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 Table of Contents  
Year : 2017  |  Volume : 3  |  Issue : 2  |  Page : 88-90

Long-term effects of childhood management of retinoblastoma using chemo-radiotherapy on dental tissues: Literature review and case report

Department of Pedodontics and Preventive Dentistry, D.Y. Patil University, School of Dentistry, Nerul, Maharashtra, India

Date of Web Publication25-Jan-2018

Correspondence Address:
Dr. Uma B Dixit
Department of Pedodontics and Preventive Dentistry, D Y Patil University School of Dentistry, Nerul - 400 706, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijohr.ijohr_38_17

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Retinoblastoma is the most common intraocular neoplasm of childhood which is caused by a defective RB1 gene. It is detected early in life by clinical presentation including leukocoria, strabismus, glaucoma, poor visual tracking, and inflammation among others. Antineoplastic therapy that children with childhood malignancies are submitted to, have long-term effects on various tissues in the body. With timely treatment, the 5 years survival rate for retinoblastoma is reported to be 99%. Dental anomalies commonly seen in these children are characterized by disturbances in shape, size, number, and mineralization in permanent teeth. The purpose of this case report is to present long-term effects of chemotherapy and radiotherapy on dental development in a patient treated for retinoblastoma in childhood and discuss its pathogenesis. Early diagnosis of presence and severity of dental anomalies will help the dentist to plan and implement an appropriate course of dental treatment to make the survivor child's life as normal as possible.

Keywords: Chemotherapy, childhood cancer, dental anomalies, microdontia, radiotherapy, retinoblastoma

How to cite this article:
Dixit UB, Tekwani K, Raina S, Bhise Patil RS. Long-term effects of childhood management of retinoblastoma using chemo-radiotherapy on dental tissues: Literature review and case report. Indian J Oral Health Res 2017;3:88-90

How to cite this URL:
Dixit UB, Tekwani K, Raina S, Bhise Patil RS. Long-term effects of childhood management of retinoblastoma using chemo-radiotherapy on dental tissues: Literature review and case report. Indian J Oral Health Res [serial online] 2017 [cited 2024 Mar 5];3:88-90. Available from: https://www.ijohr.org/text.asp?2017/3/2/88/223931

  Introduction Top

Retinoblastoma is the most common intraocular neoplasm of childhood affecting children below the age of 5 years. The incidence of retinoblastoma is reported to be approximately 3–5 per million children per year in most developed countries and accounts for 6% of cancers in this age group.[1] In India, the incidence is reported to be 4–6 per million children; however, in some parts of the country like Chennai, the incidence is higher (12 in boys and 6 in girls).[2]

Retinoblastoma is caused by a defective RB1 gene of retinal neural cells which is either inherited by autosomal pattern or due to mutational changes. Clinical presentation includes leukocoria (white pupillary reflex), strabismus, glaucoma, poor visual tracking, and inflammation. It is fairly invasive and has potential to metastasize.

Diagnosis of retinoblastoma is usually based on clinical examination, indirect ophthalmoscopy and imaging studies. Treatment of retinoblastoma depends on the staging of the tumor and may include enucleation, chemotherapy, and radiotherapy.[3] Intra-arterial chemotherapy and focal, periocular injection of carboplatin have been tried with excellent success.

Five-year survival rate for retinoblastoma is reported to be 99%.[1] Although in India, survival rates are reported to be lower,[4] due to late diagnosis and suboptimal chemotherapy regimen used,[5] they are improving over the years.

Antineoplastic therapy used in children with childhood malignancies have long-term effects on various tissues in the body including dental tissues. The aim of this case report is to present long-term effects of chemotherapy and radiotherapy on dental development in a patient treated for retinoblastoma in childhood and discuss its pathogenesis.

  Case Report Top

An 11-year-old male child presented with the chief complaint of decay and pain in a lower left tooth. His medical history revealed a prior diagnosis of retinoblastoma of the left eye at the age of two and a half years. His nonsurgical treatment included a combination of external beam radiation therapy and chemotherapy. For external beam radiation therapy, a total radiation dose of 45 Gy in 25 fractions was delivered by single lateral port with 20 mev electron over a period of 2 months. This was followed by 12 sessions of chemotherapy using Carboplatin, Etonid, Vincristine, and Cyclosporine for 1 year. Transpupillary thermotherapy was performed twice every 6 months after chemotherapy. The patient was under regular follow-up thereafter and had remained cancer-free.

On clinical examination, no obvious abnormalities of the head and neck were detected. Intra-oral examination revealed late mixed dentition with grossly carious primary mandibular left second molar. Microdontia of the second premolars was evident [Figure 1] and [Figure 2]. Permanent second molars were in eruptive phase. Both the arches showed inter-dental spaces with Angle's Class I molar relationship. No developmental anomalies of enamel or dentin were observed.
Figure 1: Maxillary occlusal view showing microdontia of second premolars

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Figure 2: Mandibular occlusal view showing alteration of shape and size of erupted second premolar and erupting second molars

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Radiographic examination revealed the presence of all teeth except third molars [Figure 3]. Microdontia and altered shape of erupting permanent second molars was evident, mandibular showing more severe alterations than the maxillary. No abnormalities of roots were observed.
Figure 3: Panoramic view showing small, conical second premolars and second molars and absence of third molar buds

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Dental treatment consisted of extraction of the carious primary molar followed by preventive management and restorative treatment of carious permanent teeth. The patient was advised to seek orthodontic correction of spaced dentition at the appropriate time. He was kept under observation to evaluate the status of permanent second molars after eruption and development of the third molars.

