Phyllode tumours of the breast. A review of 21 cases

Twenty-one cases of phyllode tumours of the breast (9 malignant, 4 borderline and 8 benign) were reviewed. Three patients with malignant tumours developed metastases, and all died. Eight patients developed local recurrence, 6 in the malignant and borderline groups and 2 in the benign group. The classification into malignant, borderline and benign groups is a reliable guide for use when deciding on the extent of surgery. Malignant and borderline tumours require local mastectomy, while tumour excision suffices for benign disease. The customary pathological separation of malignant phyllode tumours from primary sarcoma of the breast is questioned.     

Malignant tumours of bone: clinical aspects and natural course.

An accurate pathological diagnosis must be made prior to treatment of a primary malignant bone tumour. Consideration must be given to the clinical and radiologic aspects as well as the histology. Both benign and malignant tumours occur more frequently in certain decades. A search should be made for precursor lesions such as Paget's disease. The presenting manifestations of pain, a mass and dysfunction are not specific for tumours. Laboratory tests may be helpful, especially in distinguishing tumours from infections and metabolic diseases. Metastasis is usually via the blood stream to the lungs and bones. The low survival rate following amputation for osteosarcoma and radiation therapy for Ewing's sarcoma has been improved by chemotherapy. The lower-grade tumours such as aggressive giant cell tumour and low-grade chondrosarcoma can often by treated successfully by resection and insertion of an autograft, an allograft or a metallic implant.     

Clinical and immunohistochemical characteristics of benign giant cell tumour of bone with pulmonary metastases: case series

PURPOSE: Giant cell tumour of bone with pulmonary metastases is rare. However, some patients die of pulmonary metastases, and histological examination cannot distinguish between benign tumour and malignant metastases. In this study, we present clinical and immunohistochemical findings associated with giant cell tumour of bone with pulmonary metastases. METHODS: Five patients with benign giant cell tumour of bone with pulmonary metastases (one man and 4 women) were studied. Patients' ages ranged between 20 and 23 years (mean age, 21.8 years). Tumours were in the distal femur in 2 cases, and in the proximal tibia, distal tibia, and lumbar spine in one case each. The tissue specimens from primary tumours, recurrent tumours, and pulmonary metastases were studied using immunohistochemical techniques. RESULTS: Three of the 5 primary tumours were of the spontaneous regression or growth cessation type, or the continuously slow-growing type, showing 4.2% to 6.2% of positive cells for Ki-67 after immunohistochemical staining. However, 2 patients with the rapid-growing type of disease died of pulmonary metastases; their primary, recurrent, and metastatic tumour specimens contained 9.0% to 11.5% of positive cells for Ki-67. CONCLUSION: Three of the 5 primary tumours had a benign clinical pattern and immunohistochemistry. Two of the 5 patients died of pulmonary metastases, which had an aggressive clinical pattern and a high prevalence of positive cells in Ki-67. Examination of Ki-67 should be carried out for aggressive type of giant cell tumour.     

Tumours of the central nervous system

The World Health Organization (WHO) classification lists 85 types of primary tumours in the central nervous system, each grouped according to their cell lineage. Simpler classifications exist for various clinical categories. The central nervous system (CNS) comprises both brain and spine, and different tumours are commonly found in one region over another. Children tend to acquire different types of tumours to adults, although some tumours occur in both groups. Primary tumours arise from the local cells in the area, but secondary tumours (metastases) are thought to arrive by haematogenous spread. The grade of the tumour, a histological diagnosis, informs us of its nature in terms of how benign or malignant (invasive/infiltrative) it is, and so helps us predict prognosis. Benign tumours, because of size or position, can be life-threatening. In most cases the mortality rates of CNS tumours have improved over time, but despite the technological advances in medicine there are some groups of tumours whose prognosis has remained unchanged for the past 50 years. We describe the general principles of diagnosing and treating CNS tumours with regard to the common tumour types in each category.     

