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Year : 2018  |  Volume : 7  |  Issue : 3  |  Page : 228-232

An insight of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma: A rare case report

Department of Oral Pathology and Microbiology, Kalinga Institute of Dental Sciences, Bhubaneswar, Odisha, India

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Sujatha Ramachandra
Department of Oral Pathology and Microbiology, Kalinga Institute of Dental Sciences, KIIT University, Bhubaneswar - 751 024, Odisha
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Ameloblastoma is one of the most common benign odontogenic tumors of the jaw. It is generally categorized into: solid (multicystic), unicystic, and peripheral. Unicystic ameloblastoma is a single cystic cavity presenting ameloblastomatous differentiation in the lining. The clinical, radiographic, and histopathological features of unicystic ameloblastoma are usually confusing and misdiagnosed as odontogenic cysts of jaws. Increased vascularity in a tumor can not only affect its prognosis but can also increase the risk of tumor seeding and secondary tumor. Here, we present a case of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma in a 25-year-old male that is a rare entity and needs to be explored.

Keywords: Intramural, odontogenic tumor, plexiform, unicystic ameloblastoma

How to cite this article:
Dash KC, Ramachandra S, Bhuyan L, Kumar H. An insight of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma: A rare case report. J NTR Univ Health Sci 2018;7:228-32

How to cite this URL:
Dash KC, Ramachandra S, Bhuyan L, Kumar H. An insight of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma: A rare case report. J NTR Univ Health Sci [serial online] 2018 [cited 2023 Feb 6];7:228-32. Available from: https://www.jdrntruhs.org/text.asp?2018/7/3/228/241289

  Introduction Top

Ameloblastomas originate from an odontogenic epithelium arising from the rests of dental lamina, developing enamel organ, epithelial lining of odontogenic cysts, or basal cells of oral mucosa. Clinicoradiographically, there are three types—conventional solid or multicystic (about 86%), unicystic (about 13%), and peripheral or extraosseous (about 1%). The unicystic ameloblastoma (UA) is a type of intraosseous ameloblastoma that is frequently seen in younger population and associated with impacted tooth, giving it a misdiagnosis of odontogenic cyst—mostly dentigerous cyst. UA usually shows a distinct clinical, radiographic, and histopathologic behavior than the most common form, i.e., the conventional solid or multicystic.[1] Three histologic subtypes of UA with subgroups 1, 1.2, 1.2.3 and 1.3 have been proposed by Ackermann et al.[2] [Table 1]. However, histopathologic examination is always essential for correct diagnosis of such cases.
Table 1: Histologic ua subgrouping (modified after ackermann et al.)

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In the past, a hemangiomatous variant of ameloblastoma has been seen showing large endothelial-lined blood vessels in certain parts of the tumor.[3] Such case of vascular ameloblastomas was first reported by Kuhn in 1932 and was documented as ameloblastic hemangiomas or hemangioameloblastomas (HA) in the early literatures.[4],[5],[6] Here, we report a case of intramural plexiform hemangiomatous proliferation in UA in the mandible of a 25-year-old male, which was misdiagnosed as a dentigerous cyst clinically.

  Case Report Top

A 25-year-old male patient reported to the Department of Oral medicine and Radiology with a chief complaint of pain and swelling in the lower left back tooth region for 3 months. Patient was apparently normal 3 months ago after which he noticed the swelling in the left side of the jaw associated with pain. Pain was dull aching type that aggravated on mastication but relieved on rest. There was no history of trauma or past relevant dental or medical history. All vital signs were within the normal limits.

On physical examination, there was a facial asymmetry due to the swelling in left lower jaw region. The temporomandibular joint showed no abnormalities. No lymph nodes were palpable. The extraoral examination revealed diffuse, asymptomatic swelling in the left lower jaw over the angle of mandible, measuring approximately 3 × 4 cm with no secondary changes. On palpation, the swelling was firm and non-tender with no local rise in temperature [Figure 1]. The intraoral examination revealed single diffuse swelling measuring 2 × 3 cm approx. extending from distal of 37 to retromolar region obliterating the buccal vestibule. The left third molar (38) was clinically missing. On palpation, it was mildly tender, soft to firm in consistency, nonfluctuant, and nonreducible with no discharge. The routine blood examination was done and was found to be within normal limits.
Figure 1: Facial profile of the patient

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The orthopantomograph (OPG) revealed well-defined unicystic radiolucency from crown portion of 38, which was impacted at the border of mandible involving the ramus and coronoid process of the involved side [Figure 2]. Surface-rendered 3D image of cone beam computed tomography (CBCT) showed a well-defined unilocular radiolucency involving the 38 with perforation of buccal cortices of the left body of mandible [Figure 3]. The CT scan revealed well-defined lytic lesion with perforation of cortical plates and the extent of lesion (3 cm × 3 cm × 2 cm) from 38 to retromolar region [Figure 4].
Figure 2: OPG reveals unilocular radiolucency in relation to mandibular left third molar

