An 11 years old boy with dismorphical syndrome and associated malformations is reported having multiple keratocysts in both jaws. Odontogenic cysts are those which arise from the epithelium associated with the development of teeth. Also called as odontogenic ghost cell cyst or gorlin cyst. It is thought to originate from dental laminae residues. It is considered as an extremely rare cyst and accounts for only 1% of jaw cysts. They might also have a number of other medical conditions. Gorlin syndrome associated with midline nasal dermoid cyst article pdf available in journal of medical genetics 338.
Gorlin syndrome is also known as nevoid basal cell carcinoma syndrome or basal cell nevus syndrome. From thoma to gorlin fumio ide, kentaro kikuchi, yuji miyazaki, kaoru kusama, ichiro saito, and takashi muramatsu department of diagnostic pathology, tsurumi university school of dental medicine, 2 tsurumi, tsurumiku, yokohama, 2308501 japan. Gorlins cyst whonamedit dictionary of medical eponyms. Our followup radiograph did not show any signs of recurrence. It is an unusual and unique lesion, which may show characteristics of both a solid neoplasm and a cyst. Calcifying odotogenic cyst is a benign odontogenic tumor of cystic type most likely to affect the anterior areas of the jaws. As additional series were reported, it was recognized that these lesions can demonstrate a spectrum of histopathologic features, sometimes occurring as more solid tumorlike growths.
Calcifying cystic odontogenic tumor associated with an odontome a diverse lesion encountered chourasia radheshyam,1 bandyopadhyay alokenath,2 harish kumar,2 panda abikshyeet2 1shri medishine hospital, raipur, chhattisgarh, india. Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a condition that affects many areas of the body and increases the risk of developing various cancerous and noncancerous tumors in people with gorlin syndrome, the type of cancer diagnosed most often is basal cell carcinoma, which is the most common form of skin cancer individuals with gorlin syndrome typically begin to. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a. Calcifying odontogenic cyst with extensive areas of.
Ccot are cysts of primordial origin and not associated with crown of any impacted tooth. Cysts of the oral and maxillofacial regions fourth edition mervyn shear bds, mds, dsc dent, hdipdent, frcpath, frssaf, lld hc, dchd hc, hon fcd cmsa, hon fcpath cmsa professor emeritus, university of the witwatersrand, johannesburg and paul speight bds, phd, fdrcps glasg, fdsrcs eng, fdsrcs edin, frcpath professor of oral. People with this syndrome are particularly prone to developing a common and usually nonlifethreatening form of non melanoma skin cancer. It is most common in people in their second to third decades but can be seen at almost any age. The nevoid basal cell carcinoma syndrome nbccs is a rare, complex genetic disorder characterized by a wide variety of developmental abnormalities and a predisposition to developing certain forms of cancer, particularly a type of skin cancer known as basal cell carcinoma. Radiographic examination revealed six cysts in maxilla and mandible which were excised and histologically were compatable with keratocystic odontogenic tumors. Gorlingoltz syndrome nevoid basal cell carcinoma syndrome. Calcifying odontogenic cyst is an uncommon developmental odontogenic cyst first described by gorlin in 1962. The calcifying odontogenic cyst or gorlin cyst was originally identified as an independent condition by gorlin in 1962 year.
Nevoid basal cell carcinoma syndrome nbccs, also known as gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. Mim109400 a syndrome of myriad basal cell nevi with development of basal cell carcinomas in adult life, odontogenic keratocysts, erythematous pitting of the palms and soles, calcification of the cerebral falx, and frequently skeletal anomalies, particularly ribs that are bifid or broadened anteriorly. Pdf calcifying odontogenic cyst gorlins cyst report of. Gorlin was one of the founders and a diplomate of the american board of medical genetics, clinical genetics. It is characterized by keratocystic odontogenic tumors kcot in the jaw, multiple basal cell nevi carcinomas and skeletal. Aug 23, 2012 odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. Herein, we report a case of gorlin gotz syndrome who developed a meningioma, which is a rare association with this syndrome. Dec 16, 2009 this document summarizes data about gorlingoltz syndrome. Multiple maxillary and mandibular cysts are principle features of basal cell nevus syndrome gorlin goltz. These cysts should be considered in the differential diagnosis of any radiolucent area.
Gorlin syndrome definition of gorlin syndrome by medical. Odontogenic keratocysts in nevoid basal cell carcinoma. The calcifying cystic odontogenic tumor ccot, also known as calcifying odontogenic cyst coc or gorlin cyst is a rare developmental lesion which arises from odontogenic epithelium. Meningioma associated with gorlingoltz syndrome and a. The syndrome follows an autosomal dominant inheritance pattern with a gene mutation localized to 9q22.
