Retroperitoneal giant schwannoma: a case report and review of the literature

Schwannomas are rare tumours that originate in the neural sheath and account for only a small percentage of all retroperitoneal tumours. They are usually solitary, circumscribed and encapsulated lesions eccentrically located on proximal nerves or spinal nerve roots. Presentation is typically varied and non-specific, ranging from abdominal pain, an abdominal mass or an incidental finding. The preoperative diagnosis is difficult and laboratory tests are usually unremarkable. We report the case of a 66-year-old female presenting with abdominal pain in her left flank and with an ultrasonographic diagnosis of a left kidney mass. She was diagnosed as suffering from a giant retroperitoneal schwannoma after surgical exploration and complete excision. The role of CT scan and CT-guided needle biopsy is emphasised, in that ultrasonography and fine needle aspiration alone do not provide sufficient information regarding aetiology and malignancy. Radical surgical excision is curative but recurrences may occur. Careful follow-up is needed.     

Surgical management of large desmoid tumour of the anterior abdominal wall

Desmoid tumours are uncommon. They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge. Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin. The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall. We report our experience in treating large desmoid tumours of the anterior abdominal wall. Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues. This led to a considerable abdominal wall defect. The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh. All three patients recovered well with no immediate or late postoperative morbidity. Follow-up until December 2006 has not revealed any tumour recurrence or hernia development. Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours. Closure under tension of the peritoneum did not seem to produce any morbidity.     

Primary retroperitoneal sarcomas

A retrospective analysis of 20 adult patients with primary retroperitoneal sarcomas demonstrated that most patients presented with pain and a palpable abdominal mass. Liposarcomas and leiomyosarcomas were the most common tumors. Seven of the 20 patients (35 percent) had complete tumor excision. Operative morbidity was 18 percent with no mortality. After complete resection, the 5 year survival rate was 43 percent. Thirteen patients underwent partial excision of tumor, with an operative morbidity rate of 29 percent and a mortality rate of 15 percent. No patient in our series lived more than 2 years after only partial excision. Anatomic restrictions to wide resection and local recurrence were the most important factors determining survival. Aggressive, complete surgical resection in treating primary retroperitoneal sarcomas is strongly recommended.     

Effect of the method of biopsy on the prognosis of and reliability of receptor assays in patients with operable breast cancer

A comparison has been made of the survival of contemporary patients treated by mastectomy in whom the original diagnosis was made by either Tru-cut biopsy or by excision biopsy. After a minimum of 5 years of follow-up there was no difference in survival between those biopsied by either method. When subdivided by tumour size again no differences emerged. Among those patients in whom the original Tru-cut biopsy was false negative there was no difference in survival compared with those treated by excision biopsy. This suggests that Tru-cut biopsy is a safe method for obtaining a histological diagnosis in operable breast cancer. Furthermore, when the receptor status of the tumours from the two groups was determined, there was no difference in the percentage of oestrogen receptor positive tumours, nor in the mean receptor value. However, there were more progesterone receptor positive tumours among the Tru-cut group, suggesting that a better tumour sample could have been submitted for analysis. This may be yet another advantage for the use of Tru-cut biopsy for the diagnosis of breast cancer.     

Surgical strategy in primary retroperitoneal tumours

Sixty-nine patients with primary retroperitoneal tumours (17 benign, 52 malignant including 4 malignant tumours of uncertain origin) were reviewed to determine the best form of surgical strategy. Total resection was performed in 88 per cent of benign cases and in 65 per cent of malignant cases. In 62 per cent of the total resections for malignant tumours, en bloc excision included adjacent organs or anatomical structures. Operative mortality rate (in terms of the total number of operations performed) was 5 per cent. Postoperative complications occurred in 14 per cent and recurrences in 35 per cent. The overall 5-year survival rate was 67 per cent in patients with totally resected tumours and zero in patients whose tumours were treated by partial resection or biopsy. An aggressive surgical approach aimed at total excision of the tumour is the best form of therapy currently available. In the totally resected retroperitoneal tumour, the use of adjuvant radiotherapy and/or chemotherapy depends on the grade of the malignancy and clearance as assessed histologically. Careful follow-up based on the use of computerized axial tomography and ultrasound allows early identification of recurrence at a stage when the recurrence is amenable to total resection.     

Improved survival following complete excision of retroperitoneal sarcomas.

