Primary tumours of the small intestine

Tumours of the small intestine are not common. It is essential that an early diagnosis be made so that curative surgery can be carried out and the patient survive. The various clinical presentations and methods of investigation are discussed in order to create an awareness of this type of tumour. The methods of treatment are considered and also the prognosis of malignant growths, which, although poor, compares favourably with that of cancer of the stomach. The relative rarity of these tumours is probably due to the rapid passage of intestinal contents through the small bowel as compared with the stomach or colon.     

Primary tumours of the small bowel (author's transl)

In a period of 21 years we observed 28 tumours of the small bowel (14 benign, 14 malignant), what comes up to 0.85% of all tumours of the GI-tract seen at our hospital. The low rate of incidence of malignomas of the small bowel is supposed to be in relation with the carcinogenic indifference of its contents. 60% of the cases underwent operations for acute complications, such as ileus or peritonitis. Early diagnosis of these tumours is difficult due to their uncharacteristic symptoms and the lack of radiographic signs. Only 4 out of the 14 cases with malignomas underwent radical surgery. The prognosis of malignant tumours of the small bowel is not a good one due to delay of diagnosis, early metastising and local involvement of the mesentery. The 5 year survival rate was 22%. Solitary benign tumours of the small bowel after their removal have an excellent prognosis.     

Mobile tumours in the lumbar spinal canal: a diagnostic problem

In two cases of mobile tumours in the lumbar spinal canal there was difficulty and delay in clinical and radiologic diagnosis because the early symptoms did not correspond to any particular dermatome. Myelography and computed tomography (CT) are the initial diagnostic procedures used in most institutions, even where magnetic resonance imaging (MRI) is available. The purpose of these 2 case reports is to remind clinicians that it is possible for certain tumours attached to the roots in the lumbar spinal canal to migrate, because the roots tend to be redundant or lax. Multilevel search is essential in neuroradiologic studies for early diagnostic confirmation of mobile tumours.     

'Skipping' is still a problem with radiofrequency ablation of small renal tumours

OBJECTIVE: To evaluate the homogeneity and extent of necrosis obtained with next-generation radiofrequency ablation (RFA) equipment and techniques, as incomplete tumour necrosis, or 'skipping', has been documented after RFA of renal tumours and subsequent partial nephrectomy, but this was assumed to result from insufficient energy deposition with first-generation low-energy generators. PATIENTS AND METHODS: In all, 17 patients with solitary renal tumours of 0.5-1.0 cm beyond the sonographically controlled tumour borders. Target temperatures of 105 degrees C were applied in three cycles for 10-30 min at up to 150 W. Tumours were then removed by laparoscopic partial nephrectomy and specimens evaluated by detailed histology. RESULTS: The mean (range) resected tumour size was 22 (11-40) mm, the mean RFA time was 39 (27-59) min and the mean surgical resection time was 25 (12-45) min. In 13 patients, haemostasis was sufficient to avoid the renal pedicle being clamped. Intraoperative repeated positive margins in one patient required a laparoscopic radical nephrectomy. Thirteen (76%) renal masses showed histologically complete ablation of the entire tumour. Of the four RFA failures, three tumours were >3 cm in diameter, two were highly vascularized and three had a very heterogeneous tissue texture. CONCLUSION: Even with state-of-the-art technology, skipping remains a problem with RFA for small renal masses and renders the technique unreliable.     

Primary tumours of the ribs

Thirty-four primary rib tumours (24 benign, 10 malignant) were surgically treated in 1966-1985. The mean age was higher and the tumour diameter was greater in the patients with malignant, than in those with benign neoplasm. The benign tumours were excised without operative death. At follow-up after a mean of 12.3 years there was no recurrence of benign growth, but in two cases with initial diagnosis of chondroma a regrowth at the same site proved to be chondrosarcoma. Among the cases of malignant tumour there was one operative death from pulmonary embolism, after radical resection of sarcoma. None of the four patients with chondrosarcoma had recurrence 6-13 years after surgery. There was no long-term survival among the patients with other forms of sarcoma or malignant tumour of the reticuloendothelial system.     

