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  Table of Contents    
CASE REPORT  
Year : 2016  |  Volume : 59  |  Issue : 2  |  Page : 209-211
A rare case of multicentric secretory carcinoma of breast in an adult female with review of literature


Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

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Date of Web Publication9-May-2016
 

   Abstract 

Secretory breast carcinomas (SBCs) are considered one of the rarest types of BCs accounting for <0.15% of all breast cancers. The lesions are typically solitary, but rare multicentric cases have been reported. SBC have characteristic histopathological, immunohistochemical, and electron microscopic findings. Children and adolescents with SBC have a favorable prognosis, but disease seems slightly more aggressive in adults. The characteristic ETV6-NTRK3 molecular alteration, leading to a stable chimeric tyrosine kinase fusion product, may be the target of promising new treatment for this unique BC. We present a rare case of multicentric secretory carcinoma of breast with aggressive clinical behavior in a 75-year-old female.

Keywords: Immunohistochemistry, secretory breast carcinoma, multicenrtic

How to cite this article:
Kulkarni MM, Khandeparkar SG, Joshi AR, Dhande AN. A rare case of multicentric secretory carcinoma of breast in an adult female with review of literature. Indian J Pathol Microbiol 2016;59:209-11

How to cite this URL:
Kulkarni MM, Khandeparkar SG, Joshi AR, Dhande AN. A rare case of multicentric secretory carcinoma of breast in an adult female with review of literature. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Dec 16];59:209-11. Available from: http://www.ijpmonline.org/text.asp?2016/59/2/209/182021



   Introduction Top


Secretory breast carcinomas (SBCs) are considered one of the rarest types of BCs accounting for <0.15% of all breast cancers.[1] The clinical presentation is a slow growing, painless, well-circumscribed, mobile, palpable mass.[2] The lesions are typically solitary, but rare multicentric cases have been reported.[3],[4] Axillary lymph node metastasis is uncommon, especially if tumors are <2 cm. In an occasional case, in which lymph node metastasis does occur, it rarely involves more than 3 lymph nodes.[1] As a rare type of BC, there are at present, no consensus guidelines for treatment. Surgical excision is the primary mode of treatment. The characteristic ETV6-NTRK3 molecular alteration, leading to a stable chimeric tyrosine kinase fusion product, may be the target of promising new treatment for this unique BC.[5] We present a rare case of multicentric secretory carcinoma with extensive cystic degeneration and skin involvement.


   Case Report Top


A 75-year-old postmenopausal female presented with left breast lump for 1 year. The lump gradually increased to present size. Nipple discharge was present for 2–3 months. On clinical examination, the overlying skin was fixed, and there was presence of nipple retraction. Mammography showed large well-defined homogeneous radio-opaque lesion noted in the retroareolar region measuring 6 cm × 5 cm with focal area of heterogeneous opacity with irregular margins with focal surrounding desmoplastic reaction in the left retroareolar region. True cut biopsy done was given as positive for malignancy. The patient underwent left modified radical mastectomy (MRM). Grossly, the skin, nipple, and areola were involved. Tumor was noted measuring 5 cm × 5 cm × 5 cm and was almost involving the entire breast. Tumor was well-circumscribed, solid, grayish white, and showed cystic degeneration with hemorrhagic fluid. ([Figure 1]a) There were two satellite nodules measuring 3 cm × 3 cm × 2 cm and 1 cm in diameter. Microscopically, there was a partially circumscribed tumor composed of nests separated by dense collagenous stroma. ([Figure 1]b) Tumor cells were arranged in microcystic, tubular, and at places papillary pattern. Tumor cells had vesicular nuclei with inconspicuous nucleoli, moderate nuclear pleomorphism, and moderate amount of eosinophilic cytoplasm. There were intraluminal as well as intracytoplasmic eosinophilic secretions in tumor cells. ([Figure 1]c) Two out of 15 lymph nodes showed tumor deposits. ([Figure 1]d) Stroma showed areas of hyalinization. There were areas of cystic degeneration filled with hemorrhagic fluid. Few lymphovascular emboli were noted. Satellite nodules showed similar histomorphological features. Overlying skin was involved by the tymor. ([Figure 1]e) Surrounding breast showed apocrine metaplasia with epitheliosis. ([Figure 1]f) Periodic acid-Schiff (PAS) stain showed PAS-positive diastase-resistant intratubular and intracytoplasmic secretions. Immunohistochemical was done with estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2), S100, cytokeratin 5/6 (CK 5/6), epidermal growth factor receptor (EGFR), and E-cadherin. The tumor cells were negative for ER, PR, and HER2 (triple negative). Tumor cells showed immunoreactivity for S100([Figure 1]g), CK5/6([Figure 1]h), E-cadherin, and vimentin. The final diagnosis of secretory carcinoma of the left breast was given.


   Discussion Top


In 1966, McDivitt and Stewart described 7 cases of BC with a distinct morphology in young children and named it juvenile carcinoma.[6] However, subsequent studies have included many adult patients. The tumors were all morphologically characterized by the presence of abundant eosinophilic secretions in intracellular vacuoles and intercellular spaces. The descriptive term SBC, therefore replaced the original designation of juvenile carcinoma.

These tumors have been reported in both sexes. The median age at presentation is 25 years (3–87 years).[1] Mammography usually reveals a discrete tumor with smooth or irregular borders.[7] SBC is considered a form of duct carcinoma. Tumor cells, glands, and microcystic spaces contain abundant secretions, which is usually pale pink or amphophilic. Secretions react variably for mucin and with PAS and is diastase-resistant.[8] Strong positive staining has been reported for alpha-lactalbumin as well as S100 protein and CEA.[9] Secretory carcinomas in women are ER(−), PR(−), HER2/neu(−), and so are called triple negative.[1] The tumor cells are known to be immunoreactive for S100, E-cadherin, and focally for CK8/8 and CK5/6.[1] Local excision is the preferred initial treatment in children.

