Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report

Introduction: Conventional chordoma is a malignant osseous neoplasm that usually arises from the axial skeleton. It recapitulates a phenotype of embryonic notochord, usually occurring in the 5th–7th decades of life. Very rarely, chordomas can arise in the extra-axial skeleton, more so in the extra-a...

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Main Authors: P Sakthidasan Chinnathambi, G V R N Krishna Kanth, Anitha Burra, Neha Singh
Format: Article
Language:English
Published: Indian Orthopaedic Research Group 2025-04-01
Series:Journal of Orthopaedic Case Reports
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Online Access:https://jocr.co.in/wp/2025/04/01/extra-axial-soft-tissue-chordoma-of-hand-a-rare-case-report/
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author P Sakthidasan Chinnathambi
G V R N Krishna Kanth
Anitha Burra
Neha Singh
author_facet P Sakthidasan Chinnathambi
G V R N Krishna Kanth
Anitha Burra
Neha Singh
author_sort P Sakthidasan Chinnathambi
collection DOAJ
description Introduction: Conventional chordoma is a malignant osseous neoplasm that usually arises from the axial skeleton. It recapitulates a phenotype of embryonic notochord, usually occurring in the 5th–7th decades of life. Very rarely, chordomas can arise in the extra-axial skeleton, more so in the extra-axial soft tissues. We present one such case of Extra-axial soft tissue chordoma in the dorsum of the hand in an adult patient, with a short review of the literature. Case Report: A 48-year-old male patient presented with a longstanding swelling on the dorsum of the left hand, persisting for 5 years. Clinico-radiological assessment suggested differential diagnoses of a ganglion cyst or a chondroid lesion. A fine needle aspiration was performed, which was documented as a giant cell-rich soft tissue neoplasm. Subsequent excision biopsy showed histopathological features characterized by numerous multivacuolated, bubbly cytoplasmic physaliferous cells within a myxohyaline matrix, indicative of a neoplastic process. Immunohistochemistry revealed positivity for brachyury, which confirmed the diagnosis of an Extra-axial soft tissue chordoma. Conclusion: Extra-axial soft tissue chordomas are extremely rare malignant osseous neoplasms that can mimic a variety of other lesions, need a strong clinical suspicion, and keen morphological cum immunohistochemical examination for diagnosis. Given their similar imaging characteristics and overlapping histological features, making an accurate diagnosis is crucial, as treatment plans vary significantly.
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spelling doaj-art-8b35a37ca42d4091a15d97eca6b0cad62025-08-20T03:21:47ZengIndian Orthopaedic Research GroupJournal of Orthopaedic Case Reports2250-06852321-38172025-04-0115412613110.13107/jocr.2025.v15.i04.5470Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case ReportP Sakthidasan ChinnathambiG V R N Krishna KanthAnitha BurraNeha SinghIntroduction: Conventional chordoma is a malignant osseous neoplasm that usually arises from the axial skeleton. It recapitulates a phenotype of embryonic notochord, usually occurring in the 5th–7th decades of life. Very rarely, chordomas can arise in the extra-axial skeleton, more so in the extra-axial soft tissues. We present one such case of Extra-axial soft tissue chordoma in the dorsum of the hand in an adult patient, with a short review of the literature. Case Report: A 48-year-old male patient presented with a longstanding swelling on the dorsum of the left hand, persisting for 5 years. Clinico-radiological assessment suggested differential diagnoses of a ganglion cyst or a chondroid lesion. A fine needle aspiration was performed, which was documented as a giant cell-rich soft tissue neoplasm. Subsequent excision biopsy showed histopathological features characterized by numerous multivacuolated, bubbly cytoplasmic physaliferous cells within a myxohyaline matrix, indicative of a neoplastic process. Immunohistochemistry revealed positivity for brachyury, which confirmed the diagnosis of an Extra-axial soft tissue chordoma. Conclusion: Extra-axial soft tissue chordomas are extremely rare malignant osseous neoplasms that can mimic a variety of other lesions, need a strong clinical suspicion, and keen morphological cum immunohistochemical examination for diagnosis. Given their similar imaging characteristics and overlapping histological features, making an accurate diagnosis is crucial, as treatment plans vary significantly.https://jocr.co.in/wp/2025/04/01/extra-axial-soft-tissue-chordoma-of-hand-a-rare-case-report/chordomaextra-axial soft tissue chordomanotochordbrachyuryphysaliferous
spellingShingle P Sakthidasan Chinnathambi
G V R N Krishna Kanth
Anitha Burra
Neha Singh
Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
Journal of Orthopaedic Case Reports
chordoma
extra-axial soft tissue chordoma
notochord
brachyury
physaliferous
title Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
title_full Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
title_fullStr Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
title_full_unstemmed Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
title_short Extra-Axial Soft Tissue Chordoma of Hand: A Rare Case Report
title_sort extra axial soft tissue chordoma of hand a rare case report
topic chordoma
extra-axial soft tissue chordoma
notochord
brachyury
physaliferous
url https://jocr.co.in/wp/2025/04/01/extra-axial-soft-tissue-chordoma-of-hand-a-rare-case-report/
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AT nehasingh extraaxialsofttissuechordomaofhandararecasereport