腹膜后神经鞘瘤1例报告并文献复习

目的报告1例腹膜后神经鞘瘤,并复习相关文献,分析其临床诊治现状。方法通过对1例腹膜后神经鞘瘤的诊断、治疗方法及术后病理、随访的回顾,结合国内外有关文献报道,综合分析腹膜后神经鞘瘤的诊断及治疗状况。结果本例行手术治疗,术后病理证实为腹膜后神经鞘瘤,随访半年无复发。结论腹膜后神经鞘瘤诊断困难,影像学检查无特异性,确诊依靠病理检查,理想的治疗方法是通过外科手术完整切除肿瘤,其预后良好,早期复发率低。     

ADULT HEPATOBLASTOMA: CASE REPORT AND REVIEW OF THE LITERATURE

We report a case of hepatoblastoma in a 24 year old woman. The tumour was removed by an extended right lobectomy after transcatheter hepatic arterial embolization. A survey of the literature revealed 31 cases of adult hepatoblastoma. According to these reports there are no characteristic clinical features of the tumour, making a correct diagnosis before surgery or autopsy extremely difficult. Complete removal of the tumour is the only treatment which is potentially curative provided that lesion is confined within the liver capsule.     

腹膜后囊性淋巴管瘤的诊断与治疗

目的探讨腹膜后囊性淋巴管瘤的诊断与治疗。方法收集经病理证实的成人腹膜后囊性淋巴管瘤5例,回顾性分析其临床表现及影像学特征,并与手术及病理结果进行对照分析。其中男性2例,女性3例,平均年龄38．0岁。患者无特异性临床表现,均为常规体检时经B超检查发现。经CT及MRI检查均考虑：腹膜后囊性淋巴管瘤可能。结果5例均行手术治疗并完整切除,术后恢复顺利,随访3个月至16个月均无复发。结论腹膜后囊性淋巴管瘤多无特异性临床表现,CT及MRI检查对术前协助诊断及对手术均有重要指导意义。手术切除为首选治疗方式,术中应尽可能完整切除病变,以防术后复发。     

LAPAROSCOPIC VARICOCELECTOMY: INVASIVENESS AND EFFECTIVENESS COMPARED WITH CONVENTIONAL OPEN RETROPERITONEAL HIGH LIGATION

The invasiveness of laparoscopic varicocelectomy and- open retroperitoneal high ligation of the internal spermatic veins were compared and the surgical effects on fertility of these two procedures determined. 48 of 97 men diagnosed with varicocele testis underwent laparoscopic varicocelectomy, while the remaining 49 underwent open retroperitoneal high ligation of the internal spermatic vessels. Operating time, number of post-operative days to walking, length of hospital stay and analgesic use were measured as peri-operative indicators of invasiveness. In addition, seminal parameters were determined in order to evaluate the effects of these procedures on fertility. The operating time required for laparoscopic surgery was significantly longer than that for open surgery (96.6 vs 78.1 min., p = 0.0078). The patients in the laparoscopic surgery group began walking earlier post-operatively than did those who underwent open high ligation (0.97 vs 1.42 days, p = 0.00037). Length of hospital stay for the laparoscopic patients was shorter than for the open surgery group (7.05 vs 9.55 days, p=0.00001). There were no statistical differences between the groups in terms of semen quality or improvement in the post-operative rate of pregnancy of partners. These findings indicate that laparoscopic varicocelectomy is associated with a shorter period of convalescence than open high ligation of the internal spermatic vessels.     

RETROPERITONEAL MALIGNANT MESENCHYMOMA: A CASE REPORT

A case of a 37-year-old woman with a retroperitoneal tumor is reported. Angiography revealed that the tumor was partially supplied via an intercostal artery, suggesting that the cause of the tumor might be located in the rib. Histologically the tumor was diagnosed as a malignant mesenchymoma composed of chondrosarcoma and myxoid liposarcoma in addition to fibrosarcoma. The chondro-sarcomatous element was predominant, a phenomenon which is extremely rare. Pulmonary metastases developed 8 mo after surgical removal of the tumor and the patient died of the disease 2 yr postoperatively.     

THE ABDUCTOR DIGITI MINIMI FLAP: A CASE REPORT AND REVIEW

The use of an abductor digiti minimi flap is reported here for coverage of a dorso-ulnar defect of the hand following excision of a tumour. This flap has not been widely used before. The abductor digiti minimi flap appears to have minimal donor site morbidity and a reliable vascular supply, and is straightforward to raise. The authors consider it would be a useful flap to provide cover on the ulnar side of the hand and wrist in patients who do not require highly dextrous use of the little finger.     