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Figure 3: CBCT image shows well-defined unilocular radiolucency

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Figure 4: CT scan (axial view) of mandible reveals well-circumscribed radiolucent lesion

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Based on patient's history, clinical finding, and radiographic investigations, a diagnosis of dentigerous cyst was made. A differential diagnosis of ameloblastoma, calcifying epithelial odontogenic tumor (CEOT), and odontogenic myxoma was also considered based on patient's age and clinical presentations. Patient was then advised for biopsy and the sample was sent to the Department of Oral Pathology & Microbiology for its histopathological evaluation.

Histopathology revealed a cystic lesion that was lined by ameloblastic epithelium showing hyperchromatic and palisaded basal cell layer [Figure 5]. Intramural proliferation of the ameloblastomatous epithelium with tall columnar basal cells and stellate reticulum-like superficial cells was arranged in plexiform pattern [Figure 6]. Numerous endothelium-lined blood vessels were found in the connective tissue along the proliferated plexiform ameloblastomatous cells [Figure 7].
Figure 5: Microscopic features reveals cystic cavity lined by ameloblastic epithelium showing hyperchromatic and palisaded basal cell layer (10×)

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Figure 6: Intramural proliferation of ameloblastomatous epithelium in plexiform pattern with numerous vascular spaces (10×)

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Figure 7: Ameloblastomatous cells showing intramural plexiform proliferation along with endothelium-lined blood vessels (40×)

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Based on the clinical, radiograph, and histopathological findings, we came to a final diagnosis of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma. The patient was then surgically treated and is under regular follow-up.

  Discussion Top

The origin of unicystic ameloblastoma has remained a debate among researchers from several decades. It may arise de novo as a neoplasm or due to neoplastic transformation of non-neoplastic cystic epithelium.[7] According to Vickers and Gorlin, who conducted studies on such cases in the past, the diagnosis of ameloblastoma should satisfy the features like (a) hyperchromatism of the basal cell nuclei of the epithelium lining the cystic cavity, (b) palisading arrangements of basal cells, (c) polarization of nuclei of the basal cells, and (d) cytoplasmic vacuolation of the basal cells.[1] Our case satisfies all the criteria of Vickers and Gorlin.

Plexiform unicystic ameloblastomas (PUA) generally mimic a dentigerous cyst clinically and radiographically, presenting usually in younger individual. They may or may not be associated with an unerupted tooth but are seen more commonly in mandibular region with male predilection.[8] In our case, we also came across such findings where the lesion was associated with an unerupted tooth in the mandibular third molar region of a young male patient.

The radiographic appearance of UA is mostly unilocular radiolucency; however, multilocular radiolucenies may also appear especially in some recurrence cases. Differential diagnosis of dentigerous cyst and odontogenic keratocyst are considered to be most common.[8] In the present case, the clinical diagnosis was also given as dentigerous cyst considering it is associated with an unerupted tooth. On histopathological examination, it was finally diagnosed to be a case of intramural plexiform hemangiomatous proliferation in unicystic ameloblastoma.

Several theories have been formulated for the pathogenesis in the origin of vascular component of the HA; however, the exact origin, the pathogenesis, and the biological behavior are not yet clear. Abnormal induction of the profuse blood supply to the outer enamel epithelium during amelogenesis can lead to overgrowth of vascular component. Epithelial cell rests in periodontal ligament may be induced by a trauma, such as extraction, and subsequently develop tumor. A disturbance in the repair of tissue damage results in excessive granulation tissue formation and development of abnormal vascular component.[3] HA being a collision tumor has also been suggested by Jois et al. in 2011.[9] Adisa [10] in 2010 reported a case of HA in mandible and suggested repeated surgical intervention as a possible etiology for its development. Whether HA is a part of the neoplastic process, a hamartomatous malformation or a separate neoplasm, it is still to be explored.[11]

A very few cases of HA have been reported in the literature. Rensburg in 2001 reported a case with histologic features consistent with those of a UA, but radiologic features and computed tomography mimicking a fibro-osseous lesion and magnetic resonance imaging suggesting a vascular lesion.[12] Sharma et al. reported a case of HA in maxilla in 2012 in a 15-year old.[11] Sarode et al. reported a case of intraluminal plexiform HA in a unicystic ameloblastoma.[3] Hegde et al. reported hemangiomatous proliferation in a case of mural and luminal plexiform UA and plexiform ameloblastoma of mandible.[13] Kasangari et al. reported a case of HA in mandible of a 35-year-old female patient.[14] Our case was an intramural plexiform HA in UA.

