Benign retroperitoneal schwannoma in patient with antecedents of Willm's tumor in the infancy

We report a case of benign retroperitoneal schwannoma diagnosed casual in a 23 years old woman treated of a Wilm's tumour twenty years ago. A review of the on this unusual tumour is included.     

A huge malignant triton tumour

A 45 years old man presented with progressively increasing abdominal mass of two years duration. The mass had rapidly increased in size in the past five months. The patient was emaciated, with a grossly protuberant abdomen. The biopsy revealed fibrosarcoma. Extirpation of the tumour was contemplated. A huge 82 x 53 x 32 cm retroperitoneal mass was removed and the histopathology confirmed it as malignant triton tumour.     

Giant dedifferentiated retroperitoneal liposarcoma

A 72 years old lady presented with a gradually increasing abdominal mass for the last 25 years, with more rapid increase in the last 05 years. Examination revealed a large mass spanning the whole abdomen. CAT scan revealed a huge retroperitoneal tumour, with tortuous ureters passing through the tumour and three tiny nodules in the right lung field. A subtotal piecemeal excision of the tumour was done after stenting the ureters with single J stents. The excised tumour mass weighed 46 Kg. The patient had a prolonged ileus that recovered in 5 days. Histopathology revealed the mass to be dedifferentiated liposarcoma. The patient and the family were satisfied with the short-term outcome of the surgery. She was ambulating with little support and was able to manage her daily affairs.     

Rare case of retroperitoneal malignant Schwannoma

Malignant Schwannoma, recently renamed malignant peripheral nerve sheat tumor retroperitoneally localized, represents 0.01 of retroperitoneal tumours. A 41-year old woman, without pathological record--and without cutaneous neurofibromatosis--hospitalized for increased volume of the abdomen, without symptomatology, is diagnosed after the imaging and biological tests--without CT and RMN--with retroperitoneal tumour. The unusual size of tumour--6000 gr.--the macroscopic aspect suggesting malignancy (histopathologically and immunohistochemically confirmed), the relatively easy extirpation which, nevertheless, required caudal spleen- and pancreatectomy as well as transvers colectomy, the absence of proximity adenopathy and remote secondary determinations, the simple postoperative evolution represent overwhelming elements in this case. Two years after the operation, during which the patient was under chemotherapy, on a routine control we found a relapse at a distance from the initial tumour (primitive tumour ?), totally operable. For the time being, after almost five years from the first operation, there are no clinical, biological and imaging changes. Conclusions: the retroperitoneal space is quite enough for the development of large tumour masses, without symptomatology. The present case combines most characteristics of retroperitoneal neoplasms: large or very large size, quasi-absent symptomatology, difficulty in preoperative diagnosis, surgical tactics and techniques--quite often, the total extirpation of tumour mass led to the sacrifice of other organs within the limits of a justified risk--and unforeseeable evolution with relapses having the same characteristics.     

Limitations of surgery in the treatment of retroperitoneal sarcoma

A retrospective analysis was undertaken of 120 patients with retroperitoneal sarcoma referred to the Royal Marsden Hospital over a period of 20 years. The actuarial 5-year survival rate of all cases following referral was 29 per cent. On univariate and multivariate analysis the principal factors associated with an unfavourable prognosis were the presence of metastases, poor performance status at presentation, high tumour grade and incomplete excision of the primary tumour. The single most important factor affecting the ability to remove the primary tumour completely was multiple organ involvement. After apparently complete excision, however, the probability of local recurrence by 5 years was 85 per cent (95 per cent confidence interval 56-99). The prognosis of patients with retroperitoneal sarcoma is poor.     

Extragonadal germ cell tumor with "burned-out" phenomenon in the testis

The "burned-out" phenomenon in germ-cell neoplasias is defined by the presence of an extragonadal germ-cell tumour with no tumour at the testis level where a series of distinctive histological lesions can be detected indicative of the earlier presence of an already disappeared testicle tumoration. Extragonadal germ-cell tumours with "burned-out" phenomenon show better evolution than their primary counterparts and are treated similarly to primary tumours of the testis. Currently, in the presence of retroperitoneal tumoration, a scrotal ultrasound study with high frequency transducers can lead to a suspected picture of tumoral involution. This paper contributes one retroperitoneal seminoma with "burned-out" phenomenon in the homolateral testis in a 35-year old patient. Available clinical and radiological criteria were enough to reach a suspected diagnosis. Homolateral orchiectomy and biopsy of retroperitoneal tumoration were performed, rounding treatment up with polychemotherapy. Evolution was good with immediate complete response.     

