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Head and Neck Pathology Dec 2022Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic... (Review)
Review
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic review of the literature on NCMH, we also report an unusual case of NCMH in an adolescent patient. A systematic review conducted following the PRISMA guidelines. PubMed, EMBASE and manual search through references of relevant publication were utilised to gather all published case-reports of NCMH. Data collected from each case-report for patient demographics, site and size of NCMH, clinical presentation, co-morbidities, diagnostic methods, treatment options and follow-up methods. The systemic review collected sixty-two case-reports of NCMH (including our case) affecting 42 men and 21 women (2:1 male to female ratio). Mean average age was 5.1 years (age range: 1 day to 70 years). The anatomical sites of the tumor were: nasal cavity (n = 17), paranasal sinuses (n = 30), orbital region (n = 17), and the base of the skull (n = 16). The reported clinical manifestations were nasal obstruction or congestion (n = 29), nasal mass (n = 27), epistaxis (n = 6), orbital symptoms (n = 14). NCMH is a very rare cause of nasal masses in infants and toddlers. Our case and previous case reports confirm that NCMH can mimic other benign and malignant tumors, therefore we should be vigilant for rare pathologies that lead to nasal masses. Recently the link between DIECR1 mutation with NCMH has been established, so NCMH should be considered in any patient with nasal or orbital symptoms with a history of DICER1-related tumor spectrum.
Topics: Female; Male; Humans; Adolescent; Child, Preschool; Infant; Neoplasms; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 35507301
DOI: 10.1007/s12105-022-01452-7 -
BMC Neurology Feb 2022Intra-cranial schwannomas account for less than 8% of brain tumors, among which more than 80% arise from the vestibular nerve. Intra-cerebellar schwannomas are extremely...
BACKGROUND
Intra-cranial schwannomas account for less than 8% of brain tumors, among which more than 80% arise from the vestibular nerve. Intra-cerebellar schwannomas are extremely rare. Several cases have been previously reported but without remarkable degenerative changes on histology.
CASE PRESENTATION
A 61-year-old man presented with worsening disorientation, and an imaging study revealed a cystic lesion (6.5 cm in the largest diameter) in the left hemisphere of the cerebellum accompanied by a mural nodule (2.5 cm) located just inside the skull with enhancement and focal calcification, in addition to hydrocephalus. The lesion was more than 5 mm from the left acoustic nerve. The patient underwent gross total resection. Pathological examination revealed remarkable degenerative changes with various morphological features. Tumor cells were pleomorphic with rich cytoplasm containing numerous eosinophilic granules. Blood vessels and extracellular matrix showed remarkable hyalinization. Immunohistochemical staining revealed that the tumor cells were positive for S-100 protein and negative for Olig2. The tumor was diagnosed as a schwannoma with marked degenerative changes.
CONCLUSIONS
The present case is discussed with reference to a systematic review of previous reports of intra-cerebellar schwannoma. Intra-cerebellar schwannoma should be included in the differential diagnosis of cystic lesions with heterogeneous histopathological morphology in the cerebellum.
Topics: Brain Neoplasms; Calcinosis; Cerebellum; Diagnosis, Differential; Humans; Male; Middle Aged; Neurilemmoma
PubMed: 35209854
DOI: 10.1186/s12883-022-02596-3 -
Current Oncology (Toronto, Ont.) Feb 2022Dendritic cell vaccination (DCV) strategies, thanks to a complex immune response, may flare tumor regression and improve patients' long-term survival. This meta-analysis... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Dendritic cell vaccination (DCV) strategies, thanks to a complex immune response, may flare tumor regression and improve patients' long-term survival. This meta-analysis aims to assess the efficacy of DCV for newly diagnosed glioblastoma patients in clinical trials.
METHODS
The study databases, including PubMed, Web of Knowledge, Google Scholar, Scopus, and Cochrane, were searched by two blinded investigators considering eligible studies based on the following keywords: "glioblastoma multiforme", "dendritic cell", "vaccination", "immunotherapy", "immune system", "immune response", "chemotherapy", "recurrence", and "temozolomide". Among the 157 screened, only 15 articles were eligible for the final analysis.
RESULTS
Regimens including DCV showed no effect on 6-month progression-free survival (PFS, HR = 1.385, 95% CI: 0.822-2.335, = 0.673) or on 6-month overall survival (OS, HR = 1.408, 95% CI: 0.882-2.248, = 0.754). In contrast, DCV led to significantly longer 1-year OS (HR = 1.936, 95% CI: 1.396-2.85, = 0.001) and longer 2-year OS (HR = 3.670, 95% CI: 2.291-5.879, = 0.001) versus control groups. Hence, introducing DCV could lead to increased 1 and 2-year survival of patients by 1.9 and 3.6 times, respectively.
