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PubMed1998

Renal lesions in Von Hippel-Lindau disease: the benign, the malignant, the unknown.

OBJECTIVES: To describe the features and treatment of renal lesions in von Hippel-Lindau disease (VHL) from a series of patients, to highlight important issues in the management of these lesions. MATERIALS AND METHODS: We performed a retrospective study in 7 patients with VHL who underwent surgery for renal lesions between January 1990 and July 1996. The initial evaluation consisted of an abdominal CT scan and renal arteriography. RESULTS: The mean age of patients at the time of discovery was 38 (+/- 12.01 years). All cases were of type 1. Radiology assessment underestimated the gravity of the lesions in 5 patients. In all patients, unilateral surgery was performed with the kidney being conserved in 4 cases. Of the 44 lesions removed, 23 had signs of progressiveness (atypical or malignant cyst, renal cell carcinoma), most of the cancers were of low grade. 84% of the lesions did not exceed 3 cm and 48% were </=1 cm. During follow-up (42 months), 2 patients relapsed. CONCLUSION: The clinical and pathological features of renal lesions in VHL require strict and early follow-up (from the age of 15). Only under these circumstances should primary conservative surgery be performed, however, recurrences are frequent. This conservative approach remains to be validated by multicentric prospective studies.

Adult↗
PubMed1998

Clinical and radiological predictors of complete excision in breast-conserving surgery for primary breast cancer.

BACKGROUND: Local recurrence after conservative surgery for breast cancer usually results from growth of residual cancer adjacent to the excised primary tumour or from multicentric disease. Complete local excision (CLE) confirmed histologically is essential to ensure that the risk of local recurrence is minimal. This study was undertaken to determine that clinical or radiological factors may assist the surgeon at the time of surgery to achieve this aim. METHODS: A retrospective review of 101 cases treated by conservative surgery identified 70 cases of CLE and 31 of incomplete local excision (ILE). Clinical, surgical and histopathological data were taken from hospital records. Mammographic features and those of specimen X-rays were evaluated without knowledge of the histopathological outcome of surgery. RESULTS: Complete excision was significantly associated with type of operation (lumpectomy vs wide local excision/quadrantectomy, P < 0.003), absence of calcification (P < 0.03) and the presence of a mass on mammography (P = 0.05). Tumour size (> 2.5 cm) and the presence of extensive ductal carcinoma in situ (DCIS) were associated with incomplete excision (P = 0.0005). No relationship was demonstrated with patient age, breast size, breast density, tumour grade, receptor status, axillary nodal status or spicules on X-ray and completeness of excision. Specimen X-ray had a positive predictive value of 94% with CLE. CONCLUSIONS: Clinical and pre-operative mammographic parameters are important for predicting CLE for breast cancers treated by breast-conserving surgery. Specimen radiology for palpable lesions can confirm excision of the cancer and permit re-excision of breast tissue at the time of initial surgery. Its role in determining CLE should be further evaluated.

Adult↗
PubMed1992

Nasal and orbital recurrence of conjunctival melanoma 21 years after exenteration.

In our experience 5% of invasive malignant melanomas of the conjunctiva arising from areas of primary acquired melanosis with atypia spread to the ipsilateral nasal cavity and paranasal sinuses. Twenty one years after orbital exenteration for multicentric conjunctival melanoma an 82-year-old man was seen with an orbital recurrence, which had extended to the nasal cavity and paranasal sinuses through the nasolacrimal duct without invading the mucosa. This previously undescribed way of spread after the longest symptom-free interval following exenteration ever reported is illustrated.

Aged↗
PubMed2003

Valaciclovir as a single dose during prodrome of herpes facialis: a pilot randomized double-blind clinical trial.