The calcifying ghost cell odontogenic cyst cgcoc was first described by gorlin et al. The keratinizing and calcifying odontogenic cyst gorlin cyst the diagnosis and treatment of odontogenic cysts and tumors presents an interesting but difficult problem for the clinician. Calcifying ghost cell odontogenic cyst is comparatively rare in occurrence, constituting about 0. Calcifying odontogenic cyst is a rare and specific lesion with properties of a tumor that can appear anywhere on the body and have properties of a cyst. Calcifying epithelial odontogenic cyst of the mandible. Cysts are removed surgically each time they appear. The source of epithelium is from the enamel organ, the reduced enamel epithelium, the cell rests of malassez or the remnants of the dental lamina. Such diagnosis in the literature is important, as there is. Pdf the calcifying epithelial odontogenic cyst ceoc is a developmental odontogenic cyst, which was first categorized as a distinct entity by gorlin. We present cases from an affected family in which magnetic resonance imaging mri was helpful in evaluation of the cystic lesions.
The present case report briefs a case of gorlin cyst in a 21 year old. Odontogenic keratocysts make up around 19% of jaw cysts. The frequency of the syndrome varies according to the country where the study has been carried out. Gorlin syndrome is an autosomal dominant disorder characterized by a wide range of developmental abnormalities and a predisposition to various tumors, and it is linked to the alteration of several. Calcifying cystic odontogenic tumour is a developmental cyst of odontogenic origin and constitutes about 0. This case report highlights a gorlin goltz syndrome with left orbital and multiple facial basal cell carcinoma, calcified falx cerebri with cyst in the right mandible. This cyst is not common in the gnathic bones, representing about less than 6% of all odontogenic lesions 2, 3. A novel ptch1 mutation in a patient with gorlin syndrome. Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. Odontogenic keratocyst is a locally aggressive and highly recurrent lesion, which occurs mostly on the posterior mandible with a slightly higher rate in males. A 47yearold female presented with a 30 year history of slowly enlarging left periorbital mass eventually. Calcifying odontogenic cyst gorlin cyst ddmfr oral.
The most notable features of this pathologic entity are histopathological features which include a cystic lining demonstrating characteristic. An abnormal membranous sac in the body containing a gaseous, liquid, or semisolid substance. Calcifying odontogenic cyst gorlin cyst ddmfr oral and. Peripheral calcifying cystic odontogenic tumour of the. The keratinizing and calcifying odontogenic cyst kcoc, or gorlin cyst, is not actually a cyst but rather a neoplasm with cystic tendencies.
Gorlin syndrome is an autosomal dominant condition including skin, skeletal, craniofacial, neurologic, oropharyngeal, and ophthalmologic features. The peripheral calcifying odontogenic cyst pcoc accounts for less than 25% of the cases of calcifying odontogenic cysts and most commonly appears as a nodule on the gingiva. Gorlin syndrome presenting with a unilateral ovarian fibroma. Calcifying cystic odontogenic tumor may be centrally or peripherally located, and its. A case of gorlin goltz syndrome is presented here in which the abovementioned findings are evident. Dec 03, 2019 keratinizing and calcifying odontogenic cyst calcifying odontogenic cyst. Ct scan also revealed falx cerebral calcification which led to the diagnosis of gorlin syndrome confirmed on genetic testing. Peripheral calcifying cystic odontogenic tumour of the maxillary. Cysts can be congenital but are usually acquired, the most common. Cells believed to be responsible for the ccot are rests of serres.
Calcifying odontogenic cyst, as gorlin cyst was recognized earlier, was first. Jun 12, 2012 nevoid basal cell carcinoma syndrome, or gorlin syndrome, is an inherited disorder characterized by malignancies of the skin and other organs, skeletal abnormalities, and congenital malformations. In 1962 gorlin et al, was the first person to describe calcifying ghost cell odontogenic cyst cgcoc under the term calcifying odontogenic cyst coc. Meningioma associated with gorlingoltz syndrome and a short. A benign dental cyst histologically characterised by ghost body, which is asumed to be keratinised cells and an accentuated basal cell layer.
