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Conjunctival angiomyxoma—rare but needs observation

Published:September 22, 2017DOI:https://doi.org/10.1016/j.jcjo.2017.07.005
      Angiomyxomas are benign mesenchymal tumours that rarely involve periocular structures. The age at which patients present with them ranges from 4 to 60 years.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      These tumours commonly involve the pelvic area in females. Only a few cases of conjunctival and orbital angiomyxoma have been described in the literature.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      • Liu W.
      • Lin J.
      • Yu X.
      [Clinical and histopathological features of conjunctival myxoma and angiomyxoma.] [Article in Chinese.].
      • Albert D.L.
      • Brownstein S.
      • Codère F.
      Subconjunctival angiomyxoma.
      When they involve the periocular structure, they need special attention both during and after surgery. Here we describe the clinical features, diagnosis, and management in a case of conjunctival angiomyxoma.
      A 48-year-old male presented with a painless, gradually increasing, pinkish, conjunctival mass in the superior aspect of his left eye that had been present for 5 months (Fig. 1A). On examination, the patient was found to have visual acuity of 20/40 OU, with grade I nuclear sclerosis. Findings for the rest of the anterior segment and the fundus were normal, and extraocular motility was normal. Slit-lamp examination of the eye showed a pink-to-red, fleshy, uniform conjunctival mass in the left superior bulbar conjunctiva, extending to the fornix, without any corneal infiltration.
      Fig. 1
      Fig. 1(A) The patient presented with a solitary pinkish, cystic conjunctival mass involving the superotemporal aspect of his left eye. (B) Examination under light microscopy revealed myxoid changes with spindle-shaped and stellate-shaped cells (hematoxylin–eosin stain; original magnification ×200). (C) Higher magnification showed the same changes, with capillaries (hematoxylin–eosin stain; original magnification ×400). (D) After 14 months, the conjunctiva appeared healthy, without any tumour recurrence.
      B-scan ultrasonography of the orbit showed a well-defined mass without any intraocular extension. Similarly, contrast-enhanced computed tomography of the orbit revealed a well-defined, heterogeneously enhancing, mass lesion confined to the epibulbar surface without postseptal extension. Because of these findings, the initial differential diagnosis consisted of lymphoma, conjunctival myxoma, amyloidosis, and angiomyxoma. To confirm the diagnosis, we performed an incision biopsy under local anaesthesia, which showed findings consistent with angiomyxoma of the conjunctiva. We evaluated the patient for Carney’s complex, which showed normal skin and cardiac function without any evidence of cardiac myxoma.
      The tumour margins were marked, including 2 mm of surrounding healthy conjunctiva on the nasal and temporal aspects, inferiorly up to the limbus and superiorly up to the fornices. The patient mainly had infiltration along the horizontal plane along the episcleral plane, so debulking with clear margins on the anterior, lateral, and medial sides was achieved easily. It was slightly difficult to obtain a clear margin on superior, or forniceal, extension. Therefore, we decided, on the basis of previous experience, to avoid residual tissue. The superior extent was excised beyond clinically visible margins with minimal manipulation of the superior rectus muscle and the levator palpebrae superioris complex. The visible mass was excised in total with complete removal of the fleshy tissue; the bare scleral surface was grafted with amniotic membrane under local anaesthesia.
      Histopathological examination under light microscopy showed spindle-shaped cells in the myxoid stroma, along with abundant collagen and thin-walled capillaries but without any mitotic cells. The specimen also stained strongly with Alcian blue (Fig. 1B, C). The patient had no recurrence after 14 months of follow-up (Fig. 1D).
      Angiomyxoma of the conjunctiva usually presents as pink, translucent, cystic, or solid mass over the globe. Because they are rare, these tumours are not usually considered in the differential diagnosis. Furthermore, the skill and experience of an ocular pathologist are necessary for making a definite diagnosis. These tumours mostly involve the superior orbit, causing proptosis and globe dystopia.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      However, they may go unnoticed until the patient presents later with recurrence and malignant transformation
      • Jakobiec F.A.
      • Callahan A.B.
      • Stagner A.M.
      • et al.
      Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma.
      and invasion of the surrounding structures.
      • Mishulin A.
      • Lever J.F.
      • Porter W.
      • et al.
      Aggressive glabellar angiomyxoma with orbital extension.
      Our patient presented with a gradually increasing mass in the superior bulbar conjunctiva without any orbital or intraocular extension.
      The diagnoses we considered were those we had encountered in our clinical practice: lymphoma, myxoma, amyloidosis, and angiomyxoma. Incisional biopsy revealed findings consistent with angiomyxoma. Histologically these lesions are characterized by (i) stellate to spindle-shaped cells in a loose myxoid stroma along with collagen but without any atypical/mitotic cells; (ii) more abundant blood vessels than usually occur in myxomas, ranging from small capillaries to thick-walled vessels with poorly defined infiltrative margins; and (iii) positive findings on immunohistochemical staining for vimentin, CD34, and smooth-muscle actin and negative findings for S-100 desmin and cytokeratin.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      • Liu W.
      • Lin J.
      • Yu X.
      [Clinical and histopathological features of conjunctival myxoma and angiomyxoma.] [Article in Chinese.].
      • Albert D.L.
      • Brownstein S.
      • Codère F.
      Subconjunctival angiomyxoma.
      The other variant is aggressive angiomyxoma, which is mainly seen in the pelvic region in female patients, with abundant thick-walled blood vessels in the setting of myxoid stroma; this variant shows a tendency to recur but with metastasis or tumour-related death.
      • Fetsch J.F.
      • Laskin W.B.
      • Lefkowitz M.
      • et al.
      Aggressive angiomyxoma: a clinicopathologic study of 29 female patients.
      Hidayat et al. studied 3 cases of orbital angiomyxoma; 2 of them involved patients younger than 10 years, and the tumours were locally aggressive and recurred within a year.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      Similarly, Bajaj et al. described 4 cases of orbital angiomyxoma in which the tumours occurred in the patients’ third to fourth decade of life, with recurrence in 1 patient after 6 months.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      Management of these tumours involves evaluation by ultrasonography and computed tomography to determine the extent of periocular involvement. The ideal approach involves complete surgical excision, along with the inclusion of surrounding normal margins to avoid local recurrence.
      • Hidayat A.A.
      • Flint A.
      • Marentette L.
      • et al.
      Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
      • Bajaj M.S.
      • Mehta M.
      • Kashyap S.
      • et al.
      Clinical and pathologic profile of angiomyxomas of the orbit.
      • Liu W.
      • Lin J.
      • Yu X.
      [Clinical and histopathological features of conjunctival myxoma and angiomyxoma.] [Article in Chinese.].
      In our patient, the mass was well localized to the conjunctiva and tenon, limited by the fornices without any underlying scleral invasion, because complete excision with a clear 2-mm margin was possible. This case stresses the importance of considering angiomyxoma in the differential diagnosis in cases of conjunctival and orbital disease.

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