BACKGROUND: Randomized clinical trials of valaciclovir in recurrent herpes labialis are lacking. OBJECTIVES: To determine whether a single course of valaciclovir, i.e. 500, 1000 or 2000 mg, administered during the prodrome of herpes facialis, could be beneficial. METHODS: Three hundred and forty-five out-patients with herpes labialis were screened and randomized for a multicentre, double-blind clinical trial. Ninety-six patients had no recurrence after 6 months of follow-up; 249 patients were finally included in the intent-to-treat (ITT) population. The main outcome measure was the rate of aborted episodes at day 3. The three treatment groups were similar at baseline. RESULTS: There was no statistically significant difference between the groups in rates of aborted lesions at day 3 in the ITT population, in particular between the 500 mg and 2000 mg treatment groups. CONCLUSIONS: Although a placebo group was not included in this pilot study, a single dose of valaciclovir was not considered beneficial in patients with recurrent herpes facialis.

Acyclovir↗
PubMed1998

Laparoscopic colectomy for cancer: a review.

BACKGROUND: The success of laparoscopic cholecystectomy in providing patient benefits in the immediate postoperative period has led to laparoscopic techniques being used for many other intra-abdominal procedures. Colorectal resection for malignancy is one of the more contentious applications of this new technology, because the postoperative benefits are more subtle and the long-term onco logical results are as yet unknown. METHODS: A review of the English-language literature was undertaken in order to collate and analyse all published series where 20 or more laparoscopic colectomies were performed. and where the indication for resection in the majority of cases was adenocarcinoma of the colon. RESULTS: Laparoscopic colectomy for cancer can be performed safely by experienced surgeons, although there is a considerable learning curve for the procedure. The expected benefits of minimal access surgery are provided by laparoscopic colectomy, although to a lesser extent than that seen with other procedures. The oncological safety of the procedure is as yet unproven. It is clear that an equivalent resection can be performed, but not whether this translates to an equivalent recurrence and survival rate. Reports of isolated port site recurrences are of concern. CONCLUSIONS: Early results of laparoscopic colectomy for cancer are encouraging, although the fate of this procedure rests with the analysis of the large multicentre prospective randomized trials currently under way, particularly with regard to the long-term recurrence and survival rates.

Blood Loss, Surgical↗
PubMed2000

Inverted papilloma.

BACKGROUND: Inverted papilloma, a benign sinonasal lesion, constitutes 0.5% to 4% of all nasal tumors. Its local aggressiveness, high recurrence rate, association with malignancy or malignant transformation, and tendency toward multicentricity lead most physicians to advocate radical surgery. Nonetheless, conservative surgery is effective for selected patients with limited disease. This study was done to reassess the efficacy of both radical and conservative surgery. METHODS: The records of all patients with the diagnosis of inverted papilloma treated at the Taipei Veterans General Hospital between January 1986 and October 1998 were collected. A minimal follow-up period of 12 months was required for inclusion in the study. Clinical manifestations, radiologic findings, methods of treatment, pathology reports and recurrence rates were all retrospectively reviewed. RESULTS: Sixty patients with an average age at diagnosis of 58 years were studied. The follow-up period ranged from 12 to 195 months, with a mean of 43 months. The most common presenting symptom was unilateral nasal obstruction. The duration of symptoms ranged from one month to 30 years, with a mean of 36 months. The lateral wall and middle meatus were the most commonly involved sites. Bone erosion and intracranial or intraorbital extension were observed in some patients. Medial maxillectomy was the most common surgical treatment, followed by functional endoscopic sinus surgery and the Caldwell-Luc operation. The overall recurrence rate was 23%, with an average interval from initial treatment to recurrence of 42 months (range, 2-93 months). The recurrence rates for the two groups undergoing medial maxillectomy and conservative surgery were 16% and 27%, respectively. CONCLUSIONS: Early diagnosis relies on high suspicion and biopsy. The treatment modality is related to the location and extent of the disease, which is assessed by preoperative imaging studies. Any tumor with extensive involvement of the sinuses or inaccessibility to the endoscope should be treated with traditional medial maxillectomy for best control. The endoscopic technique is good for preoperative biopsy and follow-up.

