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CORRESPONDENCE
Year : 2019  |  Volume : 15  |  Issue : 5  |  Page : 1167-1169

Clear cell carcinoma of breast lipid-rich variant


1 Department of Pathology, Kasturba Medical College, Manipal Academy of Higher Education, Mangalore, Karnataka, India
2 Department of Surgery, Kasturba Medical College, Manipal Academy of Higher Education, Mangalore, Karnataka, India

Date of Web Publication4-Oct-2019

Correspondence Address:
Pooja K Suresh
Department of Pathology, Kasturba Medical College, Manipal Academy of Higher Education, Lighthouse Hill Road, Mangalore - 575 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.JCRT_870_17

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 > Abstract 


Clear cell breast carcinomas are one of the rare types of invasive carcinoma of the breast. Among them, lipid-rich variant is still rare comprising <1%. The tumor derives its name from the intracytoplasmic neutral lipid that gives the cytoplasm a vacuolated and foamy appearance. These tumors are usually hormone receptors negative and Her-2 Neu positive. Here, we report a case of lipid-rich breast carcinoma which showed hormone receptor positivity and Her-2 Neu negative.

Keywords: Breast carcinoma, clear cell, Her-2 Neu, lipid-rich


How to cite this article:
Kini H, Bhat S, Suresh PK, Thomas M. Clear cell carcinoma of breast lipid-rich variant. J Can Res Ther 2019;15:1167-9

How to cite this URL:
Kini H, Bhat S, Suresh PK, Thomas M. Clear cell carcinoma of breast lipid-rich variant. J Can Res Ther [serial online] 2019 [cited 2019 Nov 15];15:1167-9. Available from: http://www.cancerjournal.net/text.asp?2019/15/5/1167/244481




 > Introduction Top


Lipid-rich breast carcinoma (LRBC) is a type of clear cell carcinoma of the breast with the incidence of <1% of all breast carcinomas. It is defined as a tumor with >90% of the tumor cells containing abundant cytoplasmic neutral lipids. They can be easily missed or misdiagnosed in a breast core biopsy specimen. It is characterized by aggressive behavior and poor prognosis and requires prompt recognition and close follow-up.[1],[2] Most of the cases which have been described in the literature have been estrogen and progesterone receptors (ER and PR) negative and Her-2 Neu positive. In this report, we describe LRBC occurring in a 43-year-old woman exhibiting ER, PR positivity, and Her-2 Neu negativity.


 > Case Report Top


A 43-year-old female came to the surgical outpatient department with complaints of the left breast lump for 3 months. It initially started with a small lump and had grown ever since to the present size. For 15 days, it is associated with severe pain. She also complains of weight loss for 15 days. There was no significant family history of breast carcinoma in the family. There is no history of hypertension, diabetes mellitus, and tuberculosis.

Local examination revealed a nontender lump in the left central quadrant and upper and lower outer quadrant measuring 12 cm × 8 cm. The borders of the lump were irregular and hard in consistency. The skin over the swelling was unremarkable. The nipple did not show any discharge or retraction. A single axillary lymph node is seen measuring 2 cm × 1 cm, mobile and nontender.

Her blood investigation revealed a hemoglobin of 12.1 g/dl, total count 7800 cells/cumm, differential count N54 L37E3M6, and platelet count 269,000/cumm. Her renal function test and liver function tests were within normal range. Her Random blood sugar (RBS) was 131 mg/dl.

Computed tomography of the chest revealed an ill-defined mass lesion with lobulated margins measuring 53 mm × 26 mm × 56 mm in the retroareolar zone of the left breast mammary zone. Anteriorly, the lesion is extending up to the skin surface with thickening of overlying skin. Posteriorly fat planes with pectoral muscles are maintained. Few left axillary lymph nodes are noted, largest measuring 16 mm × 15 mm. No evidence of brain or bone metastasis. Ultrasound examination of the abdomen did not show any significant sonological abnormality.

