Radiotherapy in the management of gist: state of the art and new potential scenarios.

BACKGROUND: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. The main treatment for localized gastrointestinal stromal tumors is surgical resection. Unresectable or advanced GIST are poorly responsive to conventional cytotoxic chemotherapy but the introduction of tyrosine kinase inhibitors (TKIs) marked a revolutionary step in the treatment of these patients, radically improving prognosis and clinical benefit. Historically GIST has been considered radiation-resistant, and the role of radiotherapy in the management of patients with GIST is currently restricted to symptomatic palliation in current treatment guidelines. CASE PRESENTATION: Here we report two patients affected by metastatic GIST, treated with radiotherapy and radiosurgery in combination with TKIs, achieving an unexpected objective response in the first case and a significant clinical benefit associated with a local tumor control of several months in the second case. CONCLUSIONS: These and other successful experiences that are progressively accumulating, open up new scenarios of use of radiation therapy in various settings of treatment. GIST is not universally radioresistant and radiotherapy, especially if combined with molecularly targeted therapy, can improve the outcomes for patients diagnosed with GIST.

Successful selective arterial embolizations for bone metastases in renal cell carcinoma integrated with systemic therapies: A case report.

Herein is described the case of a 64-year-old patient affected by metastatic clear-cell carcinoma, with exclusive bone disease, subjected after the initial cytoreductive nephrectomy to 3 successive lines of medical treatment (sunitinib, everolimus, and sorafenib) and multiple locoregional treatments (spinal surgery, radiation therapy, and selective arterial embolization), resulting in a surprisingly long survival of over 75 months. In the era of target therapy, integration strategies, including additional locoregional treatment to medical therapy, are essential to optimize the clinical benefit, to maximize treatment duration overcoming focal progressive disease, and to improve the quality of life. In this context, we would highlight that selective transcatheter embolization of bone metastases from renal cell carcinoma should be considered as an effective and safe option in the palliative setting for patients with bone metastasis, especially for pain relief.

Natural history of honeycombing: follow-up of patients with idiopathic pulmonary fibrosis treated with single-lung transplantation.

PURPOSE: Although honeycombing is one of the key features for the diagnosis of idiopathic pulmonary fibrosis (IPF), its origin and evolution are still poorly understood. The aim of our study was to analyse the natural history of honeycombing in patients treated with single-lung transplantation. MATERIALS AND METHODS: We considered seven patients who underwent single-lung transplantation; two of them (28.6%) were excluded from our analysis because they died in the posttransplantation period, whereas the remaining five (71.4%) were evaluated with computed tomography (CT) over 67.6±38.56 months. Each CT scan was assessed for disease extension and cyst size (visual score and size of target cysts); CT scans acquired after 2006 were also assessed for native lung volume. RESULTS: All patients showed disease progression (with a concurrent reduction in lung volume in two, 40%) and a progression of honeycombing, with increased number and size of cysts in four (80%). We observed dimensional changes in all target cysts (enlargement or reduction); three patients (60%) also had radiological evidence of complications, such as spontaneous rupture with pneumothorax and development of mycetomas within the cysts. CONCLUSIONS: Honeycombing is a dynamic process in which the overall trend is represented by a dimensional increase in cystic pattern; however, single cysts may have a different evolution (enlargement, reduction or complications). This behaviour could be explained by the variety of the pathogenetic processes underlying honeycombing, with cysts that may present abnormal communication with the airway, including the development of a check-valve mechanism.

Surgical debulking of gastrointestinal stromal tumors: is it a reasonable option after second-line treatment with sunitinib?

INTRODUCTION: After imatinib treatment, the surgical management of patients affected by gastrointestinal stromal tumor (GIST) has been widely reported and often considered by many oncologists in clinical practice. Surgical results are correlated with disease responsiveness to tyrosine kinase inhibitors and with complete extirpation of all tumor sites. By now, no report specifically addressing surgical management after second-line treatment with sunitinib is still available. Most patients have an unresectable disease and do not have any other therapeutical options except for clinical trials. MATERIALS AND METHODS: We report two clinical cases of patients with metastatic GISTs, who underwent surgery after sunitinib, and discuss the surgical management option in this clinical setting. RESULTS: Both our patients had a long, durable stable disease on sunitinib, but one developed a chronic mild bleeding that does not call for emergency surgical interventions and the other one developed chronic heart toxicity. They were proposed to undergo surgery despite the unresectable diseases and received an incomplete resection because of residual metastatic lesions. They restarted sunitinib after surgery. CONCLUSIONS: The poor prognosis after sunitinib treatment and the absence of alternative validated options open the debate on the assessment of surgical management of metastatic GISTs in this setting. The role of surgery should be investigated in clinical trials; however, the enrollment may be difficult. In clinical practice and after a multidisciplinary case patient discussion, surgery could represent a reasonable choice for advanced GISTs especially if the risk of surgery-related death is not too high.

