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Year : 1986  |  Volume : 34  |  Issue : 1  |  Page : 67-68

Alveolar soft-part sarcoma of the orbit

Department of Pathology, L. T. M. Medical College & Hospital, Sion, Mumbai, India

Correspondence Address:
Cusuma M Chodankar
11/34 Kamana society Bldg, No 4, Prabhadevi Mumbai 400 028
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Source of Support: None, Conflict of Interest: None

PMID: 3443501

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How to cite this article:
Chodankar CM, Pandit SP, Joshi MG, Deodhar K P. Alveolar soft-part sarcoma of the orbit. Indian J Ophthalmol 1986;34:67-8

How to cite this URL:
Chodankar CM, Pandit SP, Joshi MG, Deodhar K P. Alveolar soft-part sarcoma of the orbit. Indian J Ophthalmol [serial online] 1986 [cited 2022 Oct 7];34:67-8. Available from: https://www.ijo.in/text.asp?1986/34/1/67/26343

Primary alveolar soft-part sarcoma of the orbit is an unusual neoplasm. Christopher­son et al[1] first reported 12 cases of a distinc­tive soft tissue tumour that they designated alveolar soft-part sarcoma. Isolated case reports of alveolar soft-part sarcoma of the orbit have appeared in the ophthalmic litera­ture[2],[3],[4]. sub To the best of our knowledge only 25 cases of orbital alveolar soft part sarcomas are reported uptil now. The case is being reported for its rarity.

  Case report Top

A 15 year old boy presented with the complaint of swelling of left lower eyelid for 1 month's duration. On examination the lower eyelid showed a growth about 2 X 1 X 1 cm. The swelling was well circumscribed, compressible and soft in nature which led the clinicians to the diagnosis of haemangioma. Rest of the occular and general examination was unremarkable. The tumor was surgi­cally excised.

Pathologic Findings

The formalin fixed mass was 2 X 1 X 1cm. The mass was reddish-brown in colour and appeared to be encapsulated. C/S was tan to reddish brown and well vascularised. Histopathology revealed a striking uniformity and pseudoalveolar pattern composed of nests of cells separated by thin fibrous septae.

The cells were large and round to polyhedral with finely granular, acidophilic cytoplasm and distinct cell boundaries. The paracentral nuclei were round to oval and vesicular [Figure - 1] Reticulin stain showed reticulin fibres clearly outlining nests of cells, reveal­ing alveolar arrangement. No reticulin fibres were seen in between individual tumour cells. [Figure - 2]. PAS stain showed presence of PAS-positive diastase-resistant crystalline structures. [Figure - 3].

  Discussion Top

The histogenesis of alveolar soft-part sarcoma remains controversial. It is thought to originate from neural tissue, striated muscle tissue, and paraganglia. It is confus­ed histologically with nor-chromaffin para­ganglioma, granular cell myoblastoma, vaso­formative tumor (mainly angiosarcoma), metastatic carcinoma, alveolar rehabdomyc sarcoma, and amelanotic malignant mela­noma with alveolar pattern. Pathognomonic findings in favour of alveolar soft-part sarcoma is the presence of PAS-positive, diastase-resistant crystalline structures within the cytoplasm of the tumour cells

Lieberman et al. reported 53 cases of alveolar soft-part sarcoma of soft tissues. In their study they found a preponderance of cases among women as well as a significant laterality for the right side of the body[5]. Font et al reported 17 cases of alveolar soft-part sarcoma of the Orbit in which they noted female sex preponderance but no significant laterality since both orbits were equally involved. Our case is a 15 year old boy with the involvement of left lower eyelid. They also noted that prognosis is better in patients less than 20 years of age. Our case has been followed up for only 9 months and until now there is no evidence of recur­rence anywhere.

Font et al have stressed that there is need for long-term follow up studies in order to evaluate the biologic behaviour of these tumours. Surgery provides the best chance of controlling the disease. Local excision indicated especially if the patient is in the first two decades of life. Radiotherapy may be considered as a combined therapeutic modality following surgery[1].

  Summary Top

A rare case of alveolar soft-part sarcoma of the left lower eyelid, has been reported.

  Acknowledgement Top

The authors are thankful to Dr. N.A. Dhabolkar, Dean L.T M. Medical College and Hospital, Sion for permission to publish this paper.

  References Top

Font Ramon L., Jurco Stephen, and Zimmer­man Lorenz F, 1912, Human Pathology 13:569.  Back to cited text no. 1
Grant G D , Shields J A, Flanagan J C, et al 1972, Amer J. Ophthalmol 87:773.  Back to cited text no. 2
Mukherjee P.K, Agrawal S, 1979, Ind. J. Ophthalmol 27:15.  Back to cited text no. 3
Gonzalez- Almaraz G , de Puen S, Tsutsumi V, 1975, Amer J. Ophthalmol 79:606.  Back to cited text no. 4
Lieberman P.H , Foote F.W., Jr, Stewart Fw et al, 1966, JAMA 198:1047.  Back to cited text no. 5


  [Figure - 1], [Figure - 2], [Figure - 3]


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