Level of Difficulty: Advanced.)Diagnostic biopsy of a left atrial mass is technically feasible but has the risk of tumor embolization causing stroke or seeding. In this case report, we highlight the technical steps for left atrial mass biopsy under transesophageal echocardiography guidance by using cerebral embolic protection. Pathologic examination disclosed low-grade B-cell lymphoma. (  She noticed multiple brief episodes of tachycardia, with her heart rate rising to approximately 130 beats/min. She denied associated dizziness or syncope. Transthoracic echocardiography (TTE) revealed a mass in the left atrium, for which she was referred to a cardiologist. Her vital signs were stable, with blood pressure of 125/85 mm Hg, and she was afebrile and not tachycardic. Physical examination showed normal heart sounds with no murmur, as well as good air entry in both lungs, absence of lower extremity edema, and no palpable masses or lymph nodes. She denied unintended weight loss or other constitutional symptoms.The patient had a history of COVID-19 about a year before presentation. She also had history of gastroesophageal reflux, for which she took pantoprazole.Laboratory data demonstrated normal cell counts with normal renal and liver function. The electrocardiogram showed normal sinus rhythm. TTE showed a large mass in the left atrium attached to the lateral wall that was compatible with tumor . TransesCardiac tumors can be broadly categorized into neoplastic and non-neoplastic. Neoplastic tumors are further categorized into primary cardiac tumors and secondary metastatic cardiac tumors. In our case, given the diffuse infiltrative nature of the mass and absence of tumor in any other part of the body, a primary cardiac malignant tumor was more likely than a secondary metastatic tumor. The differential diagnosis included angiosarcoma, rhabdomyosarcoma, leiomyosarcoma, liposarcoma, osteosarcoma, fibrosarcoma, and lymphoma.The patient’s case was discussed by a multidisciplinary team, and the decision was made to biopsy the left atrial mass through a percutaneous transseptal approach. Use of cerebral embolic protection was planned to avoid the risk of stroke caused by tumor embolization. The procedure was performed with the patient under general anesthesia with fluoroscopic and TEE guidance. A cerebral embolic protection device (Boston Scientific) was delivered through right radial access, and the filters were deployed in the right brachiocephalic and left common carotid arteries . ThroughCardiac tumors are rare but constitute an important challenge with respect to diagnosis because they may not be easily accessible for biopsy through a percutaneous approach. Primary cardiac tumors are extremely rare, with an incidence of <0.1% as compared with secondary metastatic cardiac tumors, which are more than 20 times more common.Bone marrow biopsy showed no involvement. After completion of 6 cycles of chemotherapy with bendamustine and rituximab, a PET scan showed resolution of the previously noted hypermetabolic left atrial mass .Figure 1Compared with the traditional surgical approach, percutaneous transseptal left arial biopsy with cerebral embolic protection is safe and less invasive, especially for patients who are not candidates for surgical resection. With the advancement of structural heart interventional techniques, as well as refinement of cardiac imaging modalities, percutaneous transseptal biopsy is a reliable and viable option for sampling left atrial masses. A cerebral embolic protection strategy may reduce the risk of stroke potentially associated with this procedure.The authors have reported that they have no relationships relevant to the contents of this paper to disclose.


Laparoscopic cholecystectomy is the gold standard surgical treatment of gallstone disease and a commonly performed procedure in general surgery. Retained gallstones from intraoperative spillage remain largely asymptomatic and complications are rare. Peak incidence of presentation occurs within a year; however, it is important to recognize retained gallstones as a differential for acute presentations even many years postoperatively. We present the case of a 74-year-old female who presented with a retained-gallstone-associated abdominal wall abscess 30 years after spillage during the original surgery, which was successfully treated with a stepwise extraperitoneal approach with local drainage. Laparoscopic cholecystectomy (LC) is considered the gold standard surgical treatment of gallstone disease and is one of the commonest performed abdominal surgical procedures . IntraopA 74-year-old female presented to the emergency department with a 5-day history of intermittent right-sided abdominal pain postprandially and swelling in the right flank. She had no changes in bowel habits or urinary symptoms. Physical exam revealed tenderness to the right flank without guarding, and LC scars from over 30 years ago were noted. Laboratory tests revealed an infective picture with leukocytosis of 13, neutrophilia 8.89 and C-reactive protein of 141. Her past medical history is significant for type-2 diabetes, hypertension, ischaemic heart disease on dual antiplatelet therapy (DAPT), heart failure, dyslipidemia and obesity. Her surgical history includes LC in the 1990s, with the patient noting she was aware that one stone was left behind at the time. She is a Jehovah’s Witness and will not accept any form of blood products.We proceeded with a multiphase computed tomography (CT) scan which was suggestive of a mixed density lesion measuring 62 × 86 × 98 mm arising from the right posterior transverse abdominis/internal oblique muscle, with a well-defined rounded calcific focus at its inferior aspect likely representing a dropped gallstone (GS) associated with abscess formation . She wasLC is a commonly performed procedure in general surgery. Retained GS may remain largely asymptomatic and complications are rare, occurring in a reported 0.08–8.5%, but is well recognized to account for a spectrum of presentation both in the early and late postoperative period .Acting as a nidus for infection, abscess formation from retained GS is a commonly reported complication with literature citing 60% . Most frPresenting symptoms can be non-specific with fevers and abdominal pain, which often impose a diagnostic challenge when coupled with the variable time to presentation. CT is a commonly used for initial investigation, with high calcium-containing stones identifiable like in this case . RetaineSource control must be achieved with stone removal to prevent recurrent abscess or fistula formation. In view of the medically comorbid patient with DAPT on board, we utilized a stepwise, multidisciplinary approach working closely with interventional radiology (IR). Initial sepsis was controlled with antibiotics and IR percutaneous drainage, allowing time for further workup towards delayed definitive surgery with preoperative IR localization  to preciLC was first introduced into Queensland in 1990, and this case was one of the earlier performed LCs in the state [There are no conflicts of interest to declare.None.Data sharing is not applicable to this article as no new data were created or analyzed in this study.


Rupture of unscarred pregnant uterus is a rare occurrence and its incidence is higher in developing countries. Rupture of unscarred uterus is associated higher likelihood of adverse maternal and fetal outcomes. Occurrence of rupture of an unscarred uterus lays foundation for the importance of supervised labour and to maintain a high index of suspicion even in a prim gravida.We present a case of spontaneous rupture of unscarred uterus in a primigravida with no known risk factors. Rupture was diagnosed in second stage of labour when there was cessation of contractions and loss of station. On exploratory laparotomy, hemoperitoneum of 100 ml and a 10 cm tear was found in left posterolateral aspect of uterus. The tear was repaired successfully and patient had a normal post- operative course.Presence of a uterine scar is the key factor leading to rupture. Spontaneous rupture of unscarred uterus is a rare entity and is associated with multiple factors. In our case, all these factors were ruled out. In literature search we could find 15 cases of spontaneous rupture in unscarred uterus. According to our best knowledge this is the 7th case of rupture in unscarred uterus, reported in a prim gravida without any obvious risk factors.Rupture uterus should be kept in mind in all patients, even in primigravida if there is high index of suspicion. Quick diagnosis and immediate laparotomy is the cornerstone of treatment in such cases. •Rupture of unscarred uterus is rare but is associated with significant morbidity.•High index of suspicion is needed even in absence of risk factors.•Immediate laparotomy is the gold standard management.•Delay in diagnosis and management can lead foeto-maternal morbidity. Maternal mortality ranges between 1 and 13 % and perinatal mortality between 74 and 92 % 2A 22-year-old female primigravida, who was brought in ambulance to emergency room at 39 weeks period of gestation, at a tertiary care teaching hospital. She presented with complaint of pain abdomen. On examination she was found to be in latent labour. She was a Hindu by religion and a home maker. She was a booked and investigated case and had eight antenatal visits. All antenatal investigations were unremarkable. Mid trimester scan revealed a normally appearing anterior placenta. There was no history of intrauterine instrumentation or surgeries. During her stay in hospital, she had spontaneous progress of labour and there was no use of oxytocics for labour augmentation. She was admitted in the first stage of labour (1 cm dilated) and progressed to active labour spontaneously in 10 h. She became fully dilated after 4 h of active. Labour was well supervised by maintaining partograph and close foetal monitoring by intermittent auscultation.In second stage, there was sudden cessation of contractions, loss of uterine contour and the foetal heart sound could not be localized. On per vaginum examination cervix was fully dilated and effaced with a loss of station. Patient was hemodynamically stable without any peritoneal signs. Urgent bedside ultrasound was done which revealed an empty enlarged uterus, a foetus in the abdominal cavity and foetal heart rate of 30 bpm. She was rushed to OT for an urgent laparotomy.Midline Laparotomy revealed hemoperitoneum of 100 ml. Foetus and placenta were found outside the uterus in abdominal cavity. Uterus had no structural anomalies with a normal appearing placenta. A live male neonate weighing 2280 g was delivered. APGAR was 4, 8,8 at 1, 5 and 10 min respectively. Neonate was intubated after initial steps of resuscitation. On further inspection, a 10 cm tear found in left posterolateral aspect of uterus extending deep into the lower uterine segment. The tear extended into the left broad ligament. Bladder was mobilized. To counteract blood loss, we performed a left sided uterine artery ligation before repairing the tear. We were able to repair the tear in two layers. Intra operative blood loss was 1200 ml. Surgery was concluded by doing an extensive intraperitoneal lavage , Fig. 2.Patient had an uneventful post-operative period and did not require blood transfusion. She was discharged on day 7. She was advised early antenatal visit in subsequent pregnancy and delivery by elective caesarian section.Her neonate developed grade 3 Hypoxemic ischaemic encephalopathy. After receiving assisted ventilation for a course of 3 days in Neonatal Intensive Care Unit, the neonate eventually died on day 7 of post-natal life.3Presence of a uterine scar is the key factor leading to rupture. Spontaneous rupture of unscarred uterus is a rare and is associated with trauma, grand multipara, unsupervised augmentation of labour, injudicious use of uterotonic agents, obstructed labour, Mullerian anomalies, obstetric manuevers  and placenta percreta.In our case, all these factors were ruled out. Ehler Danlos syndrome IV has been associated with uterine rupture but we didn't carry out the genetic testing. She did not have any relevant family history of connective tissue disorders. Patient was a primigravida who went in spontaneous labour without use of any oxytocics or labour inducting agents. There was no history of any kind of intrauterine instrumentation or manipulation in the past. She was a booked case and her antenatal ultrasounds ruled out possibility of Mullerian anomalies or abnormal placentation. Her labour was well supervised in a tertiary care centre. There was no undue prolongation of latent and active stage of labour. Active stage was well monitored with partograph, intermittent foetal auscultation and cardiotochography as per institutional protocol. In active labour, features of rupture were promptly detected and patient was taken for laparotomy after confirming the diagnosis on ultrasound. Due to early diagnosis and well-coordinated multidisciplinary approach, we were able to provide quick surgical intervention which is usually the key to successful management of uterine rupture In literature search we could find 15 cases of spontaneous rupture in unscarred uterus . Out of According to our best knowledge this is the 7th case of rupture in unscarred uterus, reported in a prim gravida without any obvious risk factors. In our case, the cause may be an underlying connective tissue disorder as other causes were ruled out. Although rupture was diagnosed in second stage, there were no features of uterine hyper stimulation, precipitate labour or obstructed labour.4Uterine rupture should always be considered in all cases if there is high clinical suspicion, even in primigravida. Early surgical intervention is a key to successful management.Not commissioned, externally peer-reviewed.None/exempted.No funding taken.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of written consent is available for review by the The Editor-in Chief of this journal on request.Dr. Mona Mishra: Study design, data collection and writing the paper.Dr. Y. M Mala: Study design and writing.Dr. Y. M Mala, Professor.Not applicable.No conflict of interest.


