nearly 200 cases of calcifying epithelial odontogenic tumor have been reported in literature since pindborg described it as a separate pathologic entity in 1955 .
it has been identified under different denominations , such as , ameloblastoma of unusual type with calcification , calcifying ameloblastoma , malignant odontoma , cystic complex odontoma , and has also been considered as a variant of simple ameloblastoma .
the eponym pindborg tumor was first introduced to the literature in 1967 , to describe this interesting and unique odontogenic tumor .
the calcifying epithelial odontogenic tumor is a benign odontogenic tumor of epithelial origin that accounts for approximately 1% of all odontogenic tumors .
it is more common in the posterior part of the mandible of adults in the fourth to fifth decades .
it is characterized by squamous epithelial cells , calcifying masses , and homogeneous acellular material admixed with the tumor epithelium and stroma that have been identified as amyloid .
the origin of this neoplasm is not clearly known , although it is generally accepted to be derived from oral epithelium , reduced enamel epithelium , stratum intermedium or dental lamina remnants.[13 ] the differential diagnosis for ceot should include adenomatoid odontogenic tumor ( aot ) , calcifying odontogenic cyst ( coc ) , ameloblastic fibro odontoma ( afo ) , odontoma , and in our case amyloidoma .
this article describes a case of ceot involving the left maxilla associated with multiple myeloma in a middle - aged patient .
the correlations between clinical , radiological , and histological patterns are discussed and a brief review of the literature is presented .
a 55-year - old man presented at the ent department of sri ramchandra university with a one - and - a - half year history of painless left nasal swelling .
it was hard in consistency , gradually increasing in size to attain the current size of 4 cm .
he was diagnosed to have multiple myeloma stage iiia in 2004 , for which he underwent chemotherapy for a year .
he had a fall in 2008 , and underwent left bipolar hemiarthroplasty and left lateral rhinotomy for the nasal mass .
computed tomography ( ct ) imaging of the maxillofacial region revealed a rounded heterogeneous mass over the left side of the nasal dorsum and frontal process of the left maxilla .
bone marrow aspirate and biopsy were consistent with a diagnosis of multiple myeloma ( 36% plasma cells ) .
intra - operatively , a mass was found to be over the left side of the dorsum of the nose and the frontal process of the maxilla was eroded .
ct imaging of the maxillofacial region shows a heterogeneous mass over the left side of the nasal dorsum and frontal process of the left maxilla histopathological examination revealed a well - circumscribed neoplasm , predominantly made up of acellular eosinophilic material , arranged as nodules , with extensive calcification and ossification [ figure 2 , figure 3 ] .
increased number of osteoclast type of giant cells was noted admixed with the acellular material [ figure 4 ] .
occasionally individual and cords of squamoid cells were seen with nuclear pleomorphism [ figure 5 , figure 6 ] .
congo red stained the acellular material , confirming the amyloid nature [ figure 7 ] .
the acellular material was negative for cytokeratin stain by immunohistochemistry . with these features , a differential diagnosis of amyloidoma and ceot
the presence of extensive calcification , ossification , osteoclast type of giant cells , and occasional atypical squamoid cells , strongly favored a diagnosis of ceot .
photomicrograph showing acellular eosinophilic material arranged as nodules with ossifi cation ( h and e , 20 ) photomicrograph showing acellular eosinophilic material arranged as nodules with extensive calcifi cation ( h and e , 20 ) photomicrograph showing osteoclast type of giant cells admixed with acellular material ( h and e , 20 ) photomicrograph showing cords of squamoid cells with nuclear pleomorphism ( h and e , 20 ) photomicrograph showing cords of squamoid cells with nuclear pleomorphism and acellular material ( h and e , 40 ) photomicrograph showing acellular material arranged as nodules ( congo red , 20 )
previously , the uncertainty regarding the histological characteristics of ceot was reflected in the variety of terms for the disease , including unusual ameloblastoma , cystic odontoma , and adenoid adamantinoma .
pindborg tumor is a rare , benign , but locally aggressive odontogenic tumor , which accounts for less than 1% of all odontogenic tumors .
most investigators believe that the tumor cells originate from the stratum intermedium of the normal dental germ .
this idea is based on the morphological similarity of the tumor cells to the normal cells of the stratum intermedium , and high activity of alkaline phosphatase and adenosine triphosphate .
ceot occurs most commonly between 20 and 60 years of age with the mean age around 40 years .
