Case Reports Glial Heterotopia or Ectopic Brain Masquerading as a Dacyrocystocele Mohammad Javed Ali, M.D., F.R.C.S.*, Saurabh Kamal, M.D.*, Geeta K. Vemuganti, M.D.†, and Milind N. Naik, M.D.* Abstract: Glial heterotopia is an extremely rare clinical entity mostly presenting in head and neck region secondary to an abnormal development of an isolated mature central nervous system (CNS) tissue at an abnormal location without any continuity with the brain, spinal cord, or meninges. Its origin is either due to mechanical isolation of glial tissues by the developing cranial bones or secondary to misdirected or sequestered embryonic neuroectodermal stem cells outside the CNS. The authors present an exceptionally rare case of a heterotopic glial nodule masquerading as a dacryocystocele, its radiological features along with a brief clinicopathological correlation.

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acryocystocele is a rare presentation of congenital nasolacrimal duct obstruction and accounts for 0.1% of such cases.1 Clinical diagnosis of congenital dacryocystocele can be usually made by the presence of translucent bluish cystic mass below the medial canthus and associated watering. It is important to differentiate conditions that may mimic a dacryocystocele like encephalocele, dermoid cyst, hemangioma, nasal glioma, and lymphangioma.1–3 Glial heterotopia is an extremely rare choristoma characterized by the presence of an isolated nonteratomatous central nervous system tissue in an extracranial location. The authors present an extremely rare case of a heterotopic glial nodule masquerading as a dacryocystocele, its radiological features along with a brief clinicopathological correlation.

CASE REPORT A 5-month-old baby girl presented with swelling below the medial canthus of OS since birth (Fig. 1A). It was not associated with redness, watering, pain, or breathing problem. Antenatal and postnatal history was unremarkable with normal developmental milestones, and the child was systemically healthy. On examination, visual acuity measured with Teller acuity card was normal in OU. Anterior and posterior segment examination of OU was normal, and regurgitation on pressure over lacrimal sac was absent. A nontender, hard, well-defined mass measuring 10 × 10 mm was present over left side of nasal bridge, approaching lacrimal sac area and partly blunting the medial canthal angle (Fig. 1A). Mass was fairly mobile, separate from overlying normal skin and nonreducible and nontransilluminant. CT scan showed a well-defined heterogenous mass over the nasal bone, slightly away from the lacrimal sac area (Fig. 1B). There was a small underlying bony defect with smooth edges in the lateral nasal wall without signs of erosion or intranasal extension (Fig. 1C). Brain scans were normal with no evidence of intracranial extensions or meningoencepaholcele. Accepted for publication August 28, 2013. *Dacryology Service, and †Ocular Pathology Service, L. V. Prasad Eye Institute, Banjara Hills. Hyderabad, India The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mohammad Javed Ali, M.D., F.R.C.S., Dacryology Service, L.V. Prasad Eye Institute, Road No 2, Banjara Hills, Hyderabad-500034. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000050

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Clinico-radiological differential diagnosis included nasal dermoid or atypical dacryocystocele. Intraoperative irrigation showed lacrimal system to be patent. Endoscopic nasal examination was normal with no intranasal contiguity of the mass (Fig. 1D). A transcutaneous approach revealed the mass as separate from lacrimal sac. After complete excision of the mass, a small bony defect was noted beneath with normal lateral nasal mucosa (Fig. 1E). At 3-month follow up, there was no recurrence, and the infant was doing well. Histopathology. On gross inspection, mass was glossy, ­graywhite, soft to firm, measuring 17 × 12 × 10 mm with no surface hemorrhages (Fig. 2A). Cut section was homogenously ­grayishwhite without areas of hemorrhages or necrosis. Microscopic examination showed circumscribed unencapsulated lesion composed of fibrous connective tissue with scattered mature glial tissues in a fibrillary background (Fig. 2B). Astrocytes were the predominant constituents of the glial tissue appearing as round to oval cells with uniform basophilic nuclei enmeshed in eosinophilic fibrillar network (Fig. 2C). Few multinucleated cells or gemistocytic astrocytes were noted. Immunohistochemical staining with Glial fibrillary acidic protein (GFAP) stain was positive (Fig. 2D). The histopathologic features along with clinical correlation were consistent with the diagnosis of a heterotopic glial nodule.

