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Acta Neuropathologica Communications Mar 2020Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this... (Review)
Review
Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this category of brain tumors, herein defined as low-grade, developmental, epilepsy-associated brain tumors (LEAT) is different from those frequently encountered in adults as (A): 77% of LEAT occur in the temporal lobe; (B): the vast majority of LEAT are of low malignancy and classified as WHO I°; (C): LEAT are often composed of mixed glial and neuronal cell components and present with variable growth patterns including small cysts or nodules; (D): LEAT do not share common gene driving mutations, such as IDH1 or 1p/19q co-deletions. Characteristic entities comprise the ganglioglioma (GG), the dysembryoplastic neuroepithelial tumor (DNT), the angiocentric glioma (AG), the isomorphic diffuse glioma (IDG) and the papillary glio-neuronal tumor (PGNT), representing 73.2% of 1680 tumors collected in a large German series of 6747 patients submitted to epilepsy surgery. In the realm of exciting discoveries of genetic drivers of brain tumors new genes have been also reported for LEAT. BRAF V600E mutations were linked to GG with CD34 expression, FGFR1 mutations to DNT, MYB alterations to AG and also IDG and PRKCA fusions to PGNT, suggesting the possibility to also develop a genetically driven tumor classification scheme for LEAT. Rare availability of LEAT in a single center is a challenging obstacle, however, to systematically unravel the neurobiological nature and clinical behavior of LEAT. Other challenges in need of clarification include malignant tumor progression of LEAT entities, seizure relapse in patients following bulk tumor resection and the controversial issue of associated focal cortical dysplasia as additional pathomechanism. In order to advance our understanding and promote reliable diagnostic work-up of LEAT, we recommend, therefore, international collaboration to achieve our goals.
Topics: Arachnoid Cysts; Astrocytoma; Brain Neoplasms; Central Nervous System Cysts; Dermoid Cyst; Epidermal Cyst; Epilepsies, Partial; Ganglioglioma; Humans; Molecular Diagnostic Techniques; Neoplasm Grading; Neoplasms, Neuroepithelial; Oligodendroglioma; Protein Kinase C-alpha; Proto-Oncogene Proteins; Proto-Oncogene Proteins B-raf; Proto-Oncogene Proteins c-myb; Receptor, Fibroblast Growth Factor, Type 1; Trans-Activators
PubMed: 32151273
DOI: 10.1186/s40478-020-00904-x -
Indian Journal of Ophthalmology Feb 2022Dermoid cyst, a developmental benign choristoma, is the most common orbital tumor of childhood, arising from ectodermal sequestration along the lines of embryonic fusion...
BACKGROUND
Dermoid cyst, a developmental benign choristoma, is the most common orbital tumor of childhood, arising from ectodermal sequestration along the lines of embryonic fusion of mesodermal processes, lined by keratinized stratified squamous epithelium and expanding slowly due to constant desquamation and dermal glandular elements. Approximately 80% are found in the head and neck region and comprise 3-9% all orbital masses.
PURPOSE
It is mandatory to know about the variable presentations of orbital dermoids and the surgical techniques that can be adopted based on the site, extent, age and aesthetic needs, presence of inflammation and possibility of intraoperative rupture.
SYNOPSIS
Orbital dermoids can be classified as juxta-sutural, sutural or soft tissue cysts; superficial or deep; intraosseous or extraosseous, and intraorbital or extraorbital. These smooth, painless, mobile or partially mobile lesions mostly present at the fronto-zygomatic suture with proptosis, displacement, ptosis or diplopia, depending on depth and extent. Therefore, it is important to understand the various presentations and the appropriate surgical techniques.
HIGHLIGHTS
We describe the embryological origin, types and clinical features of dermoids in this video and demonstrate the surgical and minimally invasive techniques for their management.
VIDEO LINK
https://youtu.be/-q3xD2igjcQ.
