Effective treatment for malignant mediastinal teratoma.

Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series.     

Diagnosis, treatment and outcome of parathyroid cancer. A report of eight patients.

Eight patients presented parathyroid cancer between 1963 and 1990. There were seven women and one man with a median age of 55.5 years (range 34-81). At admission the median serum calcium concentration was 3.75 mmol/l (range 3.0-5.0), and four patients presented with a hypercalcaemic crisis. Six patients had a pathological bone resorption and five had reduced kidney function or urinary stones. All cases were treated by en bloc resection of the tumour and affected adjacent structures. One patient with a local lymph node metastasis died after seven months, and one in whom it was impossible to remove the tumour died after 14 months. Two patients had recurrences three and nine years after the operation, respectively. The first was treated with mithramycin, but died 33 months later, the other was reoperated on and was still normocalcaemic 2-9 years after diagnosis.     

Right hepatic lobectomy for primary and metastatic tumour: experience in a community teaching hospital

The first eight patients who required either a total or extended hepatic lobectomy for malignant tumour at a community teaching hospital are reviewed. Two patients had primary liver tumours and six had metastatic adenocarcinomas, five from colorectal and one from an unknown primary tumour. There were no deaths perioperatively, morbidity was minimal and survival was encouraging. Three patients died of disseminated disease 9, 16 and 18 months respectively after hepatic resection. However, five patients were free of disease 19, 27, 52, 63 and 80 months after operation. The authors' initial experience compares favourably with that of others and demonstrates that major hepatic resection can be done safely and can offer prolonged survival of good quality to a selected group of patients, even when the tumour load is large.     

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.     

Extracorporeally irradiated autografts in pelvic reconstruction after malignant tumour resection

We treated 15 patients suffering from an extracompartmental malignant pelvic tumour by resecting the affected part of the bone, irradiating it extracorporeally with 300 Gy, and reimplanting it after having removed the bulk of the lesion. Adjuvant therapies were used according to the type and extension of the tumour. Follow-up was an average of 4 years 6 months. Complications were seen in 13 patients. Most mechanical complications were related to the use of hip arthroplasties. Internal fixation of the graft failed in one case only, infection was seen in three cases, and seven patients died after local recurrence. Functional scores were fair. Although many complications were seen, this method remains our treatment of choice.     

Carcinoid tumours of the thymus.

BACKGROUND--Carcinoid tumours of the thymus are rare. The clinical manifestations, radiographic findings, and cytological features of eight histopathologically verified thymic carcinoid tumours have been assessed. METHODS--One hundred and sixty two patients of mean age 52 (range 31-68) years with malignant mediastinal tumours were reviewed retrospectively and eight cases of thymic carcinoid were identified. Four of the eight patients were diagnosed by percutaneous ultrasound guided fine needle aspiration biopsy via a parasternal approach. RESULTS--Two patients had Cushing's syndrome at presentation and four had symptoms and signs secondary to mediastinal compression. Two were asymptomatic. Local extension of the tumour to pleura, pericardium, great vessels, phrenic nerve or regional lymph nodes, or both, were found in seven patients. Only one had the tumour confined to the thymus at diagnosis. Distant metastases were found in two patients, one to both lungs and the other in the iliac bone. Local recurrence or distant metastases developed 15-60 months after surgery in four of the five patients who underwent radical resection of the thymic tumour. Three patients died at 17 months, 34 months, and 10 years after diagnosis. The other five patients are alive at 9-51 months. CONCLUSION--Thymic carcinoid is a slow growing tumour with a poor prognosis because of its tendency to local and distant spread. Cytological examination of samples obtained by ultrasound guided fine needle aspiration may provide a useful method for diagnosis in selected patients.     

