Carcinoma en cuirasse of breast presenting as keloidal structures

Abstract

Carcinoma en cuirasse, also known as scirrhous carcinoma, is a form of cutaneous metastasis of breast carcinoma. The incidence of “en cuirasse” is approximately 0.6%–10% of cases. The most common sites for cutaneous metastases are the chest wall and abdomen. It is characterized by an erythematous, indurated plaque, nodules, or morphea-like plaques over the chest wall. We report a rare case of carcinoma en cuirasse of breast presenting as keloidal-like nodules in a 55-year-old woman.

Introduction

Carcinoma en cuirasse is a form of cutaneous metastasis, most commonly associated with the breast carcinoma after mastectomy. Less frequently, this is diagnosed at the same time as the primary tumor or can be present as the first manifestation of the disease.^[1]

Case Report

A 55-year-old woman with no remarkable medical history presented with asymptomatic skin-colored nodules, thick fibrotic plaques of varying sizes on left anterior chest wall, breast, and underneath the breast in a linear pattern of 3 months duration [Figure 1]. There was no palpable mass in the breast and no regional lymphadenopathy. The findings of the systemic examination were unremarkable. All hematological, biochemical, and serological tests were normal. Chest-radiograph, ultrasound abdomen, and electrocardiogram were normal. Multiple biopsies from four different sites uniformly showed histopathological features of a highly cellular infiltrative tumor in the dermis arranged in Indian file pattern, solid nests, alveolar pattern, and clusters. The tumor cells were round to oval having a fair amount of clear, vacuolated, mucinous cytoplasm showing moderately pleomorphic vesicular nuclei with a high N:C ratio. Mitoses were seen (9/10hpf). Stroma showed dense fibrosis and lymphocytic infiltration. No pagetoid spread into the epidermis was seen [Figure 2A and B]. Immunohistochemistry (IHC) revealed that the tumor cells were ER positive [Figure 3], PR and HER2 negative. Based on histopathology and IHC studies, a diagnosis of carcinoma en cuirasse from invasive lobular breast carcinoma was established. The case was referred to regional cancer institute for further management.

Figure 1:: Erythematous nodules, plaques on left anterior chest wall, breast and underneath the breast

Figure 2:: (A) (HandE 100×) Tumor cells arranged in Indian file pattern in dense fibrous stroma infiltrating into dermis. (B) (HandE 400×) Tumor cells showing cytoplasmic mucin arranged in Indian file and alveolar pattern

Figure 3:: (400×) ER positive tumor cells

Discussion

Carcinoma of the breast is the most common cancer in women and a frequent one to involve the skin. According to GLOBOCAN2018 database, the incidence, mortality, and 5-year prevalence of carcinoma breast in India are as follows: new cases 3048, deaths 2053, and prevalence 733.^[2] The incidence of cutaneous manifestation of breast carcinoma is 23.9%.^[1] In one study 6.3% of patients with breast cancer had skin involvement at the time of diagnosis; 3.5% had this as the presenting clinical manifestation.^[3] The metastases from breast cancer to the skin have varied clinical manifestations and many clinical variants are reported in the literature.^[1,4] Nodules are the most common presentation. The incidence of “en cuirasse” is approximately 0.6%–10% of cases. Carcinoma en cuirasse is characterized by an erythematous, indurated plaque, nodules, or scleromatoid-like plaques over the chest wall. Two cases of carcinoma-en-cuirasse like ours, mimicking as keloidal like nodules on the chest wall have been reported.^[5,6] The most common sites for cutaneous metastases are the chest wall and abdomen, but they can occur at the extremities and in the head/neck region. These skin metastases can rarely occur with other malignancies such as lung, gastrointestinal tract, and kidney. Histopathological findings of en cuirasse include fibrosis with a few neoplastic cells, sometimes showing a characteristic Indian file pattern between the collagen bundles of the dermis. This clinical subtype is mostly seen in cutaneous metastasis from lobular carcinoma (ILC) of the breast. Invasive lobular carcinoma originates from the lobules within the mammary glands and is the second most prevalent type of invasive breast carcinoma, the most common being invasive ductal carcinoma (IDC). Histologically, both show features consistent with the “Indian file” pattern, stromal invasion of neoplastic cells within a liner arrangement. These two types of breast carcinomas are distinguished by Immunohistochemistry: E-cadherin is usually expressed in a large percentage of IDC cases, but is rarely expressed as ILCs.^[7] Carcinoma breast as carcinoma en cuirasse presenting as keloidal-like nodules and plaques without lump in the breast is unusual and a rare presentation. Such cases should not be regarded as keloids and thoroughly investigated to avoid delay in the diagnosis of malignancy and proper treatment.

The prognosis of patients with cutaneous metastasis depends upon the type and biological behavior of the underlying primary tumor. Breast carcinoma with skin metastasis is usually associated with a poor prognosis.

Financial support and sponsorship

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Conflicts of interest

There are no conflicts of interest.

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.