Adult↗
PubMed2001

Tumor progression and survival in patients with T1G3 bladder tumors: multicentric retrospective study comparing 94 patients treated during 17 years.

OBJECTIVES: To compare tumor recurrence, progression, and patient survival in T1G3 bladder tumors treated with transurethral resection (TUR) alone, early cystectomy, or TUR with an adjuvant 6-week course of bacille Calmette-Guérin (BCG) and followed up for a minimum of 5 years. METHODS: Between 1979 and 1996, 94 patients with T1G3 bladder tumors (lamina propria invasion) were treated at nine different centers. The time to tumor recurrence, tumor stage and grade progression, number of delayed cystectomies, and patient survival were analyzed retrospectively in relation to the initial treatment. RESULTS: The mean follow-up was 62 months. Thirty patients were treated by TUR alone (32%), 50 patients by TUR plus BCG (53%), and 14 patients by primary cystectomy (15%). The recurrence, progression, and cystectomy rates were significantly different between patients treated by TUR alone and TUR plus BCG (Fisher's exact test, P = 0.0005, P = 0.02, and P = 0.005, respectively). The disease-free survival was also significantly different when comparing TUR plus BCG with TUR alone or primary cystectomy (Kaplan-Meier analysis, log-rank test, P = 0.02). CONCLUSIONS: Endoscopic resection plus BCG treatment of pT1G3 tumors allows an 80% rate of disease-free 5-year survival with bladder preservation. This conservative option has been widely accepted as first-line treatment, offering good cancer control with excellent quality of life. Very accurate surgical and pathologic evaluations before treatment and lifelong follow-up are obviously required.

Aged↗
PubMed1999

Determination of the clonal origin of multiple human hepatocellular carcinomas by cloning and polymerase chain reaction of the integrated hepatitis B virus DNA.

The poor prognosis of hepatocellular carcinoma (HCC) is partly the result of the high rate of recurrence that is caused either by intrahepatic metastasis (IM) or independent multicentric occurrence (MO). For convenience, discrimination of IM and MO is based on pathological findings, but reliable parameters are not sufficiently established. In the case of hepatitis B virus (HBV)-associated HCC, molecular discrimination of IM from MO can be achieved by comparison of integrated HBV DNAs. However, Southern blotting cannot be used for this purpose when one tumor is saved in frozen form and the other is in paraffin-embedded form. To solve this problem, we employed polymerase chain reaction (PCR) assays to confirm the clonality of primary and recurrent tumors. From the frozen tissue, we determined the junction between the integrated HBV and flanking genomic DNA by molecular cloning, and checked the existence of an identical junction in the DNA of paraffin-embedded tissue by PCR. Using this method, as well as Southern blotting, we proved in 6 of 8 patients that two nodular HCC lesions resected metachronously or simultaneously were caused by MO, while the remaining 2 cases were caused by IM. In 1 IM case, band patterns between two HCCs detected by Southern blotting were not identical.

Aged↗
PubMed1987

Regulated high frequency diathermy for bipolar electrocoagulation--a new method for the treatment of carcinoma of the bladder.

In the endoscopic treatment of bladder cancer, especially for stages beyond T2, transurethral resection does not seem to be sufficient. The risk of bladder wall perforation, the possibility of incomplete resection, seeding of tumor cells and high recurrence rates support the former statement [6]. Results of a multicentre prospective randomised study have exemplified that irradiation with the Neodymium-YAG Laser has been a reasonable alternative in the therapeutic approach to bladder cancer [2]. Diathermy coagulation must produce a sufficiently deep, homogeneous and well demarcated tissue necrosis. The coagulation depth has to be adjustable and heat production during coagulation should not lead to bladder wall perforation and consequent damage to the bowel. These criteria were investigated using regulated bipolar high frequency diathermy. The innovation in this method is, that thermal side effects on the tissue, e.g. vaporisation and carbonisation are prevented by a protective relay with constant current flow. Animal experimental studies have shown that this method meets all postulated requirements and therefore clinical trials can proceed.