The role of spiral multidetector dynamic CT in the study of Williams-Campbell syndrome.

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

Multidetector CT-urography in the study of urological complications in renal transplant.

PURPOSE: To evaluate the value of CT-urography in the diagnosis and follow-up of the urological complications of renal transplantation. MATERIALS AND METHODS: We performed 19 CT-urography examinations (3 of which were follow-up) on 15 patients by using a spiral multislice CT scanner and multiplanar reconstructions. The examinations were carried out directly after administration of 100 ml of iodinated contrast medium by slow iv infusion, with acquisitions starting 5 minutes after the end of the infusion. Surgery was regarded as the gold standard for the diagnosis of urological complications in the operated patients, whereas in patients who had undergone medical therapy or stent placement the gold standard was 1 month ultrasound and clinical follow-up with evaluation of diuresis and renal function. RESULTS: Between January 1999 and December 2001 a total of 210 kidney transplantations were performed at our hospital. There were 34 urological complications in 28 patients with a 16.1% prevalence, consistent with the major international studies. The complications detected were 14 urine leaks and 19 ureteral obstructions secondary to stones, oedema, blood clots and stricture. We observed one case of reflux in the allograft ureter. Fourteen out of 16 CT-urography examinations yielded important clues for the diagnosis of urological complications in kidney allografts, completely replacing standard urography. In particular, CT-urography correctly detected 5 urinary fistulas by demonstrating iodinated contrast material leaks along the ureteral tract, and 8 cases of obstructive uropathy due to different causes (1 submucosal tunnel edema, 1 blood clot, 1 stone and 5 cases of ureteral stricture). One case of urinary fistula and one of obstructive uropathy were not detected. DISCUSSION AND CONCLUSIONS: CT-urography proved to be an important diagnostic tool in the evaluation of urological complications of kidney allografts, showing a diagnostic accuracy over 90%; it is useful for confirming the type and site of urological lesions, and therefore to provide guidance for a targeted surgical approach. Compared with excretory urography, which is no longer used in this diagnostic field, CT-urography is more complete and precise as it provides information not only about the urinary tract, but also about the kidney parenchyma and pararenal fluid collections. The only disadvantage, which limits its use to selected cases, is the risk associated with the use of iodinated contrast agents in patients with impaired renal function.

Evaluation of the resistive index (RI) for the diagnosis of acute renal rejection in renal allografts from the same donor.

PURPOSE: To investigate the importance of the resistive index (RI) in the diagnosis of acute renal rejection, compared with the RI of the twin kidney from the same donor, transplanted in two different patients. MATERIAL AND METHODS: From January to December 2000, we studied retrospectively 25 pairs (50 patients) of renal allografts from the same donor considering the RI obtained with by eco color-Doppler ultrasound, daily diuresis and renal function (serum creatinine level) in the first six days following surgery. Improvement of diuresis and renal function after corticosteroid therapy was considered the gold standard for the diagnosis of acute rejection. RESULTS: Medical complications (acute renal rejection) in the first six days were occurred in three cases, two in the first transplanted kidney as first and one in the second; all three cases showed disappearance of the diastolic waveform component. Considering a RI variation >0.15 with respect to the initial value, the sensitivity, specificity and diagnostic accuracy in the Doppler diagnosis of acute rejection were 100%, 97.1% and 97.3% respectively, with a prevalence of 7.8%. There were no statistically significant correlations between the RI variation of the renal transplant and the twin kidney from the same donor. DISCUSSION AND CONCLUSIONS: Doppler ultrasound is an important diagnostic tool in the detection of medical complications in the immediate postoperative period and during renal transplant follow-up. RI analysis, when studied serially and in the right clinical settings, allows an early diagnosis of renal rejection with high sensibility and specificity.