Case Report
Desmoplastic ameloblastoma Sumit Majumdar, Divya Uppala, Sreekanth Kotina, Sravanthi Karukonda Veera, Rajyalakshmi Boddepalli Department of Oral Pathology, GITAM Dental College, Rushikonda,Vizag, Andhra Pradesh, India
Abstract Desmoplastic ameloblastoma is a relatively rare variety of ameloblastoma and only very few cases have been reported so far. The present case is an elderly man who had reported with a swelling in the anterior mandible which turned up to be desmoplastic ameloblastoma. Proper diagnosis is necessary to report such a case, so that the actual incidence can be noted. Key words: Ameloblastoma, anterior, desmoplastic, mandible Submission: 26-01-2014 Accepted: 25-04-2014
Introduction The tumors that arise from odontogenic epithelium are called as ameloblastomas. Its histopathologic types are plexiform, follicular, acanthomatous, granular, and desmoplastic.[1] The desmoplastic variety is characterized by abundant collagenous stroma and small strands of compressed odontogenic type of epithelium. This feature makes it unique among the ameloblastomas.[2] The other rarer variants include basal cell, keratoameloblastoma, papilliferous keratoameloblastoma, and clear cell types.[3]
Case Report A 55‑year‑old male patient presented to the Department of Oral Pathology, GITAM Dental College with the complaint of swelling in the right lower premolar region [Figure 1]. The patient had noticed the swelling 5 months back, which had slowly increased to the present size. On extraoral examination, noticeable facial asymmetry was observed due to swelling present on the right Address for correspondence: Dr. Divya Uppala, Department of Oral Pathology, GITAM Dental College, Rushikonda, Vizag ‑ 530 045, Andhra Pradesh, India. E‑mail: [email protected] Access this article online Quick Response Code:
Website: www.ijabmr.org
DOI: 10.4103/2229-516X.140743
side of the mandible. The right submandibular lymph node was also palpable and non‑tender. On intraoral examination, there was an ill‑defined solitary swelling extending from the mesial aspect of 31 to the mesial aspect of 45, superioinferiorly extending from the superior margin of attached gingival to well beyond the gingival sulcus. Radiographic examination revealed a mixed radiolucency interspersed with radiopacities with ill‑defined borders. Occlusal radiographs showed an ill‑defined radiolucent lesion interspersed with radiopacities extending from the mesial aspect of 41 to the distal aspect of 45 [Figures 2 and 3].The lesional tissue after excisional biopsy had gritty or “frozen ice‑cream”–like consistency [Figure 4]. The lower border of the mandible was preserved so that the aesthetic and functional work‑up could be done [Figure 5]. The cut surface was solid and whitish in most cases. The epithelial tumor islands were very irregular with a pointed stellate appearance or small strand like and discrete. The histopathology was characterized by stromal desmoplasia, small tumor nests and strands of odontogenic epithelium were scattered in the stroma [Figures 6-8].
Discussion The gross specimen most often consists of resected portions of jaws. More than 50% show multilocular mixed radiolucent/ radiopaque lesions with ill‑defined margins. Desmoplastic ameloblastoma represents approximately 4-13% of all ameloblastomas.[3] This lesion most commonly occurs between the ages of 17 and 72 years, with the mean age being 44.7 years and with a male predilection.[4] The prevalence of ameloblastoma in maxilla when compared to mandible is almost close to 1:1. Desmoplastic ameloblastoma s is found predominantly in anterior or premolar regions of the jaws.[5]
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
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Majumdar, et al.: Desmoplastic ameloblastoma
Figure 1: Intraoral picture of the patient
Figure 2: Intraoral periapical radiograph
Figure 3: Occlusal radiograph of the mandible
Figure 4: Gross picture of the resected area
Figure 5: Postoperative radiograph
The chief initial complaint usually is as a painless swelling of the jaw bone. It is smaller in size than other types of ameloblastoma, and occurs mainly in the alveolar region and occupies the tooth‑bearing area.[6] The resorption of tooth roots and displacement of teeth is seen frequently. This variant exhibits no typical findings of ameloblastoma. The oxytalan fibers in the stromal tissue suggest that the tumor derived from the epithelial rest of Malassez in the periodontal membrane of a related tooth.The periphery of islands consists of cuboidal cells with hyperchromatic nuclei.The columnar cells with reversed S54
