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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 312-314

Oral proliferative verrucous leukoplakia: An enigmatic lesion with emphasis on its differential diagnosis


1 Department of Oral Medicine and Radiology, SB Patil Dental College and Hospital, Humanabad Road, Naubad, Bidar, Karnataka, India
2 Private Practitioner, The Dental Specialists, Banjara Hills, Hyderabad, Telangana, India
3 Department of Oral Medicine and Radiology, Kamineni Institute of Dental Sciences, Narketpally, Telangana, India
4 Government Dental College, Hyderabad, Telangana, India

Date of Submission19-Mar-2020
Date of Decision19-May-2020
Date of Acceptance11-Jun-2020
Date of Web Publication29-Sep-2020

Correspondence Address:
Dr. M L Avinash Tejasvi
Professor, Department of Oral Medicine and Radiology, Kamineni Institute of Dental Sciences, Narketpally, Telangana - 508 254
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_44_20

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   Abstract 


Oral proliferative verrucous leukoplakia (OPVL) is a distinct clinical form of oral leukoplakia (OL). It is one of the rare clinicopathological entities that is considered as a potentially malignant oral mucosal disorder with an increased rate of progression to oral squamous cell carcinoma (OSCC) and a very high recurrence rate. Initially, the condition develops as focal clinical hyperkeratosis that progresses gradually and becomes a wide multifocal disease with gross exophytic features. It is a slow-growing, long-term progressive, and enigmatic lesion that is difficult to define and disclose. We report here, a case of OPVL in the right mandibular alveolar ridge, clinical and diagnostic aspects, differential diagnosis, and histologic features of this relatively rare entity.

Keywords: Malignant transformation, oral proliferative verrucous leukoplakia, premalignant disorder


How to cite this article:
Madki P, Khan R, Avinash Tejasvi M L, Javudi S. Oral proliferative verrucous leukoplakia: An enigmatic lesion with emphasis on its differential diagnosis. J Indian Acad Oral Med Radiol 2020;32:312-4

How to cite this URL:
Madki P, Khan R, Avinash Tejasvi M L, Javudi S. Oral proliferative verrucous leukoplakia: An enigmatic lesion with emphasis on its differential diagnosis. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 Oct 30];32:312-4. Available from: https://www.jiaomr.in/text.asp?2020/32/3/312/296583




   Introduction Top


Oral proliferative verrucous leukoplakia (OPVL) is described by WHO as a rare, but distinctive, high-risk clinical form of oral leukoplakia (OL) and was recently classified among potentially malignant disorders of the oral cavity.[1] OPVL was first described and segregated from other forms of leukoplakia by Hansen et al. in 1985.[2]

It has been described as a disease of unknown origin, and it remains a diagnostic challenge because individuals often lack the usual risk factors associated with OL and carcinoma.[3] The diagnosis is based on clinical and histologic features.

PVL grows slowly and is an irreversible process that usually progresses to cancer. Bagan et al., in their study, concluded that PVL quickly becomes malignant, on average within 4.7 years,[4] whereas Hansen et al. reported that it takes an average of 6.1 years for the progression of PVL into cancer.[2]


   Case Report Top


A 57-year-old female patient reported with a chief complaint of pain in her lower right back region of jaw since 10 days. History revealed that there was a gradual loosening of teeth followed by exfoliation in the same region 1 year ago. The patient then remained asymptomatic and now since the past one month, she noticed roughness in the same region which was associated with an intermittent, throbbing type of pain of and there was no habit history.

