Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

2021 ◽  
Author(s):  
Aïsha Sooltangos ◽  
Istvan Bodi ◽  
Prajwal Ghimire ◽  
Konstantinos Barkas ◽  
Sinan Al-Barazi ◽  
...  
Keyword(s):  
Proton Beam ◽  
Case Series ◽  
Cell Tumor ◽  
Imaging Features ◽  
Csf Rhinorrhea ◽  
Proton Beam Radiotherapy ◽  
Tertiary Referral Center ◽  
Notochord Cell ◽  
Ecchordosis Physaliphora ◽  
Imaging Surveillance

Abstract Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

P37 Do all notochordal lesions require proton beam radiotherapy? A case series of ecchordosis physaliphora

2019 ◽  
Vol 90 (3) ◽  
pp. e34.3-e33
Author(s):  
A Sooltangos ◽  
A Giamouriadis ◽  
S Barazi ◽  
K Barkas ◽  
N Thomas ◽  
...  
Keyword(s):  
Proton Beam ◽  
Clinical Course ◽  
Case Series ◽  
Proliferation Index ◽  
Csf Leak ◽  
Endoscopic Endonasal ◽  
Proton Beam Radiotherapy ◽  
Csf Fistula ◽  
Ecchordosis Physaliphora

ObjectivesEcchordosis physaliphora (EP) is a benign, usually asymptomatic, notochord tumour but may also present with a spontaneous CSF fistula. Differentiating EP from its more aggressive counterpart, chordoma is challenging but important as the clinical course and management differ significantly, with the latter requiring proton beam radiotherapy. This case series aims to further inform this discussion.DesignCase series.SubjectsPatients with suspected EP from 2015–2018.MethodsRetrospective analysis.ResultsSix patients were identified. Four presented with suspected CSF leak. MRI revealed a T1-hypointense and T2-hyperintense clival lesion without enhancement. CT showed pneumocephalus, a clival defect and no mass lesion. All underwent endoscopic endonasal repair of CSF fistula with biopsy. Histology revealed physaliphorous cells with no mitoses; immunohistochemistry confirmed notochord origin (EMA, S100, CD10 and/or MNF116) and low proliferation index. In 2 patients, EP was found incidentally on imaging and these lesions have remained static on follow-up.ConclusionsIn the absence of a clival mass, notochord remnant lesions with benign histopathology can be treated as EP and patients can avoid radiotherapy. The diagnosis of EP, however, remains challenging as no distinctive histopathological marker exists to differentiate it from chordoma and until a genetic or immunohistochemical marker is identified, the diagnosis rests solely on the presence or absence of a mass on imaging.

Steroid Cell Tumours: Rare Ovarian Tumours that Cause Hyperandrogenaemia in Postmenopausal Women

2020 ◽  
Author(s):  
xiaodan zhu ◽  
linyu zhou ◽  
jian jiang ◽  
Tian'an Jiang
Keyword(s):  
Postmenopausal Women ◽  
Clinical Manifestations ◽  
Case Series ◽  
Resistance Index ◽  
Cell Tumor ◽  
Imaging Features ◽  
Ovarian Steroid ◽  
Clinical Recurrence ◽  
Imaging Characteristics ◽  
Ovarian Tumours

Abstract Background Diagnosing hyperandrogenemia in postmenopausal women is very difficult, because it occasionally manifests as excessive hair growth or no clinical manifestations, so it is often misdiagnosed or missed diagnosis. Ovarian steroid cell tumours that cause hyperandrogenaemia in women account for approximately 0.1% of all ovarian tumours. Due to the low incidence, corresponding imaging reports are rare, so ovarian steroid cell tumours lacks typical imaging findings to differentiate it from other ovarian tumours. Therefore, we summarized its clinical and imaging characteristics through this case series ,and we also elaborated on the differential diagnosis of steroid cell tumors. We hope to help clinicians have a deeper understanding of ovarian steroid cell tumours.Case presentation we report three cases of postmenopausal women with hyperandrogenaemia.Only 1 patient showed virilization symptoms, the other two patients were completely asymptomatic. All patients underwent total hysterectomy + bilateral adnexectomy. Histological results showed one case of leyding cell tumor (case 1) and two cases of benign non-specific steroid cell tumor (case 2 and case 3). After the operation, the androgen levels of all patients returned to normal, and there was no clinical recurrence since follow-up.Conclusions Through this series of cases, we found that although virilization caused by increased serum testosterone levels is an important clinical feature of ovarian steroid cell tumors, complete asymptomatic is also one of its features. A solid, slightly hypoechoic, round or oval mass with uniform internal echo, richer blood flow in the solid part and low resistance index are typical imaging features of ovarian steroid cell tumors. Diagnosis of ovarian steroid cell tumours after menopause is challenging, but surgery can be used for both diagnosis and clear treatment.

