Recurrent left-sided heart leiomyosarcoma: should heart transplantation be legitimate?

Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poor postoperative survival that may be measured in months. A leiomyosarcoma of the left atrium was diagnosed in a 47-year-old man. Initial admission was for acute pulmonary edema requiring emergency surgery. The tumor involved the left atrial cavity, and a radical resection was performed. Six months later an isolated myxomatous recurrence was detected. Heart transplantation was then performed. The patient is in good health 20 months after operation with no evidence of residual disease or recurrence. The literature has been reviewed. Surgical resection is not an adequate treatment for leiomyosarcoma of the left atrium and early heart transplantation probably offers the only hope for these patients.     

Primary cardiac sarcoma treated by orthoptic cardiac transplantation. Apropos of a case

The authors report a new case of cardiac sarcoma treated by cardiac transplantation. This treatment has been proposed for these malignant tumours of poor prognosis when simple excision is impossible, with variable results. This patient is in good general condition 20 months after transplantation. Transplantation is a therapeutic procedure which should be considered in malignant tumours limited to the heart.     

Experiences with hemangiopericytoma in cranial base surgery

BACKGROUND: Haemangiopericytomas are rare vascular neoplasms which show either slow local tumour growth or aggressive progression in size with a high tendency of recurrence and metastasis. Manifestations of haemangiopericytomas in the area of the nose and sinuses are supposed to have a relatively benign course. To date clinical and histological parameters for prognosis are uncertain. Therefore in a quite large number of cases only the clinical course allows to evaluate the dignity of a haemangiopericytoma. PATIENTS AND RESULTS: We present our experience with five haemanglopericytomas in contact with the skull base out of a total number of 457 skull base tumours. The site of origin of these five haemangiopericytomas were: nasal septum, pterygopalatine fossa, oronasopharynx, temporal bone, and parotid gland. All neoplasms showed primary or recurrent tumor in contact with the skull base and an intracranial extension was observed twice. One patient died postoperatively of a cerebral infarction. Three patients showed one or more recurrences which were treated surgically. In one case 5 recurrences occurred. Despite of 4 surgical procedures within a period of 15 months, the tumour could be controlled only for a short period of time, and the patient died 16 months after the first operation. CONCLUSION: One has to consider that haemangiopericytomas of the head and neck show a potential malignant course. The vascular tumor may recur decades later. Adequate therapy and life-long follow-up are therefore mandatory in haemangiopericytomas. Complete surgical resection of the tumour is usually the treatment of choice, in spite of the high degree of surgical skill required, especially in advanced tumour and in case of recurrence.     

Sacrococcygeal germ-cell tumours--the Red Cross War Memorial Children's Hospital experience, 1980-1996

OBJECTIVE: To document the experience of Red Cross War Memorial Children's Hospital in the treatment of sacrococcygeal germ-cell tumours. PATIENTS: Twenty-seven patients with sacrococcygeal germ-cell tumours were treated in our hospital from 1980 to 1996. DESIGN: A retrospective review of these patients' records was undertaken. RESULTS: There were 19 female and 8 male patients. Seventeen (63%) presented in the neonatal period, 13 on the first day of life. Complete surgical resection of the tumour was achieved in all patients with mature or immature teratomas (20 patients) and in 2 neonates with malignant tumours. The first of these 2 neonates, with a malignant teratoma, was not given chemotherapy and remains well 10 years later. The second, with a yolk-sac tumour, also received no initial chemotherapy. He relapsed at the age of 9 months and was successfully treated with repeat excision and chemotherapy. All 5 patients first diagnosed after the age of 1 year had malignant tumours. These patients had incomplete surgical resection (3) or biopsy only (2), and 3 were successfully treated with chemotherapy. One patient relapsed with yolksac tumour after initial complete resection of a mature teratoma. She was successfully treated with repeat surgery and chemotherapy.     

Reconstruction of the hemipelvis after the excision of malignant tumours. Complications and functional outcome of prostheses

We treated 35 patients with primary malignant tumours of the periacetabular area by resection and prosthetic reconstruction of the defect. At a mean follow-up of 84 months, 15 patients (43%) were free from disease. The most common complications were deep infection (26%), local recurrence (24%) and recurrent dislocation of the hip (17%). The surviving patients achieved an average of 70% of their premorbid function. This method of reconstruction has a high morbidity and should be performed only at specialist centres, but the functional and oncological outcomes are satisfactory.     

A case of free jejunal graft for anastomotic recurrence after subtotal esophagectomy

A case of anastomotic recurrence after a radical operation for thoracic esophageal carcinoma is presented. A 68-year-old male was treated by subtotal esophagectomy and esophagogastrostomy through retrosternal route. One year after the operation he experienced dysphagia and anastomotic recurrence was detected by an upper gastrointestinal series and fiberscopy. He was admitted to our hospital for radiation therapy. The response was poor and dysphagia wasn't disappeared after radiation therapy at a dose of 10,200 rad. He was consulted to our surgical department for operative therapy. Cervical esophagectomy, partial resection of gastric tube and free jejunal transplantation for the reconstruction were performed. He had been eating anything of food after the operation.     

