Cellular Blue Nevus of the Sclera Taylor
R.
Smith, MD, Robert J. Brockhurst, MD
\s=b\ An isolated cellular blue nevus of the sclera was discovered and removed during a retinal detachment operation. Such benign tumors may be misdiagnosed clinically as an extension of a melanoma of the choroid or histologically as melanoma.
(Arch Ophthalmol 94:618-620, 1976)
Boniuk1 reported a similar scierai lesion with a two-year fol¬
Society,
low-up period.
This paper reports the occurrence of a large, isolated, cellular blue nevus that was noted incidentally in the sclera at the time of retinal detach¬ ment surgery.
REPORT OF A CASE
melanotic lesions involv¬ ing only the sclera have not been described until recently. Previous re¬ ports of melanotic lesions of the sclera indicate that they are usually associated with other melanotic le¬ sions. For example, Johnson1 has re¬ ported a blue nevus of the sclera that was associated with an underlying choroidal nevus. Scheie and Yanoffdescribed a patient with a melanocytoma of the ciliary body with involve¬ ment of the overlying sclera. Hagler and Brown' reported an orbital ma¬ lignant melanoma arising in a nevus of Ota, the sclera showing dendritic melanocytes characteristic of a blue nevus. We could find no report of an isolated cellular blue nevus of the sclera in the literature; however, when our specimen was presented at the 1974 meeting of the Verhoeff
Isolated
50-year-old
man was examined be¬ of loss of vision in the left eye of four weeks' duration. Past ocular history disclosed no notable eye disorders, the medical review indicated that his general health was excellent, and his only surgical experience had been a herniorrhaphy ten
A
cause
Fig 1 .—Black gery.
pigmented
mass
in sclera discovered at time of retinal detachment
Submitted for publication Dec 18, 1974. From the Eye Pathology Laboratory and Retina Service, Massachusetts Eye and Ear Infirmary, and the Department of Retina Research of the Eye Research Institute, Retina Foundation, Boston.
Reprint requests
to
years previously. On examination, corrected visual acuity measured 20/20 in the right eye and hand movements at four feet in the left eye. In¬ traocular pressure measured 18 mm Hg in the right eye and 14 mm Hg in the left. Biomicroscopic examination of the ante¬ rior segments of the eye revealed no signs of inflammation, but slight nuclear scle¬ rosis was found in each lens. The right fundus showed no important abnormality. Examination of the left fundus showed retinal detachment, including the macula, extending from the 9-o'clock to the 6o'clock positions moving clockwise as a re¬ sult of a dialysis that extended from the 1o'clock to the 4-o'clock positions. The retina was attached in the lower nasal quadrant.
Ophthalmic Library, Eye
Research Institute of the Retina Foundation, 20 Staniford St, Boston, MA 02114 (Dr Brockhurst).
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
sur¬
location of black mass under medial measured 7x15 mm and was elevated 2 mm.
Fig 2 (left).—Sketch showing rectus;
mass
Fig 3.—Tumor cells with biphasic appearance comprised of sclerotic areas (Fig 4) and cellular areas (Fig 5) (hematoxylin-eo¬
sin, original magnification x40).
4 (left).—Sclerotic areas similar to blue nevus with melanocytes and melanophages (hematoxylin-eosin, original magnifi¬ cation 1,000).
Fig
5.—Whorls of spindle cells with prominent nuclei and small nucleoli with scanty cytoplasm (hematoxylin-eosin, original mag¬ nification x250).
