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Orbit sarcoma

MedGen UID:
277449
•Concept ID:
C1335131
•
Neoplastic Process
Synonyms: orbit of skull sarcoma; Orbit Sarcoma; orbit sarcoma; Orbital Sarcoma; orbital sarcoma; Sarcoma of orbit; Sarcoma of Orbit; sarcoma of orbit; sarcoma of orbit of skull; Sarcoma of the Orbit; sarcoma of the orbit
SNOMED CT: Sarcoma of orbit (699354006)
 
Monarch Initiative: MONDO:0004943

Definition

A malignant soft tissue neoplasm that arises from the structures of the orbit. The majority of the cases are rhabdomyosarcomas. [from NCI]

Professional guidelines

PubMed

Primary Ewing's sarcoma with orbit involvement: Survival and visual outcomes after eye-sparing multidisciplinary management in eight patients.
Koka K, Rahim FE, El-Hadad C, Bell D, Debnam JM, Guo Y, Esmaeli B
Head Neck 2021 Dec;43(12):3857-3865. Epub 2021 Oct 10 doi: 10.1002/hed.26884. PMID: 34632670

Recent clinical studies

Etiology

Intramuscular Eyelid Myxoma.
Liu CK, Williamson JE 3rd, Milman T, Lally SE, Shields CL
Ophthalmic Plast Reconstr Surg 2022 Nov-Dec 01;38(6):e180-e183. Epub 2022 Jul 4 doi: 10.1097/IOP.0000000000002236. PMID: 35793663
Rhabdomyosarcoma in children.
Hayes-Jordan A, Andrassy R
Curr Opin Pediatr 2009 Jun;21(3):373-8. doi: 10.1097/MOP.0b013e32832b4171. PMID: 19448544
Retinoblastoma.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F, Desjardins L
Orphanet J Rare Dis 2006 Aug 25;1:31. doi: 10.1186/1750-1172-1-31. PMID: 16934146Free PMC Article
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992
Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease.
Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS
J Clin Oncol 2001 Jun 15;19(12):3091-102. doi: 10.1200/JCO.2001.19.12.3091. PMID: 11408506

Diagnosis

Intramuscular Eyelid Myxoma.
Liu CK, Williamson JE 3rd, Milman T, Lally SE, Shields CL
Ophthalmic Plast Reconstr Surg 2022 Nov-Dec 01;38(6):e180-e183. Epub 2022 Jul 4 doi: 10.1097/IOP.0000000000002236. PMID: 35793663
Extraskeletal Mesenchymal Chondrosarcoma.
Arora K, Riddle ND
Arch Pathol Lab Med 2018 Nov;142(11):1421-1424. doi: 10.5858/arpa.2017-0109-RS. PMID: 30407856
Ptosis Shmosis.
Kinori M, Ben Simon GJ, Zehavi-Dorin T, Robbins SL, Siatkowski RM
Surv Ophthalmol 2017 Mar-Apr;62(2):237-240. Epub 2016 Feb 26 doi: 10.1016/j.survophthal.2016.02.004. PMID: 26921808
Rhabdomyosarcoma in children.
Hayes-Jordan A, Andrassy R
Curr Opin Pediatr 2009 Jun;21(3):373-8. doi: 10.1097/MOP.0b013e32832b4171. PMID: 19448544
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Therapy

Chemotherapy Options for Poor Responders to Neoadjuvant Chemotherapy for Orbital Granulocytic Sarcoma.
Gossai N, Cafferty R, Weigel B
Curr Treat Options Oncol 2016 Jul;17(7):38. doi: 10.1007/s11864-016-0411-7. PMID: 27300546
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992
Sarcomas of the head and neck.
Potter BO, Sturgis EM
Surg Oncol Clin N Am 2003 Apr;12(2):379-417. doi: 10.1016/s1055-3207(03)00005-x. PMID: 12916461
Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease.
Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS
J Clin Oncol 2001 Jun 15;19(12):3091-102. doi: 10.1200/JCO.2001.19.12.3091. PMID: 11408506
Radiation-induced fibrosarcoma.
Gane NF, Lindup R, Strickland P, Bennett MH
Br J Cancer 1970 Dec;24(4):705-11. doi: 10.1038/bjc.1970.84. PMID: 5503597Free PMC Article