Endoscopic treatment of bilateral pheochromocytoma in MEN 2A syndrome: case report and review of the literature

The benefits of laparoscopic adrenalectomy for single adrenal lesion have been well documented in literature; less experience though has been reported with simultaneous bilateral laparoscopic adrenalectomy. This operation is indicated in case of primary hypercortisolism caused by bilateral adrenocortical hyperfunction, Cushing's disease after failure of pituitary surgery, ectopic adrenocorticotropic hormone (ACTH) production by a tumour inaccessible for surgical intervention, and pheochromocytoma when it occurs bilaterally in case of multiple endocrine neoplasia type 2A and 2B. Different laparoscopic approaches have been described to perform this operation, such as the "anterior" approach (transperitoneal), the "lateral" approach (transperitoneal and retroperitoneal), and the "posterior" approach (retroperitoneal). We report a case of bilateral laparoscopic adrenalectomy in a 33 years old female affected with bilateral pheochromocytoma due to multiple endocrine neoplasia type 2A treated with a bilateral transperitoneal laparoscopic adrenalectomy and disease free after 18 months follow-up.     

Surgical management of retroperitoneal tumours: A 12-year review

Background: The aim of the present study was to determine the long-term outcome of patients who had undergone resection of retroperitoneal tumours. Methods: This was a retrospective review of 44 patients with preoperative diagnoses of retroperitoneal tumours, who had resections carried out between April 1996 and June 2008 at our institution. Results: Forty-four patients at our hospital underwent resection with curative intent for retroperitoneal tumours. Eight patients developed recurrences, and reoperations were carried out in these patients. Merely 23.1% of the patients underwent fine-needle aspiration, and of those patients, just 15.4% received the correct diagnosis for their retroperitoneal tumour. Liposarcoma was the most common tumour (31.1%). The overall mean largest diameter of the retroperitoneal tumours was 13.4 ± 8.8 cm, and the median largest diameter was 11.0 cm (range: 2–43 cm). No significant difference was found between the mean largest diameters of benign and malignant tumours (P = 0.08). Simultaneous surgical resection of adjacent organs was required in 46.1% of the patients. The overall survival at 5 years for patients with liposarcomas, other malignancies and benign tumours was 20%, 50% and 100%, respectively. The disease-free survival at 5 years for patients with liposarcomas was zero (P = 0.013), whereas, for other retroperitoneal malignancies and benign tumours, the disease free survival at 5 years was 38% and 100%, respectively. Conclusion: Surgical management of retroperitoneal tumours often requires a multidisciplinary approach. Long-term survival rates for patients with liposarcomas are relatively poor, and recurrences after surgical resection are common.     

后腹腔镜联合经尿道电切镜治疗肾盂输尿管肿瘤疗效分析

目的探讨后腹腔镜联合经尿道电切镜治疗肾盂输尿管肿瘤的疗效。方法48例肾盂输尿管肿瘤患者,男性29例,女性19例。年龄32～72岁,平均61.4岁;肾盂肿瘤31例,输尿管肿瘤17例。Ta期5例,T1期16例,T2期19例,T3期6例,T4期2例。48例均采用后腹腔镜联合经尿道电切镜作肾、输尿管全长加膀胱袖状切除。结果48例手术均获成功,平均手术时间134min;出血80ml,住院8.5d。随访平均32个月,发生膀胱癌3例,肺或骨转移4例,局部复发2例。复发率为18.75%。结论腹腔镜联合经尿道电切镜做肾、输尿管全段及膀胱袖状切除治疗上尿路上皮细胞癌安全有效,创伤小,康复快。     

Retroperitoneal dermoid presenting as an infected pancreatic cyst

Bacteroides fragilis infection of a juxtapancreatic benign cystic retroperitoneal teratoma in a 28 year old Chinese male is reported. Preliminary drainage was followed by excision. The radiological and pathological findings of this rare tumour are reported with emphasis on the differential diagnosis which includes inflammatory and neoplastic pancreatic cysts.     

Malignant paraganglioma of the retroperitoneum with lung metastases: a 13-year survivor after radical surgery

We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.     