Rhabdomyosarcoma of the extremities in adults

Rhabdomyosarcomas are uncommon in adults and literature regarding their management is limited. Eight patients with an average age of 32.6 years (range: 21 to 75) who were treated for rhabdomyosarcomas on the extremities between 1991 and 2002 in a regional centre for the treatment of soft tissue sarcomas were studied retrospectively. Treatment consisted of en bloc resection of the primary tumour in all the patients, combined with radiotherapy or chemotherapy or both. The tumour size ranged from 5.5 cm to 15 cm (average 9.3 cm). Histologically there were four alveolar, two pleomorphic, one embryonal and one anaplastic subtype. Seven of the eight patients developed metastasis and died after an average period of 15.3 months (range 4-28 months). At the final follow-up, only one patient was disease free and alive 48 months after surgery. Extremity rhabdomyosarcoma is a highly malignant tumour and our results are poor compared to the reported results in children. Metastatic recurrence is high in adult rhabdomyosarcomas even with local control of the disease and therefore development of effective systemic therapy is an urgent priority.     

Diathermy resection and radioactive gold grains for palliation of obstruction due to recurrence of bronchial carcinoma after external irradiation.

During 1985-7 15 patients previously treated by external radiotherapy for inoperable carcinoma of the lower trachea and major bronchi underwent endobronchial insertion of radioactive gold grains as a palliative procedure for relief of symptoms. Four patients had undergone three or more endobronchial laser treatments before being referred. Under general anaesthesia diathermy was used to resect obstructing tumour before the insertion of the gold grains into the tumour and the compressed endobronchial wall. Four patients died within one month. The remaining 11 patients were symptomatically and objectively improved when assessed at one month. Both collapsed lungs and three out of six collapsed lobes had re-expanded. Fourteen of the 15 patients died within 13 months (median survival 2.5 months); one patient with recurrent symptoms after 10 months underwent a further implantation and is alive after two years.     

Prosthetic replacement of the inferior vena cava and the iliofemoral vein for urologically related malignancies

OBJECTIVE: To evaluate the feasibility and results of prosthetic venous replacement, as inferior vena cava (IVC) and iliofemoral vein resection and replacement are sometimes necessary when they are extensively involved by a large and fixed tumour thrombus from renal cell carcinoma (RCC) or other urological malignancies. PATIENTS AND METHODS: Five men and two women (age range 41-75 years) were treated over a 10-year period (1991-2001) by aggressive venous surgery to achieve complete tumour resection, with prosthetic graft replacement to re-establish venous flow. The tumours included RCC of the right kidney (two), retroperitoneal liposarcoma (two), bladder cancer (one), retroperitoneal fibrosarcoma (one) and inguino-pelvic lymphoma (one). Two patients had a vena caval replacement, of whom one had a circular reinforced PTFE and one a Dacron silver graft; five patients had either an iliofemoral or an ilio-iliac circular reinforced PTFE graft. The prosthetic diameter was 18-20 mm for the IVC grafts and 8-10 mm for the iliac grafts. In all the patients, graft patency was evaluated during the follow-up by colour flow duplex imaging, and in one it was determined by angio-computed tomography scan and venography. RESULTS: One patient died 30 days after surgery; of the remaining six patients one had no evidence of regional recurrence or metastatic disease at 12 months, and five died from recurrent tumour 8-30 months after surgery. The mean time to death was 23 months. At 3 months all six prosthesis were patent; at 6 months four were patent and at 12 months three of five prostheses were patent. CONCLUSION: Resecting and replacing the IVC allows complete tumour resection and avoids renal failure, providing durable relief from the symptoms of venous obstruction. Iliofemoral prosthetic reconstruction for urological-related malignancies represents a viable option to avoid venous engorgement and lower extremity swelling, at least in the early postoperative period. The mean time to death for the present patients must be considered the limit for these aggressive operations.     

