|Year : 2015 | Volume
| Issue : 3 | Page : 666
Primary breast lymphoma with progression to the scalp
Arundhati, Kiran Preet Malhotra, Nuzhat Husain
Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
|Date of Web Publication||9-Oct-2015|
Kiran Preet Malhotra
Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Primary lymphomas developing in the breast constitute a rare entity. A 50-year-old female presented with a breast lump for duration of 2 months. She also had a scalp swelling since 2 days. No lymph nodal involvement was present. Histopathological examination and immunohistochemistry performed on a core needle biopsy of the breast lump revealed a diffuse large B-cell non-Hodgkin's lymphoma, an aspirate of the scalp swelling revealed progression from the primary breast lymphoma (PBL). We review the relevant literature and discuss the diagnostic aspects of extranodal lymphomas arising in the breast. Disease progression to the scalp from PBL has not been reported until date. We hereby report the first case of progression to the scalp in a case of PBL.
Keywords: Breast, disease progression, extranodal lymphoma, non-hodgkin′s lymphoma, scalp
|How to cite this article:|
Arundhati, Malhotra KP, Husain N. Primary breast lymphoma with progression to the scalp. J Can Res Ther 2015;11:666
| > Introduction|| |
Primary lymphoma of the breast is an uncommon entity. Lymphomas mostly have their origin in the immune system; of them, 40% are of Hodgkin's and 60% of non-Hodgkin's type.  Primary extranodal involvement has been described in organs containing areas of lymphoid tissue, such as gastrointestinal tract, skin, bone, brain, and Waldayer's ring.  Primary breast lymphoma (PBL) accounts for 1.7-2.2% ,,,, of all extra nodal lymphomas and a meager 0.7% of all non-Hodgkin's lymphomas (NHL).  The paucity of lymphoid tissue in the breast justifies this low incidence. PBL is substantially outnumbered by the more common ductal and mesenchymal neoplasms in the breast and accounts for only 0.04-0.5% of all breast malignancies. ,
| > Case report|| |
A 50-year-old female presented with pain and a lump in the right breast, for duration of 2 months with a history of gradual increase in size [Figure 1]a. She also noted a lump in the scalp since 3-4 days [Figure 1]b. On examination, the right breast showed a firm mass of 6 cm × 5 cm in the inferolateral quadrant. The scalp swelling was firm, nodular, measured 2 cm × 2 cm and was overlying the left side of the vault. The skin was not involved grossly, and was freely mobile over both the breast and scalp lesions. The patient did not complain of B symptoms (fever, weight loss, or night sweats). She was hypertensive and on medication for last 2 years. There was no significant gynecologic and obstetric history or family history. There was no lymphadenopathy or hepatosplenomegaly. On investigation, random blood sugar, liver function tests, kidney function tests, chest X-ray, and ultrasonography of the abdomen were within normal limits. She was nonreactive for HIV antibody, hepatitis B surface antigen, and hepatitis C virus antibody.
|Figure 1: (a) Clinical photograph of the patient showing a lump in the right breast (arrows) (inset: Closer view) (b) Clinical photograph of the patient showing a lump in the scalp (inset: Closer view)|
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A skull X-ray showed a small round soft tissue mass in the left vault; no bony infiltration of the calvarium was seen. A mammography showed a fairly large, inhomogeneous opacity with ill-defined margins, seen in the inferolateral quadrant of the right breast. The skin and subcutaneous tissue looked normal. No nipple retraction or abnormal calcification was noted. On the basis of findings of parenchymal opacity, a Breast Imaging Reporting and Data System (BIRADS) category III was suggested.
Fine-needle aspiration from the breast tumor showed predominantly monomorphic medium to large cells overlying a blood mixed background and was reported as round cell tumor, requiring biopsy for further characterization. Core biopsy from the breast mass showed a diffuse infiltration of large neoplastic cells in the breast parenchyma. The cells were round with pleomorphic, hyperchromatic nuclei and prominent nucleoli. Few benign glands lined by bilayered epithelium were seen entrapped within the infiltrate. Differentials of NHL, high-grade infiltrating duct carcinoma, solid variant of lobular carcinoma and medullary carcinoma were considered [Figure 2]a. On immunohistochemistry (IHC), the neoplasm was negative for pan cytokeratin (CK), excluding epithelial malignancy [Figure 2]b. The neoplastic cells were diffusely positive for leukocyte common antigen-CD45 [Figure 2]c and CD20 [Figure 2]d. The lesion was hence diagnosed as NHL of diffuse large B-cell type. Fine-needle aspiration from the scalp lesion showed sheets of similar atypical, immature lymphoid cells suggesting progression from the PBL. A bone marrow biopsy did not reveal any lymphomatous infiltration.
|Figure 2: (a)Photomicrograph of the core needle biopsy taken from breast lesion showing small round cells in sheets (H and E, ×100) (b) Immunohistochemistry showing negative stain for cytokeratin (diaminobenzidine, ×100) (c) Immunohistochemistry showing strong positive staining of the lymphoid cells for CD45 (diaminobenzidine, ×100) (d) Immunohistochemistry showing positive stain of the lymphoid cells for CD20; an entrapped breast duct is unstained (diaminobenzidine, ×400)|
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| > Discussion|| |
Primary breast lymphoma is an uncommon neoplasm of the breast accounting for 0.04-0.5% ,,, of all breast malignancies. When compared to other tissues, breast has relatively scant lymphoid tissue, which attributes to low incidence of PBL almost always of non-Hodgkin's type. Among the extra nodal involvements of NHL, PBL accounts for 1.7-2.2%. ,,, A predilection for the right breast may exist, which was also noted in our case.  The median age for PBL has been reported as 40-67 years. Clinically, both carcinoma and lymphoma of breast present alike, usually as painless masses.
Nonspecific findings and absence of microcalcification in mammography may cause further delay in diagnosis. In this case, mammography did not show any microcalcification, and parenchymal lesion showed BIRADS category III. Biopsy and IHC remain the gold standard for establishing diagnosis. Majority of PBL are of B-cell lineage ,, as in this case. Common observed types are diffuse large B-cell lymphoma, follicular, and mantle zone lymphomas. IHC has a great role in clinching the diagnosis. As in this case, negative staining for pan CK ruled out the possibilities of poorly differentiated ductal and lobular carcinomas. Further positivity for CD45 and CD20 and negative stain for CD3 established the diagnosis of NHL of diffuse large B-cell type.
The few reported series of PBLs show a predilection of spread to the contralateral or ipsilateral breast, deep-seated and peripheral lymph nodes, central nervous system (CNS) and bone marrow.  However, no involvement of marrow, lymph node or other organ besides scalp was noted in our case.
To the best of our knowledge, no case of disease progression to the scalp from a PBL has been reported until date. On the other hand, infiltrating breast carcinomas are known to metastasize hematogenously to the cranium; however, PBLs do not seem to follow the same course. In a retrospective review, it has been observed that PBLs fare worse with surgery as the primary treatment and radiotherapy is of distinct advantage for local control of PBLs.  Systemic relapses notably in the CNS are known to occur in PBLs. In our patient, chemotherapy (CHOP regime) has been instituted and no other progression at any site has been observed over a follow-up of 2 months.
| > Conclusion|| |
We report the first case of PBL with disease progression to the scalp. This report highlights the importance of recognizing breast as a rare primary site for extranodal lymphomas. An unequivocal diagnosis of this entity requires astute pathologic examination, supported by immunohistochemical analysis. Distinct treatment protocols for usual ductal carcinomas and lymphomas attests to the importance of recognizing this rare entity.
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[Figure 1], [Figure 2]