ObjectiveTo Summarize the clinical features of retroperitoneal ganglioneuroma.MethodsA total of 18 cases of pathologically confirmed retroperitoneal ganglioneuroma, who received surgery in The Affiliated Hospital of Xuzhou Medical University from May 2013 to July 2016, were collected to reviewe the clinical features, imaging findings, pathologic characteristics, treatments, and prognosis of retroperitoneal ganglioneuroma.Results① Clinical symptoms. Of the 18 cases, 8 cases were found by physical examination, and had no symptoms; 6 cases showed abdominal pain and discomfort; 3 cases had hypertension; 1 case had bulge and discomfort in the lower abdomen. ② Imaging performance. All of the 18 cases underwent color Doppler ultrasonography, 16 cases showed clear hypoechoic mass, and hypoechoic masses showed irregular shape in 2 cases; homogeneous echo in 12 cases and inhomogeneous echo in 6 cases could be seen. Seventeen cases underwent CT scan, and homogeneous low density in 14 cases, uneven density in 3 cases, scattered calcification in 3 cases could be seen. Fifteen cases underwent CT dynamic contrast-enhanced scan, 2 cases showed no significant enhancement both in dynamic and venous phase, 8 cases showed no significant enhancement in arterial phase, 11 cases showed heterogeneous enhancement in venous phase. Three cases underwent MRI scan, T1WI images of 3 cases showed uniform low signal; T2WI images showed high signal in 1 case, T2WI images showed high signal with moderate intensity in 2 cases. ③ Surgery and prognosis. Fifteen cases underwent laparoscopic retroperitoneal tumor resection,3 cases underwent open surgery, and all the tumors were completely resected. All cases had good postoperative recovery and no significant complication occurred. All cases were followed up for 6 to 32 months with a median of 20 months, no recurrence or distant metastasis was found during follow-up period.ConclusionsRetroperitoneal ganglioneuroma has nonspecific clinical manifestation. Imaging examinations are the most effective adjuvant ways preoperatively, but patholoy is still the gold standard of final diagnosis for ganglioneuroma. Radical tumor resection is the most effective therapy with satisfactory prognosis for ganglioneuroma.
ObjectiveTo summarize the predictive factors of recurrence of retroperitoneal sarcoma.MethodsThe literatures on the recurrence of retroperitoneal sarcoma at home and abroad were reviewed and analyzed.ResultsTumor size, margin negative resection, tumor grade, multifocal, and histological types were important predictors of the recurrence of retroperitoneal sarcoma. In addition, combined organ resection, diversity of tumor growth patterns, and tumor-free surgical techniques could also affect the recurrence of retroperitoneal sarcoma.ConclusionUnderstanding the predictive factors of the recurrence of retroperitoneal sarcoma is of great significance to guide surgeons to understand and recognize the disease, to reduce the recurrence of retroperitoneal sarcoma.
ObjectiveTo evaluate the clinical features of the retroperitoneal paralangliomas.
MethodClinical data of 34 patients with retroperitoneal paragangliomas who underwent surgery in our hospital between January 2005 and January 2015 were enrolled, all patients diagnosed by pathological examination.
ResultsClinical manifestation: hypertension (n=4) and headache (n=1) were the main symptoms in all 5 patients with functional tumor. Abdominal distension (n=14) or abdominal pain (n=11) were the initial symptoms in patients with nonfunctional tumor, gastrointes-tinal obstruction was observed in 1 patient, the other 3 patients were diagnosed accidently among the 29 patients with nonfunctional tumors. Blood and urine catecholamines increased notly in 5 patients with functional tumor, increased mildly in 2 patients with nonfunctional tumor; and remained at a normal level in the other patients. All the 34 patients underwent surgery, 4 patients with nonfunctional tumors who suffered from vessel wrapped needed tumor resection combining with main vessel resection or reconstruction, 1 of the 4 patients presented with vascular wall invasion. Two patients presented with lymph-vascular invasion of patients didn't received vessel resection or reconstruction. The mean value of diameter of these tumors was (9.5±5.1) cm (3.5-18.5 cm). Twenty four tumors had complete capsule, the other 10 had partial capsule; 32 tumors were well differentiated, and other 2 patients had apparent strange nucleus. Among the 34 patients, G1 tumors were confirmed in 14 patients and G2 tumors were confirmed in 20 patients. All the 34 patients were followed up with a mean value of follow-up time of 52 months (1-105 months). During the follow-up period, 6 patients suffered from recurrence and 6 patients died. The 1-year and 5-year survival rates were 86.0% and 81.5%, respectively.
ConclusionsRadical resection may be the only measure to cure retroperitoneal paragangliomas. Tumor combining with vascular resection or reconstruction provide a chance for patients with ‘unresectable tumors’.
