Odontogenic Keratocysts in the Anterior Midline Region: A Case Series and Literature Review

ABSTRACT

This case series describes three midline odontogenic keratocysts (OKCs) and reviews the associated literature. All three patients were male; two lesions were maxillary. All lesions were surgically excised, with no recurrence. A search of PubMed, Embase, and Scopus identified 33 reported cases of midline OKC, including this series. Of these, 64% (21/33) were male, and age ranged from 6 to 80 years (mean 39.8 ± 20.3). The mandible was affected in 67% (22/33). Excision was the predominant treatment (94%, 31/33), with adjunctive cauterization in 30% (10/33) and decompression in 15% (5/33). Among cases with available follow‐up, one recurrence was reported. OKC should be considered in the differential diagnosis of midline jaw lesions, supported by combined clinical, radiographic, and histopathological assessment.

Key Clinical Message

Odontogenic keratocysts can occur in the anterior jaws and be associated with teeth roots, and are an important differential diagnosis for other intraosseous conditions.

1. Introduction

The odontogenic keratocyst (OKC) is an odontogenic cyst characterized by a stratified squamous parakeratinized epithelial lining and a locally infiltrative growth pattern with a marked tendency toward recurrence [1]. It is most commonly diagnosed between the second and fourth decades of life, with a slight male predominance and a predilection for the posterior mandible [1]. OKC is uncommon in the anterior region of the jaws [2].

When OKCs arise outside their typical location, particularly in the midline, their clinical and radiographic presentation may closely mimic a radicular cyst [3, 4, 5, 6], nasopalatine duct cyst [7], and residual cyst [2]. Such presentations remain sparsely documented in the literature and are often reported as isolated cases, limiting comprehensive understanding of their behavior and diagnostic implications.

This study reports three cases of midline OKC, defined as an OKC centered on the anatomical midline or extending across it, involving both sides of the maxilla or mandible. These cases pose diagnostic challenges due to their uncommon clinical and radiographic characteristics.

2. Case History

2.1. Case Report 1

A 54‐year‐old Caucasian man presented with anterior maxillary swelling. Upon physical examination, symmetrical normochromic swelling was observed in the vestibular fornix associated with the apical region of teeth #8 and #9.

Cone beam computed tomography (CBCT) revealed a unilocular hypodense area associated with the roots of central maxillary incisors. The alveolar cortical bone was disrupted, and the dental apex was resorbed. Nasopalatine duct communication and vestibular cortical discontinuity were also observed (Figure 1a–c).

FIGURE 1.

(a–c) Cone beam computed tomography of the anterior maxilla (coronal, sagittal, and axial sections) showing discontinuity of the vestibular cortical bone, communication with the nasopalatine duct, and disruption of the lamina dura. (d) Fine‐needle aspiration revealing yellowish‐white viscous content with blood in the syringe. (e, f) Histological sections demonstrating a thin stratified squamous epithelial lining with corrugated parakeratin and a palisaded basal layer with flattened epithelial ridges. The lamina propria consists of fibrous connective tissue with fibroblasts and sparse mononuclear inflammatory cells (×200 and ×400, hematoxylin and eosin).

Teeth #8 and #9 were nonvital and underwent endodontic treatment. However, no remission of the periapical lesion was observed. Fine‐needle aspiration (FNA) revealed a yellowish‐white viscous fluid with blood (Figure 1d). The lesion was surgically enucleated with curettage.

Microscopically, a cystic cavity lined by a thin parakeratinized stratified squamous epithelium was observed, with surface corrugation and a basal layer of palisaded cells. Additionally, the cyst wall exhibited a fibrous capsule containing epithelial islands, along with a mild inflammatory infiltrate (Figure 1e,f). The final diagnosis of an OKC was made. The patient is under follow‐up for 29 months without signs of recurrence.

