- Email: [email protected]
Pediatric case of squamous cell carcinoma arising from a keratocystic odontogenic tumor
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Pediatric case of squamous cell carcinoma arising from a keratocystic odontogenic tumor
T
Lara Nokovitcha, Anne-Gaelle Bodarda, Nadège Corradinib, Carole Crozesc, Aurélie Guyennond, Sophie Deneuvea,∗ a
Oncologic Surgery Department, Centre Léon Bérard, Lyon, France Pediatric Oncology Department, Centre Léon Bérard, Lyon, France c Pathology Laboratory Department, Centre Léon Bérard, Lyon, France d Radiology Department, Centre Léon Bérard, Lyon, France b
A R T I C LE I N FO
A B S T R A C T
Keywords: Case report Pediatric Squamous cell carcinoma (SCC) Keratocystic odontogenic tumor (KCOT)
Keratocystic odontogenic tumors (KCOT) are exceptional in children and adolescents as they usually occur in the third decade. The present study reports the case of a 15 years old girl who was diagnosed with a KCOT that underwent malignant transformation. KCOT diagnostic was based on clinical, radiological, histopathological and immunohistochemical findings. A conservative treatment by enucleation was performed. Histopathological analysis of the surgical specimen concluded to a KCOT, with an infra-centimetric focus of well-differentiated squamous cell carcinoma. Owing to the well-differentiated character of the squamous cell carcinoma, a single clinical and MRI surveillance every 3 months was decided, without complementary treatment.
1. Introduction Keratocystic odontogenic tumors (KCOTs) are exceptional in children and adolescents as they usually occur in the third decade of life [1]. It is defined as « a benign uni- or multicystic intraosseous tumor of odontogenic origin, with a potentially local aggressive and infiltrative behavior » [2]. Owing to its destructive nature and high recurrence rate, it was reclassified by the World Health Organization (WHO) in 2005 as a benign neoplasm of odontogenic origin, and not as a cyst [3]. KCOTs represent 14.5% of odontogenic lesions in the pediatric population [4], with a male preponderance, and a mean age of occurrence of 14.7 years old [5] in pediatric series. They can be sporadic, or syndromic, associated with nevoid basal cell carcinoma syndrome. Various authors described cases of squamous cell carcinoma arising from a KCOT in adults [6–10], but the exact incidence of KCOT malignant transformation is not known, and believed to be rare [2]. The present study reports the case of a 15-year-old girl who was diagnosed with a KCOT that underwent malignant transformation, forming a well-differentiated squamous cell carcinoma of the mandible. 2. Case presentation A 15-year-old female with no medical history was referred to the Head and Neck Surgery Department of the Léon Bérard Center in Lyon
∗
(France), for a cystic lesion of the right mandible developed over an included supernumerary tooth. The lesion was described by her parents as rapidly increasing in size over a period of two months, and was associated with pain and intermittent bleeding. Clinical examination revealed a right irregular mandibular mass extending from teeth numbers 42 to 47 that was tender to the touch. Dental occlusion seemed to be modified. There was no lower lip numbness, and no significantly enlarged submandibular or cervical lymph nodes on palpation. A dental CT scan showed a cystic lesion with root resorption. Although dental CT scan findings were in favor of a benign lesion, as a malignant tumor was suspected, owing to the assumed rapid growth of the tumor, clinical staging was completed through a CT scan, an MRI, and a PET-CT scan. The CT scan revealed a tissular mass 36 × 20 × 28mm in size that developed over an included supernumerary tooth, lysing the lingual cortex of the right posterior mandible, with heterogeneous enhancement after contrast material injection (Fig. 1A and B). The lesion was hyperintense on T2-weighted MRI, hypointense on T1-weighted MRI, and infiltrated the medulla of the right mandible over 4 cm (Fig. 2). It was intensely hypermetabolic on the PET-CT scan, without locoregional lymph node or distant metastatic fixation (Fig. 3). Two surgical biopsies were carried out. Microscopic examination of the tissue revealed a cystic lesion lined by hyperplastic pseudo-
Corresponding author. Oncologic surgery department, Léon Bérard Center, 28 Rue Laennec, 69008, Lyon, France. E-mail address: [email protected] (S. Deneuve).
https://doi.org/10.1016/j.ijporl.2018.06.042 Received 17 April 2018; Received in revised form 19 June 2018; Accepted 21 June 2018 Available online 30 June 2018 0165-5876/ © 2018 Elsevier B.V. All rights reserved.