Fig
No abnormalities were observed in the cho¬ roid or pars plana nasally. At the time of retinal detachment sur¬ gery, a black tumor mass was seen in the sclera (Fig 1 and 2). The tumor measured 15 mm equatorially and 7 mm anteroposteriorly and was elevated 2.0 mm. Its anterior edge was 14 mm behind the limbus at the equator. Its lower extremity was at the 7:30-o'clock position, its upper extremity at the 10-o'clock position. No vessels were seen on the surface of the tu¬ mor, but a glistening white capsule sur-
rounded its external surface. The medial rectus muscle passed over this capsule and was not adherent to the tumor. Beyond the
edges
of the elevated tumor, small, pig¬ mented, satellite deposits were seen in the superficial part of the sciera, but the clini¬ cal characteristics of melanosis oculi
were
not found. The tumor did not transilluminate when light from a fiberoptic transillu-
minator was directed through the pupil. The tumor and the adjacent sclera were dissected from the underlying sclera, which was found to be normal. There was
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
evidence of continuity of the tumor tis¬ with the long posterior ciliary nerve or the choroid. Retina surgery was then com¬ pleted. The patient has been followed-up for 18 months, the right eye remaining white and unaffected. The retina has at¬ tached with no sign of intraocular tumor and visual acuity with correction is 20/100. no
sue
HISTOLÓGICA!. FINDINGS Examination of the scierai lesion showed it to be a cellular blue nevus
with the characteristic composition of sclerotic and cellular areas (Fig 3). The cells were composed of oval and spindle-shaped melanocytes with var¬ iably sized nuclei, some of which showed inconspicuous nucleoli (Fig 4). The cytoplasm had indistinct borders that sometimes extended as long branched processes. The cells con¬ tained various amounts of melanin pigment, and some were filled with heavy pigment. Areas of spindle cells with very little pigment were found to be gathered into interlacing bun¬ dles among the more pigmented scle¬ rotic areas (Fig 5). Vascular channels were present, but mitoses and atypia were absent. Serial sections failed to show any continuity with other struc¬ tures, and the underlying scierai fi¬ bers were free of tumor. COMMENT
The differential diagnosis of a black, elevated mass in the sclera usu¬ ally includes an unsuspected staphyloma with extremely thin sclera or ex¬ traocular extension of an intraocular malignant melanoma. It is now ap¬ parent from our observations that a cellular blue nevus may exist in the superficial part of the sclera and should therefore be considered when a black mass is encountered in the sclera. Cellular blue nevus is probably a variant of blue nevus. It was de¬ scribed by Tieche5 and other au¬ thors."8 Although blue nevus is usu¬ ally considered to be a skin lesion, it has been described in the prostate,9 palate,1" and cervix.11 Its relationship
with cellular blue nevus is important, for the latter has been considered to be a variant of malignant melanoma but is characterized by a good progno¬ sis. Histologically, cellular blue nevus differs from blue nevus by the pres¬ ence of nodules composed of spindle cells in addition to the characteristic sclerotic areas of blue nevus. Rodri¬ guez and Ackerman1- studied 45 cellu¬ lar blue nevi and found among these two extracutaneous lesions, one of the spermatic cord in a 12-year-old boy, and another of the cervix, hymenal ring, and vagina of a 19-year-old girl. More than 50% of the cellular blue nevi in their series were located over the buttock and sacrococcygeal re¬ gion. Other affected areas were the scalp, face, and dorsa of the foot and hand. The patients were followed-up for from six months to more than 17 years, and no recurrences were noted except in one boy with a spermatic cord tumor; this may well have been a nodule missed at the original resec¬ tion. Of the 45 cellular blue nevus lesions reported by Rodriguez and Ackerman, seven were originally misdiagnosed as malignant melanomas. In differentiating cellular blue nevus from malignant melanoma, it is use¬ ful to observe that the borders are not infiltrating, the cellular pattern is never epithelioid, and atypia and mi¬ toses are rare. Typically the nucleoli are simple and small, and necrosis tends to be absent. It differs from simple blue nevus in that it has bun¬ dles of spindle cells showing a biphasic pattern in addition to the
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
sclerotic arrangement of melanocytes and melanophores. Cellular blue nevus is thought to be of neural origin11 and can also arise from proliferating and transformed Schwann cells.14 This study was supported by Public Health Service grant EY-00227 from the National Eye Institute, National Institutes of Health.
