DERMOID CYST (MEDSCAPE) Background The term dermoid cyst does not appear to be restricted to a single kind of lesion

nor is it used in only a single medical discipline. The term dermoid cyst can be found in the vocabulary of dermatologists, dermatopathologists, general pathologists, gynecologists, neurosurgeons, or pediatricians. If asked, all of these clinicians would most probably define and describe dermoid cysts differently. For example, gynecologists and general pathologists might say that a dermoid cyst is a cystic tumor of the ovary. In contrast, neurosurgeons tend to view a dermoid cyst is associated with a congenital cyst of the spine or an intracranial congenital cyst. For pediatricians and dermatologists, dermoid cyst means subcutaneous cysts, which are usually congenital. In all disciplines, however, the common factor is the presence of a solitary, or occasionally multiple, hamartomatous tumor. The tumor is covered by a thick dermislike wall that contains multiple sebaceous glands and almost all skin adnexa. Hairs and large amounts of fatty masses cover poorly to fully differentiated structures derived from the ectoderm. Depending on the location of the lesion, dermoid cysts may contain substances such as nails and dental, cartilagelike, and bonelike structures. If limited to the skin or subcutaneous tissue, dermoid cysts are thin-walled tumors that contain different amounts of fatty masses; occasionally, they contain horny masses and hairs. Pathophysiology Dermoid cysts in the skin and subcutis occur mostly on the face, neck, or scalp. In addition to the skin, dermoid cysts can be intracranial, intraspinal, or perispinal. Intra-abdominal cysts, such as cystic tumors of the ovary or omentum, occur as well. Epidemiology Frequency United States No information is available about the prevalence of dermoid cysts. In gynecology, the literature describes dermoid cysts as relatively rare tumors, a cystic teratoma that most often occurs in individuals aged 15-40 years. In neurosurgery, dermoid cysts are rare. In dermatology and pediatrics, dermoid cysts are relatively uncommon. International The international prevalence is the same as the prevalence in the United States. Of the 2639 eyelid tumors from a Chinese study, the 5 most common eyelid benign ones were inflammatory lesions, melanocytic nevi, papillomas, dermoid cysts and epidermoid cysts, and epithelial cysts. [1] Mortality/Morbidity Although dermoid cysts are located in connection with the spinal channel (as described in neurosurgery literature), no deaths are directly linked to ruptures of the cyst or to the spreading of fatty and occasionally, infected masses in subarachnoid, ventricular, or subdural compartments. However, rupture or spread can lead to severe neurologic complications such as secondary spinal subdural abscesses. Race No racial predilection is apparent; however, most cases of dermoid cysts in the literature are described in white persons. Sex

Dermoid cysts of the ovary are sex restricted, that is, they occur only in the female population. In other dermoid cysts, no sex predilection has been found. Age Dermoid cysts have been described in persons of all ages. Dermoid cysts on the face, neck, or scalp are subcutaneous cysts that are usually present at birth. Intracranial or perispinal dermoid cysts are most often found in infants, children, or young adolescents. Intra-abdominal dermoid cysts are described in females aged 15-40 years. For example, cystic teratoma is a relatively rare tumor that most often occurs in females aged 15-40 years. Most dermoid cysts on the floor of the mouth occur in individuals aged 10-30 years. There are few descriptions of oral dermoid cysts in newborns or children. History Dermoid cysts that are congenital and localized on the neck, head, or trunk are usually visible at birth. In some instances, careful medical examination may help to find most dermoid cysts. Intracranial, intraspinal, or intra-abdominal dermoid cysts may be suspected after specific or nonspecific neurologic or gynecologic symptoms occur. In these instances, imaging studies may help in distinguishing dermoid cysts from other tumors or organ malformations (see Imaging Studies). A congenital intracranial frontotemporal dermoid cyst may be first evident as a cutaneous fistula, although intracranial extension and cutaneous sinus tract formation are rarely seen with these dermoid cysts.[2] Unilateral upper eyelid swelling may be the first sign, with imaging studies demonstrating a soft tissue orbital dermoid cyst arising from the lacrimal gland.[3] An eyelid dermoid cyst attached to a tarsus may be evident as a firmly adherent nontender upper-eyelid nodule.[4] Dermoid tumors in the medial canthal area may present as masses adherent to the lacrimal canaliculi.[5] Rarely, a dermoid cyst evident during the newborn period clinically appears, only to become evident years later with fullthickness bone erosion and transcranial extension.[6] Physical Dermoid cysts can appear as cutaneous cysts on the head, as cysts on the floor of the mouth or elsewhere in the head, within the parotid gland,[7] or as cysts in the testes or penis. They can be quite large.[8] Specialists in various disciplines may consider dermoid cysts to be different entities.

Cutaneous cysts most commonly occur on the head (forehead), mainly around the eyes. Occasionally, they occur on the neck or in a midline region. When on the head, dermoid cysts are often adherent to the periosteum. The usual diameter of the lesions is 1-4 cm. o In one study, 25 benign tumors on the forehead and brow of children and adolescents were successfully removed by means of endoscopic excision. Of these, 6 were classified as dermoid cysts. Dermoid cysts on the forehead and brow are known to cause pressure-related erosion of the underlying bony tissue, and surgical intervention may be helpful. o In 191 children treated for congenital cysts and fistulas of the neck in 1984-1999, 21 dermoid cysts were found. Periauricular fistulas and cystic hygromas were not included in this study. [9] o Occasionally, skin-related dermoid cysts are multiple and develop over periods as long as 20 years. In one unusual case, multiple subcutaneous dermoid cysts were present in the frontal region of a 41-year-old man, none with evident intracranial extension. o In many patients, dermoid cysts occur on the floor of the mouth or elsewhere in the mouth. [10, 11, 12, 13] o Because the term dermoid is frequently used in the literature, some authors believe that this term should be used for all congenital cysts on the floor of the mouth. Three subclasses of congenital mouth cysts are described in the literature: epidermoid (simple) cysts, dermoid (complex) cysts, and teratoid (complex) cysts.

