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CASE REPORT
Ahead of print publication  

Primary intraosseous squamous cell carcinoma presenting as lytic lesion in mandible: Common presentation of an uncommon illness


 Department of Pathology, AFMC, Pune, Maharashtra, India

Date of Submission30-Apr-2022
Date of Decision08-Sep-2022
Date of Acceptance10-Oct-2022
Date of Web Publication30-Dec-2022

Correspondence Address:
Barun Kumar Chakrabarty,
Department of Pathology, AFMC, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmms.jmms_68_22

  Abstract 


Squamous cell carcinomas (SCCs) occurring in the bones are largely metastatic in nature and primary intraosseous squamous cell carcinomas (PIOSCCs) are extremely rare. They tend to involve skull bones more frequently than the other bones. We present the case of a 52-year-old male, presenting with pain in the lower jaw, which was diagnosed with PIOSCC on histopathology.

Keywords: Intraosseous, lower jaw, primary, squamous cell carcinoma



How to cite this URL:
Pendharkar C, Mehta R, Chakrabarty BK, Sen A. Primary intraosseous squamous cell carcinoma presenting as lytic lesion in mandible: Common presentation of an uncommon illness. J Mar Med Soc [Epub ahead of print] [cited 2023 Feb 6]. Available from: https://www.marinemedicalsociety.in/preprintarticle.asp?id=366393




  Introduction Top


Squamous cell carcinomas (SCCs) are a frequently occurring entity in the skin, oral cavity, and lung, but rarely involves the bones[1] due to the absence of native squamous epithelium within the bones.[2] Metastatic deposits of SCC within the bones are more common than primary intraosseous squamous cell carcinomas (PIOSCCs). The involvement of skull bones is noted more frequently than in other sites.[3] Being a rare disorder, PIOSCC is considered less frequently in the differential diagnosis of lesions of the mandible. However, considering its aggressive course, maintaining a high index of suspicion and performing biopsies proactively for definitive diagnosis enables the formulation of treatment strategies, which may help reduce morbidity. Our patient, a 52-year-old male, presented with complaints of pain in the lower jaw on the left side and was diagnosed as PIOSCC on histopathology.


  Case Report Top


A 52-year-old male, reported complaints of pain in the lower jaw on the left side, for 4 months. The pain was mild having a dull character and later increased progressively with time. On examination, the patient was found to be having restricted mouth opening, tenderness over the lower jaw on the left side, and palpable cervical lymph nodes at level IB present bilaterally. There was no ulceroproliferative lesion or precancerous lesion seen on the examination of the oral cavity and the mucosa appeared normal.

Orthopantomogram showed a radiolucent osteolytic lesion in the left posterior mandibular region, having irregular margins and multiple loculations [Figure 1]. The contrast-enhanced computed tomography of the face and neck revealed well-defined expansile lytic lesion measuring 32 mm × 18 mm × 34 mm, in the posterior part of the body and ramus of left hemimandible. Multiple discrete lymph nodes were noted in bilateral level IB stations. Based on this, the differential diagnosis of odontogenic keratocyst (OKC), ameloblastoma, and metastatic spread of unknown malignancy was considered.
Figure 1: Orthopantomogram shows osteolytic lesion with irregular margins in the left hemimandible on the posterior aspect of the body of the mandible extending into the ramus

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Whole-body positron emission tomography scan revealed a metabolically active expansile lytic lesion in the left hemimandible measuring 3.3 cm × 2.8 cm × 4.0 cm (SUV– 20.73). A biopsy was sent for the histopathological examination.

Multiple fragmented tissue bits labeled as the lining of the mandibular cyst were received, which showed dysplastic stratified squamous epithelium with subepithelium showing invading neoplastic cells in small islands and nests. These cells were large and polygonal with moderate to abundant eosinophilic cytoplasm, pleomorphic vesicular nuclei, and conspicuous nucleoli. Individual cell keratinization was noted in a few cells. Occasional keratin pearl formation was noted. Based on these histomorphological findings [Figure 2], the diagnosis of moderately differentiated keratinizing SCC was done.
Figure 2: Shows dysplastic stratified squamous lining of the mandibular cyst with infiltration of neoplastic cells into subepithelium, keratin pearl formation is evident (H and E, ×100)

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The patient underwent wide local excision and segmental madibulectomy (left) with modified radical neck dissection and placement of free fibula osteocutaneous flap.


  Discussion Top


PIOSCC is a rare entity, as metastatic SCCs are more common than the primary SCC of the bone.[2] The other histologic types of primary intraosseous carcinomas are salivary gland carcinomas and odontogenic carcinomas.[4] PIOSCC tends to involve skull bones, especially the mandible, where it is thought to arise from the remnants of the odontogenic epithelium.[5] PIOSCC in the mandible was first described by Loos.[6] Male preponderance is noted, wherein about 69.3% of the patients affected are males.[3] Our patient also showed this common presentation of the entity, which in itself is uncommon.

One of the famous medical maxims quotes, "uncommon presentations of the common illnesses are more common than the common presentations of uncommon illnesses." Hence, in our case, for the differential diagnosis of the osteolytic lesion of the mandible, OKC and ameloblastoma were considered as also the metastatic spread of unknown malignancy. PIOSCC, being rare, was not considered initially.