ILEOSIGMOID KNOTTING: A CASE REPORT AND REVIEW

Ileosigmoid knotting, or compound volvulus, has not been reported previously in Australia. A 41 year old woman of Anglo-Saxon origin presented with a short history of extreme abdominal pain that was out of proportion to her physical findings. At operation it was found that her entire ileum was tightly knotted around the redundant, twisted sigmoid colon. Both closed loops were gangrenous and it was impossible to unravel the bowel. She recovered uneventfully following resection and primary anastomosis of both portions of the intestine. Most patients with this uncommon condition have been reported from Finland and eastern Africa. An arrangement of the small bowel and sigmoid colon on long, narrow mesenteries would appear to be a prerequisite. The brevity of the history and the severity of the abdominal pain call for early laparotomy. Prolonged attempts to untie the knot are dangerous. It is safer to divide the ileum at the knot and resect it in order to release the sigmoid colon. Primary anastomosis is feasible where the history is short and the uninvolved intestine is clean and collapsed.     

MALIGNANT FIBROUS HISTIOCYTOMA REVIEW AND CASE REPORT

Malignant fibrous histiocytorna (MFH) is a rare tumour of the spermatic cord. We present the second reported case in Australia and review the literature, discussing the recommended management and prognosis of this condition.     

TORSION OF THE VERMIFORM APPENDIX: A CASE REPORT AND REVIEW OF LITERATURE

A case of torsion of the vermiform appendix is described. It is a rare cause of an acute abdomen with a clinical presentation that is indistinguishable from acute appendicitis.     

ISOLATED CONGENITAL PULMONARY INCOMPETENCE: CASE REPORT AND LITERATURE REVIEW

Although the literature fails to clearly define the prognosis of isolated congenital pulmonary incompetence (ICPI), there are suggestions that the lesion is not necessarily benign. A case report is presented of pulmonary valve replacement for ICPI which reversed not only symptoms but also electrocardiographic manifestations of right ventricular overload. A literature review is included with the case report.     

CARCINOMA OF THE THYROGLOSSAL DUCT CASE REPORTS AND A LITERATURE REVIEW

Carcinoma of the thyroglossal tract is a rare entity. Three patients with thyroglossal cyst carcinomas are presented and the features of the disease, as reported in the literature, are discussed. Epidemiologically, females are more often affected than males and the average age of the patients described lies in the fourth decade. The aetiology is obscure, although previous irradiation is a possible risk factor, Carcinoma of the thyroglossal tract should also be suspected in patients with irregular masses. Pre-operative evaluation may include a thyroid scan and fine needle aspiration cytological examination of the cyst fluid. These tests, if positive, may alter the basic approach of the Sistrunk procedure to encompass thyroidectomy or wider margins. Neck dissection is preferred for cervical nodal disease. Adjuvant radiotherapy or radio-iodine is added if indicated by the histology, and the patient receives suppressive thyroxine therapy thereafter.     

Analysis of 82 cases of retroperitoneal schwannoma

BACKGROUND: The aim of the study was to improve the diagnosis and treatment of retroperitoneal schwannoma by analysing clinical manifestations and postoperative course of this rare disease. METHODS: A retrospective analysis of 82 patients with retroperitoneal schwannoma between January 1951 and September 2004 was carried out. RESULTS: The patients were 38 (46%) men and 44 (54%) women between the ages of 6 months and 70 years. The interval between clinical manifestation and diagnosis ranged from 10 days to 2 years. The main symptoms were abdominal distension (30.5%) and abdominal pain (20.7%). Only in 13 patients (15.9%) a correct preoperative diagnosis was made by either ultrasound-guided biopsy, computed tomography scanning or magnetic resonance imaging. All patients received operative therapy. Sixty patients (73.2%) underwent a total resection; 13 patients (15.9%) subtotal resection, but 9 patients (11.0%) had only an examination and a biopsy. Two patients (2.4%) had multiple schwannomas and two others had a simultaneous malignancy (adenocarcinoma of the ascending colon and squamous-cell carcinoma of the lung, respectively). Most of the retroperitoneal schwannomas were close to the spine. Pathological results showed 81 (98.8%) were benign schwannoma and 1 (1.2%) was a malignant one. The tumour size ranged from 3 to 22 cm. One benign schwannoma recurred 3 years after the operation. The patient with malignant schwannoma died 18 months after the operation because of metastasized disease. CONCLUSION: Most of the retroperitoneal schwannomas are benign. It is difficult to make an accurate preoperative diagnosis. However, with the preoperative assessment of ultrasound-guided fine-needle aspiration, computed tomography and magnetic resonance imaging, the accuracy of diagnosis could definitely be improved. Treatment depends solely on surgery. Malignant schwannomas are insensitive to chemotherapy and radiation, resulting in poor prognosis.     