SBC is associated with a characteristic balanced translocation, t (12;15) that creates an ETV6-NTRK3 gene fusion.[1] The differential diagnosis includes a wide range of benign and malignant lesions. Cystic hypersecretory carcinoma and apocrine carcinoma are primary malignant lesions that may resemble SBC morphologically. Most cases of cystic hypersecretory carcinoma are intraductal carcinomas with no invasive component. Myoepithelial layer is present in contrast to SBC. The epithelial cells in cystic hypersecretory lesions lack intracytoplasmic secretions.

Children and adolescents with SBC have a favorable prognosis, but disease seems slightly more aggressive in adults. Axillary lymph node metastasis rarely involves more than three lymph nodes. Distant metastasis is extremely rare.[1] SBC are slow growing tumors and hence, may acquire additional genetic alterations, leading to more aggressive behavior and chemoresistance. Multicentricity, tumor size >2 cm, infiltrative margins, and >3 lymph nodes positive are indicators of a relatively worse prognosis.[5] Surgical excision is the primary mode of treatment for SBC. In children, local excision with sentinel lymph node mapping is the preferred initial treatment. In adults, because of the increased rate of recurrence, a simple mastectomy is recommended. MRM has been favored by some authors in cases with tumor size >2 cm and poor circumscription.[3] Adjuvant chemotherapy is often used in node-positive disease.

Our patient had a large tumor with extensive cystic degeneration. The cystic change has not been described in literature so far. Two lymph nodes showed tumor deposits. Overlying skin, nipple, and areola were involved by tumor representing adverse prognosis.

New definition for basal-like carcinoma includes 5 markers and is defined as those lacking ER, PR, HER2 expression, and expressing CK5/6 and/or EGFR.[10] Our case showed immunoreactivity for CK5/6 and it was triple negative thus fulfilling the definition of basal-like BC. Tumors with basal-like immunophenotype constitute a heterogeneous group of tumors and SBC is one of the basal-like tumors that has good prognosis.[11] In our case, tumor size was >2 cm and there was CK5/6 immunoreactivity. Thus, tumor size, patient's age, and CK5/6 immunoreactivity were significant determinants for prognosis in our case. The patient was not given adjuvant chemotherapy due to old age. Though SBC has good prognosis, in an adult patient, it can show high-risk behavior, especially if the size is more than 2 cm.

Our experience with the present case highlights the unusual subtype of BC in a postmenopausal female with distinct gross appearance, which needs early careful immunohistomorphological evaluation for better management and prognosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Tavassoli FA. Secretory carcinoma. In: Tavassoli FA, Devilee P, editors. Pathology and Genetics of Tumors of the Breast and Female Genital Organs. World Health Organization Classification of Tumours. Vol. 4. Lyon, France: IARC Press; 2003. p. 42-3.  Back to cited text no. 1
    
2.
Rosen PP. Secretory carcinoma. In: Rosen's Breast Pathology. 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2009. p. 563-70.  Back to cited text no. 2
    
3.
Richard G, Hawk JC 3rd, Baker AS Jr., Austin RM. Multicentric adult secretory breast carcinoma: DNA flow cytometric findings, prognostic features, and review of the world literature. J Surg Oncol 1990;44:238-44.  Back to cited text no. 3
    
4.
Beatty SM, Orel SG, Kim P, Abraham SC, Reynolds C. Multicentric secretory carcinoma of the breast in a 35-year-old woman: Mammographic appearance and the use of core biopsy in preoperative management. Breast J 1998;4:200-3.  Back to cited text no. 4
    
5.
Vasudev P, Onuma K. Secretory breast carcinoma: Unique, triple-negative carcinoma with a favorable prognosis and characteristic molecular expression. Arch Pathol Lab Med 2011;135:1606-10.  Back to cited text no. 5
    
6.
McDivitt RW, Stewart FW. Breast carcinoma in children. JAMA 1966;195:388-90.  Back to cited text no. 6
    
7.
Siegel JR, Karcnik TJ, Hertz MB, Gelmann H, Baker SR. Secretory carcinoma of the breast. Breast J 1999;5:204-7.  Back to cited text no. 7
    
8.
Alenda C, Aranda FI, Seguí FJ, Laforga J. Secretory carcinoma of the male breast: Correlation of aspiration cytology and pathology. Diagn Cytopathol 2005;32:47-50.  Back to cited text no. 8
    
9.
Hartman AW, Magrish P. Carcinoma of breast in children; case report: Six-year-old boy with adenocarcinoma. Ann Surg 1955;141:792-8.  Back to cited text no. 9
    
10.
Laé M, Fréneaux P, Sastre-Garau X, Chouchane O, Sigal-Zafrani B, Vincent-Salomon A. Secretory breast carcinomas with ETV6-NTRK3 fusion gene belong to the basal-like carcinoma spectrum. Mod Pathol 2009;22:291-8.  Back to cited text no. 10
    
11.
Cheang MC, Voduc D, Bajdik C, Leung S, McKinney S, Chia SK, et al. Basal-like breast cancer defined by five biomarkers has superior prognostic value than triple-negative phenotype. Clin Cancer Res 2008;14:1368-76.  Back to cited text no. 11
    

Top
Correspondence Address:
Maithili Mandar Kulkarni
Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Narhe, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.182021

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