2.2. Case Report 2

A 57‐year‐old male patient was referred for evaluation of a maxillary periapical radiolucent image identified in a routine radiographic exam. CBCT revealed a well‐defined, unilocular, hypodense image, located in the midline, between the roots of the maxillary central incisors, which were endodontically treated (Figure 2). The lesion caused displacement of the roots (Figure 2a), expansion of the palatine cortical plate, and discontinuity of the buccal cortical plate were also observed (Figure 2b,c). The patient reported no associated symptoms. Based on the diagnostic hypotheses of nasopalatine duct cyst and radicular cyst, surgical enucleation with curettage was performed. Histopathological analysis showed the same features observed in Case 1. Thus, the diagnosis of OKC was established (Figure 2d,e). After 2 years of treatment, no imaging signs of recurrence were observed.

FIGURE 2.

(a) Panoramic radiograph showing a well‐circumscribed radiolucent lesion displacing the roots of teeth #8 and #9. (b–c) Axial cone beam computed tomography demonstrating a well‐defined hypodense lesion between the maxillary central incisors with cortical expansion and focal perforation; panel b shows communication with the nasopalatine duct (CNP). (d) Histologic section showing a cystic lesion with luminal keratin, epithelial lining, and a vascularized connective tissue capsule (×50). (e) Higher magnification revealing thin stratified squamous epithelium with corrugated parakeratin and a palisaded basal layer (×200, hematoxylin and eosin).

2.3. Case Report 3

A 36‐year‐old man presented mobility and pressure sensation in teeth #24 and #25. Clinical examination confirmed the increased mobility in teeth #24 and #25, with a painless firm swelling on the mandibular anterior lingual surface. Thermal pulp sensitivity tests were positive in both teeth. Panoramic radiograph and CBCT showed a well‐defined unilocular midline mandibular lesion between the central incisors, causing root displacement and cortical resorption, with thin, mildly expanded buccal and lingual plates (Figure 3a–c). Based on the diagnostic hypotheses of a lateral periodontal cyst and OKC, surgical enucleation was performed, followed by peripheral costectomy. Histopathological analysis revealed the same features observed in Cases 1 and 2 (Figure 3d,e), confirming the diagnosis of OKC. No clinical or imaging signs of recurrence were observed 8 years after treatment.

FIGURE 3.

(a) Panoramic radiograph demonstrating a well‐circumscribed radiolucent lesion between the roots of teeth #24 and #25 with root displacement. (b, c) Axial cone beam computed tomography showing a hypodense lesion with anteroposterior cortical expansion. (d) Low‐power histologic view of a cystic lesion with fibrous capsule, epithelial lining, and luminal content (×20). (e) Higher magnification showing stratified squamous epithelium with corrugated parakeratin and palisaded hyperchromatic basal cells; the connective tissue capsule contains fibroblasts and capillaries (×400, hematoxylin and eosin).

3. Methods

3.1. Patient Consent

All 3 patients presented in this case series provided informed written consent for the use of clinical images, information, and biological material for research and publication purposes.

3.2. Literature Review

A literature search was performed in November 2025 for midline OKC in PubMed, Embase, and Scopus using the following strategy: (“Odontogenic Cyst” OR “Odontogenic Cysts” OR Odontogenic‐Cyst OR Odontogenic‐Cysts OR Keratocyst OR Keratocysts OR Keratocystic OR “Keratocystic Odontogenic Tumor” OR “Keratocystic Odontogenic Tumors” OR “Odontogenic Keratocyst” OR “Odontogenic Keratocysts”) AND ((mandible OR mandibular OR mandibula) AND (anterior OR symphysis OR symphyseal OR “incisor region” OR chin) OR (maxilla OR maxillary OR maxillar) AND (anterior OR “incisor region” OR “alveolar process” OR alveolar)).

Only case reports describing anterior OKCs centered on the anatomical midline or extending across it and involving both sides of the maxilla or mandible, with available epidemiological information were included in the review. The search was filtered by article type for case reports, reviews, and systematic reviews without language or publication date restriction.

