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Year : 2018  |  Volume : 29  |  Issue : 3  |  Page : 396-399
Cystadenocarcinoma of the mandible

1 Department of Oral Medicine and Radiology, Government Dental College, Kozhikode, Kerala, India
2 Department of Oral Medicine and Radiology, Government Dental College, Alappuzha, Kerala, India
3 Department of General Pathology, Government Dental College, Kozhikode, Kerala, India

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Date of Web Publication13-Jun-2018


Papillary cystadenocarcinoma of the salivary gland is a rare malignant tumor and occurs in major and minor salivary glands. Papillary cystadenocarcinoma of the mandible is exceptionally rare. It is usually a low-grade destructive tumor with a papillary and cystic architecture. This case describes a unique presentation, location, and radiographic appearance of this lesion.

Keywords: Cystadenocarcinoma, mandible, salivary gland, tumor

How to cite this article:
Madathil J, Kumar NR, Shiny P. Cystadenocarcinoma of the mandible. Indian J Dent Res 2018;29:396-9

How to cite this URL:
Madathil J, Kumar NR, Shiny P. Cystadenocarcinoma of the mandible. Indian J Dent Res [serial online] 2018 [cited 2023 Mar 22];29:396-9. Available from:

   Introduction Top

Cystadenocarcinoma is a rare salivary gland tumor which commonly arises in the major salivary glands, mainly the parotid gland.[1] It was first defined by the World Health Organization (WHO) in 1991 as a low-grade malignancy that rarely metastasizes to lymph nodes.[2] It was included as cystadenocarcinoma in the recent 2005 WHO classification of head and neck tumors.[3] They are characterized histologically by prominent cystic and frequently papillary growth but lacking features that characterize cystic variants of several more common salivary gland neoplasms.[4] Epidemiologically, the lesion comprises roughly 2% of malignant salivary gland tumors.[5] Salivary gland lesions that present intraosseously are also rare, only 4 cases being reported in the literature.[6],[7],[8],[9] The authors describe this case of papillary cystadenocarcinoma arising from a rare location with unique radiographic findings.

   Case Report Top

A 71-year-old male patient came to the Department of Oral Medicine and Radiology with a complaint of swelling in relation to the right lower back region of jaw for 2 weeks with a rapid increase in size. Extraoral examination was negative for swelling or tenderness. The patient had given a history of extraction of a tooth at the same site 6 years back due to mobility. Medical history revealed that the patient was diabetic but was not on any treatment.

Intraoral examination revealed a diffuse swelling involving the edentulous canine–molar region that extends up to lower mucobuccal sulcus obliterating the buccal sulcus buccally and up to lingual sulcus lingually. On palpation, swelling had a variable consistency and was nontender. Surface over the swelling showed an ulcerated area 0.5 cm × 0.5 cm probably due to impingement from the supraerupted canine from the opposing arch [Figure 1].
Figure 1: Swelling with ulcerated area with keratosis surrounding

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Panoramic [Figure 2] and lateral radiographs [Figure 3] showed radiolucent lesion with irregular margin extending up to lower border of mandible. Fine-needle aspiration yielded [Figure 4] thick fluid red in color mixed with blood and the smear showed chronic inflammatory cells intermingled with red blood cells.
Figure 2: Panoramic radiograph showing radiolucent area with irregular margin and bone loss

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Figure 3: Lateral radiograph

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Figure 4: Fine needle aspiration showing thick fluid red in color mixed with blood

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From the clinical and radiographical examination, a diagnosis of intraosseous carcinoma was arrived, with a differential diagnosis of metastatic lesion. The patient was sent for biopsy for further evaluation.

Histopathology report showed a neoplasm composed of numerous cysts in a desmoplastic stroma lined by mucinous and columnar cells with varying degree of papillary proliferation. They also showed moderate nuclear pleomorphism with scanty mitoses and with no areas of necrosis [Figure 5], [Figure 6], [Figure 7] These histopathological features lead to the diagnosis of cystadenocarcinoma.
Figure 5: Photomicrograph showing cystically dilated glands and small irregular glands with papillary proliferation, infiltrating into desmoplastic stroma (H and E, ×4)

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Figure 6: Photomicrograph showing cystically dialated glands and small irregular glands with papillary proliferation infiltrating into desmoplastic stroma (H and E, ×10)

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Figure 7: Columnar cells showing pleomorphism with moderate eosinophilic cytoplasm and hyperchromatic nucleus (H and E, ×40)

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The patient was referred to the Oncology Department of Medical College. However, the patient passed away before any treatment could start.

   Discussion Top

Cystadenocarcinoma is rarely mentioned in literature because of low incidence and the subestimation of the diagnosis. Cystadenocarcinoma is also termed as papillary cystadenocarcinoma, mucus-producing adenopapillary (nonepidermoid) carcinoma, malignant papillary cystadenoma, or low-grade papillary cystadenocarcinoma and papillary adenocarcinoma.[4]

Cystadenocarcinoma of the salivary gland is a distinct group of epithelial malignancy characterized by cystic and solid areas with luminal papillary projections, lined by cuboidal, columnar, or mucus-secreting cells. Malignant nature of tumor is confirmed by nuclear pleomorphism, mitosis, and infiltrative growth pattern.[10] Microscopically, all tumors revealed cystic areas with stromal invasion and 75% of cases showed clearly visible papillary structures. Immunohistochemical study is not mandatory to make the diagnosis, but some authors reported a positivity of the tumor cells with the cytokeratin, smooth muscle actin, and S100 protein antigens.[10]

