Exclusion criteria included cases with suspicion for Differences of Sex Development, lack of surgically-confirmed ovarian absence, and karyotypes other than 46XX. Our search yielded 12,120 citations, of which 79 studies were included. 10 additional studies were found by citation chasing resulting in a total 113 cases including two unpublished cases presented in this review. Abdominal/pelvic pain (30%) and infertility/subfertility (19%) were the most frequent presentations. Ovarian abnormalities were not noted in 28% of cases with pre-operative ovarian imaging results. Approximately 17% of cases had concomitant uterine abnormalities, while 22% had renal abnormalities. Renal abnormalities were more likely in patients with uterine abnormalities (p < 0.005). Torsion or vascular etiology was the most frequently suspected etiology of ovarian absence (52%), followed by indeterminate (27%) and embryologic etiology (21%). Most cases of ovarian absence are likely attributable to torsion or vascular accidents, despite many references to the condition as “agenesis” in the literature. Imaging may fail to correctly diagnose ovarian absence, and diagnostic laparoscopy may be preferable in many cases as genitourinary anatomy and fertility considerations can be assessed during the procedure. Fertility is likely minimally or not affected in women with unilateral ovarian absence.Ovarian absence is an uncommon condition that most frequently presents unilaterally. Several etiologies for the condition have been proposed, including torsion, vascular accident, and embryological defect. A systematic review was conducted to describe the clinical presentation of ovarian absence, as well as its associations with other congenital anomaliesThe online version contains supplementary material available at 10.1186/s13048-022-01090-1. Unilateral ovarian absence (UOA) is a rare finding with prevalence previously reported as 1 in 11,241 [Emx2 and Lhx1/9, the Wilms’ tumor gene Wt1, and steroidogenic factor 1 (Nr5a1 or Sf1), whose ablation in the mouse leads to severe disruption of gonadal formation [In humans, the genitourinary (GU) system derives from the intermediate mesoderm – a component of the germ layer situated between paraxial and lateral plate mesoderm. Gonadal development begins around week four of gestation when the coelomic epithelium of the intermediate mesoderm proliferates forming the genital ridges on either side of the developing embryo. These genital ridges are somatic components of the gonads which will be colonized by primordial germ cells from the posterior endoderm that form the hindgut around week six . At thisctor 1 Nra1 or Sf1SRY and its downstream target SOX9, which activate testis development. Ovarian development, once believed to occur by default, requires the action of several genes including Foxl2, Wnt4, and Rspo1 [Foxl2 and Wnt4 regulate ovarian development by downregulating Sox9 expression [Foxl2 inhibits Nr5a1 expression by antagonizing Wt1 [Rspo1 upregulates Wnt4 expression and cooperates with Wnt4 signaling [Sex determination occurs around gestation week six-eight and depends on the expression of the Y-linked gene pression . In addizing Wt1 . Rspo1 uignaling . FollowiWNT4 mutations have been associated with a minority of Müllerian anomalies cases, and ablation of Wnt4 in mice disrupts Müllerian duct development and sex determination but does not cause gonadal agenesis [The uterus develops separately. Between week seven-nine, the paramesonephric ducts fuse at the cranial end to form the uterus, and caudally to form the upper two thirds of the vagina . The latagenesis , 15.The 1-in-11,241 incidence of UOA is based on two cases observed at a single Malaysian institution in the context of 22,483 gynecological and obstetric surgeries –19. We e.The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) and Synthesis Without Meta-Analysis (SWiM) were used to guide the reporting in this study and the checklists can be found in Supplementary Table A systematic search of the literature was conducted by a medical librarian in Cochrane Library, ClinicalTrials.gov, Google Scholar, Ovid Embase, Ovid MEDLINE, PubMed, Scopus, and Web of Science Core Collection databases to identify relevant articles published from the inception of each database to April 2022. The final searches were performed in all the databases on April 6, 2022. The search was peer-reviewed by a second medical librarian using PRESS (Peer Review of Electronic Search Strategies) .Case studies or series were included if the study reported ovarian absence with surgical confirmation. Studies were limited to cases of ovarian absence described in phenotypical females that had 46 XX karyotype or, when karyotype was not stated, absence of syndromic features suggestive of a genetic condition. Studies describing cases with Differences of Sexual Development (DSD) or those not available in English language were excluded.Four independent reviewers  assessed citations returned by the literature search in pairs, selected relevant abstracts for full text review, and identified full text articles eligible for inclusion in the final review. The lead investigator (AVM) resolved all identified conflicts at each stage of the review process and reviewed the final list of full-text articles to ensure each was relevant to this study’s objectives.Three independent reviewers  extracted variables from full texts. These included age of patients, presentation symptom(s), surgical procedure, OA laterality, genetic studies, imaging studies, anatomy of the remaining reproductive tract, renal anatomy, and study design. Data items are listed in Table Manuscripts were assessed for quality of evidence based on the Oxford Centre for Evidence-Based Medicine (OCEBM): Levels of Evidence. This scale grades manuscripts from 1 (highest) to 5 (lowest) according to the level of clinical evidence that can be derived from their study design. Randomized control trials are designated 1, cases-series are 4, while case reports and expert opinions are graded 5. Case reports were subsequently assessed using the JBI Critical Appraisal Checklist for Case Reports .Statistical analysis was performed using SAS software  and Microsoft Excel . We performed quantitative, qualitative, and formal narrative syntheses of data extracted from the case reports and literature reviews included in our systematic review of OA. We performed univariate analysis on the categorical variables and reported their frequencies and relative percentages. We conducted chi-square tests to evaluate the relationships between several categorical variables. During the data analysis process, we used the fixed-effect assumption. Publications included in this systematic review were primarily case reports and case series, and there was significant variability in reporting of case details.A 15-year-old postmenarchal girl presented to Yale New Haven Children’s Hospital for a diagnostic laparoscopy due to chronic abdominal and pelvic pain. Menarche occurred at age 12. She reported regular menses with normal flow and acyclic pelvic and flank pain. She denied being sexually active. Past medical and surgical history were significant for ADHD, dyslexia, nephrolithiasis, constipation, tonsillectomy and adenoidectomy. There was no documented family history of endometriosis, but there was a suspicion for endometriosis necessitating oophorectomy in the maternal grandmother. Physical exam was notable for tenderness with deep palpation in the left upper and lower abdominal quadrants. She had age-appropriate secondary sexual characteristics with Tanner stage 5 breasts and Tanner stage 4 pubic hair. The external inspection of the genitalia revealed normal labia, clitoris, prepuce, external urethra meatus, and annular hymen. Renal ultrasound demonstrated left-sided nonobstructing kidney stones. The transabdominal pelvic ultrasound was unremarkable. Three prior ultrasounds reported normal-appearing ovaries bilaterally. The patient was started on continuous combined ethinyl estradiol-progesterone oral formulation to suppress menses due to suspicion of endometriosis. After four months, she continued to experience pelvic pain and irregular vaginal bleeding. Following a shared decision-making process, the patient and guardian elected to proceed with diagnostic laparoscopy. Surgery revealed a normal appearing uterus with bilateral round ligaments and a normal appearing right fallopian tube leading to an enlarged right ovary. Lesions suspicious for endometriosis were observed and biopsied in the pouch of Douglas, on the sigmoid colon, and the right uterosacral ligament. The left ovary, distal fallopian tube, and utero-ovarian ligament were absent. The left cornua of the uterus had a 3 cm tubular structure which was presumed to be the left fallopian tube remnant.  woman with a history of asthma and genital chlamydia infection. Her surgical history was significant for a supraumbilical laparotomy in infancy to retrieve a severed umbilical catheter remnant. She had no other abdominal surgery. She had a history of a right tubal ectopic pregnancy which was successfully treated with methotrexate. The patient presented to the hospital with severe abdominal pain and a positive home pregnancy test. On abdominal exam she had mild suprapubic tenderness with rebound. Her pelvic exam revealed normal appearing labia, clitoris, and external urethral meatus. Speculum exam demonstrated multiparous cervix, without discharge or blood. Bimanual examination revealed a small, anteverted uterus with no masses appreciated in the adnexa bilaterally, and mild tenderness in the posterior fornix. The serum beta-HCG level was 5,519 mIU/mL. The patient had two pelvic ultrasounds. The first revealed normal bilateral ovaries with normal blood flow and a complex cystic right adnexal structure possibly indicative of an ectopic pregnancy. The second ultrasound suggested torsion of the left fallopian tube, a cystic structure with a possible yolk sac in the right adnexa, and no distinct right ovary. Neither ultrasound indicated an intrauterine pregnancy. The patient elected to have diagnostic laparoscopy due to a suspected ectopic pregnancy and adnexal torsion. Operative findings were significant for the minimal adhesive disease and a right tubal ectopic pregnancy. The left ovary and adnexa appeared normal as did the bilateral round ligaments. An ectopic pregnancy was found extruding from the remnant of the right fallopian tube that had dived into the posterior leaf of the broad ligament. Notably, the right ovary, distal 2/3 of the right fallopian tube and the right IP ligament were missing. To verify the absence of the right adnexal structures, dissection of the right pelvic side wall was performed. This revealed normal appearing ureter and iliac vessels, but the right ovary and distal 2/3 of the fallopian tube were not visualized. Of note, a second gynecology attending physician was consulted intraoperatively to confirm the absence of the right ovary. The pathology of the extruded gestational tissue revealed decidualized stromal tissue with implantation site trophoblasts, consistent with an ectopic pregnancy. Postoperative recovery was uneventful.Database searches resulted in 22,813 citations Fig. .Fig. 2PRThe analysis comprised 89 studies with 113 cases of bilateral and unilateral ovarian absence, all of which were from English-language articles published from 1909 to 2022. All