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 Table of Contents  
Year : 2014  |  Volume : 26  |  Issue : 2  |  Page : 182-186

Primary de novo intraosseous carcinoma of the mandible: A rare case report

1 Department of Oral Medicine and Radiology, Mamata Dental College and Hospital, Khammam, Telangana, India
2 Department of Oral Medicine and Radiology, Sri Sai College of Dental Surgery, Vikarabad, Telangana, India

Date of Submission11-Jun-2014
Date of Acceptance09-Jul-2014
Date of Web Publication30-Oct-2014

Correspondence Address:
Kotya Naik Maloth
H No 5-5-47/1, Mustafa Nagar, Khammam - 507001, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-1363.143696

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Primary intraosseous carcinoma (PIOC) is a very rare, but well-recognized entity, which is locally aggressive with quite poor prognosis. It may arise within the jaws either from a previous odontogenic cyst or de novo, rather than from a pre-existing epithelial lesion. A case of PIOC arising in the mandible of a 63-year-old male patient is reported in this article, with the clinical, radiological, and histological features described. This rare lesion must be considered in any differential diagnosis of jaw radiolucency.

Keywords: Central epidermoid carcinoma, odontogenic carcinoma, primary intraosseous carcinoma

How to cite this article:
Maloth KN, Reddy Kundoor VK, kodangal S, Meka N. Primary de novo intraosseous carcinoma of the mandible: A rare case report . J Indian Acad Oral Med Radiol 2014;26:182-6

How to cite this URL:
Maloth KN, Reddy Kundoor VK, kodangal S, Meka N. Primary de novo intraosseous carcinoma of the mandible: A rare case report . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2020 Dec 5];26:182-6. Available from: https://www.jiaomr.in/text.asp?2014/26/2/182/143696

   Introduction Top

Primary Intraosseous Carcinoma (PIOC) is a rare malignant neoplasm of the jaws, which is locally aggressive, with quite poor prognosis. About 150 cases of PIOC have been documented to date. It was first described by Loos in 1913, as a central epidermoid carcinoma of the jaw. Wills, in 1948, renamed it as an intra-alveolar epidermoid carcinoma. Later, Pindborg, in 1971, coined the term primary intraosseous carcinoma (PIOC). [1],[2] According to the World Health Organization (WHO), PIOC is defined as, "A squamous cell carcinoma arising within the jaw, having no initial connection with the oral mucosa and presumably developing from residues of the odontogenic epithelium". [3] Hence, it is also known as odontogenic carcinoma. It may arise within the jaws either from a previous odontogenic cyst or de novo, rather than from a pre-existing epithelial lesion. [4] Primary intraosseous squamous cell carcinoma (PIOSCC) includes two different entities: PIOC and malignant change in the odontogenic cysts. After Elzay's review in 1982, [5] these odontogenic carcinomas were considered together. PIOC is now critically defined and separated from the carcinoma arising from an odontogenic cyst. Establishing a diagnosis of PIOC is often difficult, as the lesion must be differentiated from a tumor that metastasizes to the jaws from distant sites, and from gingival carcinomas that have invaded the bone from the surface. [6] Careful investigation of the systemic condition and inspection of the intraoral condition, along with a chest radiograph and bone scan are necessary to rule out metastatic carcinoma. The following strict and established criteria for diagnosing a lesion such as PIOC have been proposed by Suie et al.: (a) An intact oral mucosa prior to diagnosis, except due to trauma or tooth extraction. (b) Squamous cell carcinoma with no histological evidence of associated odontogenic cysts. (c) Ruling out a metastatic deposit from a distant primary at the time of diagnosis or during a follow-up period of more than six months. [6],[7] Asserting on the rarity of this lesion, here is a case report of PIOC de novo in the posterior region of the mandible, in a 63-year-old male patient, and the present article also reviews the existing literature.