COG4 gene. Magnetic resonance imaging revealed a critical stenosis of the cranio-cervical junction (CCJ) which required surgical treatment to attempt sufficient neurological decompression. The patient underwent decompression of CCJ under general anesthesia. There was no significant radiological and clinical improvement during the postoperative period. Conclusions: SWS is presented as an extremely rare congenital disease in children. The clinical condition of our patient confined surgical possibilities, therefore further treatment in such patients should be appropriately evaluated.Introduction: Saul Wilson syndrome (SWS) is a rare congenital syndrome characterized by a variety of symptoms, mostly skeletal changes. Saul and Wilson were the first to describe children with extremely short stature and craniofacial dysmorphism. Case report: We present a case of a 15-years-old boy with clinical and radiological characteristics of SWS. Genetic examination identified a pathogenic heterozygous variant in the  COG4 (606976) gene on chromosome 16q22, and represents a rare genetic autosomal dominant disorder typically caused by a de novo pathogenic variant. SWS is a skeletal dysplasia characterized by numerous skeletal disorders, such as profound short stature, clubfoot and short distal phalanges of fingers and toes. Besides skeletal features, patients usually have characteristic craniofacial features such as prominent forehead, prominent eyes and micrognathia, ocular disorders and hearing loss. Early development is usually delayed with normal cognition. The diagnosis of SWS is established in a proband with marked short stature, typical facial and skeletal features, and a heterozygous pathogenic variant in COG4 identified by molecular genetic testing [Saul-Wilson syndrome (SWS) OMIM#618150) is caused by recurrent de novo heterozygous missense mutations in the conserved oligomeric Golgi—8150 is cCOG4 gene result in altered proteins in the COG complex and change retrograde transport of proteins, which consequently results as SWS [COG4 variants have been reported; both resulted in a p.Gly516Arg missense change [COG4 is a subunit of the COG complex and an important intermediary in transport of proteins between the Golgi apparatus to the endoplasmic reticulum. Mutations of the s as SWS . To dateThe authors present a rare case of a boy suffering from SWS complicated with critical stenosis of cranio-cervical junction (CCJ) and atlanto-axial dislocation.A 15-year-old boy suffering from SWS was born from the third pregnancy of nonconsaguine young and healthy parents as a hypotrophic infant with a perinatal risk factor which included intrauterine growth retardation and asphyxia. Complex phenotypic dysmorphic features were observed at birth. The phenotype of our patient includes multiple facial and skeletal disorders: midface retrusion, prominent forehead, frontal bossing, shallow orbits, downslanted palpebral fissures, narrow nasal bridge, large filtrum, micrognathia, small hands with short metacarpal bones and short distal phalanges of fingers with consequent brachydactyly, knee flexion contracture, pseudoarthrosis, calcaneovalgus deformity, thoracic scoliosis, lumbar hyperlordosis, coxa valga, skeletal dysplasia, pectus carinatum, generalized hypotonia, global developmental delay, cognitive impairment, complete lack of adipose tissue with consequent pseudohypertrophy of muscles, bilateral cataracts and progeroid facial appearance . As a paComputed tomography (CT) scan of cranio-cervical junction revealed bifid arch of the atlas as a part of skeletal disorders. Additional preoperative magnetic resonance imaging (MRI) revealed a critical stenosis of cranio-cervical junction with concomitant myelopathy .COG4 gene (16-70530270-C-T NM_015386.3:c.1546G>A (NP_056201.2:p.Gly516Arg). Amino acid changes identical to a pathogenic variant have been previously reported (ClinVarID: VCV000585271). The same variant was also reported as de novo in one or more affected individuals with a consistent phenotype from multiple, unrelated families.Karyotyping excluded chromosomopathies, and no change of clear clinical significance in chromosomal microarray analysis (CMA) was found. Whole exome sequencing  identified a pathogenic heterozygous variant in The patient underwent surgical treatment under general anesthesia and in a prone position. The surgical approach was performed in the mediosagittal plane. The foramen magnum was decompressed with a micro-technique using a high-speed drill and a rongeur . The bonThe postoperative recovery went uneventfully, and the patient was released from hospital one week after surgery.Follow-up MRI scan of cranio-cervical junction after three months and one year revealed residual stenosis of CCJ and myelopathy of cervical medulla. Nevertheless, the postoperative diameter of decompressed CCJ was insignificantly wider in the sagittal and axial planes compared to preoperative MRI scan .Saul-Wilson syndrome is considered one of the rarest diseases worldwide with only fourteen known cases from the 1990s until the present. Saul and Wilson were the first to describe the syndrome, which was presented as severely short stature, craniofacial dysmorphism and microcephaly . ClinicaIn the scope of the presented case conditioned by congenital disorders, further surgical approaches should be avoided, and further treatment should be focused merely on symptomatic treatment under guidance of paediatricians.A preoperative magnetic resonance imaging (MRI) scan revealed a severe myelopathy at the level of cranio-cervical junction and radiologic signs of atlanto-axial instability. Therefore, the authors decided to obtain only bone decompression of the foramen magnum and C1 vertebra to prevent any possible neurological deterioration with the aim of achieving a better radiologic outcome, e.g., partial or nearly complete regression of myelopathy. There were no radiological signs of platybasia and basilar invagination according to radiographic measurements of cranio-cervical junction.Despite an adequate decompression of the CCJ, a postoperative follow-up MRI scan revealed only a limited widening of the diameter of the cranio-cervical junction caused by radiologically confirmed atlanto-axial instability. There was no sign of regression of myelopathy. The authors did not confirm the utmost benefit of surgery according to radiological follow-up.Ferreira et al. compared and followed-up characteristics of their patients which revealed short stature and progeroid appearance in all patients. A significant part of SWS clinical findings are skeletal abnormalities, which are described as osteoarticular pain followed by decrease range of motion. Bone fragility and poor bone healing are also presented as secondary common clinical features in patients suffered from SWS . AccordiDespite satisfying postoperative recovery, the main obstacle in further treatment of our patient remained his established clinical status in the scope of his primary disease and radiologic confirmation of persisting myelopathy. Another significant difficulty in the treatment approach was the impossibility of obtaining a prompt genetical confirmation of SWS, since it currently cannot be performed in the Republic of Croatia. A genetic exam was necessary to obtain an adequate diagnosis due to the physical features and physical development of the patient. Goel proposed a classification of atlantoaxial instability based on analysis of numerous subjects that included instabilities of all spinal segments. A novel classification defines types of central atlantoaxial instability (CAAD) with emphasis on the possibility of atlantoaxial instability without radiologic confirmation or influence of bone deformity. There are two types of atlantoaxial instability that define dislocation of the atlas in the anterior or posterior direction (types 1 and 2), and a third type that defines normal anatomic alignment with clinical and radiological evidence during the surgical procedure (type 3). Furthermore, Goel described CAAD as an independent clinical entity and the cause of cervical myelopathy, especially in abnormal conditions of cervical spine, included bifid arch of the atlas which is comparable to our case. Musculoskeletal and neurologic alterations as the most common hallmarks in patients with CAAD are not considered as embryological disorders. Contrary to this claim, based on a large number of subjects, musculoskeletal and neurological disorders in our patient have to be considered as a result of primordial growth disturbance as a part of SWS .We were unable to surgically treat our patient properly due to his physical status, which included a very low bodily height and weight, shortened neck and soft bones, creating difficulties in achieving the usual operative approach. At the moment, the treatment of SWS remains mainly in the reach of paediatricians according to the clinical symptoms of the patient, mostly systemic and metabolic conditions.In conclusion, the authors were confronted with an unusual condition in an extremely rare congenital disease. Due to its rarity, a possible preoperative radiologic confirmation of critical stenosis of CCJ in such cases should be carefully evaluated. If required, surgery should only be considered as a palliative treatment.


