Pre-operative irradiation for retroperitoneal liposarcoma: results of a pilot study.

PURPOSE: The aim of this study was to evaluate toxicity and benefit of an original method of preoperative irradiation combined with adequate surgery in the treatment of retroperitoneal liposarcoma. Instead of irradiating the whole tumor volume, 50Gy in 25 fractions was delivered to the posterior part of the tumor and the contact zone with the postero-lateral abdominal wall only. METHODS: Between mid-2000 and end of 2011, 29 patients were included in this study, 22 with a primary tumor, 7 with a first local recurrence. The results obtained were compared with a well-matched control group operated on by the same surgeon during the same period. RESULTS: This therapy was well tolerated by all included patients and no difference in toxicity was found between pilot and control group. With a median follow-up of 84 months the oncological results were similar in both groups with a 5 y disease specific survival of 79 and 81% (p: 0.61). However a very significant difference was found according to histotype: five year disease specific survival was 84% (CI 66-93) for the well- differentiated and only 35% (CI 20-51) for the dedifferentiated liposarcoma (p < 0.0001). CONCLUSIONS: This form of combined treatment is well tolerated but superiority could not be demonstrated. Our experience clarifies some of the difficulties facing a randomized clinical trial on this topic.

Pelvic exenteration: long-term oncological results in a series of 106 patients.

INTRODUCTION: The aim of this retrospective study is to present the oncological results obtained in a series of 106 patients who underwent a pelvic exenteration with curative intent. PATIENTS AND METHODS: Between December 1980 and December 2008 pelvic exenteration was performed in a series of 106 patients, in 69 for gynecologic cancer, in 29 for colorectal cancer, in 6 for urological and in 2 for skin cancer. In only 21 patients it was the primary treatment, in 85 it was for persistent or recurrent tumor. The resection was macroscopically complete in all patients. RESULTS: Overall five-year and ten-year survival was 40% and 33% respectively, disease-free survival 41 and 37%. Survival was better for gynecological tumors than for the other tumors. After supralevatoric exenteration survival was 50% and 47% and better than after infralevatoric exenteration. Exenteration with extension beyond the classical plane of dissection resulted in a 5 year survival of 32%. The only significant difference found was according to the margin status. After R1 resection the median survival was 24 months and the 5-year survival only 9% whereas R0 resection resulted in a 5-year survival of 47% and a local recurrence rate of 13.5%. Fifteen patients died from an unrelated cause. Only 12% of the patients alive 5 years after the operation suffered from recurrent tumor and surgery cured half of them. CONCLUSION: Pelvic exenteration in patients with advanced or recurrent pelvic cancer results in a long-term cure rate of about 50% if an R0 resection has been obtained.

Handling ovarian cancer FIGO III-IV : evolution over the last 30 years.

The treatment of ovarian cancer FIGO III-IV has undergone substantial changes in the last 30 years. As I was involved as an oncological surgeon in the treatment of these patients since 1979 and made my PhD thesis on this subject, I consider myself a privileged witness of this evolution. In the late 1970's two major changes took place: the introduction of combination chemotherapy containing cisplatin and the concept of debulking surgery. In 1980 we embarked on an ambitious treatment plan combining maximal cytoreductive surgery, 6 cycles of chemotherapy, second look laparotomy and panabdominal irradiation. The results were analyzed in 1991 and gave rise to the following changes. Surgical cytoreduction could only be considered optimal if no residual tumor was left and residual tumor correlated with median survival. Upfront surgery was abandoned in FIGO IV and FIGO III with a very high tumor load. Panabdominal irradiation was too toxic. A recent randomized study has established equivalency of survival in FIGO IIIc between interval debulking after 3 cycles and upfront surgery. Initial tumor load remains a determinant of long- term cure and optimal upfront surgery is critical in patients with a metastatic tumor load of less than 100 gram. Retroperitoneal node dissection becomes important when complete resection of peritoneal metastases can be obtained. In experienced hands selection for primary debulking or for interval debulking seems possible at laparoscopic exploration.

V-Y Bilateral gluteus maximus myocutaneous advancement flap in the reconstruction of large perineal defects after resection of pelvic malignancies.

OBJECTIVE: To evaluate the role of the V-Y bilateral gluteus maximus myocutaneous flap (GLM) in the reconstruction of large perineal defects after wide surgical resections for pelvic malignancies. METHOD: Twelve consecutive patients (seven females and five males), of mean age 59 years (36-78), with primary or recurrent pelvic malignancies (rectal, anal and vulvar carcinoma), underwent either abdomino-perineal rectum excision with partial sacrectomy or total pelvic exenteration. The perineal defect was reconstructed by means of a GLM flap. Intra-operative blood loss, operative time, hospital stay, postoperative complications and long-term outcome were retrospectively assessed. RESULTS: One patient died postoperatively. All the remaining patients had at least one early and/or late complication. After a mean follow-up of 31.2 months, seven patients were alive. No major functional impairment in daily activities was observed. Five patients experienced a slight discomfort in either walking, sitting or cycling. CONCLUSION: Gluteus maximus myocutaneus flap is a useful technique for the repair of perineo-pelvic defects after abdomino-perineal rectum excision with partial sacrectomy.

