BACKGROUND: Alveolar soft part sarcoma is a rare tumour of soft tissues, mostly localized in muscles or deep soft tissues of the extremities. In rare occasions, this tumour develops in deep tissues of the abdomen or pelvis.
METHODS: In this case report, we described the case of a 46 year old man who developed a primary splenic alveolar soft part sarcoma. The tumour displayed typical morphological alveolar aspect, as well as immunohistochemical profile notably TFE3 nuclear staining. Detection of ASPSCR1 Exon 7::TFE3 Exon 6 fusion transcript in molecular biology and TFE3 rearrangement in FISH confirmed the diagnosis.
CONCLUSIONS: We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.

UNASSIGNED: Alveolar soft part sarcoma (ASPS) is a rare and distinct subtype of soft tissue sarcoma. This study aims to describe the unique presentation of ASPS in the female genital tract.
UNASSIGNED: Prognostic factors for cancer-specific overall survival (CSS) were evaluated using multivariate analyses.
UNASSIGNED: In our case series, we identified a novel TFE3-PRCC gene fusion in a 24-year-old unmarried patient with cervical ASPS who underwent fertility-sparing surgery and remained recurrence-free for 41 months. The other two patients underwent radical hysterectomy and bilateral salpingo-oophorectomy. At the time of writing, the two patients had been disease-free for 49 and 71 months, fluorescence in situ hybridization showed break-apart signals for the ASPL-TFE3 gene. Among the 55 cases with available information from the PubMed/Medline database, most presented with localized disease, and at the last follow-up, all patients were alive and 45 patients showed no evidence of disease. The 5-year CSS rate in the female genital tract cohort from SEER database was 86.2%. Multivariate analysis revealed that older age was associated with a 1.042-fold increased risk of cancer-specific mortality (HR=1.042, 95% CI 1.022-1.063, P < 0.001), involvement of soft tissue including the heart was associated with a 4.786-fold higher risk (HR=4.7868, 95% CI 1.681-13.623, P= 0.003), and regional infiltration and distant metastasis were associated with approximately 8.6-fold and 18-fold higher risk of cancer-specific mortality compared to local disease, respectively (HR=8.652, 95% CI 2.529-29.63, P = 0.001; HR=18.366, 95% CI 6.153-54.817, P< 0.001). Patients who underwent radical excision did not show reduced cancer-specific mortality compared to those who underwent local excision (HR=0.492, 95% CI 0.224-1.081, P = 0.078).
UNASSIGNED: Previously unrecognized genetic diversity exists in ASPS. Patients with ASPS in the female genital tract have the lowest likelihood of presenting with a distant disease and are associated with a more favorable survival outcome.

OBJECTIVE: Female genital alveolar soft part sarcoma (ASPS) is rare and has a favourable prognosis compared to ASPS from other sites. We reported our experience to manage a case with uterine corpus ASPS (UC ASPS) and conducted a literature review on prognosis of ASPS from different sites of female genital tract.
METHODS: This report represented a 33-year-old woman who had UC ASPS. She received tumor excision with uterine preservation and had the longest follow-up time (155 months) without recurrence in the literature.
CONCLUSIONS: UC ASPS has better prognosis than ASPS from the uterine cervix, the low uterine segment, vulvovaginal area and perineum. We recommended conservative treatment for young women with UC ASPS.

Alveolar soft part sarcoma is a rare neoplasm of uncertain histogenesis that belongs to a newly defined category of ultra-rare sarcomas. The neoplasm is characterized by a specific chromosomal translocation, der (17) t(X; 17)(p11.2;q25), that results in ASPSCR1-TFE3 gene fusion. The natural history of alveolar soft part sarcoma describes indolent behaviour with slow progression in deep soft tissues of the extremities, trunk and head/neck in adolescents and young adults. A high rate of detection of distant metastasis at presentation has been reported, and the most common metastatic sites in decreasing order of frequency are the lung, bone and brain. Complete surgical resection remains the standard treatment strategy, whereas radiotherapy is indicated for patients with inadequate surgical margins or unresectable tumours. Although alveolar soft part sarcoma is refractory to conventional doxorubicin-based chemotherapy, monotherapy or combination therapy using tyrosine kinase inhibitors and immune checkpoint inhibitors have provided antitumor activity and emerged as new treatment strategies. This article provides an overview of the current understanding of this ultra-rare sarcoma and recent advancements in treatments according to the clinical stage of alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is an extremely rare type of soft tissue sarcoma. The primary sites of ASPS are mostly located in the extremities and trunk. Primary pulmonary ASPS is extremely rare. A search of the PubMed® database identified only five cases of primary pulmonary ASPS. This current case report describes the sixth case of ASPS in a 15-year-old male that presented with recurrent headaches. Head computed tomography showed space-occupying lesions in the left parietal lobe. Positron emission tomography-computed tomography confirmed the space-occupying lesions in the left parietal lobe and showed multiple nodules and masses in the two lungs and pleura, which were considered to be low-grade malignant mesenchymal tumours. The case report presents the clinical characteristics, diagnosis and treatment process. Programmed cell death protein 1 monoclonal antibody (sintilimab) combined with a tyrosine kinase inhibitor (anlotinib hydrochloride) achieved a good therapeutic effect, indicating that this combination therapy is worth exploring further. Large-scale prospective studies are needed to explore and develop standardized treatments for ASPS.