T2W fat-saturated and T1W images after administration of gadolinium. The diagnosis wasfinally confirmed by means of percutaneous biopsy with subsequenthistological/immunohistochemical study.This case report describes the clinical, imaging, and pathological features of acase of Langerhans cell histiocytosis affecting a patient suffering from chronicthoracic spine pain. Spinal localizations of Langerhans cell histiocytosis havebeen rarely described and they are usually characterized by involvement ofvertebral bodies with osteolytic lesions. Our case presented with severalunusual features that delayed the diagnosis, including the age of patient andthe involvement of left T10 costovertebral junction with relative sparing ofvertebral body and costal bone. The clues for diagnosis were represented byincreased signal intensity both on  LCH includes a broad spectrum of pathological conditions ranging from thesolitary well-treatable eosinophilic granuloma to the disseminated life-threateningvariety known as Letterer-Siwe disease. Although LCH is considered a pediatric disease, several cases have beenreported in the adult population. Moreover, spinal localization is usually considered very rare in adults. In the spine, LCH mainly involves the vertebral bodies, with a pre-dilection for the thoracic spine (54%) followed by the lumbar(35%) and cervical spine (11%).Langerhans cell histiocytosis (LCH) is a rare proliferative disease characterized byinfiltration of a single or multiple organs by dendritic cells that resemble thenormal epidermal Langerhans cells.We present a case of spinal LCH characterized by several unusual features, as typicalosteolysis of vertebral bodies and fludeoxyglucose (FDG) uptake on positron emissiontomography-CT (PET-CT) were not present.This case deals about a 50-year-old caucasian malec omplaining for a dulllong-lasting pain located in the dorsolateral region of the chest on the leftside.Medical history was unremarkable for trauma or other bone disease. He had no historyof fever, night sweats or weight loss. Physical examination revealed limitedmovement at the waist with no motor or sensor abnormalities. Neurologicalexamination was normal.Laboratory tests  and C-reactive protein (CRP) werenormal. Chest X-rays was considered normal.Baseline CT scan revealed a widening of left T10 costovertebral joint with scallopingand mild sclerosis of the left aspect of T10 vertebral body, partially involving theleft pedicle and the adjacent costal bone . 18-FDG T1W and high signal on T2W fat-saturated images , characterized by a low signal ond images , with nod images .A fluoroscopy-guided needle biopsy at the level of T10 vertebral body was thereforeperformed.Histopathology showed fibrous/myxoid tissue with reactive lymphocytes and plasmacells associated to rare “histiocytic” elements resembling anon-specific chronic osteomyelitic pattern . On the Patient was referred to the Hematology Department where, considering also thenon-active pathological pattern, was successfully treated conservatively. Despite this, in rare cases, posterior arch involvement can be present, aswell as paravertebral and/or epidural soft tissue masses. As a result of the low frequency with which spine lesions are encountered inclinical practice, there are few studies in the literature focusing on this specificdisease.LCH is a rare condition, usually affecting pediatric population. Spine can beinvolved in a minority of cases, being the thoracic vertebral bodies the preferredsites, and joint involvement is extremely rare. Usually, in LCH spinal lesions occurin a single vertebral body without extension to neural arch orparavertebral/epidural soft tissues. In advanced stages, the typical complete orincomplete collapse of the vertebral body can be seen, causing anterior wedging withthe characteristic “vertebra plana” appearance; this appearance istypical of children, as in adults only a relatively small volume of the vertebralbody is affected so that vertebral collapse usually does not occur.In our case, radiological presentation was unusual, lacking the typical osteolyticpattern. CT showed only a subtle, non-specific widening of left T10 costovertebraljunction with mild sclerosis of the adjacent bones that could be easily misdiagnosedin case of lack of chief complain and detailed clinical data. Also, the lesion didnot show any FDG uptake at PET-CT. The lack of tracer uptake could be explainedbecause of the absence of significant structural changes and absence of significantinflammation. However, normal PET/CT scan was of great value in excluding themajority of tumoral entities, narrowing the differential diagnosis. MRI changes werenon-specific as well, and somehow similar to those of a spondylodiscitis orinflammation of costovertebral joint. However, lab data were normal, without anysigns of inflammation. The prevalent involvement of pedicle and costovertebraljoint, with relative sparing of vertebral body, is another feature that needs to beunderlined.e.g. tubercolosis), inflammatory disorder(spondyloarthritis), primary tumors and metastases. In the majority of these cases,however, associated imaging findings are present.In our case, there were no signs of osteolysis, both at CT and MRI, and imagingfindings resembled those of inflammation or bone marrow edema. Differentialdiagnosis included other conditions that can involve costovertebral joints asinfections (This case demonstrates that LCH may involve any age and any level of the spine.Osteolysis and FDG-uptake on PET-CT of the involved segments are not always presentand the radiologist should be aware of atypical or unusual radiographicpresentations of the disease when evaluating the spine for evidence of Langerhans,such as involvement of the costovertebral junction. In our case, MRI was ofparamount importance because MRI not only revealed the involvement of bone marrow ofthe vertebral body but also demonstrated the absence of disc involvement, excludingspondylodiscitis. The combination of MRI findings and ancillary changes at CT,including articular space narrowing and subtle sclerosis, could represent a clue fordiagnosis.Final diagnosis relies on pathological findings with the caveat that the lesion canbe suspected on the basis of conventional staining, but only immunohistochemistry,revealing the presence of Langerhans cells stained with CD1a - normally never foundin normal bone marrow—can establish the right diagnosis. Another remarkablepoint is that the biopsy should be performed on a relatively spared area of thevertebral body, avoiding the risk of collecting necrotic or inflammatory tissuesaround the area of costovertebral joint.A variety of treatment modalities for spinal LCH have been reported, includingconservative management, intralesional steroid injection, radiation therapy,chemotherapy, currettage and surgery in selected cases with mechanical instabilityor neurological deficits due to spinal cord compression, keeping in mind that spinalLCH is self-limiting and the prognosis is usually good.LCH is a rare proliferative disease usually affecting pediatric patients,although several cases have been reported in the adult population.Spine can be involved in a minority of cases, with thoracic vertebral bodiesbeing the preferred sites.Osteolysis and FDG-uptake on PET-CT of the involved segments are not alwayspresent, and imaging findings may only include non-specific signs ofinflammation.


Sarcomas are a rare heterogeneous group of neoplasms of mesenchymal origin. In the redistribution of all head and neck malignancies, sarcomas are represented by only 1%. Herein, we report a case of a 66-year-old patient with right maxillary sinus sarcoma that spread through the ostiomeatal complex, infiltrated the septum, all ethmoid cells, frontal sinus, involved the entire right nasal cavity and penetrated to the nasopharynx. Patient was treated with neoadjuvant chemotherapy, surgery and adjuvant radiotherapy. The histopathology indicated poorly differentiated sarcoma with elements of Ewing’s sarcoma, but also with elements consistent with osteosarcoma. Molecular pathological analysis excluded Ewing's sarcoma. Samples were also sent for review to the other Pathology Clinics. They suggested poorly differentiated high-grade pleomorphic sarcoma with elements of osteosarcoma. The accurate diagnosis of the head and neck sarcoma type can be a histopathology dilemma posing a great challenge in the choice of therapeutic approach, and thus the treatment outcome. Sarcomas are rare heterogeneous group of neoplasms of mesenchymal origin. The majority (80%) of head and neck sarcomas are histogenetically soft tissue origin, and only 20% are of bone origin. In the redistribution of all head and neck malignancies, all sarcomas are represented by only 1–2% .A 66-year-old patient with a history of heart disease presented to our hospital with recurrent epistaxis, hyposmia and nasal congestion. On examination, the patient was found with an ulcer-proliferative mass that obstructed the entire right nasal cavity spreading from the epipharynx to the oropharynx. No lymphadenopathy was detectable. Computerized tomography (CT) scan of the head and neck region showed expansive mass primarily in the right maxillary sinus spreading throughout the ostiomeatal complex, infiltrating the septum, all ethmoid cells, entering into the frontal sinus through frontoethmoidal recess and penetrating the nasopharynx. The soft palate was suppressed by the described mass and the initial infiltration of the right pterygoid muscles was also visible . A biopsThe final histopathology corresponded to the biopsy findings. Due to the lack of accurate diagnosis, molecular pathological analysis of all available EWS FLI1 and EWS ERG fusion genes by polymerase chain reaction method was performed and the result was negative. Furthermore, no redistribution of the EWSR1 gene was found by the FISH method, and MDM2 was poorly amplified in 2% of tumor cells, which would exclude the diagnosis of Ewing’s sarcoma. Samples were also sent for consultation and review to the other three Pathology Clinics. Their histopathological findings corresponded to a poorly differentiated high-grade pleomorphic sarcoma with elements indicating bone differentiation and diagnostically corresponded to osteosarcoma, but without definitive diagnosis. The patient was presented again to the board for mesenchymal malignancies and a decision on adjuvant radiotherapy was made. The patient received 60 Gy of radiotherapy in 30 fractions and was free of tumor 14 months postoperatively . One monHead and neck sarcomas are extremely rare tumors that pose a challenge in treatment. Making an accurate diagnosis is often difficult, as seen in our case and in another cases of undifferentiated sphenoid sinus sarcomas, described in the literature that, unfortunately, ended in a fatal outcome . CompletAlthough for Ewing’s sarcoma and pleomorphic sarcoma chemoradiation or neoadjuvant chemotherapy is the desirable first-line treatment, for osteosarcoma the results in the literature are still the subject of debate. The Memorial Sloan Kettering Cancer Center’s experience for head and neck osteosarcomas could not conclusively confirm improved tumor local control, decrease presence of distant metastases or improved disease-specific survival with the addition of neoadjuvant chemotherapy to conventional treatment. Furthermore, they suggest that the bone architecture of the tumor does not allow the mass to ‘shrink’ , 9, 10.In patients with the N0 neck, elective neck dissection is not part of the treatment standard because metastases to regional lymph nodes in head and neck sarcoma are relatively rare . The freMaking an exact diagnosis of the sarcoma type can be a histopathology dilemma, which makes it difficult to decide on a therapeutic approach. It is clear that surgical excision with clean margins is what we strive for by the oncological postulates, but the anatomical complexity of the region and the extent of the tumor often make such an approach difficult. Therefore, diagnosis at an early stage of tumor disease is imperative. In rare cases, to improve the prognosis of treatment, surgery must be combined with other methods of oncological treatment, chemotherapy and/or radiotherapy as described in our case.All of the authors have read and approved the manuscript, and all authorship contributions have been verified to adhere to ICMJE guidelines.


