scholarly journals Diaphyseal osteomyelitis of femur with suspected ewing sarcoma in 8 years old children: a case report

Author(s):  
Panji Sananta ◽  
Hidayat Rizal

Metaphyseal osteomyelitis in children due to direct bone trauma or vascular insufficiency is a frequent problem in orthopaedic surgery. In contrast, diaphyseal osteomyelitis represents a rare entity that almost exclusively affects child with bony infarct in sickle cell anemia. Differentiating neoplasm from musculoskeletal infection can sometimes be very challenging. In particular, Ewing sarcoma can masquerade as osteomyelitis with the presenting symptoms of fever, localized bone pain, and elevated inflammatory markers common to both entities, although osteomyelitis is a totally different type of disease. In this article, we report the case of chronic osteomyelitis of the femur in an immunocompetent and otherwise healthy 8 years old boy with minor inflammation signs and misleading clinical features. The X-ray showed onion skin periosteal reaction. We evacuated about 200 cc of abscess. Biopsy report revealed polimononuclear, mononuclear, and necrotic inflammatory cells. The patient was treated with antibiotic for two weeks and discharged with improved general condition. Six months follow-up shows clinical and radiological improvement. The diagnosis had to be confirmed by surgery which allowed the initiation of a targeted therapy. A case of diaphyseal osteomyelitis of a femur, lacking predisposing factors or trauma, is unique in children and never been reported previously.

2021 ◽  
pp. 1-11
Author(s):  
Michael J. Gigliotti ◽  
Christine Mau ◽  
Charles S. Specht ◽  
Cynthia Lawson ◽  
Sarah McNutt ◽  
...  

OBJECTIVE The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.


Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.


Cancers ◽  
2021 ◽  
Vol 13 (1) ◽  
pp. 153
Author(s):  
Sabrina Daniela da Silva ◽  
Fabio Albuquerque Marchi ◽  
Jie Su ◽  
Long Yang ◽  
Ludmila Valverde ◽  
...  

Invasive oral squamous cell carcinoma (OSCC) is often ulcerated and heavily infiltrated by pro-inflammatory cells. We conducted a genome-wide profiling of tissues from OSCC patients (early versus advanced stages) with 10 years follow-up. Co-amplification and co-overexpression of TWIST1, a transcriptional activator of epithelial-mesenchymal-transition (EMT), and colony-stimulating factor-1 (CSF1), a major chemotactic agent for tumor-associated macrophages (TAMs), were observed in metastatic OSCC cases. The overexpression of these markers strongly predicted poor patient survival (log-rank test, p = 0.0035 and p = 0.0219). Protein analysis confirmed the enhanced expression of TWIST1 and CSF1 in metastatic tissues. In preclinical models using OSCC cell lines, macrophages, and an in vivo matrigel plug assay, we demonstrated that TWIST1 gene overexpression induces the activation of CSF1 while TWIST1 gene silencing down-regulates CSF1 preventing OSCC invasion. Furthermore, excessive macrophage activation and polarization was observed in co-culture system involving OSCC cells overexpressing TWIST1. In summary, this study provides insight into the cooperation between TWIST1 transcription factor and CSF1 to promote OSCC invasiveness and opens up the potential therapeutic utility of currently developed antibodies and small molecules targeting cancer-associated macrophages.


Neurosurgery ◽  
2010 ◽  
Vol 67 (2) ◽  
pp. 404-407 ◽  
Author(s):  
R. Shane Tubbs ◽  
Christoph J. Griessenauer ◽  
Todd Hankinson ◽  
Curtis Rozzelle ◽  
John C. Wellons ◽  
...  

Abstract BACKGROUND Retroclival epidural hematomas (REDHs) are infrequently reported. To our knowledge, only 19 case reports exist in the literature. OBJECTIVE This study was performed to better elucidate this pathology. METHODS We prospectively collected data for all pediatric patients diagnosed with REDH from July 2006 through June 2009. Data included mechanism of injury, Glasgow Coma Scale score, neurological examination, treatment modality, and outcome. Magnetic resonance imaging was used to measure REDH dimensions. RESULTS Eight children were diagnosed with REDH, and the hematomas were secondary to motor vehicle–related trauma in all cases. The mean age of patients was 12 years (range 4–17 years). The mean REDH height (craniocaudal) was 4.0 cm, and the mean thickness (dorsoventral) was 1.0 cm. At presentation, the mean Glasgow Coma Scale score was 8 (range 3–14), and there was no correlation between hematoma size and presenting symptoms. Two patients died soon after injury, and 2 additional patients had atlanto-occipital dislocation that required surgical intervention. No patient underwent surgical evacuation of the REDH. The mean follow-up was 14 months. At most recent follow-up, 4 patients are neurologically intact, 1 patient has a complete spinal cord injury, and 1 patient has mild bilateral abducens nerve palsy. CONCLUSION To our knowledge, this study of 8 pediatric patients is the largest series of patients with REDH thus far reported. Based on our study, we found that REDH is likely to be underdiagnosed, atlanto-occipital dislocation should be considered in all cases of REDH, and many patients with REDH will have minimal long-term neurological injury.


