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Head and Neck Pathology Mar 2021This article reviews odontogenic and developmental oral lesions encountered in the gnathic region of pediatric patients. The process of odontogenesis is discussed as it... (Review)
Review
This article reviews odontogenic and developmental oral lesions encountered in the gnathic region of pediatric patients. The process of odontogenesis is discussed as it is essential to understanding the pathogenesis of odontogenic tumors. The clinical presentation, microscopic features, and prognosis are addressed for odontogenic lesions in the neonate (dental lamina cysts/gingival cysts of the newborn, congenital (granular cell) epulis of the newborn, melanotic neuroectodermal tumor, choristoma/heterotopia, cysts of foregut origin), lesions associated with unerupted/erupting teeth (hyperplastic dental follicle, eruption cyst, dentigerous cyst, odontogenic keratocyst/keratocystic odonogenic tumor, buccal bifurcation cyst/inflammatory collateral cyst) and pediatric odontogenic hamartomas and tumors (odontoma, ameloblastic fibroma, ameloblastoma, adenomatoid odontogenic tumor, primordial odontogenic tumor). Pediatric odontogenic and developmental oral lesions range from common to rare, but familiarity with these entities is essential due to the varying management implications of these diagnoses.
Topics: Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Jaw Diseases; Male; Odontogenesis; Tooth Abnormalities
PubMed: 33723756
DOI: 10.1007/s12105-020-01284-3 -
Children (Basel, Switzerland) Oct 2022Compound odontoma is a malformation typical of young adults below the age of 20, with a slight preference for the male gender and the anterior region of the maxilla.... (Review)
Review
Compound odontoma is a malformation typical of young adults below the age of 20, with a slight preference for the male gender and the anterior region of the maxilla. Clinically asymptomatic, it can be detected during a radiological investigation in connection with the persistence of deciduous dental elements and the impaction of definitive ones. The treatment of choice is excisional surgery and recurrence is a rare event. The need for orthodontic therapy for impacted elements is usually not necessary because in most cases, odontomas are small, circumscribed lesions the size of a permanent tooth. In this article, the diagnostic and therapeutic surgical excision procedure is presented in three patients at developmental age with large compound odontomas associated with at least one retained canine, and in two of the cases, with serious transmigration to the impacted tooth elements.
PubMed: 36291445
DOI: 10.3390/children9101509 -
Orphanet Journal of Rare Diseases Oct 2009Familial adenomatous polyposis (FAP) is characterized by the development of many tens to thousands of adenomas in the rectum and colon during the second decade of life.... (Review)
Review
Familial adenomatous polyposis (FAP) is characterized by the development of many tens to thousands of adenomas in the rectum and colon during the second decade of life. FAP has an incidence at birth of about 1/8,300, it manifests equally in both sexes, and accounts for less than 1% of colorectal cancer (CRC) cases. In the European Union, prevalence has been estimated at 1/11,300-37,600. Most patients are asymptomatic for years until the adenomas are large and numerous, and cause rectal bleeding or even anemia, or cancer develops. Generally, cancers start to develop a decade after the appearance of the polyps. Nonspecific symptoms may include constipation or diarrhea, abdominal pain, palpable abdominal masses and weight loss. FAP may present with some extraintestinal manifestations such as osteomas, dental abnormalities (unerupted teeth, congenital absence of one or more teeth, supernumerary teeth, dentigerous cysts and odontomas), congenital hypertrophy of the retinal pigment epithelium (CHRPE), desmoid tumors, and extracolonic cancers (thyroid, liver, bile ducts and central nervous system). A less aggressive variant of FAP, attenuated FAP (AFAP), is characterized by fewer colorectal adenomatous polyps (usually 10 to 100), later age of adenoma appearance and a lower cancer risk. Some lesions (skull and mandible osteomas, dental abnormalities, and fibromas on the scalp, shoulders, arms and back) are indicative of the Gardner variant of FAP. Classic FAP is inherited in an autosomal dominant manner and results from a germline mutation in the adenomatous polyposis (APC) gene. Most patients (~70%) have a family history of colorectal polyps and cancer. In a subset of individuals, a MUTYH mutation causes a recessively inherited polyposis condition, MUTYH-associated polyposis (MAP), which is characterized by a slightly increased risk of