Most of these lesions occur in individuals aged 10-30 years. Only a few cases describe dermoid cysts of the mouth in newborns or children. o An unusual case of a carcinomatous transformation of a long-standing sublingual dermoid cyst has been described.[14] o Other rare dermoid cysts in the oral cavity are those on the tongue.[15, 16] As of early 2000, 17 patients with intralingual dermoid cysts are described in the English-language literature. All cases occurred in young patients. Magnetic resonance imaging (MRI) was helpful in establishing the differential diagnosis. Surgical excision corrected deglutition and speech problems in all of these patients. Dermoid cysts can occur elsewhere in the head. o Dermoid cysts in the eustachian tube are rare.[17] Only 12 patients have been described. In most cases reviewed, cysts affected female patients on the left side. MRI was useful in establishing the correct diagnosis and in selecting the surgical approach. o Reports of nasal dermoid cysts were recently published. Of 36 children with nasal dermoid sinus cysts that were treated from 1974-1994, 10 had only a midline cyst, 8 had only nasal pits, and 18 had combined cysts. Meningeal adherences have been found in only 2 patients.[18] Dermoid cysts can occur in the testes or penis. o In a review of cystic testicular lesions in the pediatric population, dermoid cysts were noted.[19] Other diagnoses for these cysts include epidermoid cyst, prepubertal teratoma, juvenile granulosa cell tumor, cystic dysplasia of the rete testis, testicular cystic lymphangioma, simple cyst, and cystic degeneration after torsion. An understanding of potentially cystic testicular lesions in children leads to the best treatment choices and often to the preservation of a substantial portion of the affected testis. o Dermoid cysts in the penis are extremely rare. Tomasini et al[3] described the first in 1997. The patient was a young white male who had significant penile swelling for several months. o For neurosurgeons, dermoid cysts are associated with congenital cysts of the spine or intracranial cysts. o Several cases involve ruptured cysts and generalized subarachnoid and ventricular spread of the contents (mostly fatty masses).[20, 21, 22] o In some patients, spinal dermoid cysts, especially those connected to dermal sinus tract, lead to severe neurologic complications such as secondary spinal subdural abscesses caused by the spread of the infection in the dermoid cyst. For gynecologists and general pathologists a dermoid cyst is primarily associated with a cystic tumor of the female ovary. o Cystic teratoma is a relatively rare tumor that most often occurs in females aged 15-40 years. A cystic teratoma consists of a thick leatherlike capsule that covers amorphous fatty masses and poorly to fully differentiated structures derived from the ectoderm. Most ovarian dermoid cysts contain skin and skin adnexa, including prominent sebaceous glands, hairs, and nails, but also teeth or eyes. Melanotic changes may also occur. Rare cases of multiple dermoid cysts of the omentum have been reported. [23] Dermoid cysts of the ovary are usually benign and easy to remove. o Malignant melanomas may originate from melanocytes in ovarian cystic teratomas. Two new cases and 17 older cases in the literature (reported from 1903-1995) are described and were critically reviewed. The present authors found 17 additional cases of benign and malignant melanotic ovarian lesions that were not associated with a dermoid cyst, including 4 melanomas, 3 benign nevi, 5 benign melanosis, and 4 benign and malignant retinal anlage tumors. The extremely rare primary ovarian melanoma was differentiated from the more common melanoma metastatic cyst of the ovary by its unilaterality, the presence of junctional change, and detailed history taking and physical examination, the findings of which excluded other primary sites. [24] o Three patients with metastasizing squamous cell carcinoma from a dermoid cyst of the ovary are described.[25] Malignant transformation in a dermoid cyst is a rare complication and mainly occurs in older individuals. Although the prognosis is poor, aggressive therapy may result in long-term remission.

Causes

Dermoid cysts are true hamartomas. Dermoid cysts occur when skin and skin structures become trapped during fetal development. Histogenetically, dermoid cysts are a result of the sequestration of skin along the lines of embryonic closure.

Imaging Studies

Radiography, CT scanning, and MRI are helpful in making the correct differential diagnosis of dermoid cysts. MRI is particularly helpful in diagnosing intracranial or intramedullary dermoid cysts and in assessing the dissemination of fatty masses or droplets.

MRI is helpful in planning surgical procedures and in assessing therapeutic success. Also see the ACR Appropriateness Criteria suspected adnexal masses.[29]