DISCUSSION Heterotopia or choristoma is the development of normal tissue at an abnormal location. Heterotopic glial tissue is an extremely rare clinical entity presenting in head and neck region secondary to abnormal development of mature central nervous system tissue at an abnormal location with a likely limited growth potential.4,5 It has been reported to occur in nasopharynx, internal auditory canal, intraoral mass, hard palate, and mastoid bone.4,5 Few cases has also been reported in orbit presenting as proptosis and strabismus.6–9 The underlying cause for heterotopic or ectopic glial tissue has been theorized to be related to the abnormality in development of facial and skull bone plates but can also be seen isolated in the form of nodules without any connection to the central nervous system.10 The isolated glial masses are believed to have originated from the misdirected or sequestered embryonic neuroectodermal stem cell rests.9,10 CT scan features described for glial heterotopic masses in the orbits include well-circumscribed hypo- to isodense masses, minimal cystic areas, minimal rim enhancement with adjacent bony scalloping but without erosions.6–9 There are no well-defined or characteristic MRI features. In contrast, both CT scan and MRI in dacryocystoceles shows fluid collection with a thin rim enhancement along the course of lacrimal sac and nasolacrimal ducts.1–3 Histopathological evaluation usually reveals fibrous and vascularized connective tissues with scattered mature glial tissue in a fibrillary background.4–10 The cells are predominantly astrocytes with sparse or absent neurons, and this differentiates it with an encephalocele where abundant neurons will be noted. As the age of the patient increases, the fibrous component of glial heterotopic masses increases significantly with minimal glial tissues. There should be efforts to look for presence of any ependymal cells, meninges, and choroidal plexus since the presence of these helps in differential diagnosis of other conditions like meningoencephaoloceles.4–10 Ophthal Plast Reconstr Surg, Vol. 31, No. 2, 2015

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FIG. 1.  A, Clinical photograph showing a swelling below the left medial canthus. B, CT scan, axial cut, showing a heterogenous mass lesion away from the lacrimal sac area. C, CT scan, axial cut showing the mass lesion with a small bony defect beneath the mass in the lateral nasal wall but no intranasal extension. D, Endoscopic examination of the left nasal cavity showing no evidence of any nasal mass or intranasal extension of the mass. E, Clinical photograph following complete excision of the mass showing edges of the bony defect with normal underlying lateral wall mucosa.

FIG. 2.  A, External photograph of the gross specimen showing a grayish-white mass without any surface hemorrhages. B, Microscopic photograph showing areas of mature glial tissue interspersed among fibrous connective tissue (hematoxylin-eosin ×100). C, Numerous round to oval astrocytes enmeshed in an eosinophilic fibrillary network (hematoxylin-eosin ×400). D, Glial tissue showing positive staining with glial fibrillary acidic protein (GFAP ×400).

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

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Treatment recommended for glial heterotopia is a complete excision of the mass even though there is no malignant or metastatic potential.4–9 There is a controversy in the literature regarding the growth potential of these masses, but many believe that conservative management risks a possible continued growth with its attendant complications depending on the site. However, incomplete excisions are noted to have a recurrence rate of around 10%.6–8 The beneficial effects of radiotherapy are unproven and questionable.6 In conclusion, although extremely rare, glial heterotopic nodule should be kept in the differential diagnosis of a dacryocystocele. The presence of the bony defect in this case gives evidence to the theory of mechanical isolation of glial tissues from the brain by the developing cranial bones.

aerodigestive tract. Herein, the authors present a ­77-yearold woman with a basaloid squamous cell carcinoma over her limbal conjunctiva in the OD.

REFERENCES

A 77-year-old Taiwanese woman had a painless conjunctival mass in the OD that increased in size gradually for many years. On ophthalmic examinations, an elastic, pedunculated mass measuring 9 × 6 × 5 mm in size originating from the limbus with corneal involvement was found (Fig. 1). Other ophthalmic findings were not remarkable. Orbital CT scan demonstrated a soft tissue mass over medial portion of conjunctiva in the OD without orbital involvement or localized lymphadenopathy. Superficial keratectomy was performed to dissect the corneal and limbal component of the lesion; the conjunctival component along with a cuff of normal conjunctival tissue was removed. The histopathologic study (Fig. 2) showed small and crowded basaloid cells with scant cytoplasm and hyperchromatic nuclei. The tumor cells clustered in variably sized lobules and nests with prominent peripheral palisading. There were stromal hyalinosis and some small intercellular cystic spaces with mucin-like materials. Some foci of conventional SCC adjacent to the basaloid component were also seen, which was compatible with BSCC. The additional immunohistochemistry staining for human papillomavirus (HPV) diagnosis were p53 positive and p16 ­negative (Fig. 3A,B), which indicates HPV-negative. Meanwhile, positive staining of p63 and Ki-67 occurred diffusely throughout the tumor mass (Fig. 3C, D), and the ­ ­pattern is suggestive of a rapid growing malignancy. Although further excision and adjuvant mitomycin-C application was suggested, the patient was reluctant to receive

