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The gastrointestinal system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Sharon J. White, Francis A. Carey
The most frequent developmental odontogenic cyst is the dentigerous cyst, which develops around the crown of an unerupted tooth. Closely related is the eruption cyst, which presents as a bluish fluctuant swelling in the gum overlying the crown of an erupting tooth. Both cysts are lined by non-keratinized stratified squamous epithelium, which may include a few mucus-secreting cells. These cysts are usually symptomless unless infected and can grow to several centimetres with considerable bone destruction. They must be differentiated from the odontogenic keratocyst which has a distinctive parakeratinized, stratified squamous epithelial lining (Figure 10.4). Its relationship to the teeth is variable and it occurs anywhere in the jaws, the most common site being the posterior mandible, often extending up into the ramus of the mandible. Although previously categorized as a benign, potentially aggressive odontogenic tumour, odontogenic keratocyst has recently been reclassified as a developmental odontogenic cyst which possesses a high recurrence rate partly due to the friable nature of the lining and the presence of small daughter cysts within the cyst wall. Multiple, recurrent, odontogenic keratocysts are seen as part of the naevoid basal cell carcinoma syndrome (Gorlin syndrome). Mutations in the PTCH1 gene are associated with syndromic and non-syndromic odontogenic keratocysts.
Test Paper 4
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Odontogenic keratocysts are believed to arise from the dental lamina and other sources of odontogenic epithelium. At radiography, an odontogenic keratocyst usually appears as a unilocular, lucent lesion with smooth, corticated borders that is often associated with an impacted tooth. Such lesions are indistinguishable from dentigerous cysts at radiography. They are more likely to show aggressive growth than other odontogenic cysts and may have undulating borders and a multilocular appearance; these characteristics make odontogenic keratocysts indistinguishable from ameloblastomas.
The Classification of Odontogenic Cysts
Published in Roger M. Browne, Investigative Pathology of the Odontogenic Cysts, 2019
The most common radiological appearance is that of an apparent dentigerous cyst,9–11 being present in 40 to 50% of lesions. However, careful examination of the radiographs suggests that the crown of the associated unerupted tooth does not project into the cyst cavity, but rather has been displaced by it (Figure 2). This relationship is confirmed when the appropriate histological material can be examined.7,15 However, in a small proportion of cysts, the odontogenic keratocyst is in true dentigerous relationship to the associated unerupted tooth.7,16 This has given rise to the term follicular primordial cyst.17 There is evidence that such lesions arise originally in an extrafollicular position, discrete from the associated tooth, and subsequently either their epithelial linings fuse with the reduced enamel epithelium, so enclosing the entire anatomical crown of the tooth,7 or the tooth actually erupts into the cyst lumen.17 In whatever way the relationship arises, such cysts should be regarded as a variant of the odontogenic keratocyst and classified with them. In addition to its radiological appearance as a dentigerous cyst, the odontogenic keratocyst can assume a variety of other presentations, including that of a lateral periodontal cyst (Figure 3), a radicular cyst (Figure 4), a primordial cyst (Figure 1) and a residual cyst (Figure 5). It is this variety of clinical and radiological appearances that has contributed to the delay in the recognition of the odontogenic keratocyst as a distinct entity. A single case report of a primary odontogenic keratocyst arising solely in the alveolar mucosa has been published.18 The use of the term peripheral odontogenic keratocyst was suggested by the authors to describe this rare occurrence.
Recurrent odontogenic keratocyst with orbital invasion
Published in Orbit, 2022
Valerie Juniat, Alistair Varidel, James Badlani, James Nolan, Paul Sambrook, Dinesh Selva
OKCs are acquired, non-choristomatous lesions which arise from the remnants of the dental lamina with secondary proliferation of enamel epithelium.1 It is difficult to know the true incidence of OKC involving the orbit due to the rarity of the condition. This is further complicated by inconsistencies in the nomenclature, with OKCs being named in 2005 by the WHO as keratocystic odontogenic tumours.1 Previously to this, OKCs have been called variously as odontogenic cysts and odontogenic keratocysts. The designation was reverted to by consensus back to odontogenic keratocyst in 2017, justified on the grounds that the PTCH mutation found in OKC can also be found in non-neoplastic lesions and resolution of the cyst after marsupialisation does not correlate with a neoplastic process.1,4 The authors have attempted to address this by including pre-2017 terms in the literature review.
Nevoid basal cell carcinoma syndrome: a case report and literature review
Published in Ophthalmic Genetics, 2022
Shripadh Chitta, Jineet Patel, Shravan Renapurkar, Christopher Loschiavo, Jennifer Rhodes, Kayla King, Kimberly Salkey, Natario Couser
Repeat scans 7 months after the initial decompression showed progressive shrinkage in size of the keratocysts (Figure 2). The oral and maxillofacial surgery team also determined there to be sufficient bone fill by this time for definitive treatment. At the age of 9, the patient underwent surgical excision by enucleation and curettage of the maxillary and mandibular OKCs and peripheral ostectomy with cryotherapy of the right maxillary and bilateral mandibular odontogenic keratocysts as well as odontectomies of full bony impacted teeth #1, 20 and 31 which were associated with the cysts. Within 13 months of this procedure, odontogenic keratocysts appeared in the right mandible and bilateral posterior maxilla with multiple full bony impacted teeth. At the age of 10, the patient underwent marsupialization of the right mandibular cyst and E&C with cryotherapy of both maxillary keratocysts with extraction of multiple teeth. The patient was found to have multiple blood pressures which were greater than 95th percentile for his age. He was referred to cardiology and nephrology for further work-up of these values.
Gorlin syndrome presenting with primary infertility and bilateral calcified ovarian fibromas
Published in Journal of Obstetrics and Gynaecology, 2019
Rashmi Bagga, Shivani Garg, Tanuja Muthyala, Jasvinder Kalra, Pradip Kumar Saha, Aashima Arora, Rimpy Singla, Tulika Singh, Nalini Gupta
Gorlin syndrome (GS) is a genetically inherited multisystem disorder with a rare incidence. We report a young woman with GS who presented with primary infertility and had undergone excision of odontogenic keratocysts of mandible. She had frontal bossing, ptosis and bilateral ovarian calcified fibromas. Calcified ovarian fibromas are present in 15–25% of women with GS and are bilateral in 75% of cases. As GS is associated with other pathologies, such as melanoma, medulloblastoma, mandibular odontogenic cysts and multiple skeletal deformities, these women need a thorough evaluation.