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Odontogenic myxoma: Diagnostic and therapeutic challenges in paediatric and adult patients – A case series and review of the literature
Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
Case report
Odontogenic myxoma: Diagnostic and therapeutic challenges in paediatric and adult patients e A case series and review of the literature Katinka Kansy a, b, c, *, Philipp Juergens a, b, Zdzislav Krol a, Michael Paulussen d, Daniel Baumhoer e, Elisabeth Bruder e, Jacques Schneider f, Hans-Florian Zeilhofer a, b, Katja Schwenzer-Zimmerer a, b a
Deptartment of Cranio-Maxillofacial Surgery (Head: Prof. Hans-Florian Zeilhofer), University Hospital Basel, Spitalstr. 21, CH-4054 Basel, Switzerland High-tech Research Center of Cranio-Maxillofacial Surgery, University Hospital Basel, Switzerland Deptartment of Cranio-Maxillofacial Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 400, D-69120 Heidelberg, Germany d Deptartment of Oncology, Children’s Hospital Basel, Switzerland e Institute of Pathology, University Hospital Basel, Switzerland f Deptartment of Radiology, Children’s University Hospital Basel, Switzerland b c
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 10 January 2011 Accepted 19 April 2011
Introduction: Odontogenic myxomas are benign but locally invasive tumours originating from primordial mesenchymal tooth forming tissues which do not metastasise. We present a series of two paediatric and two adult cases and focus on differences in diagnostic and therapeutic approaches between children and adults based on our own experience and a critical review of the literature. Ó 2011 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Odontogenic myxoma Children
1. Introduction Odontogenic myxomas are benign but locally invasive tumours originating from primordial mesenchymal tooth forming tissues which do not metastasise. They are rare tumours and account for 3.3e15.7% of all odontogenic tumours in adults (El-Ghehani et al., 2009, Araki et al., 2007, Jing et al., 2007, Buchner et al., 2006, Olgac et al., 2006, Adebayo et al., 2005, Simon et al., 2005, Ladeinde et al., 2005, Tamme et al., 2004, Ochsenius et al., 2002) and for 8.5e11.6% in children (Mortellaro et al., 2008, Guerrisi et al., 2007). They occur in all age groups with a peak incidence in the third decade. Odontogenic myxomas can be found in both the maxilla and mandible, usually associated with a tooth germ. On imaging, odontogenic myxomas mostly present as radiolucent, multilocular structures on X-ray and CT scan. Intermediate signal intensity on T1 and high signal intensity on T2 are characteristic MRI findings with good demonstration of the extent of any resorbtion. Both CT and MRI correlate well with the histological features and are considered useful tools for diagnosis (Kawai et al., 1997, Asaumi et al., 2001, Sumi et al., 2000, MacDonald-Jankowski et al., 2004). One study has shown a beneficial effect of DVT
imaging for the diagnosis of the internal structure of the lesion and the condition of margins (Araki et al., 2007). Diagnosis is most commonly established by biopsy. Histopathological features include a whitish-grey glistening or gelatinous mass with minimal true encapsulation macroscopically and spindled or stellate-shaped cells in a mucoid-rich intracellular matrix microscopically (Martínez-Mata et al., 2008; Li et al., 2006). Immunocytochemical staining is positive for vimentin, but in contrast to myosarcomas is negative for desmin, neuron specific enolase, glial fibrillary acid protein and S100 (Martínez-Mata et al., 2008, Li et al., 2006). Management varies depending on the location and size of the tumour, the age of the patient and individual experience. They range from minimally invasive excisional biopsies to an en bloc resection of a multitude of relevant structures. Long-term follow-up is crucial as myxomas have a significant tendency to reccur. Recurrence rates for myxomas in the literature vary from 5 to 10% but there is no reliable data in the existing literature.
2. Case reports * Corresponding author. Department of Cranio-Maxillofacial Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 400, D-69120 Heidelberg, Germany. Tel.: þ49 6221 5636119; fax: þ49 6221 4375. E-mail address: [email protected] (K. Kansy).