【摘要】 目的 探討子宮頸巨大平滑肌瘤在其診斷及治療上的特殊性。 方法 對2007年10月-2010年3月收治的11例子宮頸巨大平滑肌瘤患者的臨床資料進行分析，對其發病率，診斷和手術治療進行評價。 結果 11例子宮頸巨大平滑肌瘤中黏膜下2例，腹膜后9例。術前9例出現誤診，其中誤診為盆腔包塊5例，子宮體肌瘤3例，子宮肉瘤1例。6例行經腹子宮全切加雙附件切除，2例行經腹子宮切除術，1例行經腹肌瘤挖除術，1例行經陰道肌瘤摘除術，1例行經腹肌瘤挖出加宮頸殘端切除術。 結論 子宮頸巨大平滑肌瘤由于其位置的特殊性，尤其是凸向腹膜后的肌瘤，由于盆腔器官被擠壓，使盆腔解剖結構發生改變，術前易被誤診。且手術過程中易出現損傷及出血，因此術前估計充分，術中仔細認清各器官解剖關系，可有效地減少術中損傷和控制出血。【Abstract】 Objective To investigate the particularity of diagnosis and treatment for giant uterine cervical leiomyoma. Methods We analyzed the clinical data of 11 patients with giant uterine cervical leiomyoma who were admitted in our hospital from October 2007 to March 2010. The incidence, diagnosis and surgical treatment of the disease were evaluated. Results Of the 11 cases, nine were retroperitoneal leiomyoma and two were submucous leiomyoma. There were nine misdiagnosed cases before operation, including five diagnosed as pelvic mass, one as uterine sarcoma and three as uterine corpus leiomyoma. Six patients underwent abdominal hysterectomy and bilateral salpingo-oophorectomy; two underwent abdominal hysterectomy; one underwent abdominal myomectomy; one underwent transvaginal myomectomy; and one underwent abdominal myomectomy with excision of cervical stump. Conclusion The giant uterine cervical leiomyoma is easily misdiagnosed preoperatively due to its special anatomic site. A good example is the retroperitoneal leiomyoma in which the pelvic anatomic structure is changed because of the extrusion of the tumor on other pelvic organs. Furthermore, injuries and bleeding often happen during the operation. Consequently, sufficient preoperative assessment and clearly identifying regional anatomical relations can effectively reduce the damage and bleeding during the operation.
ObjectiveTo understand the pathogenesis and the research progress of comprehensive treatment of primary retroperitoneal liposarcoma (PRLPS) and to provide evidence for clinical diagnosis and treatment.MethodThe recent literatures on the pathological classification, pathogenesis of PRLPS, and comprehensive treatment including the surgery, radiotherapy, chemotherapy, and molecular targeted therapy were reviewed.ResultsThe pathological types of PRLPS were highly differentiated, dedifferentiated, mucoid/round cell, polymorphic, and mixed. The main molecular pathogenesis was the synergistic effect of MDM2 with related genes, abnormal expressions of c-myc gene and microRNAs, Prune-nm23-H1 mechanism, and abnormal protein products of FUS-CHOP fusion gene which regulated the growth of tumor. The treatment of PRLRS included the radical resection, extended resection, and palliative resection combined with radiotherapy, chemotherapy, and molecular targeted therapy.ConclusionsPRLPS is a rare malignant tumor with high recurrence rate, but early diagnosis and treatment are difficult. With the further study of the molecular mechanism of PRLPS, the treatment of PRLPS has been transformed into a comprehensive treatment based on surgery, adjuvant radiotherapy and chemotherapy, and molecular targeted therapy.
ObjectiveTo explore the application value of multidisciplinary collaborative team (MDT) model in retroperitoneal tumors involving large vessels.MethodsThree cases of retroperitoneal tumors involving great vessels admitted to Xiang’an Hospital of Xiamen University in 2019 were retrospectively analyzed. With the support of 3D visual reconstruction and virtual reality (VR) technology, we performed MDT discussion and three cases received treatment of surgery, intervention, and targeted therapy.ResultsCase 1 was discussed by MDT and concluded that, based on CT examination, 3D reconstruction, and VR virtual image results, the tumor on the right side was determined to be completely resectable. The left tumor was judged to be unresectable, and the proposed treatment plan was right metastatic tumor resection + left metastatic tumor radiofrequency ablation. After surgery case 1 had been followed up for 6 months. The symptoms of diarrhea were significantly improved. CT reexamination showed that liver lesions and left retroperitoneal lesions were the same size and the condition was stable. After discussion by MDT, radiofrequency ablation around the tumor was proposed for case 2. This case was followed up for 3 months after surgery, and CT reexamination showed no new lesion in retroperitoneum. After MDT discussion, we concluded that arteriovenous fistula of case 3 had no indications for surgery, and proposed interventional combined with targeted therapy. After treatment, the tumor was found to be smaller after reexamination in 8 months than before treatment, and the efficacy was evaluated as partial remission. The follow-up was continued.ConclusionThe future development trend of retroperitoneal tumor therapy involving great vessels is to evaluate each patient’s condition under the MDT mode by using 3D visual reconstruction and VR technology, and to formulate the individualized treatment plan of operation combined with other treatments.
