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1.

Objective

To determine the efficacy of chemotherapy for inoperable desmoid tumours associated with familial adenomatous polyposis.

Design

A review of three cases of unresectable desmoid tumours and of the literature on the subject.

Setting

The Steven Atanas Stavro Polyposis Registry at Mount Sinai Hospital in Toronto.

Patients

Three patients with symptomatic, unresectable desmoid tumours associated with familial adenomatous polyposis and unresponsive to conventional hormone therapy.

Intervention

A chemotherapy regimen of seven cycles of doxorubicin (dose ranging from 60 to 90 mg/m2) and dacarbazine (1000 mg/m2), followed by carboplatin (400 mg/m2) and dacarbazine.

Outcome Measures

Clinical improvement and tumour regression demonstrated by computed tomography.

Results

In each of the three cases significant tumour regression was seen clinically and radiologically.

Conclusions

Cytotoxic chemotherapy is an effective treatment for desmoid tumours associated with familial adenomatous polyposis. The chemotherapy should be started early in cases of symptomatic desmoid tumour unresponsive to conventional medical therapy.  相似文献   

2.

INTRODUCTION

Leiomyosarcomas are an infrequent cause of malignant superior vena cava syndrome (VCS).

PRESENTATION OF CASE

A 51-year old male patient was admitted for a three-day history of dyspnoea, dysphagia and erythema of the head and neck. Computed tomography and magnetic resonance imaging showed a lesion arising on the anterior mediastinum, which was in close proximity with a thrombus in the superior vena cava. Surgical excision was performed, including open resection of the primary tumour and an atrio-innominate vein bypass with 8-mm polytetrafluoroethylene (PTFE). Histology confirmed a leiomyosarcoma and postoperative radiotherapy sessions were performed. Due to evidence of enlargement of the thrombus, a second intervention was undertaken. In this procedure, a remainder of the primary tumour was resected and the superior vena cava reconstructed with an autologous pericardium patch. The patient recovered satisfactorily and was discharged on the seventh postoperative day, with no evidence for relapse after 10 months of follow-up.

DISCUSSION

Leiomyosarcomas comprise less than 2% of the tumours of the mediastinum and are a rare cause of paraneoplastic VCS. Male patients in their sixties are most commonly affected. Relapses seem to be common, and thus a careful follow-up is often recommended.

CONCLUSION

In spite of the limited data on the management of thoracic leiomyosarcomas, surgery is currently considered the mainstay of treatment.  相似文献   

3.

INTRODUCTION

Gastric schwannomas are rare mesenchymal tumours that arise from the nerve plexus of the gut wall. They present with non-specific symptoms and are often detected incidentally. Pre-operative investigation is not pathognomonic and many are therefore diagnosed as gastrointestinal stromal tumours (GISTs). Operative resection is usually curative as they are almost always benign, underpinning the importance of differentiating them from GISTs.

METHODS

Three cases of gastric schwannomas were identified over a seven-year period. The clinical details and management were reviewed retrospectively.

RESULTS

There were two women and one man with a mean age of 62 years (range: 51–69 years). Two patients presented with bleeding and one with abdominal pain. The mean tumour size was 5.2cm (range: 2–10cm) and the tumours were resected completely following total or wedge gastrectomies. Histology in all cases showed spindle cells with a cuff of lymphoid tissue. Immunohistochemistry confirmed positive S100 staining and negative CD117 and DOG-1 staining in all cases.

CONCLUSIONS

We report our experience with these unusual primary stromal tumours of the gut and their presentations, preoperative investigations, operative findings and pathological findings are discussed. Operative resection in all cases has been considered curative, which is supported by previous series confirming the excellent prognosis of gastric schwannomas.  相似文献   

4.

INTRODUCTION

Chordomas are rare, slow growing, locally destructive bone tumours arising from the notochord.

PRESENTATION OF CASE

Presenting a case of a 65 year old man, who presented with complaints of swelling on the right lower back for 1 year associated with pain.On, physical examination, a swelling measuring 5 cm × 4 cm was noted in the lower back with posterior wall indentation on per rectal examination.MRI revealed a mass lesion involving the sacrum (s3–s4) and coccyx. FNAC showed features of a chroma.At surgery, we excised a mass from the retrorectal space and biopsy proved it to be a chondroid chordoma, a variant of chordoma.

DISCUSSION

Chordomas are solid malignant tumours that arise from vestiges of the foetal notochord. Common locations are the clivus and the sacrococcygeus region.Annual incidence of these tumours is 1 in one million. MRI is the imaging modality of choice. Prognosis improves based on the age, resected margins and postoperative treatment.

