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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 2  |  Page : 72-74

Salivary duct carcinoma arising in pleomorphic adenoma


1 Department of Pathology, King Khalid University, Abha, Saudi Arabia
2 Department of Laboratory Medicine, Aseer Central Hospital, Abha, Saudi Arabia
3 Department of Hemato-Oncology, Aseer Central Hospital, Abha, Saudi Arabia

Date of Submission18-May-2020
Date of Acceptance18-Jun-2020
Date of Web Publication31-Oct-2020

Correspondence Address:
Dr. Sohaila Fatima
Department of Pathology, King Khalid University, Abha
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijohr.ijohr_10_20

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  Abstract 


Carcinoma ex pleomorphic adenoma (CPA) is an epithelial and/or myoepithelial malignancy developing from primary or recurrent pleomorphic adenoma. Salivary duct carcinoma is a rare aggressive malignancy of the salivary gland resembling mammary ductal carcinoma, which can occur as a component of CPA. It is a high-grade tumor with poor prognosis. Treatment modalities include surgery and postoperative (adjuvant) radiation therapy. Here, we present an old female patient with a submandibular mass which proved to be salivary duct CPA.

Keywords: High grade, pleomorphic adenoma, salivary duct carcinoma, submandibular gland


How to cite this article:
Fatima S, Sadek SA, Siddiqui WA. Salivary duct carcinoma arising in pleomorphic adenoma. Indian J Oral Health Res 2020;6:72-4

How to cite this URL:
Fatima S, Sadek SA, Siddiqui WA. Salivary duct carcinoma arising in pleomorphic adenoma. Indian J Oral Health Res [serial online] 2020 [cited 2021 Apr 16];6:72-4. Available from: https://www.ijohr.org/text.asp?2020/6/2/72/299697


  Introduction Top


Carcinoma ex pleomorphic adenoma (CPA) is a carcinomatous transformation within a primary or recurrent pleomorphic adenoma (PA). It predominantly affects the major salivary glands, with a majority of cases in the parotid and submandibular glands.[1] Salivary duct carcinoma (SDC) is a rare aggressive epithelial malignancy of the salivary gland, resembling mammary ductal carcinoma. It can occur alone or as a component of CPA. A slow-growing parotid mass that has recently exhibited a growth spurt should raise the suspicion of a CPA.[2] Microscopically, SDC resembles high-grade in situ and invasive apocrine ductal carcinoma of the breast.[3] The management of patients depends on the type of malignant component. Treatment modalities include surgery and postoperative (adjuvant) radiation therapy. Here, we present an old female patient with a submandibular mass which proved to be CPA (SDC).


  Case Report Top


An 83-year-old female patient presented with a left submandibular mass with the skin involvement of 3 months duration. It was hard and nontender on palpation with no cervical lymphadenopathy. Excisional biopsy was performed. On gross examination, three tissues were received with largest measuring 5 cm × 3.5 cm × 2.5 cm and smallest measuring 1 cm × 0.5 cm × 0.4 cm. Microscopic examination of one tissue revealed an encapsulated neoplasm composed predominantly of myoepithelial cells interspersed by the ductal epithelial cells in a chondromyxoid stroma [Figure 1]a. The other tissues showed malignant neoplasm with abrupt transition with benign tumor [Figure 1]b. The malignant component was composed of cords, nests, and cribriform glands with marked comedonecrosis infiltrated by desmoplastic stroma with rich lymphoplasmacytic infiltrate [Figure 1]c and [Figure 1]d. The tumor cells exhibit pleomorphic nuclei with prominent nucleoli, mitotic activity, eosinophilic cytoplasm, and mucin vacuoles [Figure 2]b. The tumor infiltrated soft tissue with the involvement of skin [Figure 2]a. There was frequent lymphovascular invasion with no perineural involvement. Pathological staging was pT4a Nx Mx. Immunohistochemical study showed strong androgen and gross cystic disease fluid protein (GCDFP) positivity [Figure 2]c and [Figure 2]d. The diagnosis was invasive SDC in a background of PA.
Figure 1: (a) Sections from the salivary gland showing myoepithelial cells in the sheets and ductal epithelial cells in a chondromyxoid stroma. (b) Sections showing malignant neoplasm on the right with abrupt transition from pleomorphic adenoma on the left. (c and d) Section showing the malignant component composed of cords, nests, and cribriform glands with marked comedonecrosis infiltrated by desmoplastic stroma with rich lymphoplasmacytic infiltrate (a-d: H and E, ×20)

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Figure 2: (a) Sections from the skin overlying tumor showing infiltration of the dermis by malignant cells. (b) The tumor cells exhibiting pleomorphic nuclei with prominent nucleoli, mitotic activity, eosinophilic cytoplasm, and mucin vacuoles (H and E, a: ×20 and b: ×40). Immunohistochemical study showing (c) androgen receptor positivity in the malignant cells (AR, ×40) and (d) gross cystic disease fluid protein positivity in the malignant cells (GCDFP, ×20)