   Case Report Top

A 63-year-old male patient reported to our Outpatient Department with a chief complaint of pain and swelling in the left lower back teeth region, since three months [Figure 1]. The pain was intermittent, shooting type, which aggravated on mastication and was relieved upon taking medication. The patient had undergone extraction on a previous dental visit in the left lower back teeth region, three months prior. However, the swelling persisted and grew progressively. There was no relevant habit history. On extraoral examination there was gross facial asymmetry, with diffuse swelling on the left side of the face, measuring approximately 2 × 3 cm in size, extending superoinferiorly from the tragus of the ear to the angle of the mandible, and anteroposterior from the corner of the mouth to the lobe of the ear. The overlying skin was similar to the surrounding skin. No visible pulsations and discharge were seen. On palpation the swelling was tender; no local rise of temperature was felt. It was noncompressible, nonreducible, and nonpulsatile. A single left submandibular lymph node was noted, which was hard in consistency, tender, measuring approximately 1 × 1 cm in size, and fixed to the underlying structures. On intraoral examination, partially edentulous upper and lower arches were seen with teeth present in relation to 18 - 11, 21 - 25, 33 - 31, 41, 42, 48 (root stumps in relation to 18) and a nonhealing socket, with a proliferative growth was seen at the distal margins of the lower left back edentulous 37, 38 region, measuring approximately 2 × 2 cm in size. The unhealed socket extended anteroposteriorly from the 36 region to the retromolar region and mediolaterally from the edentulous alveolar mucosa in relation to 37, 38, to approximately 1 cm lateral to it [Figure 2]. The distal surface of the unhealed socket appeared pebbly, raised, and erythematous. No bleeding or discharge was seen. On palpation the lesion was tender and showed proliferative growth of the unhealed socket, with indurated borders. Bleeding was present on palpation. On the basis of the history and clinical findings, a provisional diagnosis of carcinoma of the left alveolus in relation to the 37, 38 region was made. Differential diagnosis was given as chronic nonhealing ulcer, epulis granulomatosa, pyogenic granuloma, irritation fibroma, intra-alveolar carcinoma.
Figure 1: Extraoral view

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Figure 2: Intraoral view

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Routine investigations like a complete blood picture, blood glucose levels, human immunodeficiency virus (HIV), and Hepatitis B screening were performed, which showed no abnormality. The following radiographs were taken: Lateral oblique view of the mandible, orthopantomogram (OPG), chest radiography, and computed tomography (CT) scan.

The lateral oblique view of the mandible showed a solitary, ill-defined radiolucency involving the body, angle, and anterior border of the ramus of the mandible [Figure 3]. The OPG revealed partially edentulous arches in both the maxilla and mandible, with a solitary, irregular radiolucency involving the body of the mandible at the 36, 37, 38 edentulous region, from the angle, extending into the ramus, up to the sigmoid notch of the mandible on the left side. There was irregular destruction of the anterior border of the ramus and inferior border of the mandible [Figure 4]. Computed tomography showed irregular bone destruction in the body of the mandible and ramus on the left side [Figure 5]. The chest radiograph showed no abnormality [Figure 6]. A radiological differential diagnosis considered it to be a residual periapical cyst, malignant odontogenic tumor, ameloblastic carcinoma, metastatic carcinoma, and mucoepidermoid carcinoma.
Figure 3: Lateral oblique view

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Figure 4: Orthopantomographic view

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Figure 5: Coronal and axial views of computed tomography

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Figure 6: Chest x-ray image

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Incisional biopsy was performed. The histopathological features showed dysplastic epithelial cells invading the connective tissue stroma, abundant individual cell keratinization and keratin pearls, suggesting it to be a well-differentiated squamous cell carcinoma [Figure 7].
Figure 7: Histopathological picture

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The patient underwent surgical resection of the left body and ramus of the mandible [Figure 8] and reconstruction of the same was done with a costochondral homograft. The patient is currently kept under follow-up, for further evaluation.
Figure 8: Postoperative intraoral view

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   Discussion Top

Primary Intraosseous Carcinoma is a rare malignant neoplasm of the jaw with a low prognostic index. This tumor is believed to arise from the odontogenic epithelium, and hence, is also referred to as odontogenic carcinoma. [2] The incidence of this lesion is very low. A review of all published cases in the English language literature, by To et al., [8] revealed only 21 well-documented cases of PIOC as de novo. Hence, owing to the rarity of the reported cases, we tried to elucidate a case similar to this with a review of literature. Age, sex, and location predilection reported by Shear, McGowaan, and Elzay, indicate that the tumor occurs mainly in adults in the sixth to seventh decade of life, with a male to female ratio of 3:1, and is usually situated in the posterior mandible, [9] as in our case, except in a case reported by Coonar, [10] where the PIOC occurred in the anterior maxilla. Also in a review of 200 cases, reported up to 2010, Nomura et al., [11] reported only eight cases (4%) of solid type PIOSCC in the maxilla. In 2011, Mohyuddin and Yao [12] reported a new case of this tumor in the maxilla followed by Lino et al., [13] where PIOC occurred in the anterior maxilla. The etiology of PIOC is not clear. However, the most common factor may be reactive inflammatory stimulus subsequent to traumatic surgical procedures, with or without a predisposing genetic cofactor, similar to our case, where the patient complained of teeth extraction, three months prior. [14]

The most common clinical features in PIOC include pain and swelling of the affected area. In a pooled analysis of 33 cases recorded in world literature, performed by Thomas et al., [2] pain was the most common feature, present in 17 (54.8%) patients, followed by swelling of the jaw in 16 (51.6%), and sensory disturbance in five (16.1%) patients. In few cases, the patients had a history of a prior dental procedure, when attempting to resolve the symptoms associated with the neoplasm. [8] The diagnosis of PIOC was difficult, partly because the initial symptoms were thought to be of dental origin, [15] which resulted in a delayed diagnosis from a few weeks to as long as 18 months. [8] In several cases teeth were extracted as the initial treatment, [16],[17] as in our case.

