- 作者列表："Gennaro N","Marrari A","Renne SL","Cananzi FCM","Quagliuolo VL","Di Brina L","Scorsetti M","Pepe G","Chiti A","Santoro A","Balzarini L","Politi LS","Bertuzzi AF
:Rhabdomyosarcoma (RMS) represents more than 50% of paediatric soft tissue tumours. Conversely, it is extremely rare among adults, where it shows peculiar biological and clinical features that are still poorly investigated. RMS patients should be referred to a Sarcoma Centre, where the contribution of experienced radiologists plays a relevant role in the diagnostic assessment of the disease, including precise localisation, staging, image-guided biopsy, response evaluation after treatment and follow-up. Besides CT and MRI, hybrid imaging including positron emission tomography (PET)/CT and PET/MRI are giving an increasing contribution to provide functional insights about tumour biology and to improve the diagnostic accuracy of the imaging work-up. This review paper provides a revision of the pathology, clinical and radiological features of adult RMS, with a particular focus on the growing role of hybrid PET-based imaging.
: 横纹肌肉瘤 (RMS) 占儿科软组织肿瘤的50% 以上。相反，它在成人中非常罕见，在那里它显示出特殊的生物学和临床特征，但仍然没有得到很好的研究。RMS患者应转诊至肉瘤中心，有经验的放射科医生的贡献在疾病的诊断评估中起相关作用，包括精确定位、分期、图像引导活检、治疗后的反应评估和随访。除了CT和MRI之外，包括正电子发射断层扫描 (PET)/CT和PET/MRI的混合成像正在提供越来越多的贡献，以提供关于肿瘤生物学的功能见解并提高成像检查的诊断准确性。这篇综述论文提供了成人RMS的病理学，临床和放射学特征的修订，特别关注基于混合PET的成像的日益增长的作用。
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.