Radiation-induced sarcoma: analysis of 46 cases.

A retrospective analysis was performed of 46 cases of sarcoma treated in our institution between 1989 and 2007 that occurred in a previously irradiated area. Eight male and 38 female patients had received radiotherapy, mainly for breast cancer and genitourinary tumours. The interval between irradiation and the diagnosis of sarcoma ranged from 1 to 54 years (median 15 y). The most common clinical findings were a mass, pain and skin dislocation. Angiosarcoma and sarcoma non-otherwise-specified were the most common histological types. Surgical resection was performed in 34 patients (74%) and 5-year survival was 45% when a radical resection was obtained. No 5-year survival was noticed after non-radical resection and in the absence of surgery. Stage and location of the sarcoma were other prognostic factors. Overall 5-year survival was 27% for the whole group.

Functional evaluation of conventional 'Celsite' venous ports versus 'Vortex' ports with a tangential outlet: a prospective randomised pilot study.

GOAL OF WORK: Totally implantable venous access ports are widely accepted in cancer patient treatment, but withdrawal occlusion (WO) can hamper the use of the device. A newly designed Vortex VX port, with a tangential outlet, should allow better clearance of the chamber, thereby reducing occlusion of the device. The present study compared the Vortex port to the classically shaped Celsite port with regards to functional complications. MATERIALS AND METHODS: Two hundred cancer patients were included in a prospective, randomised controlled trial and randomly assigned to the implantation of a Vortex or a Celsite port. Insertion details such as used vein, catheter tip position and infusion or aspiration abilities were recorded. Data were collected concerning ease of access, and functional evaluation was performed each time the port was accessed, regarding the ability to infuse fluids with a syringe and to withdraw blood by measuring the filling time of a Vacutainer blood tube. MAIN RESULTS: Ninety-nine patients received a Celsite port, and 101 had a Vortex port. Demographic variables and insertion details were comparable in both groups. All functional complications, including WO, total occlusion, sluggish inflow and sluggish withdrawal, were higher in the Celsite group (16.12%) than in the Vortex group (11.36%). This difference was not statistically significant. CONCLUSIONS: This study revealed that functional problems occurred less frequently in Vortex compared to Celsite ports. Differences were small and not significant, which indicates that functional problems may be related to other factors.

Infection assessment of totally implanted long-term venous access devices.

AIM: Comparison of catheter tip versus port content culture techniques to assess infection in totally implanted vascular access devices (TIVAD). MATERIALS AND METHODS: Comparison of pocket swab, catheter-tip and port content cultures after removing the silicon puncture septum in a prospectively collected consecutive series of 102 TIVAD removed for clinical suspicion of infection, between May 2000 and March 2003. RESULTS: 102 totally implanted port-catheters in 98 patients, age ranging from 1 to 90 years (median 53 years), were removed 7 to 2616 days after insertion (median 210 days). Infection of the pocket surrounding the port was found in 21 cases, all proven by a positive culture of the pocket swab. Out of the remaining 81 cases without pocket infection, 32 had only a positive catheter tip culture, whereas 56 had a positive port content culture (p = 0.0002). Always the same microorganism was isolated in the 32 patients with positive catheter tip and port content cultures. The main organisms identified within TIVAD were Coagulase Negative Staphylococcus (CNS) (41 cases) and Candida sp (15 cases). Eight out of the 21 pocket infections were caused by Staphylococcus aureus. CONCLUSION: In the presence of local signs of infection, taking cultures of the pocket surrounding the port is sufficient for diagnostic purposes. When infection is localized within the device only, port content cultures taken after removal of the silicon septum are more often positive than cultures of the catheter tip, and constitute therefore a more reliable tool for the assessment of TIVAD infection.

Changing phenotype of gastrointestinal stromal tumours under imatinib mesylate treatment: a potential diagnostic pitfall.