References 1. Johnson B: Ocular combined nevus: Report of a case of scleral blue nevus associated with a choroidal nevus. Arch Ophthalmol 83:594-597, 1970. 2. Scheie HG, Yanoff M: Pseudomelanoma of the ciliary body: Report of a patient. Arch Ophthalmol 77:81-83, 1967. 3. Hagler W, Brown C: Malignant melanoma of the orbit arising in a nevus of Ota. Trans Am Acad Ophthalmol Otolaryngol 70:817-822, 1966. 4. Boniuk M: Unusual orbital tumor. Read before the meeting of the Verhoeff Society, Washington, DC, April 22-23, 1974. 5. Tieche M: Uber benign malanome ("chromatophorome") der Haut "blaue naevi." Virchows Arch Pathol Anat 186:212-229, 1906. 6. Webster JP, Stevenson TW, Stout AP: The surgical treatment of malignant melanoma of the skin. Surg Clin North Am 24:319-339, 1944. 7. Dorsey CS, Montgomery H: Blue nevus and its distinction from morphian spot and the nevus of Ota. J Invest Dermatol 22:225-236, 1957. 8. Gartmann H: Neuronaevus bleu Masson\p=m-\ cellular blue nevus Allen. Arch Klin Exp Dermatol 221:109-121, 1965. 9. Simard C, Rogmon LM, Pilorce G: Le probleme du naevus bleu prostatique. Ann Anat Pathol 9:469-474, 1964. 10. Harper JC, Waldron CA: Blue nevus of the palate. Oral Surg 20:145-149, 1965. 11. Goldman RL, Friedman NB: Blue nevus of the uterine cervix. Cancer 20:210-214, 1967. 12. Rodriguez HA, Ackerman LV: Cellular blue nevus: Clinicopathologic study of forty-five cases. Cancer 21:393-405, 1968. 13. Allen AC, Spitz S: Malignant melanoma. Cancer 6:1-45, 1953. 14. Masson P: Neuro-nevi "bleu." Arch Vecchi Anat Pat
14:1-28, 1950.
R.
Smith, MD, Robert J. Brockhurst, MD
\s=b\ An isolated cellular blue nevus of the sclera was discovered and removed during a retinal detachment operation. Such benign tumors may be misdiagnosed clinically as an extension of a melanoma of the choroid or histologically as melanoma.
(Arch Ophthalmol 94:618-620, 1976)
Boniuk1 reported a similar scierai lesion with a two-year fol¬
Society,
low-up period.
This paper reports the occurrence of a large, isolated, cellular blue nevus that was noted incidentally in the sclera at the time of retinal detach¬ ment surgery.
REPORT OF A CASE
melanotic lesions involv¬ ing only the sclera have not been described until recently. Previous re¬ ports of melanotic lesions of the sclera indicate that they are usually associated with other melanotic le¬ sions. For example, Johnson1 has re¬ ported a blue nevus of the sclera that was associated with an underlying choroidal nevus. Scheie and Yanoffdescribed a patient with a melanocytoma of the ciliary body with involve¬ ment of the overlying sclera. Hagler and Brown' reported an orbital ma¬ lignant melanoma arising in a nevus of Ota, the sclera showing dendritic melanocytes characteristic of a blue nevus. We could find no report of an isolated cellular blue nevus of the sclera in the literature; however, when our specimen was presented at the 1974 meeting of the Verhoeff
Isolated
50-year-old
man was examined be¬ of loss of vision in the left eye of four weeks' duration. Past ocular history disclosed no notable eye disorders, the medical review indicated that his general health was excellent, and his only surgical experience had been a herniorrhaphy ten
A
cause
Fig 1 .—Black gery.
pigmented
mass
in sclera discovered at time of retinal detachment
Submitted for publication Dec 18, 1974. From the Eye Pathology Laboratory and Retina Service, Massachusetts Eye and Ear Infirmary, and the Department of Retina Research of the Eye Research Institute, Retina Foundation, Boston.
