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Zhonghua Zhong Liu Za Zhi [Chinese... Sep 2015To explore the clinical diagnostic features and treatment of desmoplastic small round cell tumor (DSRCT), and to improve the understanding and management of this tumor. (Review)
Review
OBJECTIVE
To explore the clinical diagnostic features and treatment of desmoplastic small round cell tumor (DSRCT), and to improve the understanding and management of this tumor.
METHODS
The clinicopathological data of nine patients treated in our hospital from October 2004 to June 2014 were retrospectively analyzed and a review of the literature was made. The clinical manifestations, pathological characteristics, diagnosis and differential diagnosis, treatment and prognosis of this tumor were summarized and analyzed.
RESULTS
Nine patients with DSRCT, 5 males and 4 females, with an average age of 21 years (range 8-56 years) were included in this study. Ultrasound examination revealed irregular low-density mass shadow in the abdominal cavity. CT examination found that 6 cases had abdominal and retroperitoneal multiple solid tumor nodules, uneven density, and visible low density fluid area. Postoperative pathological examination revealed that the tumor cells were small, mostly elliptic, gathered to form clear structure of nests with clear irregular boundaries. The central portion of large tumor nests often showed necrosis. Scattered fibroblasts and large amount of hyalinization of collagen fibers were seen in the interstitial tissue around the nests. Six patients received laparotomy surgery, however, all failed to resect the tumor completely. Three patients received postoperative chemotherapy, i. e. two cases had carboplatin and paclitaxel chemotherapy, and one case of chemotherapy regimen not specified. Two patients had radiation and chemotherapy (no concrete plan was available). Another case was lost to follow-up. Two of the three patients without surgery received chemotherapy with CAP (cyclophosphamide+adriamycin+carboplatin) and total rectal lesions, pelvic and inguinal lymph nodes, ilium metastases radiation therapy. Another one patient received EP regimen (DDP+VP16) which was then changed into a TP chemotherapy alone. Eight of the nine cases died shortly after surgery, and only one patient treated with chemotherapy alone was still alive after 11 months of follow-up.
CONCLUSIONS
Desmoplastic small round cell tumor is a very rare, special type of soft tissue tumor, with very poor prognosis. This tumor may be preliminarily diagnosed according to the imaging characteristics and detection of tumor markers, however, final diagnosis is made by pathology. Surgery is the priority of treatment, combined with complementary radiation and chemotherapy.
Topics: Abdominal Neoplasms; Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Carboplatin; Child; Combined Modality Therapy; Cyclophosphamide; Desmoplastic Small Round Cell Tumor; Doxorubicin; Female; Humans; Male; Middle Aged; Paclitaxel; Prognosis; Retrospective Studies
PubMed: 26813434
DOI: No ID Found -
The American Journal of the Medical... Aug 2020Diabetic foot ulcer (DFU) is one of the most common and severe complications of diabetes mellitus, which is becoming increasingly prevalent throughout the world, with... (Review)
Review
Diabetic foot ulcer (DFU) is one of the most common and severe complications of diabetes mellitus, which is becoming increasingly prevalent throughout the world, with high mortality and morbidity. Because of the complex pathophysiological processes involved, DFU is difficult to treat effectively with traditional therapies. Ozone therapy, an emerging method, has been reported as potentially beneficial for closure of DFUs and may gradually move to the forefront of clinical practice. Possible mechanisms of action include antioxidant capacity, pathogen inactivation, vascular and endogenous growth factor modulation, and immune system activation. However, some researchers are skeptical about its safety, and clinical trials are lacking. This article reviews the current research and application of ozone therapy for DFUs.
