scholarly journals Malignant Transformation of Ossifying Fibroma into Parosteal Osteosarcoma with High-grade Component: Presentation of an Unusual Case and Review of the Literature

2018 ◽  
Vol 12 (1) ◽  
pp. 1059-1068
Author(s):  
Jahan Abtahi ◽  
Aida Ajan

Background: Parosteal Osteosarcoma of the Jaw (POSJ) is a rare entity that is associated with a high survival rate. Several case reports and case series of POSJ have been published in the literature, but few authors have described development of this tumor by possible transformation from a fibro-osseous neoplasm. Objective: We present a rare occurrence of parosteal osteosarcoma with involvement of the posterior maxilla, orbit floor, and infra-temporal fossa in a 20-year-old man. Furthermore, we performed a literature review regarding clinical, radiological, and histological features; treatment strategies; and etiology/pathophysiology. Methods: A PubMed search yielded a total of 74 articles and the articles were sorted according to their corresponding key area of focus. Results: This was a case of POSJ with high-grade component in the maxillofacial region of a 20-year old male. Co-expression of MDM2 and CDK4 was confirmed. At 2.5-year follow-up, the patient had died. The literature review revealed 18 articles including 20 cases of POSJ. Four cases represent the possible development of this tumor by transformation from a fibro-osseous neoplasm: Two cases of fibrous dysplasia, one case of cemento-ossifying fibroma, and the case of Ossifying Fibroma (OF) in the present study. Conclusion: In conclusion, we found an unusual case of POSJ of the midface in a patient with a previous diagnosis of OF in the same region. To our knowledge, there have been no previous reports of development of POSJ in OF. Furthermore, this is the first described case of high-grade surface osteosarcoma in the craniofacial region.

2018 ◽  
Vol 64 (12) ◽  
pp. 1147-1153
Author(s):  
Andrei F. Joaquim ◽  
Alpesh A. Patel

SUMMARY OBJECTIVE: The surgical management of high-grade lumbar spondylolisthesis (HGLS) is complex and aims to achieve both a solid fusion that is able to support the high shear forces of the lumbosacral junction, as well as neural decompression. We performed a systematic literature review of the safety and efficacy of posterior transdiscal (PTD) screw fixation from L5S1 for HGLS and its variations. METHODS: A systematic literature review following the PRISMA guidelines was performed in the PubMed database of the studies describing the use of PTD screw fixation for HGLS. Clinical and radiological data were extracted and discussed. Study quality was assessed with the Oxford Centre for Evidence-Based Medicine Levels of Evidence. RESULTS: Seven studies were included and reviewed; all of them were level IV of evidence. Two of them had large case series comparing different surgical techniques: one concluded that PTD was associated with better clinical outcomes when compared with standard screw fixation techniques and the other suggesting that the clinical and radiological outcomes of PTD were similar to those when an interbody fusion (TLIF) technique was performed, but PTD was technically less challenging. The remaining five studies included small case series and case reports. All of them reported the successful useful of PTD with or without technical variations. CONCLUSIONS: Our review concludes, with limited level of evidence that PTD fixation is a safe and efficient technique for treating HGLS patients. It is technically less demanding than a circumferential fusion, even though proper screw insertion is more demanding than conventional pedicle screw fixation.


Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110108
Author(s):  
Ashley D Hickman ◽  
Evandro D Bezerra ◽  
Anja C Roden ◽  
Matthew T Houdek ◽  
Jonathan D Barlow ◽  
...  

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.


2021 ◽  
Vol 3 ◽  
pp. 100071
Author(s):  
Vanesa Rodríguez-Fernández ◽  
Lucía Cameselle-Cortizo ◽  
María José Lamas González ◽  
Gonzalo José De Castro Parga ◽  
Javier Valdés-Pons ◽  
...  

Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang

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.


2019 ◽  
pp. 13-31
Author(s):  
Kevin Wong

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


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.


