后腹腔镜肾上腺肿瘤切除术22例报告

目的探讨后腹腔镜肾上腺肿瘤切除的方法和临床应用价值。方法采用腹膜后途径腹腔镜下完成肾上腺肿瘤切除术22例。记录手术时间、估计出血量、术后并发症和术后住院时间等临床资料,并对结果进行分析。结果22例均获成功。手术时间45．125min,平均70min。失血量40～360ml,平均70ml,所有患者术中、术后均未输血,无严重并发症。术后恢复顺利,术后住院5—9d,平均8d。结论腹膜后腹腔镜肾上腺切除手术具有对组织损伤小、出血少,术后恢复快,并发症少,术后患者康复快,并发症少等优点,应成为肾上腺良性疾病的首选治疗方法。     

Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm which appears to have predominancy for young, frequently Asian, women. The neoplasm is composed chiefly of HMB-45-positive epithelioid cells with clear to granular cytoplasm and usually showing a perivascular distribution. These tumors have been reported in various organs under a variety of designations. Malignant PEComas exist but are very rare. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. We report a retroperitoneal PEComa discovered during emergency surgery for abdominal pain in a 28-year-old Asian woman. The postoperative period was complicated by chylous ascites that was initially controlled by a wait-and-see policy with total parenteral nutrition. However, the chyle production gradually increased to more than 4 l per day. The development of a bacterial peritonitis resulted in cessation of production of abdominal fluid permitting normal nutrition without chylous leakage. Effective treatment for this rare complication of PEComa is not yet known; therefore, we have chosen to engage in long-term clinical follow-up.     

Benign retroperitoneal schwannoma presenting as colitis： A case report

We report a case of a patient presenting with clinical, radiological and endoscopic features of colitis due to a compressive left para-aortic mass. Total open surgical excision was performed, which resulted in complete resolution of colitis. Histopathology and immunohistochemistry revealed benign retroperitoneal schwannoma. These neural sheath tumors rarely occur in the retroperitoneum. They are usually asymptomatic but as they enlarge they may compress adjacent structures, which leads to a wide spectrum of non-specific symptoms, including lumbar pain, headache, secondary hypertension, abdominal pain and renal colicky pain. CT and MR findings show characteristic features, but none are specific. Schwannoma can be isolated sporadic lesions, or associated with schwannomatosis or neurofibromatosis type Ⅱ （NF2）. Although they vary in biological and clinical behavior, their presence is, in nearly every case, due to alterations or absence of the NF2 gene, which is involved in the growth regulation of Schwann cells. Both conditions were excluded by thorough mutation analysis. Diagnosis is based on histopathological examination and immunohistochemistry. Total excision is therapeutic and has a good prognosis. Schwannomatosis and NF2 should be excluded through clinical diagnostic criteria. Genetic testing of NF2 is probably not justified in the presence of a solitary retroperitoneal schwannoma.     

Primary solitary fibrous tumor (SFT) in the retroperitoneum

BACKGROUND: Solitary fibrous tumor (SFT) is an infrequent but distinct neoplasm, which generally arises from submesothelial connective tissue in the pleura. SFT is rarely recognized in extrathoracic sites, and histologically identical conditions have also been reported in the retroperitoneum, although their pathophysiology has not been extensively investigated. METHODS: We present four cases of primary SFT in the retroperitoneum, and review 37 similar cases in the previous literature. RESULTS: About 40% of patients were asymptomatic, and 19.2% and 15.4% presented with an abdominal mass and urinary symptoms, respectively. The tumor size ranged between 2 and 26 (mean 9.1) cm. Sixty-three percent of tumors showed nonspecific development with haphazard distribution of bland short spindle or polygonal cells with or without collagenous bundles and stromal hyalinization. In 22.0%, hemangiopericytomatous appearance was seen. About 15% of cases showed histologically malignant characteristics. The tumor cells were immunoreactive for vimentin in all cases, CD34 in 91% and Bcl-2 in 86%. All tumors were excised, and in 85.4% of cases, tumors did not recur postoperatively for 6 to 48 months. No significant difference was found between the recurrence rate of histologically benign and malignant cases. Cases positive for both CD34 and Bcl-2 had no recurrence. CONCLUSIONS: The identification of SFT in the retroperitoneum is of importance because histopathological indicators of malignancy are not necessarily associated with clinical malignant potential in many cases of retroperitoneal SFT. Retroperitoneal SFT showing typical pathological features with expression of CD34 and Bcl-2 is associated with a favorable outcome following excision.     