Alveolar soft part sarcoma (ASPS) is a rare malignancy that is morphologically characterized by a distinctive nodular, organoid, or nested growth pattern in which the cells are separated by vascularized septa. The diagnosis is based on a combination of pathologic and immunohistochemical findings and the presence of an ASPSCR1-TFE3 gene fusion revealed by next-generation sequencing. ASPS most commonly occurs as a painless mass in the lower extremity, with likely involvement in the lungs if metastasis is present. Here we report a case of ASPS that exhibited the characteristic ASPSCR1-TFE3 gene fusion along with a reciprocal fusion of TFE3-ASPSCR1, which presented in the nasolabial fold of a 31-year-old female. An intraoral approach was utilized for complete surgical resection of the malignancy, resulting in continued remission after 11 months.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare kind of malignant soft tissue tumor with undefined differentiation, of which the incidence rate accounts for only 0.5-1.0% among all kinds of soft tissue tumors. An even rarer ASPS occurs in kidney.
METHODS: Here we reported a case of a 7-year-old girl diagnosed with nephrogenic ASPS, regarding the analyses of the incidence, clinical manifestation, pathology and genetic diagnosis, in order to deepen the recognition of the disease.
CONCLUSIONS: ASPS is very rare, and tends to occur to young patients. It is very significant to precisely diagnose ASPS at an early stage, which will be the key point for the following treatment choices and prognosis.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma with unique tumor characteristics, which is rare in children. Herein, we present the immunophenotype, treatment, and prognosis of three children with ASPS from The Second Xiangya Hospital of Central South University, and 51 children with ASPS have been reported in the previous literature, along with a focused review of the clinical features, pathological features, differential diagnosis, treatment, and prognosis of ASPS in pediatric patients.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare translocation-related soft tissue sarcoma, occurring mainly in the limbs and trunk in young adults and adolescents. ASPS is rarely seen in the head and neck and one fourth of those cases described are tongue primary. Given its nonspecific symptoms, clinical findings, and rarity in this location, lingual ASPS (L-ASPS) has been reported to be commonly misdiagnosed as various benign tumors, leading to adverse outcomes.
METHODS: We report a case of L-ASPS occurring in the oldest (78 years) female patient published to date and comprehensively review the literature from 1952 to 2022.
RESULTS: She presented with a slow-growing (2-year duration) tongue mass, measuring 3.5 cm on palpation. Intraoperative frozen section could not render the definitive diagnosis. The pathological findings of the tumor were characteristic of ASPS with eosinophilic polygonal cells in an organoid/nested pattern, rich sinusoidal capillaries, and TFE3 immunoreactivity, except for the strong diffuse aberrant cytoplasmic CD68 immunoexpression and absence of intracytoplasmic crystalline inclusions on PAS with diastase. After TFE3 gene rearrangement had been identified with fluorescent in-situ hybridization, reflex testing confirmed a rearrangement of TFE3 gene with the known fusion partner ASPSCR1.
CONCLUSIONS: ASPS should be included in the differential diagnoses in cases of any slow-growing lingual masses (especially vascular ones) with non-specific clinical pictures, regardless of the patient\'s age.

UNASSIGNED: Alveolar soft part sarcoma (ASPS) is a very rare type of soft tissue sarcomas which usually occurs in the limbs and trunk.
UNASSIGNED: A 25-year-old woman presented with proptosis and redness of the right eye for 8-month. She suffered from severe right upper lid edema, conjunctival chemosis, downward displacement of the globe, and proptosis. Radiological imaging was nonconclusive. Histopathological evaluations confirmed ASPS. The patient underwent exenteration as a lifesaving procedure. At 16-month follow-up, the patient is stable without any signs of recurrence or metastasis.
UNASSIGNED: We report an extremely rare case of ASPS occurring in the superior rectus muscle. Few orbital ASPS cases have been reported in the literature. A literature review of orbital ASPS was done to shed lights on the diagnosis and management of this rare tumor.