2.1. Case 1 A 12-month-old child was referred with a four-day history of a painless left paranasal swelling and increased tearing for several
1010-5182/$ e see front matter Ó 2011 European Association for Cranio-Maxillo-Facial Surgery. doi:10.1016/j.jcms.2011.04.009
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K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
weeks. Physical examination revealed a fixed tumour of 1.5 cm in diameter. The lesion was firm, non-tender and did not enlarge with crying. Eye position and extra ocular motion were normal. Clinical examination otherwise was normal. An axial computed tomogram (CT) of the head revealed a welldefined intraosseous expansile mass involving the inferior, anterior and medial wall of the left maxillary sinus and the left infraorbital rim. The tumour reached the germ of the left permanent canine and compressed the left nasolacrimal duct without invading it. Magnetic resonance imaging (MRI) revealed a well-defined mass, 16 mm in diameter, with an intermediate intensity signal on T1- and a high intensity signal on T2-weighted image. An intraoperative fast frozen section was performed giving the preliminary diagnosis of a myxoid tumour, probably low grade. Subsequently, enucleation of the tumour was performed with curettage of the surrounding bone through a vestibular incision. Immediate postoperative MRI showed complete removal of the lesion. The patient recovered uneventfully from surgery and no scar was visible at follow-up. Final histology revealed a myxoid tumour of 1.6 cm in diameter with haphazardly arranged stellate to spindle-shaped cells with marked eosinophlic cytoplasm in a mucoid-rich intercellular matrix. Immunohistochemical staining was positive for vimentin and also for beta-catenin cytoplasmatically with a proliferation index of 5% in the MIB-1 reaction. The tumour stained negatively for Alk-1, CD31, CD34, CD68, calponin, desmin, myogenin, SMA and S-100. The final diagnosis was odontogenic myxoma. The patient showed no signs of recurrence at six months follow-up visit. An interdisciplinary team of experts decided on the following followup management: clinical examination one-monthly for the first year, two-monthly for the second year and a three-monthly MRI scan for the first two years. The patient has remained disease-free for 24 months after surgery (Figs. 1,2 and 3).
Fig. 1.
2.2. Case 2 An 11-month-old child presented with recurrent nosebleeds and a hard, painful left paranasal swelling as well as an extensive infection of the left tear duct and eye. An ultrasound and MRI scan showed a circumscribed lesion extending from the left medial canthus and the floor of the orbit to the maxillary sinus and left nasal cavity. Surgical resection involving a partial maxillectomy was performed via transantral access. Intraoperatively, the tumour filled the whole maxillary sinus and nasal cavity, displacing the septum, and showed several septae. Intraoperative fast frozen sections gave the preliminary result of a benign mesenchymal tumour. The lesion had a similar appearance to the first case and negative immunostaining for desmin and myogenin, which excluded a botryoid rhabdomyosarcoma, resulted in the diagnosis of myxoma. After uneventful postoperative healing, there was suspicion of a recurrence on the three-month postoperative MRI and ultrasound. A further resection was performed and there was a suspicion of another recurrence two months later, however only minimal remnants of myxoma were found in the specimen with extensive scar formation. Following this operation, the patient has remained tumour-free for eight years.
Fig. 2.
2.3. Case 3 A 36-year-old woman presented with a painful swelling of the right hard palate after sinusitis. On examination a swelling of the right hard palate and the right vestibule was visible. On X-ray, there was a cystic lesion in the region of the teeth 15 and 16. A biopsy was performed and revealed an odontogenic myxoma. The tumour was removed by a partial maxillectomy and in a second
Fig. 3.
operation, two years later, reconstructed via an iliac crest bone transplant. Unfortunately, the patient developed a late infection with actinomycosis of the graft and the graft had to be removed five months after reconstructive surgery. The infected area healed
K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
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Table 1 Odontogenic myxoma in children of less than 2 years in the literature. Author
Year
No of cases
Site
Patient age
Therapy
Follow-up time
Recurrence
Fenton et al. King et al.
2003 2008
1 2
Rotenberg et al.
2004
5
Wachter et al.
2003
2
Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla
17 18 17 13 18 16 13 19
Lateral rhinotomy Enucleation Enucleation Lateral rhinotomy Medial maxillectomy Lateral rhinotomy Midfacial degloving, excisional biopsy Sublabial, lateral nasal
16 months 18 months 24 months 4 years 14 years 7 years 24 months 24 months
No No No No No No No No
months months months months months months months months
well under adjuvant antibiotic therapy with clindamycin. The patient has remained recurrence-free until today, 42 months after diagnosis. A new attempt at bony reconstruction is planned in the near future.