scholarly journals Seizure Semiology, EEG, and Imaging Findings in Epilepsy Secondary to Mitochondrial Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Anthony L. Fine ◽  
Greta Liebo ◽  
Ralitza H. Gavrilova ◽  
Jeffrey W. Britton
Keyword(s):  
Status Epilepticus ◽  
Phenotypic Variability ◽  
Mitochondrial Disorder ◽  
Case Series ◽  
Mitochondrial Disorders ◽  
Imaging Features ◽  
Retrospective Case ◽  
Tertiary Referral Center ◽  
Focal Status Epilepticus ◽  
Patients With Epilepsy

Background: Identification of an underlying mitochondrial disorder can be challenging due to the significant phenotypic variability between and within specific disorders. Epilepsy can be a presenting symptom with several mitochondrial disorders. In this study, we evaluated clinical, electrophysiologic, and imaging features in patients with epilepsy and mitochondrial disorders to identify common features, which could aid in earlier identification of a mitochondrial etiology.Methods: This is a retrospective case series from January 2011 to December 2019 at a tertiary referral center of patients with epilepsy and a genetically confirmed diagnosis of a mitochondrial disorder. A total of 164 patients were reviewed with 20 patients fulfilling inclusion criteria.Results: A total of 20 patients (14 females, 6 males) aged 0.5–61 years with epilepsy and genetically confirmed mitochondrial disorders were identified. Status epilepticus occurred in 15 patients, with focal status epilepticus in 13 patients, including 9 patients with visual features. Abnormalities over the posterior cerebral regions were seen in 66% of ictal recordings and 44% of imaging studies. All the patients were on nutraceutical supplementation with no significant change in disease progression seen. At last follow-up, eight patients were deceased and the remainder had moderate-to-severe disability.Discussion: In this series of patients with epilepsy and mitochondrial disorders, we found increased propensity for seizures arising from the posterior cerebral regions. Over time, electroencephalogram (EEG) and imaging abnormalities increasingly occurred over the posterior cerebral regions. Focal seizures and focal status epilepticus with visual symptoms were common. Additional study is needed on nutraceutical supplementation in mitochondrial disorders.

Complications after Proton Beam Therapy to Skull Base Tumors: Case Series

2019 ◽  
Author(s):  
Gustavo Rangel ◽  
Mostafa Shahein ◽  
Thiago Felicio ◽  
Guilhermo Malave ◽  
Nyall London ◽  
...  
Keyword(s):  
Skull Base ◽  
Proton Beam ◽  
Case Series ◽  
Proton Beam Therapy ◽  
Skull Base Tumors

Evaluation and treatment of children with radiation-induced cerebral vasculopathy

2019 ◽  
Vol 24 (6) ◽  
pp. 680-688
Author(s):  
David S. Hersh ◽  
Kenneth Moore ◽  
Vincent Nguyen ◽  
Lucas Elijovich ◽  
Asim F. Choudhri ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Proton Beam ◽  
Tumor Imaging ◽  
Clinic Visit ◽  
Surgical Patient ◽  
Beam Radiation ◽  
Proton Beam Radiotherapy ◽  
Cranial Radiation ◽  
Delayed Complication ◽  
Radiation Induced

OBJECTIVEStenoocclusive cerebral vasculopathy is an infrequent delayed complication of ionizing radiation. It has been well described with photon-based radiation therapy but less so following proton-beam radiotherapy. The authors report their recent institutional experience in evaluating and treating children with radiation-induced cerebral vasculopathy.METHODSEligible patients were age 21 years or younger who had a history of cranial radiation and subsequently developed vascular narrowing detected by MR arteriography that was significant enough to warrant cerebral angiography, with or without ischemic symptoms. The study period was January 2011 to March 2019.RESULTSThirty-one patients met the study inclusion criteria. Their median age was 12 years, and 18 (58%) were male. Proton-beam radiation therapy was used in 20 patients (64.5%) and photon-based radiation therapy was used in 11 patients (35.5%). Patients were most commonly referred for workup as a result of incidental findings on surveillance tumor imaging (n = 23; 74.2%). Proton-beam patients had a shorter median time from radiotherapy to catheter angiography (24.1 months [IQR 16.8–35.4 months]) than patients who underwent photon-based radiation therapy (48.2 months [IQR 26.6–61.1 months]; p = 0.04). Eighteen hemispheres were revascularized in 15 patients. One surgical patient suffered a contralateral hemispheric infarct 2 weeks after revascularization; no child treated medically (aspirin) has had a stroke to date. The median follow-up duration was 29.2 months (IQR 21.8–54.0 months) from the date of the first catheter angiogram to last clinic visit.CONCLUSIONSAll children who receive cranial radiation therapy from any source, particularly if the parasellar region was involved and the child was young at the time of treatment, require close surveillance for the development of vasculopathy. A structured and detailed evaluation is necessary to determine optimal treatment.