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  Discussion Top


CPA accounts for 3.6% of all salivary gland tumors. SDC accounts for as many as 10% of all salivary gland malignancies. It can occur de novo or outcome of malignant component of CPA.[1] The malignant component of CPA is most often adenocarcinoma not otherwise specified. Sometimes, the component may be adenoid cystic carcinoma, mucoepidermoid carcinoma, or SDC.[4] SDC was first reported in 1968 by Kleinsasser et al.[5] The term “SDC” refers to the pattern of growth of the tumor rather than to its anatomic location. Most reported carcinomas of Stensen duct have not been SDC but rather mucoepidermoid, squamous cell, or undifferentiated carcinomas.[3]

CPA occurs slightly more often in women than in men, with peak incidence in the sixth and seventh decades of life.[1] However, for SDC, the median age was found to be 65 years and the majority were males.[6] SDC mainly occurs in the parotid gland (78%); followed by submandibular gland (12%) and minor salivary glands (10%).[7] In early stage of the disease, the predominant presentation is a painless mass, whereas in the advanced stage, pain and nerve paralysis are common.[8]

Microscopically, SDC resembles high-grade ductal carcinoma of the breast, including large ducts with comedonecrosis and cribriform and Roman bridge-like features.[1] The presence of a micropapillary or sarcomatoid component is associated with an aggressive behavior.[3] There is expression of keratin, carcinoembryonic antigen, epithelial membrane antigen, androgen receptors (ARs), human epidermal growth factor receptor 2 (HER-2/neu), CD117, GCDFP, and prostate-specific antigen and other prostate-related markers and negative expression for estrogen receptors.[3] Treatment strategies targeting HER-2/neu or AR may provide a more positive outcome for such patients.

CPA follows a multistep model of carcinogenesis with the progressive loss of heterozygosity at chromosomal arms 8q, then 12q, and 17p. In addition, many genes which regulate tumor suppression, cell cycle control, growth factors, and cell–cell adhesion play a role in the development and progression of CPA.[2] In SDC, mutations of the p53 gene were more frequent in tumor samples.[7]

CPA should not be considered as an isolated entity because the type and extent of the carcinomatous component impact the management of patients.[1] Intracapsular SDC ex PA appear to have an indolent clinical course. World Health Organization (WHO) criteria for minimal invasion (<1.5 mm) are not applicable to SDC ex PA. It appears that while confinement to the PA capsule has a protective effect, once the capsule is breached, the intrinsic aggression of SDC manifests itself and the presence of perineural and/or angiolymphatic invasion should probably disqualify such tumors from this category.[9] Poor prognostic factors were aged 50 years or above, tumor size, and lymph node involvement.[10] Furthermore, SDC in the parotid glands was associated with an improved prognosis compared with that of the palate and submandibular gland.[8]

In conclusion, SDC is a rare aggressive salivary gland carcinoma. It could be seen in association with PA. It is a salivary gland counterpart of mammary duct carcinoma. Site is an important prognostic criterion with parotid gland tumors having better prognosis. The WHO criterion for minimal invasion is not applicable to SDC ex PA, due to the presence of perineural and/or angiolymphatic invasion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
El-Naggar AK, Chan JK, Rubin GJ, Takata T, Slootweg PJ. WHO Classification of Head and Neck Tumours. Int Agency Res Cancer 2017;pp158-202.  Back to cited text no. 1
    
2.
Antony J, Gopalan V, Smith RA, Lam AK. Carcinoma ex pleomorphic adenoma: A comprehensive review of clinical, pathological and molecular data. Head Neck Pathol 2012;6:1-9.  Back to cited text no. 2
    
3.
Rosai J, Ackerman L. Surgical Pathology. 11th ed. New York: Mosby; 2017.  Back to cited text no. 3
    
4.
Lewis JE, Olsen KD, Sebo TJ. Carcinoma ex pleomorphic adenoma: Pathologic analysis of 73 cases. Hum Pathol 2001;32:596-604.  Back to cited text no. 4
    
5.
Kleinsasser O, Klein HJ, Hübner G. Salivary duct carcinoma. A group of salivary gland tumors analogous to mammary duct carcinoma. Arch Klin Exp Ohren Nasen Kehlkopfheilkd 1968;192:100-5.  Back to cited text no. 5
    
6.
Boon E, Bel M, van Boxtel W, van der Graaf WT, van Es RJ, Eerenstein SE, et al. A clinicopathological study and prognostic factor analysis of 177 salivary duct carcinoma patients from The Netherlands. Int J Cancer 2018;143:758-66.  Back to cited text no. 6
    
7.
Jaehne M, Roeser K, Jaekel T, Schepers JD, Albert N, Loning T. Clinical and immunohistologic typing of salivary duct carcinoma: A report of 50 cases. Cancer 2005;103:25260-33.  Back to cited text no. 7
    
8.
Huang X, Hao J, Chen S, Deng R. Salivary duct carcinoma: A clinopathological report of 11 cases. Oncol Lett 2015;10:337-41.  Back to cited text no. 8
    
9.
Griffith CC, Thompson LD, Assaad A, Purgina BM, Lai C, Bauman JE. Salivary duct carcinoma and the concept of early carcinoma ex pleomorphic adenoma. Histopathology 2014;65:854-60.  Back to cited text no. 9
    
10.
Jayaprakash V, Merzianu M, Warren GW, Arshad H, Hicks WL Jr., Rigual NR, et al. Survival rates and prognostic factors for infiltrating salivary duct carcinoma: Analysis of 228 cases from the Surveillance, Epidemiology, and End Results database. Head Neck 2014;36:694-701.  Back to cited text no. 10
    


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