Table 2 Differential diagnosis of odontogenic myxoma in children and adults. Children
Adults
Benign
Eosinophilic granuloma Histiocytosis Intraosseous haemangioma Cherubism Cranial fasciitis
Ameloblastoma Odontoma Odontogenic keratocyst Aneurysmal bone cyst Central giant cell granuloma
Malignant
Rhabdomyosarcoma Eosinophilic granuloma
Rhabdomyosarcoma Carcinoma Metastasis Ameloblastoma Metastasis
2.4. Case 4 A young woman initially presented at the age of 33 complaining of an intraoral swelling in the right retromolar mandible. An OPT revealed a polycystic lesion in the right angle of the mandible. A biopsy was performed and the diagnosis of odontogenic myxoma was established. The tumour was resected with preservation of mandibular continuity. The defect was filled secondarily with autologous bone graft from the iliac crest one week later, when the definitive diagnosis was established and the margins were tumourfree. Yearly follow-up was maintained for five years and was uneventfully. Fifteen years after the initial presentation, the patient presented again with a painless swelling in the right retromolar region and after imaging and biopsy the diagnosis of a recurrence was made. As the recurrent tumour now extended into the right ramus and condyle, a partial mandibulectomy was performed and the mandible was reconstructed in a second procedure with fibula. The patient is under follow yearly. She is fully functional and has remained disease-free six years after the recurrence and twentyone years after the initial presentation. 3. Literature review Using the earch term “odontogenic myxoma” lead to 231 results, of which 26 were review articles. 34 articles were case series, 100 were case reports, 14 were cohort studies. 16 articles focused on immunostaining, 11 focused on imaging aspects and 8 on surgical approaches. A total of 801 odontogenic myxomas are described within these articles. 178 myxomas are described in the maxilla, 238 in the mandible. For 385 tumours, no detailed localisation is given. Age is given in 144 cases, with an average of 26.4 years. There were 167 male and 232 female patients. In 402 cases no sex was given. Follow-up time was only given for 25 patients and of these 14 patients had been followed up for two years or less, 5 patients had a follow-up time of between two and five years and 6 patients had been followed up for more than five years (minimum 6 months, maximum 14 years, mean 3.5 years). Surgical procedures were reported in 35 cases. In four cases, enucleation was described and one of the four patients, a 14-year-old adolescent, had a recurrence after 2.5 years. Data on recurrence is given in 55 cases with a recurrence in five cases (9% recurrence rate). One malignant transformation is described which was finally fatal for the patient. A total of eight cases of odontogenic myxoma have been described in the literature for children of less than two years of age. All of these tumours occurred in the maxilla, more precisely, in the paranasal area with very similar extension patterns. After surgical
treatment, none of these lesions recurred. Detailed data of this sub entity is given in Table 1.
4. Discussion Odontogenic myxoma is a rare, slowly growing, non-metastasing tumour within the head and neck region. One case of malignant transformation has been described in the literature. Differential diagnosis in adults and children includes a wide variety of other benign but also malignant tumours (Table 2). This makes adequate diagnosis and management a challenging problem, having to balance between aggressive and radical intervention in case of malignancy and conservative approaches in benign lesions. Especially in children, one is hesitant to leave a functional and aesthetic defect to reduce recurrence rates. In adults, there is a tendency towards more radical operations with minimal risk of recurrence, as aesthetic and functional defects are stable. Furthermore, due to the locally destructive growth pattern, these tumours can invade critical structures like the orbital floor and the skull base, resulting in a non-resectable situation, especially in cases of recurrence. To make an accurate diagnosis, histology with immunohistochemical staining is most reliable in distinguishing myxoma from malignant tumours. Especially in children, with a short clinical history and less characteristic features on imaging, histology is essential to make a definitive diagnosis. In our two paediatric cases, the specimens showed haphazardly arranged stellate to spindleshaped cells with markedly eosinophilic cytoplasm embedded in a loose myxoid stroma. Collagen fibres were sparse and both tumours were located intraosseously. This is an important argument against cranial fasciitis, one of the most important differential diagnoses. Although cranial fasciitis can have a prominent myxoid background as well these lesions typically develop extraosseously and express smooth muscle actin which was negative in our cases. The expression of beta-catenin in the first case was only detected in the cytoplasm of the cells which is considered non-specific and also argues against the differential diagnosis of cranial fasciitis (Fig. 4a and b).
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K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
Fig. 4.
Fig. 5.
In order to plan the correct surgical approach, to plan biopsy and to estimate the correct extent of the tumour, various methods of imaging have been described. Therapy is primarily surgical, ranging from enucleation to partial and total maxillectomies (Leiser et al., 2009) and mandibulectomies. Leiser et al. could obtain good results with long disease-free follow-up with a more radical approach of a total maxillectomy in a recurrence case, a partial maxillectomy in a first presentation and a continuity resection with immediate reconstruction in a mandibular odontogenic myxoma. On the other hand Adebayo et al. point out the importance of
careful treatment selection in children as recurrence did not occur in their review following segmental resection (12 cases) or enucleation (3 cases). Although there is a tendency towards more conservative management in children and more radical approaches in adults, our analysis of reported cases in the literature does not support this management strategy. Enucleation shows recurrence rates similar to partial maxillectomies. Our case also shows that partial maxillectomy can lead to significant, not necessarily tumour-related, complications.
K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
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Fig. 6.
We therefore question the more aggressive management in adults and suggest more conservative procedures for this sub group. Only after recurrence can we see a clear indication for a more radical approach, as this is indicative of a more aggressive tumour and risk of subsequent recurrence requiring more aggressive treatment. However, reports in the literature and our own experience also show that, due to a tendency towards more extensive disease in adults with significant destruction of key structures, more radical surgery is inevitable in a large number of cases.
