Mesenchymal Chondrosarcoma of the Orbit

Hanakita, Shunya; Kawai, Kensuke; Shibahara, Junichi; Kawahara, Nobutaka; Saito, Nobuhito

doi:10.2176/nmc.52.747

Cited by 12 publications

(6 citation statements)

References 21 publications

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“…Clinically, orbital chondrosarcoma patients mainly present with progressive proptosis often developing over months to years, as seen generally in our patients 5, 7. Less frequent symptoms include: pain of variable severity, diplopia and impaired vision while ptosis, lacrimation and nasal obstruction were less consistently described 5, 7, 11. One of our patients described painful eye movements as shown in Table 1.…”

Section: Discussionsupporting

confidence: 65%

“…Orbital MCS was first described by Cardenas-Ramirez in 1959 4 . Subsequently, reports of orbital involvement by this neoplasm were published reaching 28 cases mentioned in the English-written literature mostly in adults 5 . As the orbit is a rare site of mesenchymal chondrosarcoma, here we report 3 pediatric cases with primary orbital MCS.…”

Section: Introductionmentioning

confidence: 75%

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Primary mesenchymal chondrosarcoma of the orbit: Histopathological report of 3 pediatric cases

Alkatan

Eberhart

Alshomar

et al. 2018

Saudi Journal of Ophthalmology

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Mesenchymal chondrosarcoma (MCS) is an unusual tumor mainly found in the skeleton. Around third of the cases occur in extra-skeletal sites with the orbit being the third most common site in these cases. In previous reviews of the orbital cases, it has been concluded that orbital MCS tends to occur in women in the second or third decades of life. However, 8 cases of orbital MCS have been reported so far in the pediatric age group (age less than 18 years-old) one of which has been considered congenital MCS in a 5-days old newborn girl. We describe 3 additional pediatric cases with primary orbital MCS and they were all males. Our cases presented with proptosis and calcific orbital masses on imaging studies. Histopathological examination of the excised masses shared the typical presence of undifferentiated mesenchymal cells and immature areas of cartilage. The diagnosis of MCS was further confirmed by immunohistochemical staining. Brief review of the literature in relation to this diagnosis in the orbit is also presented.

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Section: Discussionsupporting

confidence: 65%

Section: Introductionmentioning

confidence: 75%

Primary mesenchymal chondrosarcoma of the orbit: Histopathological report of 3 pediatric cases

Alkatan

Eberhart

Alshomar

et al. 2018

Saudi Journal of Ophthalmology

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“…Even if radical resection is not performed in the first surgery, the patient’s outcome could improve if the tumors were removed with safety margins in the multiple surgeries. 19) In the current case, if we had removed his tumor with safety margins less than 84 months after initial surgery, the tumor might not have recurred.…”

Section: Discussionmentioning

confidence: 70%

“…It has been reported that a series of intraorbital MC patients had good outcomes after radical removal of their tumors. 19) However, the most difficult problem is the decision to determine the appropriate timing of total removal with minimal neurological deficit. Even if radical resection is not performed in the first surgery, the patient’s outcome could improve if the tumors were removed with safety margins in the multiple surgeries.…”

Section: Discussionmentioning

confidence: 99%

Multiple Surgical Treatments for Repeated Recurrence of Skull Base Mesenchymal Chondrosarcoma

Murakami

Jinguji

Kishida

et al. 2018

NMC Case Report Journal

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We report a case of a young male who received multiple surgical treatments for repeated recurrence of skull base mesenchymal chondrosarcoma (MC). When the patient was 18 years old, we subtotally removed the skull base MC and he was treated with stereotactic radiosurgery for remnant tumors in the left cavernous sinus. After 30 months, we removed residual tumors that had regrown partially, via combined endonasal endoscopic and orbitozygomatic approaches. Over the next 65 months, the patient refused radical resection, and received six salvage surgeries, two stereotactic radiotherapies, and five stereotactic radiosurgeries for repeated recurrence. At 95 months after initial surgery, the tumors had extended to the skull base and nasal cavities. As a result, the left eye had been blinded and right visual acuity was deteriorated. We performed left anterior-middle cranial base resection, removal of nasal and intradural tumors, high flow bypass, en-bloc resection of the left cavernous sinus and clivus, and reconstruction using an abdominal flap. Even though the main tumors were removed with safety margins, tumors around the right optic nerve were removed by piecemeal to preserve right eye function. Six months after the radical resection, tumors in the right orbital apex recurred because we had been unable to remove the tumor with adequate safety margins. Skull base MC has a high tendency to recur locally, so these tumors should be radically removed with safety margins as early as possible to prevent recurrence.

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“…8,9 MCS tends to develop in young patients in their second or third decades of life, with a female predominance. 14,19,21 The age at presentation ranges from 9 to 39 years, although there are some reports in congenital 11 or middle-aged patients, 18,19 with most cases occur between 10 and 30 years of age. 11,14 MCS of the orbit mainly affects female patients, 11 and our study, which included three females and two males, supports this finding.…”

Section: Discussionmentioning

confidence: 99%