most investigators agree that the central type is usually located in the premolar and molar regions with a mandibular to maxillary ratio of 2:1 or 3:1 .
the literature reports that this intraosseous tumor usually manifests as a painless swelling that causes slow bone expansion .
when located in the maxilla , patients may sometimes complain of nasal stuffiness , epistaxis , and headache .
few ( 5% ) extra osseous cases have been described , all of which have been involved peripherally in the anterior maxillary or mandibular gingiva.[79 ] fifty - two percent of the reported cases have been associated with an unerupted or embedded tooth . in the index case
the tumor most commonly appears as either a diffuse or a well - circumscribed unilocular radiolucent area . in some cases ,
the lesion becomes multilocular with a honeycomb pattern . in others , multiple radio - opacities
there is no doubt that the epithelial cells of ceot are of squamous epithelial origin , as is manifested by their morphological and immunohistochemical characteristics .
an amyloid deposit is well documented in association with squamous cell carcinoma at different locations namely ; skin , vagina , uterine cervix , and nasopharynx .
some reports have indicated that the squamous cell carcinoma associated with amyloid is immunoreactive for cytokeratin , and amyloid is regarded as the degradation product of keratin .
it is also known that in primary cutaneous amyloidosis , the amyloid is derived from filamentous degeneration of keratin filaments .
therefore , it is reasonable to regard all these findings , including ceot amyloid , as similar expressions of the same process , that is , filamentous degeneration of keratin and conversion into amyloid . with this view and
since pindborg tumor is an odontogenic tumor , one draws back to previous suggestions that the ceot amyloid is somehow connected to enamel formation , which is related to keratin .
another idea that emerges from the analysis of the cytokeratin staining pattern of amyloid is the concept of aging .
this thought is based on several observations , among which is the resemblance between the cytokeratin staining pattern of the amyloid and the epithelial gland - like structures of the tumor .
it seems that the tumor passes through various stages of development , commencing with epithelial degeneration and conversion of keratin filaments into amyloid . at first
, the amyloid is periodic acid - schiff - negative and stains positively for cytokeratin .
however as it ages and the amyloid deposits coalesce into globules , the amyloid loses its immunoreactivity for cytokeratin and becomes pas - positive . at this stage ,
the amyloid also mineralizes and gives rise to calcifications that exhibit the liesegang ring phenomenon .
the idea of aging has been proposed in the past , but it gains a new dimension now that it relies on more facts .
however the relative significance and contribution of keratin and basement membrane protein to the development of ceot - associated amyloid are still not clear .
it is for future studies to reveal this and other secrets concerning this rare and unique tumor .
the closest differential diagnosis of ceot in the present case is amyloidoma of the bone , which is a rare condition characterized by the massive destructive deposition of al amyloid in bones .
although extensive amyloid was present , plasma cells were not seen in the maxillary lesion .
this along with the other features , mentioned wide supra , favored a diagnosis of ceot .
the literature search depicts 200 cases of ceot in the jaw and only two cases in case of amyloidoma .
differences between ceot and amyloidoma the deposits in amyloidoma proved to be composed of al amyloid showing kmno4-resistant congophilia .
immunohistochemistry showed immunoglobulin igg ( lambda ) , igg ( kappa ) , and igm ( lambda ) monoclonality of the plasma cells , and lymphoid infiltrate .
the treatment for ceot has ranged from simple enucleation or curettage to radical and extensive resection , such as , hemimandibulectomy or hemimaxillectomy.in the present case lateral rhinotomy was done .
malignant behavior is extremely rare ; only two cases have been reported in the literature so far .
although it has not been established in the literature , five years should be the absolute minimum follow - up necessary to assess the behavior of this type of odontogenic tumor .
overall , a range of clinical , imaging , and histopathological findings should be used to help in the diagnosis of ceot and to evaluate its extension to different sites .
 calcifying epithelial odontogenic tumor ( ceot ) , also known as pindborg tumor , is a rare benign odontogenic tumor of locally aggressive behavior . 
 it is more common in the posterior part of the mandible of adults , typically in the fourth to fifth decades . 
 its origin as well as its true malignant potential is not clearly known . 
 it usually starts as a painless swelling and is often concurrent with an impacted tooth . 
 a case of ceot in a 55-year - old man with multiple myeloma is presented . 
 clinical , radiological , and pathologic findings are discussed . 
INTRODUCTION
CASE REPORT
DISCUSSION