Congenital proximal radioulnar synostosis is a rare genetic malformation of the upper limb. This deformity, which is found mainly in preschool-aged children, has no recognized diagnosis and treatment. Current diagnostic methods cannot effectively assess both bone structure and soft tissue abnormalities, and most surgical treatments introduce complications and do not prevent recurrence. More work is needed; therefore, to address the diagnosis and treatment of this disease.An 8-year-old male patient was hospitalized in our department. He reported deformity and limited motion in his right elbow for the past 2 years. He denied a traumatic or family history of bony malformation. The chief complaint at the time of the hospitalization was the limitation in forearm rotation.Digital radiography of the right elbow joint showed proximal radioulnar synostosis and a valgus deformity. A 3-dimensional computed tomography scan further showed proximal ulna and radius dysplasia as well as anterior dislocation of the radius head. The patient was diagnosed with congenital right proximal radioulnar synostosis.Surgical procedures included arthrolysis of the right proximal radioulnar joint, osteotomy of the proximal radius, internal fixation with Kirschner wires, and reconstruction of the annular ligament. The right elbow was immobilized in plaster in a flexion and supination position for 2 weeks.Recurrence of the right proximal radioulnar synostosis was observed during the 6-month follow-up, but the rotation function of the patient's forearm was significantly improved.The findings from this case suggest that we should carefully monitor all patients younger than 6 years old who report long-term issues with forearm rotation. This case also highlights the need to assess soft tissue and epiphysis abnormalities in addition to bone assessments via digital radiography and 3-dimensional computed tomography. We suggest that surgery should not be performed until the proximal radius epiphysis has closed. Not all cases require surgical treatment, but when surgery is needed, a suitable method should be selected according to the individual needs of the patient. Any surgery performed should treat both the bony malformations and soft tissue abnormalities to maximize the therapeutic effect and reduce complications during and after surgery. Herein, we report a case of congenital unilateral proximal radioulnar synostosis that was recently diagnosed and treated at our hospital. This case report is presented with consent from both the patient and his parent.Congenital proximal radioulnar synostosis is a rare orthopedic malformation. Sandifort reported the first case in 1793.2An 8-year-old male patient who complained of deformity, pain, and limited range of motion in his right elbow for the past 2 years was hospitalized in our department in January 2018. The patient had a normal full-term delivery and did not report a traumatic or family history of malformation. Upon physical examination, the right elbow joint presented with a valgus deformity (carrying angle: 25°). No swelling was observed and no percussion pain was reported by the patient. The posterior cubital triangle of the right elbow was present. The range of elbow flexion and extension was 0° to 120°. Rotation of the right forearm was limited; the range of supination was 60° to 80° Fig. . Based oThe following surgical procedures were performed: arthrolysis of the right proximal radioulnar joint, proximal radius osteotomy, internal fixation with Kirschner wires, and reconstruction of the annular ligament of the radius. The posterolateral elbow approach was used. During surgery, dislocation of the radial head, synostosis of the radius and ulna, and absence of the annular ligament were confirmed. Rotation of the forearm was severely limited. The proximal fusion of the ulna and radius were separated by osteotomy after cleaning hypertrophic scar tissue under the capitulum humerus. Bone wax was applied to the surfaces of the osteotomy sites for hemostasis. Reduction of the radial head was performed via a Wedge osteotomy approximately 1.5 cm below the radial head. The radial head was fixed to the radius trunk using 2 Kirschner wires from the capitulum humerus to the radius trunk with the elbow flexed at 90° and the forearm in the supination position. A vascularized fascial patch was placed proximally between the ulna and radius to reconstruct the annular ligament Fig. . The incDR 2 weeks postoperation showed that the radial head was not dislocated and the proximal radioulnar joint had not re-fused Fig. . At 4 we3 Some