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Benign Orbital Neurilemoma: Sarcomatous Transformation in von Recklinghausen's Disease

Benign Orbital Neurilemoma: Sarcomatous Transformation in von Recklinghausen's Disease Abstract NEURILEMOMAS (benign schwannomas) are found in 1.5%1 to 18%2,3 of patients with von Recklinghausen's neurofibromatosis and make up 1% of orbital tumors.4-9 They rarely, if ever, undergo malignant change.2,10-17 This paper describes, to this author's knowledge, the first reported case of sarcomatous transformation of a benign neurilemoma of the orbit. Report of a Case A 27-year-old white woman (51-38-29) was first seen at the University of Illinois Eye Clinic on Nov 21, 1966, and was admitted to the hospital with a six-week history of a mass in the superior right orbit. Two weeks previous to admission she noted gradual proptosis and some pain. Additional history revealed the mother and sister had café-au-lait spots. Examination of the patient showed a right superior orbital mass with some proptosis and mechanical limitation of upward gaze. The mass was firm, discrete, round, and movable. Vision was 20/40 OD, 20/30 OS. References 1. Gore DOO, Rankow R, Hanford JM: Parapharyngeal neurilemmoma . Surg Gynec Obstet 103:193-201, 1956. 2. Vieta JO, Pack GT: Malignant neurilemmomas of peripheral nerves . Amer J Surg 82:416-431, 1951.Crossref 3. Stout AP: Neurofibroma and neurilemmoma . Clin Proc 5:1-12, 1946. 4. O'Brien CS, Leinfelder PJ: Unilateral exophthalmos: Etiological and diagnostic studies in 82 consecutive cases . Amer J Ophthal 18:123-132, 1935. 5. Forrest AW: Intraorbital tumors . Arch Ophthal 41:198-232, 1949.Crossref 6. Bullock LJ, Reeves RJ: Unilateral exophthalmos: Roentgenographic aspects . Amer J Roentgen 82:290-299, 1959. 7. Schultz RO, Harris RD, Hamilton HE: Asymmetric proptosis . Amer J Ophthal 52:10-15, 1961. 8. Jackson H: Orbital tumors . J Neurosurg 19:551, 1962.Crossref 9. Reese AB: Tumors of the Eye , ed 2. New York, Paul B Hoeber Inc, 1963, pp 190-202, 534-535. 10. Kragh LV, Soule EH, Masson JK: Benign and malignant neurilemmomas of the head and neck . Surg Gynec Obstet 111:211-218, 1960. 11. Evans RW: Histological Appearances of Tumours , ed 2. Edinburgh, E & S Livingston Ltd, 1966, pp 359-383. 12. Hogan MJ, Zimmerman LE: Ophthalmic Pathology , ed 2. Philadelphia, WB Saunders Co, 1962, pp 443-449, 753-757. 13. Chang KHR, Niguidula FN: Metastatic malignant schwannoma with bilateral pulmonary resection . Surgery 50:749-754, 1961. 14. Stout AP: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma) . Amer J Cancer 24:751-796, 1935.Crossref 15. Tykstra PC: The pathology of acoustic neuromas . Arch Otolaryng 80:605-616, 1964.Crossref 16. D'Agostino AN, Soule EH, Miller RH: Primary malignant neoplasms of nerves (malignant neurilemmomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease) . Cancer 16:1003-1014, 1963.Crossref 17. Stout AP: Tumors of the peripheral nervous system , in Atlas of Tumor Pathology , section 2, pt 6. Armed Forces Institute of Pathology, 1949. 18. Drescher EP, Benedict WL: Asymmetric exophthalmos . Arch Ophthal 44:109-128, 1950.Crossref 19. Iliff CE: Tumors of the orbit . Trans Amer Ophthal Soc 55:505-541, 1957. 20. Van Buren JM, Poppen JL, Horrax G: Unilateral exophthalmos: A consideration of symptom pathogenesis . Brain 80:139-175, 1957.Crossref 21. Silva D: Orbital tumors . Amer J Ophthal 64:318-339, 1968. 22. Pfeiffer RL: Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmology . Amer J Ophthal 26:724-741, 816-833, 938-942, 1943. 23. Moss HM: Expanding lesions of the orbit: A clinical study of 230 consecutive cases . Amer J Ophthal 54:761-770, 1962. 24. Foster J: The diagnosis and treatment of orbital tumors . Ann Roy Coll Surg Eng 17:114-129, 1955. 25. Holt JF, Wright EM: Radiological features of neurofibromatosis . Radiology 51:647-663, 1948.Crossref 26. Hosoi K: Multiple neurofibromatosis (von Recklinghausen's disease), with special reference to malignant transformation . Arch Surg 22:258-281, 1931.Crossref 27. Preston FW, Walsh WS, Clarke TH: Cutaneous neurofibromatosis (von Recklinghausen's disease): Clinical manifestations of incidence of sarcoma in 16 male patients . Arch Surg 64:813-827, 1952.Crossref 28. Rottino A, Kelly AS: Specific nerve sheath tumor of orbit . Arch Ophthal 26:478-488, 1941.Crossref 29. Hiranandani LH, Hiranandani NL, Hiranandani GK: Malignant schwannoma (neurogenic sarcoma) . J Laryng 81:1409-1413, 1967.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Benign Orbital Neurilemoma: Sarcomatous Transformation in von Recklinghausen's Disease