A huge lymphovenous malformation in the retroperitoneum

Vascular malformations are localized or diffuse errors of embryonic development resulting in collections of abnormal vessels with normal endothelium. Many authors have pointed out the confusing terminology of vascular anomalies that has led to improper diagnosis and therapy. This still remains confused in clinical practice, especially in cases occurring at rare regions or presenting atypical radiologic findings. We present a huge retroperitoneal lymphovenous malformation, which exhibited unusual radiologic findings and was difficult to confirm the diagnosis that occurred in a 12-year-old male.     

Retroaortic left renal vein: a report of six cases

Summary Six cases of retroaortic left renal vein have been studied, two in corpses and four by means of Computed Tomography. Any previous pathology has been rejected. The total number of dissected specimens was 116, whereas the patients with non-pathological retroperitoneum studied by CT numbered 170. In our six cases, the left renal vein formed a single trunk of considerable size, retroaortic position and directed obliquely towards the inferior vena cava.

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Veine rénale gauche rétro-aortique : a propos de six cas

Résumé Six cas de veines rénales gauches rétro-aortiques sont étudiés ; deux découverts de façon systématique lors de nécropsies et quatre lors d'examens tomo-densitométriques. Il n'existait pas de pathologie associée. 116 corps ont été disséqués, 170 patients dont la région rétropéritonéale ne présentait pas d'anomalies, ont été étudiés par examen scannographique. Dans les six cas, la veine rénale gauche se présente comme un gros tronc unique en position rétro-aortique oblique vers la veine cave inférieure.

     

Rare Coincidence of Familial Adenomatous Polyposis and a Retroperitoneal Fibromyxoid Sarcoma: Report of a Case

Purpose Familial adenomatous polyposis is an autosomal-dominant inherited disease with development of as many as thousands of adenomas within colon and rectum. All untreated patients will develop colorectal adenocarcinoma. A variety of extracolonic manifestations can occur, although malignant tumors are rare. An association of familial adenomatous polyposis and sarcomas was reported in a few cases only. Methods We present the exceptional case of a 24-year-old male with genetically verified familial adenomatous polyposis (deletion of 10 base pairs at position 228–237 of exon 15A). The patient underwent prophylactic subtotal proctocolectomy and ileal-pouch rectal anastomosis in 2003. Two years later, an obstruction of the left ureter caused by a retroperitoneal mass was diagnosed. Results Histopathologic findings after complete tumor resection showed a low-grade fibromyxoid sarcoma. CT scan and clinical follow-up through 15 months postoperatively revealed no recurrent tumor growth. Conclusions To our knowledge, this is the first reported case of familial adenomatous polyposis with metachronous retroperitoneal fibromyxoid sarcoma. Proctocolectomy or total colectomy and complete tumor resection is the treatment of choice in this case. In addition to more common semimalignant retroperitoneal desmoid tumors in familial adenomatous polyposis patients, a malignant soft-tissue tumor also has to be considered for differential diagnosis. Presented at the Cancer Congress of Saxony-Anhalt, Magdeburg, Germany, March, 23 to 24, 2007. Reprints are not available.     

Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment

PURPOSE: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. METHODS AND MATERIALS: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. RESULTS: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. CONCLUSIONS: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes.     

Computed tomography and ultrasound of renal and perirenal diseases in infants and children

Forty-seven infants and children had excretory urography, ultrasound, and computed tomography with the renal and perirenal areas as the primary regions of interest. Ultrasound is an excellent screening procedure and is often diagnostic especially in renal cystic disease. Computed tomography best demonstrated the extent of renal injury and best delineated the geography, character, and extent of solid renal and perirenal neoplasms (screened by ultrasound). Excretory urography in these patients was at times incorrect, often underestimated the extent of the pathologic process, and is often unnecessary.