AIMS: The diagnosis of gastrointesinal stromal tumours (GISTs) is widely based on morphological features and KIT (CD117) immunoreactivity. Most patients with advanced GISTs show a major clinical response after treatment with imatinib mesylate. The histopathological features of GISTs in patients on prolonged imatinib treatment have, thus far, not been addressed in detail. In this report, we present three patients with metastatic GISTs, who received more than 1 year of therapy with imatinib, and whose tumours changed their morphological and immunohistochemical characteristics during continued treatment with the drug. METHODS AND RESULTS: All three primary GISTs from these patients were classical spindle-type tumours, showing diffuse, strong CD117, CD34, and focal alpha-smooth muscle actin expression. During treatment, two clinically progressive and one clinically stable GIST revealed a diffuse epithelioid, or pseudopapillary epithelioid growth pattern, characterized by rounded cells with eosinophilic cytoplasm and uniform round-to-ovoid nuclei. In addition, GIST specimens from patients on therapy showed complete loss of CD117 immunoreactivity. Remarkably, two of these tumours also became CD34 immunonegative and in one case the progression was accompanied by desmin expression. KIT mutational analysis revealed the presence of distinct exon 11 mutant isoforms in all cases examined, while the same genotype was sustained in the base line and on-therapy tumour specimens, proving the common origin of analysed specimens. CONCLUSIONS: GISTs subject to imatinib treatment can undergo striking (immuno)phenotypic changes, which are not necessarily corroborated by new genotypic modifications. Because these may mimic other tumour types, this feature creates a differential diagnostic challenge, of which the pathologist should be aware.

Surgical interventions during STI 571 treatment of metastatic GIST: experience in six patients.

Gastrointestinal stromal tumours (GIST), previously classified as smooth muscle tumours, are the most common mesenchymal tumours of the digestive tract. Since the discovery of KIT (CD 117) expression, these tumours can be diagnosed confidently by pathology. Until recently, surgery was the only treatment available because these tumours were not sensitive to chemotherapy nor radiation therapy. In the long run most of these tumours recurred in the abdominal cavity or in the liver. Recently a new drug STI 573 (Glivec) showed very promising results in metastatic disease with response rates of about 65%. In six patients treated at our center, surgery was indicated during STI therapy because of subobstruction, skin necrosis, abdominal distention, bleeding. Surgery proved to be safe and efficient, allowing continuation of STI therapy in much better circumstances.

Vacutainer filling time through subcutaneous venous access devices.

INTRODUCTION: The functionality of subcutaneous venous access devices is evaluated at least every time the device is accessed. This evaluation is subjective and actions are only undertaken when blood withdrawal and/or fluid injection become problematic. The function of these devices has rarely been evaluated objectively. The present study tried to find an objective and standardized way to evaluate the withdrawal speed of a newly inserted port. METHODS: Between October 2001 and December 2002, a prospective randomized study of 3 types of ports was carried out. The ability to infuse heparinized normal saline and to withdraw blood was evaluated by recording the filling time of a 10 ml Vacutainer tube in 876 newly inserted central venous ports at the end of the surgical procedure. RESULTS: The patient groups were comparable in age, gender and insertion procedure characteristics (vein used, position of the port on the body and length of the catheter). The median time needed to fill a 10 ml Vacutainer tube in all ports was 17.00 sec (range 11.43-63.62 sec). The median filling time for BardPort was 16.36 sec (range 13.48-39.00 sec), for Celsite 18.35 sec (range 12.03-40.00 sec) and for Port-a-cath 16.43 sec (range 11.43-63-62 sec). A significant difference in filling time was found between the large bore catheters (BardPort, Port-a-cath) and the small bore catheter (Celsite) of 2 seconds median value. CONCLUSION: Measurement of withdrawal speed provides an objective criterion for the quality description of blood withdrawal immediately after insertion. A value of more than 20 seconds for filling a 10 ml Vacutainer tube could therefore be suggested a useful trigger for further investigation.

Fibrolipomatous hamartoma in the foot: atypical MR imaging findings.

Lipomatosis of a nerve is a well-known but uncommon entity mostly seen in the median nerve. Magnetic resonance imaging (MRI) has been shown to provide pathognomonic features, obviating the need for diagnostic biopsy. We present a case of lipomatosis of a branch of the medial plantar nerve with an atypical appearance on magnetic resonance imaging.

Delay in diagnosis of soft tissue sarcomas.

AIM: We reviewed 100 patients referred with soft tissue sarcoma between May 1999 to determine doctor- and patient-related delay. METHODS: Patient delay is defined as longer than one month from first symptoms till doctor's visit, doctor delay as longer than one month from first visit till definitive diagnosis. Sixty-eight patients had a multifactional delay. RESULTS: Forty-seven patients showed patient delay, with a median patient delay of 4 months, ranging from 2 to 240 months. The main reason for this delay is a painless mass that is mostly ignored. When pain is present, median patient delay is shorter.Twenty-seven patients experienced doctor delay, ranging from 2 to 79 months, with a median of 6 months. The most frequent reason was a misdiagnosis from the outset, on a clinical basis only, or due to a wrong diagnosis on ultrasound. Only two of these 27 patients had a biopsy, showing a benign tumour. High grade tumours are diagnosed earlier, 85% within 6 months. CONCLUSIONS: Delay in diagnosis of soft tissue sarcomas is still a problem requiring better patient and doctor education.