Cartilage tumours of the bone. Diagnosis and therapy

Primary malignant bone tumours are rare. The annual incidence of these tumours is 10 per 1 million. Nearly 30% of the primary malignant bone tumours are malignant cartilage tumours. The frequency of benign cartilage tumours cannot be definitely estimated because these tumours are normally clinically inapparent and therefore often diagnosed as an incidental finding. The cartilage tumours appear as benign lesions (e.g. chondroma), as borderline tumours (proliferative chondroma vs grade I chondrosarcoma) or as highly malignant chondrosarcoma (e.g. dedifferentiated chondrosarcoma). Commensurate with the different clinical and oncological manifestations of the cartilage tumours, there are wide differences in the treatment and clinical course of the individual tumour. This article discusses the problems in the diagnosis and treatment of cartilage tumours from an orthopaedic point of view.     

Knorpelbildende Knochentumore

Primary malignant bone tumours are rare. The annual incidence of these tumours is 10 per 1 million. Nearly 30% of the primary malignant bone tumours are malignant cartilage tumours. The frequency of benign cartilage tumours cannot be definitely estimated because these tumours are normally clinically inapparent and therefore often diagnosed as an incidental finding. The cartilage tumours appear as benign lesions (e.g. chondroma), as borderline tumours (proliferative chondroma vs grade I chondrosarcoma) or as highly malignant chondrosarcoma (e.g. dedifferentiated chondrosarcoma). Commensurate with the different clinical and oncological manifestations of the cartilage tumours, there are wide differences in the treatment and clinical course of the individual tumour. This article discusses the problems in the diagnosis and treatment of cartilage tumours from an orthopaedic point of view.     

Pathology of paediatric bone tumours

Primary bone tumours account for less than 0.2% of all neoplasms but malignant bone tumours represent the third most common cause of cancer deaths in children and adolescents. The rarity of bone tumours in itself is a diagnostic challenge but is compounded by the number of tumour subtypes on top of which the imaging and histological features of degenerative and reactive processes, and benign bone tumours can simulate bone sarcomas. Furthermore, even in children bone lesions may represent metastatic disease. Hence the assessment of a bone tumour in a child or adolescent should be performed in a specialist referral bone tumour centre which has access to a multidisciplinary team and molecular diagnostic tests: the latter provides greater diagnostic accuracy. It is now appreciated that germline alterations occur more commonly than previously recognised in children and young adults presenting with osteosarcoma and Ewing sarcoma. Awareness of this is important as genetic counselling and screening may be appropriate. In this article epidemiology, radiology, pathology, genetics, treatment and prognosis of most commonly encountered bone tumours among the paediatric population are reviewed.     

Primary solid tumors of the epididymis. Apropos of 4 cases of benign tumor and one case of malignant mesothelioma

Based on a series of 5 tumours of the epididymis, the authors recall the relative frequency of primary solid tumours of the epididymis. They present 2 benign mesotheliomas and discuss the various histogenetic theories for their development, one fibroma, one haemangio-fibro-leiomyoma and one malignant mesothelioma of the cord, invading the epididymis. These are rare tumours (Broth's review of the literature revealed 278 cases in the world, consisting of 209 benign tumours (75%) and 69 malignant tumours (25%). The most frequent benign tumour is the mesothelioma (75% of the benign tumours), formerly referred to as an "adenomatoid tumour". Its histogenesis is still controversial (epithelial or mesothelial origin). The mesothelial theory is the more widely accepted. According to the majority of authors, malignant mesothelioma of the epididymis does not exist, but our case of malignant tumour questions this concept. The other tumours consist of benign and malignant mesenchymal, epithelial or dysembryoplastic tumours. The borderline with spermatic cord tumours is not always easy to define. The authors recall the value of comparative scrotal ultrasonography for the topographical diagnosis of tumours of the epididymis and testicular tumours and the differential diagnosis with certain epididymal cysts.     

A Consecutive 7-Year Series of 1331 Benign Soft Tissue Tumours: Clinicopathologic Data. Comparison with Sarcomas

A consecutive 7-year series of 1331 benign soft tissue tumours was analysed and compared with the data of 72 sarcomas diagnosed in the same period (April 1970 to April 1977). Lipoma was the most frequent benign tumour entity, accounting for almost half of the entire series. Different entities had different male to female ratios and preferred locations and, furthermore, differed from sarcomas in this regard. The factors which discriminated most in the clinical differential diagnosis benign vs. malignant were tumour size and location.

At the first visit to the general practitioner or surgeon it is recommended that patients with large (≥50 mm) and/or deep seated tumours (beneath or fixed to superficial fasciae) or tumours obviously malignant for other reasons (invasion of nerves, vessels or bone) should be transferred, leaving their tumours untouched, to a centre for soft tissue sarcomas for further diagnosis and treatment.     

