Multiple cutaneous metastasis of sacral chordoma, mimicking neurofibromas clinically

Neha Chopra Narang,Preeti Diwaker,Salil Narang

doi:10.21276/apalm.1753

Abstract

Chordoma is a rare primary bone tumor of notochordal origin, constituting 1-4% of malignant primary bone tumors. Chordomas are slow growing tumors and show aggressive local extension, multiple recurrences and metastasis. Cutaneous metastasis of chordoma is very rare. We report a case of a 61-year-old man presenting with multiple cutaneous metastasis of primary sacral chordoma.DOI:10.21276/APALM.1753

Highlights

Chordomas are rare but aggressive tumors due to local recurrence and distant metastasis
Microscopic examination showed a lobulated moderately cellular tumor having cords of epithelioid tumor cells in a myxoid background reported as suggestive of chordoma.The patient was asymptomatic till December 2013
The incidence of distant metastasis varies between 3%-50%.[3]

Summary

Introduction

Chordomas are rare but aggressive tumors due to local recurrence and distant metastasis. They most commonly arise from notochordal remnants found throughout the axial skeleton. The common sites of metastasis include lungs, liver, bone, lymph nodes and rarely skin.[1]Cutaneous involvement is usually seen because of direct extension from primary tumor or local recurrence. Microscopic examination showed a lobulated moderately cellular tumor having cords of epithelioid tumor cells in a myxoid background reported as suggestive of chordoma.The patient was asymptomatic till December 2013. Histopathological findings: Gross examination showed three skin covered lesions measuring 3 x 2 x 1 cm, 4 x 2 x 1.5 cm and 7 x 5 x 3 cm from lip, scalp and forehead respectively.

Discussion

Metastatic tumors