One hundred years of orthopedics in the Netherlands. III. Bone tumors

In the fifties, patients with a malignant bone tumour were treated with radiotherapy, sometimes supplemented by resection, as recommended by the Bone Tumour Committee established in 1953. In the seventies, improvement of imaging techniques and chemotherapy made limb-saving surgery possible. Currently, 70% of patients with a malignant tumour of the locomotor apparatus are treated by limb saving, oncologically justified surgery. The surgical defects can be repaired with the aid of an endoprosthesis, or bone homografting or autografting. Children are often treated with an operation involving 180% rotation of the ankle to function as the knee, combined with a prosthesis. Adults are sometimes treated with this technique, but also with internal ('standard') prostheses and donor bone. The future will have to make clear which technique is to be preferred in which situation. Probably, typing and prognosis of various tumours may improve, leading to better insight into the treatment. Centralization of the treatment of rare tumours may also contribute to improvement of the treatment and its scientific research.     

Surgical treatment of solitary or common heart ventricle

On the basis of 24 cases reported in the literature and 2 personal observations of the authors the indications for surgical correction of a single ventricle of the heart are discussed. Two different surgical techniques are described: 1) haemodynamic correction of the defect comprising a closure of the venous atrioventricular valve, oversewing the proximal pulmonary artery, and anastomosing the right atrium and the distal pulmonary artery; 2) radical correction--creation of the ventricular septum. The variants of the disease permitting a radical correction are indicated, the results of surgery, the causes of complications and mortality are discussed.     

Subtotal cor triatrium with left partial anomalous pulmonary venous connection--successful correction in an infant

This report concerns a 10-month-old infant with subtotal cor triatrium associated with left sided partial anomalous pulmonary venous connection to the innominate vein. In the operation, we found that the fossa ovalis existed between the right atrium and the accessory atrial chamber. We performed a radical operation which consisted of a resection of the intra-left-atrial diaphragma and a anastomosis of the vertical vein to the left atrium. His postoperative course was uneventful, and the result was satisfactory. We have presented and reviewed our case, and then discussed the embryogenesis and the hemodynamics. To our knowledge, our case represents the first successful surgical repair of this combination of defects in Japan.     

Exposure of macrophage-like cells to titanium particles does not affect bone resorption, but inhibits bone formation

Endocrine tumours of the pancreas, even in case of liver involvement, are generally characterized by a slower evolution and a better prognosis, if compared with ductal carcinoma. This fact gives reason to a radical surgical approach, whenever possible, and to the research of any effective adjuvant treatment. For this purpose, hepatic transarterial chemoembolization (TACE) has been proposed in recent years for the treatment of metastatic endocrine tumours. Out of 80 patients suffering from endocrine tumours of the pancreas, observed between January 1985 and December 1996, 28 (35%) presented liver metastases at the time of diagnosis. Twelve of these patients were submitted to palliative resection of pancreatic tumour and one or more cycles of TACE. Overall survival was 50% (6/12); median survival was 35.4 months (range 4-75). These results suggest that chemoembolization, combined with surgical resection of primary malignancy, appears to be able to control the disease for a certain time and to increase the survival rate.     

Lung transplantation for primary pulmonary hypertension and giant pulmonary artery aneurysm

We report the case of an 18-year-old patient with a giant pulmonary artery aneurysm and primary pulmonary hypertension who was successfully treated with bilateral lung transplantation and complete reconstruction of the pulmonary artery.     

Recurrent malignant acrospiroma. Treatment by chest wall excision

Malignant acrospiroma is an uncommon tumor of the eccrine sweat gland. In contrast to its relatively more common benign counterpart, malignant acrospiroma is highly invasive, often with significant lymphatic and distant metastasis. The establishment of this diagnosis is difficult on both clinical and histopathologic grounds. After diagnosis, wide surgical excision is warranted to completely extirpate these lesions. We report the case of a 66-year-old female with a recurrent malignant acrospiroma. This patient's tumor was removed by wide radical resection, including chest wall excision, followed by reconstructive surgery and radiotherapy. After 16 months there is no evidence of local recurrence or distant metastasis.     

Surgery of soft tissue sarcoma (with the exception of the retroperitoneum)

The place of surgery in the treatment of soft-tissue sarcoma is defined in the light of a review of the recent litterature. Usual treatment combines conservative surgery and radiotherapy. The essential risk factor of local recurrence is the quality of surgical resection, defined by the definitive resection margins. The addition of radiotherapy after inadequate surgery can improve local control, but cannot ensure that obtained after adequate surgery. Some limited tumours can be treated by surgery alone, but radiotherapy remains essential in more advanced tumours. Under these conditions, local recurrence rates after surgery alone, surgery and systematic radiotherapy and surgery with radiotherapy, as required are 27%, 28% and 30% respectively. The development of a local recurrence appears to affect survival in tumour with a good initial prognosis, early recurrence determining unfavourable outcome. Serious postoperative morbidity is observed in 14% of the cases and is responsible for delayed treatment and functional disorders. The use of muscle flaps to fill the surgical defects can reduce these complications. Evaluation of the functional results must be based on predefined objective criteria.     