On extraoral examination, right submental and submandibular lymph nodes that were tender, soft, and movable. Intraorally, a solitary, sessile exophytic growth measuring 1 × 5 cm, ovoid in shape was seen in the right mandibular alveolar ridge region. The lesion was whitish pink and the surface appeared proliferative. The growth was tender [Figure 1].
Figure 1: Exophytic growth arising from the alveolar mucosa in the mandibular right posterior region

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Based on the above findings, a provisional diagnosis of proliferative verrucous leukoplakia was given and verrucous carcinoma and squamous cell carcinoma (SCC) were considered as the differential diagnosis. Panoramic radiograph showed mild cortical erosion anterior to 48 [Figure 2].
Figure 2: OPG showing mild cortical erosion anterior to 48

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Patient consent was taken and an incisional biopsy was performed [Figure 3] and the specimen was sent for histopathological examination.
Figure 3: Incisional biopsy

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Histological examination revealed parakeratinized stratified squamous epithelium exhibiting pseudoparakeratinized hyperplasia with focal epithelial entrapment at one place and bulbous rete ridges at other places. The epithelial surface revealed an exophytic growth pattern with parakeratin plugging and keratin pearl in few areas of the epithelium. The connective tissue consists of collagen bundles which are loosely arranged with extravasated, engorged blood vessels, and marked inflammatory infiltrates suggestive ofverrucous hyperplasia [Figure 4].
Figure 4: Photomicrographs illustrates OPVL showing Collagen bundles which are loosely arranged with extravasated, engorged blood vessels, and marked inflammatory infiltrates (scanner view)

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Based on the clinicopathological findings, proliferative verrucous leukoplakia was made as a final diagnosis. The patient was referred to the department of oral and maxillofacial surgery where cryosurgery was planned.


   Discussion Top


A rare form of nonhomogeneous OL known as OPVL is a progressive condition that behaves in an aggressive manner. These lesions initially develop as a white plaque of hyperkeratosis that may appear as solitary, flat homogeneously whitish-gray patches, and eventually become a multifocal disease with confluent, exophytic, and proliferative features.[5] It is not strongly associated with alcohol or tobacco use, but the possible etiology includes human papillomavirus (HPV), Epstein–Barr virus, and immunity.[6]

Considering the clinical presentation of a solitary, sessile tender exophytic, whitsh pink ovoid growth with a proliferative surface seen in the right mandibular alveolar ridge, a differential diagnosis of ---- were considered in the present case verrucous carcinoma, a papillary form of SCC, verruciform xanthoma, focal epithelial hyperplasia, and chronic hyperplastic candidiasis.

Verrucous carcinoma is a disease of slow-growing, painless, mostly seen in men >55 years of age with mandibular vestibule and buccal mucosa and hard palate the most common sites. Lesion appears as a diffuse, well-demarcated painless, thick plaque with papillary or verruciform surface projections. The lesions will be white but also may appear erythematous or pink. It also has papillary/verruciform projections and may develop from either OPVL or verrucous hyperplasia.

Verruciform xanthoma usually formed in the oral cavity, skin, and genitalia. Lesion appears as a well-demarcated, soft, painless sessile slightly elevated mass with a white, white-yellow, or red color and a papillary or roughened (verruciform) surface, and rarely flat-topped nodules are seen without surface projections. Most of the lesions are smaller than 4 cm in diameter. Multiple lesions are also seen occasionally.

Papillary squamous cell carcinoma rarely occurring variant of SCC may be due to HPV. The common site of occurrence will be in oropharyngeal, oral cavity, and larynx; they have distinctive exophytic and papillary features and a more favorable prognosis than conventional SCC.

Focal epithelial hyperplasia is a virus induced localized proliferation of oral squamous epithelium; usually, it is a childhood condition but can affect young and middle-aged adults also. Sites of occurrence include the labial buccal and lingual mucosa. They typically appear as multiple soft and non-tender, flattened, or rounded papules and are usually clustered and the color of normal mucosa, and also these lesions might appear pale, scattered, and white. Lesions also might have a papillary surface.

Hyperplastic candidiasis is clinically characterized by white plaques or patch-like appearance that cannot be removed by scraping that may have a verrucous appearance. The presence of candidal hyphae in this is confirmatory.[7]

Diagnosis of PVL is mainly based on a set of diagnostic criteria; one of the most widely used is the following Cerro Lapiedra et al.'s[8] major and minor criteria:

Major criteria

  1. A leukoplakia lesion with more than two different oral sites, which is most frequently found in the gingiva, alveolar processes, and palate
  2. The existence of a verrucous area
  3. The lesions have spread or engrossed during the development of the disease
  4. There has been a recurrence in a previously treated area
  5. Histopathologically, there can be from simple epithelial hyperkeratosis to verrucous hyperplasia, verrucous carcinoma, or OSCC, whether in situ or infiltrating.


