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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 351-353
Large lipid-rich mammary analogue secretory carcinoma of parotid gland: An unusual case


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Otolaryngology and Head Neck Surgery, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication14-Aug-2015
 

   Abstract 

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a malignant tumor which bears morphologic, immunohistochemical and molecular features similar to those of mammary secretory carcinoma. The tumor is considered as a low-grade malignancy perhaps slightly more aggressive than acinic cell carcinoma. High-grade transformation with recurrences, regional nodal involvement, metastases, and cancer-related death has been reported in a few cases. We report an unusual case of large MASC of the parotid gland in a young patient without regional lymph node involvement. To the best of our knowledge till date such a large MASC of the salivary gland has not been reported in the English literature.

Keywords: Acinic cell carcinoma, mammary analogue secretory carcinoma, parotid gland

How to cite this article:
Joshi P, Mridha AR, Singh S, Kinra P, Ray R, Thakar A. Large lipid-rich mammary analogue secretory carcinoma of parotid gland: An unusual case. Indian J Pathol Microbiol 2015;58:351-3

How to cite this URL:
Joshi P, Mridha AR, Singh S, Kinra P, Ray R, Thakar A. Large lipid-rich mammary analogue secretory carcinoma of parotid gland: An unusual case. Indian J Pathol Microbiol [serial online] 2015 [cited 2020 Jun 6];58:351-3. Available from: http://www.ijpmonline.org/text.asp?2015/58/3/351/162897



   Introduction Top


Mammary analogue secretory carcinoma (MASC) of the salivary gland is a recently described low-grade malignant neoplasm with histologic and molecular features similar to those of mammary secretory carcinoma. [1],[2],[3] Microscopically MASC exhibits microcystic, papillary­cystic, glandular, or solid pattern and is often misdiagnosed as acinic cell carcinoma (ACC). [4] The patients with this tumor have a slightly aggressive clinical course than the patients with ACC. [3] We present a rare case of a huge, recurrent MASC in parotid gland without regional lymph node involvement.


   Case Report Top


A 15-year-old male presented with a painless, progressive swelling in the right infra-auricular region for 18 months. He was operated twice outside with an approximately 1-month asymptomatic postoperative period following which the swelling reappeared and subsequently he was referred to our institution with a histologic diagnosis of ACC. Physical examination revealed a 20 cm × 20 cm firm mass in the right side of the neck. No neurologic deficit was seen. Magnetic resonance imaging revealed a 16 cm × 15 cm × 10 cm heterogeneous mass involving both lobes of the right parotid gland [Figure 1]. Metastatic work-up was negative. Right total parotidectomy was performed. The tumor was found to surround the right facial and spinal accessory nerves which were sacrificed though there was no clinical sign of nerve compression.
Figure 1: Magnetic resonance imaging image shows a large heterogeneous mass involving both the superficial and deep lobes of right parotid gland


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The tumor was well-circumscribed, solid and grayish-white with large areas of necrosis. Microscopic examination revealed epithelial cells arranged in nests and lobules with intervening thin fibrovascular septae [Figure 2]a and areas of necrosis. The individual cells were polygonal with a large intracellular clear vacuole and peripherally displaced vesicular nucleus. Dispersed among the tumor were tiny aggregates of epithelial cells with comparatively scant eosinophilic cytoplasm and elongated vesicular nuclei [Figure 2]b and c. Focal cystic area revealed periodic acid-Schiff (PAS) positive material [Figure 2]d. Large intracellular fat droplets were demonstrated [Figure 2]e. No mucin was seen [Figure 2]f. Mitotic index was approximately 3-4/10 HPF. The tumor infiltrated into periparotid soft tissue, but the interface between the tumor and surrounding tissue was well-defined. No perineural invasion, lympho-vascular embolus or nodal metastasis (0/17) was seen. The tumor cells were immunopositive with (S100 [1:800], CK7 [1:800]) (Dako, USA), CK19 (1:50) (Biocare Medical, USA), (vimentin [1:400], epithelial membrane antigen [EMA] [1:400]) (Thermo Fisher Scientific Inc., USA), GCDFP15 (1:50) (Dako, USA), and CD10 (1:25) (Spring BioScience, UK) [Figure 2]g-k; while negative for (CK20 [1:100], progesterone receptor [PR] [1:300]) (Spring BioScience, UK), (smooth muscle actin [1:400], DOG1 [1:50]) (Thermo Fisher Scientific Inc., USA), CD34 (1:200) (Spring BioScience, UK) and carcinoembryonic antigen (1:3200) (Novocastra, UK). Proliferative index (Ki67) (1:200) (Spring BioScience, UK) was approximately 15-20% in the highest proliferative areas [Figure 2]l. Electron microscopy (EM) demonstrated intracellular large electron-dense lipid droplets [Figure 3].
Figure 2: Sections (H and E) show epithelial cells in lobules with intervening thin septae (a, ×100). The tumor shows oval to polygonal cells with large intracellular clear vacuoles (b, ×200) and interspersed tiny aggregates of epithelial cells (c, ×200). Periodic acid-Schiff positive secretion is present within microcystic area (d, ×200); intracellular lipid droplets are demonstrates by Oil Red O (e, ×200). Mucicarmine stain shows no intracellular mucin (f, ×200). IHC shows positivity with S100 (g, ×200), CK7 (h, ×200), CK19 (i, ×200), vimentin (j, ×200), and GCDFP15 (k, ×200). Ki67 LI is approximately 20% (l, ×400)


