LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1314
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size
IJPM is coming out with a Special issue on "Genitourinary & Gynecological pathology including Breast". Please submit your articles for these issues


 
  Table of Contents    
LETTER TO EDITOR  
Year : 2016  |  Volume : 59  |  Issue : 4  |  Page : 563-564
Coexistence of breast carcinoma, pseudoangiomatous stromal hyperplasia, and neurofibromatosis type 1 in a male patient


Department of Pathology, Faculty of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece

Click here for correspondence address and email

Date of Web Publication10-Oct-2016
 

How to cite this article:
Christos PA, Triantafyllia KD, Prodromos HM, Ioannis KS. Coexistence of breast carcinoma, pseudoangiomatous stromal hyperplasia, and neurofibromatosis type 1 in a male patient. Indian J Pathol Microbiol 2016;59:563-4

How to cite this URL:
Christos PA, Triantafyllia KD, Prodromos HM, Ioannis KS. Coexistence of breast carcinoma, pseudoangiomatous stromal hyperplasia, and neurofibromatosis type 1 in a male patient. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Jul 21];59:563-4. Available from: http://www.ijpmonline.org/text.asp?2016/59/4/563/191773


Editor,

Pseudoangiomatous stromal hyperplasia (PASH) is a rare, histologically benign, finding in breast specimens. PASH has been described in some female patients with breast carcinoma but not in males. An exceptional case of PASH-associated breast carcinoma in a male patient with neurofibromatosis type 1 (NF1) is described.

A 42-year-old man presented with a left breast tumor (2.7 cm in greatest diameter), with features of malignancy. On physical examination, the patient had multiple cutaneous nodules. After a positive for malignancy open biopsy of the breast tumor, a modified radical mastectomy along with axillary lymph node dissection was performed. A nodule of the forearm was also excised.

Hematoxylin and eosin stained sections from the tumor led to the diagnosis of infiltrating ductal carcinoma, grade II [Figure 1]a. Immunohistochemical examination showed strong positivity for estrogen receptor, mild positivity for progesterone receptor, and focal positivity for androgen receptor. The adjacent breast parenchyma showed ductal dilatation with epithelial hyperplasia [Figure 1]b. All axillary lymph nodes were free of metastasis.
Figure 1: Infiltrative ductal carcinoma (IDC), of no special type (a). IDC, tubular dilatation with mild epithelial hyperplasia and an incidental neurofibroma (arrow) in the same section (b). Pseudoangiomatous stromal hyperplasiaPASH lesion characterized by cleft-like spaces, lined by flat cells (c), positive to CD34 antigen (d). Cutaneous neurofibroma (e) positive to S-100 protein (f). (a: H and E, ×100; b: H and E, ×20; c: H and E, ×100, inset ×400; d: IHC, ×100; e: H and E, ×20, inset ×100; f: IHC:, ×100)

Click here to view


Near the carcinoma, a lesion consisting of cleft-like empty spaces, lined by flat spindle cells in dense fibrous stroma was observed [Figure 1]c. Mitoses were absent. The spindle cells were positive for vimentin, CD34 antigen [Figure 1]d and smooth muscle actin (focally) and negative for CD31 antigen, FVIII, desmin, caldesmon, and S-100 protein. The overall features of this lesion were those of PASH. In one of the sections, an incidental small neurofibroma (0.2 cm) was found [Figure 1]b, arrow. The cutaneous lesion of the forearm was also diagnosed as a neurofibroma [Figure 1]e, positive for S-100 [Figure 1]f and CD34. The presence of multiple cutaneous nodules and histological confirmation of two neurofibromas indicated the diagnosis of NF1.

PASH is considered to be a benign lesion of myofibroblastic origin. It has been proposed to correspond to myofibroblastic response to abnormal hormonal stimuli, especially to progesterone. This is supported by the fact that usually occurs in premenopausal and in those menopausal women, who receive hormone therapy. In male patients, PASH usually coexists with gynecomastia, mainly, due to high estrogen levels. [1] Coincidental carcinoma has been reported in up to 25% of clinical evident PASH cases in women, but the correlation between PASH and breast carcinoma is not yet possible due to low number of reported cases. In addition, to the best of our knowledge, there was no reported case of breast carcinoma and PASH in male individual.

The coexistence of NF1 and breast carcinoma is proposed to be due to mutations in both NF1 and BRCA genes. [2] Controversial bibliographical data on the possible association between PASH and NF suggest that it is not yet fully accepted such correlation or a common pathogenetic mechanism. However, it is of note that five out of the six reported cases with the coexistence of PASH and NF regarded male patients. [3],[4]

This reported case is a unique case of coexistence of breast carcinoma, PASH, and neurofibromatosis in a male patient. Each of the above conditions is rare, and their coexistence calls for molecular investigation of their pathogenesis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest

 
   References Top

1.
Drinka EK, Bargaje A, Ersahin ÇH, Patel P, Salhadar A, Sinacore J, et al. Pseudoangiomatous stromal hyperplasia (PASH) of the breast: A clinicopathological study of 79 cases. Int J Surg Pathol 2012;20:54-8.  Back to cited text no. 1
    
2.
Campos B, Balmaña J, Gardenyes J, Valenzuela I, Abad O, Fàbregas P, et al. Germline mutations in NF1 and BRCA1 in a family with neurofibromatosis type 1 and early-onset breast cancer. Breast Cancer Res Treat 2013;139:597-602.  Back to cited text no. 2
    
3.
Damiani S, Eusebi V, Peterse JL. Malignant neoplasms infiltrating pseudoangiomatous' stromal hyperplasia of the breast: An unrecognized pathway of tumour spread. Histopathology 2002;41:208-15.  Back to cited text no. 3
[PUBMED]    
4.
Kimura S, Tanimoto A, Shimajiri S, Sasaguri T, Yamada S, Wang KY, et al. Unilateral gynecomastia and pseudoangiomatous stromal hyperplasia in neurofibromatosis: Case report and review of the literature. Pathol Res Pract 2012;208:318-22.  Back to cited text no. 4
[PUBMED]    

Top
Correspondence Address:
Poulios A Christos
Department of Pathology, Faculty of Medicine, Aristotle University of Thessaloniki, Thessaloniki
Greece
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.191773

Rights and Permissions


    Figures

  [Figure 1]



 

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
    Viewed1316    
    Printed10    
    Emailed0    
    PDF Downloaded47    
    Comments [Add]    

Recommend this journal