Precision Oncology in Soft Tissue Sarcomas and Gastrointestinal Stromal Tumors.

Soft tissue sarcomas (STSs), including gastrointestinal stromal tumors (GISTs), are mesenchymal neoplasms with heterogeneous clinical behavior and represent broad categories comprising multiple distinct biologic entities. Multidisciplinary management of these rare tumors is critical. To date, multiple studies have outlined the importance of biological characterization of mesenchymal tumors and have identified key molecular alterations which drive tumor biology. GIST has represented a flagship for targeted therapy in solid tumors with the advent of imatinib which has revolutionized the way we treat this malignancy. Herein, the authors discuss the importance of biological and molecular diagnostics in managing STS and GIST patients.

Sodium Tetradecyl Sulphate Sclerotherapy for Lateral Malleolar Bursitis of the Ankle.

Background: The aim of this study was to evaluate clinical outcomes of sodium tetradecyl sulphate (STS) sclerotherapy for conservative treatment of lateral malleolar bursitis of the ankle. Methods: We reviewed data from 20 consecutive patients (20 ankles) who underwent STS sclerotherapy between August 2018 and June 2019. After aspiration of fluid from the lateral malleolar bursal sac, 2 mL (20 mg) STS was injected into the sac. Clinical outcomes and side effects and complications were evaluated at 2 weeks, 3 months, 1 year, and 2 years after sclerotherapy. Responses to treatment were assessed according to degree of fluctuation, shrinkage of the bursal sac, and soft-tissue swelling. The 36-item short form survey (SF-36) was completed for each patient before and after therapy. Results: Complete response was observed in 17 patients (85%), and partial response was observed in 3 patients (15%) after STS sclerotherapy. SF-36 physical component scores improved from 62.2 (interquartile range, 5.2) before therapy to 70.0 (interquartile range, 7.9) at last follow-up (p < 0.05). One patient (5%) experienced transient hyperpigmentation at the injection site. No major complications occurred. Conclusions: STS sclerotherapy was an effective and safe treatment for patients with lateral malleolar bursitis of the ankle.

Aneurysmal bone cysts in the spine, causing neurological compromise: safety and clinical efficacy of sclerotherapy utilizing sodium Tetradecyl sulfate foam.

OBJECTIVE: To assess the clinical efficacy, technical feasibility, and safety profile of percutaneous sclerotherapy utilizing sodium tetradecyl-sulfate foam (STS) as a first-line treatment strategy for aggressive spinal aneurysmal bone cysts (sABCs) presenting with neurological compromise. MATERIALS AND METHODS: Between July 2013 and September 2019, eight consecutive patients (5 males; 3 females; mean age 22±17, range 7-52) underwent fluoroscopic/CT-guided intraosseous sclerotherapy for sABCs. Pain and/or neurological compromise was the primary indications. Procedural data, complications, imaging, and clinical results were analyzed. RESULTS: Technical success was achieved in all cases. Mean procedure time was 25±15 min (range 6-167); 1 to 5 repeat treatment cycles (mean 3.7±1.2) utilizing a mean 2.6mls±1.3 (range 1-6) of agitated 3% STS, with a DLP mean dose of 158±91 mGy*cm (range 62-331) per procedure. One reported a minor complication (pain), but no significant complications. Two patients had persistent neurological deficit due to cord compression despite successful sclerotherapy, requiring surgical resection (and were thereby excluded from the final outcome analysis). The remaining six patients demonstrated a significant reduction in tumor volume (p = 0.028), pain (p = 0.027), and SINS (spinal instability neoplastic score) (p = 0.027) at up to 5 years of follow-up (mean 20 ± 16.7, range 7-51 months). CONCLUSION: Percutaneous sclerotherapy with STS is a minimally invasive, technically feasible, safe, and effective first-line treatment for primary sABCs causing pain and neurological compromise, alleviating the need for extensive surgery. It is most effective with three or more treatment cycles, in patients with higher SINS, pain scores, or tumor volumes at the initial presentation.

Use of percutaneous foam sclerotherapy with 1.5% sodium tetradecyl sulfate for treatment of a pelvic limb venous malformation in a dog.