1. MacEwen CJ, Young JD. Epiphora during the first year of life. Eye (Lond) 1991;5(Pt 5):596–600. 2. Mansour AM, Cheng KP, Mumma JV, et al. Congenital dacryocele. A collaborative review. Ophthalmology 1991;98:1744–51. 3. Shashy RG, Durairaj VD, Durairaj V, et al. Congenital dacryocystocele associated with intranasal cysts: diagnosis and management. Laryngoscope 2003;113:37–40. 4. Tambay MC, Rodriguez IZ, Gil YR, et al. Heterotopic neuroglial tissue as a congenital laterocervical mass: a case report. Int J Oral Maxillofac Surg 2009;38:382–4. 5. Aanaes K, Hasselby JP, Bilde A, et al. Heterotopic neuroglial tissue: two cases involving the tongue and the buccal region. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;105:e22–9. 6. Newman NJ, Miller NR, Green WR. Ectopic brain in the orbit. Ophthalmology 1986;93:268–72. 7. Bajaj MS, Kashyap S, Wagh VB, et al. Glial heterotopia of the orbit and extranasal region: an unusual entity. Clin Experiment Ophthalmol 2005;33:513–5. 8. Ghose S, Balasubramaniam ST, Mahindrakar A, et al. Orbital ectopic glial tissue in relation to medial rectus: a rare entity. Clin Experiment Ophthalmol 2005;33:67–9. 9. Kiratli H, Sekeroğlu MA, Tezel GG. Orbital heterotopic glial tissue presenting as exotropia. Orbit 2008;27:165–8. 10. Skelton HG, Smith KJ. Glial heterotopia in the subcutaneous tissue overlying T-12. J Cutan Pathol 1999;26:523–7.

Basaloid Squamous Cell Carcinoma of the Conjunctiva

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asaloid squamous cell carcinoma (BSCC), which commonly arises in the upper aerodigestive tract, is an aggressive variant of squamous cell carcinoma (SCC).1,2 To date, only 1 case of BSCC of the conjunctiva has been reported in the literature.3 Our report presents a patient with conjunctival BSCC, which was recognized and further confirmed with histopathological studies. The patient had no disease recurrence during the 6-month follow-up period.

CASE REPORT

Yu Chang Liu*, Nancy Chen, M.D.†, Yung-Hsiang Hsu, M.D., M.S.‡, Min-Muh Sheu, M.D., M.T.M.†§, and Rong Kung Tsai, M.D., Ph.D.†§ Abstract: Basaloid squamous cell carcinoma is a distinct variant of squamous cell carcinoma, and it is more aggressive and has a poorer prognosis than conventional squamous cell carcinoma. Basaloid squamous cell carcinoma has been reported to arise from many organs, mainly in the upper Accepted for publication September 24, 2013. *Department of Medicine, College of Medicine, Tzu Chi University; Departments of †Ophthalmology and ‡Pathology, Buddhist Tzu Chi General Hospital; and §Department of Ophthalmology and Visual Science, Tzu Chi University, Hualien, Taiwan The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Rong Kung Tsai, M.D., Ph.D., Department of Ophthalmology, Buddhist Tzu Chi General Hospital and Department of Ophthalmology and Visual Science, Tzu Chi University, 707 Sec. 3, Chung Yung Road, Hualien 970, Taiwan. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000056

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FIG. 1. An elastic, pedunculated mass originated from the limbus with corneal invasion in OD.

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Glial heterotopia or ectopic brain masquerading as a dacyrocystocele.

Glial heterotopia is an extremely rare clinical entity mostly presenting in head and neck region secondary to an abnormal development of an isolated m...
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