articles were case reports or case series, some of which were accompanied by literature reviews.10 studies were OCEBM Level 4 (11%) and 79 studies were Level 5 (89%). Using the JBI Critical Appraisal Tool for Case Reports, the overall quality of 89 included records was generally good, with > 90% records scoring “yes” in 6 or more domains. The demographics domain was poorly reported across 59 papers, in most cases this was due to the lack of reporting on race and ethnicity. The detailed quality assessment for each study can be found in Supplementary Table n = 3) or hypoplastic/rudimentary uterus (n = 8). The most common uterine anomaly recorded was unicornuate uterus, followed by non-surgical uterine absence. Of note, patients who presented with uterine anomalies were more likely to have a renal abnormality. (p < 0.005), and patients with absent fallopian tube were more likely to have renal agenesis (p < 0.005).The average age of OA diagnosis was 22.47 years Table . A torsi, SRY and ZFY genes were negative, ruling out the presence of genomic sequences associated with streak gonads or rare intersex conditions [A small minority of cases reported bilateral ovarian absence, typically presenting as primary amenorrhea , 27. Mutnditions . These cnditions .Approximately 17% of cases of OA had concomitant uterine anomalies.Two instances of MRKH with 46,XX karyotype and bilateral ovarian absence were observed , 31.Osmanagaoglu described a 17-year-old female who presented with amenorrhea with unicornuate uterus, right tuboovarian absence, and ipsilateral renal agenesis . An inst. Kriplani et al. reported a 36-year-old G0 female with secondary amenorrhea and a 14-year history of infertility [Infertility/subfertility was the second most common presenting symptom in the diagnosis of the OA, comprising 19% of all casesertility . Laparotertility . Laparotertility , 36.Sunku et al. described a 16-year-old female with secondary amenorrhea, synchronous cervical rhabdomyosarcoma, and Sertoli-Leydig cell tumor of the ovary with unilateral tuboovarian absence . A numbeIncluding our case of the adolescent patient, we noted seven reports of UOA with concomitant endometriosis , 47–50. , is speculative at best. Cases of unknown etiology largely represent insufficient evidence to rule-in embryological causes rather than to rule-out mechanical/vascular causes. As such, it is likely that many unknown etiology cases are attributable to torsion, and the prevalence of torsion etiology in our review (52%) is understated. Categorizing all unknown cases as torsion gives an upper range of 79%, suggesting torsional etiologies were observed with approximately 2.5–4 times the frequency of embryological etiologies.Various proposed etiologies for OA fall into either embryological, vascular, or torsion hypotheses . All thragainst agenesis as an etiology for most UOA cases.While many prior cases label their findings as “agenesis”, the term “absence” is better suited to describing findings that result from mechanical or vascular incidents rather than the failure of the ovary to develop during embryogenesis due to absent primordial tissue . As most cases of UOA do not present with concomitant GU abnormalities, but rather can be explained by infarction and torsion, this suggests Ovarian torsion leading to autoamputation may be preceded by the formation of an ovarian cyst or other outgrowth that raises the risk of torsion. Torsion, in turn, would typically manifest with nausea, vomiting, and acute-onset abdominal pain. Evidence of scarring and adhesions would also be expected during surgery. While some UOA patients report a history of severe abdominal pain for which they did not seek treatment, others recall no such history , 52–54. Multiple cases in our review support the existence of such a spectrum, consistent with the natural history we proposed above. At one institution, the rate of autoamputation following antenatal ovarian torsion was > 60% . We idenOverlap between embryological and vascular causes may exist. It has been suggested that vascular compromise to the primitive ovary or the Müllerian duct could occur during elongation and spinalization of the tube during fourth to fifth month of embryologic development, resulting in ovarian aplasia or hypoplasia . The preCases in our study with suspected embryological origins were all notable for comorbid GU abnormalities– most commonly complete ipsilateral renal agenesis. Uterine abnormalities such as Müllerian agenesis, unicornuate uterus, and hypoplastic/rudimentary uterus were also frequently observed in this group. Bousfiha et al. reports on a 19-year-old female with bilateral ovarian dysgenesis, concomitant uterine aplasia (MRKH), and normal karyotype . SimilarLhx1-, Lhx9-, Emx2-, Nr5a1-, and Wt1-null mice lack gonads completely, but agenesis extends to the kidneys and sometimes other organs, including the adrenal glands, due to their common embryological origin [Foxl2, Wnt4, Tcf21 (Pod1), Six1/4, and members of the insulin/insulin-like growth factor family Insr, Igf1r and Insrr [Wt1and Nr5a1mutations in humans are associated with gonadal dysgenesis and primary ovarian insufficiency respectively, but do not impair early gonadal formation [Animal studies showed that ablation of genes involved in genital ridge development can lead to gonadal agenesis. nd Insrr –70. Howeormation , 72. HowOur results suggest that imaging alone is insufficient to diagnose UOA. Of the case reports that noted results of ovarian imaging prior to confirmatory surgery, 28% (16/57) of imaging results were inconsistent with operative findings. These findings echo Case 1, where three ultrasounds reported normal bilateral ovaries prior to surgery. As UOA is relatively uncommon, some degree of expectancy bias may contribute to these inconstancies. Imaging is also limited by the skill of the technician obtaining the study, as well as the individual who interprets it. Ultrasound was the most common imaging modality reported, and the modality most prone to error. More sensitive modalities, such as MRI, were often conducted post-operatively to ensure that the ovary was not heterotopic/ectopic or missed during surgery. Arguably the most sensitive test for UOA was laparoscopy, as it incidentally revealed every instance of UOA that was missed on imaging. While laparoscopy is generally a safe operative procedure, it still carries risks inherent to surgical intervention, and is likely unnecessary in the absence of other indications. For patients pursuing infertility evaluation, laparoscopy may be useful. It gives patients definite knowledge of their anatomy and can be performed in conjunction with procedures aimed at the evaluation of tubal patency.Questions of how UOA may affect fertility are of great importance to patients with the condition. As approximately 20% of our cases presented with fertility concerns, it may be tempting to infer a causal relationship. While fertility was a major cause of presentation, approximately 35% of our cases with ages ≥ 18 years had a history of pregnancy prior to their UOA diagnosis. Perhaps the most extreme example was an incidental UOA finding in a G11P11 woman with a history of 11 uncomplicated deliveries . AnotherAnother way to conceptualize fertility in UOA involves examining women with a surgically absent ovary. A metanalysis of 21 studies examining ovarian reserve status-post unilateral oophorectomy found decreased ovarian pool quantity, but not quality . DespiteUpon suspicion for UOA, we suggest several practices to confirm the diagnosis. 1) Rule out ovarian ectopy. An ectopic ovary may be detected along the line of descent – the presence of bilaterally intact fallopian tubes may increase the suspicion for maldescent . If a he3) Upon ruling in UOA, preservation of the remaining ovary should be considered. Prior instances where the remaining ovary was removed due to pathology have resulted in surgical menopause . StudiesWe could not comment on the inheritance pattern of the OA as, with few exceptions, family history was rarely reported in the cases we reviewed. One case report noted their patient had a healthy monozygotic twin with no known gynecological pathology . The matIn addition to documenting reported cases of UOA in-depth, our systematic review revealed a discrepancy in the literature regarding the semantics of “absence” and “agenesis” to describe patients with a solitary ovary. We also highlight the association between OA and Müllerian and renal anomalies, instances of which may denote true agenesis. We used a strict inclusion/exclusion criterion to ensure that our reported cases reflected surgically confirmed cases of UOA. By excluding articles without confirmation, we attempted to eliminate the possibility of false positive reports – in line with our finding that imaging may be insufficient to diagnose UOA.Our study has several limitations that must be considered in light of our study design. First, we chose to exclude conference abstracts and non-English language publications. Conference abstracts, generally speaking, are less rigorous than peer-reviewed articles , 90. NonUOA is a heterogenous condition that is occasionally noted during imaging or surgical exploration. Further work up should be based upon full consideration of patients’ history, symptoms, and clinical goals. Careful evaluation of clinical history may help identifying torsion or autoamputation events. UOA found incidentally during surgery should be followed up with additional imaging to rule out heterotopy/ectopy, and fully assess abdomen and pelvis for any sign of pathology or abnormal findings. If the other ovary has been removed or is absent, laboratory assessment to assess ovarian reserve could be helpful as this would suggest an unidentified, functional ovary. If fertility is desired, intraoperative assessment of tubal patency should be considered to avoid the need for separate invasive procedures. Additionally, oophoropexy of the identified ovary should be considered to prevent torsion of a single ovary. In the case of unilateral oophorectomy, caution should be taken prior to removing the sole existing ovary due to the risk of surgical menopause. Genetic analysis is not routinely beneficial in establishing a differential unless OA presents in association with congenital urogenital anomalies or true agenesis is suspected. In this case, biochemical or functional validation of the identified variants should be performed following genetic analysis. Overall, UOA does not seem to represent an independent risk factor for infertility or primary ovarian insufficiency. However, more data is required to definitively address these risks, and patients should be counseled accordingly. While true agenesis likely occurs early in embryogenesis, the possibility of predisposing heritable or environmental factors, as well as genetic associations, remains unknown. This review adds to the existing knowledge on OA and will provide guidance to clinicians, thus improving clinical care for patients with ovarian absence.Additional file 1: Supplementary Table 1. Reporting Guideline Checklists. Supplementary Table 2. Search Strategies & Additional Methodology. Supplementary Table 3. Table of Included Studies. Supplementary Table 4. Excluded Studies Table with reasons for Exclusion. Supplementary Table 5. JBI Critical Appraisal Checklist for Case Reports. Figure S1. Critical appraisal of included studies.


