- 作者列表："Lee JC","Chou HC","Wang CH","Chu PY","Hsieh TH","Liu ML","Hsieh SM","Liu YR","Kao YC
:Myoepithelial tumors of soft tissue are uncommon neoplasms characterized histologically by spindle to epithelioid cells arranged in cords, nests, and/or reticular pattern with chondromyxoid to hyaline stroma, and genetically by rearrangement involving EWSR1 (among other less common genes) in about half of the cases. The diagnosis often requires immunostaining to confirm myoepithelial differentiation, most importantly the expression of epithelial markers and S100 protein and/or GFAP. However, there are cases wherein the morphology is reminiscent of myoepithelial tumors, while the immunophenotype falls short. Here, we report 2 highly similar myoepithelioma-like tumors arising in the hands of young adults. Both tumors were well-demarcated and composed of alternating cellular areas with palely eosinophilic hyaline stroma and scattered acellular zones of densely eosinophilic collagen deposition. The tumor cells were mainly epithelioid cells and arranged in cords or small nests. Vacuolated cells encircling hyaline matrix globules were focally prominent. A minor component of nonhyaline fibrous nodular areas composed of bland spindle cells and rich vasculature was also observed. Perivascular concentric spindle cell proliferation and perivascular hyalinization were present in some areas. The tumor cells were positive for CD34 and epithelial membrane antigen (focal) by immunostaining, while largely negative for cytokeratin, S100, GFAP, p63, GLUT1, and claudin-1. By RNA sequencing, a novel OGT-FOXO3 fusion gene was identified in case 1 and confirmed by reverse transcription polymerase chain reaction and fluorescence in situ hybridization in both cases. Sharing the unusual clinicopathologic features and the novel fusion, these 2 cases probably represent a distinct tumor entity, whose relationship with myoepithelial tumors and tumorigenic mechanisms exerted by the OGT-FOXO3 fusion remain to be studied.
: 软组织的肌上皮肿瘤在组织学上是不常见的肿瘤，其特征在于纺锤体至上皮样细胞排列成索、巢和/或网状图案，软骨粘液样至透明基质，并且在约一半的病例中遗传上涉及EWSR1 (在其他不太常见的基因中) 的重排。诊断通常需要免疫染色以确认肌上皮分化，最重要的是上皮标志物和S100蛋白和/或GFAP的表达。然而，有些情况下，形态令人联想到肌上皮肿瘤，而免疫表型不足。在这里，我们报告了2个非常相似的肌上皮瘤样肿瘤发生在年轻人手中。两种肿瘤边界清楚，由交替的细胞区域组成，这些区域具有苍白的嗜酸性透明基质和密集嗜酸性胶原沉积的散在脱细胞区。肿瘤细胞以上皮样细胞为主，呈索状或小巢状排列。空泡细胞环绕透明基质小球，局灶突出。还观察到由淡薄的梭形细胞和丰富的脉管系统组成的非透明纤维结节区的次要成分。部分区域存在血管周围同心梭形细胞增殖和血管周围玻璃样变。通过免疫染色，肿瘤细胞对CD34和上皮膜抗原 (病灶) 呈阳性，而对细胞角蛋白、S100、GFAP、p63、GLUT1和claudin-1大部分呈阴性。通过RNA测序，在病例1中鉴定了新的OGT-FOXO3融合基因，并在两种情况下通过逆转录聚合酶链反应和荧光原位杂交证实。这两个病例具有不同寻常的临床病理特征和新的融合，可能代表了一个不同的肿瘤实体，其与肌上皮肿瘤的关系以及由OGT-FOXO3融合产生的致瘤机制仍有待研究。
METHODS::Malignant thymoma can be invasive and may require radical resection. Here we present a case with phrenic nerve, right upper lobe, bilateral brachiocephalic vein, and superior vena cava involvement. Total venous reconstruction was performed with a cryopreserved aortic allograft by using the aortic root, ongoing transverse arch, and innominate arterial branch. The patient then had postoperative radiotherapy for a total of 63 Gy over 35 treatment days. Follow-up imaging demonstrated no evidence of recurrence and intermediate-term patency but with homograft calcification developing at 4 years.
METHODS:OBJECTIVE:To examine the CT and MRI characteristics of extraneural perineuriomas. MATERIALS AND METHODS:With IRB approval, our institutional imaging database was retrospectively reviewed for cases of pathologically proven extraneural perineuriomas. CT and MRI features were recorded, correlative imaging analyzed, and the electronic medical record cross-referenced. RESULTS:We identified ten patients [(seven males, three females, mean age 49.4 ± 18.3 years (range, 16-70 years)]. All cases were pathologically confirmed. Nine cases were conventional soft tissue extraneural perineuriomas, including one with "reticular" features and one with histologic features of malignancy; the tenth case contained admixed Schwann cells (hybrid perineurioma/schwannoma). Six out of ten patients underwent CT and ten of ten MRI evaluation. Nine out of ten MRIs were performed with IV contrast. Five lesions were subcutaneous, four intermuscular, and one intramuscular. Mean lesion diameter was 4.3 ± 2.7 cm (range, 0.9-10.2 cm). Nine out of ten lesions were well circumscribed; one had irregular margins. On CT, five of six were hypodense and one isodense compared to skeletal muscle. Most lesions were T1 isointense (5/10) or hypointense (4/10) and T2 hyperintense (7/10) relative to skeletal muscle, and demonstrated solid enhancement (6/9). There was no evidence of muscular denervation on any MRI exam, and a nerve of origin was identified in two out of ten cases. CONCLUSIONS:Extraneural perineuriomas have a distinctly different imaging appearance from intraneural perineuriomas, manifesting as rounded or ovoid soft tissue masses, without evidence of muscular denervation, and usually without an apparent nerve of origin. Because these features mimic other benign and malignant soft tissue lesions, including sarcomas, biopsy or excision is needed for definitive diagnosis.
METHODS::We present the case of a 46-year-old woman with a presumed diagnosis of multiple unprovoked pulmonary emboli. Her cardiorespiratory function continued to deteriorate despite optimal medical therapy. At surgical thrombectomy she was identified to have an intimal soft tissue lesion arising from the right pulmonary artery. This lesion was subsequently diagnosed as a pulmonary artery sarcoma, a rare neoplasm that carries a poor prognosis. It typically presents at an advanced stage with pulmonary vascular obstruction. Surgical debulking is the mainstay of therapy to restore ventilation perfusion mismatching and relieve right-sided heart strain. Median survival of these patients is 20 months with adjuvant chemoradiotherapy. At 9 months after surgery, she has undergone 6 cycles of chemotherapy and has stable disease with no metastases.