Recommendations for the Optimal Radiation Dose in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma: A Report of the Dutch Cutaneous Lymphoma Group.

PURPOSE: To determine the optimal radiation dose for treatment of primary cutaneous anaplastic large cell lymphoma (C-ALCL) with solitary or localized, multifocal or recurrent skin lesions. METHODS AND MATERIALS: In this multicenter study, patients with C-ALCL who had been treated with radiation therapy (RT) between 1984 and 2016 were retrieved from the Dutch registry of cutaneous lymphomas. Distinction was made between patients first presenting with solitary or localized lesions (n=63), with multifocal skin lesions (n=6), and patients with a skin relapse (n=22). Radiation doses, treatment response, and follow-up were evaluated. Radiation doses were categorized as low-dose (≤20 Gy), intermediate-dose (21-39 Gy), and high-dose (≥40 Gy) RT. RESULTS: Of 63 patients presenting with solitary or localized skin lesions, 61 (97%) showed a complete response (CR). There were no differences in CR between low-dose (16 of 17), intermediate-dose (15 of 15), and high-dose RT (30 of 31). After a median follow-up of 46 months, 30 of 63 patients (48%) had a relapse, but in-field relapses were never observed. Six of 6 patients (100%) initially presenting with multifocal skin lesions showed a CR (3 of 3 low-dose, 2 of 2 intermediate-dose, 1 of 1 high-dose RT). After a median follow-up of 27 months, 3 of 6 patients had a relapse. Treatment of 33 skin relapses in 22 patients showed no differences in CR between low-dose (18 of 19), intermediate-dose (6 of 6), and high-dose RT (8 of 8). In the last 10 years there has been a decrease in radiation dose used in the treatment of C-ALCL. Treatment of multifocal and recurrent lesions with a dose of 8 Gy (2 × 4 Gy) resulted in CR of 17 of 18 lesions. CONCLUSIONS: Our results show that a radiation dose of 20 Gy (8 × 2.5 Gy) is effective in patients presenting with solitary or localized skin lesions. For patients with multifocal skin lesions and patients with a skin relapse, a dose of 8 Gy (2 × 4 Gy) may be sufficient.

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides.

IMPORTANCE: Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way. OBJECTIVE: To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF. DESIGN, SETTING, AND PARTICIPANTS: In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded. MAIN OUTCOMES AND MEASURES: Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques. RESULTS: In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival. CONCLUSIONS AND RELEVANCE: The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

Persistent reflux below the knee after stripping of the great saphenous vein.

OBJECTIVES: This prospective study investigated persistent reflux of the below knee great saphenous knee (GSV) 2 years after stripping the above knee GSV and the effect on reflux of postoperative incompetent perforating veins (IPVs). METHODS: Included were 59 consecutive ambulatory patients in a community hospital with untreated primary varicose veins and reflux of the saphenofemoral junction and at least the GSV in the upper leg. Patients underwent a preoperative clinical examination and color flow duplex ultrasound imaging. Postoperative evaluations were at 6 months and 2 years, and 67 limbs were re-evaluable. This analysis was adjusted for age, CEAP, follow-up period, and preoperative reflux using a multivariate logistic regression model. The difference in diameter of the GSV below the knee before and after surgery was measured and tested with the paired t test. RESULTS: Clearly visible varicose veins in the GSV below the knee were seen in 49 limbs (73%) before surgery and after surgery in 11 limbs (16%) at 6 months and in 15 limbs (22%) at 2 years. Reflux in the below knee main stem increased from 81% before surgery to 84% at 6 months and 91% at 2 years after surgery. Reflux in the posterior calf tributary of the GSV decreased from 67% before surgery to 64% at 6 months and 59% at 2 years after surgery. The mean diameter of the below knee GSV main stem decreased from 4.7 mm before surgery to 3.3 mm 6 months after surgery (P < .001), but increased to 3.6 mm 2 years after surgery (P < .05). The mean diameter of the posterior calf tributary of the GSV decreased from 3.5 mm before surgery to 2.7 mm at 6 months (P < .001), but increased to 2.9 mm at 2 years (P < .05). Univariate and multivariate logistic regression shows no association between reflux in one of the GSV below knee branches and postoperative IPVs. CONCLUSION: Many patients (91%) that undergo a short stripping procedure will have a persistent reflux of the remnant below knee GSV tributaries postoperatively. This incompetence of the distal GSV is independent from the proximal GSV as well from insufficient perforating veins. There seems to be a tendency to worsening of the clinical signs and symptoms between 6 months and 2 years after surgery, and this goes along with an increase of reflux and diameters of the GSV below knee remnants.

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.

