Secondary Extra-anatomic Infrainguinal Bypass following Lower Limb Tumoral Resection.

BACKGROUND: Soft tissue malignancy of lower limb can involve femoral triangle by direct tumoral invasion or secondary to ganglionic metastasis. Secondary arterial complications can appear during follow-up after initial tumoral resection and local radiation therapy. The aim of this study is to report our experience of secondary extra-anatomical lower limb revascularization following lower limb oncological resection with femoral bifurcation involvement. METHODS: This is a retrospective monocentric study including patients who underwent extra-anatomical iliopopliteal bypass, with a previous treated neoplasia involving homolateral femoral bifurcation. Proximal anastomosis was performed on the iliac artery, tunnelization was made through iliac wing, and distal anastomosis was done on distal superficial femoral or popliteal artery. RESULTS: Five patients underwent extra-anatomic iliopopliteal bypass for oncological purpose from 2008 to 2018 at our institution. Mean age at surgery time was 52 years (standard deviation = 19.3). Prosthetic graft was used in all cases. Primitive tumor involved Scarpa triangle in 3 cases (soft tissue sarcomas) and ganglionic metastasis involved Scarpa triangle in 2 cases (epidermoid carcinoma). Clinical presentation was ischemic in 4 cases and hemorrhagic in 1 case. One patient died during hospitalization. Of the 4 survivors, 3 patients had a patent bypass at the end of follow-up (2 had bypass thrombectomy, 1 patient had major amputation). CONCLUSIONS: Secondary iliopopliteal bypasses through the iliac wing following lower limb tumoral resection have acceptable results. It is a valid option for limb salvage especially after local radiation therapy and tumoral resection. Multidisciplinary management is necessary to obtain acceptable results and follow-up is mandatory.

Spontaneous Regression of Metastatic Extraskeletal Myxoid Chondrosarcoma.

Spontaneous regression of tumors is very unusual and is defined as a partial or complete disappearance of metastatic tumors without any treatment. This phenomenon has been reported in almost all types of cancer. The patient was a 25-year-old woman who presented with multiple pulmonary nodules on her bilateral lungs on the annual chest roentgenograph. Simultaneously, a swelling mass on her subcutaneous inguinal region was observed. The diagnosis of the inguinal mass was extraskeletal myxoid chondrosarcoma. The inguinal mass and pulmonary nodules spontaneously regressed without any treatment after biopsy. The patient was doing well without evidence of recurrence at 1 year after the operation without any additional therapy. Our case is the first clinical one that indicated a possibility of histologic regression of extraskeletal myxoid chondrosarcoma.

Breast Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.

BACKGROUND: Extraskeletal myxoid chondrosarcoma is a unique and distinct clinicopathological entity in terms of its origin, morphology, and biologic behavior. Despite being a slow-growing tumor, it has a high rate of local recurrences and history of metastases to uncommon sites like the mandible, liver, retroperitoneum, right ventricle, pancreas, and central nervous system. Here, we report a very unique case of extraskeletal myxoid chondrosarcoma that metastasized to the breast, which itself is a very rare site for metastases. CASE REPORT: A 58-year-old woman presented with a large, firm, and tender soft-tissue mass (6.0×7.0 cm) underneath the sole of the left foot. A computerized tomography (CT) scan showed a heterogeneous lobulated mass in the plantar aspect of the forefoot, measuring 8.6×8.0×7.1 cm. Punch biopsies revealed histology consistent with extraskeletal myxoid chondrosarcoma. Metastatic work-up was negative. The mass was fully resected with left below-knee amputation. The histology of the resected mass was consistent with extraskeletal myxoid chondrosarcoma. A follow-up CT showed a new right breast nodule along with metastases to lung and bones. The results of the core needle biopsies of the right breast masses seen on mammogram were morphologically identical to extraskeletal myxoid chondrosarcoma. CONCLUSIONS: Although rare, metastases to the breast should be considered in the differential diagnosis of a breast mass. A close long-term follow-up is needed due to the unpredictable behavior of extraskeletal myxoid chondrosarcoma and the high frequency of local recurrences, metastases, and death due to disease.

Primary malignant myopericytoma of the left atrium--a tumor of aggressive biological behavior: report of the first case and review of literature.

We report a case of malignant myopericytoma arising in the left atrium with brain, skeletal, and liver metastases, which, to our knowledge, is the first report of this rare entity in this anatomic location. A 52-year-old man presented with progressive blackening of his left field of vision. Magnetic resonance imaging and a computed tomography scan of the brain and thorax showed a heterogeneous mass in the right occipital lobe and a large left atrial floor mass. Excision of the atrial mass showed a circumscribed but unencapsulated malignant spindled neoplasm with a perivascular concentric cellular arrangement punctuated by sheets of tumor necrosis. The cells were round to spindled with eosinophilic cytoplasm and indistinct borders. Focally, the tumor infiltrated cardiac muscle. By immunohistochemistry, the cells were positive for smooth muscle actin and negative for desmin, H-Caldesmon, S-100, HMB-45, and Melan-A. The features were prototypic for malignant myopericytoma. Eight months after initial presentation, the patient is alive with metastatic disease.