developing CRC and polyps/adenomas in both the upper and lower gastrointestinal tract. Diagnosis is based on a suggestive family history, clinical findings, and large bowel endoscopy or full colonoscopy. Whenever possible, the clinical diagnosis should be confirmed by genetic testing. When the APC mutation in the family has been identified, genetic testing of all first-degree relatives should be performed. Presymptomatic and prenatal (amniocentesis and chorionic villous sampling), and even preimplantation genetic testing is possible. Referral to a geneticist or genetic counselor is mandatory. Differential diagnoses include other disorders causing multiple polyps (such as Peutz-Jeghers syndrome, familial juvenile polyps or hyperplastic polyposis, hereditary mixed polyposis syndromes, and Lynch syndrome). Cancer prevention and maintaining a good quality of life are the main goals of management and regular and systematic follow-up and supportive care should be offered to all patients. By the late teens or early twenties, colorectal cancer prophylactic surgery is advocated. The recommended alternatives are total proctocolectomy and ileoanal pouch or ileorectal anastomosis for AFAP. Duodenal cancer and desmoids are the two main causes of mortality after total colectomy, they need to be identified early and treated. Upper endoscopy is necessary for surveillance to reduce the risk of ampullary and duodenal cancer. Patients with progressive tumors and unresectable disease may respond or stabilize with a combination of cytotoxic chemotherapy and surgery (when possible to perform). Adjunctive therapy with celecoxib has been approved by the US Food and Drug Administration and the European Medicines Agency in patients with FAP. Individuals with FAP carry a 100% risk of CRC; however, this risk is reduced significantly when patients enter a screening-treatment program.
Topics: Adenomatous Polyposis Coli; Animals; Female; Humans; Male
PubMed: 19822006
DOI: 10.1186/1750-1172-4-22 -
European Journal of Paediatric Dentistry Sep 2020Odontomas are hamartomatous developmental malformations of the dental tissues. Usually asymptomatic, their presence is often revealed on routine radiographs. The study... (Review)
Review
BACKGROUND
Odontomas are hamartomatous developmental malformations of the dental tissues. Usually asymptomatic, their presence is often revealed on routine radiographs. The study aimed to establish the efficacy of this conventional approach in treating odontomas, analysing clinical outcome, follow-up, and histomorphological profile.
CASE REPORT
A case is presented with a review of the international literature. The patient, aged 8 years, had a complex odontoma localised on the front upper jaw. She was treated following the conventional surgical procedure. Post-operative course and healing were uneventful. Orthodontic treatment was necessary to realign the teeth. At the 12-month follow-up there was no recurrence or failure. Healing was excellent.
CONCLUSION
Variations in normal tooth eruption are a common finding, but significant deviations from established norms should alert the clinician to further investigate the patient's health and development.
Topics: Child; Female; Humans; Maxilla; Neoplasm Recurrence, Local; Odontoma; Tooth Eruption; Tooth, Impacted
PubMed: 32893652
DOI: 10.23804/ejpd.2020.21.03.08 -
Ear, Nose, & Throat Journal Sep 2021
Topics: Adolescent; Adult; Child; Female; Humans; Male; Mandibular Neoplasms; Medical Illustration; Odontoma; Young Adult
PubMed: 31760790
DOI: 10.1177/0145561319890175 -
BMJ Case Reports Jun 2015Ameloblastic fibro-odontoma is a slow growing, benign, expansile epithelial odontogenic tumour with odontogenic mesenchyme, accounting for 0.3-1.7% of jaw tumours,...
Ameloblastic fibro-odontoma is a slow growing, benign, expansile epithelial odontogenic tumour with odontogenic mesenchyme, accounting for 0.3-1.7% of jaw tumours, signifying its rarity. The WHO defines it as "a neoplasm composed of proliferating odontogenic epithelium in a cellular ectomesenchymal tissue with varying degrees of inductive changes and dental hard tissue formation". We report a case of an 11-year-old girl who presented to the Department of Maxillo-Facial Medicine and Radiology for the evaluation of a swelling in the left posterior mandible. Her clinical chart and investigations unveiled it as ameloblastic fibro-odontoma. After a promising presurgical evaluation, the lesion was enucleated using an intraoral approach followed by osteoplasty. Osteogenesis was attained despite of any definitive techniques to promote bone regeneration. Immediate postoperative inter-maxillary fixation was performed to prevent pathological fractures for a period of 3 weeks. In an 8-month follow-up, no untoward complications were noticed.