Figure 6: Histopathology showing odontogenic epithelium like areas in between dense collagen tissue fibers
nuclear polarity are rarely conspicuous. The central area consists of swirled, hypercellular, spindle‑shaped, or squamous epithelial cells. Microcysts that contain eosinophilic amorphous deposits or appear empty may occur centrally.The stroma has
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
Majumdar, et al.: Desmoplastic ameloblastoma
Figure 7: 40× magnification of the desmoplastic area
extensive stromal desmoplasia characterized by moderately cellular fibrous connective tissue with abundant thick collagen fibers that seem to compress or “squeeze” the odontogenic epithelial islands from the periphery. Formation of metaplastic osteoid trabeculae (osteoplasia) may be present. Evidence of a capsule is not found. In the immunoprofile, the tumor cells exhibit variable expression of S100 protein and desmin, similar to other types of s olid multicystic a meloblastoma (SMA). Keratin immunoreactivity is confined to the tumor cells showing squamous differentiation. Decreased expression of CK19 and high expression of p63 is observed.[7] Vimentin is not expressed by either squamatoid or spindle‑shaped cells. Differences in expression are attributed to diverse factors such as dedifferentiation or rate of proliferation of neoplastic cells, inherent cellular potential, or extracellular mediators.[8] Marked immunoexpression of transforming growth factor beta (TGF‑β) points to the part played in prominent desmoplastic matrix formation.[9] Compared to the stroma of SMA, the desmoplastic stroma of desmoplastic ameloblastoma shows a strong positive reaction for collagen type VI, fibronectin, and type 1 collagen. Treatment is by resection (77.1%) and, in some cases, by enucleation and/ or curettage. Long‑term follow‑up is a must after initial treatment.[10]
Conclusion As desmoplastic ameloblastoma is an uncommon lesion, it warrants proper diagnosis and treatment.
Figure 8: 20× magnification of the area exhibiting peripheral cells of tumor island columnar cells with reverse polarity. Hypercellularity of spindle-shaped or polygonal cells and microcyst formation is seen focally
Pathology and Genetics. Head and Neck Tumors. Lyon, France: IARC Press; 2005. 2. Gardner DG, Heikinheimo K, Shear M, Philipsen HP, Coleman H. Ameloblastomas. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumors. Lyon, France: IARC Press; 2005. p. 296‑300. 3. Durmus E, Kalayci A, Ozturk A, Gunhan O. Desmoplastic ameloblastoma in the mandible. J Craniofac Surg 2003;14:873‑5. 4. Kim JD, Jang HS, Seo YS, Kim JS. A repeatedly recurrent desmoplastic ameloblastoma after removal and allobone graft: Radiographic features compared with histological changes. Imaging Sci Dent 2013;43:201‑7. 5. Reichert PA, Philipsen HP. Benign neoplasm’s and tumor‑like lesions arising from the odontogenic apparatus showing odontogenic epithelium with mature fibrous stroma, without ectomesenchyme. In: Odontogenic Tumors and Allied Lesions. London: Quintessence Publishing Co. Ltd.; 2004. p. 41. 6. Savithri V, Janardhanan M, Suresh R, Kumar RV. Desmoplastic ameloblastoma with osteoplasia: Review of literature with a case report. J Oral Maxillofac Pathol 2013;17:298‑301. 7. Sherlin HJ, Natesan A, Ram P, Ramani P, Thiruvenkadam C. Immunohistochemical profiling of ameloblastomas using cytokeratin, vimentin, smooth muscle actin, CD34 and S100. Ann Maxillofac Surg 2013;3:51‑7. 8. Matsuo A, Ueno S. Immunohistochemical demonstration of keratin in ameloblastoma as an indication of tumor differentiation. J Oral Maxillofac Surg 1991;49:282‑9. 9. Takata T, Miyauchi M, Ogawa I, Kudo Y, Takekoshi T, Zhao M, et al. Immunoexpression of transforming growth factor beta in desmoplastic ameloblastoma. Virchows Arch 2000;436:319‑23. 10. Medeiros Junior R, Queiroz IV, Correia AV, Vasconcelos BC, Gueiros LA, Leao JC, et al. Mandibular desmoplastic ameloblastoma: Case report. Gen Dent 2012;60:e109‑13.
References
How to cite this article: Majumdar S, Uppala D, Kotina S, Veera SK, Boddepalli R. Desmoplastic ameloblastoma. Int J App Basic Med Res 2014;4:53-5.
1. Barnes L, Eveson JW, Reichart P, Sidransky D, Kleihues P, Sobin LH, editors. World Health Organization Classification of Tumors.
Source of Support: Nill. Conflict of Interest: None declared.
