Case Report This work is licensed under the Creative Commons Attribution 4.0 License. Published by Pacifc Group of e-Journals (PaGe) Angiomyxoma of the Lower Eyelid with Orbital Extension: A Rare Entity and Review of Literature Introduction In 1983, Steeper and Rosai described a series of distinctive soft tissue tumours of the female pelvis and perineum with the microscopic appearance of a spindle or stellate cells in a loose myxoid stroma with prominent thick-walled vessels with non-arborizing pattern and low mitotic activity. They assigned the term ‘aggressive angiomyxoma’ [1] . Allen et al., in 1988 described 30 uncommon dermal and subcutaneous angiomyxoid tumours in 28 patients. Microscopically, there were moderate to sparsely cellular angiomyxoid nodules with scattered small vessels. They coined the term ‘superfcial angiomyxoma’ for such lesions. Angiomyxomas are benign mesenchymal tumours. They rarely involve the orbit and a limited number of cases exist in literature. They can be either superfcial or deep in nature. Both these varieties have been noted in orbit with one case report documenting malignant rhabdoid transformation [2] . They can be locally aggressive, which combined with their gelatinous texture precludes complete surgical removal leading to a high risk of recurrences. These recurrences usually do not cause bony erosions or tissue destruction and therefore can be managed without much morbidity. This study adhered to the tenets of the Declaration of Helsinki, and a written informed consent form was signed by the patient. Case Report A 60-year-old male patient presented to our outpatient department with complaints of swelling in his left lower eyelid and protrusion of the eyeball associated with diminution of vision for the past six months. (Figure 1a) On ophthalmological examination, visual acuity in right eyes was 6/6 on Snellen’s visual acuity chart and in the left eye was light perception with an inaccurate projection of rays in temporal and nasal quadrants. Both upper and lower lid fullness was noted on the left side. On Hertel’s exophthalmometry, the proptosis of the left eye was found to be 13 mm with 3 mm medial dystopia. The swelling was palpable inferiorly from the midpoint of the lower lid, laterally over the lateral canthus and superiorly till beneath the brow. The superior margin could not be felt distinctly. The mass was non-tender, frm in consistency with no palpable pulsations or thrill. It showed resistance to retropulsion with no increase on Valsalva manoeuvre. Bony orbital margins were intact. No lymph nodes were clinically palpable. Extraocular movements were found to be reduced equally in all meridians. On slit-lamp examination, inferior conjunctival prolapse was noted. Pupillary reactions were sluggish, and fundus examination using indirect ophthalmoscopy revealed disc pallor and choroidal folds at the posterior pole. Ophthalmic examination for the right eye revealed no abnormalities. Systemic examination ruled out any other lesions pertaining to Carney’s complex. A clinical differential diagnosis of lymphoma, mesenchymal tumors and orbital metastasis was made. The patient underwent imaging, and on computed tomography, a heterogeneous lesion was noted in the inferotemporal orbit lying in close relation to the globe. (Figure 1c, d). An incisional biopsy was then planned under local infltrative anaesthesia. Sahil Agrawal 1 , Aarush Deora 1 , Sujeeth Modaboyina 1 , Deepsekhar Das 1 *, Mandeep Singh Bajaj 1 and Seema Sen 2 1 Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences New Delhi –110 029, India 2 Department of Ocular Pathology, All India Institute of Medical Sciences, New Delhi –110 029, India ABSTRACT A 60-year-old male sought medical advice for painless progressive swelling in the left eye associated with protrusion of the eyeball and diminution of vision for the past 6 months. A computed tomography scan was done which revealed a heterogeneous lesion in the inferotemporal orbit lying close to the globe without any bony erosions or intracranial extension. Incisional biopsy revealed spindle or stellate cells in a myxoid matrix with abundant thin-walled vessels.On Immunohistochemistry, the tumour was positive for vimentin and SMA. It was negative for Desmin and S-100. A diagnosis of superfcial angiomyxoma of the eyelid with orbital extension was thus made. Surgical excision under general anaesthesia was planned and the mass removed in toto. The histopathological examination of the same revealed fndings similar to the incision biopsy. The patient is being followed up with no recurrences till date. Keywords: Angiomyxoma, orbital tumor, proptosis, lid tumor, Carneys complex DOI: 10.21276/APALM.2981