Implication of VEGF and MMPs in hepatic metastasis of human colon cancer

In contrast to other types of cervical adenocarcinoma, well-differentiated papillary villoglandular adenocarcinoma of the uterine cervix is unique for its tendency to develop in young women and its excellent prognosis. Until now, no tumor recurrence has been reported in the English literature following surgical treatment that varies from conization to radical hysterectomy. We report a case of 47-year-old female who presented with postcoital bleeding and was treated by radical hysterectomy for FIGO (International Federation of Gynecologists and Obstetricians) Stage Ib cervical carcinoma, in which the preoperative cervical biopsy diagnosis was adenocarcinoma. The patient was well at follow-up nine months after surgery. A literature review including treatment implications is presented.     

Association study of structural mutations of the tyrosine hydroxylase gene with schizophrenia and Parkinson''s disease

Although pulmonary metastases are typical of chondrosarcoma, only 2 patients with intermediate grade tumours have been reported with bone metastases and without pulmonary involvement. We report one patient with an intermediate grade chondrosarcoma which metastasised to the lumbar spine following surgical resection of a locally recurrent tumour. The local recurrence and the metastases were resected and she is alive and well after 20 months.     

Rare pancreatic tumours in children (other than nesidioblastosis)

BACKGROUND: The rarity of pancreatic tumours other than nesidioblastosis in children is such that the experience of any one surgeon or institution is small. As a consequence, there is limited information on the appropriate management and outcome of these tumours. For this reason a review was conducted of the experience of a large paediatric surgical institution. METHODS: During a 23-year period, six patients with pancreatic tumours other than nesidioblastosis were treated at the Royal Children's Hospital, Melbourne. RESULTS: Despite the varied modes of presentation and surgery undertaken, all six patients with surgery alone, with follow-up from 1 to 18 years. Two of the tumours were malignant; the remaining four being islet cell adenomata. No form of adjuvant treatment (chemotherapy or radiotherapy) was used. CONCLUSION: These rare paediatric tumours appear to have a good prognosis, even when malignant, and respond well to radical surgical ablation. Adjuvant therapy appears to be unnecessary.     

A surgical case of cardiac malignant lymphoma in right atrium

We reported a case of malignant lymphoma originating from the right atrium. The patient was a 71-year-old man who had no symptoms associated with heart failure and arrhythmias. At the time of admission the patient was suggested the presence of a tumor in the right atrium by an echocardiogram accidentally. Coronary angiography revealed a feeding artery to a tumor. Although cytological confirmation was not obtained, diagnostic extirpation of tumor was performed under cardiopulmonary bypass. The histological diagnosis was malignant lymphoma of B-cell origin. His postoperative course was uneventful and no recurrence had been observed one year postoperatively without chemotherapy.     

Petrosectomies for invasive tumours: surgery and reconstruction

Tumours involving the temporal bone have historically carried a bad prognosis. The only prospect of cure is radical en bloc resection. Temporal bone resection for malignancies is, however, such a formidable undertaking that many centres label such tumours as unresectable. Additionally, the enormity of the surgical defect poses a major reconstructive challenge. A review of 14 petrosectomies (in 12 males and 2 females) performed for extensively invasive neoplasms in and around the ear is presented. All underwent immediate reconstruction, the majority (12/14) with free tissue transfers. 9 of the 14 patients (64%) are still alive after a mean follow-up of 70 months (range 4-8 years). With the use of free tissue transfers, an aggressive approach with regard to the resection margins can safely be adopted in the full knowledge that the eventual size of the defect need not compromise tumour clearance. Additionally, free flaps provided a reliable dural seal. This approach of radical en bloc resection with free flap reconstruction has decreased the mortality (compared to the literature), while largely reducing the morbidity to that of unavoidable cranial nerve resection.     

Giant cranial base tumours

Thirty-three patients with giant (diameter > or = 4.5 cm) cranial base tumours who underwent surgery at the Hadassah Hospital over the last ten years are described. Twenty-three of the patients had meningiomas, 4 neurinomas, one giant cell tumour, one haemangiopericytoma, and 4 had malignant meningiomas. Four tumours were at the cerebellopontine angle, 9 within the anterior cranial fossa, 8 petroclival, 8 on middle fossa floor, and 4 along the sphenoid ridge. The average pre-operative symptom duration was 31 months, range 3-180 months. Nineteen patients had a radical tumour resection, 10 subtotal, and 4 a partial resection on an average 1.7 operations per patient. The mean follow-up period from the first operation was 39 months (range 2-120). There was no mortality peri-operatively or during the follow-up period. The mean pre-operative Karnofsky score was 68 and at the last follow-up 76. There was no correlation between histology and degree of resection, complications, or status at last follow-up. The best resections (92% radical) and outcome (mean Karnofsky 92) with the least number of operations (mean 1.4) were in the anterior fossa and along the sphenoid wing. The patients requiring the most operations (mean 2.1), having the smallest percentage of radical resections (25%) and the least favourable outcomes (mean Karnofsky 52) were those with petroclival tumours. Patients with giant cranial base tumours have a good overall long-term prognosis, but especially those with petroclival tumours challenge us to improve our techniques.