Incidentally discovered distant cutaneous metastasis of sacral chordoma: a case with variation in S100 protein expression (compared to the primary tumor) and review of the literature

Abstract

Chordomas represent rare malignant primary bone tumors most often occurring in the sacral area. These tumors uncommonly involve the skin and often follow a progressive course with multiple recurrences, metastases and eventual death. Reports of cutaneous metastases from chordoma are very rare. The immunohistochemical staining characteristics of these cutaneous metastases with comparison to the primary tumors are similarly rarely addressed in the literature. We report a rare case of incidentally discovered, small, solitary distant cutaneous metastasis of sacral chordoma that developed on the right upper back of a 44-year-old man with a history of multiple completely excised melanomas who had also been previously diagnosed with chordoma involving the sacrum 12 years earlier. We describe its pathologic features with comparison to the primary tumor and briefly review the literature. Immunohistochemically, the cutaneous metastasis and primary tumor both stained positively for pancytokeratin and vimentin, as expected. However, the cutaneous metastasis unexpectedly lacked S100 protein expression, whereas the primary tumor was S100 positive. This phenomenon has only been documented in one other case report. We demonstrate that late, incidentally discovered cutaneous metastasis with unexpected immunohistochemical staining features rarely occur and can present a diagnostic challenge.

Site-Specific Analysis of Inflammatory Markers in Discoid Lupus Erythematosus Skin

“…Primary anti-human antibodies against CD3 (Cell Marque Corporation, Hot Springs, AR), CD4 (Biocare Medical, Concord, CA), CD8 (Cell Marque), CD20 (Ventana Medical Systems, Tucson, AZ), CD68 (Ventana), CXCL10 (R&D Systems, Minneapolis, MN), CXCR3 (BD Pharmingen, San Jose, CA), and TIA-1 (Biocare) were applied. Immunostaining using avidin-biotin-peroxidase complex and 3,3′-diaminobenzidine as substrate on an automated Ventana Benchmark instrument (Ventana) was performed using previously published protocols [26] with appropriately staining negative and positive control samples. Slides were counterstained with hematoxylin and bluing reagent.…”

Multiple cutaneous metastasis of sacral chordoma, mimicking neurofibromas clinically

“…[4] Face or back has been found to be the most common cutaneous metastatic site, though scalp, trunk and extremities have also been reportedly involved. [5,6] Diagnostic difficulties of metastatic chordoma are mainly due to histologic similarities with other dermal chondroid lesions. Differential diagnosis includes both primary as well as metastatic lesions having abundant mucinous/myxoid component.…”

“…[8,9] However occasional reports have stated loss of S-100 expression in metastatic lesions . [5,6] Brachyury has emerged as a novel biomarker to differentiate chordomas from other chondroid lesions with high sensitivity and specificity when combined with cytokeratins, 98% and 100% respectively. Brachyury is a transcription factor that regulates mesoderm and notochord formation in humans .…”

On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma

“…In up-to-date medical literature about 20 cases of cutaneous metastasis from sacrococcygeal chordoma were described [21]. The last one was illustrated in last year [22].…”