A total of 1152 records were identified: PubMed (n = 346), Embase (n = 321), and Scopus (n = 485). After title and abstract screening, 64 records underwent full‐text assessment. Of these, 16 duplicates, 15 ineligible studies, and 4 studies with unavailable full texts were excluded (Figure 4). A total of 29 studies remained eligible. One study reported 2 cases, resulting in 30 cases; together with the three additional cases from this series, 33 cases are presented in Table 1.

FIGURE 4.

Literature review flowchart of included and excluded studies.

TABLE 1.

Cases of midline odontogenic keratocysts published in the reviewed literature, including the current cases.

Article	Gender	Age (years)	Site	Treatment	Follow‐up (months)
Case 1	M	54	Maxilla	Excision with curettage	29
Case 2	M	57	Maxilla	Excision	24
Case 3	M	36	Mandible	Excision with peripheral ostectomy	96
Hayashi et al., 2025 [27]	M	73	Mandible	Excision	0
Khruomo et al., 2025 [8]	M	22	Mandible	Excision with peripheral ostectomy and chemical cauterization	22
Ma et al., 2025 [9]	F	21	Mandible	Excision with curettage	66
Ravi et al., 2025 [10]	M	70	Mandible	Excision	12
Suhaim et al., 2025 [11]	M	31	Mandible	Decompression	84
Upadhyaya et al., 2025 [12]	F	14	Mandible	Excision	6
Pirade et al., 2022 [21]	F	36	Maxilla	Maxillectomy	12
Grover et al., 2024 [25]	M	37	Mandible	Excision	Na
Singh et al., 2024 [13]	M	42	Maxilla	Excision with chemical cauterization	12
Barrow et al., 2024 [14]	F	14	Mandible	Excision	3
Nelke et al., 2023 [15]	M	62	Mandible	Excision	48
Chaves et al., 2021 [28]	M	33	Mandible	Excision	Na
Lunawat et al., 2020 [2]	M	80	Maxilla	Excision with electrical cauterization	3
Menon et al., 2020 [5]	M	36	Mandible	Excision with curettage and chemical cauterization	18
Prashanth et al., 2020 [6]	M	29	Mandible	Excision with chemical cauterization	18
Rodrigues et al., 2020 [26]	F	63	Mandible	Excision	0
Zamare et al., 2020 [24]	F	24	Maxilla and Mandible	Excision and peripheral ostectomy	1
Khan et al., 2019 [29]	F	35	Mandible	Decompression followed by excision with chemical cauterization	120
Medawela et al., 2017 [16]	F	50	Mandible	Excision	18
Satappa et al., 2017 [4]	M	45	Maxilla	Excision with curettage and chemical cauterization	Na
Gurugubelli et al., 2016 [17]	F	60	Mandible	Excision and oncologic treatment	Na
Garde et al., 2012 [23]	M	25	Mandible	Decompression followed by excision with curettage and chemical cauterization	36
Rajkumar et al., 2011 [18]	F	11	Mandible	Excision	9
Thamizhchelvan et al., 2011 [22]	F	27	Maxilla	Excision with chemical cauterization	Na
Veena et al., 2011 [3]	M	25	Mandible	Excision	18
Basile et al., 2010 [19]	M	44	Mandible	Decompression and excision	Na
Sulabha et al., 2013 [30]	F	11	Mandible	Excision with chemical cauterization	12
Chiang et al., 2006 [7]	M	6	Maxilla	Transnasal endoscopic decompression	Na
Woo et al., 1987 [20]	M	66	Maxilla	Excision	Na
	M	75	Maxilla	Excision	Na

Abbreviations: F, female; M, male; Na, information not available.

4. Results

Among the 33 reported cases of midline OKC, including the present series, most patients were male (21/33; 64%). Patient age ranged from 6 to 80 years (mean, 39.8 ± 20.3 years), indicating a broad demographic distribution. Cases 1 and 2 involved the maxilla, highlighting the unusual nature of this case series, given that most midline OKC reported in the literature affected the mandible (22/33; 67%). Surgical excision predominated as the treatment modality (31/33; 94%), with adjunct chemical or electrical cauterization in 30% (10/33) of cases, and decompression was performed in advanced lesions, comprising 15% (5/33) of all cases. Follow‐up was reported in 24 cases (73%), ranging from 1 to 120 months (median 18), with only 4 (12%) followed for 60 months or more [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30]. Recurrence was documented in one case, with a 66‐month follow‐up [9].

