Orbital alveolar soft part sarcoma: case report and literature review

Abstract

Orbital alveolar soft part sarcoma: case report and literature review 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. 1À4 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. 1À3 Herein we describe an unusual case of orbital ASPS primary of the orbit in a 31-year-old female with a previous history of breast cancer.A 31-year-old female presented a mild proptosis of the right eye. After magnetic resonance imaging (MRI), the preliminary diagnosis was a metastasis of her previous breast carcinoma. At age 26 years, she was diagnosed with a grade 3 invasive ductal carcinoma of the right breast, stage T2 N2.Genetic screenings were negative.The first MRI showed a solitary lesion in the right orbit measuring 16 £ 15 £ 14 mm. The location was intramuscular inferior right, and the lesion was well circumscribed and reconfigured the orbit's floor. It caused swelling of the superior right portion of the optic nerve. It was iso-intense T2 and T1 with an enhanced homogeny after gadolinium injection.Two months later, a second MRI showed an increase in size of the lesion, measuring 18 £ 18 £ 17 mm (Fig. 1A). Biopsy demonstrated a tumour composed of uniform polygonal epithelioid cells in a solid and alveolar architecture outlined by delicate sinusoidal vascular channels. The cells had abundant granular eosinophilic cytoplasm and vesicular nuclei with large prominent nucleoli and a very low mitotic rate with no necrosis (Fig. 1C and D). Periodic acidÀSchiff with diastase stains revealed needle-shaped crystals (Fig. 1E). Immunohistochemical stains performed demonstrated tumour strong positivity for Fig. 1-A, CT scan of the orbit showed a well-circumscribed lesion in the right orbit. B, Microscopic examination shows a monotonous and uniform tumour with a sinusoidal architecture (hematoxylin-eosin [H&E], 20 £). C, The tumour is composed of monomorphic cells with vesicular nuclei and a central nucleoli, eosinophilic and granular cytoplasm, with rare cellular pleomorphism and rare mitotic figures (H&E, 400 £). D, Vascular invasion was identified at the periphery of the tumor (H&E, 40 £). E, Needle-shaped crystals were identified in the cytoplasm of some of the tumoural cells by periodic acid-Schiff (PAS) with diastase stain (PAS diastase, 400 £). F, The tumoural cells show a diffuse nuclear positivity for TFE3 by immunohistochemistry (TFE3, 100 £).

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

“…There were 39 males and 45 females. [ 1 2 5 7 8 9 ] Our series showed a male: female ratio of 4:1, contrary to that reported in a majority of the studies. The most common signs were reduced visual acuity, an abaxial proptosis, and restricted extraocular movements.…”

“…[ 1 ] Numerous case reports have shown that ASPS is a highly vascular tumor, as seen in three of our cases. [ 5 8 ] This is seen as flow voids on MRI, and in such cases, a preoperative DSA can delineate the vascular supply and major feeder vessels of the tumor. It also guides in planning preoperative embolization to reduce vascularity and intraoperative bleeding, which facilitates a smooth and complete tumor excision.…”

“…Exenteration, a radical surgery that at times is unavoidable was done for 20 (27%) out of the 74 cases reported. [ 1 2 5 7 8 9 ] Embolization before exenteration was done for one patient owing to significant bleeding encountered during an incisional biopsy by Kim et al . [ 15 ] In our series, all three patients who underwent embolization had successful removal of the entire tumor with very minimal intraoperative bleeding.…”

“…deBarros reviewed 49 cases of orbital ASPS with a mean follow-up of 71 months and found that nine cases (18%) had distant metastases and six patients (12%) died of the disease. [ 8 ] The most common sites for metastasis noted by them were the lung (12%), followed by the brain (5%), and the liver (one case). Another common site of metastasis is the bone, which was seen in one of our patients.…”

“…A recent trend toward conservative globe salvage surgery has been reported in 47 (63.5%) out of 74 cases in the literature. [ 1 2 5 7 8 9 ]…”