studies have indicated that the incidence rate is higher in males, but a recent study found no significant difference between males and females.,5,6,7 Although the exact etiology remains unclear, congenital proximal radioulnar synostosis has been linked to an abnormality of the distal limb on the X chromosome. A number of exogenous factors also may play a role, however. In the present case, the patient exhibited both unilateral proximal radioulnar synostosis and radial head dislocation, but reported no family or traumatic history. In addition, symptoms did not emerge until the patient was 6 years old, which is later than the typical diagnosis of this disease. Therefore, we suspected a multifactorial etiology because the patient had no other congenital anomalies.Congenital proximal radioulnar synostosis is a rare malformation of bone development characterized by the fusion of the proximal radius and ulna. This malformation usually occurs bilaterally and is diagnosed before the patient is 5 years old.Preoperative observations from this case suggested that the fusion site involved only the proximal epiphysis of the radius. Our theory is that because the fusion was not between 2 bony structures, the resulting cartilaginous bridge provided small extent movement when the patient rotated the forearm. In addition, we hypothesize that the rotation of the forearm was partly compensated by the distal radioulnar joint. The patient's symptoms may only have emerged after 6 years of age because this is typically when ossification of the epiphysis of the radius occurs. This ossification limits the development of the radius along the longitudinal axis of the forearm. In the presence of a malformation, the closing of the epiphysis also may have contributed to the occurrence of the valgus deformity as well as dislocation of the radial head observed in this patient. In support of this theory, preoperative DR showed that the fusion site was at the original location of the proximal radialis epiphysis. During surgery, this bony fusion was confirmed at the site of the original epiphysis of the radius. It is not surprising that the final diagnosis of congenital proximal radioulnar synostosis was initially missed in this patient, given that there was no family history or obvious relevant abnormalities. Furthermore, the cartilaginous bridge would not have been clearly visible on an X-ray. This case suggests that a patient younger than 6 years old who reports long-term issues with forearm rotation, but no family history or DR abnormality, should be considered for congenital proximal radioulnar synostosis.: Type I, fusion of the medullary canals of the radius and ulna, in which the radius is longer and larger than the ulna; and Type II, anterior or posterior dislocation of the proximal radius with fusion to the proximal ulnar shaft. Similarly, Cleary–Omer described 4 types of synostosis based on radiography: Type I, fibrous synostosis; Type II, osseous synostosis with a normal position of the radial head; Type III, osseous synostosis with a posterior dislocation of the radial head; and Type IV, osseous synostosis with an anterior dislocation of the radial head. Although these fusion types can be observed clearly by DR or 3D CT, the findings from many cases suggest that synostosis often involves soft tissue abnormalities around the proximal radioulnar joint, such as contracture of the pronator and interosseous membrane and absence of the supinator muscle. Dynamic magnetic resonance (MR) more recently has been proposed in the diagnosis of synostosis because soft tissue abnormalities and cartilaginous connections cannot be observed on X-ray or 3D CT. Adoption of dynamic MR in the clinical diagnosis of synostosis has been limited; however, due to a lack of availability of equipment. The present case was diagnosed with congenital proximal radioulnar synostosis of the right elbow based on the Wilkie Type II classification and Cleary–Omer Type IV classification. Although no preoperative MR examination was conducted to assess soft tissue abnormalities in this case, surgery confirmed the absence of the annular ligament, which likely would have been observed on MR. Soft tissue abnormalities concomitant with bony malformations are common with synostosis. Given that MR is able to assess both the epiphysis and soft tissue, we suggest that preoperative MR should be considered in addition to DR and 3D CT to increase the rate of diagnosis of synostosis and better inform the treatment plan.Diagnosis and clinical classification of congenital proximal radioulnar synostosis