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 1. A) Included supernumerary tooth on a dental CT scan. B) Lysis of the lingual cortex of the right posterior mandible on a transverse CT scan.
Testing for the Gorlin-Goltz syndrome gave a negative result based on familial/clinical history and a blood sample. MRI was done one month post-operation, showing no sign of recurrence. Owing to the well-differentiated character of the squamous cell carcinoma, and its central character with large margins of cancer-free tissue, a single clinical and MRI surveillance every 3 months was decided, without complementary treatment. To date, no local or cervical recurrence of the squamous cell carcinoma was observed, with a retrospective time perspective set at 18 months after the surgery.
epitheliomatous stratified squamous epithelium in a few areas, features suggestive of a form of dysplasia such as nuclear hyperchromatism and pleomorphism. Immunohistochemical analysis indicated the presence of Ki-67-positive cells in the basal and suprabasal cell layers of the tissue samples. 60% of epithelial cells presented a nuclear and cytoplasmic expression of p16, without significant p53 expression. In situ DNA hybridization for HPV oncogene detection was negative. A diagnosis of KCOT was made. An enucleation was decided, along with the avulsion of the supernumerary tooth, and teeth 44, 45 and 46 (Fig. 4A and B). Histopathological analysis of the right mandible lesion indicated a KCOT, with an infra-centimetric focus on well-differentiated squamous cell carcinoma in the middle of the cyst (Fig. 5A–C).
3. Discussion The presumed rapid growth of the tumor led us to suspect a more
122
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 2. T2-weighted images showing a hyperintense lesion of the right mandible infiltrating the medulla.
aggressive etiology prima facie and justified the repetition of the biopy. However, after careful review of this case, the existence of preexisting dental occlusion modifications, the observed root resorption, and complex family circumstances suggested that this lesion developed over a longer period of time. Chronic inflammation might therefore have contributed to KCOT malignant transformation. In the case of KCOT diagnosed in a child, the possibility of GorlinGoltz syndrome should systematically be discussed, since syndromic variants of KCOTs usually occur at a younger age [2,13]. Kadlub et al. also reported that 80% of children with KCOTs displayed a PTCH1 germinal mutation [1], a tumor suppressor gene involved in nevoid basal cell carcinoma syndrome. The pathogenesis of KCOT malignant transformation is largely unknown. Carcinogenesis induced by chronic inflammation has been mentioned [9]. The role of PTCH mutation in the case of KCOT transformed into squamous cell carcinoma has yet to be explored. Radiological characteristics of KCOTs usually take the form of a cystic, lytic lesion involving the posterior mandible and ascending ramus, usually with a dental connection [1,14]. Root resorption is possible but rare. CT images on bone window settings ordinarily display most of the features seen on the panoramic radiographs, such as expansion and displacement of the unerupted teeth. On soft tissue windows settings, KCOTs appear hypodense to isodense compared to muscle. MRI features show low to intermediate signal intensity on T1weighted images, and high signal intensity on T2-weighted images, with a characteristic signal inhomogeneity or « signal drop out » [15]. Histopathological findings include a parakeratinised stratified squamous cell epithelium lining the cystic lumen, with hyperchromatic nuclei oriented away from the basement membrane, this last feature being characteristic of KCOTs [2]. Immunohistochemical analysis usually exhibit high Ki-67, CD34 and podoplanin expression levels, with KCOTs presenting a higher proliferation rate than other odontogenic cysts [16].
Fig. 3. PET-CT scan showing an intensely hypermetabolic lesion of the right mandible, without locoregional lymph node or distant metastatic fixation.