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Figure 3: Electron microscopic images confirm presence of intracellular large electron dense lipid droplets (a and b, ×1000)


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   Discussion Top


Mammary analogue secretory carcinoma of the salivary gland is considered as a low-grade malignancy with morphologic, immunohistochemical and molecular features reminiscent of secretory carcinoma of the breast. [1],[3] Fewer than 100 cases have been reported in the English literature. [5] Adults are commonly affected; however, these tumors have been reported in ages 13-77 years (mean 46) with slight male preponderance (male:female = 1.4:1). [1],[4],[6] The parotid and submandibular glands are involved more often than other salivary glands. The average tumor size is 2.1 (range: 0.7-5.5) cm. [1],[7] Microscopically the tumor is lobulated with microcystic, papillary­cystic, glandular, or solid growth pattern. The cells usually exhibit a low-grade vesicular nuclei, pale eosinophilic or "bubbly" cytoplasm, and intraluminal or intracellular secretion, which can be positive with PAS, mucicarmine, MUC1, MUC4 and mammaglobin. The mitotic index is usually low, and necrosis is generally absent or minimal. [1],[5] Morphologic type can be papillary, cystic, solid or cribriform variants. [3] In our case, the patient was a young male with a huge parotid mass. To the best of our knowledge, until date such a large MASC of the salivary gland has not been reported. The tumor was solid, with the presence of focal microcystic areas containing PAS-positive material. Large intracellular lipid-containing vacuoles were present in most of the cells. A unique morphology hitherto undescribed was the presence of tiny aggregates of epithelial cells without intracellular vacuoles, but with an immunophenotype similar to the rest of the tumor. MASC is usually and diffusely immunopositive with vimentin and S100. [8] Some authors, however, have demonstrated focal and variable S100 immunoreactivity. [3] Other immunopositive markers include HMWCK, CK7,CK8, CD10, CK18, CK19, EMA, GCDFP15, mammaglobin and adipophilin. [4] Adipophilin staining is diffuse and surrounds large cytoplasmic vacuoles in MASC; whereas, in ACC and other salivary carcinomas the staining is patchy, variable and around minute droplets. [8] It can also be positive in sebaceous carcinoma. [8] MASC is usually negative for estrogen receptor, PR, Her2Neu, epidermal growth factor receptor, and p63. Vimentin, S100, CK7, CK19, EMA, GCDFP and CD10 were positive in our case. The EM study of our case revealed intracellular large lipid droplets. To the best of our knowledge the ultrastructural features of MASC of salivary have not been described earlier.