Stromal tumors of the small intestine. Retrospective analysis of 9 cases surgically treated

The authors report on their experience with 9 patients with small bowel stromal tumours who underwent surgical treatment over the period 1974-2001. Seven were males and 2 females, with an average age of 63.1 years (range: 49-72 years). Histologically, 4 tumours showed evidence of differentiation towards smooth muscle elements (1 benign and 3 malignant), 4 towards neural elements (all malignant) and 1 lacked differentiation towards either cell type. Five tumours were located in the ileum, 3 in the jejunum and 1 in the duodenum. The main symptoms were abdominal pain and an abdominal mass, and the most sensitive diagnostic technique was abdominal CT scan. In the 8 jejunal or ileal stromal tumours we performed a typical intestinal resection, while undifferentiated duodenal stromal tumours were managed by pancreaticoduodenectomy. The diagnosis was only histological. There was no operative mortality, while 2 postoperative complications (1 pancreatic fistula and 1 myocardial infarction) occurred. The patient with jejunal benign muscular stromal tumour is still alive and in good health 73 months after the operation. Of the 3 patients with malignant muscular ileal stroma tumours, 1 is alive and free from disease 63 months after the operation, while the other 2 died of metastatic disease 39 and 29 months after surgery. Of the 4 patients with malignant neural stromal tumours (2 jejunal and 2 ileal) 1 with jejunal and 1 with ileal tumour were lost to follow-up, while 1 is still alive and in good health 101 months postoperatively; the 4th patient, with jejunal disease, developed liver metastasis 14 months after small bowel resection and died 12 months later. The patient with undifferentiated duodenal stromal tumour died of liver metastases 38 months after pancreaticoduodenectomy. Small bowel stromal tumours are more often than not malignant. The most frequent symptoms are abdominal pain and a palpable mass, but no specific signs have been detected. Abdominal CT scan is the most sensitive diagnostic technique in the evaluation of the location, size, invasion of adjacent organs and metastases. The treatment must be intestinal resection, and prognostic prediction on the basis of histological findings is difficult.     

Second hepatic resection for recurrent hepatocellular carcinoma

During the last 5 years, radical hepatic resection was performed in 91 patients with hepatocellular carcinoma (HCC). Thirty-one of them had tumour recurrence in the remaining liver during the follow-up period. Second hepatic resection was carried out on nine of them 4-38 months after the first hepatectomy. The ages of these patients ranged from 39 to 65 years with an average of 53.7. There were six men and three women. Eight patients had underlying cirrhosis of the liver and one chronic active hepatitis. Six patients are alive, four being free of HCC and two with disease, for 15-45 months after the first operation. Two patients died of systemic cancer dissemination. The remaining patient had tumour recurrence in the liver again and died of hepatic failure after the third laparotomy. The survival rate of these nine patients was significantly better than that of twenty-two patients who were treated by other palliative methods. The present result shows that a second hepatic resection is a possible and meaningful method of treatment for the patients with recurrent HCCs in the liver remnant.     

Repeated resection for malignant liver tumours.

Fifteen repeat hepatic resections were performed on 12 patients with either recurrent or residual malignant tumours of the liver. Of these, one patient underwent three repeat resections and another underwent two. Five had primary liver liver tumours and seven had liver metastases. Planned, ''staged'', repeat resections were performed on three patients because of multiple deposits of tumour, cirrhosis or extensive disease at initial presentation. There was no operative mortality. The period of follow-up from the time of repeat sections ranged between 4 months and 36 months during which two patients died from recurrent disease. The mean survival after the repeat resection was 16.8 months (range 4-36 months). Although technically demanding, repeat hepatectomy is feasible and provide similar benefits.     

Reconstruction and limb salvage after resection for malignant bone tumour of the proximal humerus. A sling procedure using a free vascularised fibular graft.

We assessed the intermediate functional results of eight patients after wide resection of the proximal humerus for malignant bone tumour. We used a free vascularised fibular graft as a functional spacer and a sling procedure to preserve passive scapulohumeral movement. Scapulohumeral arthrodesis was not carried out. Five patients had osteosarcoma, two achondrosarcoma and one a malignant fibrous histiocytoma of the bone. The mean duration of follow-up was 70 months (median, 76) for the seven patients who were still alive at the time of the latest follow-up. One patient died from the disease 12 months after surgery. There were no local recurrences. The functional results were described and graded quantitatively according to the rating system of the Musculoskeletal Tumour Society. Our results were satisfactory with regard to pain, emotional acceptance and manual dexterity. Function and lifting ability were unsatisfactory in two patients. One patient had delayed union between host and graft, but this united after six months without further surgery. Radiographs of the shoulder showed absorption or collapse of the head of the fibula in four of the eight patients and a fracture in another. No functional problems related to absorption or fracture of the head of the fibula were noted. There was no infection or subluxation of the head. We conclude that this is a reasonably effective technique of limb salvage after resection of the proximal humerus.     