Keratosis obturans is estimated to occur among 4 to 5 patients among 1000 new otological cases.A previously healthy 22 year-old Malay gentleman presented to our clinic with a two-month history of worsening left otalgia with bloody otorrhea. There was also left sided reduced hearing followed by asymmetry on the left side of face for the past 1 week. Patient claimed that there was no recent or any recurrent upper respiratory tract infection prior to this.Further history from patient revealed that he had similar complaints a year ago involving the right ear with no facial asymmetry. Patient was diagnosed with right aural polyp and polypectomy was done under local anaesthesia in another government hospital. However, patient defaulted his follow-up as the problem resolved completely.Upon examination, patient was comfortable, not septic looking and afebrile. Facial nerve examination revealed House–Brackmann Grade III lower motor neuron palsy as there was loss of left nasolabial fold and drooping of left angle of lip. Otoscopic examination revealed a polypoidal mass occluding the entire left ear canal covered with bloody, foul smelling otorrhea . Right eA high-resolution computer tomography (HRCT) scan of temporal was obtained, which revealed non-enhancing soft-tissue mass occupying left external auditory canal, left Prussak's space and middle ear with erosion of left scutum. The left inner ear was intact with normal right ear structures. There was no evidence of facial canal dehiscence or erosion; however signs of inflammation over the tympanic segment of facial canal were noted .Figure 2Patient underwent microscope-guided examination under anaesthesia which revealed polypoidal mass occupying entire lateral third of ear canal with whitish keratin-like debris noted medial to the mass. Polypectomy and aural toileting was commenced. As tympanic membrane appeared bulging, myringotomy and grommet insertion was performed. Histopathologic examination revealed cyst wall covered with stratified squamous epithelium containing lamellated keratin flakes suggestive of keratosis obturans  Post-opeKeratosis obturans can be of two types: Inflammatory type or silent type.Keratosis obturans usually affects younger age group, occurs bilaterally and manifests as severe otalgia, conductive hearing loss and widened ear canal.Computer tomography typically demonstrates soft tissue plug in bilateral external ear canal with evidence of ballooning of the osseous part. In our patient, his main complaint was severe otalgia and otorrhea which was followed by unilateral facial weakness and hearing loss. Although rare, few cases of keratosis obturans, presenting with facial weakness have been reported.Clinical differential diagnosis for mass in aural canal with facial nerve palsy includes external auditory canal cholesteatoma, neoplasms of external canal and malignant otitis externa. It is important to distinguish the diagnosis, as management of each of the differential diagnosis is notably different. Hence, thorough and detailed history, physical examination, radiological examination and most importantly histopathological examination is crucial prior to a diagnosis. Histopathological examination of the biopsied or excised mass is the main modality of diagnosis, more so when there is an atypical or rare presentation.As for management, keratosis obturans usually requires frequent aural toileting to remove the keratin plug and topical medication. This may be done under general anaesthesia especially among non-cooperative patients. Split skin graft and canalplasty method have also been reported for refractory keratosis obturans.Albeit commonly regarded benign, keratosis obturans may result in devastating complications including facial nerve palsy as in our patient. Hence, high clinical suspicion and prompt management among clinicians are of dire importance as absence of typical clinical features usually lands both the attending physician and patient in dilemma.The authors declare no conflicts of interest.


To date, this is the only report showing with close and consecutive magnetic resonance images the extremely rapid response of two types of pediatric low-grade gliomas (PLGG) to vemurafenib and its impact on the surgical approach.We report two cases of symptomatic PLGG treated with vemurafenib, a BRAF inhibitor: in a 12-year-old girl it was used as first-line medical treatment, reducing the tumor by 45% within a month and stabilizing to 76% after a year; in a 3-year-old boy with no improvement after SIOP LGG 2004 Protocol, vemurafenib induced in only one week a 34% shrinkage and solved the hydrocephalus, avoiding surgical operation.Our cases demonstrate how an early molecular diagnosis of BRAF mutations through the neurosurgical biopsy is essential to promptly start targeted therapies., whose effect can influence both therapeutic and surgical decisions, hopefully reducing the occurrence of second neurosurgery with associated risks of neurological sequelae. BRAF V600E is an oncogenic driver mutation that results in a loss of inhibition and gives BRAF oncogenic potential. PLGGs with this variant show a good response to targeted approaches such asvemurafenib (PLX4032), an orally available selective BRAF inhibitor [Pediatric low-grade gliomas (PLGG) are the most frequent brain tumors in children . The mosnhibitor –9, opticnhibitor , desmoplnhibitor , and hignhibitor , 12.BRAF-positive mutant PLGGs and the consequent effect on surgical decisions.Here we describe the extremely rapid radiological response to vemurafenib shown by two BRAF V600E variant, a targeted medical approach was preferred. After specific consent was obtained from our Institution and the family, vemurafenib was started adopting a dose escalating protocol, with initial dose of 480 mg/m2 daily, progressively increased up to 1080 mg/m2 strictly monitoring cutaneous and cardiological adverse effects. In two weeks motor skills clearly improved. A brain MRI performed one month after treatment showed a 45% shrinkage of the mass according to RANO criteria. It raised up to 70% in 5 months and stabilized to 76% after one year . After only 3 days, eyesight improved and a better muscle tone was found. An MRI performed 1 week later showed a 34% volume reduction; ascites decreased too. Brain MRI at two and six months of treatment proved a further shrinkage of 57% and 65% to baseline  is preferred when the lesion is unresectable, gross total resection cannot be achieved, in case of secondary lesions at radiological follow-up, hemorrhagic cysts, or if threatening neurological symptoms occur . The chootherapy , 16.BRAF V600E PLGG once treated with vemurafenib, highlighting how it favorably influenced the subsequent therapeutic and surgical decisions. We aimed to avoid further surgery, in order to reduce the risk of secondary neurological sequelae, as well as limit radiotherapy because of its well-known late effects. Importantly, in both cases a radical surgical approach would have been hard to perform due to the anatomical localization of the masses and the high risk of permanent impairment.We describe the rapidity of both clinical and radiological improvement in two patients affected by In the girl, vemurafenib was used as medical frontline therapy, obtaining a sudden reduction in mass dimension and neurological amelioration in only one month. The younger boy was first treated with conventional chemotherapy for PLGG, but after an initial response, disease progression occurred causing a misfunction of the ventriculoperitoneal shunt, hydrocephalus, and ascites. Also in this case, vemurafenib induced a very rapid clinical, neurological and visual improvement, corresponding to radiological reduction detected by MRI after only six days of treatment, thus avoiding a surgical revision of the shunt. The compliance to therapy was very strict in both patients. Notably, we started with lower doses and adopted an escalating approach, with the aim of testing its tolerability and the reducing occurrence of side effects. Importantly, both the patients presented quick shrinkage already in the first days of therapy, before reaching the full dose of medication, thus raising interest as future field of research in finding its lower effective dose.BRAF V600E is age-dependent, with a predilection for infants and young children (< 3 years of age), tumors have a higher tendency for multicentricity and in some case reports a certain propensity toward a more aggressive behaviour is described [BRAF V600E -mutant diencephalic PLGGs have a more aggressive clinical course, especially in children under the age of 13 years (5 years PFS 9% versus 46% in BRAF wildtype) [Typically, in PLGG the incidence of escribed –20. Ho eildtype) .Several authors have shown the benefits of BRAF inhibition in controlling and reducing LGG progression after first-line treatment , 21, 22,A Phase I Study including 19 patients reported fewer adverse effects in children than in adult patients, for equivalent doses. Of note, only one progressed on therapy, while 4 of the 14 that came off drug progressed in the following year (three within four months) . UnfortuVan Tilburg et al. reported on how BRAF inhibitors can induce tumor mass shrinkage even when used twice in the same subject: the first administration of vemurafenib for desmoplastic infantile astrocytoma needed three months to determine clinical improvement and volume reduction; treatment was discontinued for a year, then restarted after evidence of disease progression. The second cycle took only three weeks to produce a clinical and radiological response .In a recent study on 56 PLGG from multiple countries, objective responses were observed in 80%, after discontinuation 76.5% experienced rapid progression , but upon rechallenge with vemurafenib (± a MEK-inhibitor), 90% achieved an objective response .Other authors have already reported on clinical responses to vemurafenib, but no one assessed an imaging improvement earlier than two months after the start of the therapy , 25.BRAF mutations identification and guide subsequent targeted therapies. Being our first objective the rapid relief of symptoms due to tumor compression, such a remarkable result adds an important experience on the sparing of surgery-related procedures. The main limitations of this report are the ones expectedly pertaining to a case description involving a single or few patients, being PLGG a quite rare condition and vemurafenib having been adopted as a possible therapeutic strategy in pediatric tumors for a relatively short period. However, our cases demonstrate how a prompt radiological response to vemurafenib, and the related clinical improvement, can influence both therapeutic and surgical decisions, hopefully reducing the occurrence of second neurosurgery with associated risks of neurological sequelae.In conclusion, we report the abrupt radiological response , after only 6 days and four weeks of treatment with vemurafenib; the MRI follow-up showed further shrinkage and then stabilization in mass volume. To our knowledge, this is the first report assessing with consecutive MR images such a quick shrinkage in PLGGs treated with vemurafenib, highlighting the importance of an early investigation of BRAF status in all cases of LGG in children. We also wish to underline the pivotal role of neurosurgical biopsy for molecular diagnosis, in order to let a prompt