CONCLUSION
Antitumor regimens including DCV can effectively improve mid-term survival in patients suffering glioblastoma multiforme (GBM), but its impact emerges only after one year from vaccination. These data indicate the need for more time to achieve an anti-GBM immune response and suggest additional therapeutics, such as checkpoint inhibitors, to empower an earlier DCV action in patients affected by a very poor prognosis.
Topics: Brain Neoplasms; Dendritic Cells; Glioblastoma; Humans; Immunotherapy; Vaccination
PubMed: 35200574
DOI: 10.3390/curroncol29020075 -
Frontiers in Endocrinology 2021Basal ganglia germ cell tumors (BGGCTs) represent an extremely rare subset of tumors about which little is known. Some patients suffer from tumor dissemination, such as... (Comparative Study)
Comparative Study
BACKGROUND
Basal ganglia germ cell tumors (BGGCTs) represent an extremely rare subset of tumors about which little is known. Some patients suffer from tumor dissemination, such as sellar involvement. This study aimed to evaluate the independent prognostic risk factors of patients with BGGCTs with or without sellar involvement.
METHODS
Sixteen patients were diagnosed with BGGCTs at Peking Union Medical College Hospital from January 2000 to December 2020. A literature review was performed on the online databases Medline and PubMed, and 76 cases in the 19 retrieved articles were identified at the same time. The data regarding biochemical tests, radiological examinations, and outcomes during follow-up were analyzed.
RESULTS
Of 92 patients in this study, seven patients were clinically diagnosed as germinomas, with the remaining 85 patients receiving surgery. Fifty-two patients suffered from multifocal lesions or tumor dissemination. The patients with BGGCTs demonstrated a significant male predilection. The patients with delayed diagnosis more likely had cognitive disturbance (p = 0.028), mental disturbance (p = 0.047), and diabetes insipidus (p = 0.02). Multivariate analysis demonstrated that the independent poor prognostic risk factors of patients with BGGCTs were delayed diagnosis [odd ratio (OR) 2.33; 95% CI 1.02-5.31], focal radiotherapy (OR 4.00; 95% CI 1.69-9.49), and non-pure germinoma (OR 4.64; 95% CI 1.76-12.22).
CONCLUSIONS
The delayed diagnosis, focal radiotherapy, and non-pure germinoma were associated with a poorer prognosis for patients with BGGCTs with or without sellar involvement.
Topics: Adolescent; Adult; Basal Ganglia; Brain Neoplasms; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Neoplasms, Germ Cell and Embryonal; Sella Turcica; Skull Neoplasms; Young Adult
PubMed: 34858336
DOI: 10.3389/fendo.2021.763609 -
World Neurosurgery Oct 2021Skull base leiomyomas (LMs) and angioleiomyomas (ALMs) are rare, and the understanding of this disease is limited. We present a systematic literature review of skull...
BACKGROUND
Skull base leiomyomas (LMs) and angioleiomyomas (ALMs) are rare, and the understanding of this disease is limited. We present a systematic literature review of skull base LM and ALM and report a case of internal auditory canal (IAC) ALM.
METHODS
A systematic review was conducted following the PRISMA guidelines. PubMed and Embase were systematically queried for skull base LM and ALM, and Rayyan QCRI was used for the review. After applying exclusion criteria, individual articles were evaluated for quality control, data collection, and analysis. The presentation, management, and outcome of a 37-year-old man with a right-sided IAC ALM are described.
RESULTS
Of 68 unique entries, 27 studies were included. Thirty-four cases of skull base LM (n = 6) or ALM (n = 28) were identified. Average age at presentation was 45.1 ± 14.5 years, and 52.9% of patients were male. Tumor diameter was 2.75 ± 1.6 cm, with headaches being the most reported symptom. Commonly reported locations were the cavernous sinus and the external auditory canal. Only 3 cases of IAC ALM met the criteria for this review. All tumors were treated with surgery, and gross total resection was achieved in 27 patients. Radiation was given in 3 cases with subtotal resection.
CONCLUSIONS
Skull base LM and ALM are rare. Given the need for pathology, surgery has been the standard treatment for symptomatic skull base LM and ALM. It is important to understand the available data about this disease and consider it in the differential of skull base lesions.
Topics: Adolescent; Adult; Aged; Angiomyoma; Child; Child, Preschool; Ear Canal; Female; Humans; Leiomyoma; Male; Middle Aged; Neurosurgical Procedures; Skull Base; Skull Base Neoplasms; Young Adult
PubMed: 34182177
DOI: 10.1016/j.wneu.2021.06.096 -
Head and Neck Pathology Dec 2021Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The... (Comparative Study)
Comparative Study
Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. β-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.
