|Year : 2018 | Volume
| Issue : 6 | Page : 245-248
Occult medullary breast carcinoma in a male
Devesh Sanjeev Ballal, Gabriel Rodrigues
Department of General Surgery, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India
|Date of Submission||03-May-2018|
|Date of Decision||10-Jun-2018|
|Date of Acceptance||01-Aug-2018|
|Date of Web Publication||11-Dec-2018|
Prof. Gabriel Rodrigues
Department of General Surgery, Kasturba Medical College, Manipal Academy of Higher Education, Manipal - 576 104, Karnataka
Source of Support: None, Conflict of Interest: None
Occult breast cancer (OBC) or breast cancer presenting without an evident primary tumor is a rare presentation and can lead to a diagnostic dilemma. Further, the treatment of OBC is a subject of much controversy. We present a 47-year-old male who had been admitted with a fungating lesion in his axilla. As clinical examination was unremarkable, a punch biopsy from the lesion revealed a medullary carcinoma of breast. The purpose of this report is to highlight this unusual presentation of carcinoma breast and the treatment dilemma that it poses, especially in men, where breast cancer is seldom considered a diagnostic possibility in the setting of occult primary cancer. This is the first-ever case report of medullary carcinoma presenting as OBC in a male.
Keywords: Axillary lymphadenopathy, chemotherapy, male breast cancer, medullary carcinoma breast, occult breast cancer
|How to cite this article:|
Ballal DS, Rodrigues G. Occult medullary breast carcinoma in a male. Formos J Surg 2018;51:245-8
| Introduction|| |
Breast cancer presenting as isolated axillary nodal metastasis is very rare, with a reported incidence of 0.3%–1% among breast cancer patients.,, With the advent of more sensitive means of detecting primary tumors, such as breast magnetic resonance imaging (MRI) and high-quality mammography, the incidence of occult breast cancer (OBC) is decreasing. In a female presenting with malignant axillary lymphadenopathy, the breast is usually the most common site of primary malignancy, and this has led to many patients presenting with isolated axillary metastasis being treated with mastectomies with axillary node dissections., However, there are some clinicians who advocate breast irradiation with axillary dissection and adjuvant systemic therapy if no primary tumor can be detected on MRI. Breast cancer of male (BCM) is very rare, accounting for around 1% of the cases of breast cancer. This makes it a very unusual diagnosis is the setting of malignancy with unknown primary. There is little evidence to guide treatment in BCM, and there have been no randomized controlled trials comparing treatment options, with most recommended treatments stemming for low-level evidence. Therefore, evidence-based treatment for OBC in males, is a hard to come by.
| Case Report|| |
A 47-year-old male with no comorbid illness presented with a nonhealing ulcer of the left axilla of 4 months duration, which actually started as an asymptomatic swelling, gradually increased in size and then ulcerated discharging pus. There was no symptom of breast or metastatic disease, constitutional symptoms, or any other swellings over the body. On examination, there was a foul smelling ulceroproliferative growth in the left axilla discharging pus and enlarged lymph nodes. It was nontender but with restricted mobility [Figure 1]. Examination of the left breast revealed no palpable lump, absent left supraclavicular lymph node, and normal left upper limb. The contralateral breast, axilla, skull, and spine were normal. Systemic examination of the chest, abdomen, lymphoreticular system revealed no abnormality. Hence, a punch biopsy of the lesion was done, which showed a tumor composed of cells in syncytial sheets, marked mononuclear infiltrate, pleomorphic nuclei, circumscribed edge, and absence of glandular differentiation that was suggestive of medullary carcinoma of the breast, in a background of lymph nodal tissue [Figure 2]. The tumor was estrogen-progesterone receptor (ER-PR) positive but HER2/neu negative. The patient was then subjected to a MRI of chest and bilateral axilla, to look for any primary in the breast. The MRI did not show any lesion in the breast or chest wall but showed a fungating left axillary lymph nodal mass, other enlarged left axillary lymph nodes with areas of necrosis. Ultrasonography of the abdomen and the bone scan was also done to rule out systemic metastases and all were found to be normal.