Pancreaticopleural fistulas (PPF) are a rare etiology of pleural effusions. We describe a case of a 61-year-old man, with left chest pain with six months of progression who presented with a large volume unilateral pleural effusion. A thoracentesis was performed, which showed a dark reddish fluid(exudate) and high content of pancreatic amylase. After that an abdominal computed tomography (CT)and magnetic resonance cholangiopancreatography (MRCP) was done, revealing fistulous pathways that originated in the pancreas. The patient was admitted for conservative and endoscopic treatment by Endoscopic Retrograde Cholangiopancreatography (ERCP) and a prosthesis was placed on a fistulous path. He was discharged without complications, with the resolution of the pleural effusion and fistula.The interest of this case lies in the rarity of the event and absence of symptoms of the probable primary event (acute pancreatitis). The possible iatrogenic association with several drugs of his usual medication makes it even more complex. They occur more regularly in men in the 5th and 6th decade of life with chronic alcoholism.Despite this, any etiologic mechanism of pancreatitis can be associated with fistulization.The fistula usually occurs due to rupture or malformation of a pancreatic pseudocyst, being the rupture of a pancreatic duct a less common mechanism. Although the primary abdominal repercussion, the most frequent symptoms are respiratory. Most commonly they cause unilateral pleural effusions ( > ¾ on the left side) with high concentrations of amylase and pancreatic lipase. The most informative imaging exams are computed tomography (CT), magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrogade cholangiopancreatography (ERCP). As a rule, treatment initially passes through drainage of the pleural fluid, parenteral feeding and decreasing pancreatic secretion . It can also be advantageous to use ERCP as a therapeutic weapon (placement of stents in fistulous pathways). Only when this is not effective or the endoscopic route is technically impossible, it is decided to proceed to the surgical intervention.Pancreaticopleural fistulas (PPF) are rare complications of acute or chronic pancreatitis (approximately in 5%)We describe a case of a 61-year-old man, referred to the Emergency Room (ER) with generalized left thoracic pain . In addition, he reported tiredness in small efforts, anorexia and weight loss . For the mentioned complaints, he had gone to the ER in the first weeks of the condition. At that time, there were no signs of clinical, laboratory or imaging abnormalities and he was discharged without a definite diagnosis; surveillance was indicated.As pathological history, he had type 2 diabetes mellitus, arterial hypertension and mixed dyslipidemia (13 years of evolution), all of those with a good control in the last two years , he returned to the ED due to the reappearance of chest pain. He maintained a large volume pleural effusion, so diagnostic and evacuating thoracentesis was repeated and this time dark brown liquid was drained. On that occasion, pancreatic amylase was added to the analysis, proving to be quite high (2072 U/L). The level of serum of pancreatic amylase (108 U/L) and lipase (116 U/L) were 2 to 3 times above the reference value; transaminase value, liver function parameters and lipid profile (including triglycerides) were all normal.We decided to perform an Abdominal Computed Tomography (CT): at the pancreatic body / tail transition, a collection with 48 x 36 x 46 mm and two drainage paths were observed . Thus, tAt that consultation, an elective hospitalization (3 weeks after the first thoracentesis) for pleural drainage, medical care  and endoscopic (ERCP) treatment was decided. During ERCP, a sphincterotomy was performed and a leak of contrast (high output) was shown in the Wirsung duct; a prosthesis was placed in the proximal region of the evidenced fistulous path . At disc Despite this, existing meta-analyses and systematic reviews do not confirm this increased risk. With this patient, a relationship cannot be ruled out , but he consumed other drugs occasionally described as a cause of pancreatitis, too.The importance of this case lies in the rarity of the event and presentation. The absence of symptoms  of the probable primary event (acute pancreatitis) preceding chest pain makes it more peculiar. Therefore, it is not possible to specify the beginning of the process or the etiology of the probable acute pancreatitis that caused this complication (PPF). Although the most frequent causes  have been excluded, it is difficult to define if pancreatitis was iatrogenic or not. One of the drugs possibly implicated, Empagliflozin , belongs to a class that in recent years has been suspected of having increased risk of pancreatic pathology, with several cases of acute pancreatitis with a possible direct relationship. and is an indicator of poor prognosis , in most cases it disappears after the resolution of the acute event.Although a relevant portion of acute pancreatitis evolves with pleural effusion (up to ¼) As in this case, the levels of amylases of the pleural effusion are frankly elevated . At the imaging level, it is not always possible to visualize the fistula with CT (MRCP is more sensitive). In terms of treatment, there is often resolution only with a conservative approach ; these procedures have a success rate of 30-60%. As with our patient, an endoscopic approach is sometimes decided at the beginning, or it is performed when conservative therapy has not been effective. ERCP allows a therapeutic intervention through the placement of stents to block the fistulous pathways. This less invasive method has shown good results . In this patient, there was no need for surgical intervention , which is associated with a higher risk of complications. Until nowadays, there are no clinical, laboratory or imaging parameters that have shown a predictive ability to assess who most benefits from medical, endoscopic or surgical therapy.  The lack of effective treatment can lead to severe respiratory failure and other complications (such as empyema) with implicated vital risk.As in this case, pleural effusion caused by PPFs is more regularly unilateral (mainly left and large volume), with rapid reappearance after evacuation.With this case, we intend to draw attention to a complication of abdominal pathology with essentially respiratory manifestations, for which a high level of suspicion is needed, especially in patients with risk factors for acute or chronic pancreatitis.None.The authors have no ethical conflicts to disclose. Informed consent was obtained from the patient.None.


Giant paratesticular liposarcoma (PLS) is an uncommon tumour, often misdiagnosed pre-operatively, which presents as a painless scrotal mass. Early detection and prompt surgical management provide the best outcome. We present an 87-year-old patient with gradually enlarging, painless left scrotal swelling. Ultrasound on initial presentation suggested a benign hernia, resulting in an 11-month treatment delay. Computed tomography (CT) thereafter showed paratesticular scrotal mass measuring 14 x 8 x 7cm. Radical inguinal orchidectomy with high ligation of the spermatic cord was performed. Histopathology and cytogenetics confirmed PLS with both de-differentiated and well-differentiated features involving the spermatic cord margin. The patient had rapid progression to fatal lung metastasis within three months of surgery. Our case highlights that any suspicious fat swelling should be investigated thoroughly and excised promptly if paratesticular liposarcoma is suspected, as delayed management gives poor outcomes. Urological sarcomas account for roughly 2.7% of all soft tissue sarcomas, with the paratesticular region being the most common site of origin for such tumours . They arAn 87-year-old patient presented to his General Practitioner (GP) with a small left testicular lump 11 months ago. Ultrasound scan (USS) in September 2020 showed a small left scrotal lump with an area of echogenic tissue traceable cranially into the groin, suspicious for a fat containing hernia. The patient was managed conservatively by the GP as the 'hernia' was small and not causing any pain. Eleven months later, the patient was seen by the general surgeons in the emergency department for suspected incarcerated left inguinal hernia. The patient gave a history of gradually enlarging, painless left testicular swelling with no history of testicular trauma, lower urinary tract symptoms or urethral discharge. On examination, there was a hard lump arising from the upper pole of the left testis, which occupied the whole left hemiscrotal area and displaced the right testis. The upper border of the mass was palpable in the left inguinal area. The overlying skin showed signs of hypervascularity. A repeat doppler USS scan in August 2021 showed a large mass measuring 13 x 9 x 10cm with mixed echogenicity and vascularity attached to the left testes. Based on this finding, the patient was referred to the Urology team for management. The patient’s case was discussed in the radiology-urology multidisciplinary team (MDT). Tumour markers  for testicular cancer were all negative. Due to diagnostic uncertainty, an abdominopelvic CT was performed which showed a left paratesticular scrotal mass measuring 14 x 8 x 7cm  cells and markedly pleomorphic bizarre nuclei were present. Immunohistochemistry showed that the tumour cells were diffusely positive for desmin and vimentin but negative for MyoD1 and myogenin. The morphology and immunohistochemistry supported a liposarcoma with areas of de-differentiation Figure . MDM2 geThe tumour involved the mesothelial surface of the tunica vaginalis and well-differentiated liposarcoma (WDL) was seen in the spermatic cord Figure  right upDue to the rarity and stage of tumour, this was referred to a specialised sarcoma unit for MDT discussion. However, before an outcome was reached, the patient was admitted with left hemi-scrotal swelling and new cutaneous lesions around site of previous orchidectomy, five weeks post-operation. CT chest-abdomen-pelvis showed metastatic lung deposits and local disease progression, with cutaneous recurrences found around left inguinal and hemi-scrotal region. The patient was seen by the oncology consultant whilst an inpatient. Unfortunately, chemotherapy was not a viable option in view of the patient’s frailty. After discussion with the patient and his family, a decision was made to transfer the patient to a hospice for palliative management. The patient died shortly after, with time of diagnosis to death being 101 days.Liposarcoma is a soft tissue malignancy derived from mesodermal tissue embryologically. It is the most common histological type 46%), followed by leiomyosarcoma 20%), histiocytoma 13%), and rhabdomyosarcoma (9%) [%, and rh0%, histi6%, folloExcision specimens of any potential liposarcoma should be carefully observed and sampled, because tumour diagnosis and grading may depend on features that are focal within the specimen. Resections from retroperitoneal/paratesticular sites, in particular, should be thoroughly sampled and assessed to avoid misdiagnosis of DDL as malignant fibrous histiocytomas (MFH) or undifferentiated pleomorphic sarcomas (UPS) which they can morphologically mimic. When compared with UPS or leiomyosarcomas, DDL have a lower tendency toward local recurrence and metastasis . Most ofClinical diagnosis of PLS is difficult as it presents similarly to inguinoscrotal hernia and lipoma in the form of painless left inguinoscrotal mass. Therefore, radiological imaging is required to guide diagnosis. USS can help identify solid, hyperechoic and heterogenous lesions but cannot accurately distinguish lipoma from a small liposarcoma . In our There is no universal consensus on the management of giant PLS due to its rarity. Radical orchidectomy, wide local excision and high ligation of the spermatic cord provide the best outcome . Three-yOur case highlights that DDL is associated with a high rate of local recurrence and metastasis, in comparison to WDL which is of better prognosis. Therefore, any suspicious fat swelling should be investigated appropriately, even if asymptomatic, and excised promptly to prevent progression into DDL.Giant PLS is a rare entity and is often misdiagnosed resulting in treatment delay. The gold standard imaging for PLS is MRI, with definitive diagnosis provided by histopathological studies. Radical orchidectomy and high ligation of spermatic cord should be performed as soon as possible if PLS is clinically suspected, to prevent progression of WDL to DDL. Regular follow-up is needed in cases of DDL in view of its metastatic potential. Chemoradiotherapy can be offered on an individual basis, although the evidence in literature remains limited.