A modified radical mastectomy was done with axillary clearance and sent for histopathological evaluation. Grossly, the tumor was centrally placed involving all the four quadrants of the breast. Cut surface was solid, gray-white in color, firm in consistency involving the skin and extending up to the deeper resected margins and pectoralis muscle. Fifteen lymph nodes were recovered from the axillary pad of fat. The largest measuring 1 cm × 1 cm, cut surface being pale white [Figure 1]a.
Figure 1: Lipid rich carcinoma of breast. (a) Gross image of the breast carcinoma involving the entire breast. (b-e) Histopathology showing the lobules and nests of tumor cells having clear cytoplasm and pleomorphic hyperchromatic nucleus. (f) Sudan IV special stain showing orange red positivity in the cytoplasm suggesting neutral lipids. (g-i) Estrogen and progesterone receptor positive and Her-2 Neu Negative

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The histological sections revealed tumor tissue arranged in sheets, nest, trabeculae, and single cell pattern with abundant intervening hyalinized collagen. The tumor cells are large, polygonal with vacuolated cytoplasm and moderately pleomorphic vesicular nucleus with inconspicuous nucleoli. Tubule formation is absent. Patchy areas of necrosis with aggregates of chronic inflammatory cells are noted. Brisk mitosis (18/10 hpf) is seen [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e. The occasional intraductal component with central necrosis and calcification is also noted. Lymph node metastasis is noted in 13 axillary lymph nodes and one internal mammary lymph node.

Special stains were done to assess the content of the vacuolated cytoplasm, which showed negativity for glycogen in periodic acid Schiff stain and negativity to mucin stains. Hence, frozen section with Sudan IV staining revealed positivity for fat in the tumor cells. Hence, it was diagnosed as lipid-rich carcinoma of the breast [Figure 1]f.

Immunohistochemistry showed ER and PR positive and Her-2 Neu negative [Figure 1]g, [Figure 1]h, [Figure 1]i. The patient was taken for chemotherapy and is free of recurrence for the past 6 months.


 > Discussion Top


LRBC was first described by Aboumrad, Horn and Fine in 1963. They called this lesion as “lipid secreting carcinoma;” however, today, the terminology used is “lipid-rich” breast carcinoma describing the presence of neutral lipid in the cytoplasm of the neoplastic cells rather than the active secretion of lipid by the neoplastic cells.[2],[3],[4],[5] Till date, only approximately 70 cases of LRBC has been reported in the literature.[6]

LRBC can be seen over a wide age ranging from 33 to 81 years, while our patient is a 43-year-old female. The presenting symptoms are usually a lump in the breast and rarely with nipple discharge. The lump usually involves the upper outer quadrant of the breast with the equal incidence of occurrence in both the breast.[4] Grossly, the size of the tumor may range from 1.2–15 cm. The cut surface is usually lobulated yellow to tan in color and firm in consistency.[1],[2]

Histologically, the tumor is composed of nests, cords, or sheets of large polygonal cells, which characteristically have a clear, vacuolated cytoplasm. The nucleus is vesicular and exhibit moderate atypia with conspicuous nucleoli. The histological grade of these tumors is usually grade 3. As per the WHO classification [1] and many studies,[3],[4] the diagnosis requires the presence of neutral lipid in >90% of the neoplastic cells. The neutral lipids are highlighted mainly on frozen section with Oil red O or Sudan IV staining. Mucin and glycogen are typically less conspicuous and would be positive on mucicarmine and PAS, respectively.[2]