Primary tumours of the sternum

In 1966-1986, two men and four women (mean age 47.5 years) underwent surgery for primary sternal tumour. Three of the tumours were benign (two condromata, one osteochondroma) and three were malignant (two chrondrosarcomata, one reticulum cell sarcoma). Inflammatory or degenerative lesions impeded differential diagnosis in three additional cases (without tumour). The tumours were treated with radical resection of the affected part of the sternum, including the relevant attached structures. Marlex-mesh reconstruction of the defect was necessary in four cases. There was no operative mortality. One Marlex graft became infected. At follow-up (average 11.1 years, range 9.0-14.7 years), five patients were alive without recurrence of tumour and the sixth had died of unrelated cause.     

Bleeding schwannoma of the small intestine: a diagnostic problem. Case report

A rare case of intermittently bleeding, solitary benign schwannoma of the small intestine is presented. The tumor was identified by abdominal selective angiography. The importance of this investigation in cases of gastrointestinal bleeding without obvious cause is stressed.     

Primary ureteral tumours

The authors report on the clinical picture, diagnostics and therapy of ureteral tumours in 4 patients. The most important symptoms are haematuria and pain. Of the diagnostic possibilities retrograde ureterography is the most significant. Their own surgical solution was ureteronephrectomy with excision of the orifice. Also the conservative therapeutic measures were surveyed. The post-operative control for detecting early recurrences is considered important.     

Primary sternal tumours

Nine cases of primary sternal tumour were retrospectively reviewed in regard to clinical, radiologic and surgical features. The tumours were chondrosarcoma (4), chondroma (3), solitary plasmacytoma (1) and osteochondroma (1). All the benign lesions were excised without complication or recurrence. Three chondrosarcomas were radically excised en bloc, and one was only locally excised because of gross involvement of underlying structures. The solitary plasmacytoma was treated with incisional biopsy and radiotherapy. Two of the three patients with radical excision of sternal chondrosarcoma were alive after 5 years. Careful preoperative assessment, including use of computed tomography, is important. Wide excision should be the procedure of choice for all sternal tumours, since differentiation between benign and malignant lesions may be difficult in cartilaginous tumours, which are the commonest types.     

Primary mediastinal tumours

Al-Naaman, Y. D., Al-Ani, M. S., and Al-Omeri, M. M. (1974).Thorax, 29, 475-481. Primary mediastinal tumours. A review of 28 patients with primary mediastinal tumours seen over a five-year period is presented. Clinical and pathological features of a heterogeneous group of tumours are emphasized. Since a number of patients presented with mild symptoms or were asymptomatic (especially adults), the importance of routine chest radiographs is stressed. Complete excision was accomplished in all patients with benign lesions. Malignant lesions were usually partially resectable and carried a poor prognosis.     

Primary small intestinal tumours: increased incidence of lymphoma and improved survival.

Small intestinal malignancies are rare and may have a delayed presentation owing to insidious growth. We have reviewed the case notes of 25 patients presenting with primary small bowel tumours over a 10-year period. Abdominal pain, weight loss and vomiting were the most common symptoms. The median duration of symptoms was 6 months. Physical examination was normal in 24% of patients. An abdominal mass was present in 46% of cases. Emergency laparotomy was undertaken in 28% of patients. Lymphomas were identified in 72% and adenocarcinomas were present in 16%. The predominance of small bowel lymphoma is an unusual finding and may be related to the high incidence of coeliac disease in the region. The median survival in the lymphoma group was 36 months, which compares favourably with reported series.     