Histologic Findings Dermoid cysts are a result of the sequestration of the skin along the lines of embryonic closure. If connected with the ovary, dermoid cysts are true teratomas. In contrast to epidermal inclusion cysts, dermoid cysts in the skin are lined by an epidermis that possesses various epidermal appendages. As a rule, these appendages are fully mature. Hair follicles containing hairs that project into the lumen of the cyst are often present. The dermis of dermoid cysts usually contains sebaceous glands, eccrine glands, and, in many patients, apocrine glands. Occasionally, the lining epithelium may proliferate as papillary boundaries extend externally or inward toward the lumen of the cyst. This proliferation may have some superficial resemblance to epidermal carcinomatous proliferation, and the growth may be misclassified as a cancer. Dermoid cysts in the ovary (cystic teratomas) or those disseminated intra-abdominally may contain other structures such as nails, hairs, or cartilage and bone fragments. These cysts have cell walls that are almost identical to those of the skin, and they may contain multiple adnexal skin structures such as hair follicles, sweat glands, and occasionally, hair, teeth, or nerves. A congenital dermoid together with a bronchogenic cyst of the tongue is extremely rare but has been described in a few patients.[30] Surgical Care Surgical excision is the treatment of choice in any localization. Surgically remove dermoid cysts. In some patients, surgery should be performed even more carefully than usual because the fatty content of the cyst may spread to the surrounding tissues or anatomic structures, especially if the cyst is infected with bacteria. The spread of these contents can cause foreign body reactions and severe complications. Minimally invasive surgical techniques have been successful in removing dermoid cysts from difficult locations, such as those on the tongue or the floor of the mouth. Intralingual dermoid cysts lead to lingual motility defects and speech problems. These cysts should be surgically removed to restore normal lingual function and to correct speech problems. Intracranial, intramedullary, and ovarian dermoid cysts are difficult to treat. Sophisticated neurosurgical or gynecologic surgical techniques are often needed to remove the dermoid cyst and prevent possible complications. High-definition fiber tracking guidance may be beneficial in the resection of an intraparenchymal dermoid cyst by means of a minimally invasive endoscopic port.[31] In some patients with dermoid cysts on the forehead and brow, successful excision with endoscopy-assisted surgery have been described.[32] In the reported cases, no complications (eg, paresthesia or numbness on the scalp) occurred. The absence of visible scarring is an additional advantage of endoscopy-assisted surgery. Angular dermoid cyst excision using an eyelid crease approach may yield excellent cosmesis.[33] Frontozygomatic removal through a blepharoplasty incision has been advocated for frontozygomatic dermoid cysts.[34] Complications

Several possible complications of spontaneous or posttraumatic rupture and surgical procedures have been described. o In patients with a ruptured spinal dermoid cyst, fatty droplets can disseminate in the cerebrospinal fluid or in a dilated central canal of the spinal cord. o In other patients, subarachnoid and ventricular fat dissemination can occur after the cerebellopontine angle dermoid cyst is resected. o Spinal subdural abscesses are a possible complication because of the bacterial infection of spinal dermoid cysts in a dermal sinus tract. o A ruptured intracranial dermoid cyst be an incidental finding on an MRI performed for other purposes or because of a persistent headache.[35] Pay special attention to intralingual dermoid cysts because deglutition and speech problems may occur. Malignant transformation is an unusual complication that may occur in patients with long-standing dermoid cysts. o Carcinomatous transformation to a squamous cell carcinoma is described in sublingual and intra-abdominal dermoid cysts, most often dermoid cysts of the ovary

o
Prognosis

Metastatic malignant melanomas arising from dermoid cysts have been reported

If dermoid cysts are diagnosed early and treated with complete surgical excision, the prognosis is good, and no further complications are expected. In patients with carcinomatous transformation, the prognosis depends on the stage of the malignant disease (locally limited or spreading) and the success of therapy.

Patient Education For patient education resources, see the Procedures Center and Women's Health Center, as well as Dermoid Cyst Removal and Ovarian Cysts.

References 1. Xu XL, Li B, Sun XL, Li LQ, Ren RJ, Gao F. [Clinical and pathological analysis of 2639 cases of eyelid tumors]. Zhonghua Yan Ke Za Zhi. Jan 2008;44(1):38-41. [Medline]. 2. Scolozzi P, Lombardi T, Jaques B. Congenital intracranial frontotemporal dermoid cyst presenting as a cutaneous fistula. Head Neck. May 2005;27(5):429-32. [Medline]. 3. Kiratli H, Bilgic S, Sahin A, Tezel GG. Dermoid cyst of the lacrimal gland. Orbit. Jun 2005;24(2):145-8. [Medline]. 4. Koreen IV, Kahana A, Gausas RE, Potter HD, Lemke BN, Elner VM. Tarsal dermoid cyst: clinical presentation and treatment. Ophthal Plast Reconstr Surg. Mar-Apr 2009;25(2):146-7. [Medline]. 5. Kim NJ, Choung HK, Khwarg SI. Management of dermoid tumor in the medial canthal area. Korean J Ophthalmol. Sep 2009;23(3):204-6. [Medline]. 6. Maurice SM, Burstein FD. Disappearing dermoid: fact or fiction?. J Craniofac Surg. Jan 2012;23(1):e31-3. [Medline]. 7. Tas A, Yagiz R, Altaner S, Karasalihoglu AR. Dermoid cyst of the parotid gland: first pediatric case. Int J Pediatr Otorhinolaryngol. Feb 2010;74(2):216-7. [Medline]. 8. Kahraman A, Kahveci R. A giant dermoid cyst. Dermatol Surg. Sep 2008;34(9):1273-5. [Medline]. 9. Nicollas R, Guelfucci B, Roman S, Triglia JM. Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. Sep 29 2000;55(2):117-24. [Medline]. 10. Acree T, Abreo F, Smith BR, Bagby J, Heard JS. Diagnosis of dermoid cyst of the floor of the mouth by fine-needle aspiration cytology: a case report. Diagn Cytopathol. Feb 1999;20(2):78-81. [Medline]. 11. Bonilla JA, Szeremeta W, Yellon RF, Nazif MM. Teratoid cyst of the floor of the mouth. Int J Pediatr Otorhinolaryngol. Dec 5 1996;38(1):71-5. [Medline]. 12. Vargas Fernandez JL, Lorenzo Rojas J, Aneiros Fernandez J, Sainz Quevedo M. [Dermoid cyst of the floor of the mouth]. Acta Otorrinolaringol Esp. Jan 2007;58(1):31-3. [Medline]. 13. Zeltser R, Milhem I, Azaz B, Hasson O. Dermoid cysts of floor of the mouth: report of four cases. Am J Otolaryngol. Jan-Feb 2000;21(1):55-60. [Medline]. 14. Devine JC, Jones DC. Carcinomatous transformation of a sublingual dermoid cyst. A case report. Int J Oral Maxillofac Surg. Apr 2000;29(2):126-7. [Medline]. 15. Miles LP, Naidoo LC, Reddy J. Congenital dermoid cyst of the tongue. J Laryngol Otol. Dec 1997;111(12):1179-82. [Medline]. 16. Myssiorek D, Lee J, Wasserman P, Lustrin E. Intralingual dermoid cysts: a report of two new cases. Ear Nose Throat J. May 2000;79(5):380-3. [Medline]. 17. Sichel JY, Dano I, Halperin D, Chisin R. Dermoid cyst of the eustachian tube. Int J Pediatr Otorhinolaryngol. Apr 25 1999;48(1):77-81. [Medline]. 18. Carroll CM, Gaffney R, McShane D. Congenital nasal dermoids in children. Ir J Med Sci. Jul-Sep 1997;166(3):149-51. [Medline]. 19. Garrett JE, Cartwright PC, Snow BW, Coffin CM. Cystic testicular lesions in the pediatric population. J Urol. Mar 2000;163(3):928-36. [Medline]. 20. Calabr F, Capellini C, Jinkins JR. Rupture of spinal dermoid tumors with spread of fatty droplets in the cerebrospinal fluid pathways. Neuroradiology. Aug 2000;42(8):572-9. [Medline]. 21. Carvalho GA, Cervio A, Matthies C, Samii M. Subarachnoid fat dissemination after resection of a cerebellopontine angle dysontogenic cyst: case report and review of the literature. Neurosurgery. Sep 2000;47(3):760-3; discussion 7634. [Medline]. 22. Karabulut N, Oguzkurt L. Tetraventricular hydrocephalus due to ruptured intracranial dermoid cyst. Eur Radiol. 2000;10(11):1810-1. [Medline].

