Background Gastric schwannomas (Gs) are rare mesenchymal neoplasms of the gastrointestinal tract. Diagnosis is commonly achieved by pathological examination of resected specimen. In most cases surgical resection of submucosal gastric lesions is performed with wide margins. New minimally invasive techniques are described in an increasing number of cases. Methods A Pubmed, Cochrane and Embase systematic review of the literature has been performed. Original papers, review articles, case reports published between 1988 and 2019 were considered eligible. Only papers written in English with full text available have been included. Articles reporting a follow up period, the type of treatment of the primary tumor and the appearance of local or distant recurrence were compared and analysed. Statistical analysis of data has been performed using GraphPad Prism 7 software. Results 328 articles were found and a total of 102 were included in the analysis. Fifty-three papers reported the follow-up information, ranging from 1-417 months across different studies. Among them, 31 patients underwent endoscopic removal of the gastric lesions, 140 patients local surgery, including wedge resection or partial gastrectomy and 148 patients underwent subtotal or total gastrectomy. The median follow-up was of 27-38-33 months respectively. No recurrence or distant metastasis were detected in endoscopic group. Among local surgery group, liver metastasis was reported in one case; in extended surgery group one patient died for multiple liver metastases. Conclusions In comparison with endoscopic group, local or more extended surgery involved a larger cohort of patients and reported satisfactory long-term results. Surgical approach in absence of a definite pre-operative diagnosis is considered the gold standard treatment for resectable Gs due to the excellent long-term outcome. Further studies are warranted to define the role of endoscopic treatment. Keywords : Schwannoma, neurinoma, gastric neurinoma, gastric schwannoma.
Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue tumor that typically affects the lower limbs of men between the ages of 50 and 60. EMC of the shoulder is rare with a high risk of local recurrence and distant metastasis. A planned surgical excision in sarcoma referral centers (SRCs) is mandatory to obtain the best outcome. The role of chemotherapy (CHT) and Radiotherapy (RT) on soft tissue chondrosarcoma is still controversial. Case presentation A 47-year-old man presented to our referral center with a history of EMC in the right shoulder excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis or residual disease, but during follow-up a local recurrence was detected. After a multidisciplinary discussion, preoperative radiotherapy was administered with a total dose of 50 Gy, and then the patient underwent wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred in a 24-months post-operative follow up. Conclusions The case here described suggests the importance of patient’s management in SRCs. A planned combined treatments with both surgery and RT seems to be the best choice to improve local control. RT seems to be promising within this specific histotype. Further studies are needed to confirm if the observed efficacy of combined treatments reflects in a consistent survival benefit for EMC patients.
Background Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated. Case presentation A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up. Conclusions The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival.
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