Salivary gland papillary cystadenocarcinoma has also been reported to arise in unusual sites, including the tongue, retromolar area, and submandibular gland.[11] Salivary gland lesions that present intraosseously are also rare. The tumor is found more commonly in the mandible than in the maxilla [12] and comprises <0.4% of all salivary gland carcinomas.[13] The pathogenesis of salivary gland tumor arising centrally within the mandible is still controversial and has been proposed as [1] entrapment of salivary tissue from the major or minor salivary glands within bone during embryonic development,[2] aberrant induction of salivary tissue from the dental lamina epithelium during embryonic development,[3] metaplastic transformation of mucus-secreting cells in odontogenic cyst epithelium, or [4] entrapment of minor salivary glands from chronic osteomyelitis.[7],[12],[14],[15]

A staging system for central malignant salivary gland tumor was established by Brookstone and Huvos in 1992 based on the condition of overlying bone. Stage I is indicated by lesions with intact cortical bone and overlying periosteum without signs of cortical expansion. In Stage II, lesions are surrounded by intact cortical bone with some degree of expansion. Stage III is characterized by lesions with cortical perforation, breakdown of the overlying periosteum, or nodal metastasis.[12]

Literature reviewed showed only four cases of cystadenocarcinoma of mandible presented as a cystic lesion on radiograph in the three cases.[6],[8],[9] And, in the study by Li et al., lesion was presented with a radiolucent regions with an ill-defined margin.[7]

The appropriate treatment of the primary tumor is en bloc resection, including marginal mandibulectomy and segmental mandibulectomy based on preoperative findings and intraoperative evaluation.[7] Neck dissection is necessary when evidence of any metastatic cervical lymph node involvement is present. Adjuvant radiotherapy and chemotherapy have been reported in cases with perineural invasion, inadequate surgical margin, large tumor, rupture of cortical plate, and soft-tissue invasion.[7],[15]

   Conclusion Top

Central papillary cystadenocarcinoma of the jaw is an extremely rare tumor with only four previously reported cases in the literature. Clinical, radiographic, and histopathologic examinations should be carefully reviewed to differentiate this neoplasm from other possible malignant neoplasms. The tumor prognosis is still unsettled. Surgical excision with wide margins is the appropriate treatment, while adjuvant radiotherapy should be considered in histologically aggressive or high-stage tumor. Prolonged clinical observation is mandatory.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Metgud R, Kalburge J, Dongre S, Karle R. Papillary cystadenocarcinoma of the tongue. J Oral Maxillofac Pathol 2007;11:63.  Back to cited text no. 1
  [Full text]  
Siefert G, Sobin L. Histological classification of salivary gland tumours. International Histological Classification of Tumours. Berlin: Springer-Verlag, World Health Organization; 1991.  Back to cited text no. 2
Barnes L, Eveson J, Reichart P, Sidransky D. Classifi cation of Tumours: Pathology and Genetics of Head and Neck Tumours.IARC Press in Lyon, France: World Health Organization; 2005.  Back to cited text no. 3
Shafer, Maynard Hine, Barnet Levy. Shafer's Textbook of Oral Pathology. 17th ed. India: Elsevier; 2012.  Back to cited text no. 4
Ellis GL. What's new in the AFIP fascicle on salivary gland tumors: A few highlights from the 4th series atlas. Head Neck Pathol 2009;3:225-30.  Back to cited text no. 5
Johnston NJ, Rose DS, Lutterloch MJ. Cystadenocarcinoma of salivary gland presenting as a cystic lesion in the mandible. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:201-4.  Back to cited text no. 6
Li Y, Li LJ, Huang J, Han B, Pan J. Central malignant salivary gland tumors of the jaw: Retrospective clinical analysis of 22 cases. J Oral Maxillofac Surg 2008;66:2247-53.  Back to cited text no. 7
Takei R, Tomihara K, Arai N, Noguchi M. Central cystadenocarcinoma of the mandible. Int J Oral Maxillofac Surg 2012;41:1463-6.  Back to cited text no. 8
Srivanitchapoom C, Sittitrai P, Mahanupab P. Central papillary cystadenocarcinoma of the mandible: A case report and review of the literature. Int J Surg Case Rep 2014;5:330-4.  Back to cited text no. 9
Pollett A, Perez-Ordonez B, Jordan RC, Davidson MJ. High-grade papillary cystadenocarcinoma of the tongue. Histopathology 1997;31:185-8.  Back to cited text no. 10
Foss RD, Ellis GL, Auclair PL. Salivary gland cystadenocarcinomas. A clinicopathologic study of 57 cases. Am J Surg Pathol 1996;20:1440-7.  Back to cited text no. 11
Brookstone MS, Huvos AG. Central salivary gland tumors of the maxilla and mandible: A clinicopathologic study of 11 cases with an analysis of the literature. J Oral Maxillofac Surg 1992;50:229-36.  Back to cited text no. 12
Martínez-Madrigal F, Pineda-Daboin K, Casiraghi O, Luna MA. Salivary gland tumors of the mandible. Ann Diagn Pathol 2000;4:347-53.  Back to cited text no. 13
Bouquot JE, Gnepp DR, Dardick I, Hietanen JH. Intraosseous salivary tissue: Jawbone examples of choristomas, hamartomas, embryonic rests, and inflammatory entrapment: Another histogenetic source for intraosseous adenocarcinoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:205-17.  Back to cited text no. 14
Pires FR, Paes de Almeida O, Lopes MA, Elias da Cruz Perez D, Kowalski LP. Central mucoepidermoid carcinoma of the mandible: Report of four cases with long-term follow-up. Int J Oral Maxillofac Surg 2003;32:378-82.  Back to cited text no. 15

Correspondence Address:
Dr. Jinisha Madathil
Department of Oral Medicine and Radiology, Government Dental College, Kozhikode, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdr.IJDR_768_16

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

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