Pigmentation Process TRAINING COURSE. Pigmentation Process TRAINING COURSE The Pigmentation Process.
ORBITAL NEURILEMMOMA WITH CAFE-AU-LAIT PIGMENTATION … · Brit. J. Ophthal. (1968) 52, 262 ORBITAL...
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Brit. J. Ophthal. (1968) 52, 262 ORBITAL NEURILEMMOMA WITH CAFE-AU-LAIT PIGMENTATION OF THE SKIN*t BY ALY MORTADA Department of Ophthalmology, Faculty of Medicine, Cairo University, Egypt IN the two cases here described, a solitary orbital neurilemmoma was associated with pigmentation of the skin suggesting an association with von Recklinghausen's disease (Strachov and Shepkalova, 1941; Reese, 1963). Case Reports There was no important family history and no history of trauma or x-ray therapy, and a complete general medical examination was negative. Case 1, a 35-year-old woman (Fig. 1), complained of right proptosis of one year's duration. Her skin showed areas of cafe-au-lait pigmentation (Fig. 2). [;, >R'' i:LD:X ''?~~~~~~~~~~~~~~i g ^=~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~. ............. s FiG. I.-Case 1. Right proptosis of one year's t.....i duration due to orbital encapsulated neurilem- moma in a woman aged 35 years. FIG. 2.-Case l. Caft-au-lait areas of pigmenta- tion on the skin of the back. Examination.-The left eye was normal with visual acuity 6/12. The right eye showed proptosis 20 mm. (left 15 mm.), and deviated outwards with limitation of ocular movements inwards. The fundus showed optic atrophy and the visual acuity was hand movements. A firm mass was felt in the inner side of the orbit extending backwards. Surgery.-Through a medial fomix conjunctival incision the tumour was removed by blunt finger dissection. It was encapsulated and firm, measuring 3 x 3 cm., with a smooth surface (Fig. 3, opposite) and pink in colour. Histopathological Examination.-There were spindle Schwann cells with elongated nuclei showing the characteristic palisade arrangement. Between the cells were long slender straight or serpentine reticulum fibres giving the appearance of spindle cellular fibrillar tissue of Antoni type A (Fig. 4, opposite). There were no nerve fibrils. The appearances were those of a neurilemmoma. * Received for publication, November 18, 1966. t Address for reprints: 18A 26th July Street, Cairo, Egypt. 262 on January 19, 2021 by guest. Protected by copyright. http://bjo.bmj.com/ Br J Ophthalmol: first published as 10.1136/bjo.52.3.262 on 1 March 1968. Downloaded from
Transcript of ORBITAL NEURILEMMOMA WITH CAFE-AU-LAIT PIGMENTATION … · Brit. J. Ophthal. (1968) 52, 262 ORBITAL...
Brit. J. Ophthal. (1968) 52, 262
ORBITAL NEURILEMMOMA WITH CAFE-AU-LAITPIGMENTATION OF THE SKIN*t
BY
ALY MORTADADepartment of Ophthalmology, Faculty of Medicine, Cairo University, Egypt
IN the two cases here described, a solitary orbital neurilemmoma was associated withpigmentation of the skin suggesting an association with von Recklinghausen's disease(Strachov and Shepkalova, 1941; Reese, 1963).
Case ReportsThere was no important family history and no history oftrauma or x-ray therapy, and a complete
general medical examination was negative.Case 1, a 35-year-old woman (Fig. 1), complained of right proptosis of one year's duration. Her skinshowed areas of cafe-au-lait pigmentation (Fig. 2).
[;, >R'' i:LD:X ''?~~~~~~~~~~~~~~i g
^=~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..............s
FiG. I.-Case 1. Right proptosis of one year's t.....iduration due to orbital encapsulated neurilem-moma in a woman aged 35 years.
FIG. 2.-Case l. Caft-au-lait areas of pigmenta-tion on the skin of the back.
Examination.-The left eye was normal with visual acuity 6/12. The right eye showed proptosis 20 mm.(left 15 mm.), and deviated outwards with limitation of ocular movements inwards. The fundus showedoptic atrophy and the visual acuity was hand movements. A firm mass was felt in the inner side of theorbit extending backwards.Surgery.-Through a medial fomix conjunctival incision the tumour was removed by blunt finger
dissection. It was encapsulated and firm, measuring 3 x 3 cm., with a smooth surface (Fig. 3, opposite)and pink in colour.
Histopathological Examination.-There were spindle Schwann cells with elongated nuclei showing thecharacteristic palisade arrangement. Between the cells were long slender straight or serpentine reticulumfibres giving the appearance of spindle cellular fibrillar tissue of Antoni type A (Fig. 4, opposite). There wereno nerve fibrils. The appearances were those of a neurilemmoma.
* Received for publication, November 18, 1966.t Address for reprints: 18A 26th July Street, Cairo, Egypt.
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ORBITAL NEURILEMMOMA
FIG. 4.-Case 1. Section of neurilemmoma. x 400.
FIG. 3. -Case 1. Encapsulated tumourafter removal.
Case 2, a 60-year-old woman (Fig. 5), complained of left gradual proptosis of 2 years' duration.Examination.-The right eye showed a mature senile cataract; the visual acuity was hand-movements
with good projection of light.The left eye showed upward proptosis 20 mm. (right 15 mm.), an immature senile cataract, and visual
acuity 1/60. The fundus showed optic atrophy. There was a palpable mass between the globe and thelower orbital margin.
FIG. 5.-Case 2. Left proptosis of 2 years' dura-tion due to orbital encapsulated neurilemmoma ina woman aged 60 years.
....~~~~~~~~~~~~~~~~~~~~~..... ......
Surgery.-Through a lower fornix conjunctival incision the tumour was removed by blunt finger dis-section. It was encapsulated, firm, and pink, measuring 2 v 3 cm. (Fig. 6, overleaf).
Histopathological Examination.-The appearances were those of a neurilemmoma (Fig. 7, overleaf).A 5-year follow-up of the two cases showed neither proptosis nor recurrence of the neoplasm.
DiscussionIt is the general opinion that a neurilemmoma is an isolated entity, while a neurofibroma
may be a local manifestation of von Recklinghausen's neurofibromatosis (Duke-Elder,1952). Herbut (1959) said that it mattered little from which type of cell the tumouroriginated or what name it was given as they have similar pathological and clinical pro-perties; when the tumour is multiple the condition is referred to as neurofibromatosis. Anurilemmoma may show nerve fibres in its periphery and a neurofibroma usually containsspindle cells frequently arranged with palisading of the nuclei.
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ALY MORTADAx;&r_ 3:. X§b.w
FIG. 7.Case 2. Section of neurilemmoma. x 90.
FIG. 6.-Case 2. Encapsulated tumourafter removal.
Summary(1) Although neurilemimioma is considered to be an isolated entity not associated with
neurofibromatosis, these two solitary orbital encapsulated neurilemmomata were associatedwith patches of cafe-au-lait pigmentation.
(2) These are the first two orbital neurilemmomata to be described from Egypt.
REFERENCESDUKE-ELDER, S. (1952). "Text-book of Ophthalmology", vol. 5, p. 5579. Kimpton, London.HERBUT, P. A. (1959). "Pathology", 2nd ed., p. 1391. Kimpton, London.REESE, A. B. (1963). "Tumors of the Eye", 2nd ed., p. 202. Harper and Row, New York, Evanston, and
London.STRACHOV, V. P., and SHEPKALOVA, V. M. (1941). Vestn. oftal., 18, 12.
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