Archives of Ophthalmology , Volume 86 (3) – Sep 1, 1971

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References (33)

Publisher
American Medical Association
Copyright
Copyright © 1971 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1971.01000010270006
Publisher site
See Article on Publisher Site

Abstract

Abstract NEURILEMOMAS (benign schwannomas) are found in 1.5%1 to 18%2,3 of patients with von Recklinghausen's neurofibromatosis and make up 1% of orbital tumors.4-9 They rarely, if ever, undergo malignant change.2,10-17 This paper describes, to this author's knowledge, the first reported case of sarcomatous transformation of a benign neurilemoma of the orbit. Report of a Case A 27-year-old white woman (51-38-29) was first seen at the University of Illinois Eye Clinic on Nov 21, 1966, and was admitted to the hospital with a six-week history of a mass in the superior right orbit. Two weeks previous to admission she noted gradual proptosis and some pain. Additional history revealed the mother and sister had café-au-lait spots. Examination of the patient showed a right superior orbital mass with some proptosis and mechanical limitation of upward gaze. The mass was firm, discrete, round, and movable. Vision was 20/40 OD, 20/30 OS. References 1. Gore DOO, Rankow R, Hanford JM: Parapharyngeal neurilemmoma . Surg Gynec Obstet 103:193-201, 1956. 2. Vieta JO, Pack GT: Malignant neurilemmomas of peripheral nerves . Amer J Surg 82:416-431, 1951.Crossref 3. Stout AP: Neurofibroma and neurilemmoma . Clin Proc 5:1-12, 1946. 4. O'Brien CS, Leinfelder PJ: Unilateral exophthalmos: Etiological and diagnostic studies in 82 consecutive cases . Amer J Ophthal 18:123-132, 1935. 5. Forrest AW: Intraorbital tumors . Arch Ophthal 41:198-232, 1949.Crossref 6. Bullock LJ, Reeves RJ: Unilateral exophthalmos: Roentgenographic aspects . Amer J Roentgen 82:290-299, 1959. 7. Schultz RO, Harris RD, Hamilton HE: Asymmetric proptosis . Amer J Ophthal 52:10-15, 1961. 8. Jackson H: Orbital tumors . J Neurosurg 19:551, 1962.Crossref 9. Reese AB: Tumors of the Eye , ed 2. New York, Paul B Hoeber Inc, 1963, pp 190-202, 534-535. 10. Kragh LV, Soule EH, Masson JK: Benign and malignant neurilemmomas of the head and neck . Surg Gynec Obstet 111:211-218, 1960. 11. Evans RW: Histological Appearances of Tumours , ed 2. Edinburgh, E & S Livingston Ltd, 1966, pp 359-383. 12. Hogan MJ, Zimmerman LE: Ophthalmic Pathology , ed 2. Philadelphia, WB Saunders Co, 1962, pp 443-449, 753-757. 13. Chang KHR, Niguidula FN: Metastatic malignant schwannoma with bilateral pulmonary resection . Surgery 50:749-754, 1961. 14. Stout AP: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma) . Amer J Cancer 24:751-796, 1935.Crossref 15. Tykstra PC: The pathology of acoustic neuromas . Arch Otolaryng 80:605-616, 1964.Crossref 16. D'Agostino AN, Soule EH, Miller RH: Primary malignant neoplasms of nerves (malignant neurilemmomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease) . Cancer 16:1003-1014, 1963.Crossref 17. Stout AP: Tumors of the peripheral nervous system , in Atlas of Tumor Pathology , section 2, pt 6. Armed Forces Institute of Pathology, 1949. 18. Drescher EP, Benedict WL: Asymmetric exophthalmos . Arch Ophthal 44:109-128, 1950.Crossref 19. Iliff CE: Tumors of the orbit . Trans Amer Ophthal Soc 55:505-541, 1957. 20. Van Buren JM, Poppen JL, Horrax G: Unilateral exophthalmos: A consideration of symptom pathogenesis . Brain 80:139-175, 1957.Crossref 21. Silva D: Orbital tumors . Amer J Ophthal 64:318-339, 1968. 22. Pfeiffer RL: Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmology . Amer J Ophthal 26:724-741, 816-833, 938-942, 1943. 23. Moss HM: Expanding lesions of the orbit: A clinical study of 230 consecutive cases . Amer J Ophthal 54:761-770, 1962. 24. Foster J: The diagnosis and treatment of orbital tumors . Ann Roy Coll Surg Eng 17:114-129, 1955. 25. Holt JF, Wright EM: Radiological features of neurofibromatosis . Radiology 51:647-663, 1948.Crossref 26. Hosoi K: Multiple neurofibromatosis (von Recklinghausen's disease), with special reference to malignant transformation . Arch Surg 22:258-281, 1931.Crossref 27. Preston FW, Walsh WS, Clarke TH: Cutaneous neurofibromatosis (von Recklinghausen's disease): Clinical manifestations of incidence of sarcoma in 16 male patients . Arch Surg 64:813-827, 1952.Crossref 28. Rottino A, Kelly AS: Specific nerve sheath tumor of orbit . Arch Ophthal 26:478-488, 1941.Crossref 29. Hiranandani LH, Hiranandani NL, Hiranandani GK: Malignant schwannoma (neurogenic sarcoma) . J Laryng 81:1409-1413, 1967.Crossref

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Sep 1, 1971

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