Although the histopathological examination alone cannot differentiate between the primary or metastatic SCC, there are subtle clues which may hint at the existence of one above the other. Gardner and Hampl noted that the presence of transition between normal and abnormal epithelia is one of the most important criteria for making a diagnosis of PIOSCC.[7],[8] Histopathologically, the neoplastic squamous cells should not be contiguous with the oral mucosa or mucosa of the maxillary sinus.[1]

The presence of fludeoxyglucose avid lesion only in the mandible, without any other metabolically lesion in the body, points toward the primary disease.[1] Apart from this, the following criteria are of help-(a) the histopathological examination should show SCC, without any odontogenic cyst or metastatic tumor cells, (b) the mucosa has to be intact, (c) at the time of initial evaluation and subsequent 6-month follow-up period, there should not be another distant primary tumor diagnosed.[9] As our patient fulfilled the above criteria, the diagnosis of PIOSCC was made.

The common presenting symptoms such as vague pain and paresthesias make practitioners misdiagnose this entity as a common dental problem,[10],[11] the radiological investigations may show the ill-defined bone destructive lesions, leading to further evaluation and diagnosis. The periapical and periodontal lesions may complicate the interpretation of radiological findings.[12],[13]

Surgery alone or in combination with radiotherapy is the most commonly used approach used to treat these cases.[14] The course of PIOSCC is aggressive with a 2-year mortality rate of 38% rising to 62% at the end of 5 years.[6],[15]


  Conclusion Top


PIOSCC is a rare entity, which tends to involve the jaw bone more frequently than the other bones. In cases presenting with the dull aching pain in the mandible, with presence of an osteolytic lesion, the differential diagnosis of PIOSCC must be taken into account, considering the poor prognosis of the disease. A high index of suspicion and the importance of proactive steps to take a biopsy for obtaining a definite diagnosis on histopathological examination are the key factors in tackling these cases with early institution of definitive treatment, the importance of which cannot be overemphasized.

Acknowledgments

The authors like to acknowledge the contributions of Dr Anup Kumar Singh, Department of OMFS and Dr Kshitij Srivastav, Department of Pathology, AFMC, Pune for the collection of relevant clinical information.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sukegawa S, Matsuzaki H, Katase N, Kanno T, Mandai T, Takahashi Y, et al. Primary intraosseous squamous cell carcinoma of the maxilla possibly arising from an infected residual cyst: A case report. Oncol Lett 2015;9:131-5.  Back to cited text no. 1
    
2.
Gaston CL, Vergel de Dios AM, Dela Rosa TL, Wang EH. Case report: Primary squamous cell carcinoma of a tarsal bone. Clin Orthop Relat Res 2009;467:3346-50.  Back to cited text no. 2
    
3.
de Morais EF, Carlan LM, de Farias Morais HG, Pinheiro JC, Martins HD, Barboza CA, et al. Primary intraosseous squamous cell carcinoma involving the jaw bones: A systematic review and update. Head Neck Pathol 2021;15:608-16.  Back to cited text no. 3
    
4.
Woolgar JA, Triantafyllou A, Ferlito A, Devaney KO, Lewis JS Jr., Rinaldo A, et al. Intraosseous carcinoma of the jaws: A clinicopathologic review. Part III: Primary intraosseous squamous cell carcinoma. Head Neck 2013;35:906-9.  Back to cited text no. 4
    
5.
Geetha P, Avinash Tejasvi ML, Babu BB, Bhayya H, Pavani D. Primary intraosseous carcinoma of the mandible: A clinicoradiographic view. J Cancer Res Ther 2015;11:651.  Back to cited text no. 5
    
6.
Bodner L, Manor E, Shear M, van der Waal I. Primary intraosseous squamous cell carcinoma arising in an odontogenic cyst: A clinicopathologic analysis of 116 reported cases. J Oral Pathol Med 2011;40:733-8.  Back to cited text no. 6
    
7.
Gardner AF. The odontogenic cyst as a potential carcinoma: A clinicopathologic appraisal. J Am Dent Assoc 1969;78:746-55.  Back to cited text no. 7
    
8.
Hampl PF, Harrigan WF. Squamous cell carcinoma possibly arising from an odontogenic cyst: Report of case. J Oral Surg 1973;31:359-62.  Back to cited text no. 8
    
9.
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. 9
    
10.
Thomas G, Sreelatha KT, Balan A, Ambika K. Primary intraosseous carcinoma of the mandible – A case report and review of the literature. Eur J Surg Oncol 2000;26:82-6.  Back to cited text no. 10
    
11.
Dimitrakopoulos I, Psomaderis K, Asimaki A, Papaemanouel S, Karakasis D. Primary de novo intraosseous carcinoma: Report of two cases. J Oral Maxillofac Surg 2005;63:1227-30.  Back to cited text no. 11
    
12.
Hwang EH, Choi YS, Lee SR. Primary intraosseous carcinoma of the mandible. Imaging Sci Dent 2005;35:235-9.  Back to cited text no. 12
    
13.
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. 13
    
14.
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. 14
    
15.
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. 15
    


    Figures

  [Figure 1], [Figure 2]



 

 
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