Topics: Administration, Rectal; Administration, Topical; Anti-Bacterial Agents; Blood Transfusion, Autologous; Diabetic Foot; Hemorheology; Humans; Hydrogen Peroxide; Immunologic Factors; Intercellular Signaling Peptides and Proteins; Interferons; Interleukin-2; NF-E2-Related Factor 2; Oxidants, Photochemical; Oxidative Stress; Ozone; Reactive Oxygen Species; Tumor Necrosis Factor-alpha
PubMed: 32534720
DOI: 10.1016/j.amjms.2020.05.012 -
The Korean Journal of Gastroenterology... Jan 2017Ulcerative colitis (UC) is a chronic inflammatory bowel disorder characterized by a relapsing and remitting course. The direct and indirect costs of the treatment of UC... (Review)
Review
Ulcerative colitis (UC) is a chronic inflammatory bowel disorder characterized by a relapsing and remitting course. The direct and indirect costs of the treatment of UC are high, and the quality of life of patients is reduced, especially during exacerbation of the disease. The incidence and prevalence of UC in Korea are still lower than those of Western countries, but have been rapidly increasing during the past decades. Various medical and surgical therapies, including biologics, are currently used for the management of UC. However, many challenging issues exist, which sometimes lead to differences in practice between clinicians. Therefore, the Inflammatory Bowel Disease Study Group of the Korean Association for the Study of Intestinal Diseases established the first Korean guideline for the management of UC in 2012. This is an update of the first guideline. It was generally made by the adaptation of several foreign guidelines as was the first edition, and encompasses treatment of active colitis, maintenance of remission, and indication of surgery for UC. The specific recommendations are presented with the quality of evidence and classification of recommendations.
Topics: Administration, Oral; Anti-Inflammatory Agents, Non-Steroidal; Antibodies, Monoclonal; C-Reactive Protein; Colitis, Ulcerative; Cyclosporine; Humans; Mercaptopurine; Mesalamine; Parenteral Nutrition; Prednisolone; Severity of Illness Index; Steroids; Tumor Necrosis Factor-alpha
PubMed: 28135789
DOI: 10.4166/kjg.2017.69.1.1 -
AJR. American Journal of Roentgenology May 2022Fistulizing perianal disease, a frequent, chronic, and often debilitating manifestation of Crohn disease (CD) in adults and children, has been relatively refractory to... (Review)
Review
Fistulizing perianal disease, a frequent, chronic, and often debilitating manifestation of Crohn disease (CD) in adults and children, has been relatively refractory to treatment in the past. The advent of biologic agents such as anti-tumor necrosis factor-α and cellular therapies, used in conjunction with a range of surgical interventions, has greatly improved disease outcomes, although complete remission can still be elusive. This Special Series review considers current perianal imaging options, specifically pelvic MRI and endoanal and transperineal ultrasound, as well as their roles in the diagnosis, management, and assessment of treatment response. Pelvic MRI is the first-line modality for imaging perianal CD, given the complexity of fistulas encountered in CD. MRI technical acquisition parameters for adults and children and an approach to MRI interpretation and reporting are provided. Anatomic classification systems for fistulizing peri-anal disease are presented. We also explore the history, current landscape, and future developments of MRI features of perianal disease as imaging biomarkers, to quantify activity and severity and to consider CD MRI-based inflammatory activity scores. We discuss the reliability and validation of a number of indexes (including the pediatric MRI-based perianal CD [PEMPAC] index, the MR novel index for fistula imaging in CD [MAGNIFI-CD], the Van Assche index, and the modified Van Assche index), their potential to quantify treatment response, and possible prognostic capabilities.
Topics: Adult; Child; Crohn Disease; Humans; Inflammation; Magnetic Resonance Imaging; Rectal Fistula; Reproducibility of Results
PubMed: 34549607
DOI: 10.2214/AJR.21.26615 -
Drugs in Context 2019Ulcerative colitis (UC) is a chronic idiopathic inflammatory disorder that involves any part of the colon starting in the rectum in a continuous fashion presenting... (Review)
Review
Ulcerative colitis (UC) is a chronic idiopathic inflammatory disorder that involves any part of the colon starting in the rectum in a continuous fashion presenting typically with symptoms such as bloody diarrhea, abdominal pain, and rectal urgency. UC is diagnosed based on clinical presentation and endoscopic evidence of inflammation in the colon starting in the rectum and extending proximally in the colon. The clinical presentation of the disease usually dictates the choice of pharmacologic therapy, where the goal is to first induce remission and then maintain a corticosteroid-free remission. There are multiple classes of drugs that are available and are used based on the clinical severity of the disease. For mild-to-moderate disease, oral or rectal formulations of 5-aminosalicylic acid are used. In moderate-to-severe UC, corticosteroids are usually used in induction of remission with or without another class of medications such as thiopurines or biologics including anti-tumor necrosis factor, anti-integrins, or Janus kinase inhibitors for maintenance of remission. Up to 15% of the patients may require surgery as they fail to respond to medications and have risk of developing dysplasia secondary to longstanding colitis.
