Clinicopathological diagnosis of morphea-like carcinoma en cuirasse in the neck: a rare presentation of lung cancer

Abstract

Background

Carcinoma en cuirasse is mostly reported in breast cancer. It rarely originates from other visceral tumors such as lung cancer. In this report, we highlight the importance of skin biopsy not to make a misdiagnosis or missed diagnosis.

Case presentation

We report a 51-year-old male, diagnosed with lung adenocarcinoma 2 years ago, presenting as swelling and hardening of the face and neck. The patient was diagnosed with carcinoma en cuirasse from lung cancer and was transferred to the oncology department for further management. Unfortunately, the patient gave up treatment after 3 months and died after 1 year of follow-up.

Conclusion

In patients with tumors that present as swelling and hardening of the skin, the possibility of skin metastases should be considered, and the necessity of early skin biopsy should be taken into account.

Background

Carcinoma en cuirasse is a rare type of cutaneous metastatic carcinoma, with the breast being the most common involved site. It’s characterized by diffuse thickening and hardening of the skin, Anatomist Alfred Velpeau used the term cuirasse, because he thought that the clinical presentation resembled a medieval breastplate or a cuirass. Carcinoma en cuirasse rarely originates from other visceral tumors such as lung cancer [1]. Here we report a rare case of morphea-like cutaneous en cuirasse that originated from lung cancer and occurred in the neck. We underscore the necessity to contemplate the potential for skin metastases in patients presenting with tumors accompanied by skin swelling and hardening.

Case presentation

A 51-year-old male presented with swelling and hardening of the face and neck with dysphagia for six months. The skin on the neck was hard in texture, similar to leather, and had some patches of pale red color on the surface. The patient was unable to lift or turn the neck (Fig. 1).

He was diagnosed with lung adenocarcinoma (stage III adenocarcinomaof the right lung) at local hospital 2 years ago, and underwent surgical treatment, followed by chemotherapy and targeted combination therapy (bevacizumab + petremex + carboplatin). Initial Consideration: Systemic sclerosis? Auxiliary test: ANA > 400.00RU/ml↑, Ro-52 > 400.00RU/ml↑. Cervical skin ultrasound revealed thickening and non-uniform echo of the subcutaneous soft tissue in the front neck region. The structure appeared disordered, with some areas showing “fissure-like” changes. The fascia in each muscle space were also thickened and displayed non-uniform echo enhancement (Fig. 2). Both CT and MRI scans reveal swelling in the subcutaneous soft tissues of the neck and maxillofacial region. Bilateral neck muscles exhibit patchy areas of enhancement with indistinct margins. Additionally, the intermuscular septa in the affected areas are thickened and show enhancement, which suggests a potential diagnosis of either infection or tumor. Histopathological analysis of the neck skin revealed a widening gap between the collagen fibers in the dermis, along with the presence of diffuse mucin deposition. Additionally, abnormal cells with atypical morphology were observed infiltrating the dermis. Immunohistochemical: CK(+),CK7(+),TTF-1(+),Napsin A(+),Ki-67(40%+). (Figure3).

The patient was diagnosed with carcinoma en cuirasse from lung cancer and was transferred to the oncology department for further management. Unfortunately, due to economic reasons, the patient gave up treatment after 3 months and died after 1 year of follow-up.

Fig. 1

Diffuse thickening and hardening of skin on face and neck

Fig. 2

Skin ultrasound revealed thickening and non-uniform echo of the subcutaneous soft tissue in the front neck region. The structure appeared disordered, with some areas showing “fissure-like” changes. The fascia in each muscle space were also thickened and displayed non-uniform echo enhancement

Fig. 3

Histopathology: A widening gap between the collagen fibers in the dermis, along with the presence of diffuse mucin deposition. Additionally, abnormal cells with atypical morphology were observed infiltrating the dermis (A HE× 100, B HE× 200). Immunohistochemical: CK (+) (C), CK7(+) (D), TTF-1(+) (E), Napsin A (+) (F), Ki-67(40%+) (G)

Discussion

Cutaneous metastatic cancer is a disease that occurs when the primary malignant tumor outside the skin metastasizes through blood vessels, lymphatic vessels, or spreads directly to the adjacent skin through the tissue space, sometimes secondary to surgical implantation [1]. The prevalence of cutaneous metastatic carcinoma originating from lung cancer varies between 1% and 12% [2, 3], positioning lung cancer as the foremost primary malignancy linked to skin metastases in males.