CONCLUSION

Here, we shall discuss the literature, variants, treatment and prognosis of this rare tumour.  相似文献   

5.

INTRODUCTION

Bladder cancer recurrence occurs via four mechanisms - incomplete resection, tumour cell re-implantation, growth of microscopic tumours, and new tumour formation. The first two mechanisms are influenced by clinicians before and immediately after resection; the remaining mechanisms have the potential to be influenced by chemopreventive agents. However, the relative importance and timing of these mechanisms is currently unknown. Our objective was to postulate the incidence and timing of these mechanisms by investigating the location of bladder cancer recurrences over time.

PATIENTS AND METHODS

The topographical locations of tumours and their recurrences were analysed retrospectively for 169 patients newly-diagnosed with Ta/T1 bladder cancer, with median follow-up of 33.8 months. Tumours were assigned to one or more of six bladder sectors, and time to recurrence and location of recurrences were recorded.

RESULTS

Median time to first tumour recurrence was 40 months. Median times between subsequent recurrences were 6.6, 7.9, 8.0 and 6.6 months for recurrences 1 to 2, 2 to 3, 3 to 4, and 4 to 5, respectively. The risk of first tumour recurrence in any given bladder sector increased by nearly 4-fold if the primary tumour was resected from that sector (P < 0.001); this association was not significant for subsequent recurrences. The proportion of tumour recurrences in multiple bladder sectors increased from 13% for the first recurrence to 100% for recurrence seven onwards.

CONCLUSIONS

First tumour recurrence appears different to subsequent recurrences; incomplete resection and tumour cell re-implantation may dominate at this time-point. Only later does genuine new tumour formation appear to increase in importance. This has important implications for clinical trials, especially those involving chemopreventive agents.  相似文献   

6.

INTRODUCTION

Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney''s complex.

PRESENTATION OF CASE

A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma.

DISCUSSION

In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney''s complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney''s complex.

CONCLUSION

Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney''s complex or other familial syndrome.  相似文献   

7.

Background

Aggressive surgical resection of neuroendocrine tumour liver metastases (NET-LM) is associated with symptomatic relief. Debulking up to 90% of tumour burden, even with positive margins, may be beneficial. However, patients with diffuse hepatic metastases may not qualify for resection owing to associated insufficient remnant liver parenchyma. The purpose of this study is to describe an early experience with a hepatic parenchymal preserving (HPP) approach.

Methods

We retrospectively reviewed our institutional neuroendocrine tumours database to identify patients with NET-LM, including symptomatic patients with extensive bilobar involvement, who underwent virtual volumetric assessment (VVA) combined with HPP resection between October 2008 and July 2011.

Results

Our study involved 9 patients. The median number of liver metastases resected was 10 (range 4–50). Symptomatic improvement was observed in all patients. Immediate postoperative normalization of 5-HIAA 24-hour urine levels occurred in 89% of patients. Symptomatic and biochemical response remained stable or improved in 75% of patients at 12 months of follow-up. Four patients had postoperative complications. There was no 90-day mortality.

Conclusion

The described HPP approach is feasible and safe. Most patients experienced symptomatic and biochemical improvement. This reproducible approach could expand surgical resection options for patients with NET-LM and diffuse bilobar involvement.  相似文献   

8.

Background

Laparoscopic wedge resection has been widely accepted for small benign gastric tumours. Large gastrointestinal stromal tumours (GISTs), however, can be difficult to manipulate laparoscopically and are at risk for capsule disruption, which can then result in peritoneal seeding. Some authors have suggested that large GISTs (> 8 cm) are best approached using an open technique. However, there has been no consensus as to what the cut-off size should be. We conducted one of the largest Canadian series to date to assess outcomes and follow-up of the laparoscopic management of GISTs.

Methods

All patients with gastric GISTs presenting to Vancouver General Hospital and University of British Columbia Hospital between 2000 and 2008 were reviewed. Most lesions were resected using a wedge technique with closure of the stomach facilitated by an endoscopic linear stapling device.

Results

In all, 23 patients presented with GISTs; 19 patients underwent laparoscopic resection and, of these, 15 had a purely laparoscopic operation and 4 had a hand-assisted laparoscopic resection. Mean tumour size was 3.2 cm, with the largest tumour measuring 6.8 cm. There were no episodes of tumour rupture or spillage and no major intraoperative complications. All margins were negative. Mean follow-up was 13.3 (range 1–78) months. There was no evidence of recurrence or metastasis.

