scholarly journals Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

Diagnostics ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. 1680
Author(s):  
Francesco Fabozzi ◽  
Silvia Ceccanti ◽  
Antonella Cacchione ◽  
Giovanna Stefania Colafati ◽  
Andrea Carai ◽  
...  
Keyword(s):  
Literature Review ◽  
Case Reports ◽  
Case Series ◽  
Surgical Excision ◽  
Central Canal ◽  
Ependymal Cells ◽  
The Past ◽  
First Case ◽  
One Year

Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years.

Monoclonal antibody extravasations: Two case reports and literature review

2020 ◽  
pp. 107815522095000
Author(s):  
Alicia Rodríguez-Alarcón ◽  
David Conde-Estévez
Keyword(s):  
Monoclonal Antibody ◽  
Case Report ◽  
Literature Review ◽  
Case Reports ◽  
Rare Complication ◽  
Specific Treatment ◽  
Intravenous Chemotherapy ◽  
Chemotherapy Administration ◽  
First Case

Introduction Extravasation is a rare complication from intravenous chemotherapy administration. Literature about monoclonal antibody (MoAb) extravasations is scarce and also conflicting in how they are classified. Case report We reported two different cases of MoAb extravasations with cetuximab and nivolumab outcome respectively. The administration site appeared inflamed and patients did not report disturbances. Management and outcome: Both extravasations did not require specific treatment. General unspecific measures suffice to properly manage these extravasations and no sequels were observed after long follow-up. Both patients received all further courses of MoAb without any adverse events. Discussion To our knowledge, we reported the first case-report of nivolumab extravasation in the literature. In addition, the cetuximab extravasation management and outcome was in accordance with previously published reports. Both MoAb may be considered as non-aggressive or neutral. We reviewed published information about MoAb extravasations. In conclusion, not all MoAb should be classified in the same category when extravasated and special precautions are warranted with conjugated MoAb and bevacizumab.

scholarly journals DOP79 Primary hypogammaglobulinemia with IBD-like features: An ECCO CONFER Multicenter Case Series

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S111-S111
Author(s):  
A Albshesh ◽  
P Eder ◽  
D G Ribaldone ◽  
B Oldenburg ◽  
N K De Boer ◽  
...  
Keyword(s):  
Case Reports ◽  
Case Series ◽  
Iga Deficiency ◽  
Clinical Feature ◽  
Aminosalicylic Acid ◽  
Igg Subclass Deficiency ◽  
Selective Iga Deficiency ◽  
Appropriate Treatment ◽  
One Year

Abstract Background The most commonly recognized clinical feature of hypogammaglobulinemia is recurrent infections with high prevalence of gastrointestinal manifestations. In some cases, clinical and endoscopic features are indistinguishable from those of inflammatory bowel disease (IBD). Methods This was a multicenter case series performed as a part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. Results This report includes 25 patients with primary hypogammaglobinemia and IBD-like features [20 males and 5 females, mean age 50 years (±21.7 SD)]. Crohn’s disease-like features were noted in 22 patients, three patients had ulcerative colitis-like features. The diagnosis of hypogammaglobulinemia preceded IBD-like features diagnosis in 20 patients (mean of 7.7 years prior, range 0.3–35 years), and followed IBD-like features appearance in 5 cases (mean of one year after, 0.4- 9.1 years). Hypogammaglobinemia etiologies were common variable immunodeficiency (72%), Agammaglobulinemia (8%), selective IgA-deficiency (8%), Goods syndrome (8%), IgG subclass deficiency with IgA deficiency (4%). In addition to antibiotics and intravenous immunoglobulin (IVIG) as a treatment for hypogammaglobinemia, fifteen patients received IBD treatment during the follow-up period, of whom two were on 5-aminosalicylic acid, five on corticosteroids, three on immunomodulatory, four on anti-tumor necrosis factor, and one on vedolizumab. By the end of the follow-up [35.5 months (Interquartile range 18–75)], 20 of 25 (80%) patients were in clinical remission. Conclusion This case series illustrates a strong male and CD-like features predilection. The diagnosis of IBD-like features mainly occurs after that of hypogammaglobulinemia, the majority of cases successfully recovered after appropriate treatment.

scholarly journals LONG-TERM FOLLOW-UP OF PATIENTS UNDERGOING ENDOSCOPIC PAPILLECTOMY FOR PAPILLARY NEUROENDOCRINE TUMOR: A CASE SERIES AND LITERATURE REVIEW