The differential diagnoses of MASC include ACC, adenocarcinoma/cystadenocarcinoma (not otherwise specified), mucoepidermoid carcinoma and signet ring carcinoma. [4] This tumor is most frequently misdiagnosed as ACC as seen in our case. [9] The characteristic genetic alteration associated with MASC is the translocation t(12;15)(p13;q25) leading to formation of ETV6-NTRK3 fusion oncogene, which is also seen in mammary secretory carcinoma, infantile fibrosarcoma, congenital mesoblastic nephroma and acute myelogenous leukemia. [1]

Patients with MASC of the salivary gland are considered to have favorable outcome with a mean disease-free survival about 92 months. [3] Like ACC, some cases of MASC show high grade transformation with rapid increase in size, frequent mitoses, cellular pleomorphism, infiltrative margins, perineural invasion, high proliferation index (usually more than 45%) and p53 overexpression. [10] In our case, tumor recurrence, large size, presence of large areas of necrosis and moderately high proliferation index (20%) suggested an aggressive tumor; however, there was no nerve palsy, significant cellular atypia, or nodal metastasis. The index case thus represents a huge, lipid-rich, solid variant of MASC of the salivary gland in a young male. It also highlights an aggressive behavior of the tumor in otherwise low-grade morphology. Histopathological recognition of this entity is important to arrive at an accurate diagnosis, for proper management, and prediction of prognosis.

 
   References Top

1.
Skálová A, Vanecek T, Sima R, Laco J, Weinreb I, Perez-Ordonez B, et al. Mammary analogue secretory carcinoma of salivary glands, containing the ETV6-NTRK3 fusion gene: A hitherto undescribed salivary gland tumor entity. Am J Surg Pathol 2010;34:599-608.  Back to cited text no. 1
    
2.
Tognon C, Knezevich SR, Huntsman D, Roskelley CD, Melnyk N, Mathers JA, et al. Expression of the ETV6-NTRK3 gene fusion as a primary event in human secretory breast carcinoma. Cancer Cell 2002;2:367-76.  Back to cited text no. 2
    
3.
Chiosea SI, Griffith C, Assaad A, Seethala RR. Clinicopathological characterization of mammary analogue secretory carcinoma of salivary glands. Histopathology 2012;61:387-94.  Back to cited text no. 3
    
4.
Connor A, Perez-Ordoñez B, Shago M, Skálová A, Weinreb I. Mammary analog secretory carcinoma of salivary gland origin with the ETV6 gene rearrangement by FISH: Expanded morphologic and immunohistochemical spectrum of a recently described entity. Am J Surg Pathol 2012;36:27-34.  Back to cited text no. 4
    
5.
Bishop JA. Unmasking MASC: Bringing to light the unique morphologic, immunohistochemical and genetic features of the newly recognized mammary analogue secretory carcinoma of salivary glands. Head Neck Pathol 2013;7:35-9.  Back to cited text no. 5
    
6.
Hwang MJ, Wu PR, Chen CM, Chen CY, Chen CJ. A rare malignancy of the parotid gland in a 13-year-old Taiwanese boy: Case report of a mammary analogue secretory carcinoma of the salivary gland with molecular study. Med Mol Morphol 2014;47:57-61.  Back to cited text no. 6
    
7.
Jung MJ, Song JS, Kim SY, Nam SY, Roh JL, Choi SH, et al. Finding and characterizing mammary analogue secretory carcinoma of the salivary gland. Korean J Pathol 2013;47:36-43.  Back to cited text no. 7
    
8.
Mariano FV, dos Santos HT, Azañero WD, da Cunha IW, Coutinho-Camilo CM, de Almeida OP, et al. Mammary analogue secretory carcinoma of salivary glands is a lipid-rich tumour, and adipophilin can be valuable in its identification. Histopathology 2013;63:558-67.  Back to cited text no. 8
    
9.
Chiosea SI, Griffith C, Assaad A, Seethala RR. The profile of acinic cell carcinoma after recognition of mammary analog secretory carcinoma. Am J Surg Pathol 2012;36:343-50.  Back to cited text no. 9
    
10.
Skálová A, Vanecek T, Majewska H, Laco J, Grossmann P, Simpson RH, et al. Mammary analogue secretory carcinoma of salivary glands with high-grade transformation: Report of 3 cases with the ETV6-NTRK3 gene fusion and analysis of TP53, ß-catenin, EGFR, and CCND1 genes. Am J Surg Pathol 2014;38:23-33.  Back to cited text no. 10
    

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Correspondence Address:
Dr. Asit Ranjan Mridha
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.162897

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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