PubMed: 31065290
DOI: 10.7573/dic.212572 -
The Lancet. Gastroenterology &... Jul 2022Guidelines on Crohn's perianal fistulas recommend anti-tumour necrosis factor (TNF) treatment and suggest considering surgical closure for patients with surgically... (Randomized Controlled Trial)
Randomized Controlled Trial
Short-term anti-TNF therapy with surgical closure versus anti-TNF therapy in the treatment of perianal fistulas in Crohn's disease (PISA-II): a patient preference randomised trial.
BACKGROUND
Guidelines on Crohn's perianal fistulas recommend anti-tumour necrosis factor (TNF) treatment and suggest considering surgical closure for patients with surgically amenable disease. However, long-term outcomes following these two strategies have not been directly compared. The aim of this study was to assess radiological healing in patients who received short-term anti-TNF treatment and surgical closure compared with those who received anti-TNF treatment alone.
METHODS
The PISA-II trial was a multicentre, patient preference study done in nine hospitals in the Netherlands and one hospital in Italy. Adult patients with Crohn's disease and an active high perianal fistula with a single internal opening were eligible for inclusion. After counselling, patients with no treatment preference were randomly assigned (1:1) using random block randomisation (block sizes of six without statification), to 4-month anti-TNF therapy and surgical closure or anti-TNF therapy for 1 year, after seton insertion. Patients with a treatment preference received their preferred therapy. The primary outcome was radiological healing assessed by MRI at 18 months, defined as a complete fibrotic tract or a MAGNIFI-CD (Magnetic Resonance Index for Fistula Imaging in Crohn's Disease) score of 0, assessed according to the intention-to-treat principle. Secondary outcomes included clinical closure, number of patients undergoing surgical reintervention and number of reinterventions, recurrences, and impact on quality of life measured by the Perianal Disease Activity Index (PDAI). Analyses were performed on an intention-to-treat basis and additionally an as-treated analysis for radiological healing and clinical closure. This study was registered at the Dutch Trial Registry, NL7625, and with EudraCT, 2018-002064-15, and is closed to accrual due to completion.
FINDINGS
Between Sept 14, 2013, and Dec 7, 2019, 94 patients were enrolled onto the trial, of whom 32 (34%) were randomly assigned and 62 (66%) chose a specific treatment. 38 (40%) patients were assigned to the surgical closure group and 56 (60%) patients to the anti-TNF group. At 18 months, radiological healing was significantly more common in the surgical closure group (12 [32%] patients) than in the anti-TNF group (five [9%] patients; p=0·005). By contrast, clinical closure was not significantly different between the two treatment groups (26 [68%] patients in the surgical closure group vs 29 [52%] patients in the anti-TNF group; p=0·076). Significantly fewer patients required a reintervention in the surgical closure group than in the anti-TNF therapy group (five [13%] patients in the surgical closure group, median one reintervention [IQR one to three] vs 24 [43%] patients in the anti-TNF group, median two reinterventions [one to two]; p=0·005). Among patients who reached clinical closure during follow-up, four (14%) of 29 in the surgical closure group and five (16%) of 31 in the anti-TNF therapy group had a recurrence, which occurred only in patients without radiological healing. PDAI was significantly lower in the surgical closure group than in the anti-TNF group after 18 months (p=0·031). Adverse events and serious adverse events were similar in both treatment groups and mostly entailed reinterventions. Ten (11%) patients had side-effects associated with anti-TNF treatment. Two serious adverse events unrelated to study treatment occurred (appendicitis and myocardial infarction). One patient died from a tongue base carcinoma, unrelated to study treatment.
INTERPRETATION
Short-term anti-TNF treatment combined with surgical closure induces long-term MRI healing more frequently than anti-TNF therapy in patients with Crohn's perianal fistulas. These data suggest that patients with Crohn's perianal fistula amenable for surgical closure should be counselled for this therapeutic approach.