Conservative management in young patients is the norm, and enucleation combined with peripheral ostectomy is usually performed. However, in the case of large lesions with multiple perforations of the bony cortices, en bloc resection can be performed [17]. Recurrence rate is high, and regular follow-ups and surveillance at least once a year for 5 years after the initial treatment is recommended [2,18]. It has been reported that KCOTs may undergo malignant transformation into squamous cell carcinoma (SCC) in adults, with an estimated incidence of odontogenic cyst malignant transformation of 0.12% [2]. Also known as primary intraosseous squamous cell carcinoma (PIOSCC), these lesions usually arise within the jawbones from the odontogenic epithelium and have no initial connection to the oral mucosa [3]. Up to now, the total number of reported cases is about 30
123
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 4. A) Pre-operative view of the lesion. B) Surgical specimen and supernumerary tooth.
[9,19], with a mean age of occurrence of 60 years old, and a higher prevalence in men. Recommended treatment combines surgery, sometimes associated with neck dissection, and radio (chemo)therapy. The survival rate relative to when this tumor arises in adults is 62% at 2 years and 38% at 5 years [9]. In order to estimate the incidence of KCOT malignant transformation in the pediatric population, a systematic review of the literature using the MESH terms “Carcinoma, Squamous cell” AND “Odontogenic cyst” was performed. Out of 160 articles, there were only 5 reported cases of PIOSCC arising from an odontogenic cyst in children/adolescents [9,11,12,20]. Among those 5 pediatric clinical presentations, the histopathology of the odontogenic cyst was not specified in 2 cases [9], corresponded to a dentigerous cyst in 2 cases [11,12], and to a real KCOT in only 1 case [20]. This case involved an adolescent girl who presented a KCOT of the maxillary that underwent malignant transformation at the age of 18, a period of life when the spectrum of malignant tumors begins to be enriched by histological categories of adult type.
Fig. 5. A) Infracentimetric focus of well-differentiated squamous cell carcinoma in the middle of a KCOT. The cystic lesion is lined by a hyperplastic pseudoepitheliomatous stratified squamous epithelium in a few areas, which suggests a form of dysplasia such as nuclear hyperchromatism and pleomorphism. B) Squamous cell carcinoma focus (magnification 5). Carcinoma focus containing sheets of eosinophil cells, with dystrophic inflammatory stroma and rupture of the basement membrane. C) Ki67 immunohistochemical analysis of the squamous cell carcinoma, showing a disrupted architecture with irregular nuclei.
4. Conclusion KCOT malignant transformation is extremely rare in the pediatric and adolescent population. In the present case, which is the youngest reported, the diagnosis was based on clinical, radiological, histopathological and immunohistochemical findings. A conservative treatment by enucleation was performed, followed by regular MRI surveillance, owing to the small size and well-differentiated and central character of the squamous cell carcinoma.
Financial disclosure and products page None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.
124
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
References [11]
[1] N. Kadlub, T. Kreindel, V. Belle Mbou, A. Coudert, E. Ansari, V. Descroix, et al., Specificity of paediatric jawbone lesions: tumours and pseudotumours, J. CranioMaxillo-Fac. Surg. 42 (2014) 125–131. [2] D. Bhargava, A. Deshpande, M.A. Pogrel, Keratocystic odontogenic tumour (KCOT)–a cyst to a tumour, Oral Maxillofac. Surg. 16 (2012) 163–170. [3] L. Thompson, World Health Organization classification of tumours: pathology and genetics of head and neck tumours, Ear Nose Throat J. 85 (2006) 74. [4] Q.-G. Fang, S. Shi, C.-F. Sun, Odontogenic lesions in pediatric patients, J. Craniofac. Surg. 25 (2014) e248–251. [5] N. Li, X. Gao, Z. Xu, Z. Chen, L. Zhu, J. Wang, et al., Prevalence of developmental odontogenic cysts in children and adolescents with emphasis on dentigerous cyst and odontogenic keratocyst (keratocystic odontogenic tumor), Acta Odontol. Scand. 