EC Dental Science

Alveolar soft part sarcoma is an exceptionally discerned, malignant mesenchymal neoplasm of uncertain histogenesis. Initially scripted by Christopherson., et al. in 1952, alveolar soft part sarcoma characteristically depicts a specific genetic translocation der(17)t(X;17)(p11.2;q25) with consequent occurrence of ASPSCR1-TFE3 genetic fusion [1].

Tumour is constituted of enlarged, polygonal cells permeated with abundant, eosinophilic cytoplasm with intracytoplasmic rhomboid or rod shaped crystals which can stained with periodic acid Schiff’s (PAS) stain with diastase resistance. Neoplasm configures a nested or pseudo-alveolar tumour pattern.

Alveolar soft part sarcoma is a high grade neoplasm although devoid of applicable formal grading system. Initial biological course may appear as indolent although overall prognostic outcomes are inferior.

Alveolar soft part sarcoma manifests an estimated < 1% of soft tissue sarcomas. Median age of disease emergence is 25 years although no age of disease occurrence is exempt. Majority (~70%) of incriminated subjects appear < 30 years with neoplastic occurrence commonly discerned within 15 years to 35 years.

1. Christopherson WM., et al. “Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis”. Cancer1 (1952): 100-111.
2. Fujiwara T., et al. “Alveolar soft part sarcoma: progress toward improvement in survival? A population-based study”. BMC Cancer1 (2022) :891.
3. Tan Z., et al. “Clinical features and therapeutic outcomes of alveolar soft part sarcoma in children: A single-center, retrospective study”. Frontiers in Oncology 12 (2022): 1019911.
4. Kazuhiro Tanaka and Toshifumi Ozaki. “New TNM classification (AJCC eighth edition) of bone and soft tissue sarcomas: JCOG Bone and Soft Tissue Tumor Study Group”. Japanese Journal of Clinical Oncology2 (2019): 103-107.
5. Aksionau A., et al. “Lingual Alveolar Soft Part Sarcoma in a 78-Year-Old Woman: A Case Report and Comprehensive Review of the Literature from 1952 to 2022”. Head and Neck Pathology (2022).
6. Chang X., et al. “The current management of alveolar soft part sarcomas”. Medicine31 (2021): e26805.
7. Image 1 Courtesy.
8. Image 2 Courtesy.