Jul 24, 2010 although the lesion has been commonly recognized as a benign odontogenic cyst since gorlin et al. The calcifying odontogenic cyst coc was first described by gorlin et al. Gorlin cyst which is, also, recognised by the synonyms calcifying. The calcifying epithelial odontogenic cyst ceoc is a developmental odontogenic cyst, which was first categorized as a distinct entity by gorlin in 1962. A differential diagnosis of cystic ameloblastoma, dentigerous cyst, and odontogenic keratocyst was made. Gorlin syndrome other conditions cancer research uk. Apr 22, 20 the calcifying odontogenic cyst gorlin cyst. Calcifying odontogenic cyst was first described by gorlin et al. A patient with gorlin syndrome should be monitored regularly and a periodic recall is very essential to evaluate potential cyst recurrences, new cyst formation and development of basal cell carcinomas 2. Odontogenic cysts and tumors are mandibular and maxillary lesions that occur across all patient demographics across age, sex, race, and social economic status, as altered remnants of dental development. A 50year old female patient presented with headache and headreeling for 1 year, and pain in the right lower jaw and right side of face for 3 months. Although the lesion has been commonly recognized as a benign odontogenic cyst since gorlin et al. Calcifying odontogenic cyst gorlin cyst odontoma mixed lesion cementoblastoma. Gorlin syndrome pictures, symptoms, life expectancy.
Oral lichen planus olp is a chronic mucocutaneous disease with an unknown aetiology, affecting 0. Clark is a thirdyear resident, oral and maxillofacial surgery, bexar county hospital district teaching hospitals of the university of texas health science center, san antonio. The calcifying odontogenic cyst gorlin cyst pure facts. Gorlin syndrome is an autosomal dominant multisystem disorder characterised by multiple basal cell naevi, cysts of the jaw, pits of the palms and soles, skeletal anomalies, and various other defects.
Nevoid basalcell carcinoma syndrome nbccs, is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones. Who defined it as a cystic lesion in which the epithelial lining shows a welldefined basal layer of columnar cells, an overlying layer that is often many cells thick and that may resemble. The calcifying odontogenic cyst coc, first described by gorlin et al. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Gorlin cyst definition of gorlin cyst by the free dictionary. This is a very rare lesion with no age, sex, or location predilections.
Gorlin cyst article about gorlin cyst by the free dictionary. Colloid cyst and multiple meningiomata in gorlin syndrome. In our case, the lesion is central with expansion of buccal cortical plate and the cyst was asymptomatic. In onethird of cases, an impacted tooth is involved. In the whoiarc classification of head and neck pathology, this clinical entity had been known for years as the odontogenic keratocyst. In 1962, gorlin and associates first described an unusual type of odontogenic cyst, which they termed the calcifying odontogenic cyst. The keratinizing and calcifying odontogenic cyst gorlin cyst martin j. Gorlin cyst synonyms, gorlin cyst pronunciation, gorlin cyst translation, english dictionary definition of gorlin cyst. In 1962, gorlin described a rare cystic form termed the calcifying odontogenic cyst coc or. Epidermal cysts are not usually a presenting feature of nbcc. The estimated prevalence varies from 157,000 to 1256,000, with a maletofemale ratio of 1. Calcifying cystic odontogenic tumor accompanied by a dentigerous. Patients with gorlin syndrome have a predisposition to basal cell carcinomas and other neoplasms.
However, a clinical study with larger number of cases and longterm followup is required. May lead to the development of odontogenous tumours. They may be incidental findings from routine imaging in any office or found through workup for craniofacial surgery or injury. Who defined it as a cystic lesion in which the epithelial lining shows a welldefined basal layer of columnar cells, an overlying layer that is often many cells thick and that may resemble stellate reticulum, and masses of ghost epithelial cells that may be in the epithelial cyst lining or in the fibrous. It was thoroughly described by gorlin and coworkers in 19621 and in. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastomalike epithelium and ghost cells. Calcifying odontogenic cyst with extensive areas of dentinoid. This syndrome is also named as gorlin syndrome, multiple nevoid basal cell epithelioma, jaw cyst bifid rib syndrome, or multiple nevoid bcc syndrome.