Animals↗
PubMed2005

A rare female genital tract tumor: benign granular cell tumor of vulva: case report and review of the literature.

BACKGROUND: The granular cell tumor (GCT) of vulva is a rare female genital tract tumor. CASE: A 59-year-old female was incidentally noted to have a 1-cm sized lump in her left labium majus that subsequently increased in size to approximately 4 cm. The mass was totally excised under general anesthesia. Her postoperative follow-up was uneventful. Histologic examination of the tumor showed sheets and clusters of infiltrating tumor cells with morphologic features consistent with granular cell tumor. CONCLUSION: Although benign and slow growing, it has a tendency for recurrence and can cause morbidity and mortality when presenting with multicentric or multiple organ involvement due to the lack of effective systemic therapy. Therefore, clinicians and pathologists should be aware of its clinical and histopathologic features.

Female↗
PubMed2002

Inguinal hernia repair: where to next?

BACKGROUND: Hernia repair is one of the most frequently performed operations in general surgery - a total of 39 000 elective inguinal hernia repairs were performed in public and private hospitals in Australia between July 1998 and June 1999 - and, as such, even minor alterations in outcome and resource use have appreciable impact. However, decisions regarding choice of operation for hernia repair remain controversial. The purpose of the present paper is to critically evaluate the evidence available regarding recently introduced open mesh repair techniques and to try to identify meaningful directions for future hernia research. METHODS: A thorough search of all published surgical literature was undertaken. Medline, EMBASE and the Cochrane databases were searched by title, by key words and by author. References in review articles and in textbooks were pursued. The manufacturing companies were contacted for trials evaluating their product. RESULTS: Eight original articles evaluating either the Kugel Patch, the PerFix Plug or the Prolene Hernia System were located. None of these trials directly compared two or more of these repair systems. To date, there has been no published review of the evidence regarding the newer mesh repair techniques. With one exception, all of these articles qualify as Level IV evidence. Highlighted is the lack of evidence regarding chronic significant posthernioplasty pain - this has an incidence of 6-12%. This complication is 3-5 times more common than recurrence after open repair, is clinically relevant, is poorly understood and has been poorly studied. Arguably it is a more important end point than recurrence. CONCLUSION: Only one study comparing the newer techniques of open hernia repair (PerFix Plug) constitutes level II evidence. The PerFix Plug appears to be quicker to insert and uses a smaller incision. Chronic significant posthernioplasty pain is a more important endpoint in hernia research than is recurrence, and this review concludes with a proposal for a multicentre, randomized, controlled trial evaluating the incidence of chronic significant posthernioplasty pain following elective mesh repair of primary, unilateral -hernias.

Digestive System Surgical Procedures↗
PubMed2000

Malignant thymoma: current status and future directions.

Malignant thymomas are rare indolent tumours of the anterior superior mediastinum. Despite a benign histologic appearance, some thymomas invade nearby structures or metastasize. Patients are commonly asymptomatic, but some may present with unusual paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, or hypogammaglobulinemia. Since tumour biopsy may potentially disrupt the thymic capsule, it is often not performed. Patients are therefore diagnosed and staged at the time of definitive surgery. Thymomas can generally be categorized into two stages: non-invasive and invasive. Prognosis closely parallels the disease stage. Surgery is the principal treatment and is curative in early stage disease. Radiation therapy, either alone or in combination with chemotherapy, is an option for both incompletely or completely resected disease. Chemotherapy is offered to patients with locally advanced, recurrent, or metastatic thymoma, with excellent responses and prolonged survival. Multicentre co-operative group clinical trials are required to assess novel thymoma therapies to maximize patient resources in this uncommon tumour.

Combined Modality Therapy↗
PubMed1990

[Infantile myofibromatosis].

