Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment, and Outcome in a Series of 33 Patients at a Single Institution

Pennacchioli, Elisabetta; Fiore, Marco; Collini, Paola; Radaelli, Stefano; Dileo, Palma; Stacchiotti, Silvia; Casali, Paolo G.; Gronchi, Alessandro

doi:10.1245/s10434-010-1186-x

Cited by 86 publications

(73 citation statements)

References 18 publications

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“…The most common sites of ASPS are the deep muscles of the lower limbs. ASPSs also occur in other parts, such as the head and neck, the mediastinum, and the retroperitoneum [8]. It grows slowly, shows strong enhancement and has a slow painless progress and a tendency to metastasize [9].…”

Section: Discussionmentioning

confidence: 99%

Magnetic Resonance Features and Characteristic Vascular Pattern of Alveolar Soft-Part Sarcoma

Cui

Chen²,

Hao³

et al. 2017

Oncol Res Treat

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Objective: The aim of this study was to investigate the magnetic resonance (MR) features of alveolar soft-part sarcoma (ASPS). Methods: We studied 12 patients with ASPS confirmed by pathology in this retrospective study. MR features were analyzed, especially for the location, morphology, signals, and related enhanced features of the tumor vessels. Results: Flow voids were shown in the central part of the tumor on T2-weighted imaging (T2WI) in all patients; they were arrayed in a radiating mode gathered toward the center (8 cases), designated by us as vascular center-gathered syndrome (VCGS), or scattered like twigs (4 cases). The flow voids were accompanied by high signals in all patients, including tubular (6 cases) and platy (6 cases) signals. Slightly higher signals were shown in the peripheral part of the tumor in all patients. Flow voids in the peripheral part were shown in all patients, and the majority of the flow voids surrounded the tumor (8 cases). The vessels around the tumor in 9 patients showed high signals, and the majority of the vessels were located at the superior and inferior poles (8 cases). 6 patients underwent enhanced scanning, including moderate (5 cases) and significant enhancement (1 case). Conclusion: Low signals of radiating flow voids accompanied by high signals of slow blood flow or blood sinuses in the center part have high significance for the diagnosis of ASPS.

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Section: Discussionmentioning

confidence: 99%

Magnetic Resonance Features and Characteristic Vascular Pattern of Alveolar Soft-Part Sarcoma

Cui

Chen²,

Hao³

et al. 2017

Oncol Res Treat

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“…The status of the surgical margins is also a significant prognostic factor. Patients who underwent complete resection showed better prognoses than those who underwent incomplete or marginal resection [24].…”

Section: Discussionmentioning

confidence: 88%

“…Tumor size has been shown to be a prognostic factor. Several reports have indicated that patients with tumors less than 5 cm in diameter had significantly better prognoses than those with larger tumors [20][21][22][23][24]. The status of the surgical margins is also a significant prognostic factor.…”

Section: Discussionmentioning

confidence: 99%

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A case of primary alveolar soft part sarcoma of the uterine cervix and a review of the literature

et al. 2011

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Alveolar soft part sarcoma (ASPS) that originates from the uterine cervix is extremely rare, with only thirteen cases reported. The participation of the ASPL-TFE3 chimeric gene, translocation (X; 17) (p11; q25), has been demonstrated in ASPS. Here, we report a case of cervical ASPS, along with a review of the literature. The patient, a 56-year-old woman, was referred for a 70 × 80 mm cervical tumor. She underwent a hysterectomy and bilateral salpingo-oophorectomy, and remained disease free for 66 months without adjuvant therapy. Pathological examination revealed features consistent with ASPS. In addition, the present case demonstrated strong positive nuclear staining for TFE3, and ASPL-TFE3 fusion gene type 1 was detected by RT-PCR. In a review of fourteen cases of this tumor (including the present case), the immunohistochemical expression patterns of myogenic or neuroendocrine markers were somewhat varied among cases. In all cases except for the present case, the patients were under 40 years of age, and the tumor sizes were under 5 cm. The prognosis of ASPS in the cervix was considerably better than that of ASPS in soft tissues. Complete resection with adequate margins is thought to be important, although the appropriate surgical method, including lymph node dissection, is uncertain. The role of chemotherapy or radiotherapy as adjuvant therapy has not been defined. Cervical ASPS is extremely rare, making case series the most viable option for understanding their natural history and for developing a treatment strategy, including an optimal surgical procedure and adjuvant therapy.

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“…The development of imatinib for gastrointestinal stromaltumors(GIST) [2]canserveasanexample.Alveolar softpartsarcoma(ASPS)isanextremelyraresubtypeofsoft tissue sarcoma. It is characterized by translocation der(17) t(X:17)(p11;p25)ononehand,andausuallyindolentgrowth patternontheother [3].SimilartoGIST,itisquiteinsensitive tothecommonlyusedcytotoxicdrugs [4].…”

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confidence: 99%

Alveolar Soft Part Sarcoma: Should We Be Targeting the Tumor or Targeting the Vasculature?

Verweij¹

2012

Onkologie

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Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment, and Outcome in a Series of 33 Patients at a Single Institution

Abstract: Prognosis of ASPS is basically related to the characteristics of the disease and the quality of surgery. Overall, the occurrence of distant metastases is quite common, with a typical indolent course. New agents are eagerly needed to complement surgery to eradicate this disease.

Cited by 86 publications

References 18 publications

Magnetic Resonance Features and Characteristic Vascular Pattern of Alveolar Soft-Part Sarcoma

Magnetic Resonance Features and Characteristic Vascular Pattern of Alveolar Soft-Part Sarcoma

A case of primary alveolar soft part sarcoma of the uterine cervix and a review of the literature

Alveolar Soft Part Sarcoma: Should We Be Targeting the Tumor or Targeting the Vasculature?

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