|Year : 1991 | Volume
| Issue : 1 | Page : 35-37
Leiomyoma of the orbit
SM Betharia, Roopa Arora, Kamal Kishore, ND Patil
Dr. R.P. Centre For Ophthalmic Sciences, All India Institute Of Medical Sciences, Ansari Nagar, New Delhi - 110 029, India
S M Betharia
Dr. R.P. Centre For Ophthalmic Sciences, All India Institute Of Medical Sciences, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
A rare case of leiomyoma located anteroinferiorly in the orbit of a 39-year-old male patient is reported. The histopathogenesis and management is discussed.
|How to cite this article:|
Betharia S M, Arora R, Kishore K, Patil N D. Leiomyoma of the orbit. Indian J Ophthalmol 1991;39:35-7
| Introduction|| |
Leiomyoma, a benign tumour arising from the smooth muscle cells, is one of the rare tumours of the orbit. Only 17 case ,,,,,,,,,,,,,, have been reported so far since initial description of the condition by Lodato  in 1896. The majority of patients have been young adults in their first two decades with no sex predisposition . Most of the tumours have been located deeply in the orbit  with a slight predilection for the inferior and medial quadrants. We are reporting a case of anteriorly placed leiomyoma located in the inferotemporal quadrant of the orbit in a 39-year-old male patient.
| Case report|| |
A 39-year-old man presented to us with complaints of a painless progressive swelling in the temporal part of the left lower lid of two years duration and binocular diplopia for the past 4 months. On examination there was a firm, non-tender nodular mass involving the lateral two-thirds of the left lower lid with superomedial dis≠placement of the globe [Figure - 1]. The mass was not at≠tached to the skin, orbital margin or sclera. The ocular movements were restricted in down gaze. The rest of the eye was normal. The other eye and the general physical examination were within normal limits.
Plain radiograph showed an increased soft tissue den≠sity in the left lower orbit. On echography, the mass was solid with low to medium internal reflectivity spikes and poor sound transmission. Orbital computerised tomog≠raphy demonstrated a well-demarcated homogeneous mass of high attenuation value separate from the muscles and optic nerve. No bony erosion or destruction was noted.
Transcutaneous anterior orbitotomy was done. The in≠cision was placed near the inferior orbital margin and the mass was removed in-toto by blunt dissection. The postoperative course was un3ventful. Good cosmetic and functional results were achieved [Figure - 2]. There was no tropia or limitation of ocular movements.
| Pathologic findings|| |
The gross appearance showed a well-encapsulated oval mass
4 Cm x 3.5 cm x 2.5 cm in dimensions, firm in consistency, with a nodular external surface. The cut surface was greyish-white in colour and demonstrated a whorled pattern [Figure - 3].
Microscopic Examination : The tumour was composed of bundles of spindle shaped cells arranged in a whorled patterns [Figure - 4]. The nuclei were elongated with blunted ends with scant mitoses [Figure - 5]. A large number of dilated capillaries were seen throughout the tumour On Masson's trichrome staining, the cells revealed intense cytoplasmic fuchsinophilia without any cross-striations. The diagnosis of leiomyoma was made.
| Discussion|| |
Leiomyomas are extremely rare in the orbital region. They usually develop in young individuals in the first two decades of life. Isolated cases have been described in a 5-year-A child , and in adults aged 34 years , 41 years , and 53 years . Most of these tumours have been located deep in the orbit . Only one patient, a 17-year-old boy had an anteriorly located tumour in the medial canthal area . There seems to be a slight predilection for the inferior and medial aspects of the orbit . Clinically they present as painless proptosis or displacement of the globe progressing slowly over several months or years . Proptosis is usually less than 5 mm on initial presentation. Symmetric enlargement of the orbit may be seen on radiography due to the longstanding nature of the condition. The tumour is best diagnosed on CT scan which demonstrates a well-defined, round to oval circumscribed mass with moderate contrast enhancement . Orbital CT, how≠ever, cannot differentiate leiomyoma from other solid circumscribed benign tumours. The role of MR[ as a diagnostic modality is not yet fully established 
Histological features include bundles of spindle shaped cells arranged in whorls, elongated but blunt-tipped "cigar shaped" nuclei, retraction of cytoplasm around the nucleus, cytoplasmic eosinophilia, and, a large num≠ber. of dilated capillaries. ,,]18] Red reaction is seen on Masson's trichrome staining indicating a myogenic origin. Non-striated cytoplasmic filaments may be seen on trichrome and P.T.A.H. staining ,sub ..Neurofibroma, fibrous histiocytoma, schwannoma and amelanotic melanoma may give similar appearance on routine haematoxylin-eosin staining .However, the nuclei of leiomyoma cells have a characteristic cigar shape with blunted ends in contrast to the pointed ends of the nuclei in fibroblastic proliferation and comma shaped or wavy nuclei of Schwann cell tumours . Electron microscopy is required to establish the diagnosis in doubtful cases. Leiomyoma is characterised by a well-defined base≠ment membrane, numerous surface-connected vesicles and intercellular cytoplasmic filaments with fusiform densities ,,.