Pure epidural cavernous hemangioma (ECH) of the spine are rare and account for only 4% of all epidural spinal lesions. We report a case of epidural cavernoma at L3/4 presenting with L4 radiculopathy. Radiological, intraoperative findings and histopathology are presented.We present the case of a 56-year-old man who was admitted with a right L4 radiculopathy including an M4 paresis of the right leg, hypoesthesia L4, and radicular pain. Magnetic resonance imaging (MRI) confirmed an extradural lesion L3/4 partially expanding into the right intervertebral foramen. The lesion had a heterogeneous signal, isointense on T1-weighted and hyperintense on proton density (PD) and T2-weighted images. At surgery, an epidural, ovoid, gray-red, soft mass, lightly adherent to the dura and extending to the right L4 foramen was observed. Findings in the histological examination indicated a cavernous hemangioma without signs of hemorrhage. Symptoms and paresis improved rapidly after surgery. The follow-up MRI showed complete resection of the lesion with no signs of radicular compression.Spinal ECH should be considered as a cause of chronic lumbar radiculopathy with atypical radiological findings. Early diagnosis and total removal of the spinal ECH might prevent hemorrhage and neurological deficits. Fewer than 50 cases of lumbar epidural spinal hemangioma have been reported until today, and our case report is adding valuable knowledge to the existing literature.  Cavernous hemangioma is also known as cavernous angioma, cavernoma and cavernous malformation . It is aThe incidence of cavernous hemangioma in the spine has been reported to be 0.22 cases/million/year, accounting for 5-12% of spinal vascular lesions . About hPresentation of spinal ECH is most common in the thoracic spine in approximately 60% of cases. Other locations include the cervical and lumbar spine in approximately 30% and 10-16%, respectively ,16,17 NeCavernoma is embryologically supposed to be of endothelial cell origin, and because of that an extension of the epidural mass into the neuroforamen is possible . Other cThe clinical presentation of spinal ECH may be slow and intermittent in non-hemorrhagic cases ,8 or preHistoryA 56-year-old European man was admitted with symptoms of a right L4 radiculopathy including progressive gait disturbances without back pain. The symptoms were progressive during the last six months prior to admission. The neurological examination revealed a right M4 quadriceps paresis, as well as a right L4 hypoesthesia and dysesthesia and a positive straight leg raise test. No bowel or bladder disturbances were documented. The patient had other comorbidities: Coronary artery disease, and had undergone prior bypass surgery, familial hypercholesterolemia, and chronic inflammatory demyelinating polyneuropathy. Prior to surgery, he was prescribed Aspirin Cardio 100 mg, Atorvastatin 40 mg, and Ezetimibe 10 mg. Radiological findingsThe lumbar MRI showed an extradural lesion L3/L4 ventral to the cauda equina, compressing the right L4 nerve root at the recessus and partially expanding into the right L4 intervertebral foramen. The lesion surrounded the nerve root without any visible widening of the neuroforamen. Although there was some contrast enhancement at the vertebral body of L4, no clear affection of the adjacent vertebral bodies was visible on MRI and additional CT scan. The dumbbell-shaped, smooth lesion had an isointense signal on T1-weighted images and was hyperintense on PD and T2-weighted images Figure , 1B. A hIntraoperative findingsThe patient underwent a microsurgical right interlaminectomy L3/4. Intraoperatively, an epidural ovoid and gray-red soft mass, lightly adherent to the dura, was found. The lesion extended into the right L4 foramen, compressing the right L4 nerve root. There were no signs of epidural hematoma. Under continuous bipolar coagulation and dissection, a microsurgical gross total en bloc resection of the lesion could be achieved. Histological findingsFindings in the histological examination indicated a hemangioma of the cavernous subtype without evident signs of hemorrhage. Hematoxylin and eosin-stained microsections showed tissue composed of a tightly packed collection of variably thickened and hyalinized, dilated, blood-filled vessels with no intervening spinal cord parenchyma. The blood vessel walls consisted of endothelium and a collagenous adventitia lacking elastic tissue and with little smooth muscle cell component, as shown on EvG-stained sections Figure .Postoperative course and follow-upNo intra- or postoperative complications occurred. Symptoms including paresis rapidly diminished after surgery. The follow-up MRI and CT six months after surgery showed complete resection of the lesion with no signs of radicular compression Figure . Due to progressive symptoms of the patient's chronic inflammatory demyelinating polyneuropathy, without any recurrence of the radiculopathy, another MRI was performed 14 years after the initial surgery. The MRI revealed residual postoperative changes without unambiguous recurrence of the ECH, or progression of the intraosseus component. We present a case of a spinal lumbar ECH with radicular pain due to nerve root compression. Since the first description in 1929 by Globus and Doshay ,31, apprClinical findingsRoughly 50% -70% of the cases occurred in women ,44,56 anIn cases with a lumbar lesion, as in our case, patients present with lower back pain and radiculopathy caused by the extension of the cavernoma into the neuroforamen or the ventrolateral compartments, mimicking symptoms of a disc herniation ,20,60-62Neuroradiological appearanceAccording to the MRI findings, spinal epidural vascular lesions are categorized into four types : 1) arteDifferential diagnosisFor the differential diagnosis, radiological findings and clinical history are key points. The differential diagnosis of an EHC per se includes disc fragments, epidural fat, atypical schwannoma, an enlarged epidural venous plexus, lymphoma, sarcoidosis, chordoma, eosinophilic granuloma, multiple myeloma, meningioma and neurinoma ,70,78,80Histological considerationsCavernous hemangiomas may be mistaken macroscopically in the central nervous system for fresh, demarcated brain/spinal cord hemorrhages. Histologically, they appear as a tightly packed collection of variably thickened and hyalinized, dilated, vascular channels with no intervening spinal cord/brain parenchyma . The bloSurgical considerationsIn case of clinical or radiological evidence of acute hemorrhage, immediate spinal decompression and hematoma evacuation should be performed to avoid further neurological deterioration ,84,85. WDue to a high incidence of multiple lesions, the whole neuraxis should be screened, since approximately 40% of patients with spinal cavernomas have cranial lesions ,93. The Spinal epidural cavernous hemangioma should be included in the differential diagnosis in patients with atypical radiological findings as a benign cause of chronic lumbar radiculopathy. Early diagnosis and total removal of the lesion should be achieved to avoid the risk of hemorrhage and consecutive neurological deficits. Surgical resection is the standard of care and resulted in an excellent outcome with complete and sustainable pain relief and no new neurological deficits. Due to the high incidence of combined presentation of spinal and cranial cavernomas, screening of neuraxis is recommended in patients presenting with spinal cavernomas.