usually rely on forearm rotation dysfunction and abnormalities on imaging examinations, specifically X-ray and 3D CT. Two clinical classifications were originally defined by Wilkie In contrast; however, other clinicians assert that conservative treatment will lower the patient's quality of life. In particular, as growth occurs, the bony malformation and contraction of the soft tissue will gradually increase symptoms and also will increase the risk of surgical complications. Thus, many clinicians support early intervention (including surgery) in the treatment of congenital proximal radioulnar synostosis.,11 In our case, the rotation function of the right forearm had regressed over 2 years such that the patient was no longer able to place his elbow in the supination position. In addition, the patient reported constant pain during elbow flexion and extension. These symptoms seriously affected the quality of the patient's daily life. Surgery to separate the proximal radioulnar joint as well as to reduce the radial head and reconstruct the annular ligament relieved these symptoms and restored an appropriate forearm rotation arc that allowed for hand movement. Although the proximal radioulnar joint eventually re-fused within 6 months postoperation, the patient's forearm rotation function was improved and the patient could perform most activities of daily living. These results suggest that early-stage surgery may be the best treatment for this disease.Clinicians do not agree on the appropriate treatment for congenital proximal radioulnar synostosis. Many clinicians recognize that bony malformations and soft tissue abnormalities usually coexist in this condition, and that surgical reconstruction of the bone alone cannot completely restore the rotation function of the forearm. In addition, there is a high postoperative rate of recurrence of the fusion. Similarly, Fujimoto and colleagues reported that the optimal age for rotational osteotomy to treat congenital radioulnar synostosis was between 3 and 6 years old because union could be achieved without internal fixation and the radius could be sufficiently remodeled. Hung and colleagues reported consistent findings using derotational osteotomy, and also found better postoperative rotation function in younger versus older patients. There are limitations to these studies; however, including small sample sizes and lack of long-term radiographic follow-up. Our patient had surgery at 8 years old, which resulted in successful restoration of forearm rotation but did not prevent re-fusion. Based on this case, we suggest that surgery should be delayed until the proximal radius epiphysis has closed completely, which usually occurs around 7 years old, to avoid injury to the epiphysis and adverse effects on the bone union. Another advantage is that older children can more readily comply with post-operative exercise regimens to improve functional outcomes.If surgery is the optimal treatment, then the clinician must consider the appropriate timing of surgery as well as the indications and methods to be used. Many studies suggest that early childhood is the best time for surgical treatment. For example, in a study by Murase and colleagues, all children were younger than 5 years old. others argue that surgery should be performed whenever the patient's daily life or sporting activities are negatively impacted. In recent years, a group of surgeons proposed that surgical treatment should be indicated under 2 circumstances:Clinicians do not agree on indications for surgery among patients with proximal radioulnar synostosis. Whereas some clinicians consider surgery to be necessary only when the patient's range of supination is 20° to 35°,(1)When the forearm is locked in a position of hyperpronation .(2)When there is bilateral involvement.In reality, there are many factors that may influence the decision to pursue surgical treatment, such as whether the patient has a unilateral or bilateral malformation, is right- or left-hand dominant, uses knives or chopsticks, or plays or is learning a musical instrument. The indication for surgery also may depend on whether the shoulder and wrist joints can compensate for the forearm's rotation function or whether other deformities are present. Surgery should be performed; therefore, to relieve pain and restore whatever forearm rotation function is suitable to the individual patient's needs.In general, we suggest the following guidelines should be used to determine whether surgery is indicated:(1)The dominant-side forearm is locked in 0° to 20° pronation or 60° to 90° degrees supination.