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Otology & Neurotology : Official... Apr 2024To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in this population through a retrospective cohort review, systematic review, and meta-analysis.
DATABASES REVIEWED
PubMed, Cochrane Library, and Embase.
METHODS
We retrospectively reviewed all CI cases after head and neck (HN) XRT at our institution, noting intraoperative findings, postoperative complications, and hearing outcomes. Change in speech discrimination scores (SDSs) was the primary outcome measure. Systematic review was performed to identify all cases of CI after HNXRT. A meta-analysis was performed to assess SDS change.
RESULTS
The retrospective cohort review identified 12 patients who underwent CI after HNXRT. One patient with HN cancer (HNC) and one with central nervous system pathology (CNSP) received bilateral implants. Six had HNC, three had CNSP, and one had Langerhans cell histiocytosis. Eleven had abnormal findings during CI. There were no postoperative complications. Twenty articles with an additional 97 patients were suitable for systematic review inclusion. Of the 109 patients, 67 (61.5%) had HNC and 18 (16.5%) had CNSP. Abnormal intraoperative findings were common (30.3%), most frequently in the mastoid (66.7%). Postoperative complications, including wound dehiscence and infection with some requiring explantation, occurred in 10.1% of patients. Sixty-six patients were included in the meta-analysis. All demonstrated SDS improvement (mean increase, 56.2%).
CONCLUSION
Patients with prior HNXRT benefit from CI. Paying careful attention to surgical planning and technique, postoperative care, and patient expectations is imperative, as complications are not uncommon.
Topics: Humans; Cochlear Implantation; Retrospective Studies; Hearing Loss, Sensorineural; Cochlear Implants; Postoperative Complications; Speech Perception
PubMed: 38361317
DOI: 10.1097/MAO.0000000000004127 -
Rare Tumors 2013We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches,...
We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.
PubMed: 24179650
DOI: 10.4081/rt.2013.e38 -
JAMA Dermatology Mar 2020Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or...
IMPORTANCE
Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or lymphoproliferative disorders. Despite the morbidity of NXG, the literature is limited to case reports and small studies, and diagnostic criteria are lacking.
OBJECTIVE
To evaluate the characteristics of NXG and propose diagnostic criteria.
DESIGN, SETTING, AND PARTICIPANTS
This multicenter cross-sectional study was conducted at tertiary academic referral centers and followed by a systematic review and a consensus exercise. The multicenter cohort included patients with NXG diagnosed at the Brigham and Women's and Massachusetts General Hospitals (2000-2018), the University of Iowa Hospitals and Clinics (2000-2018), and the University of Pennsylvania Health System (2008-2018). The systematic review was conducted in 2018 and included patients with NXG identified in the Cochrane, Ovid EMBASE, PubMed, and Web of Science databases. The consensus exercise was conducted by 8 board-certified dermatologists to identify diagnostic criteria.
MAIN OUTCOMES AND MEASURES
Demographic factors, comorbidities, clinical features, and treatment response.
RESULTS
Of 235 included patients with NXG (34 from the multicenter cohort and 201 from the systematic review results), the mean (SD) age at presentation was 61.6 (14.2) years; 147 (62.6%) were female. Paraproteinemia was detected in 193 patients (82.1%), most often IgG-κ (117 patients [50.0%]). A malignant condition was detected in 59 patients (25.1%), most often multiple myeloma (33 patients [14.0%]). The overall rate of paraproteinemia and/or a malignant condition was 83.8% (197 patients). In the multicenter cohort, evolution of paraproteinemia into multiple myeloma was observed up to 5.7 years (median [range], 2.4 [0.1-5.7] years) after NXG presentation. Cutaneous lesions consisted of papules, plaques, and/or nodules, typically yellow or orange in color (113 of 187 [60.4%]) with a periorbital distribution (130 of 219 [59.3%]). The eye was the leading site of extracutaneous involvement (34 of 235 [14.5%]). In the multicenter cohort, intravenous immunoglobulin had the best treatment response rate (9 of 9 patients [100%]), followed by antimalarial drugs (4 of 5 patients [80%]), intralesional triamcinolone (6 of 8 patients [75%]), surgery (3 of 4 patients [75%]), chemotherapy (8 of 12 patients [67%]), and lenalidomide or thalidomide (5 of 8 patients [63%]). The consensus exercise yielded 2 major criteria, which were (1) clinical and (2) histopathological features consistent with NXG, and 2 minor criteria, consisting of (1) paraproteinemia, plasma-cell dyscrasia, and/or other associated lymphoproliferative disorder and (2) periorbital distribution of cutaneous lesions. In the absence of foreign body, infection, or another identifiable cause, fulfillment of both major and at least 1 minor criterion were proposed to establish the diagnosis of NXG.