2009 ◽  
Vol 65 (suppl_6) ◽  
pp. ons100-ons109 ◽  
Author(s):  
Giuseppe Mirone ◽  
Salvatore Chibbaro ◽  
Luigi Schiabello ◽  
Serena Tola ◽  
Bernard George

Abstract Objective: En plaque sphenoid wing meningiomas are complex tumors involving the sphenoid wing, the orbit, and sometimes the cavernous sinus. Complete removal is difficult, so these tumors have high rates of recurrence and postoperative morbidity. The authors report a series of 71 patients with sphenoid wing meningiomas that were managed surgically. Methods: The clinical records of 71 consecutive patients undergoing surgery for sphenoid wing meningiomas at Lariboisière Hospital, Paris, were prospectively collected in a database during a 20-year period and analyzed for presenting symptoms, surgical technique, clinical outcome, and follow-up. Results: Among the 71 patients (mean age, 52. 7 years; range, 12–79 years), 62 were females and 9 were males. The most typical symptoms recorded were proptosis in 61 patients (85.9%), visual impairment in 41 patients (57.7%), and oculomotor paresis in 9 patients (12.7%). Complete removal was achieved in 59 patients (83%). At 6 months of follow-up, magnetic resonance imaging scans revealed residual tumor in 12 patients (9 in the cavernous sinus and 3 around the superior orbital fissure). Mean follow-up was 76.8 months (range, 12–168 months). Tumor recurrence was recorded in 3 of 59 patients (5%) with total macroscopic removal. Among the patients with subtotal resection, tumor progression was observed in 3 of 12 patients (25%; 2 patients with grade III and 1 patient with grade IV resection). Mean time to recurrence was 43.3 months (range, 32–53 months). Conclusion: Surgical management of patients with sphenoid wing meningiomas cannot be uniform; it must be tailored on a case-by-case basis. Successful resection requires extensive intra- and extradural surgery. We recommend optic canal decompression in all patients to ameliorate and/or preserve visual function.


Neurosurgery ◽  
2009 ◽  
Vol 65 (5) ◽  
pp. 914-918 ◽  
Author(s):  
Aftab A. Khan ◽  
Ajay Niranjan ◽  
Hideyuki Kano ◽  
Douglas Kondziolka ◽  
John C. Flickinger ◽  
...  

Abstract OBJECTIVE Hemangiomas are rare but highly vascular tumors that may develop in the cavernous sinus or orbit. These tumors pose diagnostic as well as therapeutic challenges to neurosurgeons during attempted removal. We analyzed our increasing experience using stereotactic radiosurgery (SRS). METHODS Eight symptomatic patients with hemangiomas underwent SRS between 1988 and 2007. The presenting symptoms included headache, orbital pain, diplopia, ptosis, proptosis and impaired visual acuity. The hemangiomas were located in either the cavernous sinus (7 patients) or the orbit (1 patient). Four patients underwent SRS as primary treatment modality based on clinical and imaging criteria. Four patients had previous microsurgical partial excision or biopsy. The median target volume was 6.8 mL (range, 2.5–18 mL). The median prescription dose delivered to the margin was 14.5 Gy (range, 12.5–19 Gy). The dose to the optic nerve in all patients was less than 9 Gy (range, 4.5–9 Gy). RESULTS The median follow-up period after SRS was 80 months (range, 40–127 months). Six patients had symptomatic improvement; 2 patients reported persistent diplopia. Follow-up imaging revealed tumor regression in 7 patients and no change in tumor volume in 1 patient. All the patients improved after SRS. CONCLUSION Our extended experience confirms that SRS is an effective management strategy for symptomatic intracavernous and intraorbital hemangiomas. Our study is the first long-term report on the safety and efficacy of SRS.


2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Murat Tuğrul Eren ◽  
Hakan Özveri ◽  
Hilal Kurtoğlu

Abstract Background Penile Mondor’s disease (PMD) is thrombophlebitis of the superficial dorsal vein of the penis. Following the occurrence of thrombotic events in the affected veins, the lumen often becomes occluded with fibrin and inflammatory cells. A hyper-coagulative state is one of the underlying causes although most cases of PMD are idiopathic. Coronavirus disease-2019 infection (COVID-19) is associated with frequent thrombotic events. Inflammation and thrombosis play a central role in the course and outcome of COVID-19, which can predispose to both venous and arterial thromboembolism. In this report, we present a 33-year-old male patient diagnosed with PMD during the subacute phase of COVID-19 infection while on prophylactic antithrombotic treatment. Case Presentation A 33-year-old male patient was diagnosed as PMD which occurred during the subacute phase of COVID-19 infection, while he was on active treatment of COVID-19 by prophylactic antithrombotic Rivaroxaban 15 mg therapy and curative antiviral medication. There was no recent sexual intercourse or trauma to the genitals. His PCR test for COVID-19 had become negative, and antibody test was positive at the time of his PMD’s onset. Rivaroxaban was replaced by Enoxaparin (8000 IU/0.8 ml.), a low molecular weight heparin administered subcutaneously and twice daily. On the third day of this medication, all coagulative measurements returned to normal. PMD disappeared in the second week. Conclusion Low-dose Rivaroxaban 15 mg is not safe for some COVID-19-associated thromboembolism prophylaxis, and careful follow-up is critical due to the possibility of a wide range of pathologic thrombotic manifestations in COVID-19 infection.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11569-11569
Author(s):  
Edwards Kasonkanji ◽  
Yolanda Gondwe ◽  
Morgan Dewey ◽  
Joe Gumulira ◽  
Matthew Painschab ◽  
...  

