子宫平滑肌肉瘤盆腹腔广泛播散一例

子宫平滑肌瘤与早期子宫平滑肌肉瘤在临床表现及辅助检查方面很难鉴别,由于无瘤观念缺乏,在无保护措施下使用肌瘤分碎器,会增加子宫平滑肌肉瘤腹腔内播散的风险,并导致较差的预后.回顾性分析甘肃省妇幼保健院收治的1例患者腹腔镜下经分碎器行次全子宫切除术后出现腹胀,考虑为子宫平滑肌肉瘤盆腹腔广泛播散,行肿瘤细胞减灭术后辅助化疗的诊...     

New developments in the pathology and molecular biology of retroperitoneal sarcomas

Retroperitoneal sarcomas (RPS) refer to a heterogeneous group of malignancies of mesenchymal origin developing from retroperitoneal tissues and vessels. The most frequent RPS are well differentiated/dedifferentiated liposarcomas and leiomyosarcomas, but other rare histological subtypes can be observed. Over the last decade, significant advances have been made in the pathological and molecular characterization of sarcomas. These advances have led to major changes in their diagnostic management as well as in the development of new therapeutic strategies based on tumor biology and microenvironment. This review describes the current knowledge and recent findings in the pathology and molecular biology of the most frequent RPS subtypes.     

EJSO educational Special issue from the TARPSWG - Standard medical treatment and new options in retroperitoneal sarcoma

Retroperitoneal soft tissue sarcomas mainly consist histologically of liposarcomas and leiomyosarcomas. For the liposarcoma subgroup, the local relapse rate seems to determine patients' overall prognosis. In contrast, leiomyosarcoma patients are challenged by the development of metastatic disease; therefore, effective systemic therapies are the cornerstone to improve patients’ outcome. No doubt, the limited number of active regimens currently available makes the treatment of patients with locally advanced and/or metastatic disease challenging and results in the overall poor prognosis of this population. In this European Journal of Surgical Oncology Educational Special Issue from the Transatlantic Australasian RetroPeritoneal Sarcoma Working Group (TARPSWG), we aim to summarize state-of-the-art systemic treatments for patients with retroperitoneal sarcomas with a focus on the locally advanced and metastatic disease setting including conventional standard chemotherapies as well as new innovative treatment approaches in order to identify current unmet medical needs guiding the sarcoma community to initiate appropriate translational research projects and design innovative clinical trials.     

Leiomyosarcoma of the lower rectum managed by radiotherapy and surgery: A case report and review of literature

Rectal leiomyosarcoma is a very rare entity. Surgery is the main treatment, but the place of radiation therapy remains unclear. A 67-year-old woman was referred for a few-weeks’ history of bleeding and anal pain intensified during defecation. Pelvic magnetic resonance imaging (MRI) showed a rectal lesion and biopsies revealed a leiomyosarcoma of the lower rectum. She was free of metastasis on computed tomography imaging. The patient refused radical surgery. After discussion by a multidisciplinary team, the patient received pre-operative long-course radiotherapy followed by surgery. The tumor was treated with 50 Gy delivered in 25 fractions, within 5 weeks. The aim of radiotherapy was local control, allowing organ-preservation. Four weeks after radiation therapy, organ-preservation surgery could be performed. She had no adjuvant treatment. At 38-months follow-up, she had no local recurrence. However, distant recurrence (lung, liver, and bone) was detected 38 months after the resection and was managed by intra-venous doxorubicin 60 mg/m² and dacarbazine 800 mg/m² every 3 weeks. The patient was in a stable condition for nearly 8 months. The patient died 4 years and 3 months after the diagnosis.     

原发性卵巢平滑肌肉瘤诊断和治疗(附2例报告)

目的探讨原发性卵巢平滑肌肉瘤的临床特点及诊断、治疗方法.方法对2例原发性卵巢平滑肌肉瘤进行临床报告及分析.结果原发性卵巢平滑肌肉瘤可发生在任何年龄,临床表现不典型.诊断主要依靠病理,核分裂数为主要依据,同时结合免疫组化Desmin(-),Vimentin(+)诊断.结论原发性卵巢平滑肌肉瘤恶性度高,预后差,治疗需以手术为主,辅以合适的放疗和化疗综合性治疗.     

Posterior mediastinal epithelioid leiomyosarcoma: Case report and literature review

Epithelioid type leiomyosarcoma is rarely encountered outside of the abdomen or uterus. We present a case of posterior mediastinal leiomyosarcoma in a 45-year-old male with back pain and bilateral lower extremity weakness. Magnetic Resonance Imaging of the thoracic spine revealed a heterogeneous posterior mediastinal soft tissue mass infiltrating the vertebral body and epidural space with resultant spinal cord compression and edema. Positron Emission Tomography showed no evidence of distant metastatic spread. Histopathological characterization revealed epithelial type leiomyosarcoma. Despite multiple subtotal resections, radiotherapy, and salvage chemotherapy with successful restoration of the patient''s neurological function, the tumor burden remained significant. The patient was subsequently lost to follow up and the clinical outcome remains unknown. To our knowledge this is the first reported case of epithelioid type posterior mediastinal leiomyosarcoma presenting with spinal cord compression and edema.     

Primary leiomyosarcoma of the sigmoid colon: Case report and review of literature

Leiomyosarcomas of the sigmoid colon are aggressive and extremely rare tumors representing less than 0.1% of all colorectal malignancies. Few cases have been reported in the literature and their imaging features need to be more detailed. We report the case of a 70 year old male patient, smoker, with a history of arterial hypertension, admitted for abdominal pain and hypogastric mass. Computed tomography has shown a voluminous heterogeneously enhancing tumor process with a necrotic center, attached to the sigmoid wall, with multiple secondary peritoneal and hepatic masses. The anatomopathological examination of the biopsy sample with immunohistochemistry allowed the diagnosis of leiomyosarcoma of the sigmoid colon. Clinical presentation and radiological features of leiomyosarcomas are non-specific, and the definitive diagnosis is only established after an anatomopathological examination.