Primary tumors of ribs

An analysis of 50 consecutive patients with primary rib tumors operated on at the U.S. Naval Hospital, San Diego, is presented. Thirty-nine patients had benign and 11 had malignant tumors. Men predominated in the entire series, but a greater proportion of women had malignant tumors. The average age in the group with malignant lesions was 41 years compared with 31.3 in the benign group; however, the age range was wide, and extremes were noted in both groups. Fibrous dysplasia and osteochondroma were the most common benign lesions, and Ewing's sarcoma and metastatic lesions comprised the majority of malignancies. Only one chondrosarcoma was encountered.     

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.     

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 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 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 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 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.     

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 brain stem tumours

Summary The material consisted of 31 patients with primary brain stem tumours who were investigated retrospectively. Twenty patients were younger than 16 years of age (range: 1 1/2–65 years). The mean age was 11 years. The female/male sex ratio was 19/12. Histological diagnoses were available for 18 patients. All of the verified tumours were gliomas. The diagnoses in 12 cases were made by section or biopsy; in 15 by standard clinical and radiological examinations and in 4 by exploratory operations.Headaches, mental changes, speech and gait disturbances were the most common symptoms at the onset of the illness and at the time of diagnosis. The mean latency period between the emergence of symptom(s) until diagnosis was 4 months (range: 1/2–48 months). Amongst the dominant objective findings were failure of the V, VI and VII cranial nerves, pyramidal tract symptoms, ataxia and nystagmus, occurring singly or in combination.Pneumoencephalograms disclosed characteristic changes in 25 patients (83%).Fourteen patients (45%) received radiation treatment, from which 5 (36%) obtained a temporary remission in their symptoms. The postdiagnostic mean survival period for the patients was 15 months (range: 0–92 months).The survival time was found to depend on the number of damaged cranial nerve nuclei at the time of diagnosis and on the degree of severity and duration of the accompanying hydrocephalus. The prognosis was unfavourable, as the percentage of patients who survived for 2 years was only 6%, and a total duration of illness longer than 2 years occurred in only 7 patients. None of the patients were still surviving at the conclusion of this investigation.     

Primary bony chest wall tumours

A retrospective review of 81 cases of primary chest wall tumours was carried out to analyse their clinical, radiological and surgical features. There were 37 malignant and 44 benign lesions; 72 were found in the ribs and nine in the sternum, of which five were malignant and four benign. Benign tumours of the ribs outnumbered malignant ones by a ratio of 5:4. The distinction between benign and malignant lesions is difficult clinically and radiologically unless cortical destruction and soft tissue swelling are present. The pathological differentiation is also not possible in all cases. All the patients with benign tumours were treated by excision with no recurrences or deaths. The overall 5-year survival for primary malignant chest wall tumours was 43% and 10-year survival was 27%. These were the results of radical en bloc excisions. Based on our experiences we believe that all tumours of the bony chest wall should be considered potentially malignant and wide excision should be performed, not only to provide adequate tissue for diagnosis but also to allow the best chance of cure in malignant lesions.