5. Conclusions Odontogenic myxoma in infants seems to have a very high prevalence in the central maxillary region. If diagnosed early, it can be removed by an intraoral incision with minimal damage to the surrounding structures. In children, we recommend an intraoperative fast frozen section for diagnosis and an immediate pre and postoperative MRI scan to avoid multiple anaesthetics and to keep radiation to a minimum. In adults, treatment concepts show a wider variety due do a wider range of localisations and size of tumour. After a thorough review of the literature evaluating a total of 801 cases we cannot support the thesis that a more radical approach with wide safety margins is appropriate to decrease recurrence rates (Figs. 5 and 6).
6. Outlook Currently we lack data on recurrence rates in odontogenic myxomas, especially concerning different therapeutic approaches. The authors therefore encourage all colleagues to contribute to an improved analysis of recurrence rates and surgical procedures by sending their anonymous patient data to the corresponding author giving patient age at diagnosis, gender, the surgical procedure
performed, follow-up time and time of recurrence if applicable. All contributors will be acknowledged in the analysis. Acknowledgements The authors would like to thank Prof. Dr. Gernot Jundt for critical review and comment of specimen and diagnosis. References Adebayo ET, Ajike SO, Adekeye EO: A review of 318 odontogenic tumors in Kaduna, Nigeria. J CranioMaxillofac Surg 63(6): 811e819, 2005 Araki M, Kameoka S, Mastumoto N, Komiyama K: Usefulness of cone beam computed tomography for odontogenic myxoma. Dentomaxillofac Radiol 36(7): 423e427, 2007 Asaumi J, Konouchi H, Hisatomi M, Kishi K: Odontogenic myxoma of maxillary sinus: CT and MR-pathologic correlation. Eur J Radiol 37(1): 1e4, 2001 Buchner A, Merrell PW, Carpenter WM: Relative frequency of central odontogenic tumors: a study of 1,088 cases from Northern California and comparison to studies from other parts of the world. J Oral Maxillofac Surg 64(9): 1343e1352, 2006 El-Ghehani R, Elarbi M, Subhashraj K: Benign tumours of orofacial region at Benghazi, Libya: a study of 405 cases. J CranioMaxillofac Surg 37: 370e375, 2009 Fenton S, Slootweg PJ, Dunnebier EA, Mourits MP: Odontogenic myxoma in a 17-month-old child: a case report. J Oral Maxillofac Surg 61(6): 734e736, 2003 Guerrisi M, Piloni MJ, Keszler A: Odontogenic tumors in children and adolescents. A 15-year retrospective study in Argentina. Med Oral Patol Oral Cir Bucal 12(3): E180eE185, 2007 Jing W, Xuan M, Lin Y, Wu L, Liu L, Zheng X, et al: Odontogenic tumours: a retrospective study of 1642 cases in a Chinese population. Int J Oral Maxillofac Surg 36(1): 20e25, 2007 Kawai T, Murakami S, Nishiyama H, Kishino M, Sakuda M, Fuchihata H: Diagnostic imaging for a case of maxillary myxoma with a review of the magnetic resonance images of myxoid lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 84(4): 449e454, 1997 King 3rd TJ, Lewis J, Orvidas L, Kademani D: Pediatric maxillary odontogenic myxoma: a report of 2 cases and review of management. J Oral Maxillofac Surg 66(5): 1057e1062, 2008 Ladeinde AL, Ajayi OF, Ogunlewe MO, Adeyemo WL, Arotiba GT, Bamgbose BO: Odontogenic tumors: a review of 319 cases in a Nigerian teaching hospital. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 99(2): 191e195, 2005 Leiser Y, Abu-el-Naad I, Peled M: Odontogenic myxoma e a case series and review of the surgical management. J CranioMaxillofac Surg 37: 206e209, 2009
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Li TJ, Sun LS, Luo HY: Odontogenic myxoma: a clinicopathologic study of 25 cases. Arch Pathol Lab Med 130(12): 1799e1806, 2006 MacDonald-Jankowski DS, Yeung RW, Li T, Lee KM: Computed tomography of odontogenic myxoma. Clin Radiol 59(3): 281e287, 2004 Martínez-Mata G, Mosqueda-Taylor A, Carlos-Bregni R, de Almeida OP, ContrerasVidaurre E, Vargas PA, et al: Odontogenic myxoma: clinico-pathological, immunohistochemical and ultrastructural findings of a multicentric series. Oral Oncol 44(6): 601e607, 2008 Mortellaro C, Berrone M, Turatti G, Rimondini L, Brach Del Prever A, Canavese F, et al: Odontogenic tumors in childhood: a retrospective study of 86 treated cases. Importance of a correct histopathologic diagnosis. J Craniofac Surg 19(4): 1173e1176, 2008 Ochsenius G, Ortega A, Godoy L, Peñafiel C, Escobar E: Odontogenic tumors in Chile: a study of 362 cases. J Oral Pathol Med 31(7): 415e420, 2002 Olgac V, Koseoglu BG, Aksakalli N: Odontogenic tumours in Istanbul: 527 cases. Br J Oral Maxillofac Surg 44(5): 386e388, 2006