Minor criteria

  1. An OL lesion that occupies at least 3 cm when adding all the affected areas.
  2. The patient should be female.
  3. The patient should be a nonsmoker.
  4. Disease evolution is higher than 5 years.


In order to make the diagnosis of PVL, one of the two following combinations of the criteria mentioned before should be met:

  1. Three major criteria (E being one among them) or
  2. Two major criteria (E being one of them) + two minor criteria.


Various treatment modalities for the management of these lesions include conventional surgery, radiotherapy, cryotherapy, vitamin A therapy, antiviral therapy, carbon dioxide laser surgery, and photodynamic therapy.[9]

OPVL has a high malignant transformation rate (60%–100%), recurrence rate (87%–100%), and high mortality rates (30%–50%).[10]


   Conclusion Top


OPVL is a rare, aggressive lesion that requires special awareness. As early detection is not straight forward as in our present case, care should be taken by proper clinical examination and follow-up of OPVL cases for a long time as it has a higher recurrence rate and malignant transformation.

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.
Warnakulasuriya S, Johnson NW, van der Waal I. Nomenclature and classification ofpotentially malignant disorders of the oral mucosa. J Oral Pathol Med 2007;36:575-80.  Back to cited text no. 1
    
2.
Hansen LS, Olson JA, Silverman S Jr. Proliferative verrucous leukoplakia. Oral Surg Oral Med Oral Pathol 1985;60:285-98.  Back to cited text no. 2
    
3.
Onofre MA, Sposto MR, Navarro CM, Motta ME, Turatti E, Almeida RT. Potentially malignant epithelial oral lesions: Discrepancies between clinical and histologic diagnosis. Oral Dis 1997;3:148-52.  Back to cited text no. 3
    
4.
Bagan JV, Jimenez Y, Sanchis JM, Poveda R, Milian MA, Murillo J,et al. Proliferative verrucous leukoplakia: High incidence of gingival squamous cell carcinoma. J Oral Pathol Med 2003;32:379-82.  Back to cited text no. 4
    
5.
Abadie WM, Partington EJ, Fowler CB, Schmalbach CE. Optimal management of proliferative verrucous leukoplakia: A systematic review of the literature. Otolaryngol Head Neck Surg 2015;153:504-11.  Back to cited text no. 5
    
6.
Ge L, Wu Y, Wu LY, Zhang L, Xie B, Zeng X,et al. Case report of rapidly progressive proliferative verrucous leukoplakia and a proposal for aetiology in mainland China. World J Surg Oncol 2011;9:26.  Back to cited text no. 6
    
7.
Neville BW, Damm DD, Allen CM, Chi AC. Oral & Maxillofacial Pathology. 4th ed. Missouri: WB Saunders, Elsevier; 2016. p. 604-5.  Back to cited text no. 7
    
8.
Cerero Lapiedra R, Balade Martinez D, Moreno Lopez LA, Esparza Gomez G, Bagán JV. Proliferative verrucous leukoplakia: A proposal for diagnostic criteria. Med Oral Patol Oral Cir Bucal 2010;15:e839-45.  Back to cited text no. 8
    
9.
Neville BW, Damm DD, Allen CM, Chi AC. Oral and Maxillofacial Pathology. 4th Edition, WB Saunders, Elsevier, Missouri 2016:604-5.  Back to cited text no. 9
    
10.
Kresty LA, Mallery SR, Knobloch TJ, Li J, Lloyd M, Casto BC, et al. Frequent alterations of p16INK4a and p14ARF in oral proliferative verrucous leukoplakia. Cancer Epidemiol Biomarkers Prev 2008;17:3179-87.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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