Complications of radiofrequency coagulation of liver tumours.

BACKGROUND: Radiofrequency coagulation (RFC) is being promoted as a novel technique with a low morbidity rate in the treatment of liver tumours. The purpose of this study was to assess critically the complication rates of RFC in centres with both large and limited initial experience, and to establish causes and possible means of prevention and treatment. METHODS: This is an exhaustive review of the world literature (articles and abstracts) up to 31 December 2001; 82 independent reports of RFC of liver tumours were analysed. RESULTS: In total, 3670 patients were treated with percutaneous, laparoscopic or open RFC. The mortality rate was 0.5 per cent. Complications occurred in 8.9 per cent: abdominal bleeding in 1.6 per cent, abdominal infection in 1.1 per cent, biliary tract damage in 1.0 per cent, liver failure in 0.8 per cent, pulmonary complications in 0.8 per cent, dispersive pad skin burn in 0.6 per cent, hepatic vascular damage in 0.6 per cent, visceral damage in 0.5 per cent, cardiac complications in 0.4 per cent, myoglobinaemia or myoglobinuria in 0.2 per cent, renal failure in 0.1 per cent, tumour seeding in 0.2 per cent, coagulopathy in 0.2 per cent, and hormonal complications in 0.1 per cent. The complication rate was 7.2, 9.5, 9.9 and 31.8 per cent after a percutaneous, laparoscopic, simple open and combined open approach respectively. The mortality rate was 0.5, 0, 0 and 4.5 per cent respectively. CONCLUSION: The morbidity and mortality of RFC, while low, is higher than previously assumed. With adequate knowledge, many complications are preventable.

Is 4q13 a recurring breakpoint in solitary fibrous tumors?

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm found predominantly in the subpleural region but also in many other body sites. We report a malignant solitary fibrous tumor of the peritoneum with a 47,XY,t(4;9)(q13;p23),+5 karyotype. The chromosome 4q13 breakpoint in the presented and previously published case of pleural solitary fibrous tumor with a 46,XY,t(4;15)(q13;q26) karyotype was further characterized by fluorescence in situ hybridization analysis and localized within the 5-cM interval that was flanked by regions specific to YAC clones 761A7 and 886C11. Chromosome translocations involving chromosome 4q13 may characterize a separate cytogenetic subgroup of SFT.

Sleeve-related thrombosis: a new form of catheter-related thrombosis.

In a detailed study of central venous catheter-related sleeve and thrombosis in experimental animals, a new form of thrombosis was detected and termed sleeve-related thrombosis. A silastic catheter was placed in the jugular vein and the anterior vena cava of 22 rabbits and 54 rats. After intervals of 1, 3, 7 days, 2, 3, 4 weeks and 1, 2, 4, 6 months the veins were examined by light microscopy and by transmission electron microscopy. In about 50% of the rats a thrombus was observed at the end of the catheter sleeve. Consecutive cutting allowed the visualization of a transition from a sleeve via part of sleeve and part of thrombus to a pure thrombus. This thrombus was separated from the vein wall and could not be considered a mural thrombus. As the thrombus was only attached to the terminal part of the organized catheter sleeve we propose the name sleeve-related thrombosis.

Angiomyxolipoma shares cytogenetic changes with lipoma, spindle cell/pleomorphic lipoma and myxoma.

Angiomyxolipoma is a rare variant of lipoma, two cases of which have recently been described. We report on the hitherto unreported clonal chromosomal changes of a third case of angiomyxolipoma. The karyotype showed a 46,XX,t(7;13)(p15;q14),t(8;12)(q13;p13)[17]/46,XX[3]. The involvement of 13q14, 12p13, and 8q13 supports a relationship with other types of benign lipomatous and myxoid tumors.

Recurrent chromosome aberrations in fibrous dysplasia of the bone: a report of the CHAMP study group. CHromosomes And MorPhology.

The nosologic status of fibrous dysplasia (FD), a well-known and relatively common bone lesion, is controversial. Information collected by the CHromosomes And MorPhology (CHAMP) study group on published and unpublished cases of fibrous dysplasia shows the presence of clonal chromosome changes in at least a proportion of these lesions. The chromosome aberrations found in FD lesions have been quite variable and have included both structural and numerical changes. Two of the three cases investigated at the study group had trisomy 2 as the sole acquired anomaly. Combined with previously published data, +2 and rearrangements involving chromosome band 12p13 have each been detected in 3 of 8 cases with abnormal karyotype of 11 in which chromosomal analysis has been performed, suggesting that FD is a neoplastic lesion rather than a "dysplastic" process, as has been generally believed and as implied by its very name.