(2)The dominant-side forearm is locked in 20° to 60° pronation or 20° to 60° degrees supination and the dysfunction cannot be compensated by the ipsilateral shoulder or wrist.(3)There is bilateral forearm rotation dysfunction that cannot be compensated by the shoulder or wrist and has a significant effect on the patient's quality of daily life.(4)The patient has any of the above in combination with dislocation of the radial head and significant pain.,14,15 Hung and colleagues reported on the use of derotational osteotomy in 34 cases, all of which experienced significant improvement in rotation function with no complications. This procedure is complicated to perform; however, which limits its widespread use. In addition, like radioulnar rotation osteotomy, recovery requires a long period of immobilization of the forearm. Others have reported the use of vascularized fascia-adipose layer transplantation, in which the fusion site is separated and the radial head is wrapped with pedicled fascia. While this treatment has been reported to improve the rotation function of the forearm and reduce the rate of recurrence, a few cases developed transient posterior interosseous nerve palsy postoperation.,17 In addition, Kanaya and colleagues, who performed free vascularized fascio-fat grafting in 7 cases, reported recurrence of synostosis in 3 of the cases.There is no gold-standard method for treating congenital proximal radioulnar synostosis. Radioulnar rotation osteotomy is common, but requires a long period of immobilization of the forearm post-operation and is associated with an increased risk of delayed union, nerve injury, and ischemic muscle contracture.In our case, we separated the ossified proximal radioulnar joint, performed a Wedge osteotomy, and fixed the caput of the radius to the radialis trunk with 2 Kirschner wires. We then used a lateral vascularized fascial patch to reconstruct the annular ligament. The patient's forearm was immobilized for 2 weeks postoperation. To reduce the possibility of re-fusion, we smeared bone wax on the radioulnar surfaces during surgery and administered indomethacin postoperation. We simplified the osteotomy to minimize the risk of delayed union, nerve injury, and ischemic muscle contracture. We chose a fixation approach that considered the patient's trait of growth and reduced the immobilization time. Despite the use of the vascularized fascial patch, bone wax, and indomethacin to prevent recurrence; however, the proximal radioulnar joint was re-fused at the 6-month follow-up. Based on our findings, we propose the following guidelines to improve postoperative outcomes:(1)Correct any radial head dislocation.(2)Choose an appropriate fixation, if osteotomy is needed, to reduce the immobilization time and prevent delayed union or nonunion.(3)Correct as much of the supination deformity as possible, and maintain the forearm in a neutral position  during and after the operation.(4)Protect the ulna and radius epiphysis from injury in cases when the surgery occurs before the proximal radius epiphysis has closed.(5)Repair soft tissue abnormalities around the proximal radioulnar joint to reinforce the stability of the bone structure.(6)Inform the patient and their parents before the operation that the prognosis may be unsatisfactory due to the high rate of recurrence.In conclusion, congenital proximal radioulnar synostosis is a rare genetic disease. There is no consensus regarding its diagnosis or treatment. Soft tissue abnormalities involving the epiphysis usually accompany the bony malformation, indicating that an assessment of both the soft tissue and epiphysis is necessary. The findings from our case suggest that we should consider congenital proximal radioulnar synostosis in patients younger than 6 years old if they report long-term forearm rotation dysfunction and no prior family history, as early-stage surgery may benefit these patients. The surgical methods performed should be selected according to the individual needs of the patient, and should treat both the bony malformation and the soft tissue abnormality with an appropriate fixation to reduce complications and maximize the therapeutic effect. We believe that treatment of congenital proximal radioulnar synostosis will continue to be improved as orthopedic technology continues to develop.The authors thank the patient and his family for their participation and the BioScience Writers for their editing.Data curation: Chunyuan Geng.Supervision: Bin Dai.Writing – original draft: Yuqing Jia.Writing – review & editing: Zikai Song, Shijie Lv.