Cutaneous metastases of malignant tumours feature three main forms: Nodular, sclerodermoid (carcinoma en cuirasse), and inflammatory cancer [1]. The nodular type is primarily characterized by erythematous, infiltrating papules and nodules, either solitary or multiple, which is the most common presentation of cutaneous metastatic carcinoma. The inflammatory response type, on the other hand, is characterized by localized flushing and pronounced edema, closely mimicking the appearance of erysipelas, and is likewise a commonly encountered presentation. Sclerotic cutaneous metastasis carcinoma, also known as carcinoma en cuirasse, are unusual and have been reported in a very limited number of cases since Velpeau first described them in 1838. Cuirasse is a French word, derived from the Latin “leather” (meaning “leathery”), used to describe the breastplates of ancient warriors. Carcinoma en cuirasse is characterized by diffuse thickening and hardening of the skin, hence the name [4]. It can also masquerade postirradiation morphea, inflammatory breast cancer, radiation dermatitis, and other cutaneous metastases.

Skin metastases tend to occur near the primary cancer site, and the most common sites include the chest, back, neck, abdomen, vulva, and scrotum [5]. This uncommon type of skin metastasis is mostly observed in breast cancer cases, less frequently, in primary lung, gastrointestinal, or genitourinary cancers, It is also observed that patients eventually develop metastatic disease following surgery and/or chemotherapy, radiotherapy, hormonal therapy [4, 6,7,8,9,10,11]. Carcinoma en cuirasse comprises 3–6% of cutaneous metastases in breast cancer patients. Our patient developed neck skin metastasis from lung cancer, which is extremely rare in clinical practice.

Pathogenesis of carcinoma en cuirasse is unknown, though researchers hypothesize it is due to the activation of stromal fibroblasts by an extracellular signaling molecule known as pleiotrophin. Certain investigators posit that its development could be linked to genetic mutations, aberrations in the immune system, and the influence of various growth factors. Pleiotropic growth factors, which are a group of extracellular signaling molecules pivotal in orchestrating cell proliferation and differentiation, exhibit elevated expression levels within the skin lesions characteristic of carcinoma en cuirasse. It is conjectured that these factors might play a role in the expedited tumor expansion and the pronounced metastatic potential associated with this malignancy [12].

Histologically, the tissue shows characteristics similar to fibrosis, with only a few tumor cells arranged in an ‘Indian file’ pattern. In this pattern, the tumor cells form small lines between the collagen bundles. The similarity of tumor cells to fibroblasts often leads to misdiagnosis or oversight, emphasizing the importance of combining immunohistochemistry for accurate diagnosis. The majority of previously reported cases were confirmed to be of lung origin through immunohistochemistry. However, for some patients, immunohistochemical testing was not performed, and diagnosis was based on medical history and histopathological findings [1]. Ideally, if conditions allow, immunohistochemistry is recommended to accurately determine the tumor’s origin. The dense fibrotic matrix and reduced blood vessels make it difficult for systemic chemotherapy drugs to reach effective concentrations, leading to high resistance to chemotherapy [13, 14]. So far, there are few reports about carcinoma en cuirasse, A vigilant approach is essential to diagnosis of carcinoma en cuirasse relies on clinical and histopathologic features to distinguish it from other entities described herein.

Cutaneous metastases indicate advanced disease progression and often suggest a poor prognosis [15]. The median survival time for lung cancer with cutaneous metastases is only 3 months [16, 17].

Conclusion

For clinicians, it is imperative to entertain the notion of cutaneous metastatic carcinoma in tumor patients presenting with skin swelling and sclerosis, irrespective of the primary site’s proximity. This underscores the necessity of initiating early skin biopsies to preclude missed or erroneous diagnoses, thereby expediting appropriate clinical interventions.