23. Mazumdar A, Vaiphei K, Verma GR. Multiple dermoid cysts of omentum. J Postgrad Med. Apr-Jun 1997;43(2):41-2. [Medline]. 24. Davis GL. Malignant melanoma arising in mature ovarian cystic teratoma (dermoid cyst). Report of two cases and literature analysis. Int J Gynecol Pathol. Oct 1996;15(4):356-62. [Medline]. 25. Ayhan A, Tuncer ZS, Bilgin F, Kucukali T. Squamous cell carcinoma arising in dermoid cyst. Eur J Gynaecol Oncol. 1996;17(2):144-7. [Medline]. 26. Ro EY, Thomas RM, Isaacson GC. Giant dermoid cyst of the neck can mimic a cystic hygroma: using MRI to differentiate cystic neck lesions. Int J Pediatr Otorhinolaryngol. Apr 2007;71(4):653-8. [Medline]. 27. Thway K, Polson A, Pope R, Thomas JM, Fisher C. Extramammary Paget disease in a retrorectal dermoid cyst: report of a unique case. Am J Surg Pathol. Apr 2008;32(4):635-9. [Medline]. 28. Arishima H, Takeuchi H, Kitai R, Yamauchi T, Kikuta KI. Vascular Leiomyoma of the Scalp with a Small Deformity on the Skull Mimicking a Dermoid Cyst. Pediatr Dermatol. Feb 22 2012;[Medline]. 29. [Guideline] Bohm-Velez M, Fleischer AC, Andreotti RF, et al. Expert Panel on Women's Imaging. Suspected adnexal masses. National Guideline Clearinghouse. 2005;[Full Text]. 30. Gleizal A, Abouchebel N, Lebreton F, Beziat JL. Dermoid cyst of the tongue: an association of dermoid cyst with bronchogenic epithelium. J Craniomaxillofac Surg. Mar 2006;34(2):113-6. [Medline]. 31. Fernandez-Miranda JC, Engh JA, Pathak SK, et al. High-definition fiber tracking guidance for intraparenchymal endoscopic port surgery. J Neurosurg. Nov 27 2009;[Medline]. 32. Lin SD, Lee SS, Chang KP, Lin TM, Lu DK, Tsai CC. Endoscopic excision of benign tumors in the forehead and brow. Ann Plast Surg. Jan 2001;46(1):1-4. [Medline]. 33. Cozzi DA, Mele E, d'Ambrosio G, Totonelli G, Frediani S, Spagnol L. The eyelid crease approach to angular dermoid cysts in pediatric general surgery. J Pediatr Surg. Aug 2008;43(8):1502-6. [Medline]. 34. Nelson KE, Mishra A, Duncan C. Upper blepharoplasty approach to frontozygomatic dermoid cysts. J Craniofac Surg. Nov 2011;22(6):e41-4. [Medline]. 35. Jacquin A, Bejot Y, Hervieu M, et al. [Rupture of intracranial dermoid cyst with disseminated lipid droplets.]. Rev Neurol (Paris). Oct 19 2009;[Medline]. 36. Tatlidede S, Egemen O, Ozkaya O, Erol O. Witch nose: an embarassing metaphor for nasal tip dermoid cysts. J Craniofac Surg. Sep 2011;22(5):1948-51. [Medline]. 37. Akita S, Hirano A, Fujii T. Recurrent, discharging congenital frontotemporal dermoid cyst. Ann Plast Surg. Apr 2000;44(4):465-6. [Medline]. 38. Chakravarti A, Vishwakarma SK, Arora VK, Singh I. Dermoid (hairy polyp) of the nasopharynx. Indian J Pediatr. May-Jun 1998;65(3):473-6. [Medline]. 39. Chen CY, Lin KL, Wang HS, Lui TN. Dermoid cyst with dermal sinus tract complicated with spinal subdural abscess. Pediatr Neurol. Feb 1999;20(2):157-60. [Medline]. 40. Coppit GL 3rd, Perkins JA, Manning SC. Nasopharyngeal teratomas and dermoids: a review of the literature and case series. Int J Pediatr Otorhinolaryngol. May 30 2000;52(3):219-27. [Medline]. 41. Cramer H, Lampe H, Downing P. Dermoid cyst of the floor of the mouth diagnosed by fine needle aspiration cytology: a case report. Acta Cytol. Mar-Apr 1996;40(2):319-26. [Medline]. 42. Denoyelle F, Ducroz V, Roger G, Garabedian EN. Nasal dermoid sinus cysts in children. Laryngoscope. Jun 1997;107(6):795-800. [Medline]. 43. Nocini PF, Barbaglio A, Dolci M, Salgarelli A. Dermoid cyst of the nose: a case report and review of the literature. J Oral Maxillofac Surg. Mar 1996;54(3):357-62. [Medline]. 44. Ogle RF, Jauniaux E. Fetal scalp cysts--dilemmas in diagnosis. Prenat Diagn. Dec 1999;19(12):1157-9. [Medline]. 45. Rohrich RJ, Lowe JB, Schwartz MR. The role of open rhinoplasty in the management of nasal dermoid cysts. Plast Reconstr Surg. Dec 1999;104(7):2163-70; quiz 2171. [Medline]. 46. Santos-Briz A Jr, Serrano R, del Canizo A, Santos-Briz A, de Agustn PP. Liesegang rings in a dermoid cyst of the floor of the mouth. Report of a case with cytologic findings. Acta Cytol. Nov-Dec 2000;44(6):1062-5. [Medline]. 47. Sathananthan N, Moseley IF, Rose GE, Wright JE. The frequency and clinical significance of bone involvement in outer canthus dermoid cysts. Br J Ophthalmol. Dec 1993;77(12):789-94. [Medline]. 48. Shehadi JA, Alorainy IA, Johnston KM. Temporal dermoid cyst with a partial dermal sinus tract. Can J Neurol Sci. Nov 1999;26(4):321-4. [Medline]. 49. Tateshima S, Numoto RT, Abe S, Yasue M, Abe T. Rapidly enlarging dermoid cyst over the anterior fontanel: a case report and review of the literature. Childs Nerv Syst. Dec 2000;16(12):875-8. [Medline]. 50. Tomasini C, Aloi F, Puiatti P, Caliendo V. Dermoid cyst of the penis. Dermatology. 1997;194(2):188-90. [Medline]. 51. Vajtai I, Sutak I, Varga Z. Melanotic progonoma as a component of ovarian teratoma. Histopathology. Mar 2000;36(3):283-5. [Medline]. 52. Yamaki T, Higuchi R, Sasaki K, Nozaki M. Multiple dermoid cysts on the forehead. Case report. Scand J Plast Reconstr Surg Hand Surg. Dec 1996;30(4):321-4. [Medline]. 53. Yap EY, Hohberger GG, Bartley GB. Pilomatrixoma of the eyelids and eyebrows in children and adolescents. Ophthal Plast Reconstr Surg. May 1999;15(3):185-9. [Medline]. 54. Yilmaz M, Vayvada H, Demirdover C. Dermoid cyst at the suprasternal notch. Ann Plast Surg. Sep 2000;45(3):343. [Medline].

