Breast masses include benign as well as malignant lesions and occur less frequently in male as compared to female. Malignant neoplasms account for 0.6 % of all breast carcinomas and less than 1% of all malignant lesions in men [1-2]. Papillary lesions are far more uncommon in men than women. The exact aetiology of male breast cancer is unknown. However, gynecomastia, imbalance in oestrogen-testosterone ratio, radiation exposure, obesity, BRCA2 mutations have all been implicated as risk factors [3-6]. It has been observed that intra-cystic papillary carcinoma (IPC) in particular may present more often in men with gynecomastia [7,8]. IPC is rare in male breast cancer, reported comprising 2.6-5% of male breast cancers [9,10]. Here, the present study reports a case of intra-cystic papillary carcinoma of the breast in a 37 years old male patient.

A 37-year-old male presented with a mass (lump) over the retro-areolar region of left breast which according to the patient was of small size initially but rapidly increased in size within 15-20 days. On examination, the mass was of variegated consistency i.e., soft to cystic at one place and hard in consistency at another. Also, it was non-tender and free from underlying structures with no nipple retraction. However, the bloody discharge was present (Figure 1).

Fig-1: Clinical appearance of the lesion.

Fig-2c: H and E (40x).

Fig-2d: H and E (40x).

Fig-2e: H and E (40x).

Fig-5: Immunohistochemistry: Her-2/neu negative.

Fig-6: Immunohistochemistry: P-63 non-specific.

Based on these findings, a final diagnosis of intra-cystic papillary carcinoma was made. The patient had received one cycle of chemotherapy but did not turn up for further follow-up.

Invasive Papillary Carcinoma of the breast is a rare tumour and constitutes fewer than 1% in most symptomatic series. Intra-cystic Papillary Carcinoma (IPC) is a variant of papillary carcinoma and accounts for 0.5-1% of breast cancers. The prevalence of male breast cancer increases with age [2]. Male patients are usually diagnosed in their 70s and 80s [6]. Approximately 15-20% of male patients with breast cancer have a positive family history [1].

IPCs do not appear to have a basal myoepithelial cell (MEC) layer, and MEC markers, such as p63, actin, and calponin can be useful in differentiating between IPCs from papilloma [15,16]. Hill et al. suggested a spectrum of progression from an in-situ disease to an invasive disease by using myoepithelial cell staining which is considered as “gold standard” method, as it has relatively high sensitivity and denotes the invasiveness of the tumour cells in malignant papillary breast lesions [7,9]. Lobular carcinoma is much less common in men than in women and it represents only 1% of all the cases [4]. The rarer subtypes such as the medullary, tubular, mucinous and the squamous carcinomas have all been reported in men. The tumours with high nuclear grades, histologic grades and large surface areas are more likely to metastasize to the lymph nodes or to recur locally [6,7]. The frequency of lymph node involvement, local recurrence and distant recurrence is 0% to 11%, 3% to 70% and 0% to 4% respectively [11-13].

The carcinomas of the male breast have higher rates of oestrogen and progesterone receptor positivity than are found in women, but similar percentages may be expressed for Her-2 and p53 in men and women [1]. Our case was Her-2 negative. The standard treatment of IPC should be based on the associated pathology [10]. There are no definite guidelines for the treatment of IPC, however, a radical surgical excision with a clear resection of margin is the important mainstay of the treatment.

Mastectomy is usually unessential unless it is required because IPC has an excellent prognosis, a low local recurrence, and a rare distant metastasis [9]. Long-term follow-up is necessary after the surgical treatment. Clinical assessment, however, could not be done in our case as the patient absconded after the first cycle of chemotherapy. There has been no clear indication for adjuvant endocrine therapy, even among the patients with oestrogen receptor-positive tumours [17]. Lefkowitz et al reported a 10-year disease-free survival rate of 91% [14].

To conclude, invasive papillary breast carcinoma of males is an uncommon disease with a peak incidence in the seventh decade of life, however, it is extremely rare in comparatively younger patients of the third to the fourth decade.