5. Discussion

As a locally infiltrative condition, OKC usually grows along medullary spaces, causing bulging of the buccal cortical plate, as presented in Case 3, without dental root resorption, communication with adjacent structures, or cortical perforation [10, 18, 30]. Although cortical perforation and infiltrative growth are not exclusive to OKC, their presence in an unusual anatomical location, as observed in Cases 1 and 2, deviates from these patterns reported in the literature [6, 30], leading to the hypothesis of a radicular cyst or nasopalatine duct cyst.

Beyond the radiographic and cortical findings, in most cases, OKC does not result in pulp vitality loss [19, 20]. However, when located in the periapex, it can lead to pulp necrosis by compromising the blood supply [31]. Among the analyzed reports, 5 (15%) cases exhibited loss of tooth vitality associated with an OKC located in the periapical region [3, 4, 5, 8].

Histopathological examination is mandatory for definitive diagnosis, particularly in atypical presentations. The microscopic features of an OKC were confirmed in all cases; additionally, the presence of satellite cysts within the cyst wall was identified, a finding that is associated with clinically aggressive behavior and an increased risk of recurrence [1, 9]. Moreover, the locally infiltrative growth pattern of these lesions adds complexity to clinical management, particularly when lesions are located near critical facial structures [7].

Surgical excision with peripheral ostectomy, or marsupialization followed by enucleation, is the standard treatment of OKCs [32]. Although chemical cauterization has been proposed after a surgical procedure, it was not performed in our cases due to the risk of paresthesia and wound dehiscence. Where cortical discontinuity placed the cyst capsule in direct contact with soft tissue, careful dissection ensured complete enucleation. All cases were treated by surgical enucleation. In Cases 1 and 2, the involved teeth had been endodontically treated prior to the OKC diagnosis.

Midline OKC is rare but clinically significant, and can present with cortical perforation and pulp vitality loss, increasing misdiagnosis risk. This case series highlights the midline location of the OKC, with cortical perforation in all three cases and dental vitality loss in two. An integrated approach with clinical, radiographic, and histopathological evaluation, combined with long‐term follow‐up, remains essential for accurate diagnosis and recurrence prevention. The variable follow‐up duration and the absence of follow‐up information in several cases may limit detection and estimation of recurrence rates. Treatment frequencies are presented as descriptive findings from the published reports, without implying comparative treatment effectiveness.

Author Contributions

Isabella Souza Malta: conceptualization, methodology, investigation, data curation, writing – original draft, writing – review and editing, formal analysis. Bruno de Albuquerque Hayasida: data curation, investigation, writing – review and editing. Ana Lia Anbinder: investigation, data curation, writing – original draft. Elaine Judite de Amorim Carvalho: investigation, data curation, writing – review and editing. Marcelo Marotta Araújo: investigation, data curation, writing – review and editing. André Caroli Rocha: writing – review and editing. Danyel Elias da Cruz Perez: conceptualization, methodology, writing – original draft, supervision. Estela Kaminagakura: supervision, writing – original draft, conceptualization, methodology.

Funding

We acknowledge the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior—Brasil (CAPES) for the Master's scholarship granted to Isabella Souza Malta, Funding Code 88887.209472/2025‐00.

Disclosure

Case series: This series comprises 3 new reports and 30 cases retrieved from the literature, completing a total of 33 cases.

Ethics Statement

Formal approval was obtained from the institutional Ethics Committee for this retrospective case series (CAAE: 00210826.8.0000.0077). The patients provided informed written consent for the use of clinical images, information, and biological material for research and publication purposes.

Conflicts of Interest

The authors declare no conflicts of interest.

Data Availability Statement

The datasets generated and/or analyzed during the current study are available from the corresponding author on reasonable request.