2021 ◽  
Author(s):  
Facundo GALETTI ◽  
Jessé Clementino de ARAÚJO-FILHO ◽  
Eliza Ribeiro Alves de ANDRADE ◽  
Eloy TAGLIERI ◽  
Otávio MICELLI-NETO ◽  
...  
Keyword(s):  
Literature Review ◽  
Case Reports ◽  
Case Series ◽  
Primary Treatment ◽  
Tertiary Referral Center ◽  
Endoscopic Papillectomy ◽  
Long Term Follow Up ◽  
Comprehensive Search ◽  
Well Differentiated

ABSTRACT BACKGROUND: Duodenal papilla neuroendocrine tumors (DP-NET) are rare. Surgical therapy may be recommended for their treatment. However, they have high rates of morbidity and mortality. Endoscopic papillectomy (EP) is safe and effective for complete resection of tumors at this site. OBJECTIVE: This study aimed to describe a case series of DP-NETs resected by EP and perform a literature review. METHODS: A series of patients with DP-NETs underwent EP as primary treatment between Jan/2008 and Mar/2020 at a tertiary referral center. A comprehensive search was made on the MEDLINE primary electronic database. The search strategy was designed to find all articles related to DP-NETs published in the literature. RESULTS: Six patients underwent EP for presenting DP-NETs, four of whom were women (mean age, 63 years). The mean diameter of DP-NETs was 1.6±0.3 cm. Four of six patients were followed up, one of whom suffered relapse at the resection site after 3 months and was referred to surgery (pT3n1b) and the remaining three patients experienced no endoscopic or histological recurrence during follow-up periods of 10, 7, and 4 years, respectively. Eighteen articles were found in the literature search in MEDLINE. The articles included case reports of endoscopically treated DP-NETs. CONCLUSION: EP is safe and effective for DP-NETs that are ≤20 mm, confined to the submucosal layer, well-differentiated, and without local or remote metastasis. Adequate endoscopic follow-up and definitive surgical treatment in the presence of relapse are necessary.

Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series

2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  
Keyword(s):  
Systematic Review ◽  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Mechanical Thrombectomy ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Neurological Outcomes

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.

Highly selective diversion with proactive leakage management after low anterior resection for rectal cancer

2021 ◽  
Author(s):  
K Talboom ◽  
I Vogel ◽  
R D Blok ◽  
S X Roodbeen ◽  
C Y Ponsioen ◽  
...  
Keyword(s):  
Low Anterior Resection ◽  
Case Series ◽  
Fecal Diversion ◽  
Single Center ◽  
Single Centre ◽  
Diverting Stoma ◽  
Faecal Diversion ◽  
One Year ◽  
Diversion Rate

Abstract In this single center case series with nine percent primary diversion, 86 of 94 patients alive and with complete follow-up at one year had a functioning anastomosis. Seventy-five of the initial 99 patients never had a stoma. Meaning: Highly selective fecal diversion in combination with proactive leakage management, low anastomoses can be preserved safely, and the majority of patients will be spared all disadvantages of a diverting stoma. In this single-centre case series, with a primary diversion rate of 9 per cent, 86 of 94 patients who were alive and had complete follow-up at 1 year had a functioning anastomosis. Seventy-five of the initial 99 patients never had a stoma. The results indicate that, with highly selective faecal diversion in combination with proactive leakage management, low anastomoses can be preserved safely, and the majority of patients will be spared the disadvantages of a diverting stoma.

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

2021 ◽  
Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang
Keyword(s):  
Literature Review ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Subtotal Resection ◽  
Study Cohort ◽  
Primitive Neuroectodermal Tumors ◽  
Pathologic Features ◽  
Neuroectodermal Tumors ◽  
The Mean

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.