72 (2014) 795–800. [6] R.M.S.H.B. Medawela, N.S.S. Jayasuriya, D.R.D.L. Ratnayake, A.M. Attygalla, B.S.M.S. Siriwardena, Squamous cell carcinoma arising from a keratocystic odontogenic tumor: a case report, J. Med. Case Rep. 11 (2017) 335. [7] M.-R. Bai, T. Shen, Y.U. Chen, N. Geng, Primary intraosseous squamous cell carcinoma in pre-existing keratocystic odontogenic tumor: a case report and literature review, Mol. Clin. Oncol. 4 (2016) 187–190. [8] O.M. Lukandu, C.S. Micha, Primary intraosseous squamous cell carcinoma arising from keratocystic odontogenic tumor, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 120 (2015) e204–e209. [9] L. Bodner, E. Manor, M. Shear, I. van der Waal, Primary intraosseous squamous cell carcinoma arising in an odontogenic cyst: a clinicopathologic analysis of 116 reported cases, J. Oral Pathol. Med. 40 (2011) 733–738. [10] H. Matsuzaki, N. Katase, T. Matsumura, M. Hara, Y. Yanagi, H. Nagatsuka, et al., Solid-type primary intraosseous squamous cell carcinoma of the mandible: a case
[12]
[13] [14] [15]
[16]
[17] [18] [19]
[20]
125
report with histopathological and imaging features, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 114 (2012) e71–77. M. Charles, T. Barr, I. Leong, B.Y. Ngan, V. Forte, G.K.B. Sándor, Primary intraosseous malignancy originating in an odontogenic cyst in a young child, J. Oral Maxillofac. Surg. 66 (2008) 813–819. S.H. Gulbranson, J.D. Wolfrey, J.M. Raines, B.P. McNally, Squamous cell carcinoma arising in a dentigerous cyst in a 16-month-old girl, Otolaryngol. Head Neck Surg. 127 (2002) 463–464. A.H. Kamil, B. Tarakji, Odontogenic keratocyst in children: a review, Open Dent. J. 10 (2016) 117–123. J.M. Abrahams, S.A. McClure, Pediatric odontogenic tumors, Oral Maxillofac. Surg. Clin. 28 (2016) 45–58. L.J. van Rensburg, M. Paquette, J.A. Morkel, C.J. Nortjé, Correlative MRI and CT imaging of the odontogenic keratocyst: a review of twenty-one cases, Oral Maxillofac. Surg. Clin. 15 (2003) 363–382. T. Naruse, K. Yamashita, S. Yanamoto, S. Rokutanda, Y. Matsushita, Y. Sakamoto, et al., Histopathological and immunohistochemical study in keratocystic odontogenic tumors: predictive factors of recurrence, Oncol. Lett. 13 (2017) 3487–3493. J.M. Abrahams, S.A. McClure, Pediatric odontogenic tumors, Oral Maxillofac. Surg. Clin. 28 (2016) 45–58. B.E. Kinard, S.-K. Chuang, M. August, T.B. Dodson, How well do we manage the odontogenic keratocyst? J. Oral Maxillofac. Surg. 71 (2013) 1353–1358. S. Acharya, A.S. Tayaar, K. Hallkeri, S. Adirajaiah, K. Gopalkrishnan, Squamous cell carcinoma emerging in an orthokeratinized odontogenic cyst: a case report and brief review, J. Oral Maxillofac. Surg. Med. Pathol. 26 (2014) 563–568. F. Falaki, Z. Delavarian, J. Salehinejad, S. Saghafi, Squamous cell carcinoma arising from an odontogenic keratocyst: a case report, Med. Oral Patol. Oral Cir. Bucal 14 (2009) E171–E174.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Pediatric case of squamous cell carcinoma arising from a keratocystic odontogenic tumor
T
Lara Nokovitcha, Anne-Gaelle Bodarda, Nadège Corradinib, Carole Crozesc, Aurélie Guyennond, Sophie Deneuvea,∗ a
Oncologic Surgery Department, Centre Léon Bérard, Lyon, France Pediatric Oncology Department, Centre Léon Bérard, Lyon, France c Pathology Laboratory Department, Centre Léon Bérard, Lyon, France d Radiology Department, Centre Léon Bérard, Lyon, France b
A R T I C LE I N FO
A B S T R A C T
Keywords: Case report Pediatric Squamous cell carcinoma (SCC) Keratocystic odontogenic tumor (KCOT)
Keratocystic odontogenic tumors (KCOT) are exceptional in children and adolescents as they usually occur in the third decade. The present study reports the case of a 15 years old girl who was diagnosed with a KCOT that underwent malignant transformation. KCOT diagnostic was based on clinical, radiological, histopathological and immunohistochemical findings. A conservative treatment by enucleation was performed. Histopathological analysis of the surgical specimen concluded to a KCOT, with an infra-centimetric focus of well-differentiated squamous cell carcinoma. Owing to the well-differentiated character of the squamous cell carcinoma, a single clinical and MRI surveillance every 3 months was decided, without complementary treatment.