Conclusion

The laparoscopic management of gastric GISTs is safe and effective with short hospital stays and good results over a mean follow-up of 13.3 months. We believe that it should be the preferred technique offered to patients.  相似文献   

9.

Objectives

To review the experience at a children’s hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other.

Design

A retrospective case series.

Setting

British Columbia’s Children’s Hospital a tertiary-care pediatric centre.

Patients

All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years.

Main outcome measures

The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma.

Results

One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar.

Conclusions

Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.  相似文献   

10.

INTRODUCTION

Neuroendocrine tumours (NETs) are tumours that commonly involve the gastrointestinal system. Common primary sites in the gastrointestinal system include the small intestine, appendix, rectum and pancreas. Mesenteric NETs are extremely rare entities and are sparsely reported in the literature.

CASE HISTORY

We report the case of a 62-year-old woman with ectopic Cushing’s syndrome due to excessive adrenocorticotropic hormone secretion by a primary mesenteric tumour in the small intestine and its liver metastases.

CONCLUSIONS

Although rare, the mesentery can be a primary site for NETs. It can cause similar symptoms and require similar treatment options. Tumour resection and debulking are acceptable ways to improve both the survival and symptoms.  相似文献   

11.

Objective

To evaluate the safety and efficacy of ultrasound-guided cryosurgery to treat malignant tumours of the liver.

Design

A prospective nonrandomized trial. The follow-up was complete and ranged from 8 to 35 months.

Setting

A university-affiliated hospital.

Patients

Ten patients with secondary malignant tumours of the liver; 1 with primary hepatoma.

Interventions

Computed portography for preoperative staging; laparotomy and ultrasonographic examination of the liver; cryosurgical ablation of liver tumours with or without a concomitant resection. Thirteen procedures were performed on 11 patients.

Main Outcome Measures

Preoperative morbidity, disease-free and overall survival.

Results

Of 24 lesions frozen, the procedure on 4 lesions was considered a technical failure because of persistent disease. There were no perioperative deaths. One patient had a liver abscess that resolved with percutaneous drainage. One patient had a biliary fistula that resolved spontaneously, and one had a transient rise in the serum creatinine level. Of 11 patients treated, 7 had a recurrence in the liver (persistent disease in 2 and new liver metastases in 5); 2 of these patients died. One patient died of distant disease with no local recurrence. At the time of writing, one patient was alive with extrahepatic disease and no local recurrence and two were free of disease.

Conclusions

Cryosurgery of the liver is a relatively safe procedure that allows treatment of otherwise un-resectable malignant disease. Proof of long-term benefit requires further experience and follow-up.  相似文献   

12.

Background and objectives

We present a large study of patients with proximal fibula resection. Moreover we describe a new classification system for tumour resection of the proximal fibula independent of the tumour differentiation.

Methods

In 57 patients the functional and clinical outcomes were evaluated. The follow-up ranged between six months and 22.2 years (median 7.2 years). The indication for surgery was benign tumours in ten cases and malignant tumours in 47 cases. In 13 of 45 patients, where a resection of the lateral ligament complex was done, knee instability occurred. In 32 patients a resection of the peroneal nerve with resulting peroneal palsy was necessary.

Results

Patients with peroneal resection had significantly worse functional outcome than patients without peroneal resection. An ankle foot orthosis was tolerated well by these patients. Three of four patients with pathological tibia fracture had local radiation therapy. There was no higher risk of tibia fracture in patients with partial tibial resection.

Conclusions

Resection of tumours in the proximal fibula can cause knee instability, peroneal palsy and in cases of local radiation therapy, a higher risk of delayed wound healing and fracture. Despite the risks of proximal fibula resection, good functional results can be achieved.  相似文献   

13.

Purpose

Study reports clinical and functional outcomes of surgical treatment in a case series of nine patients with distal fibular tumours.

Methods

Nine patients with distal fibular tumours were observed between 2005 and 2010. A PubMed search was performed using the terms “fibula”, “lower limb tumour [cancer]”, “sarcoma”, “Ewing”, “peroneal”, “fibular metastasis”, and “limb-salvage surgery”.

Results

In all our patients, lesions were unilateral. All patients complained of pain; limping was present in 5 of 9 tumours. Patients were managed surgically, except one who underwent local radiotherapy. In six patients, a benign or tumor-like lesion was detected. Malignancies consisted of metastatic lung adenocarcinoma (two cases) or multifocal mesenchymal cancer (one case). Non-malignant lesions were treated by curettage and filling, followed by internal fixation when needed. In malignant or locally aggressive lesions, metadiaphyseal fibular resection was performed. The literature search retrieved either case reports or small case series, reflecting the rarity of distal fibular tumours. Surgical treatment was successful in all patients with benign lesions, whereas the rate of success was 40–100 % in case of malignancies.