Tumors of the ribs: experience with 47 cases.

OBJECTIVES: To emphasise the existing difficulties in differentiating benign from malignant rib tumours, and especially the problems that a clinical doctor encounters when dealing with a hyperplastic rib. METHODS: Forty-seven patients with rib tumour underwent surgery in a period of 12 years (1984-1996). In 40 cases (85%), the lesion was benign and in seven (15%) was malignant. Twenty-one benign tumours originated from cartilage and bone, seven were inflammatory, six originated from the bone marrow, and minor percentages (2.5-5%) had vascular, neurogenous, degenerative or miscellaneous origin. Three of the malignant tumours were primary chondrosarcomas and two were metastatic from kidney. The rest were metastatic from stomach (adeno-Ca), and skin (melanoma). The mean age in the benign group was 25.2 years and in the primary malignant group was 20.7 years. Related symptoms were pain (47%) and swelling (42.5%). One-third (32%) of the patients were asymptomatic and the lesion was accidentally found during routine chest radiography. All patients were treated surgically with wide excision of the tumour and the diagnosis was established histologically. RESULTS: Resection was complete and curative in all cases without recurrence. CONCLUSIONS: Since the likelihood of malignancy cannot be excluded, all rib tumours should be considered malignant until proven otherwise. Therefore, prompt intervention is necessary and wide and radical initial excision of the involved rib is advocated.     

Primary Tumours in the Spine and Pelvis in Adolescents: Clinical and Radiological Features

In 34 patients in their two first decades of life with primary bone tumours in the spine and pelvis, the most common benign tumour was histiocytosis X and the most common malignant tumour, Ewing's sarcoma. X-rays were positive in 32 out of 34 cases but of little diagnostic value and primary complaints were without significance. The final diagnosis can only be made after a biopsy.     

Primary tumours in the spine and pelvis in adolescents: clinical and radiological features.

In 34 patients in their two first decades of life with primary bone tumours in the spine and pelvis, the most common benign tumour was histiocytosis X and the most common malignant tumour, Ewing's sarcoma. X-rays were positive in 32 out of 34 cases but of little diagnostic value and primary complaints were without significance. The final diagnosis can only be made after a biopsy.     

Medulloblastoma metastasizing to a breast fibroadenoma: a case report

The extracranial spread of primary intercranial tumours is infrequent, occurring most commonly with medulloblastoma. The spread of one malignant tumour to a second tumour, benign or malignant, is also very rare. This case appears to be unique, in that a primary malignant cerebral tumour metastasized to a benign extracranial tumour.     

Periacetabular limb salvage for malignant bone tumours

PURPOSE: To evaluate treatment outcomes in primary malignant periacetabular bone tumour removal and limb salvage with or without bone-graft reconstruction. METHODS: A total of 13 patients were treated for malignant periacetabular bone tumours at Siriraj Hospital, Bangkok, Thailand. The diagnoses were chondrosarcoma (n=8), Ewing's sarcoma (n=2), osteosarcoma (n=1), well-differentiated osteosarcoma (n=1), and malignant giant cell tumour (n=1). 11 patients did not undergo reconstruction following tumour resection; 2 patients received fibular bone grafts bridging the periacetabulum to the remaining sacrum. Adjuvant chemotherapy was administered for high-grade malignant tumours, and postoperative radiation therapy was performed on patients with a closed surgical margin. RESULTS: At a mean follow-up of 24.3 months (range, 8.9-43.9 months), 9 patients remained disease-free, 3 had died of the disease, and one was alive with disease. According to the Musculoskeletal Tumor Society classification system, the mean functional analysis at final follow-up was 68.7%. Patients who underwent internal hemipelvectomy experienced a subsequent leg-length discrepancy ranging from 3 to 10 cm. Four patients had complications (one each for deep wound infection, skin necrosis, seroma, and vascular spasms) and were successfully treated with multiple debridements and appropriate antibiotics. Three patients had local recurrences; one required a classic hemipelvectomy. CONCLUSION: Malignant periacetabular tumours are difficult to manage. Functional results of our patients with no reconstruction or with bone-graft bridging were fair. Patients undergoing internal hemipelvectomy may experience leg-length discrepancies, which can be balanced with shoe lifts.     