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
55 S55
Copyright of International Journal of Applied & Basic Medical Research is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Desmoplastic ameloblastoma Sumit Majumdar, Divya Uppala, Sreekanth Kotina, Sravanthi Karukonda Veera, Rajyalakshmi Boddepalli Department of Oral Pathology, GITAM Dental College, Rushikonda,Vizag, Andhra Pradesh, India
Abstract Desmoplastic ameloblastoma is a relatively rare variety of ameloblastoma and only very few cases have been reported so far. The present case is an elderly man who had reported with a swelling in the anterior mandible which turned up to be desmoplastic ameloblastoma. Proper diagnosis is necessary to report such a case, so that the actual incidence can be noted. Key words: Ameloblastoma, anterior, desmoplastic, mandible Submission: 26-01-2014 Accepted: 25-04-2014
Introduction The tumors that arise from odontogenic epithelium are called as ameloblastomas. Its histopathologic types are plexiform, follicular, acanthomatous, granular, and desmoplastic.[1] The desmoplastic variety is characterized by abundant collagenous stroma and small strands of compressed odontogenic type of epithelium. This feature makes it unique among the ameloblastomas.[2] The other rarer variants include basal cell, keratoameloblastoma, papilliferous keratoameloblastoma, and clear cell types.[3]
Case Report A 55‑year‑old male patient presented to the Department of Oral Pathology, GITAM Dental College with the complaint of swelling in the right lower premolar region [Figure 1]. The patient had noticed the swelling 5 months back, which had slowly increased to the present size. On extraoral examination, noticeable facial asymmetry was observed due to swelling present on the right Address for correspondence: Dr. Divya Uppala, Department of Oral Pathology, GITAM Dental College, Rushikonda, Vizag ‑ 530 045, Andhra Pradesh, India. E‑mail: [email protected] Access this article online Quick Response Code:
Website: www.ijabmr.org
DOI: 10.4103/2229-516X.140743
side of the mandible. The right submandibular lymph node was also palpable and non‑tender. On intraoral examination, there was an ill‑defined solitary swelling extending from the mesial aspect of 31 to the mesial aspect of 45, superioinferiorly extending from the superior margin of attached gingival to well beyond the gingival sulcus. Radiographic examination revealed a mixed radiolucency interspersed with radiopacities with ill‑defined borders. Occlusal radiographs showed an ill‑defined radiolucent lesion interspersed with radiopacities extending from the mesial aspect of 41 to the distal aspect of 45 [Figures 2 and 3].The lesional tissue after excisional biopsy had gritty or “frozen ice‑cream”–like consistency [Figure 4]. The lower border of the mandible was preserved so that the aesthetic and functional work‑up could be done [Figure 5]. The cut surface was solid and whitish in most cases. The epithelial tumor islands were very irregular with a pointed stellate appearance or small strand like and discrete. The histopathology was characterized by stromal desmoplasia, small tumor nests and strands of odontogenic epithelium were scattered in the stroma [Figures 6-8].
Discussion The gross specimen most often consists of resected portions of jaws. More than 50% show multilocular mixed radiolucent/ radiopaque lesions with ill‑defined margins. Desmoplastic ameloblastoma represents approximately 4-13% of all ameloblastomas.[3] This lesion most commonly occurs between the ages of 17 and 72 years, with the mean age being 44.7 years and with a male predilection.[4] The prevalence of ameloblastoma in maxilla when compared to mandible is almost close to 1:1. Desmoplastic ameloblastoma s is found predominantly in anterior or premolar regions of the jaws.[5]
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
53 S53
Majumdar, et al.: Desmoplastic ameloblastoma
Figure 1: Intraoral picture of the patient
Figure 2: Intraoral periapical radiograph
Figure 3: Occlusal radiograph of the mandible
Figure 4: Gross picture of the resected area
Figure 5: Postoperative radiograph
The chief initial complaint usually is as a painless swelling of the jaw bone. It is smaller in size than other types of ameloblastoma, and occurs mainly in the alveolar region and occupies the tooth‑bearing area.[6] The resorption of tooth roots and displacement of teeth is seen frequently. This variant exhibits no typical findings of ameloblastoma. The oxytalan fibers in the stromal tissue suggest that the tumor derived from the epithelial rest of Malassez in the periodontal membrane of a related tooth.The periphery of islands consists of cuboidal cells with hyperchromatic nuclei.The columnar cells with reversed S54
