LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1112
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
LETTER TO EDITOR  
Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 430-432
Male breast fibromatosis


Department of Pathology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India

Click here for correspondence address and email

Date of Submission09-Sep-2019
Date of Decision18-Feb-2020
Date of Acceptance08-Jun-2020
Date of Web Publication9-Apr-2021
 

How to cite this article:
Uttam P, Gupta RK, Hussain N. Male breast fibromatosis. Indian J Pathol Microbiol 2021;64:430-2

How to cite this URL:
Uttam P, Gupta RK, Hussain N. Male breast fibromatosis. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 9];64:430-2. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/430/313261




Dear Editor,

Fibromatosis is an infiltrative proliferation of fibroblastic and myofibroblastic cells with significant risk for local recurrence, but no metastatic potential. It originates mainly from the fascia or aponeuroses of the abdominal wall muscles or from the muscles of the shoulders and pelvic girdles.[1] Extra-abdominal desmoid tumors account for 3% of all soft-tissue tumors, with an incidence of 2.4-4.3 per million each year. Fibromatosis of the chest wall represents 8%–10% of all cases.[2] Fibromatosis is uncommon in the mammary gland and accounts for less than 0.2% of all primary breast lesions. Although several series of this condition have been reported in women, mammary fibromatosis in men is extremely rare.[1]

A 40-year-old male presented in the outpatient department of surgery with the chief complaints of a slowly progressive, painless right breast lump for 3 years. The findings of the physical examination revealed a firm painless smooth swelling in the upper inner quadrant of the right breast just above and medial to right nipple/areola, measuring 5 cm × 5 cm, with no clinically palpable axillary lymph nodes. Family history was noncontributory. The patient was a known case of coronary artery disease with a history of percutaneous transluminal coronary angioplasty and stenting in the right coronary artery 5 years back. Positron emission tomography scan revealed a homogeneously enhancing soft-tissue mass lesion measuring 30 mm with fluorodeoxyglucose uptake of low intensity in the subareolar region of the right breast, pattern most likely representing a benign lesion. Fine-needle aspiration cytology performed outside and reported as spindle cell neoplasm. A core needle biopsy was also done in a private hospital and reported as low-grade spindle cell lesion with collagen trapping likely fibroblastic/myofibroblastic in origin, possibly fibromatosis. A wide local excision of the mass (lumpectomy) was planned in our institute after preanesthetic clearance. Patient is disease free with no apparent sign of recurrence and doing well at 8 months of follow up.

We received a globular firm soft-tissue mass measuring 4.5 cm × 4 cm × 3 cm. Cut section of the mass showed a nonencapsulated firm grey-white tumor measuring 3.7 cm × 3 cm × 2.7 cm. No areas of necrosis or hemorrhage were noted [Figure 1].
Figure 1: Gross images showing (a) A globular circumscribed soft tissue mass, (b) Cut section of the mass showing a nonencapsulated homogenous grey-white tumor

Click here to view


The sections showed a tumor comprising of spindle cells with bland nuclei, arranged in interlacing fascicles separated by thick collagen bundles along with mild focal lymphocytic infiltrate [Figure 2]a [Figure 2]d. At the periphery of the lesion, focal irregular and infiltrating finger-like projections extending into the adjacent adipose tissue noted. Occasional mitotic figures were seen; however, no necrosis was noted. Further immunohistochemistry (IHC) studies were performed for characterization which revealed diffuse strong nuclear positivity for β-catenin [Figure 2]e, and diffuse cytoplasmic positivity for smooth muscle actin. The IHC for cytokeratin-7, CD34 [Figure 2]f, estrogen receptor (ER), and progesterone receptor (PR) were negative.
Figure 2: Images showing (a) A spindle cells tumor with bland nuclei, arranged in intersecting fascicles separated by thick collagen bundles (HE x40), (b) The periphery of lesion showing irregular and infiltrating finger-like projections extending into adjacent adipose tissue (HE x40), (c) Mild focal lympho-plasmacytic infiltrate (Arrow) (HE x20), (d) High power image showing spindle cells with vesicular chromatin, prominent nucleolei and moderate amount of cytoplasm (HE x100), (e) Diffuse strong nuclear positivity for β-catenin (IHC x100), and (f) CD34 stain highlighting blood vessels (IHC x100)

Click here to view


The World Health Organization defines desmoid-type fibromatosis as an intermediate grade soft-tissue tumor that is characterized by clonal fibroblastic proliferation arising in the deep soft tissues, which tend infiltration into the local tissues and local recurrence, but usually do not metastasize.[2]

Mammary fibromatosis is a rare entity, accounting for less than 0.2% of all breast tumors. Patient's age ranges from 13 to 83 years with a reported mean age at diagnosis 37–50.3 years. While the vast majority of cases occur in women, rare cases have been reported in men.[3]