Rotenberg BW, Daniel SJ, Nish IA, Ngan BY, Forte V: Myxomatous lesions of the maxilla in children: a case series and review of management. Int J Pediatr Otorhinolaryngol 68(10): 1251e1256, 2004 Simon EN, Merkx MA, Vuhahula E, Ngassapa D, Stoelinga PJ: A 4-year prospective study on epidemiology and clinicopathological presentation of odontogenic tumors in Tanzania. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 99(5): 598e602, 2005 Sumi Y, Miyaishi O, Ito K, Ueda M: Magnetic resonance imaging of myxoma in the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90(5): 671e676, 2000 Tamme T, Soots M, Kulla A, Karu K, Hanstein SM, Sokk A, et al: Odontogenic tumours, a collaborative retrospective study of 75 cases covering more than 25 years from Estonia. J CranioMaxillofac Surg 32: 161e165, 2004 Wachter BG, Steinberg MJ, Darrow DH, McGinn JD, Park AH: Odontogenic myxoma of the maxilla: a report of two pediatric cases. Int J Pediatr Otorhinolaryngol 67(4): 389e393, 2003
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
Case report
Odontogenic myxoma: Diagnostic and therapeutic challenges in paediatric and adult patients e A case series and review of the literature Katinka Kansy a, b, c, *, Philipp Juergens a, b, Zdzislav Krol a, Michael Paulussen d, Daniel Baumhoer e, Elisabeth Bruder e, Jacques Schneider f, Hans-Florian Zeilhofer a, b, Katja Schwenzer-Zimmerer a, b a
Deptartment of Cranio-Maxillofacial Surgery (Head: Prof. Hans-Florian Zeilhofer), University Hospital Basel, Spitalstr. 21, CH-4054 Basel, Switzerland High-tech Research Center of Cranio-Maxillofacial Surgery, University Hospital Basel, Switzerland Deptartment of Cranio-Maxillofacial Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 400, D-69120 Heidelberg, Germany d Deptartment of Oncology, Children’s Hospital Basel, Switzerland e Institute of Pathology, University Hospital Basel, Switzerland f Deptartment of Radiology, Children’s University Hospital Basel, Switzerland b c
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 10 January 2011 Accepted 19 April 2011
Introduction: Odontogenic myxomas are benign but locally invasive tumours originating from primordial mesenchymal tooth forming tissues which do not metastasise. We present a series of two paediatric and two adult cases and focus on differences in diagnostic and therapeutic approaches between children and adults based on our own experience and a critical review of the literature. Ó 2011 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Odontogenic myxoma Children
1. Introduction Odontogenic myxomas are benign but locally invasive tumours originating from primordial mesenchymal tooth forming tissues which do not metastasise. They are rare tumours and account for 3.3e15.7% of all odontogenic tumours in adults (El-Ghehani et al., 2009, Araki et al., 2007, Jing et al., 2007, Buchner et al., 2006, Olgac et al., 2006, Adebayo et al., 2005, Simon et al., 2005, Ladeinde et al., 2005, Tamme et al., 2004, Ochsenius et al., 2002) and for 8.5e11.6% in children (Mortellaro et al., 2008, Guerrisi et al., 2007). They occur in all age groups with a peak incidence in the third decade. Odontogenic myxomas can be found in both the maxilla and mandible, usually associated with a tooth germ. On imaging, odontogenic myxomas mostly present as radiolucent, multilocular structures on X-ray and CT scan. Intermediate signal intensity on T1 and high signal intensity on T2 are characteristic MRI findings with good demonstration of the extent of any resorbtion. Both CT and MRI correlate well with the histological features and are considered useful tools for diagnosis (Kawai et al., 1997, Asaumi et al., 2001, Sumi et al., 2000, MacDonald-Jankowski et al., 2004). One study has shown a beneficial effect of DVT
imaging for the diagnosis of the internal structure of the lesion and the condition of margins (Araki et al., 2007). Diagnosis is most commonly established by biopsy. Histopathological features include a whitish-grey glistening or gelatinous mass with minimal true encapsulation macroscopically and spindled or stellate-shaped cells in a mucoid-rich intracellular matrix microscopically (Martínez-Mata et al., 2008; Li et al., 2006). Immunocytochemical staining is positive for vimentin, but in contrast to myosarcomas is negative for desmin, neuron specific enolase, glial fibrillary acid protein and S100 (Martínez-Mata et al., 2008, Li et al., 2006). Management varies depending on the location and size of the tumour, the age of the patient and individual experience. They range from minimally invasive excisional biopsies to an en bloc resection of a multitude of relevant structures. Long-term follow-up is crucial as myxomas have a significant tendency to reccur. Recurrence rates for myxomas in the literature vary from 5 to 10% but there is no reliable data in the existing literature.