The primary intraosseous carcinomas of the jaws are classified, based on their possible origins, into four types:

Type 1: PIOC Ex-odontogenic cysts

Type 2: PIOC Ex-ameloblastoma

a: Malignant ameloblastoma

b: Ameloblastic carcinoma

Type 3: PIOC arising de novo

a: Keratinizing

b: Non-keratinizing

Type 4: Intraosseous mucoepidermoid carcinoma.

According to this classification our case belongs to Type 3 (a) PIOC.

Radiographic examination is one of the most effective means of detecting PIOC. [7] However, PIOC shows great variation in size and shape, and in the appearance of its border. [6] Nolan [18] reported that PIOCs that grew slowly usually had a well-defined, rather smoothly contoured border, while those that grew more rapidly showed a poorly defined, ragged border. In our case the lesion had an ill-defined, unilocular radiolucency, with ragged borders. A PIOC can be one of the differential diagnosis for radiolucent lesions of the jaw bones and the lesion that is radiographically demonstrated to be entirely surrounded by bone can be considered to be of intraosseous origin, although, multiple radiographs are necessary to confirm that the lesion is in fact inside the bone. Although the role of a CT scan and magnetic resonance imaging (MRI) in PIOC is not clear, the CT scan of the head and neck may help in delineating the lesion and may also help in demonstrating soft tissue infiltration. [2]

Histologically, they vary from well-differentiated tumors exhibiting significant keratinization to non-keratinizing poorly differentiated carcinomas. Yamada et al., [19] in his clinicopathological study of PIOC found three cases of well-differentiated carcinomas and one moderately differentiated carcinoma; all these three had arisen de novo and the other one had arisen from an odontogenic cyst. In our case, the tumor was a well-differentiated squamous cell carcinoma, with no evidence of any odontogenic cystic component.

Around 66% of the patients with de novo PIOC has clinical and/or histological evidence of regional metastasis, either initially or during the course of the disease. [10] Metastasis to the regional lymph nodes, at the time of presentation, has been seen in 31.4% of the cases, in the analysis made by Thomas et al. [2] Metastatic spread to the cervical lymph nodes has been discussed by Elzay [5] and Muller and Waldron. [15] It is important to rule out metastasis to the jaws in cases of suspected PIOC. As a majority of metastatic squamous cell carcinomas to the jaws arise within the lungs, it is necessary to evaluate the patient thoroughly, including chest radiography, in an attempt to detect any occult primary tumor. Ideally a bone scan should be performed to locate the primary or the metastatic lesions. On account of the patient's financial constraints, higher investigations could not be performed. We had to rely on the chest radiograph alone.

Our case fulfilled almost all the criteria suggested by Suei et al., [6] and moreover, there was no evidence of any cystic component histologically, suggesting that our case was a primary de novo intraosseous keratinizing squamous cell carcinoma.

Primary Intraosseous Carcinomas are currently managed by wide surgical resection with a safety margin. Other treatment modalities, such as, radiotherapy or chemotherapy, should be considered only for lesions that cannot be controlled by surgery. [2] However, no standard indications or protocols for radio- or chemoradiotherapy have been established. [20]

The prognosis of PIOC is quite poor and importance should be given to early diagnosis so that suitable treatment can be given at the earliest. [2] In the 12 cases of PIOC reported by Elzay, [5] a 40% two-year survival was noted. Sixty-six percent of these patients had regional metastasis. Similarly, Thomas et al. reported that the overall survival rate of one, two, and three years were 75.7%, 62.1%, and 37.8%, respectively, and in a review of 28 cases of PIOC reported by To et al., [8] 46% of the patients survived for a period varying from six months to five years.

   Conclusion Top

The importance of this case was that it illustrated the central origin of the tumor, which presented as a dental problem, and it was a relatively rapidly growing tumor. [9] The diagnosis of PIOC is rare, but it is often worth considering in any differential diagnosis of a jaw radiolucency. We would advocate that any radiolucency be either biopsied at an early stage or be followed up closely, with regular radiographs. [8] The prognosis of PIOC is quite poor and importance should be given to early diagnosis, so that suitable treatment can be given at the earliest.