Figure 6: Histopathology showing odontogenic epithelium like areas in between dense collagen tissue fibers
nuclear polarity are rarely conspicuous. The central area consists of swirled, hypercellular, spindle‑shaped, or squamous epithelial cells. Microcysts that contain eosinophilic amorphous deposits or appear empty may occur centrally.The stroma has
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
Majumdar, et al.: Desmoplastic ameloblastoma
Figure 7: 40× magnification of the desmoplastic area
extensive stromal desmoplasia characterized by moderately cellular fibrous connective tissue with abundant thick collagen fibers that seem to compress or “squeeze” the odontogenic epithelial islands from the periphery. Formation of metaplastic osteoid trabeculae (osteoplasia) may be present. Evidence of a capsule is not found. In the immunoprofile, the tumor cells exhibit variable expression of S100 protein and desmin, similar to other types of s olid multicystic a meloblastoma (SMA). Keratin immunoreactivity is confined to the tumor cells showing squamous differentiation. Decreased expression of CK19 and high expression of p63 is observed.[7] Vimentin is not expressed by either squamatoid or spindle‑shaped cells. Differences in expression are attributed to diverse factors such as dedifferentiation or rate of proliferation of neoplastic cells, inherent cellular potential, or extracellular mediators.[8] Marked immunoexpression of transforming growth factor beta (TGF‑β) points to the part played in prominent desmoplastic matrix formation.[9] Compared to the stroma of SMA, the desmoplastic stroma of desmoplastic ameloblastoma shows a strong positive reaction for collagen type VI, fibronectin, and type 1 collagen. Treatment is by resection (77.1%) and, in some cases, by enucleation and/ or curettage. Long‑term follow‑up is a must after initial treatment.[10]
Conclusion As desmoplastic ameloblastoma is an uncommon lesion, it warrants proper diagnosis and treatment.
Figure 8: 20× magnification of the area exhibiting peripheral cells of tumor island columnar cells with reverse polarity. Hypercellularity of spindle-shaped or polygonal cells and microcyst formation is seen focally
Pathology and Genetics. Head and Neck Tumors. Lyon, France: IARC Press; 2005. 2. Gardner DG, Heikinheimo K, Shear M, Philipsen HP, Coleman H. Ameloblastomas. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumors. Lyon, France: IARC Press; 2005. p. 296‑300. 3. Durmus E, Kalayci A, Ozturk A, Gunhan O. Desmoplastic ameloblastoma in the mandible. J Craniofac Surg 2003;14:873‑5. 4. Kim JD, Jang HS, Seo YS, Kim JS. A repeatedly recurrent desmoplastic ameloblastoma after removal and allobone graft: Radiographic features compared with histological changes. Imaging Sci Dent 2013;43:201‑7. 5. Reichert PA, Philipsen HP. Benign neoplasm’s and tumor‑like lesions arising from the odontogenic apparatus showing odontogenic epithelium with mature fibrous stroma, without ectomesenchyme. In: Odontogenic Tumors and Allied Lesions. London: Quintessence Publishing Co. Ltd.; 2004. p. 41. 6. Savithri V, Janardhanan M, Suresh R, Kumar RV. Desmoplastic ameloblastoma with osteoplasia: Review of literature with a case report. J Oral Maxillofac Pathol 2013;17:298‑301. 7. Sherlin HJ, Natesan A, Ram P, Ramani P, Thiruvenkadam C. Immunohistochemical profiling of ameloblastomas using cytokeratin, vimentin, smooth muscle actin, CD34 and S100. Ann Maxillofac Surg 2013;3:51‑7. 8. Matsuo A, Ueno S. Immunohistochemical demonstration of keratin in ameloblastoma as an indication of tumor differentiation. J Oral Maxillofac Surg 1991;49:282‑9. 9. Takata T, Miyauchi M, Ogawa I, Kudo Y, Takekoshi T, Zhao M, et al. Immunoexpression of transforming growth factor beta in desmoplastic ameloblastoma. Virchows Arch 2000;436:319‑23. 10. Medeiros Junior R, Queiroz IV, Correia AV, Vasconcelos BC, Gueiros LA, Leao JC, et al. Mandibular desmoplastic ameloblastoma: Case report. Gen Dent 2012;60:e109‑13.
References
How to cite this article: Majumdar S, Uppala D, Kotina S, Veera SK, Boddepalli R. Desmoplastic ameloblastoma. Int J App Basic Med Res 2014;4:53-5.
1. Barnes L, Eveson JW, Reichart P, Sidransky D, Kleihues P, Sobin LH, editors. World Health Organization Classification of Tumors.
Source of Support: Nill. Conflict of Interest: None declared.
International Journal of Applied and Basic Medical Research, 2014, Vol 4, Supplement 1
55 S55
Copyright of International Journal of Applied & Basic Medical Research is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