CASE DESCRIPTION: A 6-year-old 17-kg (37.4-lb) spayed female mixed-breed dog was evaluated because of swelling and intermittent lameness of the right pelvic limb and perianal and vulvar bleeding caused by a suspected arteriovenous malformation. CLINICAL FINDINGS: The right pelvic limb had a diffuse, raised, cobblestone-like appearance with lameness, edema, and multifocal ulcerations. The abdominal skin had multifocal circular erythematous lesions, the perianal region was erythematous, and the vestibule had superficial distended vessels. Ultrasonography and CT did not reveal the presence of an arteriovenous malformation; however, digital subtraction venography confirmed the presence of a venous malformation (VM) throughout the limb. TREATMENT AND OUTCOME: A mixture of foam sclerosant (1.5% sodium tetradecyl sulfate) and contrast medium was agitated with air and injected percutaneously into the VM. The dog received an injection of corticosteroid solution, and a soft-padded bandage was applied to the limb for 3 days. Six weeks later, the dog would intermittently hop when running, and the limb was mildly edematous with ecchymotic lesions; the swelling and lameness had improved considerably. Perianal and vulvar bleeding and dilation of the vestibular vessels had resolved. At 21 months after the procedure, examination revealed no right pelvic limb lameness related to the VM; only small cyst-like lesions and edema around the tarsus remained. CLINICAL RELEVANCE: The favorable clinical outcome for this dog for a 21-month period after treatment of a pelvic limb VM with foam sclerotherapy has suggested that foam sclerotherapy may be used to successfully treat limb VMs in some dogs.

Safety and efficacy of foam sclerotherapy for treatment of low-flow vascular malformations in children.

OBJECTIVE: Congenital vascular malformations are a heterogeneous group of lesions with the potential to cause significant lifelong morbidity in children. Diagnosis and treatment of these lesions may be complex and require a multidisciplinary approach. Sclerotherapy is widely used for the treatment of low-flow vascular malformations (LFVMs) as an alternative to surgical resection in adults; however, limited data of its use in a pediatric setting are available. The purpose of this study was to evaluate the efficacy and safety of sclerotherapy for pediatric LFVMs. METHODS: In this retrospective study, we reviewed our multidisciplinary vascular malformations team database for all patients younger than 18 years treated for congenital vascular malformations from 2008 to 2017. Of these, patients with LFVM treated with foam sclerotherapy were included. Dynamic contrast-enhanced magnetic resonance imaging was used to select patients for sclerotherapy by the multidisciplinary team. Foam sclerotherapy was performed with either polidocanol or sodium tetradecyl sulfate. Patients' characteristics, including demographics, presenting symptoms, and anatomic location of malformation, were assessed. Outcomes included treatment response, number of procedures, and postprocedural complications. RESULTS: The 61 patients with 61 LFVMs included 27 boys (44.3%) and 34 girls (55.7%), with mean age of 10.3 years (standard deviation, ± 5.3 years). The cohort included 32 venous (52.5%), 16 lymphatic (26.2%), and 8 mixed venous and lymphatic (13.1%) malformations along with 5 (8.2%) associated with Klippel-Trénaunay syndrome. Primary indications for intervention included pain and swelling (n = 12 [19.6%]), pain alone (n = 23 [37.7%]), swelling alone (n = 15 [24.6%]), functional impairment (n = 8 [13.1%]), and bleeding (n = 3 [4.9%]). Anatomic distributions varied, with 13 head and neck (21.3%), 5 truncal (8.2%), 10 upper extremity (16.4%), 27 lower extremity (44.3%), and 6 diffuse (9.8%). Among the head and neck lesions, 8 (13.1%) extended to the face; and of the extremity lesions, 5 (8.2%) extended to the hand and 17 (27.9%) to the foot. Overall, sclerotherapy resulted in significant improvement or complete resolution of symptoms in 53 patients (86.9%). Complications were observed in seven patients (11.4%); six cases (9.8%) of superficial skin ulceration resolved without intervention, and one infection (1.6%) required antibiotics. No patients experienced adverse hemodynamic consequences or venous thromboembolism. CONCLUSIONS: This series of pediatric LFVMs, the largest of its kind to date, demonstrates that sclerotherapy with foam-based agents effectively reduces symptoms with an acceptable rate of complications. Further study is needed to determine the optimal sclerosing agents for individual subsets of LFVMs in the pediatric population.