2. Case reports * Corresponding author. Department of Cranio-Maxillofacial Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 400, D-69120 Heidelberg, Germany. Tel.: þ49 6221 5636119; fax: þ49 6221 4375. E-mail address: [email protected] (K. Kansy).
2.1. Case 1 A 12-month-old child was referred with a four-day history of a painless left paranasal swelling and increased tearing for several
1010-5182/$ e see front matter Ó 2011 European Association for Cranio-Maxillo-Facial Surgery. doi:10.1016/j.jcms.2011.04.009
272
K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
weeks. Physical examination revealed a fixed tumour of 1.5 cm in diameter. The lesion was firm, non-tender and did not enlarge with crying. Eye position and extra ocular motion were normal. Clinical examination otherwise was normal. An axial computed tomogram (CT) of the head revealed a welldefined intraosseous expansile mass involving the inferior, anterior and medial wall of the left maxillary sinus and the left infraorbital rim. The tumour reached the germ of the left permanent canine and compressed the left nasolacrimal duct without invading it. Magnetic resonance imaging (MRI) revealed a well-defined mass, 16 mm in diameter, with an intermediate intensity signal on T1- and a high intensity signal on T2-weighted image. An intraoperative fast frozen section was performed giving the preliminary diagnosis of a myxoid tumour, probably low grade. Subsequently, enucleation of the tumour was performed with curettage of the surrounding bone through a vestibular incision. Immediate postoperative MRI showed complete removal of the lesion. The patient recovered uneventfully from surgery and no scar was visible at follow-up. Final histology revealed a myxoid tumour of 1.6 cm in diameter with haphazardly arranged stellate to spindle-shaped cells with marked eosinophlic cytoplasm in a mucoid-rich intercellular matrix. Immunohistochemical staining was positive for vimentin and also for beta-catenin cytoplasmatically with a proliferation index of 5% in the MIB-1 reaction. The tumour stained negatively for Alk-1, CD31, CD34, CD68, calponin, desmin, myogenin, SMA and S-100. The final diagnosis was odontogenic myxoma. The patient showed no signs of recurrence at six months follow-up visit. An interdisciplinary team of experts decided on the following followup management: clinical examination one-monthly for the first year, two-monthly for the second year and a three-monthly MRI scan for the first two years. The patient has remained disease-free for 24 months after surgery (Figs. 1,2 and 3).
Fig. 1.
2.2. Case 2 An 11-month-old child presented with recurrent nosebleeds and a hard, painful left paranasal swelling as well as an extensive infection of the left tear duct and eye. An ultrasound and MRI scan showed a circumscribed lesion extending from the left medial canthus and the floor of the orbit to the maxillary sinus and left nasal cavity. Surgical resection involving a partial maxillectomy was performed via transantral access. Intraoperatively, the tumour filled the whole maxillary sinus and nasal cavity, displacing the septum, and showed several septae. Intraoperative fast frozen sections gave the preliminary result of a benign mesenchymal tumour. The lesion had a similar appearance to the first case and negative immunostaining for desmin and myogenin, which excluded a botryoid rhabdomyosarcoma, resulted in the diagnosis of myxoma. After uneventful postoperative healing, there was suspicion of a recurrence on the three-month postoperative MRI and ultrasound. A further resection was performed and there was a suspicion of another recurrence two months later, however only minimal remnants of myxoma were found in the specimen with extensive scar formation. Following this operation, the patient has remained tumour-free for eight years.
Fig. 2.
2.3. Case 3 A 36-year-old woman presented with a painful swelling of the right hard palate after sinusitis. On examination a swelling of the right hard palate and the right vestibule was visible. On X-ray, there was a cystic lesion in the region of the teeth 15 and 16. A biopsy was performed and revealed an odontogenic myxoma. The tumour was removed by a partial maxillectomy and in a second
Fig. 3.
operation, two years later, reconstructed via an iliac crest bone transplant. Unfortunately, the patient developed a late infection with actinomycosis of the graft and the graft had to be removed five months after reconstructive surgery. The infected area healed
K. Kansy et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 271e276
273
Table 1 Odontogenic myxoma in children of less than 2 years in the literature. Author
Year
No of cases
Site
Patient age
Therapy
Follow-up time
Recurrence
Fenton et al. King et al.
2003 2008
1 2
Rotenberg et al.
2004
5
Wachter et al.
2003
2
Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla Maxilla
17 18 17 13 18 16 13 19
Lateral rhinotomy Enucleation Enucleation Lateral rhinotomy Medial maxillectomy Lateral rhinotomy Midfacial degloving, excisional biopsy Sublabial, lateral nasal
16 months 18 months 24 months 4 years 14 years 7 years 24 months 24 months
No No No No No No No No
months months months months months months months months
well under adjuvant antibiotic therapy with clindamycin. The patient has remained recurrence-free until today, 42 months after diagnosis. A new attempt at bony reconstruction is planned in the near future.