The treatment of such cases is vital due to its high recurrence, for which enucleation, marginal and aggressive resection, is needed. CT helps in detecting the extension of the lesion.[15] UA has better prognosis than other variants, but sometimes the prognosis is bad as it can breach the periphery of the capsule and can infiltrate the surrounding bone behaving more aggressively at any stage.[16]

  Conclusion Top

Though vascularity is essential for the growth, development, and functioning of the body, its disturbances can cause pathologies. It aids in the growth of the lesion through its nutrition supply and affects the prognosis of the lesion. Pathologies related to blood vessels and vascularity are well established. Vascularity might be one of the marked components along with other diagnostic components of an established disease. Such lesions vary in biological behavior and prognosis in comparison to that of an established entity and are diagnosed with special names. HA thus is a similar condition whose nature is uncertain due to the rarity of the lesion and the insufficient scientific evidence. Hence, this is a case of unicystic ameloblastoma, an established entity, with significant vascularity whose nature is indeterminate. The multilocular radiographic appearance and histopathologic variations of such lesions also make diagnosis difficult which in turn affects the treatment and prognosis.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rajendran R, Sivapathasundaram B. Shafer's Textbook of Oral Pathology, 6th Edition; Elsevier 2009. ISBN: 978-81-312-1570-8; p. 276.  Back to cited text no. 1
Ackermann GL, Altini M, Shear M. The unicystic ameloblastoma: A clinicopathologic study of 57 cases. J Oral Pathol 1988;17:541-6.  Back to cited text no. 2
Sarode GS, Sarode SC, Vaidya K. Intraluminal plexiform hemangioameloblastomatous proliferation in unicystic ameloblastoma: An unusual case report. Indian J Dent Res 2013;24:390-2.  Back to cited text no. 3
[PUBMED]  [Full text]  
Kuhn A. A combination of adamantinoma with hemangioma as a central jaw tumor. Dtsch Mschr Z 1932;50:49-56.  Back to cited text no. 4
Shafer WY, Hine MK, Levy BM. A textbook of oral pathology. 4th edition. Philadelphia: WB Saunders; 1983.  Back to cited text no. 5
Oliver RT, Mckenna WF, Shafer WG. Hemangio-ameloblastoma: Report of a case. J Oral Surg Anesth Hosp Dent Serv 1961;19:245-8.  Back to cited text no. 6
Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3rd Edition; Elsevier 2013. ISBN: 978-81-312-1946-1; p. 708.  Back to cited text no. 7
Kasat VO, Karjodkar FR, Ladda R. Plexiform unicystic ameloblastoma-a case report and review of literature. J Health Res 2014;1:103-6.  Back to cited text no. 8
Jois H, Kumar KPM, Kumar M, Waghrey S. A mixed neoplasm of intraosseous hemangioma with an ameloblastoma: A case of collision tumor or a rare variant?. Clin Pract 2011;2:e5.  Back to cited text no. 9
Adisa AO, Lawal AOS, Olusanya AA, Arotiba JT. Hemangiomatous ameloblastoma: Report of a case. Niger Dent J 2010;18:73-5.  Back to cited text no. 10
Sharma VK, Verma SK, Goyal L, Chaudhary PK. Hemangiomatous ameloblastoma in maxilla: A report of a very rare case. Dent Res J 2012;9:345-9.  Back to cited text no. 11
Rensburg LJ, Thompson IOC, Kruger HEC, Norval EJG. Hemangiomatous ameloblastoma: Clinical, radiologic, and pathologic features. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:374-80.  Back to cited text no. 12
Hegde U, Sheshanna SH, Chandan SN, Mahima VG. Enriched vascularity in ameloblastomas, an indeterminate entity: Report of two cases. Indian J Cancer Res Ther 2015;11:946-9.  Back to cited text no. 13
Kasangari MD, Gundamaraju K, Jyothsna M, Subash AV, Aravind K. Hemangiomatous Ameloblastoma-A Case Report of a Very Rare Variant of Ameloblastoma. J Clin Diagn Res 2015;9:ZD08-10.  Back to cited text no. 14
Unlu G, Tari V, Alan H. Unicystic Ameloblastoma in 8 Years old Child: A Case Report Review of Unicystic Ameloblastoma. Int Dent Med Disord 2008;1:29-33.  Back to cited text no. 15
Kiran Kumar KR, George GB, Padiyath S, Rupak S, Kumar KR, George G, et al. Mural Unicystic Ameloblastoma Crossing the Midline: A Rare Case Report. Int J Odontostomat 2012;6:97-103.  Back to cited text no. 16


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

  [Table 1]


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