TC-325 hemostatic powder versus current standard of care in managing malignant GI bleeding: a pilot randomized clinical trial.

BACKGROUND AND AIMS: TC-325 (Hemospray; Cook Medical, Winston-Salem, NC, USA), an endoscopic hemostatic powder, exhibits possible benefits in patients with malignant GI bleeding. Our aim is to assess feasibility and determine estimates of efficacy of TC-325 compared with standard of care (SOC) in terms of initial hemostasis and recurrent bleeding rates in comparable groups of patients with malignant GI bleeding. METHODS: Adult patients presenting with acute malignant upper or lower GI bleeding were randomized to TC-325 or SOC. Measured outcomes included feasibility of recruitment and randomization in the urgent care setting, immediate hemostasis, recurrent bleeding, need for additional treatment modalities, and mortality. RESULTS: A preplanned 20 patients (upper GI source in 85%) were randomized 1:1 to TC-325 or SOC (25% women, age 67.2 ± 15.9 years, oozing in 95%) over 20 months. Immediate hemostasis was achieved in 90% of patients treated initially with TC-325 versus 40% in the SOC group (P = .057). Overall, 83.3% crossed over to TC-325, with hemostasis then achieved at index endoscopy in 80%. Overall, hemostasis at index endoscopy (before or after crossover) was obtained in 87.7% of patients treated with TC-325. Recurrent bleeding over the next 180 days was 20% in the TC-325 group compared with 60% in the SOC group (P = .170). CONCLUSIONS: This pilot trial demonstrates the feasibility of TC-325 in malignant GI bleeding and provides results to help inform a larger randomized trial. Although not powered for such, results suggest that use of TC-325 is a very promising modality in malignant GI bleeding in achieving immediate hemostasis and may even result in decreased subsequent recurrent bleeding. (Clinical trial registration number: NCT02135627.).

Simultaneous intra-operative sclerotherapy and surgical resection of cervicofacial venous malformations.

OBJECTIVES: To review simultaneous intra-operative sclerotherapy (IOS) with immediate surgical resection for the treatment of cervicofacial venous malformations (VMs) at a single institution. While pre-operative sclerotherapy (POS) has been reported in the literature, simultaneous intra-operative sclerotherapy and surgery in the operating room has not. METHODS: The database from the Hemangioma and Vascular Birthmarks Clinic was reviewed. All patients in both groups had biopsy-proven VMs. RESULTS: IOS was used in 11 surgical patients with average age 17 years. Sclerotherapy was performed with sodium tetradecyl sulfate 3%, absolute alcohol or bleomycin. Immediately after IOS, and under the same anesthetic, all patients had either complete resection or debulking of the VMs. Eight patients had complete resolution of their VM and 3 had improvement. Average duration of the combined procedures done under a single anesthetic was 121 min. The POS approach was used for 6 surgical patients with average age 7 years. Sclerotherapy agents used were absolute alcohol or sodium tetradecyl sulfate 3%. All patients underwent complete resection of the VM 24-72 h after sclerotherapy under a separate surgical session. Five patients experienced complete resolution of their VM and one has had further sclerotherapy for recurrent disease. Interventional Radiology suite sclerotherapy times were on average 70 min. Surgical times were on average 142 min. Total combined anesthesia times for the two procedures added together were 212 min. Treatment time was significantly shorter in the IOS group (p = 0.0015). CONCLUSIONS: Simultaneous IOS at the time of surgical resection has been successful in our hands. IOS has the advantage of a single procedure and decreased cost to the patient. In the era of reducing pediatric exposure to anesthesia, this approach is especially attractive in the pediatric population. As well, at approximately $100/minute cost to the patient to be in either the Interventional Radiology Suite or in the operating room, the reduced length of the procedures seen in the IOS approach results in lower overall cost to the patient.

Conjunctival Retention Cysts: Outcomes of Aspiration and Sclerotherapy With Sodium Tetradecyl Sulfate.