11569 Background: Kaposi sarcoma (KS) is the leading cancer in Malawi (34% of cancers). Outside of clinical trials, prospective KS studies from sub-Saharan Africa (SSA) are few and limited by loss to follow up. We conducted a prospective KS cohort study of standard of care bleomycin/vincristine (BV) at Lighthouse HIV clinic, in Lilongwe, Malawi. Methods: We enrolled pathologically confirmed, newly diagnosed, HIV+ KS patients from Feb 2017 to Jun 2019. We collected clinical and treatment characteristics, toxicity, and outcomes of KS with follow-up censored Jun 2020. Patients were treated with bleomycin (25 mg/m2) and vincristine (0.4 mg/m2) every 14 days for a planned maximum of 16 cycles. STATA v13.0 was used to calculate descriptive statistics and Kaplan Meier survival analysis. Toxicity was graded using NCI CTCAE v5.0. Results: We enrolled 138 participants, median age 36 (IQR 32-44) and 110 (80%) male. By ACTG staging, 107 (78%) were T1 (tumour severity), 46 (33%) were S1 (illness severity) and 46 (33%) had Karnofsky performance status ≤70. Presenting symptoms included edema in 69 (53%), visceral disease in 9 (7%), and oral involvement in 43 (33%). Prior to KS diagnosis, 70 (51%) participants were aware of being HIV+ for median 17 months (IQR 6-60) and had been on ART for median 16 months (IQR 6-60). Median CD4 count was 197 (IQR 99-339), median HIV-viral load was 2.6 log copies/mL (IQR 1.6 – 4.8) and 57% were HIV-suppressed ( < 1000 HIV copies/ml). The median number of cycles was 16 (IQR 7-16). 62 (45%) participants missed at least one dose due to stock out. Amongst patients with missed doses, the median number was 3 (IQR 2-4) for bleomycin and 2 (IQR 1-3) for vincristine. 14 (10%) participants experienced at least one reduced dose due to toxicity. 5 (4%) participants suffered grade ≥3 anaemia, 13 (9%) grade ≥3 neutropenia, and one participant had grade 4 bleomycin-induced dermatitis. There was no reported grade ≥3 bleomycin lung toxicity or vincristine-induced neuropathy. Of 115 evaluable participants, responses at the end of therapy were: complete response in 52 (45%), partial response in 27 (23%) stable disease in 5 (4%), and progressive disease in 31 (28%). Median duration of follow-up was 20 months. At censoring, 69 (50%) were alive, 36 (26%) dead, and 33 (24%) lost to follow-up. Overall survival is shown Table as crude and worst-case scenario; worst-case assumes all participants lost to follow up died. Conclusions: Here, we present one of the most complete characterizations of KS presentation and treatment from SSA. As in other studies from the region, the majority of patients presented with advanced disease, chemotherapy stock-outs and loss to follow up were common, and mortality was high. Studies are planned to understand the virologic characteristics, improve therapies, and better implement existing therapies.[Table: see text]


2021 ◽  
pp. 112067212110528
Author(s):  
Lan Zhou ◽  
Juanjuan Wang ◽  
Guihua Xu ◽  
Dingding Wang ◽  
Xiaoyi Wang ◽  
...  

Purpose To describe an atypical nodular episcleritis mimicking a solitary giant episcleral mass, which is not attributed to any systemic diseases and identified only after immunohistochemical examination. Case report A sixty-year-old Chinese woman with systemic hypertension presented with 6-month history of giant, solitary and redness epibulbar mass arising from the superior aspect of her left eye. The lesion gradually enlarged, even with 6-month history of irregular topical steroid eye drops treatment. Imaging studies and laboratory test revealed a 10 mm × 8 mm episcleral mass absence of any infection indicator and autoimmune antibody changes. The mass was completely removed before its extension through the deep scleral, histopathologic examination revealed a nodular episcleritis composed of various chronic inflammatory cells infiltration. Topical steroid eye drops treatment combined with oral steroidal anti-inflammatory drugs was then administrated regularly for 1 month, and no recurrence occurred after 1-year follow-up. Conclusion Nodular anterior episcleritis is characterized by underlying chronic inflammation of the anterior episclera and can be presented as asymptomatic episcleral mass. Besides a thorough investigation systemically, tissue biopsy is required for definite diagnosis.


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