Intracystic papillary carcinoma associated with ductal carcinoma in situ in a male breastDM Dragoumis, AP Tsiftsoglou
Breast Division, Department of General Surgery, St Luke's Hospital, Panorama, 55 236 Thessaloniki, Greece
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0022-3859.39191
Source of Support: None, Conflict of Interest: None
Intracystic papillary carcinoma (IPC) represents a small distinctive subgroup of noninvasive breast cancer, accounts for <0.5% of breast malignancies and is extremely rare in men. Careful selection of operative procedure is needed after IPC associated with ductal carcinoma in situ (DCIS) around the main tumor is diagnosed.
A 75-year-old man became aware of a lump, located just below his left subareolar region. He had first noticed this mass 2 months ago and since then it slowly grew and hindered his daily activity. On physical examination, the patient had a palpable, mobile, and soft mass. It was nontender, approximately 3 cm in diameter and was detected below his left breast nipple. The lesion seemed to have clear borders and a flat surface, mimicking a breast cyst. A puncture aspiration provided 25 mL of bloody fluid content. Fine-needle aspiration cytology from the breast cystic lesion raised the suspicion of a papillary carcinoma, but yielded no definite diagnosis.
Considering the patient's age, the bloody feature of the fluid and the presence of residual mass after inspiration, we decided to perform an excisional biopsy. The histological essay revealed a cystic lesion with papillary structures lined by layers of epithelial malignant cells and surrounded by a thick fibrous wall. Large cribriform cells, with irregular nuclei and high-mitotic index were observed within the cyst wall. Similar atypical cells, arranged in a solid and sometimes cribriform pattern, were also present within the ducts. The confirmed final diagnosis was IPC with associated DCIS [Figure - 1],[Figure - 2].
One month later, the patient underwent a segmental resection of the left breast, including axillary lymph node dissection, as the surgical margin after initial excisional biopsy was positive for carcinoma. The additional microscopic pattern consisted of ductal epithelial hyperplasia with foci of atypia and progression to ductal carcinoma in one area of the specimen. There were no metastatic deposits within the axillary lymph nodes. We did not consider that adjuvant treatment was necessary in the presence of adequate local control and in the absence of metastatic spread of disease.
Approximately half of IPCs arise in the retroareolar region of the breast and the usual clinical manifestation is a palpable mass or nipple discharge. The IPC tends to be well defined on mammography, while an irregular margin suggests the presence of invasion. Solid or complex cystic masses are often seen on sonography, with mural nodularity and papillary projections. 
FNA cannot always rule out between benign and malignant papillary breast tumors. It is strongly postulated that the difficulty in obtaining a definite diagnosis of malignancy by FNA can be attributed to the cystic and hemorrhagic nature of these lesions. Some studies have suggested that core needle biopsy (CNB) has been proved to be more effective in distinguishing papillary neoplasms from other diseases and benign papillomas from papillary carcinoma. 
The IPC is divided to three subtypes: (i) pure IPC, (ii) IPC with associated DCIS, and (iii) IPC with associated invasive cancer. The majority of patients with IPC will have associated DCIS or invasive cancer or both, and should be treated on the basis of this associated pathology. 
The nature of the associated lesions to IPC is essential for prognostic reasons and for assessment of the margins. Moreover, IPC accompanied by DCIS is an important precursor to invasive carcinoma and further treatment is indicated, if medically feasible. 
Nowadays, there is still no clear consensus regarding optimal treatment of IPC. Most papers reinforce the importance of an adequate surgical margin in conservative treatments. It is true that low frequency of axillary node metastases with pure IPC does not justify axillary lymph node dissection. Although, the role of sentinel node biopsy has not been evaluated in this disease, it seems that sentinel node biopsy may be an excellent alternative to full axillary dissection in patients with IPC and associated DCIS or invasive carcinoma. ,
As regards our patient, IPC was associated with intraductal spread of high-grade DCIS, in the periphery of the main tumor. It was necessary to perform a more radical surgical therapy for the complete resection of IPC in the present case.
The patient eventually underwent segmental excision of the left breast with axillary lymph node dissection and he remains well 48 months later, without tumor recurrence.
The authors express their gratitude to Dr. Anthoula Assimaki, Pathology Division, St Luke's Hospital, for the photomicrographs.
[Figure - 1], [Figure - 2]