Eight cases of infantile myofibromatosis, 5 male and 3 female, are reported. The ages ranged from 8 days to 3 years at diagnosis. The tumor was detected in 6 cases at birth and in 2 at the age of 3 and 11 months. The tumor occurred mostly in extremities, trunk, orbit, nose, soft tissue and bone. Spontaneous regression of all the tumor nodules was observed in 1 case of the multicentric type without evidence of visceral involvement within 3 years. Recurrence developed in 2/7 cases of solitary type 2 and 3 years after excision. Histologically, the tumor nodules were composed of myofibroblasts; blood vessel was abundant in a pericytoma-like pattern; and prominent necrosis or calcification was seen. By immunohistochemical stain for desmin, diffuse weak reactivity was shown in the tumor cells and granular cytoplasmic positivity in the actively proliferating tumor foci, suggesting the existence of muscle-specific intermediate filaments in the tumor cells.

Child, Preschool↗
PubMed1999

Castleman disease presenting with ophthalmic signs and symptoms.

PURPOSE: To describe a patient with multicentric Castleman disease who was initially examined with ophthalmic signs and symptoms. METHODS: Case report. A 71-year-old man was initially examined with swelling of both upper eyelids and diplopia of 2 months' duration. RESULTS: Medical evaluation and right axillary lymph node biopsy disclosed Castleman disease. Systemic corticosteroid treatment temporarily resolved signs and symptoms, but the patient died of recurrence with cytomegalovirus and Aspergillus infection 10 months after initial examination. CONCLUSIONS: Multicentric Castleman disease is a rare but distinct disorder that may present initially with ocular signs and symptoms. This disease must be included in the differential diagnosis of orbital pseudotumor and lymphoma.

Aged↗
PubMed2006

Prediction of risk of seizure recurrence after a single seizure and early epilepsy: further results from the MESS trial.

BACKGROUND: The MRC Multicentre trial for Early Epilepsy and Single Seizures (MESS) showed a reduced risk of further seizures in patients, for whom treatment with antiepileptic drugs was uncertain, who were randomly assigned immediate treatment compared with delayed treatment. However, there was no evidence of an effect on [corrected] long-term remission rates. This study was undertaken to assess the role of patient characteristics and treatment in the prediction of seizure recurrence. This will enable decision-making on the basis of the perceived risk of treatment compared with the benefit of reducing the risk of further seizures in the initial years after diagnosis. METHODS: A prognostic model was developed based on individual patient data from MESS to enable identification of patients at low, medium, or high risk of seizure recurrence. A split-sample approach was used in which the model was developed on a subsample of the full data and validated on the remainder of the sample. Distinction of the prognostic groups and predictive accuracy of the model were assessed. FINDINGS: Number of seizures of all types at presentation, presence of a neurological disorder, and an abnormal electroencephalogram (EEG) were significant factors in indicating future seizures. Individuals with two or three seizures, a neurological disorder, or an abnormal EEG were identified as the medium-risk group, those with two of these features or more than three seizures as the high-risk group, and those with a single seizure only as the low-risk group. INTERPRETATION: The model shows that there is little benefit to immediate treatment in patients at low risk of seizure recurrence, but potentially worthwhile benefits are seen in those at medium and high risk.

Adolescent↗
PubMed1985

Combined modality management of local and disseminated adult soft tissue sarcomas: a review of 257 cases seen over 10 years at the Christie Hospital & Holt Radium Institute, Manchester.