1. Introduction Keratocystic odontogenic tumors (KCOTs) are exceptional in children and adolescents as they usually occur in the third decade of life [1]. It is defined as « a benign uni- or multicystic intraosseous tumor of odontogenic origin, with a potentially local aggressive and infiltrative behavior » [2]. Owing to its destructive nature and high recurrence rate, it was reclassified by the World Health Organization (WHO) in 2005 as a benign neoplasm of odontogenic origin, and not as a cyst [3]. KCOTs represent 14.5% of odontogenic lesions in the pediatric population [4], with a male preponderance, and a mean age of occurrence of 14.7 years old [5] in pediatric series. They can be sporadic, or syndromic, associated with nevoid basal cell carcinoma syndrome. Various authors described cases of squamous cell carcinoma arising from a KCOT in adults [6–10], but the exact incidence of KCOT malignant transformation is not known, and believed to be rare [2]. The present study reports the case of a 15-year-old girl who was diagnosed with a KCOT that underwent malignant transformation, forming a well-differentiated squamous cell carcinoma of the mandible. 2. Case presentation A 15-year-old female with no medical history was referred to the Head and Neck Surgery Department of the Léon Bérard Center in Lyon
∗
(France), for a cystic lesion of the right mandible developed over an included supernumerary tooth. The lesion was described by her parents as rapidly increasing in size over a period of two months, and was associated with pain and intermittent bleeding. Clinical examination revealed a right irregular mandibular mass extending from teeth numbers 42 to 47 that was tender to the touch. Dental occlusion seemed to be modified. There was no lower lip numbness, and no significantly enlarged submandibular or cervical lymph nodes on palpation. A dental CT scan showed a cystic lesion with root resorption. Although dental CT scan findings were in favor of a benign lesion, as a malignant tumor was suspected, owing to the assumed rapid growth of the tumor, clinical staging was completed through a CT scan, an MRI, and a PET-CT scan. The CT scan revealed a tissular mass 36 × 20 × 28mm in size that developed over an included supernumerary tooth, lysing the lingual cortex of the right posterior mandible, with heterogeneous enhancement after contrast material injection (Fig. 1A and B). The lesion was hyperintense on T2-weighted MRI, hypointense on T1-weighted MRI, and infiltrated the medulla of the right mandible over 4 cm (Fig. 2). It was intensely hypermetabolic on the PET-CT scan, without locoregional lymph node or distant metastatic fixation (Fig. 3). Two surgical biopsies were carried out. Microscopic examination of the tissue revealed a cystic lesion lined by hyperplastic pseudo-
Corresponding author. Oncologic surgery department, Léon Bérard Center, 28 Rue Laennec, 69008, Lyon, France. E-mail address: [email protected] (S. Deneuve).
https://doi.org/10.1016/j.ijporl.2018.06.042 Received 17 April 2018; Received in revised form 19 June 2018; Accepted 21 June 2018 Available online 30 June 2018 0165-5876/ © 2018 Elsevier B.V. All rights reserved.
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 1. A) Included supernumerary tooth on a dental CT scan. B) Lysis of the lingual cortex of the right posterior mandible on a transverse CT scan.