HEREDITY,MOLECULAR GENETICS AND COLORECTAL CANCER: A REVIEW

It is estimated that the hereditary polyposis and non-polyposis colorectal cancer (CRC) syndromes, which have an autosomal dominant pattern of inheritance, represent less than 10% of the total CRC burden. Thus, more than 90% of all cases of CRC have previously been considered to arise ‘sporadically’, with no identifiable genetic link. However, recent clinical evidence now suggests that a significant proportion of CRC seen in the general population may involve an inherited genetic susceptibility. Therefore, constructing an accurate family tree on all patients with a family history of CRC is an essential part of identifying families with an increased risk for CRC who could then be offered screening. Also. molecular genetic study of colorectal adenomas and carcinomas has led to a proposed genetic model of colorectal tumorigenesis which involves interactions between oncogenes and tumour suppressor genes. This information has important potential implications for screening, determining prognosis and for providing multiple targets for altering the sequence of malignant transformation.     

CALCULI IN A MECKEL'S DIVERTICULUM: A CASE REPORT AND A REVIEW

A case is reported of multiple calculi occurring in a Meckel's diverticulum which caused chronic abdominal pain in a 48-year-old man. The problem was diagnosed before operation by radiological studies. The features of this condition are well illustrated in this patient. The published literature is reviewed.     

Intracranial intraparenchymal schwannoma: Report of three cases

Summary Intracranial intraparenchymal schwannomas are rare. We report three patients with an intracranial intraparenchymal schwannoma and discuss the clinical and neuroradiological aspects of this particular tumour. The patients were a 21-year-old male, a 64-year-old female and a 17-year-old male. The tumours were located in cerebrum in two patients and the cerebellum in one patient. Computerized tomography (CT) scans demonstrated a slightly high density area with homogeneous enhancement by contrast medium. Magnetic resonance imaging (MRI) showed slightly low signal intensity on the T1-weighted image, high or mixed signal intensity on the T2-weighted image and homogeneous enhancement by gadolinium diethylene triamine penta-acetic acid (Gd-DTPA). Radiological studies revealed cystic components in 2 of the 3 patients. All tumours were firm, well-demarcated, and completely removed. The diagnosis of schwannoma was derived from histological and immunohistochemical studies in all 3 cases; 2 cases were also examined by electron microscopy.     

Retroperitoneal lymph node dissection after chemotherapy in patients with elevated tumour markers: indications, histopathology and outcome

OBJECTIVE

To evaluate the factors affecting outcome and the pathological findings in patients who had retroperitoneal lymph node dissection (pcRPLND) after chemotherapy with elevated tumour markers, as such patients have an unfavourable prognosis, with further salvage chemotherapy being the usual treatment of choice.

PATIENTS AND METHODS

Information on the preoperative treatment, tumour markers, histopathology and outcome data of the patients who had pcRPLND were extracted from the hospital databases. Survival was analysed using the Kaplan‐Meier method and multivariate analysis with Cox regression model.

RESULTS

In all, 358 patients had pcRPLND between September 1992 and April 2006, by one surgeon. In 48 patients the tumour markers were elevated at the time of surgery, they were on a ‘rising trend’ in 26 (54%) and ‘downward or stable’ trend in 22 (46%). The overall incidence of active germ cell tumour, differentiated teratoma and necrosis in the resected specimens was 58%, 25% and 17%, respectively. The median follow‐up was 51.5 months and the overall 5‐year survival was 69%. The favourable prognostic factors assessed by univariate analysis were elevation of α‐fetoprotein alone, complete resection of residual disease, histological finding of differentiated teratoma in the resected tissues and normalization of tumour markers after pcRPLND. By multivariate analysis the only statistically significant independent survival factor was the normalization of the tumour markers after pcRPLND.

CONCLUSION

For selected patients with elevated tumour markers after chemotherapy, RPLND can offer a significant chance of cure with no need for further chemotherapy. The patients most likely to benefit are those with elevations of α‐fetoprotein alone. In this group, pcRPLND can offer the prospect of long‐term survival and should be considered in the management of selected patients.     

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.