Metastases Of Alveolar Soft-part Sarcoma Research Articles

Oral metastasis of alveolar soft-part sarcoma: a case report and review of literature

Alveolar soft-part sarcoma (ASPS) is a rare malignant neoplasm with uncertain histogenesis and with a distinctive morphology. It has been described in the oral cavity, but this is the first report of ASPS metastasizing to the maxillary tuber region. A 27-year-old male patient, who was under chemotherapy treatment for ASPS of the thigh, presented in our dental clinic with a painless and pedunculated nodule on the right tuber maxillae. The nodule was erythematous with smooth and lobular surface, measuring 3 cm in maximum diameter. An incisional biopsy was performed and the diagnosis of metastatic ASPS was made. Histologically, the tumor was characterized by a proliferation of polyhedral cells in pseudoalveolar pattern. Tumor cells were large, showing granular cytoplasm, periodic acid-Schiff positive diastase-resistant intracytoplasmic material, and vesicular nuclei with prominent nucleoli. Unfortunately, the patient died 2 months after the diagnosis of the oral metastasis. Metastases of ASPS to the mouth are very rare and indicate a poor prognosis.

Myofibroblasts in Pulmonary and Brain Metastases of Alveolar Soft-Part Sarcoma: A Novel Target for Treatment?

Alveolar soft-part sarcoma (ASPS) is a rare neoplasm with chromosomal translocation that results in ASPL-TFE3 fusion. It is a slow-growing lesion associated with a high incidence of pulmonary and brain metastases indicating poor survival. We demonstrated that the ASPS metastases include also stromal myofibroblasts. These cells pro-liferate, express smooth-muscle genes, and synthesize extracellular matrix proteins, all of which are characteristics of activated myofibroblasts. The tumor cells also exhibited stromal components such as transforming growth factor beta (TGFβ)–dependent, hypoxia-regulated cytoglobin (stellate cell activation association protein, cytg/ STAP) and prolyl 4-hydroxylase, a collagen cross-linking enzyme. The pulmonary ASPS myofibroblasts synthesize serum response factor (SRF), a repressor of Smad3-mediated TGFβ signaling essential for myofibroblast differentiation and Smad3. The phosphorylated active Smad3 was found mostly in the tumor cells. The brain tumor cells express cytg/STAP, but in contrast to the lung metastases, they also express SRF, Smad3, and phospho-Smad3. Halofuginone, an inhibitor of myofibroblasts’ activation and Smad3 phosphorylation, inhibited tumor development in xenografts derived from renal carcinoma cells harboring a reciprocal ASPL-TFE3 fusion transcript. This inhibition was associated with the inhibition of TGFβ/SRF signaling, with the inhibition of myofibroblasts’ activation, and with the complete loss in TFE3 synthesis by the tumor cells. These results suggest that the myofibroblasts may serve as a novel target for treatment of ASPS metastases.

Pancreatic metastasis of alveolar soft-part sarcoma: A case report and review of the literature

Alveolar soft-part sarcoma is a rare tumour. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. We present a case of pancreatic metastasis, occurring more than six years after diagnosis. Treatment consisted in subtotal pancreatoduodenectomy with pylorus resection. Both specific patterns of relapse and treatment opportunities of this uncommon feature are discussed.

Lingual metastasis of alveolar soft-part sarcoma

A patient with lingual metastasis of alveolar soft-part sarcoma, which originated in the brachioradial muscle, is presented and the literature, is reviewed.