Testing for the Gorlin-Goltz syndrome gave a negative result based on familial/clinical history and a blood sample. MRI was done one month post-operation, showing no sign of recurrence. Owing to the well-differentiated character of the squamous cell carcinoma, and its central character with large margins of cancer-free tissue, a single clinical and MRI surveillance every 3 months was decided, without complementary treatment. To date, no local or cervical recurrence of the squamous cell carcinoma was observed, with a retrospective time perspective set at 18 months after the surgery.
epitheliomatous stratified squamous epithelium in a few areas, features suggestive of a form of dysplasia such as nuclear hyperchromatism and pleomorphism. Immunohistochemical analysis indicated the presence of Ki-67-positive cells in the basal and suprabasal cell layers of the tissue samples. 60% of epithelial cells presented a nuclear and cytoplasmic expression of p16, without significant p53 expression. In situ DNA hybridization for HPV oncogene detection was negative. A diagnosis of KCOT was made. An enucleation was decided, along with the avulsion of the supernumerary tooth, and teeth 44, 45 and 46 (Fig. 4A and B). Histopathological analysis of the right mandible lesion indicated a KCOT, with an infra-centimetric focus on well-differentiated squamous cell carcinoma in the middle of the cyst (Fig. 5A–C).
3. Discussion The presumed rapid growth of the tumor led us to suspect a more
122
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 2. T2-weighted images showing a hyperintense lesion of the right mandible infiltrating the medulla.
aggressive etiology prima facie and justified the repetition of the biopy. However, after careful review of this case, the existence of preexisting dental occlusion modifications, the observed root resorption, and complex family circumstances suggested that this lesion developed over a longer period of time. Chronic inflammation might therefore have contributed to KCOT malignant transformation. In the case of KCOT diagnosed in a child, the possibility of GorlinGoltz syndrome should systematically be discussed, since syndromic variants of KCOTs usually occur at a younger age [2,13]. Kadlub et al. also reported that 80% of children with KCOTs displayed a PTCH1 germinal mutation [1], a tumor suppressor gene involved in nevoid basal cell carcinoma syndrome. The pathogenesis of KCOT malignant transformation is largely unknown. Carcinogenesis induced by chronic inflammation has been mentioned [9]. The role of PTCH mutation in the case of KCOT transformed into squamous cell carcinoma has yet to be explored. Radiological characteristics of KCOTs usually take the form of a cystic, lytic lesion involving the posterior mandible and ascending ramus, usually with a dental connection [1,14]. Root resorption is possible but rare. CT images on bone window settings ordinarily display most of the features seen on the panoramic radiographs, such as expansion and displacement of the unerupted teeth. On soft tissue windows settings, KCOTs appear hypodense to isodense compared to muscle. MRI features show low to intermediate signal intensity on T1weighted images, and high signal intensity on T2-weighted images, with a characteristic signal inhomogeneity or « signal drop out » [15]. Histopathological findings include a parakeratinised stratified squamous cell epithelium lining the cystic lumen, with hyperchromatic nuclei oriented away from the basement membrane, this last feature being characteristic of KCOTs [2]. Immunohistochemical analysis usually exhibit high Ki-67, CD34 and podoplanin expression levels, with KCOTs presenting a higher proliferation rate than other odontogenic cysts [16].
Fig. 3. PET-CT scan showing an intensely hypermetabolic lesion of the right mandible, without locoregional lymph node or distant metastatic fixation.
Conservative management in young patients is the norm, and enucleation combined with peripheral ostectomy is usually performed. However, in the case of large lesions with multiple perforations of the bony cortices, en bloc resection can be performed [17]. Recurrence rate is high, and regular follow-ups and surveillance at least once a year for 5 years after the initial treatment is recommended [2,18]. It has been reported that KCOTs may undergo malignant transformation into squamous cell carcinoma (SCC) in adults, with an estimated incidence of odontogenic cyst malignant transformation of 0.12% [2]. Also known as primary intraosseous squamous cell carcinoma (PIOSCC), these lesions usually arise within the jawbones from the odontogenic epithelium and have no initial connection to the oral mucosa [3]. Up to now, the total number of reported cases is about 30
123
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
Fig. 4. A) Pre-operative view of the lesion. B) Surgical specimen and supernumerary tooth.