Conclusions

Given the low incidence of distal fibular tumours, controversies exist about the optimal surgical management. Clinical observation and imaging should be reserved to asymptomatic benign lesions. In non-malignant tumours causing pain, limping, and pathological fractures; in malignancies, surgery is recommended. Finally, in patients with asymptomatic lesions of uncertain nature, biopsy and histological examination should be performed to plan appropriate management.  相似文献   

14.

INTRODUCTION

Spontaneous ureteric rupture is a rare entity that presents as an extravasation of urine from the ureter without previous surgery, ureteric manipulation and external trauma of the ureter. We report the case of a desmoid tumour presenting as spontaneous ureteric rupture which was managed in our institution.

PRESENTATION OF CASE

A 28 years old healthy male presented with a four day history of generalised abdominal pain secondary to spontaneous right ureteric rupture. Patient was initially managed via insertion of nephrostomy tube and antibiotics. After unsuccessful attempts of retrograde and antegrade ureteric stent insertion, patient was subsequently managed via elective surgical intervention. The excised specimen revealed desmoid tumour as cause of the ureteric rupture.

DISCUSSION

Desmoid tumours are rare benign tumours arising from fascial or musculoaponeurotic structures that do not metastasise, but tend to invade locally. It is often initially managed medically prior to undertaking a definitive surgical intervention. To our knowledge this is the first reported case of ureteric perforation secondary to a desmoid tumour of the mesentery.

CONCLUSION

Spontaneous rupture of the ureter is often misdiagnosed as other conditions. History taking and examination can be unreliable, hence a high level of suspicion and further investigations should be utilised. Once the diagnosis is made, treatment can be individualised based on aetiology.  相似文献   

15.

Objective:

To present the clinical management of a ganglion cyst presenting on the dorsolateral aspect of the foot.

Clinical Features:

A 45-year-old female cyclist complaining of ganglion cyst following training period.

Intervention and Outcome:

Patient was treated with high-frequency electroacupuncture in four consecutive sessions over four weeks, and reported resolution of the cyst following therapeutic intervention.

Conclusions:

Ganglion cysts of the foot are relatively rare connective tissue tumours with variable treatment approaches. Electroacupuncture may be a novel and non-invasive conservative approach for the treatment of ganglion cysts. Further evaluation of the efficacy of such treatment is warranted.  相似文献   

16.

Background

Sublobar resection for non–small cell lung cancer (NSCLC) remains controversial owing to concern about local recurrence and long-term survival outcomes. We sought to determine the efficacy of wedge resection as an oncological procedure.

Methods

We analyzed the outcomes of all patients with NSCLC undergoing surgical resection at the Cancer Centre of Southeastern Ontario between 1998 and 2009. The standard of care for patients with adequate cardiopulmonary reserve was lobectomy. Wedge resection was performed for patients with inadequate reserve to tolerate lobectomy. Predictors of recurrence and survival were assessed. Appropriate statistical analyses involved the χ2 test, an independent samples t test and Kaplan–Meier estimates of survival. Outcomes were stratified for tumour size and American Joint Committee on Cancer seventh edition TNM stage for non–small cell lung cancer.

Results

A total of 423 patients underwent surgical resection during our study period: wedge resection in 71 patients and lobectomy in 352. The mean age of patients was 64 years. Mean follow-up for cancer survivors was 39 months. There was no significant difference between wedge resection and lobectomy for rate of tumour recurrence, mortality or disease-free survival in patients with stage IA tumours less than 2 cm in diameter.

Conclusion

Wedge resection with lymph node sampling is an adequate oncological procedure for non–small cell lung cancer in properly selected patients, specifically, those with stage IA tumours less than 2 cm in diameter.  相似文献   

17.

Objective

To document the epidemiologic and clinical features of benign skull lesions.

Design

A case series.

Setting

St. Michael’s Hospital, a tertiary care facility affiliated with the University of Toronto.

Patients

Thirty-one patients who had a neurosurgical consultation and were discharged from hospital after excision of a benign skull lesion during a 10-year period.

Main outcome measures

Patient demographics, clinical signs and symptoms, radiographic and pathological tumour characteristics, surgical procedure, length of hospital stay, outcome and follow-up.

