Orbital alveolar soft part sarcoma: A report of 8ï¿½cases and review of the literature

Huang, Yan; Li, Kang; Yang, Xinji; Wang, Yi; Lu, Xiao-zhong; Li, Yueyue; Zhang, Wei; Xiao, Lan

doi:10.3892/ol.2017.7286

Cited by 17 publications

(45 citation statements)

References 40 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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 immunohistochemistry, 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 Canada. This tumour can be misdiagnosed due to very low incidence and the absence of characteristic clinical findings.…”

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 67%

“…Out of the 49 cases, 6 patients (12%) died of the disease. 1,2,6 Increasing age, tumour size, and metastatic disease at presentation seem to indicate a poor prognosis. Herein, the patient was 31 years old.…”

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 99%

“…This differentiation is important before surgery to ensure adequate resection margins. 12,14 The tumour is frequently well circumscribed 1 and has already been diagnosed as cavernous hemangioma on imaging examination. 5 Histopathologically, ASPS has a distinguished morphology composed of monotonous cells that have a vesicular nucleus with a small nucleolus and abundant granular eosinophilic cytoplasm.…”

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 99%

See 2 more Smart Citations

Orbital alveolar soft part sarcoma: case report and literature review

Barros

Hakim

Passos

et al. 2019

Canadian Journal of Ophthalmology

View full text Add to dashboard Cite

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 £).

show abstract

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 67%

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 99%

Section: T a G G E D H 1 Case Reportt A G G E D E N Dmentioning

confidence: 99%

See 1 more Smart Citation

Orbital alveolar soft part sarcoma: case report and literature review

Barros

Hakim

Passos

et al. 2019

Canadian Journal of Ophthalmology

View full text Add to dashboard Cite

show abstract

“…In the cheek ASPS, the mean size was 3.7 cm (range 1.2-10 cm) and mean age was 33.7 years (range 5-57 years), whereas, in general ASPS, the mean size was 6.5 cm (range 1.2-24 cm) and mean age was 25 years (range 1-78 years) [1,[4][5][6][7][8][9][10][11][12]. The median size of lingual ASPS was 2.5 cm and that of orbital ASPS was 2.85 cm [19,20]. The median age of lingual ASPS was 5 years and that of orbital ASPS was 12 years [19,20].…”

Section: Discussionmentioning

confidence: 99%

“…The median size of lingual ASPS was 2.5 cm and that of orbital ASPS was 2.85 cm [19,20]. The median age of lingual ASPS was 5 years and that of orbital ASPS was 12 years [19,20]. The rarity of ASPS in the head and neck region makes the determination of prognostic factors for survival difficult.…”

Section: Discussionmentioning

confidence: 99%

Primary Alveolar Soft Part Sarcoma of Cheek: Report of a Case and Review of the Literature

Hirose

Naniwa

Usami

et al. 2021

Head and Neck Pathol

View full text Add to dashboard Cite

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma characterized by an alveolar or organoid arrangement of polygonal tumour cells separated by fibrovascular septa. A specific fusion gene [ASPS critical region 1 (ASPSCR1)-TFE3] was detected in ASPS. Despite being a slow-growing tumour without pain and dysfunction, ASPS is characterized by early metastasis, which leads to poor prognosis. Herein, we report a rare case of primary ASPS of the cheek harbouring ASPSCR1 (exon 7)-TFE3 (exon 5) fusion gene in a 21 year-old woman. This tumour was a well-circumscribed, smooth, round mass that was clinically suspected as a benign tumour. However, histologically, it was observed that the polygonal tumour cells were arranged in solid and alveolar growth patterns. Post-operative examination of the whole body excluded the possibility of metastasis at other sites. Thus, careful immunohistochemical and genetic analyses, as well as whole-body examination, demonstrated that the tumour was a primary ASPS of the cheek.

show abstract