Gastric stromal tumours: a practical approach

Recent findings on the pathological diversity of gastric stromal tumours and their unpredictable behaviour prompted us to review our series of 16 patients who had undergone surgery for these tumours from 1991 to 1998. There were 13 benign and 3 malignant lesions. The majority of patients presented with either upper gastrointestinal bleeding or anaemia alone (12 of 16). Endoscopy was an extremely useful diagnostic tool, revealing the lesion as an intraluminal protuberant tumour with or without ulcer in 10 cases and as an ulcer alone in 4 cases, and in 1 case features suggesting an extrinsic mass. All the patients in the series underwent surgery. We used staplers (AutosutureR TA 55) to excise the tumours in 7 cases, all of which on histological examination were benign with clear resection margins. Gastric resections were performed in 5 cases for either large tumours or those situated at the fundus or antrum and local excision of the remaining 4. The mean follow-up of these patients was 24 months. Two patients with malignant lesions died of irresectable recurrences, one 2 months and one 18 months after surgery. There have been no recurrences in the tumours diagnosed as benign on histology. Tumour size, position and the ability to apply the stapler leaving adequate margin below the tumour should be the determinants of extent and type of excision. Reliable determinants of behaviour are tumour size, grade and mitotic index.     

Prosthetic arthroplasty of the knee after resection of a sarcoma in the proximal end of the tibia. A report of sixteen cases

The results of a specific type of prosthetic reconstruction of the knee (total replacement arthroplasty) after resection of a sarcoma of the proximal part of the tibia in sixteen patients were retrospectively reviewed. The diagnosis was stage-IIB osteogenic sarcoma in nine patients, stage-IIB malignant fibrous histiocytoma in three patients, and stage-IB sarcoma of various types in four patients. The length of tibial resection ranged from 100 to 257 millimeters. Of the eleven patients who were available for functional examination (mean duration of follow-up, sixty-three months), three patients had an excellent result, seven had a good result, and one had a fair result. Of the five patients who were not available for functional testing, one who was doing well was lost to follow-up at eighty months, one had died of metastases at sixteen months, and three had had a secondary amputation for infection or for loosening of the prosthesis.     

Tumour induction as a consequence of immunosuppression after renal transplantation

Immunosuppressed recipients of organ transplants have a higher incidence of carcinoma than the general population. A retrospective analysis was made at the Department of Urology of Bonn University, investigating 236 renal allograft recipients as to the incidence of neoplasms before and after transplantation. Eleven patients developed malignant tumours after transplantation. In 4 out of these 11 patients, case history showed pre-existing malignancies. Two of the 4 patients developed a second tumour, while the other two had tumour progression (latency period 21–77 months). Three of the 4 patients died of their tumours 21, 42 and 77 months after transplantation, whereas one female patient is still alive and free of neoplasms 32 months after transplantation. In 7 out of these 11 patientsde novo tumours were diagnosed (latency period 3–88 months). All of them are still alive (NED between 15 and 85 months), six of them with good transplant function. There was no difference to be seen in the incidence of malignancies between kidneys supplied by Eurotransplant (n=40) and ABO compatible kidneys from our own donors (n=196). The higher incidence rate of neoplasms in transplant recipients requires high standards in preventive measures. Any suspicious change that may occur in the course of a thorough follow-up of transplant recipients must be removed and examined histologically. Patients with previous malignant diseases must be payed special attention, since they frequently tend to develop another malignant tumour and progression of existing tumours, respectively. As far as immunosuppression is concerned, therapeutic guidelines for the treatment of transplant recipients do not differ from those set up for patients on haemodialysis. Since immunosuppression with increased rates of tumour incidence can also be observed in dialysis patients, the mere fact of increased incidence of neoplasms cannot be taken as an argument against transplantation. With a more or less equal risk of tumour incidence the crucial factor should be the higher quality of life for transplant recipients.     