|Figure 2: Photomicrograph showing a tumor composed of cells in syncytial sheets, marked mononuclear infiltrate, pleomorphic nuclei, circumscribed edge, and absence of glandular differentiation in a background of lymph nodal tissue (H and E, ×100)|
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He received three cycles of induction (neoadjuvant) chemotherapy: Taxotere, adriamycin, and cyclophosphamide (TAC) regimen with docetaxel (taxotere) 75 mg/m2 intravenous (IV0 on day 1 plus doxorubicin (adriamycin) 50 mg/m2 IV on day 1 plus Cyclophosphamide 500 mg/m2 IV on day 1 every 3 weeks after which the lesion was amenable for surgery and underwent a left modified radical mastectomy, the final histopathology report of which revealed no tumor tissue in the breast but malignant axillary lymph nodal metastasis from a medullary carcinoma of the breast. Postoperatively, he received three more cycles of adjuvant TAF chemotherapy. Given the tumor characteristics, he also received radiation to the operated area (60 Gy over 5 weeks). As the tumor was ER-PR positive, he has been on Tamoxifen 10 mg once daily. He has been kept under close follow-up and is disease free for the last 3 years.
| Discussion|| |
While only 0.5%–1% of all BCs occur in men, the incidence appears to be rising and men are as likely to develop BC as chronic myeloid leukemia. The most common subtype, like in women, is infiltrating ductal carcinoma (92%), with colloid and papillary accounting for 2% each. Medullary carcinoma is rare, accounting for 0.2% of all the BCM. The current treatment recommendations for BCM have been largely extrapolated from studies on its counterpart in women or based on data from small case series.,, Although there are a very few reported cases of OBC occurring in males, there seems to be no prior reported case of medullary carcinoma of the breast presenting as an OBC. In all the previous cases, treatment was based on the principles described for OBC in women, with three patients undergoing mastectomies with axillary dissection and one patient undergoing a mastectomy without axillary dissection.,,
OBC is a rather rare presentation of breast cancer, the pathogenesis of which is poorly understood and the first case of OBC was reported by Halstead as early as in 1907. Recent studies suggest that the genesis of OBCs is due to metastasis from subclinical primary tumors in which angiogenesis was switched on in the axilla but not in the breast. However, this differs from earlier concepts, wherein a number of possible explanations were given for OBC: the focus of invasive cancer is missed in the breast, invasion not detected by light microscopy, metastasis from contralateral breast, carcinoma from ectopic breast tissue that may be present in a lymph node, the presence of invasive carcinoma in the axillary fat that was not examined, and misinterpretation of heterotopic glands in a lymph node as carcinoma.,,
A certain percentage of cases that would have previously been thought to be occult have been found to have a primary tumor after MRI or high-quality mammography, leading to the suggestion that MRI be instituted in the diagnostic workup of OBC. Breast MRI has been estimated to detect a primary in approximately 80% of mammogram-negative patients and would enable more patients to be treated with breast conservation therapy. However, the benefits of picking up more patients to receive less morbid treatment options because of good sensitivity need to be balanced with the unnecessary procedures it leads to due to relatively low specificity.,,
There is a therapeutic dilemma while managing patients with OBC even after extensive imaging. While there is a consensus that the involved axilla needs to be addressed with a formal axillary dissection, whether or whether not to perform a mastectomy on the ipsilateral breast remains a dilemma. However, a few studies suggest that a mastectomy may be omitted, and the breast can be addressed with radiation. A recent review reported that up to 72% of mastectomy specimens for OBCs contained an occult malignancy on pathological examination, with the authors advocating routine mastectomies in OBCs to maximize cytoreduction and preclude the need of frequent breast surveillance. Axillary dissection is not only therapeutic in these patients but also gives valuable prognostic information. The number of involved lymph nodes is a direct indicator of 5-year survival according to studies, with survival of more than 90% if less than four nodes are involved.
The use of adjuvant systemic therapy has been an integral part of the treatment of OBC, but its role has been called into question. A recent meta-analysis showed that no type of chemotherapy conferred a definitive survival benefit in patients with OBC. In addition, the advent of targeted therapy in the form of HER2 blockers and endocrine ablation has provided a far less morbid alternative to conventional chemotherapy.
In the attempt to standardize treatment protocols, it is important to remember that OBC may represent a far more heterogeneous entity than previously thought, with studies showing that OBC with isolated axillary metastasis is a vastly different entity from OBC with visceral metastasis, with the former behaving more like AJCC stage II (node positive) disease, while the latter harbors diverse genetic aberrations making it behave very differently, necessitating different treatment strategies. This makes it particularly suited for personalized breast cancer treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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