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Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review.

Abstract Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic histological biphasic pattern. Radical resection with negative margins is the mainstay of treatment; role of adjuvant chemotherapy and radiotherapy is yet not well defined. We report a rare case of 18-year-old man who was diagnosed to have orbital MC. He presented with locally advanced disease with no vision in the affected eye. He underwent right orbital exenteration; a transcranial intradural approach was used to divide the optic nerve, and the temporalis muscle flap was utilised to fill the exenterated orbit. Though optic nerve involvement is rare in orbital MCs, a transcranial approach may be used effectively to avoid traction on optic chiasma and ensure margin-free resection in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis remains dismal in MCs despite treatment.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title bmj case reports
Publication Year Start




PMID- 28258179
OWN - NLM
STAT- MEDLINE
DA  - 20170304
DCOM- 20170313
LR  - 20170313
IS  - 1757-790X (Electronic)
IS  - 1757-790X (Linking)
VI  - 2017
DP  - 2017 Mar 03
TI  - Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini
      review.
LID - bcr2016218744 [pii]
LID - 10.1136/bcr-2016-218744 [doi]
AB  - Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and
      highly aggressive tumour. It has characteristic radiological features and
      pathognomic histological biphasic pattern. Radical resection with negative
      margins is the mainstay of treatment; role of adjuvant chemotherapy and
      radiotherapy is yet not well defined. We report a rare case of 18-year-old man
      who was diagnosed to have orbital MC. He presented with locally advanced disease 
      with no vision in the affected eye. He underwent right orbital exenteration; a
      transcranial intradural approach was used to divide the optic nerve, and the
      temporalis muscle flap was utilised to fill the exenterated orbit. Though optic
      nerve involvement is rare in orbital MCs, a transcranial approach may be used
      effectively to avoid traction on optic chiasma and ensure margin-free resection
      in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis
      remains dismal in MCs despite treatment.
CI  - 2017 BMJ Publishing Group Ltd.
FAU - Jakhetiya, Ashish
AU  - Jakhetiya A
AD  - Department of Surgical Oncology, All India Institute of Medical Sciences,
      NewDelhi, India.
FAU - Shukla, Nootan Kumar
AU  - Shukla NK
AD  - Department of Surgical Oncology, All India Institute of Medical Sciences,
      NewDelhi, India.
FAU - Muduly, Dillip
AU  - Muduly D
AD  - Department of Surgical Oncology, All India Institute of Medical Sciences,
      NewDelhi, India.
FAU - Kale, Shashank S
AU  - Kale SS
AD  - Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi,
      India.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20170303
PL  - England
TA  - BMJ Case Rep
JT  - BMJ case reports
JID - 101526291
SB  - IM
MH  - Adolescent
MH  - Chondrosarcoma, Mesenchymal/diagnosis/radiotherapy/*surgery
MH  - Craniotomy/methods
MH  - Humans
MH  - Male
MH  - Orbital Neoplasms/diagnosis/radiotherapy/*surgery
MH  - Radiotherapy, Adjuvant/methods
MH  - Treatment Outcome
EDAT- 2017/03/05 06:00
MHDA- 2017/03/14 06:00
CRDT- 2017/03/05 06:00
AID - bcr-2016-218744 [pii]
AID - 10.1136/bcr-2016-218744 [doi]
PST - epublish
SO  - BMJ Case Rep. 2017 Mar 3;2017. pii: bcr2016218744. doi: 10.1136/bcr-2016-218744.

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