Primary malignant tumours of the bone

Primary bone tumours are rare, with approximately 400 new cases per year in the UK. The diagnosis of bone tumours are hampered by delays in presentation. Radiographic changes are often characteristic for each type of bone tumour and should alert the physician to investigate further. Investigations should include blood tests, local staging and systemic staging. MRI, CT of the chest and isotope bone scans are important in evaluating the tumour. The most common types of bone tumour are osteosarcoma, chondrosarcoma, Ewing sarcoma, spindle cell sarcoma of bone and chordoma. The important pathological features and treatment of each type of tumour are described in this article. Early contact to a supra-regional bone tumour unit is mandatory when a bone tumour is suspected, where a multidisciplinary team approach is employed for an accurate diagnosis and appropriate management. Biopsy and surgical treatment should only be undertaken in such a unit and all patients should be enrolled in international clinical trials, where feasible, to gather data that will ultimately improve outcomes. The survival rates from most bone tumours are of the order of 60–80% with appropriate treatment.     

Primary malignant tumours of the bone

Primary bone tumours are rare, with a prevalence of approximately 550 cases per year in the UK. Late presentation and identification of tumours delays diagnosis and negatively impacts on the survival of these patients. Characteristic clinical and radiological features of bone tumours should alert the physician to investigate further. Investigations should include blood tests and local and systemic imaging. MRI, CT and isotope bone scans are important in evaluating the tumour. The most common types of bone tumour are osteosarcoma, chondrosarcoma, Ewing sarcoma, spindle cell sarcoma of bone and chordoma. The important pathological features and treatment of each type of tumour are described in this article. Early contact with a supra-regional bone tumour unit is mandatory when a bone tumour is suspected, where a multidisciplinary approach to management is employed. Biopsy and surgical treatment should be carried out in these units wherever possible. Patients should be enrolled in international clinical trials, where feasible, to gather data that will ultimately improve outcomes. The survival rates from most bone tumours are 60–80% with appropriate treatment.     

Primary endocrine tumours of the liver

BACKGROUND: In patients with hepatic endocrine tumours, a primary neoplasm is not always found elsewhere despite extensive investigations, raising the possibility that the hepatic lesion is the primary tumour. The aim of this study was to assess the incidence, characteristics and prognosis of patients with primary hepatic endocrine tumours. METHODS: Patients with histologically confirmed hepatic endocrine tumours identified since 1993 were reviewed. All those with no primary tumour identified by computed tomography of the thorax, abdomen and pelvis, upper and lower digestive endoscopy, duodenopancreatic endoscopic ultrasonography or somatostatin receptor scintigraphy (SRS) were included. Clinical and tumour characteristics were assessed retrospectively. RESULTS: Of 393 patients with digestive endocrine tumours, 17 (seven men; median age 55 (range 26-69) years) had hepatic endocrine tumours without evidence of an extrahepatic primary lesion either at diagnosis or during a median follow-up of 43 (range 12-108) months. Ten patients had multiple and seven had single tumours. The tumours were non-functional in 13 patients and well differentiated in 14 patients. SRS was positive in the liver in 11 patients. Curative resection was performed in seven. Overall actuarial survival rates were 100, 69 and 51 per cent at 1, 3 and 5 years respectively. Only poor differentiation was associated with an unfavourable outcome (relative risk 20.8; P < 0.001). CONCLUSION: Primary hepatic endocrine tumours were identified in almost 5 per cent of patients with digestive endocrine tumours. Poor differentiation was the only factor associated with unfavourable outcome.     

Primary malignant tumours of the bone

Primary bone tumours of bone are rare, with approximately 400 new cases per year in the UK. The diagnosis of bone tumours are hampered by delays in presentation and diagnosis. There are x-ray changes which are often characteristic for each type of bone tumour and x-rays should alert the physician to investigate further. Investigations should include blood tests, local staging and systemic staging. MRI, CT of the chest and isotope bone scans are important in evaluating the tumour. The most common types of bone tumour are Osteosarcoma, Chondrosarcoma, Ewing’s sarcoma, Spindle Cell Sarcoma of bone and Chordoma. The important features and treatment of each type of tumour are described in the article. Early contact to a tertiary referral bone tumour unit is mandatory when a bone tumour is suspected, where a multidisciplinary team approach is employed. Biopsy and surgical treatment should only be undertaken in such a unit and all patients should be enrolled in international clinical trials in an attempt to improve outcomes. The survival rates from most bone tumours are of the order of 60-80% with appropriate treatment.