Congenital knee dislocation (CKD) is a rare disease with an estimated incidence of 1 per 100,000 live births, characterized by a rare musculoskeletal malformation in genu recurvatum deformity present at birth, affecting one or both lower limbs. The diagnosis may be suspected during ultrasound when observing that the situation of the extremities is not correct, and is confirmed by physical examination at birth, with plain radiography being helpful to establish the degree of severity. At present there are controversies regarding treatment and there is no definitive protocol. We present a new case of CKD, observed in the city of Manizales, diagnosed immediately after birth. Congenital knee dislocation (CKD) is a rare disease characterized by anterior and outward displacement of the tibia in relation to the femur. Three degrees of severity are recognized: congenital hyperextension of the knee, congenital hyperextension with anterior subluxation of the tibia over the femur and congenital hyperextension with anterior dislocation of the tibia over the femur. It was first described by Chanssier in 1812 and later by Chatelain in 1822 and Bord in 1834 . In 1964The known epidemiological information on this disease is not very extensive, but its incidence is estimated to be 1 per 100,000 live births , or apprThe diagnosis is generally made at birth, given the position of the knee, which is confirmed radiologically . For thiA rare case of bilateral congenital knee dislocation (BCKD), diagnosed after birth, with a history of previous delivery with the same pathology, is presented. Therapeutic generalities and new treatment modalities under study are also presented.This study presents a female patient in her fourth decade of life, in her second pregnancy, with a medical history of congenital dislocation of the knee and a firstborn with the same pathology. Prenatal control ultrasounds were performed without alterations; there was adequate adherence to micronutrients; the pregnancy was uneventful and in week 40 a female baby was born spontaneously, with adequate weight and height, with a neonatal apgar score of 8 at one minute and 10 at five minutes, and adequate neonatal adaptation. The initial physical examination showed hyperextended knees associated with genu valgum and flat feet, corresponding to grade III; the rest of the examination was normal . The ortLarsen’s syndrome. This describes patients with facial dysmorphism, joint hyperelasticity and multiple dislocations and whose frequency is 1 per 100,000 live births .Down’s syndrome. Patients may have muscular hypotonia and ligamentous hyperlaxity, which favors the appearance of conditions of this type .Arthrogryposis. This consists of congenital, non-progressive and symmetrical joint contractures affecting at least two different areas of the human body .Myelomeningocele, in the condition of paralysis .These have also been associated, but with less prevalence, with camptodactyly, Ehlers–Danlos syndrome, mongolism, cryptorchidism, angiomas, facial palsy and imperforate anus .As previously expressed, according to  the etioAmong the extrinsic causes are mechanical anomalies, such as fetal malposition associated with oligohydramnios, primary contracture of the quadricipital tendon, anterior cruciate ligament malformation and dislocation during delivery . This paClinical assessment should be performed by exploring the entire lower extremity to quantify the fixation and axis of both lower limbs, but diagnostic imaging is also necessary. Second trimester ultrasound would be indicated for prenatal diagnosis, where the relationship of the baby to the foot and head usually suggests a congenital dislocation. Radiography is useful to determine the degree of involvement, while computerized tomography (CT) and magnetic resonance imaging (MRI) are rarely used ,15. In oGrade I. Represents hyperextension of the knee joint at birth without displacement of the articular surfaces of the femur in relation to the tibia (the axes of the long bones are opposite each other in the joint line).Grade II. Represents subluxation, the tibial epiphysis slipping on the anterior aspect of the femur over the articular surface of the condyle.Grade III. Represents total dislocation of the tibial epiphysis in front of the femoral condyles. In our case, the patient corresponded to a grade III.In 1946, Leveuf and Pais classified the deformity into three groups: hyperextension, subluxation and dislocation. In 2011, Abdelaziz and Samir made a classification based on range of motion, independent of joint relationship ,13:GradeIf >90° of passive flexion. Treatment will be by serial casts, which are maintained for about 2–4 weeks. Immobilization is performed under control with entry scopy without maintained traction.If flexion is 30° to 90°. Initially treated with weekly casts and mobility is re-evaluated after 4 weeks. If >90° is achieved, conservative treatment with casts is maintained, while a quadricipital tenotomy is recommended if flexion after those 4 weeks is still <90°.If flexion less than 90° is still maintained after tenotomy, V-Y plasty associated with arthrotomy is recommended. If more than 30° of passive flexion is not achieved, and in case of recurrences, a V-Y plasty associated with an arthrotomy would be performed to release the ligamentous structures that are displaced anteriorly.Treatment should be performed immediately and is focused according to the level of involvement and degrees of passive flexion and coaptation of the joint. Manual reduction and flexion cast immobilization are recommended to be initiated within the first 24 h, especially for grade 1 and 2 deformities, and surgical treatment is recommended for grades 3, to loosen the quadriceps, iliotibial band, adductor and collaterals, and anterior cruciate ligament [BCKD is a rare disease of varied etiology, which can be diagnosed without difficulty and even prenatally if done accurately. However, an exhaustive search for other pathologies that may occur in these cases should be made, because the effectiveness of treatment is hindered by comorbid conditions, especially diseases with genetic syndromes that decrease the effectiveness of conservative measures. Treatment is usually effective if carried out early, but it depends on the degree of involvement and the condition of the structures involved in the deformity, and if it is not sufficient, the early initiation of more complex measures, in search of the least number of interventions possible, is still the best way to avoid complications and the appearance of comorbidities related to the condition. One must take into account the aesthetic impact for parents as a trigger that allows for accelerating the intervention process, and the need to follow-up between 3 and 5 months after the first intervention to prevent late identification of recurrence of the condition. It is recommended to generate a centralized registry of cases in Latin America in order to contribute to a discussion that favors early intervention and the identification of common characteristics in this patient population.


Melanosis coli is a benign condition, often identified as an incidental finding during colonoscopy, characterized by brown or black pigmentation of the colonic mucosa due to lipofuscin deposition within the cytoplasm of cells. It has been linked to the excessive use of laxatives, particularly those that are anthraquinone-based but also stimulant laxatives and herbal remedies. White patches on colonoscopy in this condition are an extremely rare finding.We present two cases of 31- and 38-year-old, male Nigerians, with a history of chronic constipation and prolonged stimulant laxative use in whom colonoscopy findings of white patches on the colonic mucosa were confirmed on histology to be melanosis coli.Melanosis coli should be considered in the differential diagnosis of patients with chronic constipation and/or prolonged use of laxatives or herbal remedies who exhibit mucosal changes on colonoscopy even if these changes are not black or brown discolorations. Melanosis coli (MC) is a benign condition, often identified incidentally during colonoscopy, characterized by brown or black pigmentation of the colonic mucosa due to lipofuscin deposition within the cytoplasm of cells . This coThe typical presentation of melanosis is a brown or jet-black appearance of the colonic mucosa on endoscopy ,2,8,9. WCase 1A 31-year-old male presented with recurrent abdominal pain of more than five years duration, and a history of similar duration for the intermittent use of the stimulant laxative, bisacodyl, and the osmotic laxative, lactulose. He had a colonoscopy done, which showed whitish, patchy lesions on the mucosae of the caecum and transverse colon suspected to be candidiasis, and biopsies were taken  histochemical, and human melanoma black 45 (HMB45) stains were applied to sections from the paraffin wax blocks of the specimen, which stained positive and negative, respectively Figures , 4.He was counseled on the need to use laxatives sparingly, and to improve the constipation he suffered from by increasing fiber intake.Case 2A 38-year-old male presented with constipation and chest pain for seven years. He was known to pass hard, pellet-like stools with associated perianal pain but no hematemesis or melaena. Over this seven-year period, he used oral bisacodyl, a stimulant laxative, once or twice a week to obtain relief from constipation. He also complained of epigastric pain, frequently radiating to the back associated with bloating, and relieved minimally by taking antacids. He also took over-the-counter antibiotics and herbal medication with no relief. He had no co-morbidities and no significant medical or surgical history. The colonoscopy showed the mucosa of the cecum appearing granular with punctuate whitish deposits Figure .The mucosae of the ascending colon, transverse colon, descending colon, sigmoid colon, and rectum/anal canal were normal. A provisional diagnosis of chronic constipation to rule out intestinal candidiasis was made. Biopsies were taken from the whitish patches on the cecal mucosa and sent for histology.Histology showed irregular fragments composed of colonic mucosa with unremarkable epithelium, which overlies lamina propria with quiescent goblet cell-rich glands. Numerous pigment-laden macrophages with golden-brown, granular cytoplasm were seen in the lamina propria. Mixed inflammatory infiltrates, including lymphocytes, which form occasional aggregates, plasma cells, neutrophils, and eosinophils were also seen as well as occasional foci of congested, thin-walled vascular channels. Fungal hyphae and/or spores were not seen. No atypical cells were seen Figure .A diagnosis of melanosis coli was made. For confirmation, PAS histochemical and human melanoma black 45 (HMB45) immunohistochemical stains were applied to sections from the paraffin wax blocks of the specimen, which stained positive and negative, respectively Figures , 8.No treatment was given to the patient, and he has since been referred back to his primary physician after being counseled.MC is a benign condition of the colon characterized by brown or black pigmentation of the lamina propria as a result of lipofuscin deposition intracellularly; and is most frequently attributed to chronic anthraquinone-based laxative use, particularly in older persons and in those with chronic constipation ,3,8. HowThe incidence of MC is said to increase with age, and is, therefore, more common in the elderly ,3, but bThe clinical features of MC are nonspecific. It is frequently an incidental finding on colonoscopy in patients with a history of chronic constipation, especially those who use anthraquinone-based laxatives over a prolonged period -3,5,7,10On colonoscopy, MC is typically noted in the large bowel, especially on the right side of the colon ,6. Both The histologic diagnosis of MC is usually evident on light microscopy using hematoxylin and eosin (H&E) stained sections where it is characterized by the presence of golden-brown pigment-laden macrophages in the lamina propria, often termed melanized ceroid . The pigIt is not exactly clear why our patients’ MC manifested as whitish patches on colonoscopy but they both reported prolonged use of the stimulant laxative, bisacodyl. While the mechanism by which anthraquinone-based laxatives produce the lesions of MC has been studied and reported in the literature ,6; the mMC should be considered in the differential diagnosis of color changes on colonoscopy, including whitish patches, in the colonic mucosa of patients, especially those with a history of chronic constipation and prolonged laxative use, even in the absence of the characteristic brown-black patches. Biopsies should always be taken, and the diagnosis confirmed by histology.


