Leiomyosarcoma of the renal vein: analysis of outcome and prognostic factors in the world case series of 67 patients.

BACKGROUND: Leiomyosarcoma is a rare malignant mesenchymal tumour. Some cases of leiomyosarcoma of the renal vein (LRV) have been reported in the literature, but no analysis of data and search for prognostic factors have been done so far. The aim of this review was to describe the LRV, to analyse overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS) in LRV world case series and to identify significant predictors of OS, LRFS and DMFS. METHODS: Cases from the literature based on PubMed search and a case from our institution were included. RESULTS: Sixty-seven patients with a mean age of 56.6 years were identified; 76.1% were women. Mean tumour size was 8.9 cm; in 68.7% located on the left side. Tumour thrombus extended into the inferior vena cava lumen in 13.4%. All patients but one underwent surgery (98.5%). After a median follow up of 24 months, the OS was 79.5%. LRFS was 83.5% after a median follow up of 21.5 months and DMFS was 76.1% after a median follow up of 22 months. Factors predictive of OS in univariate analysis were surgical margins, while factors predictive of LRFS were inferior vena cava luminal extension and grade. No factors predictive of DMFS were identified. In multivariate analysis none of the factors were predictive of OS, LRFS and DMFS. CONCLUSIONS: Based on the literature review and presented case some conclusions can be made. LRV is usually located in the hilum of the kidney. It should be considered in differential diagnosis of renal and retroperitoneal masses, particularly in women over the age 40, on the left side and in the absence of haematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma multidisciplinary team. LRV should be surgically removed, with negative margins.

Radiotherapy for inverted papilloma: a case report and review of the literature.

BACKGROUND: Sinonasal inverted papilloma (IP) is a rare, usually benign tumor arising from the respiratory mucosa of the sinonasal tract. Surgical resection is the treatment of choice. In histologically overt benign IPs (i.e. without associated malignancy) irradiation was employed only anecdotally. The patient with gross residual of benign IP after up-front surgery that was subsequently treated with irradiation is presented and the literature reports on the use of radiotherapy (RT) in this tumor type are reviewed. CASE REPORT: After the surgical treatment the residuum in the region of the sphenoid and adjacent cavernous sinus was irradiated to 54 Gy in 1.8 Gy daily fractions. No recurrence or deterioration of olfaction, hearing or vision was observed 2.6 years post-RT. REVIEW OF THE LITERATURE: In the literature, six reports were identified with 16 patients describing necessary details on RT and outcome. Twelve of 14 cases (our case included) with gross or subtotal tumor resection and postoperative RT were locally controlled. The lowest and the median irradiation doses were 47.15 Gy and 56.5 Gy, respectively, and the follow-up period ranged between 0.5-20.5 years (median 7.8 years). CONCLUSIONS: RT is safe and valuable treatment option in histologically overt benign IPs. It is indicated when the risk of tumor recurrence after surgery is increased and in inoperable tumors.

Impact of delays in radiotherapy of head and neck cancer on outcome.

BACKGROUND: In head and neck cancer (HNC), the relationship between a delay in starting radiotherapy (RT) and the outcome is unclear. The aim of the present study was to determine the impact of the amount of time before treatment intervention (TTI) and the growth kinetics of individual tumors on treatment outcomes and survival. METHODS: Two hundred sixty-two HNC patients with 273 primary tumors, treated with definitive (chemo) RT, were retrospectively analyzed. The TTI was defined as the time interval between the date of histopathologic diagnosis and the first day of the RT course. Volumetric data on 57 tumors were obtained from diagnostic and RT planning computer tomography (CT) scans in order to calculate the tumor growth kinetic parameters. RESULTS: No significant association between locoregional control or cause-specific hazards and TTI was found. The log hazard for locoregional recurrence linearly increased during the first 40 days of waiting for RT, although this was not significant. The median tumor volume relative increase rate and tumor volume doubling time was 3.2%/day and 19 days, respectively, and neither had any impact on locoregional control or cause-specific hazards. CONCLUSION: The association between a delay in starting RT and the outcome is complex and does not harm all patients waiting for RT. Further research into imaging-derived kinetic data on individual tumors is warranted in order to identify patients at an increased risk of adverse outcomes due to a delay in starting RT.