Vulva and Vagina tumors: an overview

Classification
Note Neoplasms of the vulva and vagina together account for less than 5% of all female genital tract cancers. Staging and grading of the lesions follows the TNM (Tumor, regional lymphoNode, Metastasis) and FIGO (International Federation of Gynecology and Obstetrics) recommendations. According to WHO recommendations, the main Vulva and Vagina categories are: VULVA NEOPLASIA: I. Epithelial neoplasms A. Squamous and related Tumors and precursors 1. Squamous cell carcinoma not otherwise specified 2. Basal cell carcinoma 3. Squamous intraepithelial neoplasia 4. Benign squamous lesions B. Glandular Tumors 1. Paget disease 2. Bartholin gland Tumors: carcinomas, adenoma and adenomyoma 3. Tumor arising from specialized ano-genital mammary-like glands 4. Adenocarcinoma of Shene gland origin 5. Adenocarcinoma of other types 6. Adenoma of minor vestibular glands 7. Mixed Tumors of the vulva 8. Tumors of skin appendage origin II. Soft tissue Tumors 1. Embryonal rhabdomyosarcoma (sarcoma botryoides) 2. Leiomyosarcoma 3. fibrous histiocytoma 4. Proximal epithelioid sarcoma 5. Alveolar soft part sarcoma 6. Liposarcoma 7. Dermatofibrosarcoma protuberans 8. Deep angiomyxoma 9. Superficial angiomyxoma 10. Angiomyofibroblastoma 11. Cellular angiofibroma 12. Leiomyoma 13. Granular cell Tumor 14. Other III. Melanocytic Tumors 1. Malignant melanoma 2. Congenital melanocytic naevus 3. Acquired melanocytic naevus 4. Blue naevus 5. Atypical melanocytic naevus of genital type 6. Dysplastic melanocytic naevus