Changes in lactate dehydrogenase isoenzyme patterns in patients with tumours of the central nervous system

Summary The total activities of lactate dehydrogenase (LD) and the LD isoenzyme distribution were determined on homogenates from 61 benign and malignant intracranial tumours, and in serum and cerebrospinal fluid from 45 and 28 of the tumour patients respectively.The LD activity and the LD isoenzyme distribution in serum were not changed in patients with intracranial tumours. The LD activity in the cerebrospinal fluid was raised in about 50% of the tumour patients, but without any significant differences between patients with benign and malignant tumours.Except for very low activities in acoustic schwannomas and in pituitary adenomas, the tumour tissue did not differ significantly from normal brain in total LD activity. The LD isoenzyme distribution showed a pronounced cathodal shift in the grade 3–4 astrocytomas and the metastatic carcinomas, but an anodal shift in the grade 1–2 astrocytomas and the oligodendrogliomas.The meningiomas and the acoustic schwannomas showed a midzone isoenzyme pattern with a dominant LD₃ fraction.Knowledge of the LD isoenzyme distribution can thus sometimes be a complement to the histological examination in the classification of brain tumours.     

Recurrent phyllodes tumours of the breast: pathological features and clinical implications

BACKGROUND: Phyllodes tumours (PT) of the breast are fibro-epithelial neoplasms that are known to recur locally in up to 19% of patients. The failure to achieve adequate surgical margins is an important risk factor for local recurrence. This, however, is a common problem as PT are clinically similar to the more common fibro-adenoma and are therefore often locally excised without any gross surgical margins. It is still debatable as to whether it is necessary to subject the patient to repeat surgery to obtain pathologically negative margins after a diagnosis of a benign or borderline PT is made. Although the majority of recurrences are histologically similar to the initial tumour, a malignant recurrence is possible. Malignant tumours can metastasize through the haematogenous route and metastases are associated with a poor prognosis as they are poorly responsive to conventional chemotherapy. METHODS: We retrospectively reviewed 37 women who presented with local recurrence over a 10-year period to the Singapore General Hospital. Data, including age at the time of diagnosis, clinical presentation, histological features, type of surgery carried out, clinical progression and characteristics of locally recurrent disease, were analysed. Comparisons were made between those with benign, borderline and malignant tumours, as well as between those who developed a malignant recurrence and those who did not. RESULTS: The mean age at the time of diagnosis was 39.6 +/- 7.4 years and the mean tumour size was 6.0 +/- 5.1 cm. A total of 22 patients were classified as having benign tumour, 9 as having borderline tumour and 6 as having malignant tumour. Tumour grade did not influence the tumour size, the adequacy of surgical margins or the time interval to local recurrence or the number of recurrences. Local recurrence occurred after a median interval of 20 months. Although malignant tumours tended to recur earlier, this was not found to be statistically significant. The majority of recurrent tumours were histologically similar to the initial tumour; however, seven patients (19%) developed a malignant recurrence from an initially benign or borderline tumour. Although these tumours were larger, recurred more frequently and within a shorter interval, no significant predictive factor was found on multivariate analysis. Distant metastasis developed only in patients with malignant tumours and accounted for all three mortalities in the study. CONCLUSIONS: It may be acceptable to use an expectant management towards benign and borderline tumours that are excised without adequate surgical margins. However, surgery for locally recurrent tumours, as well as malignant tumours, should aim to achieve adequate surgical margins to reduce the risk of local recurrence, particularly that of a malignant recurrence.     

Malignant tumours of the foot and ankle

Tumours of the foot and ankle constitute about 4–5% of all musculoskeletal tumours with the majority of them being benign. Diagnosis of malignant soft tissue or bone tumours is frequently delayed because of the relatively low prevalence and uncommon presentation at the foot and ankle. Suspicious lesions or lumps should be subjected to CT, MRI and biopsy. Subfascial location, lump size more than 5 cm, increase in size, painful and recurrent tumours should raise the suspicion of malignancy and lead to patient referral to a designated tumour centre. Neoadjuvant and adjuvant systemic chemotherapy and/or radiation therapy, isolated limb perfusion (ILP) and hyperthermia for malignant tumours have to be discussed in a multidisciplinary tumour board. With the advances in local and free tissue transfer, limb-sparing techniques have increasingly replaced primary amputations thus preserving lower limb function as much as possible without compromising on the principles of oncologic resection.     