A leiomyosarcoma of the gastrointestinal tract is extremely rare. We report a case of jejunal leiomyosarcoma with intestinal intussusception at the angle of Treitz that was successfully treated with laparoscopic resection followed by intracorporeal reconstruction using a delta-shaped anastomosis.A 54-year-old man was referred to our hospital due to fatigue and loss of appetite. Blood tests showed anemia. Enteroscopy and subsequent enterography using meglumine sodium amidotrizoate showed easily hemorrhagic tumor (10 cm in diameter) in the jejunum just beyond the angle of Treitz. Contrast-enhanced computed tomography revealed jejunojejunal intussusception. Histopathological examination of a biopsy specimen revealed a leiomyosarcoma. Laparoscopic resection of the tumor without reduction of the intussusception was performed. The resected line of the proximal intestine was very close to the ligament of Treitz in the present case. Intracorporeal jejunojejunostomy was completed using a delta-shaped anastomosis, wherein anastomosis was performed between the posterior walls of the proximal and distal jejunums after minimal mobilization around the ligament of Treitz. The patient’s postoperative course was uneventful, and he was discharged at 10 days postoperatively. No recurrence has been observed within 2 years after surgery.We present a case in which a totally laparoscopic surgery for leiomyosarcoma located at the angle of Treitz with jejunojejunal intussusception was performed successfully. A leiomyosarcoma (LMS) of the gastrointestinal (GI) tract belonging to soft tissue sarcomas is extremely rare and accounts for approximately 1% of malignant mesenchymal tumors in the GI tract .A 54-year-old man with loss of appetite and fatigue 3 weeks prior was admitted to our hospital due to vomiting and anemia. His medical history included depression and arrhythmia. He had no family history of malignant neoplasia. During presentation, he had tachycardia with a heart rate of 95/min was present, but his other vital signs were within normal limit. Abdominal examination showed normal findings with no palpable mass or tenderness. The blood tests showed a low hemoglobin level 6.2 g/dL) with microlytic anemia, whereas the levels of carcinoembryonic antigen and carbohydrate antigen 19–9 were within the normal range. An esophagogastroduodenoscopy showed no obvious abnormalities. A double-balloon enteroscopy and enterography using meglumine sodium amidotrizoate showed a large circumferential tumor located in the jejunum just beyond the angle of Treitz  of 1:50–65 , 8, and Although the number of reports on the LMS of the GI tract has been limited, the number of reports of minimal invasive surgery for the LMS of the GI tract have been recently increasing with favorable outcomes. A PubMed search using the key words “leiomyosarcoma” and “laparoscopic surgery” since 1998 revealed six case reports (seven patients) of laparoscopic resection of the LMS in the GI tract , 15–17. Contrarily, there have been some reports of totally laparoscopic surgery for tumors of the angle of Treitz , 18. TanIn the present case, we performed tumor resection without reducing intussusception. Although it is controversial whether the intussuscepted intestine is reduced before resection for malignant tumors concurrent with the intussusception, many studies suggest that the reduction procedure can potentially increase the risk of unfavorable outcomes, which are as follows: (1) intraluminal seeding and venous tumor dissemination, (2) perforation and seeding of microorganisms and tumor cells to the peritoneal cavity, and (3) anastomotic complications of the manipulated friable and edematous bowel tissue –23. In cWe present a case in which a totally laparoscopic surgery for leiomyosarcoma located at the angle of Treitz with jejunojejunal intussusception was successfully performed. In the present case report, this experience is too less to prove the procedure efficacy. Therefore, further studies and investigations to assess the safety and long prognosis of laparoscopic surgery for LMS at the angle of Treitz are necessary.


Schwannomas are most commonly located on the VIII CN(80%). They are more rarely found in the nasal region (4%). On the nose, they may rise from the autonomous nervous fibers or from branches of the V CN. Malignant transformation is rare 2%). According to Hillstrom%. AccordNasal schwannomas may present progressive unilateral nasal obstruction, with or without epistaxis, hyposmia and headaches. On physical examination we usually only see a grayish mass in the nasal cavity. It may resemble a vegetative lesion or polyp, much vascularized and bleeding excessively. Nasofibroscopy and CT scan are necessary in order to assess tumor extension and surgical approach. Histopathology provides the final diagnosis. Treatment is based on total tumor excision. Pre-operative biopsy helps in the differential diagnosis of other nasal-born tumors.18 year old woman with volume increase in the nasal pyramid to the right side and recurrent epistaxis that ended spontaneously for 6 months, nasal obstruction and hyposmia. Anterior rhinoscopy showed a purple-like soft mass, painless to palpation, totally occluding the right nasal cavity. Paranasal cavities CT scan showed a highly vascularized lesion on the anterior region of the nasal cavity that did not extend to the nasopharynx. Biopsy was carried out in an outpatient basis and the pathology exam diagnosed inflammatory polyp with metaplastic cover. A lateral rhinotomy was carried out with tumor exeresis and cauterization of the resection bed. Histopathology exam of the surgical specimen proved it to be a benign nasal schwannoma. After 18 months there was no evidence of local recurrence.There are about 70 reports of nasal schwannomas in the literature. Most in adults between forty and sixty years of life, while our patient is 18 years old. According to HasegawaSince it is a rare tumor, just like in literature reports, diagnosis suspicion of nasal schwannoma was not considered at first, such diagnosis was confirmed by histology.


Maxillary myxomas are rare benign odontogenic tumors with a high potential for infiltration and destruction. Clinical and radiological manifestations are variable and non-specific and often lead to confusion with other benign and malignant lesions. We present here two cases of odontogenic myxoma of different localization (maxilla and mandible). In both cases, the patient presented with a progressively enlarging facial swelling without any neurologic disturbance or lymph nodes. On computed tomography, the lesions appeared as a large unilocular, homogeneous image causing a cortical thinning and rupture along with repression of surrounding tissues. The clinical and radiological features were poorly suggestive of precise nature, and only histological examination of biopsy specimen was able to reveal the diagnosis of myxoma. Given the large size of the tumors and the locally aggressive pattern, we have opted for large maxillecetomy and marginal mandibulectomy. In conclusion, myxoma is a slow growing but locally aggressive tumor that the clinician should keep in mind. As there is a lack of consensus regarding their treatment, we can suggest a conservative treatment for small non-extensive lesions, which consists of enucleation and curettage of the tumor; and a radical excision in case of large tumors given the high risk of recurrence. Maxillary myxomas are rare benign odontogenic tumors of mesenchymal origin with a high potential for infiltration and destruction . Their CPatient information: a 25-years-old patient, with no particular pathological history, consulted for right cheek swelling that had been evolving for 1 year, without ocular or neurological signs or nasal obstruction.Clinical findings: clinical examination showed facial asymmetry, due to non-painful swelling of the right cheek, fixed to underlying structures, with healthy covering skin. The intrabuccal examination showed a gingival mass located in the upper left premolar-molar region measuring 4 cm on the long axis, occupying the corresponding vestibule area and extending to the palatal side (tal side . The lesDiagnostic assessment: the facial computed tomography revealed a unilocular osteolytic image of the upper maxilla, of a tissue density crossed by elongated and thin septa. The mass caused a cortical thinning of maxillary sinus walls with rupture and pushed back the surrounding soft tissues ( tissues . In fronTherapeutic intervention: given the large tumoral size, a right maxillectomy was performed under general anesthesia via a para-latero-nasal approach. Histological examination of the surgical specimen revealed a mesenchymal proliferation made up of stellate cells arranged on a myxoid background.Follow-up: the postoperative course was without complications.Informed consent: it was obtained from the patient.Patient information: a 35-years-old patient, with no particular pathological history, presented with progressively increasing swelling on the right side of the mandible.Clinical findings: clinical examination showed slight facial asymmetry. The intraoral examination revealed swelling extending from tooth 46 to the ramus covered by healthy-looking skin. The mass occupied the corresponding vestibule and caused lingual displacement. The covering mucosa looked normal (d normal . Upon paDiagnostic assessment: the panoramic X-ray discovered a relatively well-defined bilocular radiolucent image, directly located above tooth 46 and extending to the ramus. On facial computed tomography, the lesion appeared as a homogeneous bone formation with regular contours of the right half mandible, involving the horizontal branch and the lower 2/3 of the ramus. The mass caused a thinning and rupture of both inner and outer cortical.Therapeutic intervention: given the aggressive and locally destructive aspect of the tumor, a marginal mandibulectomy was performed using a trans-labial approach (approach . The anaFollow-up: two months after, the patient presented with a pathological fracture of the right angle fixed by a manipulation. As the follow-up showed no complications, a bony graft from the iliac crest was performed six months later.Informed consent: it was obtained from the patient.Myxoma is a benign, rare and locally invasive mesenchymal tumor, composed of stellate and spindle-shaped cells sitting in a myxoid stroma, with some collagen fibers; when the fibers are more abundant, it is called fibromyxoma . It reprRadiologically, it results in a clear, unilocular or multilocular radio image, with a “soap bubble” appearance. The bony septa located inside the lesion are thin and elongated. The cortical bone is thinned and deformed. In advanced lesions, this cortex is ruptured, leading to invasion of the adjacent soft tissues. The lesion pushes back the teeth, whose roots are rarely eroded. A computed tomography examination is essential to determine the modalities of the surgical intervention according to the three-dimensional topography of the lesion and its connexion to adjacent structures. On magnetic resonance imaging, myxomas have a variable, often heterogeneous signal. A hyposignal in T1 and hypersignal T2 is very evocative but inconstant ,8. MacroThe diagnosis of myxoma is histological, the appearance of these tumors is characterized by the presence of round and angular cells; basophilic cytoplasm, scanty, with a small hyperchromatic nucleus; disseminated in an abundant myxoid stroma containing mucopolysaccharides and a network of reticular fibres . For somRecurrence is more frequently observed in the two years following the surgical treatment; hence the importance of regular and prolonged postoperative clinical and radiological monitoring. A six-monthly rhythm for five years is advocated.The diagnosis of myxoma can be suggested based on clinical and radiological data, but the confirmation is histological. Although this lesion is rare, it should not be overlooked. It´s a slow-growing but locally aggressive tumor since it is a poorly circumscribed non-encapsulated tumor. Its treatment is exclusively surgical; we can suggest a conservative treatment for small non-extensive lesions, which consists of enucleation and curettage of the tumor; and a radical excision in case of large tumors.