PURPOSE: To assess the outcome of aspiration and sclerotherapy with sodium tetradecyl sulfate in the management of conjunctival inclusion cysts. METHODS: Retrospective interventional case series of 6 patients with clinical diagnosis of conjunctival inclusion cysts treated with cyst aspiration and foam sclerotherapy with 3% sodium tetradecyl sulfate. The volume of the sclerosant was 20% of the aspirated cyst volume. RESULTS: Four patients had an inclusion cyst in anophthalmic sockets and 2 patients in sighted eyes. Average time lag between primary surgery and cyst formation was 14.6 months (range 2-30 months). Average amount of fluid aspirated from cyst was 3.07 ml (range 1-9 ml). Average volume of sclerosant injected was (20% of the aspirated volume) 0.55 ml (range 0.2-1.1 ml). All 6 patients showed complete resolution of cyst at a mean follow-up period of 15.6 months (range 9-24 months). All but one showed complete resolution of cyst with single injection sclerosant. Only 1 patient required a second sclerosant injection. There was no ocular surface or implant-related complications in this cohort. CONCLUSIONS: Cyst aspiration and sodium tetradecyl sulfate foam sclerotherapy is a minimally invasive procedure for the management of conjunctival inclusion cysts in anophthalmic sockets and sighted eyes. The injection of sodium tetradecyl sulfate in a dose of 20% of the aspirate is effective in the management of conjunctival inclusion cysts over a follow-up period of 13 months. The procedure is safe, with insignificant inflammation and without ocular surface or implant complications.

An institution-wide algorithm for direct-stick embolization of peripheral venous malformations.

OBJECTIVE: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality. METHODS: During 36 months, patients with symptomatic congenital peripheral VMs underwent contrast-enhanced magnetic resonance imaging. Hematologic monitoring for localized intravascular coagulopathy was performed in all. Perioperative anticoagulation was administered accordingly. When applicable, venous duplex ultrasound was performed to assess for presence and patency of a deep venous system and superficial venous reflux. If superficial venous reflux was identified, radiofrequency ablation was performed per standard protocol before or at the time of initial embolization. Direct-stick embolizations (DSEs) were performed by a single operator using two concentrations (1% and 3%) of sodium tetradecyl sulfate (STS; Sotradecol; AngioDynamics, Latham, NY) without foam preparation. Patients were followed up clinically for resolution of symptoms, coagulopathic monitoring, and development of complications. All data were prospectively maintained and retrospectively reviewed. RESULTS: There were 71 DSEs performed in 40 patients (1.8 procedures per patient [range, 1-8]; 12 male patients; mean age, 22 years [range, 2-53 years]). Mean follow-up was 17.1 months (range, 0.8-31.6 months). Presenting symptoms included pain (n = 40 [100%]), swelling (n = 36 [90%]), and cosmetic disfigurement (n = 32 [80%]). Anatomic distribution was upper extremity (n = 16 [23%]), lower extremity (n = 37 [52%]), head and neck (n = 7 [10%]), trunk (n = 10 [14%]), and visceral (n = 1 [1%]). There were 33 sporadic cases, 4 (10%) Klippel-Trénaunay syndrome cases, 2 (5%) blue rubber bleb nevus syndrome cases, and 1 (2.5%) CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities) syndrome case. Four patients presented with localized intravascular coagulopathy, two of whom required perioperative enoxaparin. Twenty-six patients (65%) required a single DSE session with complete symptom relief. Fourteen patients (35%) required repeated DSE. Two patients (5%) required adjunctive surgical excision. There was one postoperative death (1.4%) secondary to massive pulmonary embolism. Complications were otherwise limited to skin necrosis (n = 2 [3%]). Mean volume of sclerosant per session was 7 mL of 1% STS (range, 3-14 mL), and 15 mL of 3% STS (range, 3-42.5 mL). CONCLUSIONS: In the absence of allergic reactions, most congenital peripheral VMs can be safely embolized with liquid STS, thereby avoiding the well-documented toxicity of ethanol. Venous thromboembolism remains a major source of morbidity and mortality in this population of patients despite close hematologic scrutiny. Prospective randomized trials are needed for embolotherapeutic standardization.