Reprint requests
to
years previously. On examination, corrected visual acuity measured 20/20 in the right eye and hand movements at four feet in the left eye. In¬ traocular pressure measured 18 mm Hg in the right eye and 14 mm Hg in the left. Biomicroscopic examination of the ante¬ rior segments of the eye revealed no signs of inflammation, but slight nuclear scle¬ rosis was found in each lens. The right fundus showed no important abnormality. Examination of the left fundus showed retinal detachment, including the macula, extending from the 9-o'clock to the 6o'clock positions moving clockwise as a re¬ sult of a dialysis that extended from the 1o'clock to the 4-o'clock positions. The retina was attached in the lower nasal quadrant.
Ophthalmic Library, Eye
Research Institute of the Retina Foundation, 20 Staniford St, Boston, MA 02114 (Dr Brockhurst).
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
sur¬
location of black mass under medial measured 7x15 mm and was elevated 2 mm.
Fig 2 (left).—Sketch showing rectus;
mass
Fig 3.—Tumor cells with biphasic appearance comprised of sclerotic areas (Fig 4) and cellular areas (Fig 5) (hematoxylin-eo¬
sin, original magnification x40).
4 (left).—Sclerotic areas similar to blue nevus with melanocytes and melanophages (hematoxylin-eosin, original magnifi¬ cation 1,000).
Fig
5.—Whorls of spindle cells with prominent nuclei and small nucleoli with scanty cytoplasm (hematoxylin-eosin, original mag¬ nification x250).
Fig
No abnormalities were observed in the cho¬ roid or pars plana nasally. At the time of retinal detachment sur¬ gery, a black tumor mass was seen in the sclera (Fig 1 and 2). The tumor measured 15 mm equatorially and 7 mm anteroposteriorly and was elevated 2.0 mm. Its anterior edge was 14 mm behind the limbus at the equator. Its lower extremity was at the 7:30-o'clock position, its upper extremity at the 10-o'clock position. No vessels were seen on the surface of the tu¬ mor, but a glistening white capsule sur-
rounded its external surface. The medial rectus muscle passed over this capsule and was not adherent to the tumor. Beyond the
edges
of the elevated tumor, small, pig¬ mented, satellite deposits were seen in the superficial part of the sciera, but the clini¬ cal characteristics of melanosis oculi
were
not found. The tumor did not transilluminate when light from a fiberoptic transillu-
minator was directed through the pupil. The tumor and the adjacent sclera were dissected from the underlying sclera, which was found to be normal. There was
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
evidence of continuity of the tumor tis¬ with the long posterior ciliary nerve or the choroid. Retina surgery was then com¬ pleted. The patient has been followed-up for 18 months, the right eye remaining white and unaffected. The retina has at¬ tached with no sign of intraocular tumor and visual acuity with correction is 20/100. no
sue
HISTOLÓGICA!. FINDINGS Examination of the scierai lesion showed it to be a cellular blue nevus
with the characteristic composition of sclerotic and cellular areas (Fig 3). The cells were composed of oval and spindle-shaped melanocytes with var¬ iably sized nuclei, some of which showed inconspicuous nucleoli (Fig 4). The cytoplasm had indistinct borders that sometimes extended as long branched processes. The cells con¬ tained various amounts of melanin pigment, and some were filled with heavy pigment. Areas of spindle cells with very little pigment were found to be gathered into interlacing bun¬ dles among the more pigmented scle¬ rotic areas (Fig 5). Vascular channels were present, but mitoses and atypia were absent. Serial sections failed to show any continuity with other struc¬ tures, and the underlying scierai fi¬ bers were free of tumor. COMMENT
