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.

Primitive neuroectodermal tumors of the brainstem in children treated according to the HIT trials: clinical findings of a rare disease

2015 ◽  
Vol 15 (3) ◽  
pp. 227-235 ◽  
Author(s):  
Carsten Friedrich ◽  
Monika Warmuth-Metz ◽  
André O. von Bueren ◽  
Johannes Nowak ◽  
Brigitte Bison ◽  
...  
Keyword(s):  
Survival Rate ◽  
Residual Disease ◽  
Case Series ◽  
Population Based ◽  
Postoperative Treatment ◽  
Subtotal Resection ◽  
Maintenance Chemotherapy ◽  
Primitive Neuroectodermal Tumors ◽  
Axial Diameter ◽  
Neuroectodermal Tumors

OBJECT Primitive neuroectodermal tumors of the central nervous system (CNS-PNET) arising in the brainstem are extremely rare, and knowledge about them is limited. The few existing case series report fatal outcomes. The purpose of this study was to analyze clinical characteristics of and outcome for brainstem CNS-PNET patients treated according to the consecutive, population-based HIT studies covering a 19-year time period. METHODS Between September 1992 and November 2011, 6 eligible children with histologically proven brainstem CNS-PNET not otherwise specified and 2 children with brainstem ependymoblastomas (3, partial resection; 3, subtotal resection; 2, biopsy), median age 3.3 years (range 1.2–10.6 years), were treated according to consecutive multimodal HIT protocols for CNS-PNET/medulloblastoma. Postoperative treatment was according to maintenance chemotherapy protocols (3, craniospinal irradiation [CSI] followed by maintenance chemotherapy), sandwich chemotherapy protocols (2, neoadjuvant chemotherapy, CSI, maintenance chemotherapy), or a therapy protocol for children younger than 4 years (3, postoperative chemotherapy followed by CSI). RESULTS The median duration of prediagnostic symptoms, predominantly cranial nerve deficits (n = 7), pyramidal tract signs (n = 5), or ataxia (n = 5), was 5 weeks (range 1–13 weeks). The tumors were all located in the pons. Most involved more than half of the pontine axial diameter and were sharply marginated. All patients had postoperative residual disease, including metastasis in 1 case. With 1 exception all tumors progressed early during treatment within 3.9 months (range 2.5–10.4 months), leading to a 1-year event-free survival rate (± standard error) of 13% ± 12%. After progression, patients succumbed early to their disease resulting in a 1-year overall survival rate of 25% ± 15%. The only surviving patient had a partially resected CNS-PNET, received a sandwich chemotherapy protocol, and is without disease progression 14 months after diagnosis. CONCLUSIONS CNS-PNET is a rare but important differential diagnosis in childhood brainstem tumors. So far, efficient therapies are lacking. The sampling of tumor material for improved biological understanding and identification of new therapeutic targets is important.

scholarly journals Wound myiasis in Western Europe: prevalence and risk factors in a changing climate scenario

2021 ◽  
Author(s):  
Erika Andreatta ◽  
Luigi Bonavina
Keyword(s):  
Risk Factors ◽  
Literature Review ◽  
Western Europe ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Climate Scenario ◽  
Cutaneous Myiasis ◽  
Artery Disease ◽  
Wound Myiasis

Summary Background Myiasis is an infestation of neglected open wounds by fly larvae. Global warming, related climate changes, and increased traveling in tropical countries may contribute to the worldwide diffusion of new fly species. Common risk factors for myiasis are advanced age, poor hygiene, malnourishment, social isolation, diabetes, cancer, and peripheral artery disease. The aim of this study was to review the characteristics of cases of myiasis reported in Europe. Methods A narrative review of cutaneous myiasis was performed using PubMed, EMBASE, Web of Science database, and the search terms “human,” “myiasis,” “cutaneous myiasis,” “maggot,” “Europe,” “wound,” with the combination of “AND” and “OR.” In addition, hospital charts were reviewed to add personal experience to the literature review. Results Overall, 52 articles, both single case reports and case series reporting cutaneous myiasis, were found in the medical literature of the past two decades. A total of 64 patients living in Europe were identified, of whom 36% had wound myiasis. In addition to the literature review, we report the case of a male patient who presented with myiasis within a deep scalp ulcer occurring after radiotherapy for basal cell carcinoma. Conclusion Myasis is rare and possibly under-reported in Europe. The epidemiology of this infestation may be changing in the future due to climate variability, a rise in travel to tropical areas, and the migration of fly species.