The second branchial cleft cyst (BCC) is the most common type of BCC. Bailey proposed a classification of the second BCC into four types, among which a Bailey type II cyst is the most common presenting lateral to the carotid space. A Bailey type III cyst, which extends between internal and external carotid arteries is an extremely rare occurrence. Complete surgical excision is the treatment modality of choice for branchial cysts and this warrants thorough imaging to see the detailed extent of the cyst. Contrast-enhanced computed tomography and Magnetic resonance imaging, both aid in diagnosis. Here we report a rare case of Type III second BCC, which presented with dysphagia, along with an elaboration of findings on imaging and treatment details. Major head and neck structures are created during the fifth week of fetal development. The five pharyngeal arches (tissue bands) are significant structures that develop. Several malformations or deformities in the neck originate from incomplete or failing, during the embryonic development of these arches. Cysts, sinuses, and fistulae are the three types of branchial arch abnormalities -3.Sinuses showing as an aperture in the lower neck are the most prevalent presentation of branchial arch abnormalities. First branchial cleft anomalies present as recurrent painful swellings around the ear and sometimes may be associated with pus discharge from the sinus. In 1832, Ascherson coined the term “branchial cyst.” He hypothesized that these cysts were the result of poor branchial cleft obliteration . PersistWe report a rare case of large Bailey type III second branchial cleft cyst (BCC) with an extension between internal and external carotid arteries and having overlapping features with type IV cyst, presenting with dysphagia as its deeper part formed relation with pyriform fossa, an occurrence which has probably not been reported previously.An 11-year-old boy had been complaining for two months about swelling on the left side of his neck and difficulties swallowing. A 3 cm × 2.5 cm globular enlargement, anterior to the upper and middle part of the sternocleidomastoid, superiorly extending 2 cm below the ramus of mandible, 5 cm above the sternoclavicular joint, with smooth surface and well-defined edges, was present on clinical examination Figures , 1B. A nA contrast-enhanced CT scan of the neck revealed a well-defined peripherally enhancing cystic mass measuring 4.7 x 2.9 x 4.8 cm, anterior to the sternocleidomastoid and extending medially up to the left lateral wall of the pharynx Figure . SplayinThe cyst was torn from its attachment, along with its tract that led to the pyriform fossa, and the patient had surgical excision. The cyst between the internal and external carotid arteries was meticulously dissected, with the glossopharyngeal nerve preserved Figure . The posBailey  classifiAround 90%-95% of the cases are second branchial cleft abnormalities, which usually manifest clinically between the ages of 20 and 40 -8. We reAnywhere along the route that passes posterior to the carotid arteries, pierces the thyrohyoid membrane, penetrates the larynx, and ultimately terminates on the lateral face of the pyriform sinus is where BCCs can be detected. To differentiate between the two, consider the superior laryngeal nerve's relationship to the sinus tract. The third arch sinuses start at the base of the pyriform sinus and run posteriorly to the carotid artery, passing superior to the glossopharyngeal nerve but inferior to the superior laryngeal nerve and the hypoglossal nerve. From the top of the pyriform sinus, the fourth arch sinuses continue inferior to the superior laryngeal nerve and descend the tracheoesophageal groove . The secOn computed tomography, they appear as fluid-attenuated masses with distinctive edges. When proteinaceous debris is present, magnetic resonance imaging exhibits varying cyst wall thickness and contents that may be either hypo- or hyperintense to muscle. BCCs are rather intense on T2-weighted scans.The therapy of choice is surgical excision, which has a favorable prognosis. The diagnosis of a third BCC was verified by a histopathologic study of the tumor removed from our patient. The inflammatory squamous mucosa and surrounding lymphoid tissue are typical BCC pathologic features.Second branchial cysts exhibit a variety of sonographic appearances that can be confusing to the untrained eye. For an appropriate diagnosis, the clinician performing the investigation must be well-versed in this area. There is no gender predilection or proclivity for the position of branchial abnormalities (right or left). The overlapping features of type III and IV Bailey Classification for type 2 BCC were found in our case. The use of imaging in the identification and treatment of the lesion was critical. The pre-operative knowledge of the extent of the cyst facilitates complete surgical clearance.


TFE3gene rearrangement and are associated with TSC with a preference for an occurrence at ayounger age. We report a case of a young male who had progressive chronic hip pain and wasfound to have a TFE3-associated PEComa in his pelvic region.Perivascular epithelioid cell tumors (PEComas) are related to the tuberous sclerosiscomplex (TSC) and are commonly benign. When malignant, they can be aggressive with localinvasion and metastatic spread. Conventional PEComas do not have  They are mostly benign and curable by surgical resection when benign. Here, we describe a case of a young male who had progressive chronic hip pain thatwas found to have a PEComa associated with TFE3 translocation in his pelvicregion. We also describe a rare subset of PEComas that has an absence of association withTSC and has a striking nuclear positivity for TFE3.Perivascular epithelioid cell tumors (PEComas) are a family of rare tumors showingperivascular epithelioid cell differentiation that originates in the soft tissues of anyorgan in the body. The most common site of origin is in the abdominopelvic region,gastrointestinal tract, retroperitoneum, and uterus.The patient is a 33-year-old Caucasian male who presented after a motor vehicle accidentand was found to have multiple fractures from pelvic trauma. On imaging, the patient wasincidentally found to have a large pelvic mass. The patient underwent embolization forinternal bleeding along with placement of hardware in the pelvic region to address thefractures. Postoperatively, the patient mentioned experiencing chronic right hip pain forthe last 2 years. Since the pain did not functionally limit, immediate medical attention wasnot sought early.The magnetic resonance imaging (MRI) of the pelvis showed a large soft tissue mass centeredwithin the right ischiorectal fossa that measured 6.6 cm × 6.9 cm × 10.8 cm. Inferiorly, themass extended 2.3 cm across the midline with displacement of the rectum toward the left and 2.TFE3. AlthoughTFE3 is not truly specific, in the context of the morphology and desminpositivity, the pathologic appearance was consistent with PEComa, likely associated withTFE3 gene rearrangement. The Ki-67 proliferation index was 15.3% andtissue necrosis was visible in many parts of the biopsy.The biopsy of the pelvic mass showed a smooth muscle tumor with epithelioid morphology. Thepathology showed the tumor to consist of cohesive clusters of vague nests of epithelioidcells with eosinophilic cytoplasm, which was somewhat granular, and had uniform vesicularnuclei. Immunostaining the tissue biopsy showed striking positivity for desmin, while SMA,caldesmon, HMB45, Melan-A, microphthalmia-associated transcription factor (MITF), and MYOD1were negative. The granular character of the cytoplasm favored the diagnosis of PEComa.There was striking nuclear positivity for After discussion with both radiation oncology and orthopedic surgery, a shared decision wasmade with the patient to start neoadjuvant chemotherapy. He was started on doxorubicin,ifosfamide, and mesna with the intent of decreasing the size of the mass in anticipation ofsurgical resection. A positron emission tomography (PET)/computed tomography (CT) after 2cycles demonstrated a mild decrease in the size of the mass, which measured approximately6.7 cm × 5.7 cm . NodistThe plan moving forward would be to consider surgical resection once sufficient tumorshrinkage is achieved.5 Perivascular epithelioidcell tumors may contain epithelioid or spindle-shaped cells while their cytoplasm can rangefrom being clear to eosinophilic. Nearly all PEComas show immunoreactivity for both melanocytic (HMB-45 and/or melan-A)and smooth muscle markers.The most common form of PEComas is angiomyolipoma (AML), specifically renal AML. Otherforms include lymphangioleiomyomatosis (LAM) and clear cell “sugar” tumor of thelung.6 The size of the tumor in our patient alongwith the histopathologic characteristics provides a potential for malignant spread. However,cellularity and the mitotic rate were not calculated because the tissue biopsy sample wasnot completely viable due to the preoperative embolization. Nevertheless, based on the size,possibility of true necrosis on biopsy, and the high proliferation index of the tumor, thepatient was treated for a potential malignant PEComa with neoadjuvant chemotherapy with planfor surgical resection in the future.The criteria currently used for the malignant classification of PEComas include tumor size>5 cm, infiltration, nuclear grade, cellularity, necrosis, vascular invasion, and mitoticrate as important prognostic factors.TFE3 immunoreactivity. In contrast, thereis a subset of lesions classified as PEComas but harbor TFE3 gene fusions.Distinctive features of TFE3-associated PEComas include a tendency to occurat a younger age, no association with TSC, minimal immunoreactivity for actin or desminmarkers, predominant epithelioid cytology, and strong TFE3 immunoreactivity.TFE3-associated PEComas are mutually exclusive to those associated with TSC. It has been suggested that PEComas that express TFE3immunoreactivity but do not involve the TSC2 gene may be biologicallydistinctive from conventional PEComas. These patients may not benefit from mammalian targetof rapamycin (mTOR) inhibitors. One study found TFE3 rearrangements in 23% of all diagnosed PEComa cases. Our case represents one such nonconventional PEComa. This case differed from otherreported TFE3-rearranged PEComas in that immunostaining showed positivityfor desmin and TFE3. Our patient did not have any clinical features orknown family history of TSC.Conventional PEComas are typically associated with TSC in young patients, but alsofrequently have a spindle cell component. They stain positive for muscle markers such asactin and desmin and lack strong TSC1 on chromosome 9q34 and TSC2 onchromosome 16p13.3, which serve to regulate cell division and differentiation. In conventional PEComas, there is a loss of TSC2. Inactivation of TSC1 and TSC2 genes, seen in TSC aswell as in sporadic PEComas, is associated with subsequent activation of the mTOR pathway.Therefore, treatment with mTOR inhibitors such as sirolimus  has shown a clinical response in a number of patients with AML, LAM, malignantPEComas, and other TSC-related lesions. However, radical resection is still the primary treatment for PEComas.Most PEComas are benign and occur in association with TSC. In TSC, the genes that undergomutations are 2 showedan overall response rate (ORR) of 39%. While 1 patient had a complete response, 52% had stable disease. This represented animportant alternative treatment option for patients with malignant PEComas and was approvedby Food and Drug Administration (FDA) based on this trial. Traditional doxorubicin-based chemotherapeutic regimes have shown response rates <20%. Comparative trials remain elusive due to the uncommon nature of the disease.In the recently published phase II AMPECT  clinical trial, 31 patients treated with nab-sirolimus 100 mg/mThe overall clinicopathologic features of the nonconventional subset of PEComas remainpoorly understood due to their extreme rarity. The treatment for malignant PEComas is stillnot defined and requires further studies. Closer follow-up of patients with nonconventionalPEComas as they undergo treatment will provide much-needed insight and prognosticinformation for the development of future protocols.