IV. Miscellaneous Tumors 1. Yolk sac Tumor 2. Merkel cell Tumor 3. Peripheral primitive neuroectodermal Tumor/Ewing sarcoma V. Haematopoietic and lymphoid Tumors 1. Malignant lymphoma 2. Leukemia VI. Secondary tumors

VAGINA NEOPLASIA:
I. Epithelial neoplasms A. Squamous Tumors and precursors 1. Squamous cell carcinoma not otherwise specified 2. Squamous intraepithelial neoplasia 3. Benign squamous lesions (condyloma acuminatum, squamous papilloma, fibroepithelial polyp) B.Glandular lesions 1. Adenocarcinoma, NOS 2. Clear cell adenocarcinoma 3. Endometrioid adenocarcinoma 4. Mucinous adenocarcinoma 5. Mesonephric adenocarcinoma 6. Mullerian papilloma 7. Adenoma not otherwise specified C.Other epithelial Tumors 1. Adenosquamous carcinoma 2. Adenoid cystic carcinoma 3. Adenoid basal carcinoma 4. Carcinoid 5. Small cell carcinoma 6. Undifferentiated carcinoma II. Mesenchymal Tumors 1. Sarcoma botryoides 2. Leiomyosarcoma 3. Endometrioid stromal sarcoma, low grade 4. Undifferentiated vaginal sarcoma 5. Alveolar soft part sarcoma 6. Leiomyoma 7. Deep angiomyxoma 8. Post-operative spindle nodule III. Mixed epithelial and mesenchymal Tumors 1. Carcinosarcoma (Malignant Mullerian Mixed tumors; metaplastic carcinoma) 2. Adenosarcoma 3. Malignant mixed Tumors resembling synovial sarcoma 4. Benign mixed Tumors IV. Melanocytic Tumors 1. Malignant melanoma

2. Blue naevus 3. Melanocytic naevus V. Miscellaneous Tumors A. Tumor of germ cell type 1. Yolk sac Tumor 2. Dermoid Cyst B. Others 1. Peripheral primitive neuroectodermal Tumor/Ewing sarcoma 2. Adenomatoid Tumor 3. Malignant lymphoma 4. Granulocytic sarcoma VI Secondary Tumors

Clinics and Pathology

Disease Note Tumor of the vulva and vagina Benign and malignant solid tumors at these sites are rare. The malignant lesions may have epithelial (squamous and glandular) and mesenchymal (soft tissue) origin. Etiology The high-risk (HR) human papillomaviruses (HPVs) infections have been identified as an essential although not sufficient factor in the pathogenesis of vulval and vagina carcinoma. It has been demonstrated that HPV integration sites are distributed over the whole genome, with a preference for genomic fragile site. It has been also hypothesized that, at the early stages of infection, the virus genome, still in an not integrated state, expresses oncoproteins E6 and E7 which interfer with the mechanisms of chromosome segregation during mitosis. This phenomenon, would favour the virus genome integration into chromosomal DNA. However, no evidence for targeted disruption of critical cellular genes by the integrated viral sequences has been found. According to this, two categories of affected patients can be distinguhished: Malignant lesions of the vulva Older age (mean 77): no vulva intraepithelial neoplasia (VIN) pre-existing premalignant condition, not Human Papilloma Virus (HPV) related, unknown etiology. Younger age (mean 55): usually associated with VIN, HPV-related (usually type 16). Malignant lesions of the vagina The strongest association is between squamous cell carcinoma and HPV types 16 and 18 infection. Association with a pre-malignant lesion, known as vaginal intraepithelial neoplasia (VAIN), was reported. Association with previous history of cervical intraepithelial neoplasia (CIN), invasive cervical carcinoma, or invasive vulvar carcinoma has been reported. Epidemiology Malignant neoplasms of the vulva together with neoplasms of the vagina account for less than 5% of all genital tract cancers. Squamous cell carcinoma (approximately 90% and 80% of the malignant neoplasms of the vulva and the vagina, respectively) is the most commonly found, and it is primarily a disease of elderly women, although it may be also observed in premenopausal women. Pigmented vulvar and vaginal lesions may occur, including nevi and melanoma, which accounts for 9% of vulvar and 5% of vaginal