Charts and slides of 47 patients with primary retroperitoneal sarcomas (excluding pediatric rhabdomyosarcoma) were reviewed to determine clinical presentation, histologic features, extent of surgical resection, operative morbidity and mortality, use of radiation and/or chemotherapy, and survival data. Most patients presented with pain and a palpable mass. Leiomyosarcomas and liposarcomas were the most common tumors. Eighteen of the 47 patients (38%) had complete tumor excision; 68% required resection of adjacent organs. Operative morbidity was 33% with no mortality. After complete resection, the disease-free 5-year survival was 50% and the overall survival was 70% at 5 years; 10-year disease-free survival was 25% with an overall 58% survival at 10 years. Eleven patients (61%) developed recurrent disease with a median interval of 5 years following complete excision. Six patients received adjuvant radiation and/or chemotherapy with four remaining disease-free from 46 to 61 months. Eighteen patients underwent partial excision of tumor and 11 patients underwent biopsy only; these groups had similar survival curves with only 4% alive at 5 years. Their operative morbidity was 18% and mortality was 7%; median time to clinical evidence of tumor progression was 12 months. Sixty per cent of these patients received therapeutic radiation and/or chemotherapy, but their survival was the same as those undergoing surgery alone. These data emphasize the importance of an aggressive surgical approach in the treatment of retroperitoneal sarcomas. Complete tumor resection and total excision of recurrences will allow many patients long-term survival.     

Major vessel excision in retroperitoneal lymph node dissection.

Retroperitoneal malignant tumours, both primary and metastatic, may involve surrounding structures such as the aorta and vena cava, making complete tumour excision difficult. En bloc resection of major blood vessels should be considered in such cases. The authors describe three patients who underwent excision of major blood vessels with retroperitoneal lymph node dissection. Two patients had aortic resection with placement of a Dacron tube graft, and one had excision of the vena cava from above the renal vessels to the level of the common iliac veins with distal venous ligation. The low complication rate confirms the feasibility of excising major blood vessels to accomplish complete retroperitoneal lymphadenectomy.     

Diagnostic and therapeutic approaches to carotid body tumours: report of three cases and review of the literature

BACKGROUND: Carotid body tumour is a rare neoplasm of the carotid body. Three cases of carotid body tumour presenting as a painless progressive mass in the neck region are reported here. A review of the relevant literature regarding carotid body tumours is also presented. METHODS: Angiographic features were diagnostic of carotid body tumour and complete surgical excision was done. RESULTS: There was no mortality and minimum morbidity. There were no malignant tumours. All three patients belong to the high-altitude area of Himachal Pradesh. CONCLUSIONS: A high degree of clinical suspicion of upper posterior triangle neck masses and an accurate diagnostic work-up are needed for operative planning.     

Can preoperative factors predict for residual malignancy after breast biopsy for invasive cancer?

The presence of malignancy at the resection margins of a malignant breast biopsy requires difficult therapeutic decisions about whether a re-excision biopsy is necessary. The aim of this study was to determine the factors predisposing to the involvement of the resection margins in 280 women undergoing breast biopsy for invasive malignancy from a single breast screening practice. Resection margins were assessed independently by a single pathologist who noted either the presence of tumour at the margins of the biopsy specimen or in the shavings taken from the biopsy cavity. Resection margin involvement (RMI) occurred in 113 patients. Mammographic microcalcification (MM) was seen in 87 women with invasive cancer and RMI occurred in 53 (61%) compared with 60/193 invasive cancers without MM (P < 0.001). If RMI was present the patients underwent a second procedure to ensure complete tumour excision, and 68% of re-excision specimens from tumours with MM and 36% of tumours without MM contained residual malignancy (P < 0.005). Statistical analysis demonstrated that these observations were independent of tumour size, grade, type, and axillary node status. The presence of mammographic microcalcification therefore indicates that wider than usual surgical resection margins should be taken.     

恶性肿瘤切除后巨大腹壁缺损的修复

目的 探讨原发于腹壁或侵犯腹壁的恶性肿瘤切除术后巨大腹壁缺损的修复方法 .方法 本组20例,腹壁恶性肿瘤12例,其中腹壁横纹肌肉瘤9例、恶性纤维组织细胞瘤3例;腹膜后及腹腔恶性肿瘤侵犯腹壁8例,其中腹膜后恶性肿瘤3例、横结肠癌1例、升结肠癌右半结肠切除术后2～3年局部复发3例、肾癌术后5年腹壁种植复发1例.采用自膨式聚丙烯和膨化聚四氟乙烯复合补片行修复手术.观察术后并发症、修复成功率.结果 术后一期愈合20例,无皮下积液,无切口感染、裂开和切口疝发生,未见修补材料与肠管粘连,修复成功率100%.随访20例,随访时间6～18个月,平均随访(9.3±3.4)个月.补片与腹壁相容性良好,无局部炎症反应;均未发现材料与肠管粘连,无切口疝形成,腹壁修补区未见肿瘤复发.结论 自膨式聚丙烯和膨化聚四氟乙烯复合补片具有抗张力强度大、良好的组织相容性、修补术后并发症少等特点,是一种良好的肿瘤切除后腹壁缺损修复材料.     