Problem-solving is an essential human endeavor. It involves problem definition, followed by solution design, solution implementation, and, finally, review. Healthcare is a prime and often successful example of problem-solving. In medical terminology, the problem definition is termed the diagnosis, the solution design is called the treatment plan, and the solution itself is the provision of treatment. The case review is used to assess the efficacy of the solution. This process is sequential and consequential. Obviously, if a problem’s definition is absent or incorrect, then an appropriate solution cannot be designed or implemented. Unfortunately, missed or incorrect diagnoses are not uncommon in healthcare and can cause considerable harm and economic cost. Minimizing these diagnostic errors would confer great benefits to patients, clinicians, and healthcare organizations. Understanding the nature of diagnosis and why it can be so difficult are some of the first steps in reducing these diagnostic errors. One issue is that diagnosis may seem to be more of an art than a science when the exact mechanisms of diagnosis are often not clearly described. Clinicians typically learn their diagnostic craft by mimicry of their mentors over years of hard experience. This absence of a clear method not only makes diagnosis difficult to do well it also makes diagnosis difficult to teach well. The purpose of this paper is to try to better articulate the fundamental principles and methods of diagnosis and to make diagnosis more intellectually understandable, and so more teachable, and more achievable by clinicians. Problem-solving is an important part of human activity. Typically, problems are identified, solutions are designed and implemented, and the outcome is reviewed. In healthcare, the main problem to be solved is a disease. Identification of disease is termed diagnosis, the solution design is called treatment planning, and treatment where appropriate is then implemented as the solution. Case review determines the outcome. These four stages are linearly dependent. Therefore, an error in diagnosis can lead to consequent errors in treatment planning, hence in treatment given, which then leads to a poor outcome on review. Incorrect diagnoses may cause patient harm, operator stress, and economic loss . UnfortuThe language of diagnosisgnosis (“to know”) and dia (“apart”) (Figure The term diagnosis is part of the fabric of medical language, and indeed elsewhere. Despite this ubiquity, many users may be oblivious to the origin of the word, which remarkably tells us its fundamental meaning. It is no accident that in the 1600s the word diagnosis was derived from two Latin words, which, in turn, came from ancient Greek. The English-language versions of the two words are So, we now know the meaning of the word diagnosis. But the word alone does not instruct us how to make a diagnosis, or how to teach a diagnostic method. Making a diagnosis is done by system analysis.System analysisTo understand a diagnostic method, it is essential to consider the fundamental system in which a diagnosis is to be made . This caEach element in the system can be working well or may be malfunctioning. In a healthy system, all elements will be in their correct state. Alternatively, if one or more elements are malfunctioning, then the system may malfunction, and in the medical sense, the disease is present . Many ofLogic applied to a known element and its relationship to another element is the primary method to obtain a diagnosis -17. An eWhile useful, one logic statement about one system element may not be enough to clearly conclude a specific diagnosis. Several diagnoses could still be possible with that one element’s status. For example, reddened skin could be an indication of a traumatic injury, a sunburn, a noxious chemical, or merely a person being healthy but just too hot. Fortunately, the diagnostic method can be improved by considering multiple elements together. Typically, a malfunction propagates through the system from one or a few initial elements to affect multiple other dependent elements and thus magnifying the effect. This causes a cascade of malfunctioning elements to occur together, some of which then become detectable, all together. This situation gives the opportunity to cross-reference individual logic statements at the same time. One element may logically preclude or alternatively dictate some disease diagnoses. However, multiple logic statements together can then deduce a narrower subset of diagnoses, or even a single specific diagnosis. Considering multiple elements together becomes a more powerful way of discriminating any disease present.Rather than laboriously reasoning each logic statement with its peers, an intellectual shortcut for this technique is simply to identify the defined set of failing elements that are known to occur in a specific pattern for a specific disease. Hence, pattern recognition is the second method by which a diagnosis is made . All oneIn the example above, clearly, redness, heat, swelling, and pain are not the initial failed elements causing the inflammatory disease. The disease usually occurs as a cascade of failing elements. It is common that the initial elements which fail often are microscopic, biochemical, internal, and difficult to detect clinically. However, their failure often leads to later consequential downstream failures of external elements, which may be clinically detectable. In such cases, the hidden internal failing elements can then be logically predicted from the altered and detectable external elements in the clinical domain. We call these proxy external elements symptoms if reported by our patient, and signs if detected by the clinician, perhaps with further tests. Some elements may be both a symptom and a sign if both clinician and patient detect them. Such external signs and symptoms exist as the result of the logic rules and fall into the patterns detected by the clinical diagnostic method.Furthermore, it can be seen that any disease process can be considered at two levels. A pathologist may describe the failing internal elements in great detail, typically on a microscopic, histological, or biochemical level . The cliInexperienced clinicians may need to consciously think through each logic step or pattern matching, but with training, the diagnostic process may occur without explicit thought ,21. SubcHow clinicians obtain and then ascribe value to clinical information has also been extensively studied -29. The Challenges in diagnosisImperfect System ModelsTherefore, the diagnostic method seems to be relatively straightforward to do. It is just logic statements and pattern recognition of disease models. Simple, right? So, how come diagnosis can often be very difficult? This is because there are some serious practical problems that can very much hinder the process. First, disease systems are usually very complicated, and not all diseases are fully described or understood. Their etiology and pathogenesis may not have been clearly established as clear logic statements or as recognizable patterns ,30. ThisUndetectable ElementsSecond, the availability of relevant system elements is crucial to making a diagnosis. Some elements may never be detectable by any means . TherefoMisinformationThe clinician also may have to cope with some misinformation Figure . Some ofNatural VariationWorkplace LimitationsThe fourth practical problem is the natural variation found in all of nature. Charles Darwin explained his theory of evolution on the basis that system elements would be typically variable in a population of organisms . As the Medical workplaces are often stressful and busy environments where the workload is excessive ,34. RepeThe limits of diagnostic methodThe above problems of incomplete knowledge, hidden or misinformation, and natural variation have very real consequences. They set a limit on the diagnostic process. Clinician fatigue is also a detriment. Realistically then, in some cases, a specific diagnosis may not be possible. Often the data available may lead to only a provisional list of more likely diagnoses. This list is termed a differential diagnosis. It is essential that when a diagnosis is not certain that iatrogenically damaging treatments are not carried out. In all but emergency situations, it is better to seek more information with further testing, with further opinions from more experienced colleagues, or with more time, such that the true state of the patient can become apparent ,36.Diagnosis and educationIt can be seen from the above analysis that like many other skills, initially, the diagnosis has to be consciously learned . At firsClinical diagnostic adagesNo account of the clinical diagnostic process would be complete without mentioning some clinical adages that are in common use.First, it has been said that “one’s ears are the best diagnostic tool,” with evidence from a patient’s history often trumping complex and expensive special investigations . More emSecond, any reported symptoms should be confirmed with a related clinical sign. Reproducing the symptom with an associated clinical sign confirms the validity of the history.Third, another useful concept is, where appropriate, to try to deduce an individual diagnosis for each tissue, process, or body part under investigation. This more detailed diagnosis by individual sites can lead to a more precisely tailored treatment plan. Each of these tissues or body parts may need specific but different management at different time stages.Fourth, the adage that “common things are common” reminds us of the greater probability of a routine condition compared with an extraordinarily rare disease, notwithstanding the need to discount rare conditions.Primum non nocere.Finally, when a diagnosis is still in doubt, in all but the most extreme circumstances, it is critical that harmful intervention is not provided until a dependable diagnosis is obtained. Repetition of the diagnostic process, additional investigations, further opinions from colleagues, or just allowing more time may lead to a clear diagnosis. In summary, misdiagnoses in healthcare are common and cause much harm and cost. The diagnostic method uses two fundamental reasoning techniques: individual or multiple logic statements, and pattern recognition of failing disease elements. Many elements are out of view of the clinician who must reverse translate the external signs and symptoms into internal diseased elements to deduce the internal diagnosis. While experienced clinicians may be able to do much of this reasoning subconsciously, inexperienced clinicians must consciously deliberate each logic step and pattern. Although observation of clinical cases is a valuable learning exercise, clinical teachers will accelerate their students’ learning by providing the deconstructed reasoning behind each diagnosis. The aim here is to better rationalize the diagnostic process, to allow it to be better taught, and to allow clinicians to become better diagnosticians. Without knowing disease from health, or one disease from another, reliable design and delivery of therapy are not possible. It is sobering to realize that someone over 300 years ago knew this when they coined the word “diagnosis” to “know apart.”