Topics: Blepharoptosis; Choristoma; Dermoid Cyst; Humans; Orbital Diseases; Orbital Neoplasms
PubMed: 35086291
DOI: 10.4103/ijo.IJO_145_22 -
The Journal of International Advanced... Dec 2020A case of mastoid dermoid cyst (DC) was presented, and differences with cases of other temporal bone DCs were analyzed. The mastoid DC was also compared with mastoid... (Review)
Review
A case of mastoid dermoid cyst (DC) was presented, and differences with cases of other temporal bone DCs were analyzed. The mastoid DC was also compared with mastoid congenital cholesteatoma. We reported a case of a patient with mastoid DC, evaluating her clinical, radiological, and surgical findings. A review of the literature was performed to compare our findings with those reported. The preoperative radiological evaluation prompted us to plan a surgical approach to the lesion, suspecting the presence of a mastoid congenital cholesteatoma. The surgical findings were in line with the presence of a mastoid DC. Only two cases reported in the literature presented features that fulfilled the criteria of a true mastoid DC. A DC confined to the mastoid region is an extremely rare clinical entity, with asymptomatic and slow growth. Preoperative radiological differentiation between congenital cholesteatoma and DCs with atypical features can be difficult. However, surgical excision is the treatment of choice in both cases. Diagnosis is confirmed by the histological evaluation.
Topics: Cholesteatoma, Middle Ear; Dermoid Cyst; Female; Humans; Magnetic Resonance Imaging; Mastoid; Middle Aged
PubMed: 33136032
DOI: 10.5152/iao.2020.7854 -
Laryngo- Rhino- Otologie May 2024The following review article highlights key topics in pediatric rhinology that are currently the focus in research and at conferences as well as in the interdisciplinary... (Review)
Review
The following review article highlights key topics in pediatric rhinology that are currently the focus in research and at conferences as well as in the interdisciplinary discussion between otorhinolaryngologists and pediatricians. In particular, congenital malformations such as choanal atresia or nasal dermoid cysts are discussed, followed by statements on the current procedures for sinogenic orbital complications as well as on the diagnosis and therapy of chronic rhinosinusitis in children. Furthermore, updates on the role of the ENT specialist in the care for children with cystic fibrosis and primary ciliary dyskinesia are provided.
Topics: Humans; Child; Choanal Atresia; Rhinitis; Sinusitis; Dermoid Cyst; Cystic Fibrosis; Chronic Disease
PubMed: 38697148
DOI: 10.1055/a-2178-2957 -
The Journal of Craniofacial SurgeryEpidermoid and dermoid cysts are benign developmental anomalies that can form anywhere in the body. Despite the rarity of incidence in the head and neck, they can arise... (Review)
Review
BACKGROUND
Epidermoid and dermoid cysts are benign developmental anomalies that can form anywhere in the body. Despite the rarity of incidence in the head and neck, they can arise at a variety of craniofacial locations. The purpose of this study was to analyze the clinical features of epidermoid and dermoid cysts arising in the craniofacial region with a literature review.
METHODS
A retrospective study was designed, and clinical features and surgical considerations were investigated from a literature review. Cases of epidermoid cysts in the scalp, temporal area, glabellar area, mouth floor, and buccal mucosa were described.
RESULTS
Dermoid cysts in more lateral regions of the scalp are rarely associated with intracranial extension. Because temporal dermoid cysts have a high rate of intracranial extension, radiological evaluation of the lesions in the temporal area is imperative. Epidermoid cysts in the glabellar area are usually superficial. Consideration of the surgical approach for an epidermoid cyst of the mouth floor is important. Because epidermoid cysts in the buccal mucosa are extremely rare, differential diagnosis was emphasized. Epidermoid cysts in the scalp, in the temporal intradiploic area, on the glabellar area in the periorbital region, in the mouth floor, and in the buccal mucosa were surgically excised considering the depth and location. Ten cases of epidermoid cysts in the buccal mucosa were retrieved from the literature review.
CONCLUSIONS
Consideration of the anatomic locations of epidermoid and dermoid cysts in the craniofacial region might help facilitate accurate diagnosis and treatment.