Fertility and Pregnancy in Women With Hypopituitarism: A Systematic Literature Review

2019 ◽  
Vol 105 (3) ◽  
pp. e53-e65 ◽  
Author(s):  
Greisa Vila ◽  
Maria Fleseriu
Keyword(s):  
Literature Review ◽  
Ovulation Induction ◽  
Single Case ◽  
Case Reports ◽  
Hormone Replacement ◽  
Case Series ◽  
Live Birth Rate ◽  
Normal Pregnancy ◽  
Human Reproduction ◽  
And Function

Abstract Context Human reproduction is mainly governed from the hypothalamic–adrenal–gonadal (HPG) axis, which controls both ovarian morphology and function. Disturbances in the secretion of other anterior pituitary hormones (and their respective endocrine axes) interfere with HPG activity and have been linked to fertility problems. In normal pregnancy, maintenance of homeostasis is associated with continuous changes in pituitary morphology and function, which need to be considered during hormone replacement in patients with hypopituitarism. Design We conducted a systematic PubMed literature review from 1969 to 2019, with the following keywords: fertility and hypopituitarism, pregnancy and hypopituitarism, and ovulation induction and hypopituitarism. Case reports or single-case series of up to 2 patients/4 pregnancies were excluded. Results Eleven publications described data on fertility (n = 6) and/or pregnancy (n = 7) in women with hypopituitarism. Women with hypopituitarism often need assisted reproductive treatment, with pregnancy rates ranging from 47% to 100%. In patients achieving pregnancy, live birth rate ranged from 61% to 100%. While glucocorticoids, levothyroxine, and desmopressin are safely prescribed during pregnancy, growth hormone treatment regimens vary significantly between countries, and several publications support a positive effect in women seeking fertility. Conclusions In this first systematic review on fertility, ovulation induction, and pregnancy in patients with hypopituitarism, we show that while literature is scarce, birth rates are high in patients achieving pregnancy. However, prospective studies are needed for evaluating outcomes in relationship to treatment patterns. Replacement therapy in hypopituitarism should always mimic normal physiology, and this becomes challenging with changing demands during pregnancy evolution.

scholarly journals Post-Infectious Guillain–Barré Syndrome Related to SARS-CoV-2 Infection: A Systematic Review

Life ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 167
Author(s):  
Pasquale Sansone ◽  
Luca Gregorio Giaccari ◽  
Caterina Aurilio ◽  
Francesco Coppolino ◽  
Valentina Esposito ◽  
...  
Keyword(s):  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Guillain Barre Syndrome ◽  
Response To Treatment ◽  
Comprehensive Overview ◽  
Fisher Syndrome ◽  
Immunomodulating Therapy ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Background. Guillain-Barré syndrome (GBS) is the most common cause of flaccid paralysis, with about 100,000 people developing the disorder every year worldwide. Recently, the incidence of GBS has increased during the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) epidemics. We reviewed the literature to give a comprehensive overview of the demographic characteristics, clinical features, diagnostic investigations, and outcome of SARS-CoV-2-related GBS patients. Methods. Embase, MEDLINE, Google Scholar, and Cochrane Central Trials Register were systematically searched on 24 September 2020 for studies reporting on GBS secondary to COVID-19. Results. We identified 63 articles; we included 32 studies in our review. A total of 41 GBS cases with a confirmed or probable COVID-19 infection were reported: 26 of them were single case reports and 6 case series. Published studies on SARS-CoV-2-related GBS typically report a classic sensorimotor type of GBS often with a demyelinating electrophysiological subtype. Miller Fisher syndrome was reported in a quarter of the cases. In 78.1% of the cases, the response to immunomodulating therapy is favourable. The disease course is frequently severe and about one-third of the patients with SARS-CoV-2-associated GBS requires mechanical ventilation and Intensive Care Unit (ICU) admission. Rarely the outcome is poor or even fatal (10.8% of the cases). Conclusion. Clinical presentation, course, response to treatment, and outcome are similar in SARS-CoV-2-associated GBS and GBS due to other triggers.