The abdominal wall is the location of a wide spectrum of pathological conditions, from benign to malignant ones. Imaging is often recommended for the evaluation of known palpable abdominal masses. However, abdominal wall pathologies are often incidentally discovered and represent a clinical and diagnostic challenge. Knowledge of the possible etiologies and complications, combined with clinical history and laboratory findings, is crucial for the correct management of these conditions. Specific imaging clues can help the radiologist narrow the differential diagnosis and distinguish between malignant and benign processes. In this pictorial review, we will focus on the non-neoplastic benign masses and processes that can be encountered on the abdominal wall on cross-sectional imaging, with a particular focus on their management. Distinctive sonographic imaging clues, compared with computed tomography (CT) and magnetic resonance (MR) findings will be highlighted, together with clinical and practical tips for reaching the diagnosis and guiding patient management, to provide a complete diagnostic guide for the radiologist. Benign abdominal wall lesions comprise a wide spectrum of conditions, including benign masses, tumors, and mimickers of neoplastic conditions. Abdominal wall masses generally present as unexpected incidental findings during radiological examinations performed for other clinical reasons, most commonly during computed tomography (CT), and therefore usually represent a clinical dilemma. In many cases, the likely diagnosis can be reached by combining clinical history  and imaging features . HoweverUltrasonography (US) is commonly the first-line exam performed for palpable superficial lesions, providing information on echogenicity, gross vascularization, relationships with nearby vessels, and elasticity. US is also usually used to guide interventional procedures and biopsies. Computed tomography (CT) provides anatomical information on mass extension and involvement of deeper abdominal wall structures, and it is usually preferred in emergency settings or abdominal traumas. Magnetic resonance (MR) is a second-line examination that helps understand the content and nature of a mass and is also useful in selected cases of benign non-neoplastic diseases that require further diagnostic assessment, such as endometriosis .The differential diagnosis among abdominal wall masses can be performed by adopting a clinical–radiologic flowchart. The diagnostic flowchart first includes the differentiation between a mass and a mass-like process. Then, the internal composition of the mass should be determined . Then, according to the main components, laboratory results, and clinical history, a differential diagnosis should be made. Based on the origin, abdominal non-neoplastic diseases include infectious processes , congenital conditions , acquired vascular conditions , and storing and miscellaneous diseases  ,2,3,4.This pictorial review aims to depict the spectrum of benign non-neoplastic diseases that may be encountered in the abdominal wall, providing tips for differential diagnosis and their management. For each case, key imaging aspects will be highlighted, drawing a diagnostic guide for the radiologist.Infective conditions present as recently appearing lesions accompanied by clinical signs suggestive of inflammation and infections, such as fever, increase in white blood count or C-reactive protein, and redness. Surgical history has an important role, as does the presence of risk factors such as immunocompromised statuses or open wounds .Abdominal wall abscesses can manifest as an extension of intra-abdominal processes or as primary infections ,5,6,7. RAbdominal wall abscesses present on US as superficial fluid-filled complex lesions, with ill-defined margins, predominantly hypoechoic to surrounding muscles ,8. PeripOver time, an abscess becomes walled off by vascularized connective tissue, which corresponds to an enhancing rim, best seen on the venous phase . The enhNecrotizing fasciitis (NF) is a soft tissue necrotic infection that involves the fascia and subcutaneous tissue . If not On CT, the most suggestive findings of NF are the thickening of the fascia and a large amount of subcutaneous gas, although the latter is not specific ,11,12 F.Focal or diffuse non-enhancing areas ,11 with On MR, high-signal-intensity areas may be observed on T2-weighted sequences, representing necrosis and edema, alternated with intralesional gas. On T1-weighted sequences, loss of muscle texture and possible high signal intensity compatible with intramuscular hemorrhage might be seen ,11. A neGranulomas are protective responses to destroy or sequester particles that are deemed harmful to the body, frequently seen in the context of chronic infections, inflammatory diseases, and foreign bodies. On US, retained foreign bodies are usually hyperechoic with posterior acoustic shadowing, although their appearance depends on their content . As granGossypibomas are secondary to the retention of surgical sponges or gauze. The abdominal wall is a rare localization, but this diagnosis should be suspected in post-surgical patients with recurrent infection, fever, and pain. On CT, abdominal wall gossypibomas are thick-walled collections in the abdominal muscles or subcutaneous fat, with an air-like density center and a small fluid component, sometimes with metallic parts .In both conditions, until the foreign body is surgically removed, the inflammation will continue. If unrecognized, they can evolve into systemic infection and septic shock .Enterocutaneous abdominal wall fistulae (ECFs) are abnormal connections between a bowel loop and the nearby skin that may be secondary to abdominal surgery or inflammatory bowel diseases. ECFs can either appear collapsed or patent with gaseous or fluid content (“tram-track” appearance) .Abdominal wall fistulae are seen as hypoechoic or anechoic duct-like structures on US but are better studied on MR or CT, as these latter depict the connection between the bowel loops and the peritoneum or the cutis .Fistulae need to be surgically repaired; otherwise, chronic infections may develop. Abscesses can indeed complicate ECFs: lesions above 2 cm, irregularly large, with thickened walls and patent peripheral sections should raise suspicion for superimposed abscesses .Urachal anomalies are due to an incomplete obliteration of the urachus, the embryologic communication between the bladder and the umbilicus ,18. AccoThe differential diagnosis includes malignancies, metastases, and endometriosis. Usually diagnosed during childhood, they are commonly studied on US.In the case of patent urachus, US shows a tubular structure with hypoechoic walls and anechoic content, traced along the anterior abdominal wall ,19. UracUmbilical–urachal sinus occurs at the umbilical end of the urachus and is seen as a fusiform outpouching structure into the abdominal wall. Imaging reveals a thickened, fusiform blind dilatation below the navel, with no communication with the bladder. Superinfection can complicate all urachal anomalies, and in that case, peripheral contrast enhancement will be seen . Stone fThe most common congenital defect of the abdominal wall is the absence of transversus abdominis muscle, followed by internal oblique and external oblique absence , which mNeuromuscular dystrophies can also present defects of the abdominal wall. Duchenne muscular dystrophy is the most common one and is clinically characterized by progressive muscle weakness, starting from the proximal pelvic girdle , whose pArteriovenous malformations (AVMs) are congenital lesions consisting of a tangle of arteries and veins connected by one or more fistulae ,24.It is important to distinguish between isolated and syndromic AVMs  .AVMs of the abdominal wall are often unilateral, interdigitated with fat, and adjacent to or within the anterolateral muscle groups . Color DAcquired shunts in the abdominal wall might be a sign of portal hypertension or inferior vena cava obstruction, which are both conditions to assess and not underestimate. Indeed, if untreated, the first one may lead to cirrhosis and hepatocellular carcinoma, and the latter can lead to pulmonary embolisms. Acquired shunts are caused by dilatation of the paraumbilical veins within the round ligament ,27,28.The paraumbilical veins will connect the superior and inferior epigastric veins in the rectus sheath with the left branch of the portal vein at the umbilicus , with anCT should be preferred, in order to study all of the other signs of portal hypertension . TreatmePseudoaneurysms, or false aneurysms, originate from the disruption of intimal and medial layers of an arterial vessel, after traumas, infections, or iatrogenic procedures ,31,32. OCT angiography is the gold standard for the diagnosis, as it precisely depicts the vascular structures involved  and demoAll pseudoaneurysms should be regarded as surgical urgencies, as they are considered at risk of rupture and therefore should be promptly treated to avoid bleeding . TreatmeAbdominal wall hematomas are a common—though often misdiagnosed—cause of acute abdominal pain ,35. TheyAbove the arcuate line, RSHs are circumscribed and spindle-shaped ; below tHematomas are well-defined masses, with higher attenuation than muscles  on unenhThe main consequence of undiagnosed or untreated rectus hematomas is pain and bleeding, which can become life-threatening. Severe bleeding should be promptly identified and aggressively treated with urgent arterial embolization or surgical intervention. Another potential complication is abscess formation, as in any blood collection that is not drained .Injection granulomas may develop as an inflammatory response to repeated injections and lead to localized fat necrosis, scar formation, and dystrophic calcification, simulating malignant diffuse processes ,11,12,13On CT, granulomas are well-defined small nodules with soft-tissue attenuation, often with peripheral calcifications . MR showFree silicone and paraffin injections are dangerous beauty techniques, disused but still performed illegally . SeriousFree silicone and paraffin are seen as irregularly scattered collections extended along fascial planes, mimicking sarcomatous or metastatic processes ,39.On US, they are heterogeneously echoic, with marked posterior acoustic shadowing . On CT, MR appearance comprises a main, plaque-like, ill-defined lesion with a low-to-intermediate signal on T1- and T2-weighted images, but also marked loss of signal on fat-suppression and dual gradient echo sequences in paraffinomas . Even yeLipohypertrophy is a classical sequela of subcutaneous insulin injections. On CT and MR, it is seen as focal subcutaneous soft-tissue masses, with fat proliferation in a symmetric fashion  and calcManagement is different between these two conditions: while lipohypertrophy disappears with the suspension of insulin therapy, insulin balls tend