Carcinoma en cuirasse

Carcinoma en cuirasse (CEC) is a rare, aggressive form of cutaneous cancer metastasis with poor prognosis.

Usually represents a tumor recurrence but occasionally it can present as an initial finding before diagnosis of a primary malignancy. 

Breast cancer is most common cancer to present with CEC

It is rarely seen in the setting of primary lung cancer, gastrointestinal cancers, and genitourinary malignancies. 

Most commonly occurs on the anterior chest wall but occasionally has been reported to involve the abdomen and rarer sites, including the scrotum. 

Clinically described as encasing the body in breastplate armor (en cuirasse). 

Initially presents as small papules and nodules that coalesce to form indurated, sclerotic plaques. 

It can progress to restrict movement of the chest wall and impede respiration. 

Pruritus, pain, bleeding, or a foul-smelling discharge may accompany cuirasse.

Treatment of CeC focuses on palliation with no consensus on therapy guidelines.

Treatment modalities that have been explored include chemotherapy, radiotherapy, with or without local hyperthermia, and hormonal antagonists. 

Breast cancer is not only the most common malignancy in women but is the primary tumor in about 70% of cutaneous metastases.

CeC comprises 3% to 6% of cutaneous metastases in breast cancer patients.

CeC presents clinically in two stages: 

The first is erythema and pitting edema of the skin, often confused with a benign dermatitis. 

Next is formation of thick leathery skin that hardens.

The histologic appearance is of a fibrotic stroma formed secondary to tumor deposition.

Fibrosis eventually overtakes the area, leaving few identifiable tumor cells, which are seen in an “Indian-filing” pattern with cells lined up in single rows within collagen bundles of the dermis.

Differential diagnosis: radiation-induced morphea, inflammatory breast cancer, radiation dermatitis, and other cutaneous metastases. 

CeC is not associated with inflammatory changes.

No consensus on treatment for CeC due to the small number of cases, but modalities used include chemotherapy, radiotherapy, hyperthermia, and hormonal antagonists.

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