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ISSN 1561-3070 (Electronic)

2016, Number 2

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Rev Cub Oftal 2016; 29 (2)

Single block orbitectomy in a patient with neurogenic orbital sarcoma

Abreu PFA, Cruz GO, Ortíz RDL, Santos SD, Sánchez WL, Lamadrid GJ

Language: Spanish
References: 18
Page: 345-353
PDF size: 302.29 Kb.


ABSTRACT

Neurogenic sarcoma is a malignant tumor that starts in Schwann cells of the peripheral nerves sheath and is rarely found in the orbit. Here is a 23 year old, male, Caucasian patient, with a history of Type-I Neurofibromatosis, and a left eye fore and lower side displacement of the left eyeball, intense pain and loss of vision for 4 months. A severe proptosis and the lowering of the left eyeball was detected during the ophthalmologic examination, as well as total ophthalmoplegia, severe chemosis, hyperemia, corneal opacity and increased upper eyelid volume. Imaging studies revealed a tumor lesion occupying the whole orbital compartment, with no bone wall infiltration, but causing the displacement of the eyeball out of the orbit border. A combined surgical approach was performed consisting in a single block orbitectomy with total tumor resection, as well as the reconstruction with the ipsilateral temporal muscle rotated flap. The histopathology study showed the presence of an neurogenic orbital sarcoma, so an adjuvant radiotherapy treatment was indicated. The patient was followed up for a year, after which the sequential occurrence of several lesions began.


REFERENCES

  1. Carroll G, Haik B, Fleming J, Weiss R, Mafee M. Peripheral nerve tumors of the orbit. Radiologic Clinics of North America. 2009;37:195-202.

  2. Romero AE, Díaz JA, Lozano A. Tumor maligno de la vaina del nervio periférico (MPNST) glandular de la órbita: primera descripción de la literatura de localización orbitaria en un paciente con neurofibromatosis tipo 1. Murcia Neurocirug. 2010 [citado 20 de junio de 2012];21:1. Disponible en: http://dx.doi.org/10.4321/S1130-14732010000100005

  3. Anghileri M, Miceli R, Fiore M. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006;107:1065-74.

  4. Kachole V, Maria DL, Khalique A, Solanki BR. Malignant schwannoma of orbit. Indian J Ophthalmol. 1979 [cited 2012 Jun 20];27:43-5. Available from: http://www.ijo.in/text.asp?1979/27/1/43/31547

  5. Schmalfuss I. Malignant lesions of the central and posterior skull base. Med Radiol. 2012:261-90.

  6. Grover AK, Rastogi A, Chaturvedi KU, Gupta AK. Schwannoma of the orbit. Indian J Ophthalmol. 1993;41:128-9.

  7. Izumo T, Otsuka M, Okunaga T, Yoshioka T, Yokoyama H, Nagata I. Intraorbital schwannoma arising from the supraorbital nerve. No Shinkei Geka. 2012;40(2):167-71.

  8. Caro E, Martínez SR, García RM. Abordaje transciliar supraorbitario en lesiones intra-axiales. México: Arch Neurocien. 2010;15(2):71-6.

  9. Lambade PN, Lambade D, Saha TK, Dolas RS, Dhobley A. Unusual intramaxillary plexiform schwannoma. Oral Maxillofac Surg. 2013;17(2):137-40.

  10. Zubillaga I, Falguera MI, Sánchez G, Montalvo JJ, Díez R. Abordaje subcraneal. Consideraciones técnicas y aplicaciones en patología traumática craneofacial. Neurocir Murc. 2010 [citado 20 de junio de 2012];21(6). Disponible en: http://dx.doi.org/10.4321/S1130-14732010000600004

  11. Mogedas A, Gutiérrez J, Rosa Palhua RL, Sahuquillo J, Ramón S, Malet D. Abordaje subfrontal para el tratamiento de un estesioneuroblastoma. Rev Esp Cir Oral Maxil. 2012 [citado 7 de noviembre de 2012]; Disponible en: http://dx.doi.org/10.1016/j.maxilo.2012.09.007

  12. Yoshida K, Kawase T, Tomita T, Ogawa K, Kawana H, Yago K, Asanami S. Surgical strategy for tumors located in or extending from the intracranial space to the infratemporal fossa —Advantages of the transcranial approach (zygomatic infratemporal fossa approach and the indications for a combined transcranial and transcervical approach—. Tokyo: Neurol Med Chir. 2009;49(12):580-6.

  13. Santos JA, Mercado R, Ortiz I, García C, Gómez S, Barquet EI, Pane C. Schwannoma trigeminal intracraneal con extensión a la fosa infratemporal, espacio parafaríngeo, órbita, seno maxilar y fosa nasal. A propósito de un caso. Neurocirugía. 2013;16(1):67-74.

  14. Liu JK, Eloy JA. Expanded endoscopic endonasal transcribriform approach for resection of anterior skull base olfactory schwannoma. J Neurosurg. 2012;32(Suppl.):3.

  15. Torres-Mora J, Dry S, Li X, Binder S, Amin M, Folpe AL. Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of "melanotic schwannoma". Am J Surg Pathol. 2014;38(1):94-105.

  16. Masui F, Yokoyama R, Soshi S. A malignant peripheral nerve sheath tumor responding to chemotherapy. J Bone Joint Surg Br. 2004;86:113-5.

  17. Coindre J, Terrier P, Bui N. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol. 1996;14:869-77.

  18. Coscarón B, Pardal JL, Zamora T. Leiomiosarcoma primario nasosinusal con origen en cornete medio. Otorrinolaringol Cast León. 2012;3:115-29.




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