Skin Reaction to Cetuximab as a Criterion for Treatment Selection in Head and Neck Cancer.

BACKGROUND/AIM: It can be hypothesized that in patients with locally advanced head and neck cancer and prominent cetuximab (CMb)-induced skin rash, immunoradiotherapy would result in a survival advantage over chemoradiotherapy with cisplatin (CP). PATIENTS AND METHODS: After a loading dose of CMb, one weekly cycle of CMb and CP concurrently with RT, patients who developed a grade ≥2 rash proceeded with immunoradiotherapy, and those with a grade 0-1 rash had chemoradiotherapy. RESULTS: A grade 3-4 allergic reaction to CMb developed in 11/39 (28.2%) patients and further recruitment was stopped. These patients proceeded treatment with CP. In early assessment of skin rash 10/28 patients qualified for chemoradiotherapy and 18/28 patients for immunoradiotherapy. There was no difference in survival between the two groups. CONCLUSION: Rate of serious CMb-induced hypersensitivity reactions was unacceptably high. Even though immunoradiotherapy was administered only to the prognostically most favorable group of patients, it resulted in no advantage over chemoradiotherapy.

A prospective, randomized comparison between combined (deep and superficial) and superficial cervical plexus block with levobupivacaine for minimally invasive parathyroidectomy.

BACKGROUND: Minimally invasive parathyroidectomy (MIP) can be performed under cervical plexus block (CPB). Superficial CPB has been reported to be easier to perform with similar efficacy and less anesthesia-related complications than combined deep and superficial CPB. In this study, we compared the efficacy of superficial and combined (deep and superficial) CPB in patients undergoing MIP. METHODS: Forty-two patients with primary hyperparathyroidism due to a solitary adenoma were randomized to receive either a superficial (group superficial, n = 20) or a combined deep and superficial CPB (group combined, n = 22) using 0.35 mL/kg of 0.5% levobupivacaine. The primary end-point was the amount of supplemental fentanyl required to complete surgery. RESULTS: There were no differences in onset of block, pain scores during surgery, or time to first analgesic request between groups. Fentanyl consumption was similar in both groups, i.e., 50 (0-200) microg in group superficial and 50 (0-100) microg in group combined (P = 0.60). Six patients [1 in group superficial (5%) and 5 in group combined (22.7%)] were converted to general anesthesia for surgically required bilateral neck dissection (P = 0.18). General anesthesia for block failure was reported in three superficial (15%) and two combined group patients (9%) (P = 0.99). In group combined, only one patient (4.5%) showed diaphragmatic paresis after the block (P = 0.99). CONCLUSION: Superficial CPB is an alternative to combined block for MIP.

PKD1 and PKD2 mutations in Slovenian families with autosomal dominant polycystic kidney disease.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogeneous disorder caused by mutations in at least two different loci. Prior to performing mutation screening, if DNA samples of sufficient number of family members are available, it is worthwhile to assign the gene involved in disease progression by the genetic linkage analysis. METHODS: We collected samples from 36 Slovene ADPKD families and performed linkage analysis in 16 of them. Linkage was assessed by the use of microsatellite polymorphic markers, four in the case of PKD1 (KG8, AC2.5, CW3 and CW2) and five for PKD2 (D4S1534, D4S2929, D4S1542, D4S1563 and D4S423). Partial PKD1 mutation screening was undertaken by analysing exons 23 and 31-46 and PKD2 . RESULTS: Lod scores indicated linkage to PKD1 in six families and to PKD2 in two families. One family was linked to none and in seven families linkage to both genes was possible. Partial PKD1 mutation screening was performed in 33 patients (including 20 patients from the families where linkage analysis could not be performed). We analysed PKD2 in 2 patients where lod scores indicated linkage to PKD2 and in 7 families where linkage to both genes was possible. We detected six mutations and eight polymorphisms in PKD1 and one mutation and three polymorphisms in PKD2. CONCLUSION: In our study group of ADPKD patients we detected seven mutations: three frameshift, one missense, two nonsense and one putative splicing mutation. Three have been described previously and 4 are novel. Three newly described framesfift mutations in PKD1 seem to be associated with more severe clinical course of ADPKD. Previously described nonsense mutation in PKD2 seems to be associated with cysts in liver and milder clinical course.