Topics: Age Factors; DNA Copy Number Variations; Humans; Jaw Neoplasms; Keratins; Ki-67 Antigen; Mutation; Neoplasm Recurrence, Local; Odontogenic Tumors; Sex Factors; Tumor Suppressor Protein p53; beta Catenin
PubMed: 34128137
DOI: 10.1007/s12105-021-01347-z -
Journal of Neuro-oncology Jun 2021We aim to systematically review and summarize the demographics, clinical features, management strategies, and clinical outcomes of primary and radiation-induced... (Review)
Review
PURPOSE
We aim to systematically review and summarize the demographics, clinical features, management strategies, and clinical outcomes of primary and radiation-induced skull-base osteosarcoma (SBO).
METHODS
PubMed, Scopus, and Cochrane databases were used to identify relevant articles. Papers including SBO cases and sufficient clinical outcome data were included. A comprehensive clinical characteristic review and survival analysis were also conducted.
RESULTS
Forty-one studies describing 67 patients were included. The median age was 31 years (male = 59.7%). The middle skull-base was most commonly involved (52.7%), followed by anterior (34.5%) and posterior (12.7%) skull-base. Headache (27%), exophthalmos (18%), and diplopia (10%) were common presenting symptoms. Sixty-eight percent of patients had primary SBO, while 25% had radiation-induced SBO. Surgery was the main treatment modality in 89% of cases. Chemotherapy was administered in 65.7% and radiotherapy in 50%. Median progression-free survival (PFS) was 12 months, and the overall 5-year survival was 22%. The five-year survival rates of radiation-induced SBO and primary SBO were 39% and 16%, respectively (P < 0.05).
CONCLUSION
SBO is a malignant disease with poor survival outcomes. Surgical resection is the primary management modality, in conjunction with chemotherapy and radiotherapy. Radiation-induced SBO has a superior survival outcome as compared to its primary counterpart. Complete surgical resection showed a statistically insignificant survival benefit as compared to partial resection.
Topics: Humans; Osteosarcoma; Progression-Free Survival; Skull Base; Skull Base Neoplasms; Treatment Outcome
PubMed: 33999382
DOI: 10.1007/s11060-021-03757-z -
Pituitary Oct 2021Postoperative cerebrospinal fluid rhinorrhoea (CSFR) remains a frequent complication of endonasal approaches to pituitary and skull base tumours. Watertight skull base...
PURPOSE
Postoperative cerebrospinal fluid rhinorrhoea (CSFR) remains a frequent complication of endonasal approaches to pituitary and skull base tumours. Watertight skull base reconstruction is important in preventing CSFR. We sought to systematically review the current literature of available skull base repair techniques.
METHODS
Pubmed and Embase databases were searched for studies (2000-2020) that (a) reported on the endonasal resection of pituitary and skull base tumours, (b) focussed on skull base repair techniques and/or postoperative CSFR risk factors, and (c) included CSFR data. Roles, advantages and disadvantages of each repair method were detailed. Random-effects meta-analyses were performed where possible.
RESULTS
193 studies were included. Repair methods were categorised based on function and anatomical level. There was absolute heterogeneity in repair methods used, with no independent studies sharing the same repair protocol. Techniques most commonly used for low CSFR risk cases were fat grafts, fascia lata grafts and synthetic grafts. For cases with higher CSFR risk, multilayer regimes were utilized with vascularized flaps, gasket sealing and lumbar drains. Lumbar drain use for high CSFR risk cases was supported by a randomised study (Oxford CEBM: Grade B recommendation), but otherwise there was limited high-level evidence. Pooled CSFR incidence by approach was 3.7% (CI 3-4.5%) for transsphenoidal, 9% (CI 7.2-11.3%) for expanded endonasal, and 5.3% (CI 3.4-7%) for studies describing both. Further meaningful meta-analyses of repair methods were not performed due to significant repair protocol heterogeneity.
CONCLUSIONS
Modern reconstructive protocols are heterogeneous and there is limited evidence to suggest the optimal repair technique after pituitary and skull base tumour resection. Further studies are needed to guide practice.
Topics: Cerebrospinal Fluid Leak; Cerebrospinal Fluid Rhinorrhea; Endoscopy; Humans; Postoperative Complications; Retrospective Studies; Skull Base; Skull Base Neoplasms
PubMed: 33973152
DOI: 10.1007/s11102-021-01145-4 -
Cancer Reports (Hoboken, N.J.) Dec 2021Management of the node-negative neck in oral maxillary squamous cell carcinoma (SCC), encompassing the hard palate and upper alveolar subsites of the oral cavity, is... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Management of the node-negative neck in oral maxillary squamous cell carcinoma (SCC), encompassing the hard palate and upper alveolar subsites of the oral cavity, is controversial, with no clear international consensus or recommendation regarding elective neck dissection in the absence of cervical metastases.
AIM
To assess the occult metastatic rate in patients with clinically node negative oral maxillary SCC; both as an overall metastatic rate, and a comparison of patients managed with an elective neck dissection at index surgery, compared to excision of the primary with clinical observation of the neck.