Activity of sunitinib in extraskeletal myxoid chondrosarcoma.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma, marked by NR4A3 rearrangement. Herein we report on the activity of sunitinib in a series of 10 patients, strengthening what initially observed in two cases. PATIENTS AND METHODS: From July 2011, 10 patients with progressive metastatic translocated EMC have been consecutively treated with sunitinib 37.5mg/day, on a named-use basis. In an attempt to interpret the activity of sunitinib in EMC, genotype/phenotype correlations were carried out by fluorescence in situ hybridization (FISH) analyses. Moreover, transcriptome, immunohistochemical and biochemical analyses of a limited set of samples were performed focusing on some putative targets of sunitinib. RESULTS: Eight of 10 patients are still on therapy. Six patients had a Response Evaluation Criteria in Solid Tumours (RECIST) partial response (PR), two were stable, two progressed. Positron emission tomography (PET) was consistent in 6/6 evaluable cases. One patient underwent surgery after sunitinib, with evidence of a pathologic response. At a median follow-up of 8.5 months (range 2-28), no secondary resistance was detected. Median progression free survival (PFS) has not been reached. Interestingly, all responsive cases turned out to express the typical EWSR1-NR4A3 fusion, while refractory cases carried the alternative TAF15-NR4A3 fusion. Among putative sunitinib targets, only RET was expressed and activated in analysed samples. CONCLUSIONS: This report confirms the therapeutic activity of sunitinib in EMC. Genotype/phenotype analyses support a correlation between response and EWSR1-NR4A3 fusion. Involvement of RET deserves further investigation.

[Cardiac metastase of a soft tissue sarcoma: a case report and a review of the literature]. / Métastase cardiaque d'un sarcome des tissus mous: cas clinique et revue de la literature.

Soft tissue sarcomas represent rare tumors. They recur most often locally and in the lungs. We report the case of a 58-year woman who was treated for awith chemotherapy and then surgery. About a year after the end of the treatment an intracardiac mass was identified during a follow up chest CT-scan. The patient underwent a surgical resection of that mass found to bel myxoid chondrosarcoma metastasis. Only 2 cases of cardiac dissemination of extraskeletal myxoid chondrosarcoma have been described. The differential diagnosis of intracardiac masses is discussed. In some carefully selected cases atrisks of obstructive shock or embolization and unique metastatic location, cardiac surgery should be considered.

Extraskeletal myxoid chondrosarcoma of the thigh in a child: a case report.

Extraskeletal myxoid chondrosarcoma (EMC) is aggressive in children. The condition in children differs to that in adults and to skeletal myxoid chondrosarcoma. We report on a 9-year-old girl with EMC in her left thigh. She underwent above-knee amputation. Five months later, a small mass was noted at the right lower lobe of the lung. The patient underwent one course of ifosfamide, carboplatin, and etoposide chemotherapy, followed by resection of the mass and 8 more courses of chemotherapy. At the 2-year follow-up, she was in remission radiologically.

[Correlation analysis of gastric cancer with extranodal metastasis].

OBJECTIVE: To evaluate the correlations between extranodal metastasis(EM)and clinicopathologic features of gastric cancer and the relationship between EM and prognosis of gastric cancer. METHODS: Data of patients with histologically proven adenocarcinoma were studied retrospectively to evaluate the prognostic factors in gastric cancer by univariate and multivariate analyses of Cox regression with SPSS 13.0 software. Two hundred and seventy-six patients with primary gastric cancer undergone operation in Tianjin Cancer Hospital from Jan. 2001 to Dec. 2001 were studied and followed up until Dec. 2006 or death. RESULTS: EMs were found in 58(21.0%) of the 276 patients. The overall 2-, 3-, and 5-year survival rates of the patients without EM were 71.2%, 55.4%, and 45.1% respectively. The overall 2-, 3-, and 5-year survival rates of the patients with EM were 24.1%, 15.5%, and 8.0% respectively. Postoperative overall survival rates were significantly lower for patients with EM than those without EM(P=0.000). EM was correlated with differentiation (r=0.163, P=0.008), invasive depth (r=0.215, P=0.003), lymph node metastasis (r=0.368, P=0.000), distant metastasis (r=0.375, P=0.000), advanced stage(r=0.441, P=0.000), and tumor size (r=0.167, P=0.007). Multivariate analysis identified EM as an independent prognostic factor. CONCLUSIONS: EM is correlated with many clinicopathological features of gastric cancer. EM is an independent prognostic factor of gastric cancer.