Resection arthrodesis of the knee with a vascularised fibular graft. Medium- to long-term results

We present the results in 12 patients of arthrodesis of the knee using a vascularised fibular graft after resection of a malignant bone tumour. At a mean follow-up of 95 months (60 to 178) all patients were free from disease although 11 had had at least one complication, with stress fracture of the graft in five patients, nonunion in two and deep infection requiring above-knee amputation in one. Despite the high rate of complications, satisfactory results can be obtained using this technique. Careful preoperative counselling is required.     

Angiographically discovered, non-operated renal artery aneurysms

Angiographies (a total of approximately 9 500) and patient records revealed 56 patients with renal artery aneurysm during the period 1960 to 1974. Fifteen patients were operated upon electively and 3 were lost for follow-up. Rupture occurred in four leading to acute nephrectomy. Thus, 34 patients were left for studies on the natural course. They were 29-82 years old (mean 58 years) at diagnosis and were followed for at least 90 months. Twelve were males and 22 females. The aneurysms had a diameter of 3-25 mm (mean 12 mm), were fusiform in 7 and saccular in 27 cases; 9 were calcified. Repeated angiographies were carried out in 7 patients at intervals of up to 73 months showing slight enlargement of one aneurysm, disappearance of another and no change in 5. Sixteen patients were alive at the end of the follow-up period 90-220 months (mean 157 months) after the first or only angiography. Eighteen patients died after 2-204 months (mean 84 months), 14 of cardiovascular disease unrelated to the renal arteries, 3 of malignant tumour and one of pancreatitis. Autopsy in 12 patients did not show rupture of any renal artery aneurysm. Thus, in a considerable number of patients the disorder was uncomplicated and compatible with a long life.     

Osteoarticular allograft reconstruction of the distal radius after bone tumour resection

Resection of the distal end of the radius is indicated in the treatment of locally aggressive primary benign and malignant bone tumours. The aim of this study was to evaluate the technique of osteoarticular allograft reconstruction of this bone defect. We analysed 12 patients retrospectively with a minimum follow-up of 2 years (range 26-145 months, median 52 months). Three patients had a malignant tumour and nine had a giant cell tumour. The patients ages ranged from 13 to 65 years. The mean resected length of the radius was 6.6 (range 4-14)cm. Non-union of the osteotomy line was diagnosed 6 months after surgery in one case and needed bone grafting. Distal radio-ulnar joint instability was observed in eight cases. Subchondral bone alterations and joint narrowing were present in all cases but were painful in only one patient. The mean range of motion was 51 degrees of flexion and 37 degrees of extension.     

Clinicopathologic studies on mesothelioma of the pleura. Differentiated approach to treatment

A surgical series of 23 patients with pleural mesothelioma is reviewed. Three who had benign localized mesothelioma of fibrous type are alive and well at least 10 years postoperatively. In two others, radically extirpated localized mesothelioma was histologically classified as benign, but later proved to be malignant, causing death from recurrent disease 27 and 79 months postoperatively. Four patients with diffuse malignant mesothelioma underwent pleurectomy or open biopsy and survived for 2-9 months. Radical en-bloc pleuropneumonectomy was performed on 14 patients with diffuse malignant mesothelioma. One patient died postoperatively and the others succumbed to the disease after 3-51 (mean 20) months. The survival time was greater than or equal to 1 year in 62% of the patients and greater than 3 years in 23%. Patient age, histologic tumour type and extent of disease seemed to be important prognostic factors. Despite the generally poor prognosis, the results of radical surgery in this study appear to warrant an aggressive approach to treatment of benign or localized malignant pleural mesothelioma, and possibly also to stage I diffuse malignant mesothelioma of epithelial type.