In 1964, the first reported case of mammary fibromatosis in a patient with Gardner's syndrome was noted. The β-catenin pathway is implicated in sporadic lesions, with dysfunction of the CTNNB1 gene. Overall, sporadic cases are more common, and only 5%–15% cases show association with familial adenomatous polyposis syndrome (FAP).[4]

Clinically, it presents as a palpable, firm, painless mass with skin dimpling and nipple retraction in superficial and retroareolar quadrant. Usually, nipple discharge and axillary lymphadenopathy are not found in breast fibromatosis.[5] Our case had a similar presentation with painless firm retroareolar lump, but nipple retraction and skin dimpling were not noted. Mammography typically reveals a stellate spiculated mass that may be indistinguishable from carcinoma. In this patient, mammography was not done. Microcalcifications are rarely found but may be present in the entrapped benign breast tissue.[6]

Grossly, the lesion appears as a rubbery, poorly vascularized grayish-white mass. Although cellular atypia may be present, the spindle cells are typically uniform with a low mitotic index. The histopathologic differential diagnosis includes myofibroblastoma, scar formation, low-grade myofibroblastic sarcoma, nodular fasciitis, and fibromatosis-like metaplastic spindle cell tumor.[7] In the present case, no significant nuclear atypia was noted, mitosis was occasional, no epithelioid differentiation was noted and IHC for cytokeratin was also negative.

Fibromatosis in the breast differs from fibromatosis arising in other parts of the body due to its hormone receptor profile. Although 30% of extramammary fibromatosis are positive for ERs, only one of the previously reported cases of mammary fibromatosis expressed hormonal receptors.[1]

Because of the consistent absence of immunoreactivity for ERs and PRs in mammary fibromatosis, a positive reaction for these receptors in spindle cell neoplasms of the breast might help exclude fibromatosis from its differential diagnoses.[1] In the present case, ER and PR markers were negative.

Treatment consists primarily of wide local excision with clear margins. Re-excision for positive margins should be undertaken to prevent the invasion of the chest wall. Negative margins do not completely preclude the possibility of recurrence.[3],[8] The roles of adjuvant radiotherapy, chemotherapy, and hormone therapy are less well defined and the optimal regimes, doses, and durations of systemic treatment of the disease require further elucidation.[9]

To conclude, male breast desmoid-like fibromatosis is very rare neoplasm with the potential for aggressive local behavior. Breast imaging is nonspecific which often imitates breast cancer. Fibromatosis should be ruled out in all the cases of male breast masses showing spindle cell tumors in FNAC/biopsy which can be further confirmed with an appropriate IHC panel.

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.
Macchetti AH, Marana HR, Ribeiro-Silva A, de Andrade JM, Melo CF. Fibromatosis of the male breast: A case report with immunohistochemistry study and review of the literature. Clinics (Sao Paulo) 2006;61:351-4.  Back to cited text no. 1
    
2.
Benej R, Mečiarová I, Pohlodek K. Desmoid-type fibromatosis of the breast: A report of 2 cases. Oncol Lett 2017;14:1433-8.  Back to cited text no. 2
    
3.
Schwarz GS, Drotman M, Rosenblatt R, Milner L, Shamonki J, Osborne MP. Fibromatosis of the breast: Case report and current concepts in the management of an uncommon lesion. Breast J 2006;12:66-1.  Back to cited text no. 3
    
4.
Bhat D, Wear V, Weisenberg E, Alvarado R. Desmoid-type fibromatosis of the breast: A case report. Breast Dis 2016;36:149-2.  Back to cited text no. 4
    
5.
Abdelwahab K, Hamdy O, Zaky M, Megahed N, Elbalka S, Elmetwally M, et al. Breast fibromatosis, an unusual breast disease. J Surg Case Rep. 2017;2017:rjx248.  Back to cited text no. 5
    
6.
McMenamin ME, DeSchryver K, Fletcher CD. Fibrous lesions of the breast: A review. Int J Surg Pathol 2000;8:99-08.  Back to cited text no. 6
    
7.
Ha KY, Deleon P, Hamilton R. Breast fibromatosis mimicking breast carcinoma. Proc (Bayl Univ Med Cent) 2013;26:22-4.  Back to cited text no. 7
    
8.
Rana C, Ramakant P, Babu S, Singh K, Mishra A, Mouli S. Unusual breast neoplasm with diagnostic and management challenges. Indian J Surg Oncol 2018;9:328-35.  Back to cited text no. 8
    
9.
Greenberg D, McIntyre H, Ramsaroop R, Arthur J, Harman J. Aggressive fibromatosis of the breast: A case report and literature review. Breast J 2002;8:55-7.  Back to cited text no. 9
    

Top
Correspondence Address:
Rakesh Kumar Gupta
Department of Pathology, Third Floor, Adminstrative Block, All India Institute of Medical Sciences, Raipur - 492 099, Chhattisgarh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_649_19

Rights and Permissions


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed76    
    Printed0    
    Emailed0    
    PDF Downloaded4    
    Comments [Add]    

Recommend this journal