FUNDING
Netherlands Organisation for Health Research and Development and Broad Medical Research Program.
Topics: Adult; Crohn Disease; Humans; Patient Preference; Quality of Life; Rectal Fistula; Treatment Outcome; Tumor Necrosis Factor Inhibitors; Tumor Necrosis Factor-alpha
PubMed: 35427495
DOI: 10.1016/S2468-1253(22)00088-7 -
World Journal of Gastroenterology Apr 2020Infliximab (IFX), as a drug of first-line therapy, can alter the natural progression of Crohn's disease (CD), promote mucosal healing and reduce complications,... (Review)
Review
Infliximab (IFX), as a drug of first-line therapy, can alter the natural progression of Crohn's disease (CD), promote mucosal healing and reduce complications, hospitalizations, and the incidence of surgery. Perianal fistulas are responsible for the refractoriness of CD and represent a more aggressive disease. IFX has been demonstrated as the most effective drug for the treatment of perianal fistulizing CD. Unfortunately, a significant proportion of patients only partially respond to IFX, and optimization of the therapeutic strategy may increase clinical remission. There is a significant association between serum drug concentrations and the rates of fistula healing. Higher IFX levels during induction are associated with a complete fistula response in these patients. Given the apparent relapse of perianal fistulizing CD, maintenance therapy with IFX over a longer period seems to be more beneficial. It appears that patients without deep remission are at an increased risk of relapse after stopping anti-tumor necrosis factor agents. Thus, only patients in prolonged clinical remission should be considered for withdrawal of IFX treatment when biomarker and endoscopic remission is demonstrated, especially when the hyperintense signals of fistulas on T2-weighed images have disappeared on magnetic resonance imaging. Fundamentally, the optimal timing of IFX use is highly individualized and should be determined by a multidisciplinary team.
Topics: Crohn Disease; Drug Administration Schedule; Humans; Infliximab; Magnetic Resonance Imaging; Proctoscopy; Rectal Fistula; Recurrence; Remission Induction; Secondary Prevention; Time Factors; Treatment Outcome; Wound Healing
PubMed: 32327905
DOI: 10.3748/wjg.v26.i14.1554 -
Langenbeck's Archives of Surgery Nov 2023Delayed coloanal anastomosis (DCAA) is a two-stage procedure. DCAA has been increasingly reused in recent years in the management of rectal cancer. Such increased use of...
INTRODUCTION
Delayed coloanal anastomosis (DCAA) is a two-stage procedure. DCAA has been increasingly reused in recent years in the management of rectal cancer. Such increased use of DCAA has highlighted the complications associated with this procedure. We aimed to evaluate the risk and risk factors of ischemia/necrosis of the colonic stump between the two stages of DCAA.
PATIENTS AND METHODS
All patients who underwent a proctectomy with a DCAA were included in this retrospective single-centre study from November 2012 to June 2022. Two groups of patients were defined: patients with a well vascularized colonic stump (well vascularized group) and those who experienced ischemia or necrosis of the colonic stump (ischemic group). The primary endpoint was the rate of ischemia or necrosis of the colonic stump and an evaluation of the associated risk factors.
RESULTS
During the study period, 43 patients underwent DCAA. Amongst them, 32 (75%) had a well-vascularized colonic stump (well-vascularized group) and 11 (25%) ischemia of the colonic stump (ischemic group). Relative to patients in the well-vascularized group, those in the ischemic group were more often men (81.8% vs 40.6%, p = 0.034), had a higher BMI (29.2 kg/m vs 25.7 kg/m, p = 0.03), were more frequently diabetic (63.6% vs 21.9%, p = 0.01) and more frequently had had preoperative radiotherapy (100% vs 53.1%, p = 0.008). On the preoperative CT scan, the interspinous diameter was shorter in the ischemic group (9.4 ± 1.01 cm vs 10.6 ± 1.01 cm, p = 0.001), the intertuberosity diameter was shorter (9.2 ± 1.18 cm vs 11.9 ± 1.18 cm, p < 0.0001), and the length of the anal canal was longer (31.9 ± 3 mm vs 27.4 ± 3.2 mm, p < 0.0001).