Sacrococcygeal teratoma is one of the most frequently prenatally diagnosed neoplasias. Obstetric ultrasound has a role in the diagnosis and management of these tumors during pregnancy. In this report, we describe a multidisciplinary approach in a case of a patient with sacrococcygeal teratomas and preterm delivery, as well as postnatal outcomes.A 26-year-old Caucasian woman at 20.3 weeks of gestation with a normal gestational course and no relevant medical or surgical history was referred to our institution with a sacrococcygeal mass diagnosis. Magnetic resonance imaging confirmed the diagnosis of sacrococcygeal teratoma type I according to the Altman classification. Follow-up with ultrasound showed an increase in the size of the mass up to 190 × 150 mm, high Doppler flow, and severe polyhydramnios. At 35.1 weeks of gestation, the patient had premature rupture of membranes, and an emergency cesarean section was performed due to recurrent late decelerations detected by fetal heart rate monitoring. Afterward, surgery was performed successfully at 36 hours of life. Posterior controls revealed normal and healthy child growth.This case report demonstrates the importance of a multidisciplinary approach to offer the best neonatal outcomes by performing early surgery, as well as the need for follow-up by ultrasound in order to minimize complications by assessing mass growth, Doppler flow, and amniotic fluid. Sacrococcygeal teratomas (SCTs) are the most common extragonadal germ cell tumors (GCTs) in infants and young children, with an estimated incidence of 1 in 27,000 fetuses [In adulthood, GCTs are often located in other sites, such as the mediastinum and retroperitoneum, and the incidence of malignant elements is higher than in children .SCTs are comprised of different types of tissues that come from at least two of the three germ cell layers, and, depending on the tissues that are included, they are divided into mature, immature, or malignant teratomas  .As shown in our patient’s case, obstetric ultrasound let us make a prenatal diagnosis, normally in the second trimester by routine sonography, and it is also an important tool in the evaluation and monitoring of the tumor over the course of gestation in order to identify fetuses at increased risk of complications and to plan a multidisciplinary treatment or intervention, when appropriate . What ouA 26-year-old Caucasian woman, gravida 2, para 1, with a spontaneous normal vaginal delivery 2 years ago was referred to our institution at 20.3 weeks of gestation after a sonographic finding of a sacrococcygeal mass of 26 × 24 mm in a male fetus.The patient had no family history of birth defects or genetic disorders. She did not have any medical or surgical history, and she had no alcohol or smoking habit. She had no relationship with the father of her fetus and received no drug therapy while pregnant.She had a normal gestational course with low risk of aneuploidies in the first-trimester screening and a normal first trimester scan at 13 weeks. Her sonographic examination revealed a single intrauterine pregnancy with an estimated gestational age of 20 weeks. The study revealed an exophytic, mixed echogenic mass arising from the sacrococcygeal region with high vascularization seen on Doppler flow , as was her physical examination. Treatment with a corticosteroid  and atosiban was started. The tocolysis was effective, and an elective cesarean section was scheduled at 35 weeks of gestation in conjunction with the neonatal service and the pediatric surgeon. However, 1 day before the scheduled cesarean section, the patient had premature rupture of membranes. Fetal heart rate monitoring revealed recurrent late decelerations at that moment, and an emergency lower segment cesarean section was performed.A male infant was born at 35.1 weeks with an SCT of 200 mm. The combined weight of the baby and teratoma was 4030 g Fig. . His ApgExcision of the teratoma was performed at 36 hours of life, after embolization of the middle sacral artery. Surgery was done without incident, with an operative time of 3 hours. The reconstruction was done without any excess skin Fig. . PatholoThe baby was discharged at 25 days after birth with normal results of abdominal, cerebral, and kidney ultrasound scans. Neonatal follow-up was performed during the first 16 months, and no long-term neurological deficits have appeared.We present a case of SCT that was correctly diagnosed at