Prognosis

Intramuscular Eyelid Myxoma.
Liu CK, Williamson JE 3rd, Milman T, Lally SE, Shields CL
Ophthalmic Plast Reconstr Surg 2022 Nov-Dec 01;38(6):e180-e183. Epub 2022 Jul 4 doi: 10.1097/IOP.0000000000002236. PMID: 35793663
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma.
Cortes Barrantes P, Jakobiec FA, Dryja TP
Semin Ophthalmol 2019;34(4):243-251. Epub 2019 May 30 doi: 10.1080/08820538.2019.1620802. PMID: 31146616
Extraskeletal Mesenchymal Chondrosarcoma.
Arora K, Riddle ND
Arch Pathol Lab Med 2018 Nov;142(11):1421-1424. doi: 10.5858/arpa.2017-0109-RS. PMID: 30407856
Retinoblastoma.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F, Desjardins L
Orphanet J Rare Dis 2006 Aug 25;1:31. doi: 10.1186/1750-1172-1-31. PMID: 16934146Free PMC Article
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Clinical prediction guides

Low-grade myofibroblastic sarcoma of the orbit: A case report and literature review.
Zhang S, Ma Y, Ma T, Wang Z
Medicine (Baltimore) 2017 Dec;96(51):e9172. doi: 10.1097/MD.0000000000009172. PMID: 29390454Free PMC Article
Prognostication in eye cancer: the latest tumor, node, metastasis classification and beyond.
Kivelä T, Kujala E
Eye (Lond) 2013 Feb;27(2):243-52. Epub 2012 Dec 21 doi: 10.1038/eye.2012.256. PMID: 23258307Free PMC Article
From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit.
Chung EM, Smirniotopoulos JG, Specht CS, Schroeder JW, Cube R
Radiographics 2007 Nov-Dec;27(6):1777-99. doi: 10.1148/rg.276075138. PMID: 18025517
Retinoblastoma.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F, Desjardins L
Orphanet J Rare Dis 2006 Aug 25;1:31. doi: 10.1186/1750-1172-1-31. PMID: 16934146Free PMC Article
Sarcomas of the head and neck.
Potter BO, Sturgis EM
Surg Oncol Clin N Am 2003 Apr;12(2):379-417. doi: 10.1016/s1055-3207(03)00005-x. PMID: 12916461

Recent systematic reviews

Ophthalmologic aspects of Dermatofibrosarcoma Protuberans: A systematic review in the context of a rare case of primary orbital involvement.
Eshraghi B, Jahanbani-Ardakani HR, Abtahi SM, Abtahi SH
J Fr Ophtalmol 2019 Oct;42(8):913-924. Epub 2019 Apr 10 doi: 10.1016/j.jfo.2018.11.012. PMID: 30981544
Publication trend, resource utilization, and impact of the US National Cancer Database: A systematic review.
Su C, Peng C, Agbodza E, Bai HX, Huang Y, Karakousis G, Zhang PJ, Zhang Z
Medicine (Baltimore) 2018 Mar;97(9):e9823. doi: 10.1097/MD.0000000000009823. PMID: 29489679Free PMC Article
Treatment of orbital solitary fibrous tumour with gamma knife radiosurgery and systematic review of literature.
Tata A, Cohen-Inbar O, Sheehan JP
BMJ Case Rep 2016 Oct 7;2016 doi: 10.1136/bcr-2016-217114. PMID: 27758816Free PMC Article
Management of neonatal proptosis: a systematic review.
Erickson BP, Tse DT
Surv Ophthalmol 2014 Jul-Aug;59(4):378-92. Epub 2013 Dec 3 doi: 10.1016/j.survophthal.2013.11.002. PMID: 24929905

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