Clinics

Pathology

Treatment

Prognosis

malignant lesions. Diethylstilbestrol (DES)-Associated Disease of the vagina are described: DES is a synthetic non-steroidal estrogen used in the early 1970s to prevent miscarriage. The female fetuses delivered by the mothers taking DES suffered from severe vaginal lesions including vaginal adenosis (benign) and clear cell adenocarcinoma. Malignant mesenchimal tumors of the vulva or vagina are rare: leiomyosarcoma is the most common vulvar lesion (mean age 35), dermatofibrosarcoma is one of the rarest: 25 cases reported, mean age 54. Cancer of the vulva and vagina at the very early stages tends to be asymptomatic. Delay in diagnosis is common, partially due to disease rarity and to delay in relating patient symptoms to the disease origin. Vulva. Major symptoms are: painless bleeding unrelated to the menstrual cycle, appearing of vulvar skin white and rough. Vagina. Major symptoms are painless vaginal bleeding (65-80% of all cases), difficult or painful urination, pain in the pelvic area. Mainly post-menopausal women (70%) are affected. Many vulvar or vaginal growths are not neoplastic and may be treated by monitoring or simple excision. Suspicious growths require diagnostic biopsy and in case of cancer diagnosis surgical ablation is mandatory. The histopathology of vulva and vagina neoplasms reflect the different cell origins of the Tumors (see classification). Examples of both gross and microscopic images of these clinical entities can be viewed at http://www.gfmer.ch/selected_images_v2/level1.php?cat1=8&stype=n Immunohistochemical studies demonstrate that monoclonal antibodies to MIB-1 (Ki67), a proliferation-associated marker, distinguish two different labeling patterns in the vulvar lesions : diffuse pattern, associated with poor prognosis, or localized pattern. Vulva. Small primary lesions less than 2 cm in diameter with superficial invasion are usually treated with wide local excision with adequate surgical margins For tumors larger than 2 cm, or deeply growing into the underlying inguinal, lymphadenectomy is performed in order to plan a further partial or total vulvectomy. Radiation, with or without chemotherapy, may be used to treat advanced tumors or tumor recurrences, although there is not general consensus on the advantage of post-operative radiation therapy. Vagina. According to the FIGO, a vaginal lesion arises solely from the vagina : a vaginal lesion involving the external os of the cervix should be considered cervical cancer, and a tumor involving both vulva and vagina should be considered vulvar cancer, and they should be treated as such. Radiotherapy is the most commonly used treatment for cancer of the vagina. Indication for diverse surgical interventions (radical hysterectomy, total or subtotal vaginectomy, vulvectomy, inguinal lymphadenectomy, etc), often accompained by radiation therapy, depends on the lesion type, stage, location, size and patients history. Vulva. As with many other types of cancer prognosis depends on several factors, including the histological type of the lesion. In general, patients with increasing tumor stage have a lower rate of survival. The overall 5-year survival rate ranges from 90% to 33%, depending upon whether and how many lymphonodes are involved (not in a directly proportional way). Recurrences are seen in a high percentage of patients within the first two years of follow-up. Vagina. The histologic type, size (Tumors less than 4cm seem to be associated with a

significantly better survival rate), stage and grade and location of the tumor influence the survival rate. The overall 5-year survival rate is about 61% , with about 54% survivig for 10 years or more.

Cytogenetics
Cytogenetics Data on cytogenetics of vulva and vagina cancer are scarse. Epithelial malignancy of Morphological both lesions show cytogenetic abnormalities, although no specific chromosome markers have been identified so far, and no consistent association between cytogenetic subgroups and histological differentiation have been observed. Complex karyotypes are frequent, however simple karyotypes have been observed in a number of cases as well. Cytogenetically unrelated clones, as well as closely related clones, were found in both in situ and infiltrating squamous cell carcinoma (SCC). Structural changes of chromosome 3,8,9,11,13,14,19 and 22 have been frequently observed. Cytogenetically unrelated, abnormal clones, characterized by simple changes (chromosome X and 7 aneuploidy) have been described in Pagets disease. The karyotypes of melanoma and dermatofibrosarcoma protuberans, arising in the vulva and/or vagina, substantially do not differ from the karyotypes of the same entities arising at other sites. A single case of vagina leiomyoma has been reported recently and a t(7;8)(p13;q11.2) translocation without PLAG1 alteration has been described. Cytogenetics Fluorescence in situ hybridization (FISH) supports the cytogenetic pattern observed by Molecular conventional techniques, confirming the gain of chromosome 3q as an early and consistent change in carcinomas of the vulva, and the presence of EWS/FLI-1 fusion in extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors of both vulva and vagina. CGH profiles are also confirmatory: chromosome imbalance with gains from the long arm of chromosome 3,5,8,9 and losses from the 11q have been frequently observed. A comparison between papillomavirus-negative and papillomavirus-positive vulvar cancer indicated that chromosome 8q was more commonly gained in the positive cases.

Genes involved and Proteins

Note No specific genes involved in vulva or vagina carcinogenesis have been found so far. An isolated study indicated a prominent role of the common IL1RN intron 2 polymorphism in vulvar carcinogenesis.

This paper should be referenced as such : Vanni R, Parodo G . Vulva and Vagina tumors: an overview. Atlas Genet Cytogenet Oncol Haematol. February 2007 . URL : http://AtlasGeneticsOncology.org/Tumors/VulVaginaCarcID5274.html

Biomedical Research 17(2): 95-97 Short communication:

A rare case of vaginal dermoid cyst: A case report and review of literature
Mesfer S. Al-Shahrani and Mark Heywood Department of Obstetrics and Gynecology, and Reproductive Sciences, St Boniface General Hospital, and Univer-sity of Manitoba, Winnipeg, Manitoba, Canada Key words: Dermoid cyst, Vaginal cyst
Accepted May 03 2006

Abstract
Vaginal dermoid cyst is a rare condition. Ultrasound is the investigative tool for such an entity which may be treated surgically through a transvaginal approach.

Introduction
The finding of paravaginal dermoid cyst is rare in gyne-cology practice. The key in the management is the preop-erative assessment using transvaginal ultrasound, and the appropriate surgical intervention. We report a case of vaginal dermoid cyst and the surgical procedure in its management.