Sodium Tetradecyl Sulfate: A Review of Clinical Uses.

BACKGROUND: Sodium tetradecyl sulfate (STS) is Food and Drug Administration approved for treatment of varicose veins, but numerous other off-label applications have been reported. OBJECTIVE: To describe the clinical uses of STS, as well as efficacy and adverse effects. METHODS: Review of studies searchable on PubMed from 1938 to 2016 describing clinical uses of STS to determine efficacy and adverse effects associated with various applications. RESULTS: Sodium tetradecyl sulfate has shown efficacy in the treatment of varicose veins, telangiectasias, hemangioma, pyogenic granuloma, cherry angioma, Kaposi sarcoma, lymphangioma circumscriptum, digital mucous cyst, ganglion cyst, glomangioma, angiokeratoma of Fordyce, pseudocyst of the auricle, and verruca. Commonly reported side effects include pain, erythema, swelling, hyperpigmentation, telangiectatic matting, and ulceration. Serious side effects such as anaphylaxis, pulmonary embolism, stroke, and myocardial infarction have also been reported. Most sources were case reports and small prospective studies, as such the strength of data supporting many uses is limited by small sample sizes and lack of controls. CONCLUSION: Although not always the most effective method of treatment in off-label usage, use of STS has been frequently selected for a variety of applications for reasons of simplicity, low cost, lack of availability of technologically advanced equipment, and intricacies related to anatomic location.

Sclerotherapy for Orbital Lymphangioma - Case Series and Literature Review.

Orbital lymphangioma is a lymphatic system lesion that commonly presents in childhood. Management of these lesions is complex. Sclerotherapy is a therapy used to treat and shrink lesions prior to or as an alternative to surgery. We present three cases of orbital lymphangioma that were treated with sclerotherapy. Case 1: A 6-month-old boy was presented in 2010 with right ptosis and proptosis. Magnetic resonance imaging (MRI) identified a lesion involving the right orbit and face. Case 2: A 3-year-old girl was presented in 2011 with intermittent right periorbital swelling and medial canthal bleeding. MRI identified a soft-tissue lesion in the right orbit, extending into the face. Case 3: A 3-year-old girl was presented in 2012 with vomiting, and painful right proptosis. MRI identified an intra-conal lesion in the right orbit with fluid filled levels. All three patients were treated with sclerotherapy (sodium tetradecylsulfate). Sclerotherapy is a promising treatment for orbital lymphangioma. Its use may prevent the need for, or minimise the amount of surgical management. Several sclerosants are now commonly used to treat these lesions.

Complications After Treatment of Head and Neck Venous Malformations With Sodium Tetradecyl Sulfate Foam.

PURPOSE: The aim of this study was to evaluate complications in patients with head and neck venous malformations (VMs) treated with foam sclerotherapy using sodium tetradecyl sulfate (STS). METHODS: The authors retrospectively evaluated the complications, pain. and degree of satisfaction in 69 consecutive patients affected by cervicofacial VM managed with STS using the Tessari method in a single institution. RESULTS: The average number of procedures for each patient was 2.1. The most frequent complication was blistering. We observed 1 patient of temporary weakness of a facial nerve branch, 1 paradoxical embolism, and 1 orbital compartment syndrome.The average pain score was 0 (no pain at all) (51.5%). There was no statistically significant correlation between patient satisfaction and the presence of complications or the degree of pain. CONCLUSIONS: Sclerotherapy with STS is an effective treatment that yields to very high patient satisfaction. This procedure has an overall low complication rate and is usually effective within a few sessions. However, severe complications may occur; these must be pointed out in the informed consent and the surgeon must be aware of and ready to quickly treat them to prevent long-term sequelae.

Media Damage Following Detergent Sclerotherapy Appears to be Secondary to the Induction of Inflammation and Apoptosis: An Immunohistochemical Study Elucidating Previous Histological Observations.