睾丸成人型粒层细胞瘤伴肺和腹膜后淋巴结转移一例报告

报告1例睾丸成人型粒层细胞瘤伴肺和腹膜后淋巴结转移病例。患者,26岁,行患侧睾丸切除术,术后放疗并定期随访,术后5年出现肺和腹膜后淋巴结转移。睾丸成人型粒层细胞瘤具有恶性潜能,一旦出现远处转移,提示预后不良。     

Retroperitoneal Malignant Fibrous Histiocytoma

Authors review the case history and follow-up of a rare malignant fibrous histiocytoma patient, based on the relevant literary data. The tumour filled the retroperitoneum on the right side, in front of the right kidney. Intravenous urography and computer tomography revealed a 10×15 cm sized mass, suspect of being a kidney tumour. Upon surgery, the tumour was found to be a retroperitoneal malignant fibrous histiocytoma. In connection with the case, a brief review is given of the storiform type of malignant fibrous histiocytoma, regarding its aetiological, clinical and pathological aspects, the difficulties in diagnosis, as well as the therapeutic possibilities. Authors regard their case worthy of publication because of the retroperitoneal location and significant size of the tumour, and because of the unproven diagnosis prior to surgery. Even after 4 years the patient is symptom- and complaint-free, and CT has revealed no metastases.     

Primary retroperitoneal hydatid cyst: report of 2 cases and review of 41 published cases

This paper gives an overview of the literature between 2000 and 2010 on primary retroperitoneal hydatid cyst. We reported 2 cases of primary retroperitoneal hydatid cyst, and studies published in English literature on hydatid cyst developing in the retroperitoneal space were accessed via Pubmed and Google Scholar databases. Forty-one published primary retroperitoneal hydatid cyst cases were evaluated, and 2 patients (1 man, 78 years old; 1 woman, 75 years old) who presented with abdominal mass caused by retroperitoneal hydatid cyst were reported. Twenty-five of the patients were men (including our patient), and 18 were women; patients ranged in age from 3 to 80 years, and the median +/- standard deviation age was 41.37 +/- 20.4 years. On presentation, 72% of the patients complained of back or abdominal pain; 13.9% had urinary tract symptoms, and 65.1% were determined as having a palpable mass. Ultrasonography was performed on 93% of the patients, computed tomography was performed on 81.4%, magnetic resonance imaging was performed on 18.6%, and intravenous pyelography test was performed on 13.9%. The results of these tests showed a cystoid mass located on the left in 32.5% of the patients, on the right in 37.2%, and in the retrovesical area in 16.2%. Serologic tests determined 67.8% of the patients were indirect hemagglutination positive, and 71.4% were positive on enzyme-linked immunosorbent assay. As a surgical approach, total exision was performed on 55.8% of patients, partial cystectomy was performed on 39.5%, and 4.6% of patients underwent unroofing. If a cystic lesion is determined in the retroperitoneal area in a patient living in an area of endemic hydatid disease, a differential diagnosis of hydatid cyst should be considered. Clinical, radiologic, serologic, and histopathologic evaluations should be made for a differential diagnosis.     

Chemotherapy of advanced seminoma: clinical significance of radiological findings before and after treatment

The predictive significance of the mass detected following chemotherapy was assessed in 46 patients with advanced seminoma. Patients with residual viable seminoma in the post-chemotherapy operation specimen or who developed recurrent disease were regarded as chemotherapy failures. This group included 1 of 20 patients in whom the retroperitoneal masses were less than or equal to 10 cm2 3 to 4 weeks after chemotherapy and 4 of 15 patients whose residual masses were greater than 10 cm2. Four of 11 patients with mediastinal tumours achieved a complete remission (mediastinal masses less than or equal to 1 cm2). However, 2 of these 4 patients relapsed, as did 2 of the 4 who achieved a partial remission. In no case was the original size of the tumour significantly related to treatment failure. Three patients had residual lung masses; 1 of these contained histological evidence of viable tumour. In one-third of the irradiated relapse-free patients, slightly enlarged masses were visible on follow-up computed tomography scans taken several years after treatment, even in patients without tumour activity. There is a 25% risk of relapse in patients with advanced seminoma who have retroperitoneal masses greater than 10 cm2 following cisplatin-based chemotherapy. They should be followed up regularly for many years.     