the second-term scan and controlled by serial ultrasound examinations with a preterm termination that concluded with a successful multidisciplinary approach leading to good maternal and infant outcomes.SCTs are an extragonadal neoplasm presenting in the presacral area as a mass in the midline caudal end of the fetus, and they can have solid, cystic, or mixed components. In most cases, they can be asymptomatic or cause rectal or bladder obstruction due to the growth of the tumor. Although more frequently reported in female infants, the malignant degeneration affects male infants in most cases , 7.Histologically, SCTs can be classified as mature teratomas , immature teratomas , and malignant teratomas, which contain one or more of the malignant GCTs  –10.SCTs are also classified according to the American Academy of Pediatrics Surgical into four types, depending on the external or internal components of the tumor. While type I is primarily external, type II is external with a significant intrapelvic component, type III is external with a pelvic mass, and type IV is completely internal. The prenatal detection is easier in types I and II tumors, whereas the malignancy is more prevalent in type IV tumors , 12.Prenatal diagnosis is usually made in the second trimester by routine sonography, even though it is also possible to be detected in the first trimester. The usual finding is a mass near the distal spine, but it could also present as an erosion in the vertebral bone or a group of calcifications. Besides the mass, other structural abnormalities, such as hydronephrosis, rectal stenosis, or cardiomegaly, could be found as a consequence of the presence of the tumor .Magnetic resonance imaging can be useful in determining the extension of the tumor, the compression of adjacent organs, and in differentiating this pathology from a distal neural tube defect (myelocystocele or myelomeningocele), which is the most important differential diagnosis. The main difference is the location of the mass effect, which is presacral in SCTs and posterior in neural tube defects .Ultrasound evaluation is used not only for the diagnosis but also for the monitoring of the tumor and its complications during the whole pregnancy. This evaluation should help in detecting which tumors are at high risk of causing complications that could cause hydrops and, in the end, fetal demise. Serial ultrasound should be performed to evaluate the tumor size, the solid or cystic portion, and the amount of amniotic fluid, as well as the vascular flow using Doppler. High-risk tumors are the ones that are larger or with a rapid growth, tumors that are mostly solid, and tumors with high vascular flow, because they can create a vascular steal phenomenon that can cause cardiomegaly and hydropic changes . In thesPerinatal morbidity and mortality in fetuses affected with SCTs is still high, mostly due to the associated prematurity that is usually involved. More important perinatal complications are preterm labor, malignant invasion, tumor bleeding or rupture, obstruction of the amniotic fluid, and heart failure .in utero as a temporary measure in specialized centers and very selected cases that have a tumor with high risk of developing hydrops and a gestational age earlier than 32 weeks of gestation [The main treatment of SCTs is the surgical resection of the tumor and the coccyx to prevent the recurrence. This surgery is undertaken postnatally in most cases, and it is only performed estation . Deliverestation .Normally, for SCTs without malignant elements, complete surgical resection is sufficient, followed by 3 years of measuring hormone levels of AFP and beta-human chorionic gonadotropin . In maliIn our patient’s case, the ultrasound follow-up allowed us to identify the growth of the mass, high Doppler flow, and severe polyhydramnios, and, in this way, it let us plan the delivery, which was scheduled together with the neonatal service and the pediatric surgeon. This multidisciplinary approach permitted a premature surgery and consequently excellent neonatal outcomes.As shown in our patient’s case, prenatal ultrasound examination is important in the diagnosis as well as the monitoring of the tumor in order to detect those that could present a high risk of perinatal complications such as heart failure and hydrops. This prenatal evaluation is important to making an accurate diagnosis, an appropriate treatment, and a multidisciplinary approach.