2019 ◽  
Vol 13 ◽  
pp. 117955651987052 ◽  
Author(s):  
Sara Sinno ◽  
Adel M Assaad ◽  
Nina Salem Shabb

Oropharyngeal small cell carcinomas (OPSmCC) are rare with only few case reports and case series published in the literature. More recently, an association of these tumors with human papillomavirus (HPV) infection has been detected. However, unlike oropharyngeal squamous cell carcinomas which have a better outcome when associated with HPV, OPSmCC exhibit an aggressive behavior. In this article, we report a case of tonsillar carcinoma arising in a 14-year-old boy that was associated with HPV infection. The tumor exhibited morphologic features of small cell carcinoma with no overt squamous differentiation. Yet, by immunohistochemistry, it showed diffuse and strong co-expression of both squamous and neuroendocrine markers. In addition, we present the clinicopathologic features of all the cases of OPSmCC reported in the literature for which p16 and/or HPV testing have been done.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Olivier Dupuis ◽  
Laura Delagrange ◽  
Sophie Dupuis-Girod

Abstract Background Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder that has prevalence of 1:5000 to 1:8000, and which is characterised by recurrent epistaxis, cutaneous telangiectasia, and arteriovenous malformations (AVMs) that affect many organs including the lungs, gastrointestinal tract, liver, and central nervous system. The aim here was to carry out a review of the literature on HHT complications during pregnancy in order to guide management decisions. Main body A literature review was carried out to analyse all publications on complications that occurred during pregnancy in women with HHT. The PubMed/Medline and Scopus databases were searched. The complications observed in HHT women during pregnancy were then described. The authors identified 5 case series and 31 case reports that describe the evolution of 1577 pregnancies in 630 women with HHT. The overall maternal death rate described in the case series was estimated at 1.0% of pregnancies in the case series and 2 maternal deaths occurred in 31 pregnancy case reports. Severe maternal complications occurred in 2.7 to 6.8% of pregnancies in the case series. Severe complications occurred mostly in the second and third trimester in non-diagnosed and non-screened HHT patients. Severe complications were related to visceral involvement. The most frequent complications were related to pulmonary arteriovenous malformations (PAVMs) (haemothorax (n = 10), haemoptysis (n = 4), and severe hypoxaemia (n = 3)). Neurological complications were related to PAVMs in one case (right to left shunt) and to cerebral arteriovenous malformations (CAVM) and intracranial haemorrhage in 2 cases. Complications were related to hepatic arteriovenous malformations (HAVMs) in 8 cases (acutely decompensated heart failure due to hepatic involvement (n = 1), dyspnoea related to heart failure (n = 5), and hepatobiliary necrosis (n = 2)). Conclusion Based on the literature review, most pregnancies in HHT women occur normally. However, these pregnancies should be considered high-risk, given the potential life-threatening events related to AVM rupture. Furthermore, there is currently no international consensus regarding the medical follow-up of pregnancy in women with HHT and the aim here was to carry out a review of the literature in order to guide screening and management decisions for this rare disease.


2017 ◽  
Vol 8 (2) ◽  
pp. 129-135 ◽  
Author(s):  
Anna Elizabeth Sutherland ◽  
John Curtin ◽  
Victoria Bradley ◽  
Olivia Bush ◽  
Maggie Presswood ◽  
...  

ObjectivesTo report the results of a combined case series analysis of subcutaneous levetiracetam (Keppra) for the management of seizures in palliative care patients.MethodsA comprehensive literature review on the use of subcutaneous levetiracetam was performed, and these data were combined with a prospective observational audit of its use in terminal care undertaken in a regional palliative care network.Results7 papers were identified from the literature review-four case reports and three observational case series-reporting on a total of 53 cases where subcutaneous levetiracetam was administered.We report 20 further cases of subcutaneous levetiracetam administration from a prospective observational audit. Doses ranged from 250mg to 4000 mg daily. Oral to subcutaneous conversion ratios where stated were 1:1. Levetiracetam was reported as the sole administered antiepileptic drug (AED) in eight cases, and no seizures were reported until death in five cases. Five were switched back to enteral levetiracetam. In seven cases, levetiracetam was combined with AEDs to provide seizure control at the end of life. There was one report of a sterile abscess after 25 days of continuous subcutaneous administration.ConclusionsCombined analysis of 73 reported cases of subcutaneous levetiracetam suggests this treatment may have a role in the management of seizures at the end of life. However, randomised controlled trials are urgently needed to establish the efficacy and tolerability of subcutaneous levetiracetam administration. If proven to be safe and effective, subcutaneous levetiracetam offers the potential to prevent and treat seizures without causing unnecessary sedation at the end of life.


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