The differential diagnosis of a black, elevated mass in the sclera usu¬ ally includes an unsuspected staphyloma with extremely thin sclera or ex¬ traocular extension of an intraocular malignant melanoma. It is now ap¬ parent from our observations that a cellular blue nevus may exist in the superficial part of the sclera and should therefore be considered when a black mass is encountered in the sclera. Cellular blue nevus is probably a variant of blue nevus. It was de¬ scribed by Tieche5 and other au¬ thors."8 Although blue nevus is usu¬ ally considered to be a skin lesion, it has been described in the prostate,9 palate,1" and cervix.11 Its relationship
with cellular blue nevus is important, for the latter has been considered to be a variant of malignant melanoma but is characterized by a good progno¬ sis. Histologically, cellular blue nevus differs from blue nevus by the pres¬ ence of nodules composed of spindle cells in addition to the characteristic sclerotic areas of blue nevus. Rodri¬ guez and Ackerman1- studied 45 cellu¬ lar blue nevi and found among these two extracutaneous lesions, one of the spermatic cord in a 12-year-old boy, and another of the cervix, hymenal ring, and vagina of a 19-year-old girl. More than 50% of the cellular blue nevi in their series were located over the buttock and sacrococcygeal re¬ gion. Other affected areas were the scalp, face, and dorsa of the foot and hand. The patients were followed-up for from six months to more than 17 years, and no recurrences were noted except in one boy with a spermatic cord tumor; this may well have been a nodule missed at the original resec¬ tion. Of the 45 cellular blue nevus lesions reported by Rodriguez and Ackerman, seven were originally misdiagnosed as malignant melanomas. In differentiating cellular blue nevus from malignant melanoma, it is use¬ ful to observe that the borders are not infiltrating, the cellular pattern is never epithelioid, and atypia and mi¬ toses are rare. Typically the nucleoli are simple and small, and necrosis tends to be absent. It differs from simple blue nevus in that it has bun¬ dles of spindle cells showing a biphasic pattern in addition to the
Downloaded From: http://archopht.jamanetwork.com/ by a University of Manitoba User on 06/06/2015
sclerotic arrangement of melanocytes and melanophores. Cellular blue nevus is thought to be of neural origin11 and can also arise from proliferating and transformed Schwann cells.14 This study was supported by Public Health Service grant EY-00227 from the National Eye Institute, National Institutes of Health.
References 1. Johnson B: Ocular combined nevus: Report of a case of scleral blue nevus associated with a choroidal nevus. Arch Ophthalmol 83:594-597, 1970. 2. Scheie HG, Yanoff M: Pseudomelanoma of the ciliary body: Report of a patient. Arch Ophthalmol 77:81-83, 1967. 3. Hagler W, Brown C: Malignant melanoma of the orbit arising in a nevus of Ota. Trans Am Acad Ophthalmol Otolaryngol 70:817-822, 1966. 4. Boniuk M: Unusual orbital tumor. Read before the meeting of the Verhoeff Society, Washington, DC, April 22-23, 1974. 5. Tieche M: Uber benign malanome ("chromatophorome") der Haut "blaue naevi." Virchows Arch Pathol Anat 186:212-229, 1906. 6. Webster JP, Stevenson TW, Stout AP: The surgical treatment of malignant melanoma of the skin. Surg Clin North Am 24:319-339, 1944. 7. Dorsey CS, Montgomery H: Blue nevus and its distinction from morphian spot and the nevus of Ota. J Invest Dermatol 22:225-236, 1957. 8. Gartmann H: Neuronaevus bleu Masson\p=m-\ cellular blue nevus Allen. Arch Klin Exp Dermatol 221:109-121, 1965. 9. Simard C, Rogmon LM, Pilorce G: Le probleme du naevus bleu prostatique. Ann Anat Pathol 9:469-474, 1964. 10. Harper JC, Waldron CA: Blue nevus of the palate. Oral Surg 20:145-149, 1965. 11. Goldman RL, Friedman NB: Blue nevus of the uterine cervix. Cancer 20:210-214, 1967. 12. Rodriguez HA, Ackerman LV: Cellular blue nevus: Clinicopathologic study of forty-five cases. Cancer 21:393-405, 1968. 13. Allen AC, Spitz S: Malignant melanoma. Cancer 6:1-45, 1953. 14. Masson P: Neuro-nevi "bleu." Arch Vecchi Anat Pat
14:1-28, 1950.