Over a 10 year period, between 1974-1984, 257 adult cases of tissue sarcoma have been evaluated in the Department of Medical Oncology, Christie Hospital, Manchester. At registration locally advanced or metastatic diseases was present in 162 (63%). The male/female ratio was 1.5:1 and median age 54 years (range 14-85). The commonest sites were lower limb (33%), visceral (21%), trunk (13%), retroperitoneum (12%) and upper limb (10%). Leiomyosarcoma (27%), liposarcoma (14%) malignant fibrous histiocytoma (10%) and neuro plus fibrosarcomas (15%) were the most frequent histological subtypes. A high proportion of uterine sarcomas (17%) is a point of distinction from many other series. Histological grade was specified in 72% of cases and the distribution (Grade I--27%; II--6%; III--67%) reflected a referral bias towards advanced disease. Local resection of the primary tumour was performed in 76% of cases. In many instances this only amounted to 'shelling out' and true compartmental excisions were rare. Amputation was performed in 31% of patients with limb sarcomas. Ninety-eight patients (38%) had experienced one or more local recurrences prior to referral and the overall local recurrence rate was 56%. Suitable patients (78%) received chemotherapy, 50% entering multicentre trials in collaboration with the EORTC. The commonest regime used in patients with advanced disease was CYVADIC which produced an overall response rate of 37%. Ifosfamide, used as a single agent in 16 patients, induced 3CR and 5PR for an overall response rate of 50%. When used in combination with MTX and VADIC, there was no difference in response rate, but numbers in these pilot studies were small. Seventeen high risk patients received adjuvant chemotherapy with VAC, but the results (11 relapses) were disappointing. An EORTC trial, comparing adjuvant CYVADIC chemotherapy with control has accrued 307 patients, 49 of these from the Christie Hospital. Preliminary results within this centre - 13/25 relapses in the control arm, 5/23 in the chemotherapy arm-suggest an advantage for chemotherapy but the data are statistically not significant. Post-operative radical radiotherapy after resection of the primary tumour or local recurrence was performed in 51 patients, with local control in 65% of cases, although metastases developed in 41%. At the time of analysis (1st April 1984) 98 (38%) were alive, of whom 72 showed no evidence of disease and 52 had never relapsed. Malignant disease was the cause of death in 92%. Overall survival was not influenced by sex, but patients less than 40 years of age fared significantly better (P less than 0.001).(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1997

Multiple frequent recurrences in superficial transitional cell carcinoma of the bladder: is survival compromised by a conservative management strategy?

OBJECTIVE: To examine whether a strategy of bladder conservation is reasonable in patients with multiple frequent superficial recurrences of transitional cell carcinoma (TCC) of the bladder. PATIENTS AND METHODS: Fifty-four patients with pTa/pT1. G1/G2 tumours at diagnosis, with five or more recurrences at two or more cystoscopies within 2 years of diagnosis and a minimum follow-up of 4 years were identified. The patients were categorized according to outcome, i.e. disease settled, continuing high-activity disease and disease progression. RESULTS: Forty-four patients did not progress, of whom 16 continued to have high-activity disease and 28 settled to a lower disease activity. One patient had a cystectomy for superficial disease. Nine patients progressed, six with muscle invasion in the bladder and three elsewhere in the urinary tract. Neither grade nor stage were predictive of recurrence. All but one of the patients with progression had both multicentric tumours at diagnosis and a positive cystoscopy at 3 months. Three patients died from their bladder cancer. CONCLUSION: A policy of endoscopic resections and intravesical chemotherapy or bacille-Calmette-Guèrin, with cystectomy reserved until muscle-invasive disease develops, does not significantly compromise survival in patients with high-activity superficial TCC. Cystectomy for superficial disease is rarely necessary.

Administration, Intravesical↗
PubMed1996

Multicentric pleomorphic xanthoastrocytomas: case report.

We report the case of a 22-year-old woman who developed multicentric pleomorphic xanthoastrocytomas (PXAs) in the cerebral hemisphere. She underwent a first operation for a PXA in the right parietal lobe at the age of 7 years and a second operation at the age of 15 years in the right frontal lobe, remote from the previous tumor site. At age 22 years, she was found to have a tumor, which was a newly formed PXA, in the left occipital lobe. There was no recurrent tumor in the right frontal lobe. Clinical and pathological aspects of multicentric PXAs are reviewed and discussed.

Adolescent↗