to progressively enlarge and require surgical excision . RepeateSystemic alterations in calcium metabolism can lead to extensive subcutaneous calcinosis , either Calcinosis Universalis is the main subtype seen in collagen vascular diseases and is recognized on CT as sheet-like deposits of calcifications in the subcutaneous tissue and fascial planes, mostly with intramuscular distribution ,45 Figu.Currently, we lack specific guidelines for managing calcinosis cutis in autoimmune connective tissue disorders. An intensification of immunosuppressive therapy was suggested, but no specific treatment was recommended. Rituximab and bisphosphonates have been increasingly used with success in dermatomyositis skin lesions . SurgerySplenosis is the heterotopic auto-transplantation of splenic tissue that occurs after a traumatic or surgical rupture of the spleen; it is usually asymptomatic and without any degeneration risk . The abdUS appearance is non-specific as it shows round or oval-shaped, hypo- to isoechoic solid masses with posterior acoustic enhancement and an incomplete hyperechoic rim . CT and Abdominal wall endometriosis (AWE) manifests as solid nodules, with occasional cystic changes due to intralesional bleeding . AWE oriUS demonstrates heterogeneously hypoechoic, round or oval-shaped nodules or masses, with scattered internal echoes, and small cystic areas ,50,53,54CT shows solid masses with ill-defined borders, isoattenuating to the surrounding muscles . After cThe clinical presentation is heterogeneous. No particular constellation of clinical risk factors has been identified, and the histological report is the major diagnostic tool for confirmation. Imaging including ultrasound and magnetic resonance imaging can assist with the localization of the lesions and aid in surgical excision, which is the first line of therapy . AWE lesAdhesions at the level of the abdominal wall after surgery may occur in about 39% of patients at risk for adhesions . Indeed,The spectrum of benign non-neoplastic abdominal wall diseases is wide. Some benign conditions are easily diagnosed and, in most cases, do not require any further diagnostic workup or follow-up. However, some cases can be challenging, may require further diagnostic assessment, and may need proper urgent treatment or follow-up. Radiologists must be aware of the key imaging features that allow a prompt differential diagnosis among these different diseases, to avoid unnecessary follow-up or overtreatment on one hand and to avoid misdiagnosis or delayed treatment on the other hand. Even though in some cases histology will still be needed to reach the final diagnosis, the role of the radiologist is fundamental to curtail the differential diagnosis and correctly interpret these processes non-invasively.


This case report describes the diagnosis, management and outcome of three dogs with peripheral nerve sheath tumors (PNSTs) involving the brachial plexus, C7 (case 1), C8 (case 2), and C8 and T1 (case 3) spinal nerves and nerve roots with intrathoracic invasion. Surgical resection required thoracic limb amputation and removal of the first rib, facilitating a novel lateral approach to the spinal nerves and foramina in all cases. This was followed by hemilaminectomy and rhizotomy in cases 1 and 2. Adjunctive radiotherapy was then performed in all dogs. All three dogs regained a good quality of life in the short-term following surgery. Two were euthanased after 3 and 10 months, following detection of a pulmonary mass in one case and multiple thoracic and abdominal masses in the other. The third dog was alive and well at the time of writing (7 months post-surgery). This surgical approach facilitated good access and allowed gross neoplastic tissue to be resected. The ease of surgical access was dependent, to a degree, on the size of the patient. This surgical approach can be considered in cases of PNSTs involving the caudal cervical or cranial thoracic spinal nerves and nerve roots. Adjunctive radiotherapy should be considered as part of a multi-modal approach to these challenging tumors due to the difficulty of achieving clean margins, particularly proximally, even with optimal surgical access. Peripheral nerve sheath tumors (PNSTs) are malignant mesenchymal tumors arising from the myelin sheath or connective tissues surrounding nerves. Historically, terms such as Schwannoma and neurofibroma have been used , 2. Sincvia CT . The dog was reported to be bright and happy at home for a further 6 weeks, before acutely becoming painful. There were no obvious neurological deficits on re-examination, but the dog was painful on being handled. Thoracic radiographs were performed which revealed a single 2.5 cm diameter well-defined soft tissue opacity in the right middle lung lobe most consistent with a neoplastic mass. The owner declined further investigations including advanced imaging and biopsy of the mass and instead requested euthanasia, declining post-mortem.A 7-year old, male entire, English Springer Spaniel weighing 20 kg presented with a 10 month history of progressive left thoracic limb lameness unresponsive to meloxicam and gabapentin. Examination revealed non-weight bearing lameness of the left thoracic limb. In this limb, the postural reactions were absent, the withdrawal reflex was incomplete and the muscle mass was markedly reduced. The cutaneous trunci reflex was present on the right and absent on the left. Miosis and third eyelid protrusion was seen in the left eye . A firm, round, painful mass was palpable in the left axilla. Hematology and biochemistry profiles were unremarkable.Pre-referral CT of the neck, thorax, and elbows had revealed the presence of an ovoid, 8 × 7 cm diameter soft tissue attenuating mass in the left axilla, extending to the thoracic inlet where it displaced the trachea, esophagus, common carotid, and left subclavian arteries. It surrounded the left axillary artery and circumference of the first rib, where it was causing cortical lysis and periosteal reaction. The C8 spinal nerve was thickened and was extending into the vertebral canal where it was indenting the spinal cord . The axiForequarter amputation and first rib removal were performed in the same manner as in case 1. The mass was followed proximally and the abnormal C8 spinal nerve identified, prior to transecting it and removing the distal part of the mass and the limb. The approach to the hemilaminectomy site was as in case 1. A left C7–T1 hemilaminectomy was then performed. The enlarged C8 nerve roots were resected ~1–2 mm distal to their attachment to the spinal cord, which appeared macroscopically normal. Lavage, chest drain placement and closure were performed as in case 1.2/48 h) and meloxicam (0.1 mg/kg/24 h) was started 3 months post-surgery. The dog was reported to be well, without obvious abnormalities, for 9 months, before becoming acutely dyspnoeic. Investigations revealed multiple thoracic and abdominal masses, which were not sampled, leading to euthanasia.The dog was comfortable the day following surgery and was discharged after 5 days. At 2-week re-examination there were no neurological abnormalities present and the Horner syndrome had resolved. Definitive intent radiotherapy was then performed (49.4 Gy over 19 daily fractions). Adjunctive metronomic chemotherapy  and prednisolone (0.5 mg/kg/24 h).Pre-referral radiographs of the thorax, cervical spine, and thoracic limbs were normal. MRI of the cervical and cranial thoracic vertebral column, spinal cord and brachial plexuses revealed that the right C8 and T1 nerve roots, spinal nerves, and brachial plexus were enlarged and irregularly marginated, were STIR hyperintense, mildly T2 hyperintense and T1 isointense compared to normal muscle, and showed mild contrast enhancement . The abnForequarter amputation and first rib removal were performed in the same manner as in case 1. The enlarged, hard and nodular brachial plexus and C8 and T1 spinal nerves were identified and followed proximally, then transected, allowing removal of the distal part of the mass. The approach to a planned C7–T2 hemilaminectomy was similar to that described in case 1, though the size of the patient made muscle dissection and retraction challenging. The C7–T2 vertebrae were identified and the hemilaminectomy was commenced but during drilling significant hemorrhage occurred from the vertebral venous sinus. The drilling of the hemilaminectomy window was incomplete at this stage and it was thought that the sinus was displaced due to the presence of the mass. Bleeding was controlled using bone wax and fibrillar collagen (Lyostypt). Given this complication, the decision was made to discontinue with the hemilaminectomy and transect the abnormal nerves as close as possible to their entry to their respective foramina. Lavage, chest drain placement and closure were performed as in case 1.The dog was comfortable the morning following surgery and was discharged after 4 days. At 2-week re-examination there were no neurological abnormalities present. Definitive intent radiotherapy was performed (50 Gy over 20 daily fractions). At time of writing, 7 months after surgery, the dog was coping well on three legs without any other abnormalities.All three masses showed similar histopathological features. All were moderately cellular and composed of fusiform cells arranged in intersecting bundles, streams and whorls, supported by a moderate amount of fibrovascular stroma. Neoplastic cells had indistinct borders, a small to moderate amount of pale eosinophilic cytoplasm, ovoid nuclei with finely stippled chromatin and 1–2 distinct nucleoli. There was moderate anisocytosis and anisokaryosis with 4–19 mitotic figures in 10 400 × fields. There was individual cellular and regional necrosis. There were areas of myxomatous fibrovascular stroma and peripheral infiltration of lymphocytes. In cases 1 and 3 the mass was unencapsulated whereas that in case 2 was encapsulated. In cases 1 and 2 neoplastic cells were seen extending to the borders of the nerve root samples. In case 2 neoplastic cells were also seen in adipose tissue surrounding the nerve roots. In case 3, neoplastic cells were separated from the peripheral tissue borders by a rim of adipose tissue, ~1 mm in width, with no neoplastic cells at the proximal border. The histopathological diagnosis in all three cases was malignant PNST.This report demonstrates the feasibility of a lateral approach to remove PNSTs involving the C7, C8, and T1 spinal nerve and nerve roots, with intra-thoracic invasion. Crucial to the procedure is the input of a multidisciplinary team for limb amputation, thoracotomy and rib removal. Patient positioning and tilting of the table was key as it allowed optimal, adaptable positioning throughout the procedure. The lateral approach facilitated good access, particularly in the two smaller patients, allowing gross tumor resection and in case 3, complete removal.The objective of the surgery