  Discussion Top

Developmental dental anomalies observed in our patient can be characterized as alteration of size (microdontia) of second premolars and second permanent molars and shape (conical mandibular permanent second molars) along with delayed development or agenesis of all third molars. Dental developmental anomalies secondary to childhood antineoplastic therapy reported in the literature include microdontia, anodontia, alteration in shapes such as conical teeth and taurodontism, root shortening or blunting, disturbances of eruption and enamel or dentinal defects.[6],[7],[8]

Chemotherapeutic agents and radiotherapy targeted at neoplastic cells also affect metabolically active dental tissues which are in active development from birth till completion of the eruption. In our patient, the chemoradiotherapy coincided with the time when the second premolars begin calcification after the complete crown shape is established.[9] Permanent mandibular second molars, however, would be at an earlier stage of development leading to alteration in both their shape and size. This can be attributed to the effect of antineoplastic therapy on the enamel knots which are signaling centers in the tooth germ epithelium that determine the location and height of tooth cusps.[10]

Calcification of mandibular third molars begins around age 8–10 years [7] or later by 13 years.[11] Their absence in the panoramic radiograph at the age of 11 years might indicate their agenesis or delayed development.

Chemotherapeutic agents used in our patient for the treatment of retinoblastoma are known to affect odontogenesis. They may affect tooth development either through direct toxic effects on the odontogenic cells or by disturbing cell communication between epithelial and mesenchymal tissues during tooth development.[12],[13]

Radiotherapy dose >20 Gy is shown to have greater chances of affecting more than one dental tissues in a dose-dependent pattern.[14] Total radiation dose used in our patient was 45 Gy which might have contributed to the dental anomalies present in this patient.

  Conclusion Top

This case report demonstrates age-dependent effects of chemoradiotherapy on dental tissues. Careful clinical and radiographic examination at regular intervals will help in diagnosing the presence and severity of dental anomalies in children treated for childhood cancer so that appropriate course of dental treatment can be planned and implemented to make the survivor child's life as normal as possible.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

American Cancer Society. Cancer Facts and Figures: Special Section. Atlanta: American Cancer Society; 2014.  Back to cited text no. 1
National Cancer Registry Programme. Three-year Report of the Population Based Cancer Registries 2009-2011. Indian Council of Medical Research, New Delhi: National Cancer Registry Programme; 2013.  Back to cited text no. 2
Hurwitz RL, Shields CL, Shields JA, Chévez-Barrios P, Gombos D, Hurwitz MY. Retinoblastoma. In: Pizzo PA, Poplack DG, editors. Principles and Practice of Pediatric Oncology. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2010. p. 809-37.  Back to cited text no. 3
Arora RS, Eden TO, Kapoor G. Epidemiology of childhood cancer in India. Indian J Cancer 2009;46:264-73.  Back to cited text no. 4
[PUBMED]  [Full text]  
Sahu S, Banavali SD, Pai SK, Nair CN, Kurkure PA, Motwani SA, et al. Retinoblastoma: Problems and perspectives from India. Pediatr Hematol Oncol 1998;15:501-08.  Back to cited text no. 5
Remmers D, Bokkerink JP, Katsaros C. Microdontia after chemotherapy in a child treated for neuroblastoma. Orthod. Craniofac Res 2006;9:206-10.  Back to cited text no. 6
Kaste SC, Hopkins KP, Bowman LC, Santana VM. Dental abnormalities in children treated for neuroblastoma. Med Pediatr Oncol 1998;30:22-7.  Back to cited text no. 7
Venkataraghavan K, Patil S, Guvva S, Karthik S, Bhandi S. Abnormal odontogenesis following management of childhood cancer (retinoblastoma): Review and a new variant. J Contemp Dent Pract 2013;14:360-4.  Back to cited text no. 8
Logan WH, Kronfeld R. Development of the human jaws and surrounding structures from birth to the age of fifteen years. J Am Dent Assoc 1933;20:379-427.  Back to cited text no. 9
Jernvall J, Keranen SV, Thesleff I. Evolutionary modification of development in mammalian teeth: Quantifying gene expression patterns and topography. Proc Natl Acad Sci USA 2000;97:14444-8.  Back to cited text no. 10
Hegde S, Patodia A, Dixit U. Staging of third molar development in relation to chronological age of 5-16 year old Indian children. Forensic Science International 2016;269:63-9.  Back to cited text no. 11
Holtta P, Alaluusua S, Saarinen-Pihkala UM, Wolf J, Nystro MM, Hovi L. Long-term adverse effects on dentition in children with poor-risk neuroblastoma treated with high-dose chemotherapy and autologous stem cell transplantation with or without total body irradiation. Bone Marrow Transplant 2002;29:121-7.  Back to cited text no. 12
Hölttä P, Alaluusua S, Saarinen-Pihkala UM, Peltola J, Hovi L. Agenesis and microdontia of permanent teeth as late adverse effects after stem cell transplantation in young children. Cancer 2005;103:181-90.  Back to cited text no. 13
Kaste SC, Goodman P, Leisenring W, Stovall M, Hayashi RJ, Yeazel M, et al. Impact of radiation and chemotherapy on risk of dental abnormalities: A report from the childhood cancer survivor study. Cancer 2009;115:5817-27.  Back to cited text no. 14


  [Figure 1], [Figure 2], [Figure 3]


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