   References Top

Shambhulingappa P, Sheikh S, Puri N, Jindal SK. Primary intraosseous carcinoma of mandible: An update on review of literature with a case report. J Clin Exp Dent 2010;2:e91-5.  Back to cited text no. 1
Thomas G, Pandey M, Mathew A, Abraham EK, Francis A, Somanathan T, et al. Primary intraosseous carcinoma of the jaw: Pooled analysis of world literature and report of two new cases. Int J Oral Maxillofac Surg 2001;30:349-55.  Back to cited text no. 2
Kramer IR, Pindborg JJ, Shear M. Histological Typing of Odontogenic Tumours (International histological classification of tumours). 2 nd ed. Berlin: Springer-Verlag; 1992. p. 24-7.  Back to cited text no. 3
Chaisuparat R, Coletti D, Kolokythas A, Ord RA, Nikitakis NG. Primary intraosseous odontogenic carcinoma arising in an odontogenic cyst or de novo: A clinicopathologic study of six new cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:194-200.  Back to cited text no. 4
Elzay RP. Primary intraosseous carcinoma of the jaws. Review and update of odontogenic carcinomas. Oral Surg Oral Med Oral Pathol 1982;54:299-303.  Back to cited text no. 5
Suei Y, Tanimoto K, Taguchi A, Wada T. Primary intraosseous carcinoma: Review of the literature and diagnostic criteria. J Oral Maxillofac Surg 1994;52:580-3.  Back to cited text no. 6
Kaffe I, Ardekian L, Peled M, Machtey E, Laufer D. Radiological features of primary intra-osseous carcinoma of the jaws. Analysis of the literature and report of a new case. Dentomaxillofac Radiol 1998;27:209-14.  Back to cited text no. 7
To EH, Brown JS, Avery BS, Ward-Booth RP. Primary intraosseous carcinoma of the jaws. Three new cases and a review of the literature. Br J Oral Maxillofac Surg 1991;29:19-25.  Back to cited text no. 8
Van Wyk CW, Padayachee A, Nortjé CJ, vonder Heyden U. Primary intraosseous carcinoma involving the anterior mandible. Br J Oral Maxillofac Surg 1987;25:427-32.  Back to cited text no. 9
Coonar HS. Primary intraosseous carcinoma of maxilla. Br Dent J 1979;147:47-8.  Back to cited text no. 10
Nomura T, Monobe H, Tamaruya N, Kishihsita S, Saito K, Miyomoto R, et al. Primary intraosseous squamous cell carcinoma of the jaw: Two new cases and review of the literature. Eur Arch Otorhinolaryngol 2013;270:375-9.  Back to cited text no. 11
Mohyuddin N, Yao M. Primary intraosseous carcinoma of the anterior maxilla: An unusual case and review of the literature. Ear Nose Throat J 2011;90:E35-7.  Back to cited text no. 12
Iino M, Ishikawa S, Ozaki H, Kobayashi T, Tachibana H, Sakurai H, et al. Solid type primary intraosseous squamous cell carcinoma in the maxilla: Report of a new case. BMC Ear Nose Throat Disord 2013;13:13.  Back to cited text no. 13
Punnya A, Kumar GS, Rekha K, Vandana R. Primary intraosseous odontogenic carcinoma with osteoid/dentinoid formation. J Oral Pathol Med 2004;33:121-4.  Back to cited text no. 14
Waldron CA, Mustoe TA. Primary intraosseous carcinoma of the mandible with probable origin in an odontogenic cyst. Oral Surg Oral Med Oral Pathol 1989;67:716-24.  Back to cited text no. 15
McGowan RH. Primary intra-alveolar carcinoma. A difficult diagnosis. Br J Oral Surg 1980;18:259-65.  Back to cited text no. 16
De Lathouwer C, Verhest A. Malignant primary intraosseous carcinoma of the mandible. Oral Surg Oral Med Oral Pathol 1974;37:77-83.  Back to cited text no. 17
Nolan R, Wood NK. Central squamous cell carcinoma of the mandible: Report of case. J Oral Surg 1976;34:260-4.  Back to cited text no. 18
Yamada T, Ueno T, Moritani N, Mishima K, Hirata A, Matsumura T. Primary intraosseous squamous cell carcinomas: Five new clinicopathologic case studies. J Craniomaxillofac Surg 2009;37:448-53.  Back to cited text no. 19
Lugakingira M, Pytynia K, Kolokythas A, Miloro M. Primary intraosseous carcinoma of the mandible: Case report and review of the literature. J Oral Maxillofac Surg 2010;68:2623-9.  Back to cited text no. 20


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]


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