Cutaneous Metastasis From Sacral Chordoma

Abstract

Chordoma is a rare primary bone malignancy of notochord origin, representing 1-4% of malignant bone tumors., Typically, chordomas follow a slow progressive course with aggressive local extension, multiple recurrences, and metastases. Of particular interest to this case, cutaneous metastasis is exceedingly rare. Diagnosis of this entity can be a challenge due to the rarity of chordoma, as well as the infrequent presentation of distant cutaneous metastasis and non-specific clinical skin findings. We report a case of a 61-year-old male with a history of sacral chordoma treated by wide local excision 8 years prior to presentation developed a nodule on his scalp for 6 weeks. Physical examination revealed a 1 cm rubbery, pink, shiny dome-shaped nodule on his left occipital scalp. Hematoxylin and eosin sections revealed a lobular dermal proliferation of small ovoid cells and larger physaliferous cells with hyperchromatic, displaced nuclei and finely vacuolated "soap-bubble" cytoplasm in a myxoid stroma. Immunohistochemistry of tumor cells showed positivity for both S-100 protein and pancytokeratin (AE1/AE3), while smooth muscle actin (SMA), P63, and CK7 were negative. Additionally, tumor cells stained positive for brachyury. The medical history, clinical presentation, histopathological appearance and immunohistochemical profile are consistent with cutaneous metastasis from sacral chordoma, known as chordoma cutis. This case illustrates the integral role of dermatopathology in the diagnosis of a rare and critical condition.

Alopecia neoplastica as a sign of visceral malignancies: a systematic review

“…Median time between primary tumour and AN diagnosis was 10 months (ranging from 0 to 240 months; IQR: 0–36). Finally, AN was the first metastatic event in 65.3% (79/121) of cases, specifically in 39 women vs. 40 men …”

“…Alopecia neoplastica was diagnosed after the diagnosis of the primary visceral tumour in 72.7% (88/121) cases and only in 2.5% (3/121) cases before the diagnosis of the visceral tumour, while in 24.8% (30/121) cases, the diagnosis was synchronous …”

“…Women were more represented with the 53.7% (66/123) of cases, compared to the 46.3% (57/123) of men . Median age of AN at onset was 59 (IQR: 47–68).…”

“…Regarding the primary site of malignancy, gastrointestinal (GI) tract accounted for 24.4% (30/123), breast for 17.9% (22/123) of cases, kidney for 8.1% (10/123), lung for 7.3% (9/123), thyroid for 7.3% (9/123), uterus for 6.5% (8/123), central nervous system (CNS) for 6.5% (8/123), liver for 3.3% (4/123) and other anatomic areas for 18.7% (23/123) of cases …”

“…Concerning histologic subtype of the primary tumour, carcinoma was the most reported with 53.7% (66/123) cases; of these, 38.2% (47/123) were adenocarcinomas, 7.3% (9/123) were squamous cell carcinomas and 0.8% (1/123) was a small cell carcinoma; while, in 7.3% (9/123) of cases, the type of carcinoma was not further specified . Regarding the remaining cases, 3.3% (4/123) primary tumours were leyomyosarcomas, 1.6% (2/123) were meningiomas, 40.7% (50/123) were other tumour subtypes and in one case of breast cancer, the histologic subtype was unknown (Table ) …”