Case
A 36 year-old non-pregnant (G0P0) woman presented to her family physician for treatment refill of her bronchial asthma medications. A gynecological exam had not been performed for the last three and half years. Her family physician decided to perform a well women exam which was difficult to do because of a mass in her vagina. An ultrasound was requested which showed a solid non-vascular mass measuring 8.4 cm. by 7.5 cm by 6.1 cm , with low level echogenic material as well as echogenic bands, situated within the posterior wall of the vagina , in the rectovaginal space; the rest of the pelvis was normal ; with an impression of a dermoid cyst. The patient was referred to the Gynecology Oncology clinic for further evaluation and management. She was asymptomatic, and had not been sexually active for the past two years. Prior to that she has been sexually Fi.g. 1: Vaginal mass ( left Vaginal wall ). Fig. 2: Vaginal mucosa over tha mass was opened . Al-Shahrani / Heywood 962 Fig. 3: Cyst capsule ruptured, revealing a sebaceous ma-terial . Fig. 4: Sebaceous materials extruded from the cyst . active without any problems. The remainder of her his-tory was non-contributory. Examination revealed a smooth mass on the left side, just above the vaginal introitus, extending up to the cervix, and was non tender. Pelvirectal exam revealed an approximately 8 by 8 centimeters mass filling the rectovaginal septum to the left. Under general anesthesia this cyst was removed. Incision was made on the vaginal mucosa on top of the mass ex-tended toward the cervix, measuring approximately 6 cm (Figure 1). The mass surface was identified, blunt dissec-tion was carried out (Figure 2), the mass ruptured reveal-ing a sebaceous material (Figures 3, 4) which was ex-truded and collected in a bowl. The cyst wall was then dissected free from the rectovaginal space with good ho-meostasis . Incision was sutured with a running locked 0 Vicryl suture, and a vaginal pack was inserted. The patient was discharged next day after removal of the vaginal pack in good condition. Histopathological exami-nation of the cyst wall, showed keratinized stratified squamous epithelium. There were few sebaceous and apocrine glands noted. The periphery showed chronic inflammation and occasional skeletal muscle tissue. No immature cellular elements were noted. The findings were consistent with a diagnosis of a dermoid cyst.

Discussion

A dermoid cyst ( Benign cystic teratoma) is a benign germ cell tumor that contains well-differentiated derivatives of all three germ cell layers. Ovaries are the commonest site for this tumor , where it is the commonest neoplastic tumor in children and adoles-cent accounting for more than half of ovarian neoplasms in women younger than 20 years of age. More than 80% of ovarian benign dermoid cysts occur during reproduc-tive years. A dermoid cyst has also been reported from other parts of the human body other than the ovaries, with rare occur-rence. It can be any where in the gastrointestinal tract from the floor of the mouth to the colon (2,3,6,7). Dermoid cysts have also been reported in male patients, in different parts of their body (6). Vaginal dermoid is a rare condition. Only five cases have been reported in English literature. First observed in 1899 by Stokes (9), who reported a 44 year old woman who had a 1 centimeter cyst removed from just within the hymen. The cystic contents showed neumerous seba-ceous glands and a few hair follicles. Curtis (1) described an ulcerated orange-sized necrotic cyst, containing hair and sebaceous materials in the vaginal mucosa. Johnston (5) described a 4-inch cyst that passed from the vagina in a woman following delivery of her second child. The cyst was filled with thick sebaceous material with matted hair and had been attached to the vaginal wall by a narrow stalk. Hirose et al (4) reported another case, having re-peated painful right vaginal wall cyst, which was excised and was found to be a dermoid cyst confirmed by histopa-tological examination. Preoperative diagnosis of the exact nature of a vaginal cyst can be difficult. S.S. N. SIU et al (8) reported the sonographic characteristics of a vaginal cyst, which was consistent with a dermoid cyst and was confirmed by histopathological exam after surgical excision of the cyst .The same sonographic features were found in our case. A rare case of vaginal dermoid cyst 97 Transvaginal excision of this type of cyst appears to be an appropriate surgical treatment option which was incon-formity with some of the previous cases (4, 8, 9).

Conclusion
Vaginal dermoid cyst is a rare condition, which can be diagnosed with ultrasound. Transvaginal excision appears to be an appropriate surgical treatment option.

References
1. Curtis AH. Transactions of Societies. Chicago Gyneco-logical Society. Surg Gynecol Obstet 1913; 16: 715. 2. Fernandez-Cebrian JM, Carda P, Morales V, Galindo J. Dermoid cyst of the pancreas: a rare cystic neoplasm. Hepatogastroenterology 1998; 45: 1874-1876. 3. Fujita K, Akiyama N, Ishizaki M, Tanaka S, Ohsawa K, Sugiyama H, et al. Dermoid cyst of the colon. Dig Surg 2001; 18: 335-337. 4. Hirose R, Imai A, Kondo H, Itoh K, Tamaya T. A der-moid cyst of the paravaginal space. Arch Gynecol Ob-stet 1991; 249: 39-41. 5. Johnston HW. A dermoid cyst of the vagina compli-cated by pregnancy. Can Med Assoc J 1939; 41: 386. 6. Longo F, Maremonti P, Mangone GM, De Maria G, Califano L. Midline (dermoid) cysts of the floor of the mouth: report of 16 cases and review of surgical tech-niques. Plast Reconstr Surg 2003; 112: 1560-1565. 7. Schuetz MJ, 3rd, Elsheikh TM. Dermoid cyst (mature cystic teratoma) of the cecum. Histologic and cytologic features with review of the literature. Arch Pathol Lab Med 2002; 126: 97-99. 8. Siu SS, Tam WH, To KF, Yuen PM. Is vaginal der-moid cyst a rare occurrence or a misnomer? A case re-port and review of the literature. Ultrasound Obstet Gynecol 2003; 21: 404-406. 9. Stokes JE. The etiology and structure of true vaginal cysts. Johns Hopkins Hosp Rep 1899; 7: 109-136.

Correspondence: Mesfer Al-Shahrani Department of Obstetrics and Gynecology St. Josephs Health Care London 268 Grosvener Street London, ON, Canada N6A 4V2 Phone: 00 1 519 646 6106 Fax 00 1 519 646 6213 e- Mail [email protected]