Imaging Features of Primary Tumors of the Hand

2020 ◽  
Vol 16 ◽  
Author(s):  
Filippo Boriani ◽  
Edoardo Raposio ◽  
Costantino Errani
Keyword(s):  
Soft Tissue ◽  
Soft Tissues ◽  
Epithelioid Sarcoma ◽  
Case Series ◽  
Malignant Neoplasm ◽  
Benign Tumors ◽  
Imaging Features ◽  
Primary Tumors ◽  
Therapeutic Decisions ◽  
Tumors Of The Hand

: Musculoskeletal tumors of the hand are a rare entity and are divided into skeletal and soft tissue tumors. Either category comprises benign and malignant or even intermediate tumors. Basic radiology allows an optimal resolution of bone and related soft tissue areas, ultrasound and more sophisticated radiologic tools such as scintigraphy, CT and MRI allow a more accurate evaluation of tumor extent. Enchondroma is the most common benign tumor affecting bone, whereas chondrosarcoma is the most commonly represented malignant neoplasm localized to hand bones. In the soft tissues ganglions are the most common benign tumors and epithelioid sarcoma is the most frequently represented malignant tumor targeting hand soft tissues. The knowledge regarding diagnostic and therapeutic management of these tumors is often deriving from small case series, retrospective studies or even case reports. Evidences from prospective studies or controlled trials are limited and for this lack of clear and supported evidences data from the medical literature on the topic are controversial, in terms of demographics, clinical presentation, diagnosis prognosis and therapy.The correct recognition of the specific subtype and extension of the tumor through first line and second line radiology is essential for the surgeon, in order to effectively direct the therapeutic decisions.

scholarly journals GCT-08. PROTON BEAM RADIOTHERAPY FOR PEDIATRIC AND YOUNG-ADULT PATIENTS WITH INTRACRANIAL GERM CELL TUMOR

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii329-iii329
Author(s):  
Minako Sugiyama ◽  
Takayuki Hashimoto ◽  
Takashi Mori ◽  
Kazuya Hara ◽  
Yukayo Terashita ◽  
...  
Keyword(s):  
Young Adult ◽  
Adverse Events ◽  
Germ Cell ◽  
Cell Count ◽  
Proton Beam ◽  
Adult Patients ◽  
Short Term ◽  
Intracranial Germ Cell Tumor ◽  
X Ray ◽  
Proton Beam Radiotherapy

Abstract BACKGROUND To reduce treatment-related adverse events in pediatric and young-adult patients with brain tumors, proton beam radiotherapy (PBT) has recently been performed instead of conventional X-ray radiotherapy. However, whether PBT is as effective as X-ray radiotherapy has not been sufficiently investigated, especially in patients receiving whole-ventricular irradiation. METHODS We report a retrospective observation of 15 patients with intracranial germ cell tumors (GCT), who received PBT at our institution from April 2014 to September 2019. We evaluated their clinical course, short-term adverse events, and prognosis. RESULTS/ CONCLUSION Fifteen patients (9 males and 6 females; median age 13 years) who received PBT following induction chemotherapy were analyzed. Nine patients received 23.4–27.0 GyE of whole-ventricular irradiation due to GCT in the pituitary gland, pineal body, or hypothalamic area. Three patients received 23.4 GyE of whole-brain irradiation: one of them had boost irradiation for basal ganglia. Three patients received 30.6 GyE of craniospinal irradiation (CSI). Six of the 15 patients experienced nausea (grade 2, according to the CTCAE version 4.0). Four patients, including two who received CSI, showed myelosuppression: decrease in white blood cell count, lymphocyte cell count, and neutrophil count (grade 3). No other severe short-term adverse events of >grade 2 was observed in any of the patients. At a median follow-up of 21 months (2-62 months) after irradiation. all patients are alive without recurrence. Our results may be encouraging and further investigations with a larger scale is warranted.