in all cases was to achieve resection of as much of the PNST as possible, while recognizing that complete resection would be very challenging, particularly in cases with spinal cord involvement. Hence, adjunctive radiotherapy was recommended. We sought to follow the principle of oncological surgery of reducing tumor burden as far as possible, thereby increasing the efficacy of non-surgical adjuvant therapies intended to eliminate microscopic disease, and consequently reducing the likelihood of local spread or widespread metastasis . In caseThis surgical procedure was planned to permit optimal access for efficacious tumor removal. It did not, however, lead to complete proximal resection in cases 1 and 2. It is possible that performing a durotomy would have allowed more neoplastic tissue to be removed. Durotomy in these cases, while possible, would be challenging as the extent of the hemilaminectomies had to be relatively conservative. This was due to limitations imposed by muscle mass and vascular anatomy. The vertebral venous sinuses were likely to be displaced due to the enlarged infiltrated nerve root and had to be avoided. The vertebral artery and vein located within the transverse foramen of C6 were another impediment to ventral access . DurotomDespite the decision not to proceed with a hemilaminectomy in case 3, this was the only tumor that was completely removed. On the basis of the MRI, nerve root involvement was thought possible and the clean histological margins were unexpected. It is possible that MRI is relatively non-specific for detection of nerve root involvement. Abnormalities on electromyography (EMG) of the epaxial muscles in dogs with PNSTs have been shown to be significantly associated with nerve root or spinal cord involvement . InterprIn order to remove the thoracic limb to permit access and visualization for the hemilaminectomy, the affected spinal nerve had to be transected, contrary to the broad principle of oncological surgery of avoiding incision into macroscopic tumor  which coDue to the difficulty of clean resection, radiotherapy may be valuable in cases of PNST with involvement of the vertebral canal. Radiotherapy could be considered even in cases with clean histopathological margins, due to the typical narrow margin of resection and the possibility of “skip” lesions . These aThe immediate post-operative outcome in all cases was favorable as relief of pain occurred very quickly in both painful dogs (cases 1 and 2) and case 3 has continued to enjoy an excellent quality of life. In case 1 and 2, good quality of life was achieved but this was only of relatively short duration due to the development of masses in the lungs (cases 1 and 2) and abdomen (case 2). The disease-free and survival times in these two cases fell in the range that have been previously reported for surgery  and radiIt could not be determined whether the new masses in cases 1 and 2 were related to the PNSTs, as post-mortem, and histopathology were not performed. While PNSTs are typically described as of low metastatic potential, distant metastases have occasionally been reported , and conOther possible differential diagnoses for the brachial plexus lesions in these cases included neuritis and lymphoma. Neuritis was thought unlikely as this would not typically be associated with intrathoracic involvement as seen in cases 1 and 2. In case 3, the clinical signs progressed despite corticosteroid administration, making inflammatory disease less likely. Lymphoma was also a possibility, though brachial plexus lymphoma appears to be very rare in dogs. It may occur as part of a more widespread neoplastic disease . In our At our clinic, chemotherapy has generally not previously been employed for PNSTs, due to assertions , 4 that Very little information is available regarding chemotherapy for canine PNST. Some response has been reported in isolated cases treated with nitrosylcobalamin , and cycIn conclusion, the reported cases demonstrate the feasibility of a lateral approach for resection of PNSTs involving the C7–T1 spinal nerves. The completeness of tumor resection, as well as disease-free and survival times, was variable. Two of three cases developed presumed metastasis or secondary neoplasia, which was unexpected considering previously available data on PNST. The benefit of radiotherapy in addition to surgery remains to be confirmed. A larger study is required to determine the efficacy of this treatment regime for canine PNST.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the animal study because this report provides only a description of clinical care provided to patients according to best practice. Owners consented for data related to the patients to be published. Written informed consent was obtained from the owners for the participation of their animals in this study.AU, OM, and NS were involved in the surgeries. SM planned and oversaw radiotherapy. AU, OM, NS, and SM involved in the assessment and management of the cases reported. All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.Linnaeus Veterinary Limited supported the costs of the open access publication charges.All authors were employed by company Linnaeus Veterinary Limited. The handling Editor declared a past co-authorship and past collaboration with one of the authors, OM.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Dentigerous cysts are benign, slow-growing odontogenic cysts that are considered to be developmental in origin. They are twice as common in men than in women, and most often occur in people between the ages of 20 and 40 years. They are rarely found in young children. They almost exclusively occur in permanent dentition and over 70% of total number of cases are located in the mandible. Dentigerous cysts most commonly involve the mandibular third molar and are infrequent in maxillary canines. They can grow to a considerable size causing painless expansion of the jaw leading to its deformity.We report a rare and challenging case of ectopically erupted massive dentigerous cyst of maxillary canine in the anterolateral wall of left maxilla leading to facial deformity and shed a light on its surgical management by the endonasal endoscopic approach in a female pediatric patient. Dentigerous cysts are benign, slow-growing odontogenic cysts that are considered to be developmental in origin. They are more than twice common in males than in females and frequently occur in individuals between 20 and 40 years of age and are seldom discovered in young children ,2.They almost exclusively occur in permanent dentition and over 70% of total number of cases are located in the mandible. The most involved teeth in descending order of occurrence are the mandibular third molars, maxillary third molar, maxillary canine, and mandibular second premolars . DentigeA sine qua non for the development of the cyst is the hydrostatic force exerted by the accumulation of fluid between reduced enamel epithelium and the tooth crown of unerupted teeth which encloses the crown and is attached to the neck at the cementoenamel junction . DentigeWe report a rare and challenging case of ectopically erupted massive dentigerous cyst of maxillary canine in the anteromedial wall of left maxilla leading to facial deformity and shed a light on its surgical management by the endonasal endoscopic approach in a female pediatric patient.A 12-year-old female was referred to our hospital with a complaint of left facial swelling adjacent to the nose in the nasofacial region, of two years duration, painless, and was gradually increasing in size. The child was initially treated with a course of antibiotics at the previous hospital. Following unresponsiveness of the swelling to the initial medical treatment, an x-ray paranasal sinus was advised which revealed a radiopaque mass in the left maxillary sinus near the floor of the orbit with cloudy appearance of the sinus.On examination, the child was asymptomatic. There was evidence of a single, smooth, hard swelling approximately 1x2 cm in size, non-tender on palpation, and not adherent to the overlying skin present over the left maxillary sinus in the left infraorbital region just lateral to the nose in the nasofacial region . The post-operative recovery was uneventful with significant reduction in the size of external facial swelling.Dentigerous cysts, also known as follicular cysts, are the second most frequent odontogenic cysts after those associated with the roots of the teeth , originating from the weakened enamel epithelium during tooth crown development. They are most frequently found in the mandible (70%) than the maxilla (30%). The incidence is 14-20% and is slightly more prevalent among males (1.6:1) occurring in the second and third decades of life [A dentigerous cyst is frequently asymptomatic and hence may go undiagnosed for several years ,4. It isAn average follicular space is between 3 and 4 mm, when it exceeds 5 mm, a dentigerous cyst is anticipated. Dentigerous cysts can grow to a considerable size and an extensive lesion can cause facial asymmetry .The extensive literature review undertaken by Marino et al. describes numerous applications of the endoscopic approach for the treatment of dentigerous cysts, with or without ectopic teeth, and reported no associated significant complications or recurrences with these procedures . EmanuelAlthough the Caldwell-Luc procedure gives direct visualization to the maxillary sinus, it is associated with greater risk of complications and morbidities including damage to the sinus mucosa, retraction of the soft tissues of the cheek, oroantral fistula, infraorbital nerve injury, and damage to the nasolacrimal duct . Thus, iDentigerous cysts have the potential to grow into painful, aggressive lesions in the context of persistent infection. Persistently enlarging dentigerous cyst may result in alveolar bone widening, tooth displacement, severe root resorption, extension of the buccal and lingual cortex, and pain. If left untreated, possible consequences include the development of cellulitis, deep neck infection, ameloblastoma, mucoepidermoid carcinoma, or epidermoid carcinoma .A dentigerous cyst of maxillary canine extending into the maxillary sinus is uncommon. The unerupted tooth involved located near the anteromedial wall and impinging the floor of orbit is infrequent in a female belonging to the pediatric age group. All these rare findings coming together in one case makes it a unique clinical situation that was managed endoscopically with complete cyst excision in a minimally invasive manner considering the age of the patient. In our opinion, early diagnosis with both traditional and advanced imaging modalities is crucial to reduce morbidity and avoid more aggressive surgical procedures to lower the risk of complications. A comprehensive endonasal endoscopic technique, ideally via a middle meatal antrostomy, can be used to extract the tooth and cyst in the maxillary sinus.