METHODS AND RESULTS
A systematic review was performed by two independent investigators for studies relating to oral maxillary SCC and analysed according to PRISMA criteria. Data were extracted from Pubmed, Ovid MEDLINE, EMBASE, and SCOPUS via relevant MeSH terms. Grey literature was searched through Google Scholar and OpenGrey. Five hundred and fifty-three articles were identified on the initial search, 483 unique articles underwent screening against eligibility criteria, and 29 studies were identified for final data extraction. Incidence of occult metastases in patients with clinically node negative oral maxillary SCC was identified either on primary elective neck dissection or on routine follow up. Meta-analyses were performed. Of 553 relevant articles identified on initial search, 29 were included for analysis. The pooled overall rate of occult metastases in patients initially presenting with clinically node-negative disease was 22.2%. There is a statistically significant effect of END on decreasing regional recurrence demonstrated in this study (RR 0.36, 95% CI 0.24, 0.59).
CONCLUSION
The results of this systematic review and meta-analysis suggest elective neck dissection for patients presenting with hard palate or upper alveolar SCC, even in a clinically node negative neck.
Topics: Animals; Head and Neck Neoplasms; Humans; Lymphatic Metastasis; Maxillary Neoplasms; Mouth Neoplasms; Squamous Cell Carcinoma of Head and Neck
PubMed: 33963809
DOI: 10.1002/cnr2.1410 -
JAMA Otolaryngology-- Head & Neck... Apr 2021Overall, the prognosis of sinonasal squamous cell carcinoma (SCC) is poor. This malignancy can arise de novo or from inverted papillomas, but it is unclear whether... (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Overall, the prognosis of sinonasal squamous cell carcinoma (SCC) is poor. This malignancy can arise de novo or from inverted papillomas, but it is unclear whether survival differences between the 2 pathologies exist.
OBJECTIVE
To assess for survival differences between patients with sinonasal de novo SCC (dnSCC) and those with inverted papilloma-associated SCC (IPSCC).
DATA SOURCES
A search of Ovid MEDLINE, Embase, Scopus, and the Cochrane Library from inception to January 23, 2020, with cross-referencing of retrieved studies, was performed. Additional data were requested from authors.
STUDY SELECTION
Inclusion and exclusion criteria were designed to capture studies with survival outcomes of adults with sinonasal SCC who underwent regular treatment. Clinical trials, cohort studies, case-control studies, and case series with more than 10 adults aged 18 years or older with sinonasal SCC were included. Exclusion criteria were studies on non-SCC sinonasal neoplasms, studies without histopathologic diagnoses, non-English language articles, nonhuman animal studies, and abstract-only articles. Two blinded investigators (J.J.L., A.M.P., T.W.E., or N.S.W.) screened each abstract and full text, and a third investigator (J.J.L. or P.P.) adjudicated discrepancies. Of 729 unique citations, 26 studies of 1194 total patients were included.
DATA EXTRACTION AND SYNTHESIS
Meta-Analysis of Observational Studies in Epidemiology (MOOSE) guidelines were followed. The Methodological Index for Nonrandomized Studies (MINORS) criteria were used to assess study quality. Two blinded investigators (J.J.L., A.M.P., T.W.E., or N.S.W.) independently extracted data from each study. Data were pooled using a random-effects model.
MAIN OUTCOMES AND MEASURES
The primary outcome was overall survival, and secondary outcomes were disease-free and disease-specific survival. Before data collection, it was hypothesized that the dnSCC cohort would have worse survival outcomes than the IPSCC cohort.
RESULTS
One study of patients with dnSCC, 12 studies of patients with IPSCC, and 5 studies with both cohorts were included in the meta-analysis of overall survival. The pooled 5-year overall survival rate for 255 patients with dnSCC was 56% (95% CI, 41%-71%; I2 = 83.8%) and for 475 patients with IPSCC was 65% (95% CI, 56%-73%; I2 = 75.7%). Five comparative studies of both cohorts totaling 240 patients with dnSCC and 155 patients with IPSCC were included in another meta-analysis. The pooled overall survival hazard ratio was 1.87 (95% CI, 1.24-2.84; I2 = 0%).
CONCLUSIONS AND RELEVANCE
This systematic review and meta-analysis found that patients with dnSCC had almost a 2-fold increased risk of mortality compared with those with IPSCC. Large, multicenter studies are necessary to validate these findings before considering treatment alterations such as de-escalation based on histopathology.
Topics: Carcinoma, Squamous Cell; Humans; Nasal Cavity; Nose Neoplasms; Papilloma, Inverted; Paranasal Sinus Neoplasms; Survival Rate
PubMed: 33507208
DOI: 10.1001/jamaoto.2020.5261