Fatal Metastatic Cutaneous Squamous Cell Carcinoma Evolving from a Localized Verrucous Epidermal Nevus
A 50-year-old Asian male presented with a partially pigmented 2-year-old lesion on the upper left side of his back. The lesion measured 3 cm in diameter, was round and had an irregular contour and surface. It had a verrucous pigmented rim on its medial side that measured 6–8 mm, while the prominent exophytic erythematous part measured 2.1 × 2.2 cm (fig. ). The exophytic part was almost round with an irregular surface. It was friable, bled easily, was though but not indurated and it was not attached to the underlying structures. The trunk also showed few post-pubertal lentigines, but no other nevi or seborrheic keratosis. The patient had no other co-morbidities and he was not immune-compromised. He had no personal or family history of skin or any other cancer. The lymph nodes were not enlarged at that time. An immediate total excision biopsy was done. The sutures were removed after 1 week with minimal scarring.\nThe pathological examination confirmed an epidermal nevus at the periphery of the lesion (fig. ). The epidermis showed bowenoid features with irregularly sized nuclei, a loss of polarity, dyskeratosis and mitotic figures (fig. ). Some sections from the periphery of the specimen showed multiple intraepidermal eddies of atypical cells or the Borst-Jadassohn phenomenon (fig. ). Sheets of poorly differentiated, atypical, pleomorphic keratinocytes migrated from the epidermis into the whole dermis, hair follicles (fig. ) and eccrine glands, including a perivascular and perineural invasion (fig. ). The tumour exhibited a maximal depth of 7 mm (fig. ).\nAtypical cells showed positive staining with cytokeratin markers and other squamous cell carcinoma markers, but they were negative for MART1 and S100. Focal, mild and sparse, subepidermal, lymphocytic infiltrates were found on some sections but were absent in many areas of the lesion. Thorough laboratory and radiological investigations were carried out. The results were: only mildly increased liver enzymes, an elevated C-reactive protein level and a mild impairment in the pulmonary function tests. The diagnosis was CSCC, stage T4N0M0.\nThe patient was immediately referred to the surgical department for the removal of sentinel lymph nodes. However, the surgeon decided not to excise any lymph nodes as long as the tumour could be totally removed in the absence of enlarged adjacent lymph nodes, demonstrated by clinical and ultrasound examination. The initial bone scan was negative for metastasis.\nSix weeks later (8 weeks after his first visit), the patient had a follow-up. Unfortunately, the regional lymph nodes were palpable, while the scar of the primary lesion and the surrounding area remained clear. The swelling was so extensive that it also infiltrated the surrounding subcutaneous tissue, measuring 12 × 17 cm (fig. ). An excision biopsy was taken from the lymph nodes and the surrounding subcutaneous tissues confirming the diagnosis of metastatic CSCC. The patient was again operated on for a wide removal of the axillary lymph nodes and adjacent tissues. After this surgical excision, the patient's general condition started to deteriorate. He was transferred to the Al Amal Cancer Hospital for chemotherapeutic and/or radiotherapeutic options; however; he was not eligible for either option and plans for intervention were postponed. The patient's general condition continued to decline with additional refractory anaemia, lymphocytopenia and hypoalbuminaemia.\nAfter another 4 weeks, a bone scan, CT and MRI images showed metastasis in both lungs (fig. ) and then 3 weeks later in the spinal cord (fig. ) and brain (fig. ), while the liver was spared. The patient received only palliative care during his entire stay, mainly to treat his anaemia and to control the pain. Eventually, he went into coma and then passed away.