Primary cutaneous follicle center lymphoma (PCFCL) is a neoplasm with differentiation of centrocytes and centroblasts presenting in the skin. At the time of initial manifestation, extracutaneous involvement is absent. PCFCL is considered as an indolent variant of primary cutaneous B-cell lymphomas since dissemination to extracutaneous sites is rare and the prognosis is favorable. Here we describe a 30-year-old man who was diagnosed with a cutaneous FCL and did not show extracutaneous affection at the time of occurrence. Six months later, however, he developed a diffuse large B-cell non-Hodgkin lymphoma localized in several lymph nodes of the neck that most likely reflects the occurrence of a second primary tumor in the same patient.

Outcomes and side effects of duplex-guided sclerotherapy in the treatment of great saphenous veins with 1% versus 3% polidocanol foam: results of a randomized controlled trial with 1-year follow-up.

BACKGROUND: Eighty subjects were treated with either 1 or 3% polidocanol foam to compare the efficacy and adverse sequelae of each concentration of polidocanol foam. OBJECTIVE: The objective was to compare the effects of two different concentrations of polidocanol foam. MATERIAL AND METHODS: During a 6-month period, we treated 80 consecutive patients with primary incompetent great saphenous veins in combination with saphenofemoral junction incompetence. These patients were treated with foam of either 1 or 3% polidocanol. Duplex analyses were made before treatment and in follow-up visits to determine the presence or absence of reflux. RESULTS: After 1 year, there was a clinically relevant difference in percentage of patients with occlusion of the treated great saphenous vein between both groups: 69.5% in the 1% foam group versus 80.1% in the 3% foam group; however, this difference was not statistically significant (p=.249). After 1 year of follow-up, patients in the 3% polidocanol group noticed a larger cosmetic improvement than patients in the 1% group. CONCLUSIONS: In the treatment of primary incompetent greater saphenous veins, 3% polidocanol foam seems to be more effective than 1% polidocanol foam. The side effects were approximately similar in both groups.

Posterolateral thigh perforator varicosities in 12 patients: a normal deep venous system and successful treatment with ultrasound-guided sclerotherapy.

OBJECTIVES: To determine whether in patients with posterolateral thigh perforator (PLTP) varicosities ultrasound-guided sclerotherapy (USGS) is successful and to determine any deep venous anomaly. PATIENTS: Twelve consecutive patients with PLTP varicosities were investigated and treated. DESIGN: In this prospective intervention study USGS of the PLTP with polidocanol microfoam 1-2% was performed with a maximal number of three treatments. Clinical pictures and Duplex imaging were performed before and after treatment. Ascending phlebography was performed in 9 patients. RESULTS: Ten patients showed a marked success, defined as no PLTP varicosities visible and no reflux detectable. In 2 patients there was moderate success. The mean number of treatments was 1.58, the mean diameter of the PLTP vein was reduced from 4.06 (range, 2.2-6.1) to 1.97 (range, 0-3.3) mm (p=0.003, Wilcoxon signed rank test). No serious side effects were noted. No deep venous anomaly was found in the phlebographic study. CONCLUSION: In patients with PLTP varicosities, USGS is very successful, with very few side effects. No deep venous anomalies were found in our study, which conforms to the literature.

State-of-the-art treatment of chronic leg ulcers: A randomized controlled trial comparing vacuum-assisted closure (V.A.C.) with modern wound dressings.

BACKGROUND: Current treatment modalities for chronic leg ulcers are time consuming, expensive, and only moderately successful. Recent data suggest that creating a subatmospheric pressure by vacuum-assisted closure (V.A.C., KCI Concepts, San Antonio, Texas) therapy supports the wound healing process. METHODS: The efficacy of vacuum-assisted closure in the treatment of chronic leg ulcers was prospectively studied in a randomized controlled trial in which 60 hospitalized patients with chronic leg ulcers were randomly assigned to either treatment by V.A.C. or therapy with conventional wound care techniques. The primary outcome measure was the time to complete healing (days). Statistical analysis was performed on the intention-to-treat basis. RESULTS: The median time to complete healing was 29 days (95% confidence interval [CI], 25.5 to 32.5) in the V.A.C. group compared with 45 days (95% CI, 36.2 to 53.8) in the control group (P = .0001). Further, wound bed preparation during V.A.C. therapy was also significantly shorter at 7 days (95% CI 5.7 to 8.3) than during conventional wound care at 17 days (95% CI, 10 to 24, P = .005). The costs of conventional wound care were higher than those of V.A.C. Both groups showed a significant increase in quality of life at the end of therapy and a significant decrease in pain scores at the end of follow-up. CONCLUSIONS: V.A.C. therapy should be considered as the treatment of choice for chronic leg ulcers owing to its significant advantages in the time to complete healing and wound bed preparation time compared with conventional wound care. Particularly during the preparation stage, V.A.C. therapy appears to be superior to conventional wound care techniques.