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.     

Surgical management of carotid body tumours: a 24-year surgical experience

BACKGROUND: Carotid body tumours (CBT) are rare tumours, best treated by complete surgical resection. However, there is no uniform agreement on the method of resection. The aim of this study was to review our 24 years' experience of meticulous subadventitial excision of CBT. METHODS: A retrospective study, from March 1980 to September 2004 of patients with CBT was undertaken, detailing presentation, diagnosis and treatment and postoperative complications. RESULTS: Twenty-five patients (six men and 19 women) with an age range of 23-72 years had been operated on, and all were treated by surgical excision. All patients had neck mass. No patient had a positive family history. Angiography was the main method of diagnosis. All of the patients had unilateral tumours. There was no postoperative permanent neurological deficit. Temporary neurological problems developed in only four patients. External carotid artery was ligated in three patients to facilitate excision of the tumour. Surgical care limited blood loss to an average of 480 mL. CONCLUSIONS: Subadventitial excision, carried out meticulously, allowed complete resection to be achieved in all of the patients with minimal morbidity and no surgical mortalities. This method is therefore recommended. Facilities for shunting and arterial repair should always be available.     

Percutaneous biopsy of adrenal and extra-adrenal retroperitoneal lesions: beware of catecholamine secreting tumours!

BACKGROUND: Many patients undergo abdominal imaging for non-specific symptoms. An increasing number of these patients are discovered to have incidental adrenal or retroperitoneal tumours. Approximately 5% of all incidentally detected adrenal lesions are phaeochromocytomas and 25% of phaeochromocytomas are discovered during imaging studies for unrelated disorders. 10% of phaeochromocytomas are extra-adrenal. METHODS: Retrospective case notes review of three patients with adrenal/retroperitoneal lesions who had percutaneous biopsy before biochemical testing and tertiary referral. FINDINGS: Adrenal/retroperitoneal lesions are still being biopsied without prior biochemical testing. One patient with phaeochromocytoma had a critical event. The others were found to have a phaeochromocytoma and a ganglioneuroma. CONCLUSIONS: The possibility of a catecholamine secreting tumour should be considered in adrenal and extra-adrenal retroperitoneal lesions. If a biopsy is planned, rarely required in adrenal lesions, phaeochromocytoma must be excluded by biochemical testing prior to the biopsy to avoid potential life threatening complications.     

Thoraco-abdominal approach to large retroperitoneal tumours

OBJECTIVES: To evaluate the thoraco-abdominal approach for resection of retroperitoneal tumours, as this approach is rarely used because, although exposure is excellent, morbidity is presumed to be increased. PATIENTS AND METHODS: From October 2003 to September 2005, 21 patients (six female, 15 male), aged 14-76 years, underwent resection of very large and/or T4 retroperitoneal tumours through a thoraco-abdominal approach. RESULTS: In 16 (76%) patients tumour resection was complete. There were no significant complications during surgery. After surgery, there were complications in six patients (29%), in four of whom there was no long-term impairment. One patient died at 75 days after surgery from a complicated retroperitoneal haematoma. The mean (range) estimated blood loss was 2883 (50-20 000) mL, the intensive-care unit stay was 3.85 (0-30) days and the intermediate-care unit stay 2.6 (0-9) days. With a mean follow-up of 9.6 (1-19) months, 15 patients (72%) are recurrence-free, two (10%) have progressive disease, and four (19%) have died from malignancy. CONCLUSIONS: The thoraco-abdominal approach permits excellent exposure of the retroperitoneum for large and/or T4 tumours, allowing radical surgery in cases considered otherwise inoperable. Additional advantages are the possibilities of early vascular control and easy surgical extension of the procedure. These facts, combined with the reasonable morbidity found in our series, support the integration of the thoraco-abdominal approach in the regular options for urological surgery.     