scholarly journals Effectiveness of the Validation Method in Work Satisfaction and Motivation of Nursing Home Care Professionals: A Literature Review

2020 ◽  
Vol 18 (1) ◽  
pp. 201
Author(s):  
Iván Sánchez-Martínez ◽  
Raül Vilar ◽  
Javier Irujo ◽  
Duna Ulsamer ◽  
Dolors Cano ◽  
...  
Keyword(s):  
Job Satisfaction ◽  
Literature Review ◽  
Older People ◽  
Single Case ◽  
Case Series ◽  
Nursing Home Care ◽  
Validation Method ◽  
People With Dementia ◽  
Cochrane Database ◽  
Quasi Experimental

The purpose of this study was to carry out a literature review on the effectiveness of the validation method (VM) in job satisfaction and motivation of care professionals working with older people in nursing homes. The review was carried out in specialised databases: Scopus, PsychINFO, PubMed, Web of Science (WOS), Google Scholar, Scielo, and Cochrane Database of Systematic Reviews. 9046 results were obtained, out of which a total of 14 studies met the inclusion criteria: five quantitative, four qualitative, one single case series, two quasi-experimental and two mixed methods studies. The results of the analysed studies report that the VM can be an effective tool that facilitates communication and interaction in care, reducing levels of stress and job dissatisfaction among care professionals. The VM facilitates communication between professionals and older people with dementia, and improves the management of complex situations that may arise in care, directly influencing a reduction in work stress and increasing job satisfaction.

scholarly journals Schwannoma of the abdominal wall: updated literature review

2021 ◽  
Author(s):  
Tommaso Panici Tonucci ◽  
Andrea Sironi ◽  
Eleonora Pisa ◽  
Benedetta Di Venosa ◽  
Luigi Bonavina
Keyword(s):  
Differential Diagnosis ◽  
Literature Review ◽  
Abdominal Wall ◽  
Single Case ◽  
Case Reports ◽  
Rectus Abdominis ◽  
Malignant Neoplasms ◽  
Palpable Mass ◽  
Surgical Specimen ◽  
Benign Schwannoma

Summary Background Schwannoma is a benign tumor arising from Schwann cells of the peripheral nerves. It is often asymptomatic and can develop in the retroperitoneum, mediastinum, head and neck region, and upper and lower extremities. Schwannoma of the abdominal wall is extremely rare, but differential diagnosis with malignant neoplasms is important to reduce the risk of undertreatment. Methods A narrative review of abdominal wall schwannoma was performed using PubMed, EMBASE, and Web of Science database and the search terms “schwannoma”, “neurinoma”, “neurilemmoma”, “soft tissue tumors”, “neurogenic tumor”, “rectus abdominis mass”, “abdominal wall”. In addition, the hospital charts were reviewed to report the personal experience. Results Only 9 single case-reports of benign schwannoma of the abdominal wall were found in the English medical literature over the past decade. None of the patients received preoperative biopsy and all were resected with clear margins. In addition to the literature review, we report the case of a 58-year-old man referred for a palpable mass in the left upper abdominal quadrant. Ultrasonography and magnetic resonance imaging revealed a solid and well-encapsulated mass inside the left rectus abdominis muscle. A core biopsy of the lesion provided the diagnosis of cellular schwannoma and this was confirmed by histopathologic examination of the surgical specimen. Conclusions Benign schwannoma of the abdominal wall is extremely rare. Percutaneous core needle biopsy is important for the differential diagnosis with more common and biologically more aggressive malignancies, such as desmoid tumors and sarcomas, and may be relevant for planning the most appropriate management.

Management of Refractory Intracranial Hypotension Using Percutaneous Fibrin Sealant Patch – A Case Series and Review of the Literature

2019 ◽  
pp. 13-31
Author(s):  
Kevin Wong
Keyword(s):  
Literature Review ◽  
Fibrin Sealant ◽  
Intracranial Hypotension ◽  
Case Reports ◽  
Case Series ◽  
Retrospective Case ◽  
Percutaneous Injection ◽  
Retrospective Case Series ◽  
Blood Patch