Vascular smooth muscle cells in the orbit are currently believed to be responsible for the histogenesis of this tumour ,,. Some of the tumours could also arise from Muller's muscle in the upper lid. There appears to be a spectrum of tumours ranging from vascular leiomyomas with a large component of capillaries , to rather solid smooth muscle tumours that do not feature a conspicuous vascular component . Pericyte is a pos≠sible source of origin for all these tumours. Electron microscopic studies have demonstrated that the pericyte shows several evolutionary states ranging from the poorly differentiated mesenchymal cell of capillary walls to the highly differentiated smooth cell of venular walls .
Treatment consists of complete surgical excision. A ring of surrounding normal tissue should also be removed because of multiple lobulations of the tumour . Incom≠plete excision has resulted in local recurrences ,, although the prognosis seems to be excellent  even after local recurrence. Leiomyomas are radioresis≠tant . A case of fatal radiation induced sarcoma has been described following radiotherapy for recurrent orbital leiomyoma with intracranial extension 
Our case is significant because it represents a primary orbital leiomyoma at an uncommon location in a middle aged patient. Complete surgicthl excision resulted in cure. There hasn't been any recurrence during the past 4 years of follow-up.
| References|| |
Lodato, G., Fibromyoma dell'orbita, Arch. Ottal., 1896, 4, 120-129.
DeQuervain, F., Leiomyoma der Orbita, Schweiz. Med. Wochenschr.. 1920.1, 628.
Siegrist. Klin. Monatsblt. Augenheilkd., 1923, 71, 233.
Shoda, M., Tumoren der Orbita, Albrecht Von Graefes Arch. Klin. Exp. Ophthalmol., 1925, 347-375.
Cattaneo, .. Leiomyoma e fibroleiomyoma dell' orbita, Atti. Cong. Oftal. Ital., 1925, 318-326.
Lewis, P.M., Leiomyoma of orbit, Abstracted. Am.J. Ophthalmol., 1938, 21, 788-789.
Wright. R.E.. "Selected pictures of extraocular affections from the Museum of the Government Ophthalmic Hospital, Madras, India, Government Press. 1938, p 10.
Nath, K., Shukla, B.R., Orbital leiomyoma and its origin, Br. J. Ophthalmol., 1963, 47, 369-371.
Wolter, J.R., Hemangio-leiomyoma of the orbit, Eye Ear Nose Throat Mo.. 1965. 44, 42-46.
Henderson, J.W., Harrison, E.G.Jr., Vas-ular Leiomyoma of the orbit Report of a case, Trans. Am. Acad. Ophthalmol. Otolaryngol., 1970, 74, 970-974.
Jakobiec, F.A., Jones, I. S., TAnnenbaum, M., Leiomyoma : An unusual tumour of the orbit, Br. J. Ophthalmol., 1973. 57. 825-831.
Jakobiec, F.A., Howard, G.M., Rosen, M, et al, Leiomyoma and leiomyosarcoma of orbit, Am.J. Ophthalmol., 1975. 80.1028-1042.
Sanborn, G.E., Vatenzuela, R.E., Green. W.R., Leiomyoma of the orbit. Am. J. Ophthalmol. 1979, 87, 371-375.
Neetens, A., Smet, H., Orbital Leiomyoma, Bull. Soc. Beige. Ophthalmol., 1984, 210, 73-77.
Vigstrup. J., Glenthi, A., Leiomyoma of the orbit. Acta Ophthalmol.. 1982. 60, 6. 992-997.
Saga. T.. Takeuchi. T.. Tagawa. Y., Orbital leiomyoma accompanied by orbital pseudotumour, 1982, Q6. 175-182.
Shields, J.A., Diagnosis and management of orbital tumours, W.B. Saunders, 1989. 252-255. 1
Jakobiec, F.A., Jones, I.S.. Mesenchymal and fibro-osseous tumours. in Jones, I.S., Jakobiec. F.A., (eds.) Disease of the orbit. Harper & Row. Second edition. 1979. 473-477.
Meyer, S.L.. Fine, B.S.. Font. R.L., Zimmermann, L.E., Leiomyoma of ciliary body : Electron microscopic verification, Am. J. ophthalmol.. 1968. 66.1061-1068.
Rhodin, J., Ultrastructure of mammalian venous capillaries, venules and small collecting veins, J. Ultrastruct. Rev.. 1968. 25, 452.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
|This article has been cited by|
||Orbital leiomyoma: A case report with clinical, radiological and pathological correlation
| ||Merani, R., Khannah, G., Mann, S., Ghabrial, R. |
| ||Clinical and Experimental Ophthalmology. 2005; 33(4): 408-411 |
||Leiomyoma of the orbit and periocular region: A clinicopathologic study of four cases
| ||Arat, Y.O., Font, R.L., Chaudhry, I.A., Boniuk, M. |
| ||Ophthalmic Plastic and Reconstructive Surgery. 2005; 21(1): 16-22 |
||Orbital leiomyoma: Report of a case and review of the literature
| ||GŁndŁz, K., GŁnalp, I., Erden, E., Erekul, S. |
| ||Survey of Ophthalmology. 2004; 49(2): 237-242 |
||Orbital apex leiomyoma with intracranial extension
| ||Kulkarni, V., Rajshekhar, V., Chandi, S.M. |
| ||Surgical Neurology. 2000; 54(4): 327-330 |
||Myolipoma of the eyelid
| ||Sharara, N., Lee, W.R., Weir, C. |
| ||Graefeśs Archive for Clinical and Experimental Ophthalmology. 1998; 236(8): 630-634 |