Results

The 31 patients (6 men, 25 women) had 32 lesions excised. The mean age of the patients was 41.9 years. Osteomas accounted for 63% of the tumours. The most frequent location was the parietal bone. Neurologic symptoms were absent in the majority of calvarial tumours. Useful diagnostic studies included plain skull radiography and computed tomography. Nuclear bone scanning was done in 7 patients. All patients underwent craniectomy, with cranioplasty in most cases. Three patients had new neurologic symptoms postoperatively and 1 patient had incomplete resolution of symptoms.

Conclusions

Benign skull lesions are infrequent, but they require neurosurgical intervention. When necessary, surgical excision can serve to confirm the diagnosis, improve cosmesis and retard the progression of neurologic dysfunction. Of primary importance is the recognition of such lesions by primary care physicians and referral to the surgeon so that an appropriate treatment plan can be made.  相似文献   

18.

Background

With modern advancements in preoperative imaging for liver surgery, intraoperative ultrasonography (IOUS) may be perceived as superfluous. Our aim was to determine if IOUS provides new information that changes surgical strategy in hepatic resection.

Methods

We retrospectively analyzed 121 consecutive liver resections performed at a single institution. Preoperative computed tomography and/or magnetic resonance imaging determined the initial surgical strategy. The size, location and number of lesions were compared between IOUS and preoperative imaging. Reviewing the operative report helped determine if new IOUS findings led to changes in surgical strategy. Pathology reports were analyzed for margins.

Results

Of 121 procedures analyzed, IOUS was used in 88. It changed the surgical plan in 15 (17%) cases. Additional tumours were detected in 10 (11%) patients. A change in tumour size and location were detected in 2 (2%) and 3 (4%) patients, respectively. Surgical plans were altered in 7 (8%) cases for reasons not related to IOUS. There was no significant difference (p = 0.74) in average margin length between the IOUS and non-IOUS groups (1.09 ± 1.18 cm v. 1.18 ± 1.05 cm).

Conclusion

Surgical strategy was altered owing to IOUS results in a substantial number of cases, and IOUS-guided resection planes resulted in R0 resections in nearly all procedures. The best operative plan in hepatic resection includes IOUS.  相似文献   

19.

INTRODUCTION

Although examination under anaesthesia and panendoscopy (EUAP) has traditionally been used in the assessment of patients presenting with oral cavity and oropharyngeal squamous cell carcinoma (SCC), the era of modern medicine with its advanced imaging techniques has meant that the indications for this technique have potentially reduced.

SUBJECTS AND METHODS

In an attempt to quantify the current use of EUAP in the UK, a structured telephone questionnaire was undertaken of 50 maxillofacial units. Information was gathered regarding whether the technique was adopted on a routine or selective basis. Likewise perceived disadvantages were sought.

RESULTS

Twenty-two units (44%) carried out EUAP on all patients presenting with oral cavity and oropharyngeal SCC. Of the remaining 28 units, all employed EUAP on a selective basis, the most commonly for the assessment of the primary tumour. The most common perceived disadvantage of carrying out EUAP routinely was its potential to increase the waiting time to definitive treatment.

CONCLUSIONS

These results suggest a gradual move towards the selective use of EUAP in patients presenting with oral cavity and oropharyngeal SCC.  相似文献   

20.

Objective:

Primary spinal cord tumours are rare causes of low back pain but can be a significant cause of morbidity if undiagnosed and untreated. The following is a case of a young male patient presenting with low back pain and radicular symptoms caused by myxopapillary ependymoma.

Clinical Features:

A nineteen year old male presented to an orthopaedic surgeon with a long history of back pain. He was initially diagnosed with soft tissue injuries and discharged. He began to experience erectile and bowel dysfunction two years later and was re-referred to the orthopaedic surgeon by his family physician but was lost to follow-up. The patient did not present to the surgeon until two years after his symptom profile changed. At that point, MRI examinations revealed a large myxopapillary ependymoma extending from T12 to L4 that was confirmed by a pathologist.

Intervention and Outcome:

The tumour was surgically resected with subsequent adjuvant radiotherapy. After one year, the patient required continued catheterization and had poor anal tone. His back and leg complaints were almost normal. Follow-up MRI examinations revealed no disease progression or new spinal lesions at 4 years after the initial diagnosis.

Conclusion:

The clinical presentation of primary spinal cord tumours is non-specific and can easily be missed. In cases of chronic back pain, signs and symptoms should be regularly monitored for changes indicative of progressive neurological compromise such as sensory, motor and bowel/bladder dysfunction. If there is deterioration of clinical signs and symptoms, a spinal tumour should be considered in the list of differential diagnoses. Delayed diagnosis and treatment of these rare causes of back pain could lead to poor outcomes; therefore, a referral to a surgeon should be done immediately with proper follow up to ensure continuity of care.  相似文献   

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