The gorlin goltz syndrome, also known as nevoid basal cell carcinoma syndrome nbccs, is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. He had multiple okcs, calcification of falx cerebri, bifid ribs, frontal bossing. Letter to editor gorlingoltz syndrome diagnosis hatem zein ldeen, r. Calcifying odontogenic cyst or gorlin cyst, was first described by gorlin. Inside the cyst is a thick, sticky, clear, colorless, jellylike material. Tokyo, japan for providing language editing services. The early history of odontogenic ghost cell lesions. It was previously described by gorlin et al in 1962 as a calcifying odontogenic cyst. Mar 10, 2015 the calcifying epithelial odontogenic cyst ceoc is a developmental odontogenic cyst, which was first categorized as a distinct entity by gorlin in 1962. Who defined it as a cystic lesion in which the epithelial lining shows a welldefined basal layer of columnar cells, an overlying layer that is often many cells thick and that may resemble stellate reticulum, and masses of ghost epithelial cells that may be in the epithelial cyst lining or in the. Nevoid basal cell carcinoma syndrome is rare in indian population or may be unreported.
Dec 23, 2016 cysts of the oral and maxillofacial regions 4th. Gorlin cyst which is also known as calcifying odontogenic cyst coc, calcifying cystic odontogenic tumor ccot, calcifying ghost cell odontogenic cyst, and dentogenic ghost cell tumor, is a rare developmental lesion that arises from the odontogenic epithelium and represents about 2% of all odontogenic pathological changes in the jaw. This is the first report to describe the coexistence of gorlin syndrome and a nasal dermoid cyst. Nevoid basal cell carcinoma syndrome nord national. Considering the rarity of this syndrome, we present a 12yearold boy affected by this syndrome. Cutaneous epidermal cysts as a presentation of gorlin. To our best knowledge, this is the fourth published report associating colloid cyst and gorlin syndrome. A ganglion cyst is a small sac of fluid that forms over a joint or tendon tissue that connects muscle to bone. Sashikiran 3, debasis sahu 4 1 department of oral and maxillofacial medicine and radiology, saraswatidhanwantari dental college and hospital and postgraduate research institute, parbhani, maharashtra, india 2 department of oral and maxillofacial medicine and. It is a rare genetic disorder characterized by the development of multiple basal cell carcinomas resulting in multiple defects of the body in general. Nevoid basal cell carcinoma syndrome gorlin syndrome. Gorlin syndrome associated with midline nasal dermoid cyst. Calcifying cystic odontogenic tumor associated with an odontome. However, also called gorlin cyst, calcifying odontogenic cyst coc, keratinizing and calcifying odontogenic cyst commonly used terminologies.
Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a condition that affects many areas of the body and increases the risk of developing various cancerous and noncancerous tumors. Gorlin goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. A calcifying cystic odontogenic tumor ccot is a proliferation of. Chronic osteomyelitis mixed florid cementoosseous dysplasia mixed fibrous dysplasia mixed cementoossifying fibroma 2nd stage paget disease mixed osteogenic sarcoma mixed chondrosarcoma mixed metastatic tumors mixed. Gorlin syndrome is a rare condition in which many people develop a type of skin cancer called basal cell cancer of the skin. The gorlin goltz syndrome is rare but wellknown multisystemic syndrome. Nevoid basal cell carcinoma syndrome, a rare autosomal dominant disorder, comprises a number of abnormalities such as multiple nevoid basal cell carcinomas, skeletal abnormalities and multiple odontogenic keratocysts. On radiographs, the calcifying odontogenic cyst appears as a unilocular radiolucency dark area. Calcifying odontogenic cyst gorlins cyst report of. Radiographically, it may appear unilocular or multilocular, with or without defined borders. Has many features of odontogenic tumor, therefore it is placed in the category of tumors in the latest who classification of odontogenic cysts and tumors. Malignant transformation of calcifying cystic odontogenic.
Pdf calcifying epithelial odontogenic cyst of the mandible. The calcifying odontogenic cyst coc was first described by gorlin and colleagues in 1962, which described a cyst lined by an ameloblastomalike epithelium that contained variable amounts of ghost cells and calcifications. Gorlin syndrome is also called naevoid basal cell carcinoma syndrome nbccs. The association between colloid cyst with gorlin syndrome was first raised by nishino in 1991 and two more cases have been identified. The keratinizing and calcifying odontogenic cyst gorlin cyst. The keratinizing and calcifying odontogenic cyst gorlin. In people with gorlin syndrome, the type of cancer diagnosed most often is basal cell carcinoma, which is the most common form of skin cancer. Barnes l, eveson jw, reichart p, sidransky d, editors. Gorlins syndrome with associated odontogenic cysts. Gorlin cyst definition of gorlin cyst by medical dictionary. The present paper highlights the importance of diagnostic criteria and histopathology in early and prompt diagnosis which will lead to proper treatment and genetic counseling of the patient. Calcifying cystic odontogenic tumor associated with an.
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