The current management of alveolar soft part sarcomas.

OBJECTIVE: Alveolar soft part sarcomas (ASPS) which has high potential ability of metastasis, is a rare and slowly growing malignant tumor, and mainly primary localized in limbs. To date, little is known about the best treatment of ASPS. This study aims to review the current management and advance of ASPS. METHODS: WANFANG MED ONLINE, CNKI, and NCBI PUBMED were used to search literature spanning from 1963 to 2020, and all cases of ASPS about "ASPS, diagnosis, treatment, surgery, radiotherapy, chemotherapy, target therapy or immune therapy" with detailed data were included. RESULTS: Complete surgical resection remained the standard management strategy, radiotherapy was reported to be used for the patients of micro- or macroscopical incomplete residue or the surgical margin was questionable. Chemotherapy was controversial. Some target drugs and immune checkpoint inhibitors had produced antitumor activity. CONCLUSION: Complete surgical resection is the cure treatment for ASPS, and adjuvant chemotherapy is not recommended excepted clinical trials. For the patients with micro- or macroscopical incomplete residue, radiotherapy should be appreciated. Furthermore, for recurrence, distant metastasis, and refractory of ASPS, combination therapy, especially combination with multiple target agents and/or immune checkpoint inhibitors may prolong survival time.

FDG PET of alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is a very rare, but distinctive type of soft tissue sarcoma, whose name is derived from the pseudoalveolar appearance of its histology. In this report, the FDG PET/CT findings of ASPS are described in a 17-year-old asthmatic female who presented with worsening respiratory symptoms and a pelvic mass. The staging PET showed heterogeneous intense incorporation of FDG within the mass and variable FDG incorporation within the multiple lung nodules. In concordance with other soft tissue sarcomas, PET/CT helped to confirm the anatomic origin of the ASPS, to direct its biopsy, and to assess the distribution of disease.

Minute alveolar soft part sarcoma of the endocervix: the smallest ever published case.

Alveolar soft part sarcoma (ASPS) is a distinctive mesenchymal tumor of uncertain histogenesis, which is exceedingly rare in the uterine cervix. We herein report the smallest (1.6 mm) ever published case of ASPS of the endocervix with the characteristic histomorphology and immunohistochemical findings. One-year follow-up was uneventful. Because of its seemingly much better prognosis, it is important in routine practice to distinguish ASPS from adenocarcinoma of the cervix. This is facilitated by the awareness that ASPS can occur in this location and if there is doubt about the diagnosis on routinely stained sections, the appropriate immunohistochemical study should be performed. Owing to the small number of published ASPSs in the cervix, the optimal treatment strategy has yet to be determined.

Alveolar soft-part sarcoma presenting with eosinophilia and shunt.

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour found in young adults that usually arises in skeletal muscle and occurs most frequently in the lower limbs. Radiological and pathological findings of ASPS in a 34-year-old man who presented with increasing shortness of breath over a period of four to six weeks with peripheral blood eosinophilia, hypoxemia and a significant arteriovenous shunt are reported. The present article is the fourth report of eosinophilia in association with sarcoma, and the first involving ASPS.

Lingual alveolar soft part sarcoma; 14 cases: novel clinical and morphological observations.

AIMS: Alveolar soft part sarcoma (ASPS) is a rare sarcoma in the buttocks or thigh of young adults, often with metastases to lung, brain, or bone. This study examines the morphological and clinical features of lingual ASPS. METHODS AND RESULTS: Fourteen cases, in eight males and six females (ages 3-21 years, median 5 years), ranged from 8 to 50 mm, median 25 mm. All tumours were intramuscular, circumscribed and multinodular. Tumours from all but the oldest patient exhibited a predominantly solid (non-alveolar) growth pattern. Vascular invasion was common. Crystals varied in number from none or extremely rare to nearly 100% of tumour cells. Immunohistochemical results: Fifty percent desmin positive, all focally smooth muscle antigen (SMA) positive; negative for vimentin, neural/melanocytic, myoid, histiocytic, and epithelial markers. All tumours were surgically excised; only two patients received chemotherapy. Follow-up on 10 patients showed that all patients were alive without disease (4-32, median 22 years). Only one patient had a microscopic metastasis to lung (3 years) but was without disease at 11 years. CONCLUSIONS: Lingual ASPS is a tumour of childhood with a distinctive, predominantly solid growth pattern. Despite typical vascular invasion, the early diagnosis and small tumour size may explain its relatively good outcome.