[9,19], with a mean age of occurrence of 60 years old, and a higher prevalence in men. Recommended treatment combines surgery, sometimes associated with neck dissection, and radio (chemo)therapy. The survival rate relative to when this tumor arises in adults is 62% at 2 years and 38% at 5 years [9]. In order to estimate the incidence of KCOT malignant transformation in the pediatric population, a systematic review of the literature using the MESH terms “Carcinoma, Squamous cell” AND “Odontogenic cyst” was performed. Out of 160 articles, there were only 5 reported cases of PIOSCC arising from an odontogenic cyst in children/adolescents [9,11,12,20]. Among those 5 pediatric clinical presentations, the histopathology of the odontogenic cyst was not specified in 2 cases [9], corresponded to a dentigerous cyst in 2 cases [11,12], and to a real KCOT in only 1 case [20]. This case involved an adolescent girl who presented a KCOT of the maxillary that underwent malignant transformation at the age of 18, a period of life when the spectrum of malignant tumors begins to be enriched by histological categories of adult type.
Fig. 5. A) Infracentimetric focus of well-differentiated squamous cell carcinoma in the middle of a KCOT. The cystic lesion is lined by a hyperplastic pseudoepitheliomatous stratified squamous epithelium in a few areas, which suggests a form of dysplasia such as nuclear hyperchromatism and pleomorphism. B) Squamous cell carcinoma focus (magnification 5). Carcinoma focus containing sheets of eosinophil cells, with dystrophic inflammatory stroma and rupture of the basement membrane. C) Ki67 immunohistochemical analysis of the squamous cell carcinoma, showing a disrupted architecture with irregular nuclei.
4. Conclusion KCOT malignant transformation is extremely rare in the pediatric and adolescent population. In the present case, which is the youngest reported, the diagnosis was based on clinical, radiological, histopathological and immunohistochemical findings. A conservative treatment by enucleation was performed, followed by regular MRI surveillance, owing to the small size and well-differentiated and central character of the squamous cell carcinoma.
Financial disclosure and products page None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.
124
International Journal of Pediatric Otorhinolaryngology 112 (2018) 121–125
L. Nokovitch et al.
References [11]
[1] N. Kadlub, T. Kreindel, V. Belle Mbou, A. Coudert, E. Ansari, V. Descroix, et al., Specificity of paediatric jawbone lesions: tumours and pseudotumours, J. CranioMaxillo-Fac. Surg. 42 (2014) 125–131. [2] D. Bhargava, A. Deshpande, M.A. Pogrel, Keratocystic odontogenic tumour (KCOT)–a cyst to a tumour, Oral Maxillofac. Surg. 16 (2012) 163–170. [3] L. Thompson, World Health Organization classification of tumours: pathology and genetics of head and neck tumours, Ear Nose Throat J. 85 (2006) 74. [4] Q.-G. Fang, S. Shi, C.-F. Sun, Odontogenic lesions in pediatric patients, J. Craniofac. Surg. 25 (2014) e248–251. [5] N. Li, X. Gao, Z. Xu, Z. Chen, L. Zhu, J. Wang, et al., Prevalence of developmental odontogenic cysts in children and adolescents with emphasis on dentigerous cyst and odontogenic keratocyst (keratocystic odontogenic tumor), Acta Odontol. Scand. 72 (2014) 795–800. [6] R.M.S.H.B. Medawela, N.S.S. Jayasuriya, D.R.D.L. Ratnayake, A.M. Attygalla, B.S.M.S. Siriwardena, Squamous cell carcinoma arising from a keratocystic odontogenic tumor: a case report, J. Med. Case Rep. 11 (2017) 335. [7] M.-R. Bai, T. Shen, Y.U. Chen, N. Geng, Primary intraosseous squamous cell carcinoma in pre-existing keratocystic odontogenic tumor: a case report and literature review, Mol. Clin. Oncol. 4 (2016) 187–190. [8] O.M. Lukandu, C.S. Micha, Primary intraosseous squamous cell carcinoma arising from keratocystic odontogenic tumor, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 120 (2015) e204–e209. [9] L. Bodner, E. Manor, M. Shear, I. van der Waal, Primary intraosseous squamous cell carcinoma arising in an odontogenic cyst: a clinicopathologic analysis of 116 reported cases, J. Oral Pathol. Med. 40 (2011) 733–738. [10] H. Matsuzaki, N. Katase, T. Matsumura, M. Hara, Y. Yanagi, H. Nagatsuka, et al., Solid-type primary intraosseous squamous cell carcinoma of the mandible: a case
[12]
[13] [14] [15]
[16]
[17] [18] [19]
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