Topics: Humans; Epidermal Cyst; Dermoid Cyst; Retrospective Studies; Face; Scalp
PubMed: 37487139
DOI: 10.1097/SCS.0000000000009561 -
The Journal of Craniofacial SurgeryDermoid cyst is a congenital and benign disease with most occur on the head and neck. It is rarely that occur on the nasal tip and nasal septum at same time and rarely...
Dermoid cyst is a congenital and benign disease with most occur on the head and neck. It is rarely that occur on the nasal tip and nasal septum at same time and rarely repair of using nasal septum mucosa. The authors treated a child with dermoid cyst in the nasal tip and septum. Only the dermoid cyst at the tip of the nose caused the change of appearance. Dermoid cyst of nasal septum did not cause any clinical symptoms. She underwent excision of the dermoid cyst at the tip of the nose and endoscopic surgery for the dermoid cyst in the nasal septum and used the nasal septum mucosa for repair at the same time. After 6 months of recovery, the appearance of the nasal tip recovered well without obvious scar, the nasal septum area recovered well, and the local stoma was unobstructed without recurrence. The authors found that this kind of nasal septal cyst with no clinical symptoms can achieve good therapeutic effect through endoscopic surgery and repair of using nasal septum mucosa, with less damage, rapid recovery, and good prognosis.
Topics: Child; Dermoid Cyst; Endoscopy; Female; Humans; Nasal Septum; Nose Diseases; Nose Neoplasms
PubMed: 34690325
DOI: 10.1097/SCS.0000000000008281 -
Medical Journal, Armed Forces India Dec 2015The dermoid cyst is an uncommon clinicopathological lesion of developmental origin. The term dermoid cyst is used to describe 3 cysts that are closely related... (Review)
Review
The dermoid cyst is an uncommon clinicopathological lesion of developmental origin. The term dermoid cyst is used to describe 3 cysts that are closely related histologically: dermoid cyst, epidermoid cyst, and teratoma. Epidermoid and dermoid cysts are benign nature, which may occur anywhere in the body, but most predominantly in the ovary and scrotal regions. Only about 7% are found in the head and neck. The occurrence of such cysts in the oral cavity is extremely rare, with approximately 1.6% located in this area. The floor of the mouth is one of the most commonly affected area, however, these cysts can also be found in the tongue, lips, buccal mucosa and jaw bones. There is always a difficulty of making a correct diagnosis of these lesions with clinical examinations and conventional radiography. To achieve a diagnosis and to develop correct surgical strategy specialized imaging examinations such as ultrasonography (US), computed tomography (CT), Magnetic Resonance Imaging (MRI) and histopathological examination should be carried out. Treatment comprises total surgical excision the approach remains dictated logically by the cyst's location. Ample understanding and vigilance about this slow growing painless mass is essential not only because of the symptoms it produces but also due to its malignant potential. When dermoid cysts occur on the floor of the mouth, they may enlarge to such an extent that they can interfere with deglutition and produce respiratory obstruction. Early diagnosis and treatment are essential for these cystic entities.
PubMed: 26843755
DOI: 10.1016/j.mjafi.2013.11.004 -
Children (Basel, Switzerland) Nov 2020Caudal Regression Syndrome (CRS) or Caudal dysgenesis syndrome (CDS) is characterized by maldevelopment of the caudal half of the body with variable involvement of the...
Caudal Regression Syndrome (CRS) or Caudal dysgenesis syndrome (CDS) is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. CRS affects 1-3 newborn infants per 100,000 live births. The prevalence in infants of diabetic mothers is reported at 1 in 350 live births which includes all the variants. A related condition is sirenomelia sequence or mermaid syndrome or symmelia and is characterized by fusion of the legs and a variable combination of the other abnormalities. The Currarino triad is a related anomaly that includes anorectal atresia, coccygeal and partial sacral agenesis, and a pre-sacral lesion such as anterior meningocele, lipoma or dermoid cyst. A multidisciplinary management approach is needed that includes rehabilitative services, and patients need a staged surgical approach.
PubMed: 33158301
DOI: 10.3390/children7110211