Management of optic disc pit-associated maculopathy: A case series from a tertiary referral center

2021 ◽  
pp. 112067212110237
Author(s):  
Ilkay Kilic Muftuoglu ◽  
Ecem Onder Tokuc ◽  
V Levent Karabas
Keyword(s):  
Optic Disc ◽  
Case Series ◽  
Internal Limiting Membrane ◽  
Maximum Height ◽  
Optic Disc Pit ◽  
Tertiary Referral Center ◽  
Number Of Patients ◽  
The Mean ◽  
Foveal Center

Purpose: To report outcomes of pars plana vitrectomy (PPV) combined with internal limiting membrane (ILM) stuffing technique in patients with optic disc pit associated maculopathy (ODP-M). Methods: Data including best-corrected visual acuity (BCVA), central macular thickness (CMT), foveal center point thickness (FCP), and maximum height of fluid (max_fluid) (intraretinal or subretinal) were collected from the medical records of the patients. Results: Six eyes of six patients with a mean age of 28.0 ± 17.68 years (range: 9–53 year) underwent PPV + ILM plug surgery. The mean follow-up duration was 25.62 ± 26.11 months (range: 11.80–78.00 month) duration. The mean BCVA increased from 1.25 ± 1.04 logMAR (20/355, Snellen equivalent) to 0.86 ± 1.09 logMAR (20/144, Snellen equivalent) at last follow-up ( p = 0.043). Compared to baseline, CMT, FCP, and max_fluid significantly decreased at all visits after the surgery ( p < 0.05 for all visits). At last follow-up, 66.6% of the eyes (four eyes) showed complete resolution of fluid at a mean of 5.25 ± 4.99 months (range: 1–12 months) after the surgery. Conclusion: PPV with ILM plug seemed to be an effective surgical technique in ODP-M. Studies with longer follow-up and higher number of patients are needed to confirm our results.

scholarly journals ARE SPORTS INJURIES THE MOST COMMON CAUSE OF MOREL-LAVALLEE LESIONS IN THE PEDIATRIC POPULATION?

2021 ◽  
Vol 9 (7_suppl3) ◽  
pp. 2325967121S0002
Author(s):  
Indranil Kushare ◽  
Ramesh Babu Ghanta ◽  
Nicole A. Wunderlich
Keyword(s):  
Lower Extremity ◽  
Conservative Management ◽  
Sports Injuries ◽  
Pediatric Population ◽  
Case Series ◽  
Lower Leg ◽  
Functional Score ◽  
Imaging Features ◽  
Associated Injuries ◽  
Common Cause

Background: Traumatic internal degloving injury i.e. Morel-Lavallée lesion (MLL) develops as a result of blunt trauma with tangential shear forces. In the pediatric population, these have been described only as small case reports till date. Purpose: To describe the largest case series of lower extremity Morel-Lavallée lesion (MLL) in the pediatric population, to determine its etiology (especially its relationship to sports), treatment and outcomes. Methods: Retrospective study of patients <18 years presenting to a tertiary children’s hospital with MLL between 2013-2019. Demographics, clinical data, imaging features, treatment and outcomes data was collected. Descriptive statistical analysis was conducted. Results: 38 patients (21 males, 17 females) having MLL with mean age 14.6 years were classified into 2 groups- hip/thigh MLL (9 patients) and lower leg MLL (29) (Table 1.1). Most common cause of lower leg MLL was sports injury (79%). Most commonly implicated sports in MLL were football and baseball(24% each). Associated injuries were seen in 9 (23%) patients. Radiographs done in 58% of patients showed no bony abnormalities. Imaging modality of choice was MRI for lower leg MLLs (72%) and US for hip/thigh MLL (67%)(Fig.1.2). MLL size was larger for hip/thigh MLL (168 cm3) as compared to leg MLL (38.6 cm3). 25 (65.8%) of MLLs were treated with conservative management,12 (31.5%) with minimally invasive methods and 1(2.6%) needed surgical management. 2/9 (22.2%) of the associated injuries required operative management. 2/38 (5%) patients were given prophylactic antibiotics. 72.4% Lower leg MLLs were treated with conservative management while intervention was needed more in hip/thigh MLLs (55.6%). Patients returned to activities at 14.3 weeks for hip/thigh MLL;9.1 weeks for lower leg MLL. 24 patients with adequate documentation demonstrated lower extremity functional score (LEFS) and pain level of 74/80 (92.5%) and 0.7/10 respectively at mean 12.5 months follow-up. Leg cellulitis was seen as a complication in one patient. Conclusion: The largest case series on MLL exclusively in the pediatric population suggests that they are more common in knee/leg region region and usually caused by sports injuries which is notably different as compared to adult population. Most pediatric injuries are treated conservatively, especially sports related MLLs. Although return to activities takes longer for hip/thigh injuries, majority of patients regain satisfactory functionality post-injury. [Table: see text][Figure: see text]

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