CONCLUSION
This study highlights clear risk factors for ischemia/necrosis of the colonic stump after proctectomy with DCAA.
Topics: Male; Humans; Retrospective Studies; Treatment Outcome; Anastomosis, Surgical; Proctectomy; Rectal Neoplasms; Anal Canal; Colon; Risk Factors; Necrosis; Postoperative Complications
PubMed: 37910292
DOI: 10.1007/s00423-023-03157-z -
Beijing Da Xue Xue Bao. Yi Xue Ban =... Aug 2023To summarize and analyze the clinical characteristics of patients diagnosed with hepatic portal venous gas (HPVG). (Observational Study)
Observational Study
OBJECTIVE
To summarize and analyze the clinical characteristics of patients diagnosed with hepatic portal venous gas (HPVG).
METHODS
This was a single center retrospective observational study. All of the patients were diagnosed with HPVG. The patients were admitted to Peking University Third Hospital from January 2017 to January 2021. Demographic characteristics, clinical manifestations, laboratory tests, abdominal imaging, treatment of the primary disease, and clinical outcomes of the patients were collected via electronic medical records. The study was approved by institutional review board and the information of all the patients was kept de-identified.
RESULTS
A total of seven cases were included in the study. The median age of the patients was 67 (63, 81) years. Six of the patients were male. The seven patients all presented with sudden onset of severe abdominal pain, which was the most common symptom. Six patients developed septic shock after admission. The signs of HPVG were detected by CT scans in all the patients, showing gas embolization. It might also be found as unique "aquarium sign" in abdominal ultrosonography. Four cases were caused by intestinal lesions, including acute volvulus, intestinal obstruction, and rectal abscess. Two were caused by ischemic bowel disease and the other one was caused by severe acute pancreatitis. The gas accumulation could disappear after effective anti-shock therapy and surgery (Cases 1, 2, and 6). Two patients had good postoperative outcomes, and one patient was discharged after non-surgical treatment. However, the prognosis was poor in the patients with intestinal ischemia necrosis accompanied by shock and multiple organ dysfunction (Cases 3, 4, 5, and 7 all died).
CONCLUSION
The HPVG patients generally have acute abdominal pain and show up at Emergency Department. The prognosis depends on the potential cause of HPVG. The mechanism and clinical management for the appearance of gas in the portal vein is not well understood. Patients complicated with shock, ascites, and peritonitis may have intestinal necrosis, which indicates surgical intervention and higher mortality. CT is the preferred diagnostic method in standard clinical practice. Physicians need to have a comprehensive understanding of the proactive diagnostic strategy, and active treatment for the primary disease.
Topics: Humans; Male; Female; Acute Disease; Pancreatitis; Portal Vein; Vascular Diseases; Necrosis; Abdominal Pain
PubMed: 37534661
DOI: 10.19723/j.issn.1671-167X.2023.04.028 -
Canadian Journal of Gastroenterology &... 2016Crohn's disease (CD) is a complex disorder with important incidence in North America. Perianal fistulas occur in about 20% of patients with CD and are almost always... (Review)
Review
Crohn's disease (CD) is a complex disorder with important incidence in North America. Perianal fistulas occur in about 20% of patients with CD and are almost always classified as complex fistulas. Conventional treatment options have shown different success rates, yet there are data indicating that these approaches cannot achieve total cure and may not improve quality of life of these patients. Fibrin glue, fistula plug, topical tacrolimus, local injection of infliximab, and use of hematopoietic stem cells (HSC) and mesenchymal stem cells (MSC) are newly suggested therapies with variable success rates. Here, we aim to review these novel therapies for the treatment of complex fistulizing CD. Although initial results are promising, randomized studies are needed to prove efficacy of these approaches in curing fistulizing perianal CD.
Topics: Crohn Disease; Cutaneous Fistula; Fibrin Tissue Adhesive; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Infliximab; Mesenchymal Stem Cell Transplantation; Rectal Fistula; Tacrolimus; Tumor Necrosis Factor-alpha
PubMed: 28053967
DOI: 10.1155/2016/1651570