Primary orbital Alveolar soft part sarcoma papers

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Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The incidence of orbital involvement is the highest in ASPS arising in the head and neck region. However, limited information is available regarding its clinical characteristics. The present study presents the clinical manifestations, imaging findings, pathological features, treatment strategies and prognosis records of 8 cases of orbital ASPS over the last 15 years in a single hospital, along with a review of the literature. Included were 3 male and 5 female patients, with the median age at presentation being 9.5 years. The mean average largest tumour diameter was 3.6 cm. A total of 5 patients underwent surgical excision of the tumour, with 2 undergoing orbital exenteration and 1 undergoing partial orbital exenteration. In total, 6 patients received postoperative radiotherapy and 2 received chemotherapy. Upon follow-up, 6 patients were doing well with no evidence of recurrence or metastasis. Local recurrence developed in 2 patients, of whom 1 succumbed following withdrawal from treatment. According to the present series and the cases mentioned in the literature, orbital alveolar soft part sarcoma has characteristics distinct from those of alveolar soft part sarcoma which arises in other locations. Orbital alveolar soft part sarcoma presents itself in a younger population with a shorter course of disease, smaller tumour size, improved prognosis, a marked association with the extraocular muscles and with the Ki-67 proliferation index possibly associated with prognosis of the disease.

Keywords: alveolar soft part sarcoma, extraocular muscle, orbit, pathology, prognosis

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738708/


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Orbital alveolar soft part sarcoma: case report and literature review
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Post Sun Feb 16, 2020 8:52 am

Abstract
Alveolar soft part sarcoma (ASPS) is a malignant soft part tissue tumour of uncertain phenotypic expression that mainly affects young adults and children and represents less than 1% of all sarcomas.14 There are only 63 cases of primary orbital ASPS reported in the PUBMED, none from Canada, with a female preponderance and a predilection for affecting the left eye.13
Herein we describe an unusual case of orbital ASPS pri- mary of the orbit in a 31-year-old female with a previous his- tory of breast cancer.
Histopathologically, ASPS has a distinguished morphol- ogy composed of monotonous cells that have a vesicular nucleus with a small nucleolus and abundant granular eosinophilic cytoplasm. Individual cells are separated by thin-walled vascular channels lined by flattened endothe- lial cells. Fibrous septa divide the tumour in a homoge- neous lobular way.15 ASPS is characterized by a specific chromosomal alteration, t(X:17)(p11:q25), resulting in fusion of the TFE3 with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25, and by immunohistochem- istry, most of the cells show positivity for TFE3, which is considered a very sensitive and specific maker for ASPS.1 In conclusion, we report the first orbital ASPS from Can- ada. This tumour can be misdiagnosed due to very low incidence and the absence of characteristic clinical find- ings. Microscopic examination and immunohistochemis- try are crucial for differentiation from other types of tumors. Aggressive surgical resection seems to be the most appropriate treatment for a better survival.
https://www.canadianjournalofophthalmol ... 0833-0/pdf


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Re: Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature
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Post Sun Feb 16, 2020 9:09 am

Orbital Alveolar Soft Part Sarcoma

Kaustubh Mulay, MD1, Sonal S. Chougule, MD2, Santosh G. Honavar, MD2
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https://doi.org/10.1016/j.ophtha.2016.03.019

“A 7-year-old boy presented with progressive left upper eyelid swelling of 2-months duration (Fig 1A). Computed tomography showed a hyperdense, orbital mass extending to the apex and indenting the globe (Fig 1B). Microscopically, the tumor comprised of organoid nests of polygonal to round cells separated by delicate capillary-sized vascular channels (Fig 1C). Cellular dyscohesion within nests resulted in pseudoalveolar pattern (Fig 1C). Periodic acid-Schiff-positive, diastase resistant crystalline structures (Fig 1D, arrow) and granules (Fig 1D, circle) were evident along with tumor emboli. The patient was treated with surgical excision and postoperative external beam radiation.”