腹膜后腹腔镜肾盂成形术治疗肾盂输尿管连接处梗阻的临床价值

目的:探讨腹膜后腹腔镜肾盂成形术治疗输尿管连接部(ureteropelvic junction,UPJ)梗阻的适应证、手术技巧及临床应用价值。方法:回顾分析腹膜后腹腔镜肾盂成形术治疗17例UPJ梗阻患者的临床资料。其中男11例,女6例,22～38岁,高位输尿管开口2例,UPJ完全闭锁4例,单纯重度肾积水7例,异位血管压迫4例,合并肾盂结石3例。结果:17例手术均获成功,手术时间80～240min,平均120min,出血50～130ml,平均85ml,术后平均住院8.5d(7～10d)。围手术期无并发症发生。随访3～12个月,UPJ吻合无狭窄,肾积水、肾功能均获改善。结论:基层医院行腹膜后腹腔镜肾盂成形术是治疗UPJ梗阻有效、安全的术式,可代替开放手术,作为UPJ梗阻患者的首选术式。     

Modified extended pancreatoduodenectomy: en bloc resection of the peripancreatic retroperitoneal tissue and the head of pancreas

Malignant periampullary tumours often invade into retroperitoneal peripancreatic tissues and a positive resection margin following pancreatoduodenectomy is associated with a poor survival. For complete extirpation of the tumour, en bloc resection of the pancreatic head with all retroperitoneal peripancreatic tissue is essential to achieve negative resection margin. A modified radical pancreatoduodenectomy technique that aims to resect all peripancreatic retroperitoneal tissue en bloc with the head of the pancreas is described. We have used this new technique in the last 30 consecutive cases of pancreatoduodenectomy with excellent results as presented in this paper. This technique allows complete en bloc resection of retroperitoneal peripancreatic tissues while preserving normal functional tissue. This technique's advantage is that the resection can be carried out without breaching the retroperitoneal tumour extension plane, thereby minimizing tumour cell spillage.     

Primary retroperitoneal mucinous cystadenoma-A case study and review of the literature

INTRODUCTIONPrimary retroperitoneal tumours of mucinous type are extremely rare and can be further sub-divided into benign, borderline or cystadenocarcinoma. Prompt diagnosis of retroperitoneal tumours is important as the majority are malignant.PRESENTATION OF CASEOur case describes a 30 year old woman, presenting with a 3 month history of intermittent right iliac fossa pain. Abdominal examination demonstrated a mass palpable in the right iliac fossa. Ultrasonography of the abdomen demonstrated a cystic mass with a magnetic resonance imaging (MRI) scan of the pelvis further defining the lesion. Laparoscopy was performed to further evaluate and ultimately remove the retroperitoneal mass. Macroscopic and microscopic examination reported mucinous epithelium of endocervical type with no evidence of invasion. Findings were consistent with primary retroperitoneal mucinous cystadenoma.DISCUSSIONThis is the 19th reported case of a benign primary retroperitoneal mucinous cystadenoma in the English literature. The origin of mucinous cystadenomas in the retroperitoneum is widely debated with multiple theories suggested. Diagnosis of retroperitoneal tumours is important but difficult as serological investigations, ultrasonography, computed topography and magnetic resonance imaging, although useful, cannot allow a confident diagnosis.CONCLUSIONPrimary retroperitoneal mucinous cystadenoma is a benign tumour, however because of the malignant nature of the majority of mucinous retroperitoneal tumours they should be considered in the differential of chronic abdominal pain despite their rarity.     

原发性腹膜后副神经节瘤5例报告并文献复习

目的：探讨腹膜后副神经节瘤的临床和病理特点、诊治方法及预后.方法回顾性分析2013年3月至2015年7月收治的5例腹膜后副神经节瘤患者的临床资料.其中男3例,女2例,年龄32~68岁,平均46岁;有高血压症状者3例,无症状体检发现者2例.5例均接受手术治疗,术后随访6个月~3年.结果5例患者均在腹腔镜下手术完整切除肿瘤,术中见肿瘤位于肾上极区3例,肾门区1例,肾下极区1例.术后病理证实为副神经节瘤.4例患者随访期间均未见复发与转移.2例高血压患者术后血压下降但未降至正常值范围,患者口服降压药辅助降压.1例术后1．5年肿瘤复发再次行手术切除瘤体.结论腹膜后副神经节瘤临床罕见,尽早手术切除是其最有效的治疗方法,术中应尽可能的完整切除肿瘤组织,避免肿瘤组织的残留,预后主要取决于肿瘤是否复发与转移.     

Extragonadal presentations of germ cell tumours

This study reviews our experience over the past 25 years with unusual presentations of germ cell tumours and emphasises the need to consider the diagnosis of germ cell malignancy in any male patient who presents under the age of 60 years with a retroperitoneal or mediastinal tumour reported to be an "undifferentiated carcinoma" of apparently unknown origin.