Penile Lymphoepithelioma-Like Carcinoma: A Rare Case With PD-L1 Expression

2021 ◽  
pp. 106689692098834
Author(s):  
Raquel Machado-Neves ◽  
Bernardo Teixeira ◽  
Elsa Fonseca ◽  
Pedro Valente ◽  
Joaquim Lindoro ◽  
...  
Keyword(s):  
Human Papillomavirus ◽  
Tumor Cells ◽  
Rare Case ◽  
Malignant Tumors ◽  
Squamous Cell Carcinomas ◽  
The Third ◽  
The Past ◽  
First Case ◽  
Lymphoplasmacytic Infiltrate

Most malignant tumors of the penis are squamous cell carcinomas (SCC), being divided in 2 groups, one human papillomavirus (HPV)-related and another non-HPV-related, with lymphoepithelioma-like carcinoma (LELC) being one of the rarest HPV-related SCC. In this article, we report a case of a 50-year-old man who presented testicular swelling and pain for the past 3 months. A penile mass was identified, and the patient was submitted to a total penectomy. The penectomy specimen showed an ulcerated lesion at the glans reaching the cavernous bodies. Microscopic examination showed undifferentiated epithelial cells with syncytial growth pattern mix with a dense lymphoplasmacytic infiltrate, consistent with LELC. The tumor cells expressed p16 and all 3 different clones of PDL1 (22C3, SP263, and SP142). The patient is alive and well with a follow-up of 3 months. To our knowledge, this is the third LELC of the penis reported in literature and the first case reported with PDL1 expression.

scholarly journals Digital Glomus Tumor: Bibliographic Review of the Studies Published over the Past 10 Years

2021 ◽  
Vol 49 (01) ◽  
pp. 046-055
Author(s):  
Victoria Hernández ◽  
Tania Lena ◽  
Eliana Camacho ◽  
Matías Craviotto
Keyword(s):  
Glomus Tumor ◽  
Case Reports ◽  
Low Frequency ◽  
Benign Neoplasm ◽  
Surgical Excision ◽  
Soft Tissue Tumors ◽  
Glomus Tumors ◽  
The Past ◽  
Pubmed Search ◽  
Magnetic Resonance Imaging Mri

AbstractGlomus tumors are a mostly benign neoplasm that constitutes less than 4% of upper-limb soft-tissue tumors. Its unspecific clinical presentation, added to its low frequency, leads to a late diagnosis.The objective of the present study is to update the clinical-paraclinical approach and the surgical technique used in the treatment.We carried out a literature review from 2014 to 2019 on digital glomus tumor in the hand in adult patients using the PubMed search engine.In most of the publications analyzed, the diagnosis was clinical, with a delay of 1 to 10 years. Plain radiography is the most requested study; of the 16 articles reporting its indication, only half evidenced compatible changes. Magnetic resonance imaging (MRI) was requested in 15 articles, presenting normal results in 3 of them. The treatment of choice was surgical excision using a transungual approach. Only 4 articles report recurrence after excision.Although there is diversity in the approach to these tumors, we conclude that the diagnosis is clinical, and the treatment surgical, and there is no consensus regarding the paraclinical indication. The information available comes mainly from case reports, publications that contribute to the generation of evidence for the clinical practice in rare diseases such as this one.

One-year Follow-Up Results with Hydrogel Implant in Therapy of Hallux Rigidus: Case Series with 44 Patients

2021 ◽  
Author(s):  
Christoph Zanzinger ◽  
Norbert Harrasser ◽  
Oliver Gottschalk ◽  
Patrick Dolp ◽  
Florian Hinterwimmer ◽  
...  
Keyword(s):  
Learning Algorithm ◽  
Case Series ◽  
Metatarsophalangeal Joint ◽  
Hallux Rigidus ◽  
Therapy Failure ◽  
Retrospective Case ◽  
Aofas Score ◽  
Medium Level ◽  
One Year

Abstract Background The Cartiva implant (CI) is being increasingly used in the surgical therapy of hallux rigidus. Despite a growing number of studies, numerous questions regarding patient selection remain unanswered. Patients and Methods As part of a retrospective case series with prospective follow-up (average follow-up period: 12 months), a total of 44 patients (male/female = 16/28; mean age at the time of surgery: 55.4 years) with 44 CI were analysed (VAS, EFAS-, AOFAS-score). Using a correlation analysis and a machine learning algorithm, risk factors for therapy failure were investigated. Results The overall survival rate of the CI was 93% at 12 months. The VAS, EFAS and AOFAS scores showed a significant improvement in comparison to the preoperative condition. The mobility of the metatarsophalangeal joint showed no increase. Patients with a medium osteoarthritis grade and a medium level of clinical restraint showed the greatest improvement in relation to their preoperative condition. Conclusion The CI can be seen as an effective therapy for hallux rigidus. Nonetheless, realistic patient expectations must be communicated.

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