Mesenchymal Chondrosarcoma of the Orbit: A Report of Two Cases and Review of the Literature

Kaur, Apjit; Kishore, Poonam; Agrawal, Ajai; Gupta, Arnav

doi:10.1080/01676830601169007

Cited by 16 publications

(6 citation statements)

References 9 publications

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“…8,9,14,20) Intraorbital MC is extremely rare, with only a few reported cases. [2][3][4][5][6][9][10][11][15][16][17][18][19][20][21][22][23] We describe a case of intraorbital MC successfully treated with intraorbital exenteration.…”

Section: Introductionmentioning

confidence: 99%

Mesenchymal Chondrosarcoma of the Orbit

Hanakita

Kawai

Shibahara

et al. 2012

Neurol. Med. Chir.(Tokyo)

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A 20-year-old woman presented with a rare case of intraorbital mesenchymal chondrosarcoma manifesting as a 6-month history of progressive ptosis and exophthalmos of her left eye. Computed tomography and magnetic resonance imaging revealed a partially calcified round mass occupying the postbulbar space. Partial removal of the tumor via a left fronto-orbital approach was performed. The histological diagnosis was mesenchymal chondrosarcoma, and additional intraorbital exenteration was performed. Neither chemotherapy nor radiotherapy was performed. She was free from tumor recurrence at the 6-year follow-up examination. Radical resection, including exenteration if possible, is recommended for intraorbital mesenchymal chondrosarcoma.

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Section: Introductionmentioning

confidence: 99%

Mesenchymal Chondrosarcoma of the Orbit

Hanakita

Kawai

Shibahara

et al. 2012

Neurol. Med. Chir.(Tokyo)

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“…Common presentations are proptosis of eye ball with decreased visual acuity, pain in eye, headache and restriction of eyeball movements. Distant metastasis is exceptional at presentation 3 5 6 13. Radiological examination is usually warranted for diagnosis and anatomical localisation to access operability.…”

Section: Discussionmentioning

confidence: 99%

“…However, if the tumour is surgically unresectable or histologically highly aggressive, preoperative chemotherapy and radiation therapy may be considered 3 16 21. Adjuvant radiation can be considered in case of incomplete resection, close margins and recurrent tumours 5 6 13 21–23. Adjuvant chemotherapy may be considered in cases with aggressive behaviour and high-grade lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Owing to rarity of these tumours, there are no definitive management guidelines available. Radical resection with negative margins is the primary modality of treatment; the role of adjuvant therapy is yet to be clearly defined 2 3 5 6. Though orbital location is associated with better survival compared to other locations, overall prognosis of MCs remains dismal 2 3…”

Section: Introductionmentioning

confidence: 99%

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Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review

et al. 2017

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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.

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“…Immunohistochemical analysis often reveals positivity for vimentin, S100 protein and CD99; meanwhile, actin, cytokeratin, and EMA are typically negative [9, 10]. The differential diagnosis of orbital MCS includes hemangiopericytoma, myxochondrosarcoma, osteogenic sarcoma, and osteochondroma as primary tumors and lymphoma, neuroblastoma, synovial cell sarcoma, and chondrosarcoma as tumors affecting the orbit secondarily (direct invasion or metastasis) [11]. This case presented no difficulty in the histopathological diagnosis since it showed a characteristic morphological pattern composed of round cells with an abrupt transition to well-differentiated hyaline cartilage and a hemangiopericytoma-like vascular pattern.…”

Section: Discussionmentioning

confidence: 99%

Primary Orbital Mesenchymal Chondrosarcoma: Case Report and Review of the Literature

Herrera

Ortega

Reyes

et al. 2012

Case Reports in Medicine

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Orbital mesenchymal chondrosarcoma is a very uncommon lesion of the bone and extraskeletal tissue. To our knowledge, approximately 30 cases have been described. We present the case of a 52-year-old male who presented with a history of progressive proptosis and chemosis of the right eye caused by an orbital tumor. He underwent exenteration of the right orbit, and the histological examination revealed a mesenchymal orbital chondrosarcoma. This paper attempts to describe a rare entity that should be considered in the differential diagnosis of calcified orbital lesions, especially in young adults. Complete removal of the tumor is the mainstay of treatment, but adjuvant radiation therapy and chemotherapy should be considered.

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Mesenchymal Chondrosarcoma of the Orbit: A Report of Two Cases and Review of the Literature

Cited by 16 publications

References 9 publications

Mesenchymal Chondrosarcoma of the Orbit

Mesenchymal Chondrosarcoma of the Orbit

Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review

Primary Orbital Mesenchymal Chondrosarcoma: Case Report and Review of the Literature

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