Table 2 Differential diagnosis of odontogenic myxoma in children and adults. Children
Adults
Benign
Eosinophilic granuloma Histiocytosis Intraosseous haemangioma Cherubism Cranial fasciitis
Ameloblastoma Odontoma Odontogenic keratocyst Aneurysmal bone cyst Central giant cell granuloma
Malignant
Rhabdomyosarcoma Eosinophilic granuloma
Rhabdomyosarcoma Carcinoma Metastasis Ameloblastoma Metastasis
2.4. Case 4 A young woman initially presented at the age of 33 complaining of an intraoral swelling in the right retromolar mandible. An OPT revealed a polycystic lesion in the right angle of the mandible. A biopsy was performed and the diagnosis of odontogenic myxoma was established. The tumour was resected with preservation of mandibular continuity. The defect was filled secondarily with autologous bone graft from the iliac crest one week later, when the definitive diagnosis was established and the margins were tumourfree. Yearly follow-up was maintained for five years and was uneventfully. Fifteen years after the initial presentation, the patient presented again with a painless swelling in the right retromolar region and after imaging and biopsy the diagnosis of a recurrence was made. As the recurrent tumour now extended into the right ramus and condyle, a partial mandibulectomy was performed and the mandible was reconstructed in a second procedure with fibula. The patient is under follow yearly. She is fully functional and has remained disease-free six years after the recurrence and twentyone years after the initial presentation. 3. Literature review Using the earch term “odontogenic myxoma” lead to 231 results, of which 26 were review articles. 34 articles were case series, 100 were case reports, 14 were cohort studies. 16 articles focused on immunostaining, 11 focused on imaging aspects and 8 on surgical approaches. A total of 801 odontogenic myxomas are described within these articles. 178 myxomas are described in the maxilla, 238 in the mandible. For 385 tumours, no detailed localisation is given. Age is given in 144 cases, with an average of 26.4 years. There were 167 male and 232 female patients. In 402 cases no sex was given. Follow-up time was only given for 25 patients and of these 14 patients had been followed up for two years or less, 5 patients had a follow-up time of between two and five years and 6 patients had been followed up for more than five years (minimum 6 months, maximum 14 years, mean 3.5 years). Surgical procedures were reported in 35 cases. In four cases, enucleation was described and one of the four patients, a 14-year-old adolescent, had a recurrence after 2.5 years. Data on recurrence is given in 55 cases with a recurrence in five cases (9% recurrence rate). One malignant transformation is described which was finally fatal for the patient. A total of eight cases of odontogenic myxoma have been described in the literature for children of less than two years of age. All of these tumours occurred in the maxilla, more precisely, in the paranasal area with very similar extension patterns. After surgical
treatment, none of these lesions recurred. Detailed data of this sub entity is given in Table 1.
4. Discussion Odontogenic myxoma is a rare, slowly growing, non-metastasing tumour within the head and neck region. One case of malignant transformation has been described in the literature. Differential diagnosis in adults and children includes a wide variety of other benign but also malignant tumours (Table 2). This makes adequate diagnosis and management a challenging problem, having to balance between aggressive and radical intervention in case of malignancy and conservative approaches in benign lesions. Especially in children, one is hesitant to leave a functional and aesthetic defect to reduce recurrence rates. In adults, there is a tendency towards more radical operations with minimal risk of recurrence, as aesthetic and functional defects are stable. Furthermore, due to the locally destructive growth pattern, these tumours can invade critical structures like the orbital floor and the skull base, resulting in a non-resectable situation, especially in cases of recurrence. To make an accurate diagnosis, histology with immunohistochemical staining is most reliable in distinguishing myxoma from malignant tumours. Especially in children, with a short clinical history and less characteristic features on imaging, histology is essential to make a definitive diagnosis. In our two paediatric cases, the specimens showed haphazardly arranged stellate to spindleshaped cells with markedly eosinophilic cytoplasm embedded in a loose myxoid stroma. Collagen fibres were sparse and both tumours were located intraosseously. This is an important argument against cranial fasciitis, one of the most important differential diagnoses. Although cranial fasciitis can have a prominent myxoid background as well these lesions typically develop extraosseously and express smooth muscle actin which was negative in our cases. The expression of beta-catenin in the first case was only detected in the cytoplasm of the cells which is considered non-specific and also argues against the differential diagnosis of cranial fasciitis (Fig. 4a and b).
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Fig. 4.
Fig. 5.