Topics: Child; Epithelium; Female; Humans; Mandible; Mandibular Neoplasms; Mesoderm; Odontogenic Tumors; Odontoma
PubMed: 26045519
DOI: 10.1136/bcr-2015-209739 -
Orphanet Journal of Rare Diseases Mar 2006The otodental syndrome also named otodental dysplasia, is characterised by a striking dental phenotype known as globodontia, associated with sensorineural high frequency... (Review)
Review
The otodental syndrome also named otodental dysplasia, is characterised by a striking dental phenotype known as globodontia, associated with sensorineural high frequency hearing loss and eye coloboma. Globodontia occurs in both primary and permanent dentition, affecting canine and molar teeth (i.e. enlarged bulbous malformed posterior teeth with almost no discernable cusps or grooves). The condition appears to be inherited in an autosomal dominant mode, although sporadic cases have been reported. It is a rare disease, a few families have been described in the literature. In the British family, the locus for oculo-oto-dental syndrome was mapped to 20q13.1 within a 12-cM critical chromosomal region. Dental management is complex, interdisciplinary and will include regular follow up, scheduled teeth extraction and orthodontic treatment. Hearing checks and, if necessary, hearing aids are mandatory, as well as eye examination and ad hoc treatment if necessary.
Topics: Abnormalities, Multiple; Adult; Child; Coloboma; Cuspid; Dental Enamel Hypoplasia; Diagnosis, Differential; Face; Hearing Loss, Sensorineural; Humans; Malocclusion; Molar; Odontoma; Syndrome; Tooth Abnormalities
PubMed: 16722606
DOI: 10.1186/1750-1172-1-5 -
Journal of Oral and Maxillofacial... 2016The term odontoma has been used as a descriptor for any tumor of odontogenic origin. It is a growth in which both epithelial and mesenchymal cells exhibits complete...
The term odontoma has been used as a descriptor for any tumor of odontogenic origin. It is a growth in which both epithelial and mesenchymal cells exhibits complete differentiation. Odontomas are considered as hamartomas rather than true neoplasm. They are usually discovered on routine radiographic examination. Odontomas, according to the World Health Organization, are classified into complex odontoma and compound odontomas. The present paper reports a case of compound composite odontomas.
PubMed: 27194882
DOI: 10.4103/0973-029X.180982 -
International Journal of Surgery Case... 2014Ameloblastic fibro-odontoma (AFO) is a quite rare, mixed odontogenic tumour generally seen in the early stages of life. Frequent signs of this tumour are asymptomatic...
INTRODUCTION
Ameloblastic fibro-odontoma (AFO) is a quite rare, mixed odontogenic tumour generally seen in the early stages of life. Frequent signs of this tumour are asymptomatic swelling, delayed tooth eruption and mixed radiological appearance within well-defined borders. Management of the lesion includes enucleation of the tumour and long-term follow-up.
PRESENTATION OF CASE
A 10-year-old girl was referred to our oral and maxillofacial surgery clinic with an incidental radiological finding of radiopaque mass in the posterior region of maxilla. OPG showed unerupted tooth bud of upper right second molar and was being prevented from eruption by the odontome. Under general anaesthesia, the lesion was enucleated and the permanent right upper second molar tooth bud removed.
DISCUSSION
Mixed odontogenic tumours are a group of rare and interesting lesions which can mislead the clinician to variety of differential diagnosis. Adequate clinical and radiological investigations, proper surgical excison, accurate histopathological diagnosis, and long term follow up will ensure the right treatment plan for the patient.
CONCLUSION
The possibility of a mixed rare tumour should be kept in mind by the clinician where they deal with the swellings of posterior maxilla in children. Histological assessment revealed a final diagnosis of ameloblastic fibro-odontoma.
PubMed: 25437658
DOI: 10.1016/j.ijscr.2014.11.025