Clinico-pathological spectrum of alveolar soft part sarcoma: case series from a tertiary care cancer referral centre in india with a focus on unusual clinical and histological features

• DOI: 10.5146/tjpath.2023.01605
• Publication Date: 2023-05-22T13:47:53Z
• Authors: Kanwalpreet Kaur; Amisha Gami; Ashini Shah; Jahnvi Gandhi; Priti Trivedi
• Keywords: Pleomorphism (cytology)

Objective: Alveolar soft part sarcoma (ASPS) is characterized by distinctive histomorphology of variably discohesive epithelioid cells arranged in nests and translocation t(x;17) (p11.2;q25)resulting ASPSCR1-TFE3 fusion.The aim the present study to review clinical, histopathological, immunohistochemical profile ASPS with a focus on unusual histological features. Material Method:The retrospective descriptive.All cases diagnosis were retrieved clinical radiology details.Results: 22 patients identified.The most common site was lower extremity size range 3-22 cm.54.5% had metastasis, lung as site.Metastasis preceded detection primary tumour two cases.All showed similar histopathology monomorphic encircled sinusoidal vasculature.Architecturally, organoid pattern (81.8%) followed alveolar pattern.68.2% apple bite nuclei predominant nuclear feature.Rare features included binucleation (n=13), multinucleation (n=8), pleomorphism (n=4), grooves three intranuclear inclusion one case, mitosis (n=5), focal necrosis (n=6).All positive for TFE3 negative AE1/AE3, EMA, HMB45, PAX8, MyoD1, SMA, synaptophysin, chromogranin.Only S100 positivity while desmin positivity. Conclusion:Diffuse strong sensitive an appropriate clinicoradiological context.Due high propensity early complete metastatic work-up long term follow up recommended.