This is a clinical image submission depicting hemithorax white-out due to massive pleural effusion. A 79-year-old female was sent from the acute rehab facility where she was recovering from pneumonia due to decreased breath sounds on the left. The patient had mild shortness of breath, but otherwise did not have complaints. Her medical history was significant for dementia, atrial fibrillation, hypertension, chronic kidney disease, and congestive heart failure. Imaging revealed a complete opacification of the left hemithorax consistent with a large pleural effusion Figs. , and 3.FAbout 1.5 million Americans experience pleural effusions annually [It is often diagnosed with chest radiographs and computed tomography (CT) scans. Chest CT can detect pleural fluid as little as 2 mL as well as underlying abnormalities, such as pneumonia, abscess, or malignant masses . Pleural


Actinomyces species bacteria. Gastric actinomycosis is extremely rare but has been identified on endoscopy, typically presenting as erythema or ulceration. Standard therapies include prolonged antibiotics, and when these fail, gastric actinomycosis often requires surgical resection. We present a case of recalcitrant gastric actinomycosis, which presented as a subepithelial lesion and the first demonstration of treatment with endoscopic resection through over-the-scope clip.Actinomycosis is an infrequent infection caused by  Actinomyces species bacteria.2 Because of a low virulence, it is uncommon for actinomycosis to occur in an immunocompetent host, instead Actinomyces species commonly prey on those with immunocompromised condition or disrupted mucosa from surgical procedures.6 Actinomycosis can present with a variety of symptoms and findings based on the area involved and in some scenarios mimic malignancy.2 The most common locations for actinomycosis include the cervicofacial region, abdominopelvic region, and the respiratory tract.1 Gastric actinomycosis is rare and generally limited to case reports.8 In 1 series of 15 patients with actinomycosis, only 1 patient had gastric involvement.9 Detection during endoscopy is even more infrequent.10 We present a case of actinomycosis, which was found during endoscopy, and to the best of our knowledge, the first case to be treated with an over-the-scope clip (OTSC).Actinomycosis is a rare infection caused by Gram-positive Actinomyces . During the procedure, the lesion was identified and targeted for EFTR; however, several devices were unsuccessful in grasping and pulling the lesion into the cap. This was believed to be due to the subepithelial lesion containing a firm, calcified core with soft overlying tissues. Because the lesion could not be pulled fully into the cap, the decision was made to deploy the OTSC alone without subsequent snare resection. After deployment, the lesion appeared above the clip, and the intention was to allow the lesion to slough off as the area necrosed , Olympus (consultant and advisor), and Cook Medical (consultant and research support). All other authors have no financial disclosures to report.Informed consent was obtained for this case report.


Leiomyoma of the bladder is a rare diagnosis and accounts for 0.43% of all bladder tumors. We herein present a case of a thirty-nine-year-old female diagnosed with bladder leiomyoma presenting with urinary retention. Workup of her urinary retention revealed a midline bladder neck mass. Transurethral resection of her tumor revealed pathology consistent with bladder leiomyoma. Surgical resection is generally the recommended management of this lesion to make a histopathological diagnosis despite low potential of malignant transformation. Although these tumors are benign, they can cause severe obstructive or irritative symptoms. Further research is needed to determine the pathophysiology of these tumors Leiomyoma of the bladder is a rare diagnosis and accounts for 0.43% of all bladder tumors. Although they are rare, they are the most common benign soft tissue neoplasm of the bladder.2The patient is a 39-year-old premenopausal female with a history of multiple urinary tract infections who presented to her primary care physician with urinary retention. She had a six-month history of small volume voids with straining. She had an elevated post void residual and straight catheterized for over 1000 mLs. She was then referred to urology.She reported a history of overactive bladder symptoms three years prior. She had previously taken anticholinergics but developed recurrent urinary tract infections, suprapubic pain, nocturia, double voiding, and frequency every hour with no gross hematuria. Her post void residual remained elevated at 381 mL. She was started on self-catheterizations and was instructed to stop taking anticholinergics for alleviation of her urinary retention.Renal/bladder ultrasonography was significant for a solid posterior bladder mass with vascularity measuring 4.1 × 3.8 × 3.0 cm . FurtherCystoscopy revealed a well circumcised spherical lesion at the bladder neck with overlying normal appearing urothelium. Bladder cytology did not reveal any malignant or atypical cells. Her urodynamic studies revealed terminal detrusor overactivity with bladder filling and an obstructed pattern with high detrusor pressure (detrusor pressure at peak flow = 124 cm/water) and a low flow rate (average flow rate of 2.1 mL/sec) with voiding. Further evaluation with contrast-enhanced MRI Pelvis revealed an ovoid smooth mass at the bladder neck with heterogeneous isointense T1 and hypointense T2 signals, with no transmural extension .Fig. 2CoShe was taken to the operating room for transurethral resection with pathology revealing a submucosal leiomyoma. Postoperatively, she passed a trial of void. Her only residual symptoms included urgency which was not bothersome enough to start anticholinergic medications. Follow up cystoscopy 1 year post resection revealed a recurrent bladder neck spherical lesion; however, with no urinary retention. She underwent repeat transurethral resection of her 5 cm × 2 cm bladder tumor with pathology revealing a benign leiomyoma . She ret3,Uterine leiomyomas are the most common benign pelvic tumors in women. Leiomyomas are benign smooth muscle tumors that can be found in any organ. Leiomyoma of the bladder is a rare diagnosis that also occurs more frequently in women.,,These tumors may present similarly to other bladder tumors as either asymptomatic or with either obstructive or irritative symptoms. Bladder leiomyomas in specific tend to produce symptoms based on their size rather than location.,Any bladder lesion requires a thorough evaluation including appropriate imaging and diagnostic evaluation which may include biopsy, cystoscopy, and resection. Initial imaging may include ultrasonography or contrast-enhanced computed tomography. If a leiomyoma is suspected, further imaging with magnetic resonance imaging may be done. Bladder leiomyomas are known to occur most commonly endovesically but may also occur intramurally, and extravesically.In terms of management, leiomyomas of the bladder are generally treated by surgical resection given the need to make a definitive histopathological diagnosis and given the good prognosis with no evidence of any malignant transformation associated with resection.There have been few reports of recurrent bladder leiomyomas that required repeat resection. These seem to be related to a difficult and incomplete resection of the original mass given the challenging location for resection  rather than due to a true recurrence.4Leiomyoma of the bladder is a rare diagnosis that accounts for <0.5% of all bladder tumors. These tumors tend to have excellent prognosis after surgical resection with no evidence of any malignant transformation. Further research is needed to determine the pathophysiology of these tumors.This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.JK and AP were involved in the design and conception of this manuscript. JK performed the literature search. JK compiled the primary manuscript. JK compiled the figures. All authors critically revised the manuscript. All authors have approved the manuscript as it is written.All data pertaining to this research article are included within the manuscript as written.The authors have no personal or institutional interest with regards to the authorship and/or publication of this manuscript.


Aneurysmatic bone cysts (ABCs) are rare, non-neoplastic lesions firstly described by Lichtenstein in 1945. These bony-cystic lesions are frequently filled with blood and destroy the architecture of the affected bone and surrounding tissue.Usually metaphysis of long bones and the vertebral column are affect by ABCs. Only about 2% of ABCs occur in the head and neck region, mainly affecting the mandible.4Other benign bony lesions such as non-ossifying fibroma, giant cell granuloma, fibrous dysplasia and fibromyxoma can be associated with these pseudocysts.Due to the rarity of ABCs in paranasal sinuses and the emerging role of endonasal endoscopic sinus surgery (ESS) over the last decades we want to report about an extensive lesion treated with ESS.A 12-year-old girl was referred to our department because of persisting nasal obstruction and occasional epistaxis.Endoscopy showed a cystic tumor like mass  obstructMagnetic Resonance Imaging (MRI) as well as Computed Tomography (CT) for computer assisted navigation was performed showing a 6.6 × 5.1 × 5 cm large expansion in the nasal cavity reaching skull base . The rigPreoperative examinations revealed that radical tumor resection would not be achieved, thus, the lesion was maximally debulked under endoscopically with minimal blood loss. Histologic examination revealed an ABC .Figure 3As expected postoperative MRI showed a residual tumor in the area of the clivus and ethmoidal bone , neverthABCs are benign cystic lesions that destroy and expand the affected bone.Symptoms are mostly described with headache, ptosis, rhinorrhoea, strabismus, exophthalmos, swelling, vision loss and nasal obstruction.Age of diagnosis of ABCs of the paranasal sinuses in previous cases was mainly below 20 years but also cases of considerably older patients were reported.After clinical examination, radiologic investigations must be performed for further diagnosis. In CT scans these lesions show a typical multicystic appearance with bony architecture and well demarked margins. Cysts are often filled with blood clots. In T2 weighted imaging ABCs show intracystic components of heterogenic enhanced signals with fluid levels and peripheral septae that appear isodense with marked enhancement.In histological examination malignancy of the tumor must be ruled out and potential underlying precursor lesions such as e.g. osteoblastoma, chondroblastoma, giant cell tumors, etc. should be defined or excluded; histological appearances of ABCs in the skull are the same is in the long bones. Blood filled pseudocysts surrounded by fibrous tissue in which giant cells are commonly find are typical for these bony lesions. They are defined as pseudocysts due to the missing epithelial layer on the surface of the cystic formations.8As performed in our case, definitive diagnosis of ABCs can only be obtained in synopsis of interviews, clinical examination, imaging and histopathologic examination.Gold-standard treatment for ABCs is the total resection of the lesion if possible.5Adjuvant arterial embolization to avoid intraoperative bleeding is described previous reports . In the 10For tumor resection different surgical approaches according to lesion size and location can be used.The endoscopic approach is less invasive, leads to faster recovery of patients and to a better postoperative cosmetic outcome particularly important in young patients. Major parts of the tumor were resected endoscopically.Two years after the surgery our patient was totally free of symptoms. In nasal endoscopy no tumor progression was observed although MRI still showed a minimal residual tumor in the area of the clivus and ethmoid bone. Due to the good results in this case we prefer endonasal, endoscopic surgery to other surgical procedures described for tumor debulking, e.g. enucleation, cryosurgery or curretages.Although ABCs rarely affect sinuses and/or skull base it should be considered as differential diagnosis of bony lesions of the skull. With clinical, histologic and radiologic examinations ABCs can be diagnosed. Minimal invasive ESS may be used for tumor debulking as symptomatic treatment in radically non resectable lesions.The authors declare no conflicts of interest.