OBJECTIVE/BACKGROUND: Traditionally, sclerotherapy has been thought to work by the cytotoxic effect of the sclerosant upon the endothelium alone. However, studies have shown that sclerotherapy is more successful in smaller veins than in larger veins. This could be explained by the penetration of the sclerosant, or its effect, into the media. This study aimed to investigate intimal and medial damage profiles after sclerosant treatment. METHODS: Fresh human varicose veins were treated ex vivo with either 1% or 3% sodium tetradecyl sulphate (STS) for 1 or 10 minutes. The effect of the sclerosant on the vein wall was investigated by immunofluorescent labelling of transverse vein sections using markers for endothelium (CD31), smooth muscle (α-actin), apoptosis (p53) and inflammation (intercellular adhesion molecule-1 [ICAM-1]). Polidocanol (POL; 3%) treatment at 10 minutes was similarly investigated. RESULTS: Endothelial cell death was concentration- and time-dependent for STS but incomplete for both sclerosants. Time, but not concentration, significantly affected cell death (p > .001). A 40% and 30% maximum reduction was observed for STS and POL, respectively. Destruction of 20-30% of smooth muscle cells was found up to 250 µm from the lumen after 3% STS treatment for 10 minutes. POL treatment for 10 minutes showed inferior destruction of medial cells. Following STS treatment and 24-hour tissue culture, p53 and ICAM-1 were upregulated to a depth of around 300 µm. This effect was not observed with POL. CONCLUSION: Inflammatory and apoptotic markers show the same distribution as medial cell death, implying that sclerotherapy with STS works by inducing apoptosis in the vein wall rather than having an effect restricted to the endothelium. Incomplete loss of endothelial cells and penetration of the sclerosant effect up to 250 µm into the media suggest that medial damage is crucial to the success of sclerotherapy and may explain why it is less effective in larger veins.

Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience.

BACKGROUND: Percutaneous sclerotherapy is an alternative to surgery for the treatment of orbital lymphatic malformations (LMs). We present a large series of patients undergoing sclerotherapy for macrocystic LMs with detailed visual acuity (VA) outcome data. METHODS: Data were collected prospectively in all patients with macrocystic orbital LMs undergoing sclerotherapy. Sclerotherapy was performed under general anaesthesia, instilling sodium tetradecyl sulfate under imaging control. Procedures were repeated at 2-week to 6-week intervals, depending on clinical response. Patients underwent ophthalmological assessment, ultrasound and/or MRI before and after treatment. PRIMARY OUTCOME MEASURE: change in maximal radiological diameter of the LM. SECONDARY OUTCOME MEASURE: change in VA after treatment. RESULTS: 29 patients underwent 71 procedures (1-8 procedures per patient) over 6.7 years. Mean age=7.31 years. 11 patients (37.9%) had undergone previous treatment, including excision biopsy, drainage and decompression. All patients presented with proptosis and/or pseudoptosis. 23 patients (79.3%) had decreased VA at presentation. Average follow-up was 21.8 months (range 3-75 months). All patients achieved a reduction in maximal lesion diameter of ≥50%, with complete radiological resolution in 51.7% (n=15). VA improved in 18/23 patients (78.2%). Average logMAR before treatment=0.43 (SD ±0.47); average after treatment=0.25 (SD ±0.32); p<0.01. There was one complication (1.4%): one patient required a lateral canthotomy for an intralesional haematoma 2 h after sclerotherapy. CONCLUSIONS: Sclerotherapy is a safe and highly effective treatment for orbital LMs with excellent VA outcomes. It should be considered as the first-line treatment for this condition.

Chemical Substances Used in the Treatment of Ganglions Located in the Hand and Wrist.

The aim of this work was to compare different chemical substances used in the treatment of ganglions located in the hand and wrist region. Their basic properties and mechanisms of action have been described. Moreover, the risks associated with the use of particular substances have been highlighted and potential complications connected with their administration have been discussed. On the basis of the available literature, the results of ganglion aspiration treatment followed by an injection of a chemical substance into the cyst cavity have been assessed. In the authors' opinion, due to the existing risk of complications associated with this treatment, as well as the relatively high rate of ganglion recurrence, this procedure should only be performed by qualified medical personnel. The authors recommend observation in cases of asymptomatic ganglions of the hand and wrist, and operative treatment in cases in which pain, restriction of limb mobility and weakening of handgrip strength are observed.