Retroperitoneal schwannoma

BACKGROUND: Retroperitoneal schwannomas are rare, benign tumors. The aim of this study is to present our surgical experience with 7 such tumors. METHODS: Between 1989 and 2004, 7 patients with pathologically proven retroperitoneal schwannomas were reviewed retrospectively. RESULTS: There were 6 male patients and 1 female patient, with a mean age of 43 years (range, 23 to 58 years). Two patients were symptomatic and presented with abdominal discomfort, and none of the patients suffered from von Recklinghausen's disease. All the patients underwent computed tomography scanning, which showed a heterogenous retroperitoneal mass, 4 of which were thought to arise from the adrenals. In 2 patients, calcification was seen in the tumors. All 7 of the patients had a preoperative diagnosis of a retroperitoneal tumor including 3 patients who were thought to have adrenal neoplasms (1 patient had a diagnosis of an adrenal neoplasm excluded on magnetic resonance imaging). Laparotomy and complete excision of tumors were performed in all the patients, and there was no morbidity or mortality. The schwannomas had a mean maximum diameter of 7.3 cm (range, 4 to 14 cm), and they were all benign. At a mean follow-up of 17 months (range, 3 to 48 months) postresection, all the patients remained free from recurrence. CONCLUSION: Retroperitoneal schwannomas are rare tumors that are difficult to diagnose preoperatively. Radiologic findings are usually nondiagnostic. The treatment of choice is complete surgical excision.     

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.     

Laparoscopic resection of locally advanced gastrointestinal stromal tumour (GIST) of the stomach following neoadjuvant imatinib chemoreduction

IntroductionLaparoscopic resection of locally advanced gastrointestinal stromal tumours (GISTs) is rarely offered to patients as a first line of treatment.Presentation of casesWe present two cases of locally advanced gastric GISTs successfully treated with neoadjuvant imatinib and followed up by complete laparoscopic excision of the residual tumour mass. There was no evidence of local recurrence or distant metastases after a mean follow up of more than 40 months.DiscussionOver the last decade, the development of imatinib has totally revolutionized management of metastatic GISTs and it is now possible to achieve primary tumour downstaging of more than 80%. Unfortunately, current literature on laparoscopic excision of locally advanced gastric GISTs following neoadjuvant treatment of imatinib remains scarce. The present cases strongly suggest that this new therapeutic approach might become the preferred medical option in such clinical situation.ConclusionPatients with locally advanced non-metastatic gastric GISTs should be offered first-line neoadjuvant. Imatinib-based cytoreductive chemotherapy as an alternative to radical debulking surgery, as a substantial proportion of them will experience significant tumour shrinkage and therefore benefit from a much less invasive laparoscopic approach.     

Complete remission of primary retroperitoneal transitional cell carcinoma after radiotherapy and oral chemotherapy: a case report

Primary retroperitoneal transitional cell carcinomas (TCCs) are extremely rare neoplasms for which prognosis is very poor. We present a case that underwent complete remission after radiotherapy and concurrent oral chemotherapy. A 68-year-old woman presented with acute onset of bloody stool. Urgent colonoscopy only detected haemorrhoids. Subsequent abdominal ultrasonography revealed a mass of 7cm in maximal diameter in the left iliac fossa. Laparotomy disclosed a retroperitoneal mass that could not be dissected and therefore only incision biopsy was performed. After a final diagnosis of primary retroperitoneal TCC, chemotherapy with tegafur-uracil (UFT) was initiated but was not effective. Subsequently, radiotherapy was initiated concurrently with UFT at a total dose of 50Gy in 25 fractions. At 20 months after radiotherapy, the tumour seemed to have completely remitted. At the last follow-up, ten years from radiotherapy, computed tomography revealed no recurrence.We identified only three single case reports regarding primary retroperitoneal TCC over the last five decades. All patients died from the tumour 8−24 months after diagnosis or treatment. Based on the success of our case, radiotherapy with concurrent oral chemotherapy should be considered as an option for unresected cases.     

Haemorrhagic Shock from the Spontaneous Rupture of an Adrenal Cortical Carcinoma. A Case Report

Adrenal cortical carcinoma is a rare endocrine neoplasm which can be either functioning or non-functioning. Usually, patients refer to the doctor because of abdominal pain or symptoms associated with the mass effect. We present an unusual case of a patient with adrenal cortical carcinoma who was immediately operated due to massive retroperitoneal haemorrhage following the spontaneous rupture of the tumour. Adrenal cortical carcinoma should enter in differential diagnosis of retroperitoneal haemorrhage. Surgeons should be familiar with this clinical entity and attempt complete resection if possible.     

Haemorrhagic shock from the spontaneous rupture of an adrenal cortical carcinoma. A case report

Adrenal cortical carcinoma is a rare endocrine neoplasm which can be either functioning or non-functioning. Usually, patients refer to the doctor because of abdominal pain or symptoms associated with the mass effect. We present an unusual case of a patient with adrenal cortical carcinoma who was immediately operated due to massive retroperitoneal haemorrhage following the spontaneous rupture of the tumour. Adrenal cortical carcinoma should enter in differential diagnosis of retroperitoneal haemorrhage. Surgeons should be familiar with this clinical entity and attempt complete resection if possible.