are slow-growing, presenting in early adult life as an asymptomatic swelling that may occasionally cause protrusion of the tongue and give the appearance of a double chin. Attempts have been m a d e to classify them according to site or histological characteristics 1'9. Sub-lingual d e r m o i d cysts may occur superficial or deep to the geniohyoid muscle and they may penetrate through the mylohyoid muscle. Surgically they can be approached either extra-orally or intraorally 4. A midline lip-splitting incision followed by symphyseal osteotomy has been advocated as providing superior access to the sub-lingual d e r m o i d in difficult cases 8. Malignant transformation o f a derm o i d cyst to squamous cell carcinoma is exceptionally rare and has previously been described in ovarian 3, intracranial 7,1° and lumbar 5 d e r m o i d cysts, but until now there have been no reported cases of such change in the head and neck. It is unlikely that the lesion was metastatic in origin, since the neoplastic change was only seen in squamous epithelial elements o f the cyst lining and there was no evidence of dysplasia of the other cellular elements. The prognosis following complete excision of this lesion is uncertain. However, the prognosis of malignant
change in dermoid cysts at other sites is reported as being very p o o r s,7,11, resulting in patient death in several cases. In view of this, prophylactic external b e a m radiotherapy to the wound bed was thought to be justified. This case also highlights the need for surgical intervention in cases of unexpected change in size or new symptoms attributable to a long-standing lesion.
Acknowledgments. We would like to thank Mr O. A. Pospisil, Consultant Maxillofacial Surgeon, for allowing us to present this case history and Dr A. Triantafyllou, Oral Pathologist, for reporting the histopathology.
References 1. BARKERAE. Sebaceous or dermoid cysts of the tongue, removal by submental incision; cure. Trans Clin Soc Lond 1883: 16: 225. 2. BATSAKISJG. Nomenclature of developmental tumors. Acta Otol Rhinol Laryngol 1984: 93:98 9. 3. CHANGCmEN C-C, CrmN L, ENG H-L. Sebaceous carcinoma arising in a benign dermoid cyst of the ovary. Acta Obstet Gynecol Scand 1994: 73:355 8. 4. DI FRANCESCOA, CHIAPASCOM, BIGLIOLI F, ANCONAD. Intraoral approach to large dermoid cysts of the floor of the mouth: a technical note. Int J Oral Maxillofac Surg 1995: 24: 233-5.
5. INO M, NAGASEM, TSUGE K, KAMATA M, UDAGAWAE. Malignant squamous cell carcinoma arising in a dermoid cyst. Int Orthop 1995: 19:185 6. 6. KATZ AD. Midline dermoid tumors of the neck. Arch Surg 1974: 109: 822-3. 7. KOBAYASm T, KucrnwAKI H, INAO S, NAKASmMA N. A squamons cell carcinoma originated from intracranial dermoid cyst. Neurochirurgia 1993: 36: 269. 8. McGREGOR IA. Symphyseal mandibular osteotomy in the approach to sublingual dermoid cysts. Br J Plast Surg 1991: 44: 544-5. 9. M~mR I. Dermoid cysts (dermoids) of the floor of the mouth. Oral Surg Oral Med Oral Pathol 1955: 8: 1149. 10. NrSHIO S, TAKESHITAI, MORIOKAT, FvKtn M. Primary intracranial squamous cell carcinomas: report of two cases. Neurosurgery 1995: 37: 329-32. 11. STEPHENSONGC, IRONSIDEJW. Squamous cell carcinoma arising in a subcutaneous dermoid cyst. Postgrad Med J 1991: 67: 84-6.
Address:
J. C. Devine Regional Centre for Maxillofacial Surgery University Hospital Aintree Longmoor Lane Liverpool L9 7AL UK