ObjectiveTo discuss the treatment of retroperitoneal tumors infiltrating celiac trunk.
MethodFifteen cases of retroperitoneal tumors who underwent surgery in our hospital between December 2014 to December 2015 were reviewed and analyzed.
ResultsAll of the 15 cases had underwent operation, and the blood loss was 300-2200 mL (mean of 600 mL), operation time was 125-600 minutes (mean of 240 minutes). Length of stay in cases of celiac trunk resected was 10-23 days (mean of 15.1 days), and length of stay in cases of celiac trunk nonresected was 15-132 days (mean of 35.2 days). Thirty cases of them were complete resection, and 2 cases were mostly resection. Eight cases of them did not injure celiac trunk, 1 case suffered from complication, 7 cases injured celiac trunk and all of them suffered from complication. There were significant statistical difference in incidence of complications (χ2=8.14, P=0.01) in cases of celiac trunk injury or not. All the cases were followed up for 3-13 months (mean of 6.3 months), 2 cases recurred during the follow-up period, 1 case was leiomyosarcoma, and another 1 was malignant fibrous histiocytoma who underwent celiac trunk resection.
ConclusionsWhen retroperitoneal tumors infiltrates celiac trunk, we can resect the celiac trunk, but we must follow a certain principle.
【摘要】 目的 評價大分割適形放射治療對腹膜后軟組織腫瘤術后患者的治療作用。 方法 對1998年10月-2003年4月收治的16例腹膜后軟組織急性腫瘤術后患者行大分割適形放射治療，設計臨床靶區等效生物劑量為55～62 Gy，觀察放療后2、5年局部控制率、生存率和無病生存情況。 結果 2、5年局部控制率較未行放療患者明顯提高并和其他放射治療方式達到較高治療劑量者近似；遠期生存無改善，無病生存率較未行放療患者有提高。 結論 大分割適形放射治療方式對腹膜后軟組織急性腫瘤術后患者有較好的局部控制作用，無瘤生存率有提高，遠期生存無改善，無嚴重的遠期放療后遺癥。【Abstract】 Objective To observe the effect of high-dose three-dimensional conformal radiotherapy combined with surgery on primary retroperitoneal soft tissue sarcoma. Methods A total of 16 patients with primary retroperitoneal soft tissue sarcoma underwent high-dose three-dimensional conformal radiotherapy after sarcoma excision from October 1998 to April 2003. The biologically effective dose was 55-62 Gy for CTV. The local control rate and long-term survival rate and disease free survival after 2 and 5 years were observed. Results The local control rate obviously raised in these patients after 2 and 5 years; but the long-term survival rate didn’t improve and the disease free survival improved in these patients compared with those wasn’t radiated. Conclusion High-dose three-dinensional comfomal radiotherapy is effective on the patients with retroperitoneal soft tissue sarcoma in local control rate and disease free survival, but long-term survival rate is not improved and the side-effect is not serious.
Objective To investigate the application and clinical efficacy of orthotopic autologous renal transplantation combined with inferior vena cava (IVC) resection and reconstruction in retroperitoneal tumor. Methods The clinical data of a patient with complex retroperitoneal tumor was analyzed retrospectively. Abdominal CT examination showed that the tumor originated from IVC and invaded the retrohepatic IVC and bilateral renal vein trunks. Intraoperative ultrasound exploration revealed mechanized thrombosis in the IVC and bilateral renal vein trunks. After blocking the left renal vein, no significant hemodynamic changes were seen before and after intraoperative ultrasound exploration and contrast-enhanced ultrasound. Results After complete removal of the left renal vein and suture of the severed end, the right renal vein was successfully reconstructed with an orthotopic autologous right kidney graft combined with IVC resection after removal of the mechanized thrombus. The patient recovered well after surgery, and a repeat CT showed that the reconstructed artificial IVC was patent, and the color Doppler ultrasonography of both kidneys showed good perfusion and no obstruction of return. The patient was given oral rivaroxaban anticoagulant therapy after operation, and discharged at 19 days after operation. The postoperative pathological findings suggested inferior vena cava smooth muscle sarcoma. Conclusion Orthotopic autologous renal transplantation combined with IVC resection and reconstruction for complex retroperitoneal tumor is safe and feasible, and the left renal vein can be ligated and dissected intraoperatively, but a comprehensive evaluation with intraoperative ultrasound (imaging) is required.
Schwannoma originating in the retroperitoneal space is relatively rare. The author reported a case of retroperitoneal Schwannoma confirmed by surgery and pathology, presented the typical CT and MRI manifestations of Schwannoma——“target signs”, described the pathophysiological mechanism and image differential diagnosis of the disease, so as to strengthen readers’ understanding of the typical signs of Schwannoma and improve the correct diagnosis rate of the disease.