Background: Intracranial hypotension (IH) among patients with persistent cerebrospinal fluid (CSF) leakage remains a challenging problem. The majority of these cases resolve spontaneously with conservative measures. The customary treatment for IH is epidural blood patch (EBP). In some cases, CSF leaks can persist for months or even years despite multiple trials of EBP. To date, there are only a limited number of published studies documenting the percutaneous injection of fibrin sealant for treatment of IH refractory to conservative measures and EBP. Objective: Our objective was to perform a literature review and retrospective case series regarding patients who underwent percutaneous injection of fibrin sealant for treatment of refractory IH at our institution. Study Design: This case series used a singlecentered retrospective observational study design and literature review. Setting: Patients in this case series were treated at a community-based tertiary care medical center. Methods: Five consecutive patients with the diagnosis of IH refractory to conservative measures and EBP who underwent percutaneous patching with fibrin sealant were identified at our institution between January 1, 2000 and January 1, 2016. A retrospective chart review was performed and data including demographics, characteristics, interventions, clinical outcomes, and complications were collected. A critical review of the current literature regarding the percutaneous use of fibrin sealant for treatment of IH was conducted. Results: Four of the 5 patients (80%) experienced no further symptoms of IH and no adverse events were noted. One patient (20%) ultimately required surgical duroplasty. Review of the current literature showed a total of 2 prospective case series, 4 retrospective case series, and 11 case reports. Our present case series and literature review demonstrated that fibrin sealants were well-tolerated by most patients and associated with low incidences of complications and recurrence. Limitations: This study is limited by the small retrospective case series of 5 patients. Conclusions: Percutaneous injection of fibrin sealant may be considered in refractory cases of IH when repeated trials of EBP have persistently failed. It appears to be a highly effective, safe, and easy-touse alternative therapy for patients with refractory IH in an ambulatory setting. Our review of the literature revealed only studies with low quality of evidence, including case series and case reports. There is a substantial need for high-quality studies and clinical evidence to corroborate the efficacy and safety of this percutaneous technique. However, this ideal is very challenging because of the relative rarity and heterogeneous etiologies of cases. Keywords: Fibrin sealant, intracranial hypotension, CSF leak, epidural blood patch, orthostatic headache, refractory, quality of life, percutaneous

Conservative Management in Two Penetrating Fishbone Injuries: A Case Series and Literature Review

2021 ◽  
Keyword(s):  
Foreign Body ◽  
Literature Review ◽  
Conservative Management ◽  
Case Reports ◽  
Case Series ◽  
Foreign Body Ingestion ◽  
Medical Referral

Foreign body ingestion is a regular medical referral. Patients present with different objects such as chicken bones, nails, coins, and fishbones. It is usually managed in causality and passes without any intervention. However, occasionally, we come across fishbone complications requiring intervention. We discuss the course and management of two case reports of fishbone injuries in different abdominal regions.

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

2018 ◽  
Vol 79 (05) ◽  
pp. 408-415 ◽  
Author(s):  
Jan Victor ◽  
Thibault Poujade ◽  
Nadine Hollevoet ◽  
Gregoire Chick
Keyword(s):  
Systematic Review ◽  
Consensus Statement ◽  
Case Reports ◽  
Case Series ◽  
Spinal Tumors ◽  
The Mean ◽  
Tumor Distribution ◽  
Key Aspects ◽  
Number Of Individuals

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected. Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria. Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%). Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

scholarly journals Renal primitive neuroectodermal tumour: Case series and brief review

2014 ◽  
Vol 8 (3-4) ◽  
pp. 241
Author(s):  
Tadeusz Kroczak ◽  
Rajan Sharda ◽  
Darrel Drachenberg ◽  
Turki Al-Essawi
Keyword(s):  
Literature Review ◽  
Inferior Vena Cava ◽  
Primitive Neuroectodermal Tumor ◽  
Inferior Vena ◽  
Vena Cava ◽  
Case Series ◽  
Primitive Neuroectodermal Tumour ◽  
Pathologic Features ◽  
Rare Malignancy ◽  
Pathologic Analysis

Renal primitive neuroectodermal tumor is a rare malignancy. These tumours rarely present with caval involvement. We report 2 cases of primitive neuroectodermal tumours (PNETs) with inferior vena cava involvement. The initial presentation and outcomes differed significantly. The diagnosis was confirmed using histologic and pathologic analysis. We present a brief literature review and an outline of typical clinical and pathologic features of renal PNETs.

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