Indian Journal of Dental Research

: 2013  |  Volume : 24  |  Issue : 3  |  Page : 390--392

Intraluminal plexiform hemangioameloblastomatous proliferation in unicystic ameloblastoma: An unusual case report

Gargi S Sarode, Sachin C Sarode, Kedar Vaidya 
 Department of Oral Pathology and Microbiology, Dr. D. Y. Patil Dental College and Hospital, Maheshnagar, Pimpri, Pune, Maharashtra, India

Correspondence Address:
Gargi S Sarode
Department of Oral Pathology and Microbiology, Dr. D. Y. Patil Dental College and Hospital, Maheshnagar, Pimpri, Pune, Maharashtra


The authors report a case of unicystic ameloblastoma with intraluminal plexiform hemangiomatous proliferation in the right mandible of an 18-year-old Indian male who suffered from painful swelling for 2 months. A radiograph showed a well-circumscribed multilocular radiolucent lesion associated with the angle and ramus of the mandible. Microscopic examination revealed the cystic lesion lined with ameloblastic epithelium with intraluminal plexiform hemangioameloblastomatous proliferation. The possible pathogenic mechanism for hemangiomatous appearance of this case reflects the previous history of extraction. The prognosis is perhaps the same as expected for conventional unicystic ameloblastoma.

How to cite this article:
Sarode GS, Sarode SC, Vaidya K. Intraluminal plexiform hemangioameloblastomatous proliferation in unicystic ameloblastoma: An unusual case report.Indian J Dent Res 2013;24:390-392

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Sarode GS, Sarode SC, Vaidya K. Intraluminal plexiform hemangioameloblastomatous proliferation in unicystic ameloblastoma: An unusual case report. Indian J Dent Res [serial online] 2013 [cited 2022 Sep 27 ];24:390-392
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Unicystic ameloblastoma comprises approximately 6% of ameloblastomas. [1] The epithelium lining the cystic cavity of the neoplasm shows typical cytomorphologic features that are recognizable as ameloblastoma, with a basal cell layer composed of columnar cells displaying hyperchromatic, palisaded nuclei. Reverse polarity of the nuclei is present, and a subnuclear vacuole usually is noted between the basement membrane and nucleus. In some instances, the ameloblastic epithelium may be proliferative, with extension of the ameloblastic epithelium into the lumen of the cystic cavity. This feature has been termed intraluminal proliferation, and in many instances, the intraluminal growth resembles the plexiform type of ameloblastoma which is referred to as plexiform unicystic ameloblastoma. [1]

In the literature, a rare variant called hemangiomatous ameloblastoma (HA) was originally described as an ameloblastoma in which a part of the tumor contained spaces filled with blood or large endothelial-lined capillaries. [2] The first case of vascular ameloblastoma was reported by Kuhn [3] in 1932 as a combination of adamantinoma and hemangioma; further cases have been recorded by Oesterreich [4] and Aisenberg [5] as adamantinohemangiomas. It was also documented in the early literature as ameloblastic hemangiomas [6] and hemangioameloblastomas. [7] Authors here describe a rare association of unicystic ameloblastoma with unusual intraluminal plexiform hemangiomatous proliferation.

 Case Report

An 18-year-old male patient presented with a chief complaint of painful swelling in the right mandibular area. He had discomfort for approximately 2 months. The patient gave a history of root canal treatment in the permanent mandibular right second molar about 1 year ago and stated that it had been extracted 6 months later following the complaint of pain and pus discharge. His medical and family history was unremarkable. Physical examination revealed 10 cm × 5 cm, diffuse, non-tender, smooth-surfaced, and bony hard swelling on the right side of the body of mandible [Figure 1]. The patient had no paresthesia or limitation of functional mandibular movements. Mucosal ulceration and cervical lymphadenopathy was not detected. Panoramic radiograph revealed a well-circumscribed multilocular radiolucent lesion in the ramus and angle of the mandible [Figure 2]. Based on the clinical and radiographic findings, a presumptive diagnosis of ameloblastoma or odontogenic keratocyst was made. The lesion was biopsied and sent for histopathologic examination. It showed cystic lesion lined by ameloblastic epithelium with hyperchromatic, palisaded basal cell layer. The overlying epithelial cells were loosely cohesive and resembled stellate reticulum. The subepithelial hyalinization was seen at many places, which is a characteristic feature of unicystic ameloblastoma [Figure 3]. At focal areas, intraluminal proliferation of ameloblastomatous epithelium in plexiform pattern was seen [Figure 4]. The loose connective tissue stroma associated with intraluminal proliferation showed degenerative changes. Numerous endothelium-lined vascular spaces were seen at most of the areas, giving hemagiomatous appearance [Figure 5]. The final diagnosis of unicystic ameloblastoma with intraluminal plexiform hemangioameloblastomatous proliferation was made. The curettage of the lesion was done with the help of Carnoy's solution. The patient was lost to follow-up.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}


The origin of the vascular component of the HA is not clear and several theories have been formulated to explain its pathogenesis. During amelogenesis, capillaries associated with the outer enamel epithelium provide the profuse blood supply necessary for enamel completion. In the HA, there is abnormal induction of blood vessels and they probably become part of the tumor. [5] Excessive stimulation of angiogenesis during tumor development by inductive influences such as those that occur during odontogenesis or by other factors may result in the overgrowth of vascular elements in the odontogenic ectomesenchyme or in adjacent connective tissue. [8]

A trauma such as a tooth extraction may provide the stimulus for proliferation of epithelial cell rests in the periodontal ligament and subsequent tumor development. [5] Tissue damage is repaired involving the formation of granulation tissue in which proliferating endothelial cells and new capillaries are prominent. A disturbance in the repair of neoplastic odontogenic tissue may result in excessive granulation tissue formation or the development of an abnormal vascular component. [8] In the present case, the history of extraction suggests the possible reason for the hemangiomatous appearance.

It has been suggested that the HA represents a collision tumor. [7] According to Lucas, [9] the unusual vascularity is not due to a neoplastic process. He believed that there is an entire absence of vasoformative activity. In the process of formation of stromal cysts in the ordinary type of plexiform ameloblastoma, the blood vessels often persist and dilate instead of disappearing; thus, it is likely to represent a purely secondary change.

Smith [10] regarded the HA as histologically similar to one of the other recognized types of ameloblastoma and not as a distinct histologic entity. He thought the blood supply to these tumors was variable and that circumstances other than the number and size of the vessels influenced the blood supply. Tumors that demonstrate vascular features are, however, well documented, and vascular variants of these neoplasms are accepted as histologic entities. Examples are the angiomatoid malignant fibrous histiocytoma and the telangiectatic osteosarcoma. The angiomatoid malignant fibrous histiocytoma combines the features of both a fibrohistiocytic tumor and a vascular tumor, while the telangiectatic osteosarcoma contains large blood-filled spaces, thrombus formation, organization, and massive areas of necrosis. [8]

Whether the vascular component of the HA is a part of the neoplastic process, represents a separate neoplasm, or is a hamartomatous malformation has not been satisfactorily resolved. Lucas [9] has suggested that the term should be reserved until such time as it can be employed for a tumor in which the vascular changes form an essential part of the neoplastic process.


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