In a study of 17 cases of lipid-rich carcinomas, all were negative for hormonal receptors and positive for HER-2 Neu.[4] Similarly, in another study of 49 cases of lipid-rich carcinoma, ER and/or PR positivity was found in 10.2% cases, while 71.4% of cases were HER-2 positive.[7] Machalekova et al.[3] reported a single case of lipid-rich carcinoma with invasive ductal carcinoma in the same breast; which showed negativity for hormonal receptors, and exhibited positive staining for HER-2 and p53, with a high Ki-67 proliferative index. Together, these data suggest that LRBC usually exhibit hormonal receptors negativity and HER-2 positivity. In the present case, we found the neoplastic cells were hormonal receptors positive and HER-2 Neu negative. This was supported by two case reports which also showed positivity for one of the hormonal receptors and negative for Her-2 Neu.[5],[6] Hence, this would be the third reported case with this finding. Electron microscopy study done in a few cases showed well-developed Golgi apparatus and lipid droplets and globules of different sizes in the cytoplasm of the tumor cells.[1],[4]

Many reports have suggested that these tumors are more aggressive than the usual ductal carcinomas and have different biopathological behavior. However, most patients were treated with mastectomy and axillary dissection as per the standard protocol. More than half of the patients show metastatic tumor in the axillary lymph nodes.[3] Our patient found to have negative axilla, despite the large size of the primary tumor. In view of the aggressiveness of the tumor, systemic therapy needs to be given. The 2- and 5-year overall survival rates of these tumors are 64.6% and 33.2%, respectively.[5],[6]

The differential diagnoses for breast carcinoma with clear cells are hidradenocarcinoma, histiocytoid lobular carcinoma, apocrine carcinoma, clear cell “sugar” carcinoma, glycogen-rich carcinoma, secretory carcinoma, adenomyoepithelioma, signet ring carcinoma, sebaceous carcinoma, and metastatic renal cell carcinoma and benign conditions such as fat necrosis and xanthogranulomatous mastitis.[1]

Lipid-rich carcinomas of the breast are extremely rare and unique cancer that should be recognized by pathologists and clinicians owing to its aggressive clinical behavior. This can be easily missed on a trucut biopsy, and a benign diagnosis can be rendered. Hence, it is very essential to keep the all the differentials of clear cells in mind while analyzing a breast carcinoma with clear cells. Early diagnosis and standard oncological treatment may be of use for increasing the overall survival of these patients. Furthermore, this is the 3rd case showing ER positivity as LRBCs are usually negative for ERs. Our case suggests that few cases of LRBC are ER-positive and hormonal therapy can be useful in this subset of patients.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Lakhani SR, Ellis IO, Schnitt SJ, Tan PH, Van de Vijver MJ. WHO Classification of Tumours of the Breast. 4th ed. Lyon: IARC Press; 2012  Back to cited text no. 1
    
2.
Sirohi D, Swimley K, Profit AL, Riddle ND. Her-2 Neu negative lipid rich breast carcinoma in an immunocompromised patient. Hum Pathol Case Rep 2015;2:99-102.  Back to cited text no. 2
    
3.
Machalekova K, Kajo K, Bencat M. Unusual occurrence of rare lipid-rich carcinoma and conventional invasive ductal carcinoma in the one breast: Case report. Case Rep Pathol 2012;2012:387045.  Back to cited text no. 3
    
4.
Guan B, Wang H, Cao S, Rao Q, Wang Y, Zhu Y, et al. Lipid-rich carcinoma of the breast clinicopathologic analysis of 17 cases. Ann Diagn Pathol 2011;15:225-32.  Back to cited text no. 4
    
5.
Cong Y, Lin J, Qiao G, Zou H, Wang X, Li X, et al. Lipid-rich carcinoma of the breast: A report of two cases and a literature review. Oncol Lett 2015;9:1729-32.  Back to cited text no. 5
    
6.
Shi P, Wang M, Zhang Q, Sun J. Lipid-rich carcinoma of the breast. A clinicopathological study of 49 cases. Tumori 2008;94:342-6.  Back to cited text no. 6
    
7.
Oba T, Ono M, Iesato A, Hanamura T, Watanabe T, Ito T. Lipid-rich carcinoma of the breast that is strongly positive for estrogen receptor: A case report and literature review. Onco Targets Ther 2016;9:1641-6.  Back to cited text no. 7
    


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