In order to plan the correct surgical approach, to plan biopsy and to estimate the correct extent of the tumour, various methods of imaging have been described. Therapy is primarily surgical, ranging from enucleation to partial and total maxillectomies (Leiser et al., 2009) and mandibulectomies. Leiser et al. could obtain good results with long disease-free follow-up with a more radical approach of a total maxillectomy in a recurrence case, a partial maxillectomy in a first presentation and a continuity resection with immediate reconstruction in a mandibular odontogenic myxoma. On the other hand Adebayo et al. point out the importance of
careful treatment selection in children as recurrence did not occur in their review following segmental resection (12 cases) or enucleation (3 cases). Although there is a tendency towards more conservative management in children and more radical approaches in adults, our analysis of reported cases in the literature does not support this management strategy. Enucleation shows recurrence rates similar to partial maxillectomies. Our case also shows that partial maxillectomy can lead to significant, not necessarily tumour-related, complications.
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Fig. 6.
We therefore question the more aggressive management in adults and suggest more conservative procedures for this sub group. Only after recurrence can we see a clear indication for a more radical approach, as this is indicative of a more aggressive tumour and risk of subsequent recurrence requiring more aggressive treatment. However, reports in the literature and our own experience also show that, due to a tendency towards more extensive disease in adults with significant destruction of key structures, more radical surgery is inevitable in a large number of cases.
5. Conclusions Odontogenic myxoma in infants seems to have a very high prevalence in the central maxillary region. If diagnosed early, it can be removed by an intraoral incision with minimal damage to the surrounding structures. In children, we recommend an intraoperative fast frozen section for diagnosis and an immediate pre and postoperative MRI scan to avoid multiple anaesthetics and to keep radiation to a minimum. In adults, treatment concepts show a wider variety due do a wider range of localisations and size of tumour. After a thorough review of the literature evaluating a total of 801 cases we cannot support the thesis that a more radical approach with wide safety margins is appropriate to decrease recurrence rates (Figs. 5 and 6).
6. Outlook Currently we lack data on recurrence rates in odontogenic myxomas, especially concerning different therapeutic approaches. The authors therefore encourage all colleagues to contribute to an improved analysis of recurrence rates and surgical procedures by sending their anonymous patient data to the corresponding author giving patient age at diagnosis, gender, the surgical procedure
performed, follow-up time and time of recurrence if applicable. All contributors will be acknowledged in the analysis. Acknowledgements The authors would like to thank Prof. Dr. Gernot Jundt for critical review and comment of specimen and diagnosis. References Adebayo ET, Ajike SO, Adekeye EO: A review of 318 odontogenic tumors in Kaduna, Nigeria. J CranioMaxillofac Surg 63(6): 811e819, 2005 Araki M, Kameoka S, Mastumoto N, Komiyama K: Usefulness of cone beam computed tomography for odontogenic myxoma. Dentomaxillofac Radiol 36(7): 423e427, 2007 Asaumi J, Konouchi H, Hisatomi M, Kishi K: Odontogenic myxoma of maxillary sinus: CT and MR-pathologic correlation. Eur J Radiol 37(1): 1e4, 2001 Buchner A, Merrell PW, Carpenter WM: Relative frequency of central odontogenic tumors: a study of 1,088 cases from Northern California and comparison to studies from other parts of the world. J Oral Maxillofac Surg 64(9): 1343e1352, 2006 El-Ghehani R, Elarbi M, Subhashraj K: Benign tumours of orofacial region at Benghazi, Libya: a study of 405 cases. J CranioMaxillofac Surg 37: 370e375, 2009 Fenton S, Slootweg PJ, Dunnebier EA, Mourits MP: Odontogenic myxoma in a 17-month-old child: a case report. J Oral Maxillofac Surg 61(6): 734e736, 2003 Guerrisi M, Piloni MJ, Keszler A: Odontogenic tumors in children and adolescents. A 15-year retrospective study in Argentina. Med Oral Patol Oral Cir Bucal 12(3): E180eE185, 2007 Jing W, Xuan M, Lin Y, Wu L, Liu L, Zheng X, et al: Odontogenic tumours: a retrospective study of 1642 cases in a Chinese population. Int J Oral Maxillofac Surg 36(1): 20e25, 2007 Kawai T, Murakami S, Nishiyama H, Kishino M, Sakuda M, Fuchihata H: Diagnostic imaging for a case of maxillary myxoma with a review of the magnetic resonance images of myxoid lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 84(4): 449e454, 1997 King 3rd TJ, Lewis J, Orvidas L, Kademani D: Pediatric maxillary odontogenic myxoma: a report of 2 cases and review of management. J Oral Maxillofac Surg 66(5): 1057e1062, 2008 Ladeinde AL, Ajayi OF, Ogunlewe MO, Adeyemo WL, Arotiba GT, Bamgbose BO: Odontogenic tumors: a review of 319 cases in a Nigerian teaching hospital. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 99(2): 191e195, 2005 Leiser Y, Abu-el-Naad I, Peled M: Odontogenic myxoma e a case series and review of the surgical management. J CranioMaxillofac Surg 37: 206e209, 2009
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