CONCLUSIONS AND RELEVANCE
Necrobiotic xanthogranuloma is a multisystem disorder associated with paraproteinemia and malignant conditions. The proposed diagnostic criteria may advance clinical research and should be validated.
Topics: Aged; Cohort Studies; Cross-Sectional Studies; Female; Humans; Male; Middle Aged; Multiple Myeloma; Necrobiotic Xanthogranuloma; Paraproteinemias; Retrospective Studies
PubMed: 31940000
DOI: 10.1001/jamadermatol.2019.4221 -
World Neurosurgery Jun 2021A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment.
BACKGROUND
A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment.
OBJECTIVE
This study aims to evaluate the feasibility, safety, efficacy, and complication profile of stereotactic radiosurgery for intracranial histiocytosis.
METHODOLOGY
The authors reviewed PubMed, Scopus, Web of Science, and Embase for "radiosurgery" and "histiocytosis" in the English/Japanese language following preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The patient profile, radiosurgical parameters (dose and isodose), target volume, and mode of radiosurgery (Gamma knife, LINAC radiosurgery, etc.) were collected. Its use as primary or adjuvant therapy, clinical and radiological outcome was also evaluated.
RESULTS
We identified 7 studies (9 patients); mean age: 41.9 years (24-57 years). Six patients received Gamma-knife radiosurgery, whereas 3 received CyberKnife radiosurgery. The Langerhans cell histiocytosis variants were eosinophilic granuloma in 3, whereas 4 were not defined. Two cases had Rosai-Dorfman disease, and 2 different yet pathogenetically related histiocytic disorders. Four patients harbored lesions in the pituitary stalk and posterior pituitary, 2 patients in the petrous region, 1 patient had a pontine lesion, and 2 patients had multiple lesions. The dose delivered ranged from 8 to 28 Gy. A total of 18 lesions (9 patients) were followed for 81.67 patient-years: 7 (39%) disappeared, 8 (44.4%) showed radiological reduction, and 2 (11%) remained stable. One lesion (5%) showed an increase in size needed surgical excision. There were no adverse effects.
CONCLUSION
The role of stereotactic radiosurgery needs to be further evaluated as the current cohort with only 9 cases (2 are Rosai-Dorfman disease) is insufficient to make conclusions. It may be a viable alternative in localized disease, along with chemotherapy and targeted surgery.
Topics: Adult; Female; Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Male; Middle Aged; Radiosurgery; Treatment Outcome; Young Adult
PubMed: 33744421
DOI: 10.1016/j.wneu.2021.03.047 -
The Journal of Clinical Endocrinology... Jan 2022Pituitary stalk thickening (PST) is often identified on magnetic resonance imaging (MRI), either incidentally or during diagnostic workup of hypopituitarism. However,... (Meta-Analysis)
Meta-Analysis
CONTEXT
Pituitary stalk thickening (PST) is often identified on magnetic resonance imaging (MRI), either incidentally or during diagnostic workup of hypopituitarism. However, the neoplastic etiology and natural course of PST are not fully understood, although this knowledge is required to establish diagnostic and surveillance strategies.
OBJECTIVE
This work aimed to investigate the neoplastic etiology and natural course of PST.
METHODS
MEDLINE/PubMed and EMBASE databases were searched up to February 2021 to identify original research investigating the etiologies of PST. The proportion of neoplastic etiology in patients with PST was meta-analytically pooled. Supplementary analysis exploring factors suggesting neoplasm was also performed. For initially indeterminate cases without confirmed diagnosis, the proportion of patients showing progression of PST during follow-up was evaluated.
RESULTS
Eighteen studies covering 1368 patients with PST were included. The pooled proportion of neoplasm was 45.2% (95% CI, 33.3%-57.8%), with substantial heterogeneity across studies (I2 = 93%). The most common neoplasm was germ cell tumor (14.0% of study population), followed by Langerhans cell histiocytosis (10.2%) and metastasis (4.7%). The studies on pediatric populations and those with more than 50% of patients having at least one pituitary hormone deficiency tended to show a higher proportion of neoplasm. The pituitary stalk was thicker in neoplasms, but the difference was not significant (pooled mean difference, 2.08 mm; P = .08). In initially indeterminate cases, 18.5% (95% CI, 7.6%-38.3%) showed progression of PST during follow-up.