Gastric stromal tumors. Results of a multicenter study. French Associations of Surgery Research

AIM OF THE STUDY: Gastric stromal tumours are not perfectly known. The aim of this retrospective multicenter study (29 centers) was to improve knowledge of these tumours. PATIENTS AND METHODS: From 1986 to 1994, 159 patients were operated on for leiomyomas (50), leiomyosarcomas (24), malignant/benign schwann cell tumours (10/29), automatic nerve tumours (4), leiomyoblastomas (28), spindle cell tumours (14). The mean duration of follow-up was 5 years. Presenting symptoms, diagnostic procedures, operative and pathological findings, evolution (recurrence, death) were recorded for each patient. RESULTS: Gastrointestinal bleeding and epigastric pain were the most common presenting symptoms (54% and 50% of patients, respectively). Endosonography was the most sensitive examination (97%). Malignant tumours size was greater than benign tumours size (12.6 cm versus 5.2 cm). Extension to contiguous organs or metastases were frequent (33% and 26% of patients, respectively). In 16 patients, pathological examination could not differentiate between malignant and benign tumour. Seven patients who had been operated on for a benign tumour (6%) developed a local (n = 4) or a metastatic (n = 3) recurrence. The 5-year survival rate was 40% for leiomyosarcomas, 28% for schwannosarcomas and 90% for malignant leiomyoblastomas. CONCLUSION: The main feature of stromal gastric tumours is the frequent difficulty to differentiate between malignant and benign tumours. The prognosis of malignant tumours depends on pathological types. The prognosis of benign tumours is uncertain since recurrences may develop.     

Accuracy of computed tomography guided core needle biopsy of musculoskeletal tumours

BACKGROUND: The accurate diagnosis of musculoskeletal tumours is important for successful treatment. Image guided biopsy is gaining increasing acceptance for obtaining tissue for diagnosis. The aim of the present study is to assess the accuracy of computed tomography (CT)-guided core needle biopsy of musculoskeletal tumours. METHODS: This is a retrospective study on a series of 127 patients with a musculoskeletal tumour. The biopsies were performed over a 4-year period from 1998 to 2001. The accuracy of the CT-guided core needle biopsy was determined by comparing the histology of the biopsy with the final histology of the specimen obtained at open biopsy or surgical resection of the tumour. The effective accuracy was determined by the accuracy of the biopsy to distinguish between a benign and malignant tumour. RESULTS: Computed tomography guided core needle biopsy in the present series has an overall accuracy of 80.3%. The effective accuracy as determined by a malignant versus benign lesion was 89%. There were 86 malignant tumours with a biopsy accuracy of 81.4% and there were 41 benign tumours with a biopsy accuracy of 78%. The positive predictive value (PPV) of a malignant tumour is 98.9% and the PPV of benign tumour 90.2%. The most common site of biopsy was from the femur and thigh, together accounting for 39.4% of the tumours. The most common tumours in this series were liposarcoma (n = 12), osteosarcoma (n = 11) and giant cell tumour (n = 11). There were no reported complications arising from the biopsy. CONCLUSION: Computed tomography guided core needle biopsy is a safe and effective procedure that is important in the diagnosis and management of musculoskeletal tumours. It should be performed in a specialized institution with a multidisciplinary musculoskeletal tumour team.     

Primary bone tumours of the thoracic skeleton: an audit of the Leeds regional bone tumour registry.

An audit of the Leeds regional bone tumour registry found that primary bone tumours of the thoracic skeleton constituted 90 of the 2004 cases (4.5%). Thirty seven per cent occurred in the ribs, 32% in the scapulae, 11% in the thoracic vertebrae, 11% in the sternum, and 9% in the clavicles. Malignant tumours were more common than benign (54 v 36) and occurred in an older population (mean ages 47 and 31 years). The scapula was the most common site for malignant lesions and the ribs the most common site for benign tumours. Chondrosarcoma was the commonest tumour in older patients, fibrous dysplasia and plasmacytoma in the middle age group, and eosinophilic granuloma in children. Presenting symptoms were a poor guide to whether the lesion was malignant or not. This and the small proportion of correct preoperative diagnoses indicate the need for early biopsy. Bone tumour registries provide a valuable source of cumulative information about uncommon tumours and facilitate accurate diagnosis, teaching, and research.