CONCLUSION
PST was commonly confirmed to be neoplastic, especially in pediatric populations. As isolated PST frequently progresses, follow-up imaging is essential in initially indeterminate cases.
Topics: Diagnosis, Differential; Disease Progression; Follow-Up Studies; Humans; Hypopituitarism; Incidental Findings; Magnetic Resonance Imaging; Organ Size; Pituitary Gland; Pituitary Neoplasms
PubMed: 34614160
DOI: 10.1210/clinem/dgab732 -
Oral Surgery, Oral Medicine, Oral... Jan 2022The purpose of this study was to describe an unusual case of intraoral juvenile xanthogranuloma (JXG) and to perform a systematic review to investigate the available... (Review)
Review
OBJECTIVE
The purpose of this study was to describe an unusual case of intraoral juvenile xanthogranuloma (JXG) and to perform a systematic review to investigate the available literature regarding oral JXGs.
STUDY DESIGN
We present a new case of oral JXG arising in a 36-year-old Italian woman and conducted a systematic literature review in PubMed, Web of Science, and Scopus, according to the PRISMA guidelines.
RESULTS
Our review of the English-language literature yielded 34 cases of oral JXG, which included our case report.
CONCLUSIONS
JXG is a non-Langerhans cell histiocytosis. Oral JXG has been reported, but it is a rare manifestation. Because of the rarity of oral lesions and possible variations in the clinical and histologic presentation, the correct diagnosis can be challenging, requiring a careful clinical and histopathologic evaluation with adjuvant immunohistochemical studies.
Topics: Adult; Female; Humans; Mouth Diseases; Xanthogranuloma, Juvenile
PubMed: 34736900
DOI: 10.1016/j.oooo.2021.09.015 -
Journal of International Society of... 2020This study aimed to review the success or remission of intralesional medicaments in the management of intraosseous lesions in the oral cavity.
AIMS AND OBJECTIVES
This study aimed to review the success or remission of intralesional medicaments in the management of intraosseous lesions in the oral cavity.
MATERIALS AND METHODS
A comprehensive search was performed in two databases (PubMed and Scopus). Research articles, case reports, case series, and clinical trials were included. Review articles, lesions not involving the bone, incomplete reporting, any other treatment other than intralesional medicaments to treat intraosseous bone lesions, publications without any treatment, and letter to editor were excluded. Data on remission (complete, partial, or no remission), details and regimen of the intervention, number of participants, and follow-up in months were recorded.
RESULTS
A total of 653 publications were available for title and abstract screening after the removal of duplicates. Seven articles were excluded, which were not in English. After title and abstract screening, a total of 88 publications were available for full-text screening. Fifty-five articles were included in qualitative synthesis. A total of 168 patients from 55 publications were evaluated. Minimum follow-up was 1 month and maximum was 264 months. More than two-third ( = 38) of the publications were case reports on single patient. More than two-third ( = 38) of the publications had complete remission.
CONCLUSION
Intralesional medications have shown variable success rates. Extensive lesions may undergo intralesional medications followed by surgical management.
PubMed: 32181219
DOI: 10.4103/jispcd.JISPCD_295_19 -
Neurosurgery Oct 2019Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these...
BACKGROUND
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses.
OBJECTIVE
To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation.
METHODS
Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors' institution since January 1, 1996 was also performed to supplement these data.
RESULTS
Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%).
CONCLUSION
Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and/or surgical debulking to relieve mass effect.
Topics: Adrenal Cortex Hormones; Adult; Erdheim-Chester Disease; Female; Humans; Male; Middle Aged; Radiosurgery; Retrospective Studies; Skull Base
PubMed: 30828728
DOI: 10.1093/neuros/nyz027 -
Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review.Cardiology Research Apr 2020Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic xanthogranulomatous infiltration. We described the case of a female...
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic xanthogranulomatous infiltration. We described the case of a female adult presenting with pericardial effusion. Pericardial infiltration is the most frequent cardiac manifestation of ECD and is the one discussed in this article. We found that the majority of patients with pericardial infiltration needed a cardiovascular procedure.
PubMed: 32256919
DOI: 10.14740/cr1025 -
Child's Nervous System : ChNS :... Aug 2023Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based...
INTRODUCTION
Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited.
METHODS
Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG.
RESULTS
In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination.
CONCLUSION
Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.
Topics: Humans; Eosinophilic Granuloma; Conservative Treatment; Skull; Histiocytosis, Langerhans-Cell; Radiography; Remission, Spontaneous
PubMed: 36939904
DOI: 10.1007/s00381-023-05926-z