A patient with end-stage primary central nervous system lymphoma presented with acute deterioration of consciousness. MRI showed a diffuse high-intensity lesion involving the right thalamus and mesencephalon on FLAIR image. She had just begun to take tirabrutinib. Emergent high-dose intravenous methylprednisolone for three consecutive days was given, which resulted in complete recovery of consciousness. Tirabrunitib and maintenance dexamethasone was continued thereafter. Follow-up MRI disclosed improvement of diffuse abnormality at 1 month after this treatment. For patients with acute progression of recurrent CNS malignant lymphoma, tirabrutinib and corticosteroid could be one of the treatment options.A 60-year-old woman was transferred urgently to our hospital due to her acute comatose state. She had been under treatment for primary central nervous system lymphoma (PCNSL) of diffuse large B cell type during the previous 2 years, including five courses chemotherapy using high dose of methotrexate, followed by whole brain irradiation for an intracranial metastasis at the right occipital lobe, 12 months after the initial presentation. About 5 months after that, dissemination to the fifth lumbar nerve was treated with focal irradiation. She had already been prescribed dexamethasone (0.5 mg/day).A small focal lesion enhanced with gadolinium (Gd) in the right thalamus had already been observed at the time of lumbar irradiation therapy , and this was followed up closely by neuroimaging. Two months later, MRI showed that the lesion had grown slightly, but she was still able to walk with assistance, and her KPS score was 70. One and a half months later, this homogenously enhanced lesion appeared to show a small ring enhancement, and the accompanying high intensity lesion had shrunk according to FLAIR imaging.1 because her daily living activities had deteriorated although her consciousness preserved. After admission, she was emergently given intravenous methylprednisolone (1000 mg/day) for three consecutive days, then a gradual decrease to the maintenance dose of dexamethasone (8 mg/day). Soon after the 3 days of methylprednisolone, she recovered consciousness to a state of full wakefulness. Motor weakness in all limbs persisted in practical voluntary movements, restricting her to a wheelchair. MRI disclosed that the hyperintensity lesion on DW and FLAIR images had diminished  and had isocoric pupils with a sluggish reaction to the light reflex. Withdrawal response to pain stimuli was observed in all extremities, with no lateral asymmetry. MRI without Gd revealed a diffuse high-intensity lesion involving the right thalamus and mesencephalon on DW and FLAIR images  and 1b. minished  and 1d, In this present case, acute progression of recurrent CNS malignant lymphoma involving thalamo-mesencephalon was reversed, at least palliatively, via tirabrutinib and corticosteroid . Acute deterioration might be a progression of the pre-existing recurrent lymphoma rather than radiation necrosis or leukoencephalopathy, given the neuroimaging features of the right thalamic lesion showing spontaneous remission followed by acute relapse. It is notably uncertain whether the reversal from apparently lethal brainstem damage is due to tirabrutinib or corticosteroid or both, based on this single case. As no treatment options exist other than corticosteroids in end-stage PCNSL, tirabrutinib might be an optional treatment to add.2 Tirabrutinib  is a newly developed another drug for relapsed/refractory PCNSL, which highly selectively inhibits BTK irreversibly. Its safety, tolerability, and efficacy have only recently been reported.1 Further study is needed to learn more about real-world deployment of tirabrutinib for progressive stages of PCNSL.Recently, the high efficacy of an irreversible selective inhibitor of Bruton’s tyrosine kinase (BTK), ibrutinib, on relapsed/refractory PCNSL has already been reported.


Klebsiella pneumoniae liver abscess (KPLA) is often associated with accompanying metastatic complications such as septic pulmonary embolism, brain abscess, and endophthalmitis. Pleural empyema secondary to a KPLA is a very unusual finding, made even more rare with the presence of a hepatopleural fistula.An 81-year-old woman presented with aggravated dyspnea.The patient was diagnosed with KPLA with empyema through computed tomography (CT) scan findings and pleural fluid culture.The empyema was drained by thoracostomy, and treatment with empirical antibiotics was initiated. After early removal of the chest tube, the liver abscess as well as the empyema increased. An additional liver abscess drainage procedure was performed.The fever resolved and dyspnea improved following drainage of effusion. Three days later, the follow-up chest radiograph showed decreased pleural effusion.Pleural empyema is a rare but fatal complication secondary to KPLA. Additionally, the discovery of a hepatopleural fistula on a CT scan (multiplanar reconstruction image) made this case even more rare. Both, the liver abscess and pleural empyema, were effectively drained through the fistula tract with drainage procedure, thoracostomy, and additional liver abscess drainage. Prompt diagnostic evaluation, using an imaging modality such as CT, and early drainage management with intravenous antibiotics can improve clinical outcome. Here, we report a unique case of pleural empyema secondary to KPLA with a hepatopleural fistula, that was treated with drainage of the pleural empyema and liver abscess.Patients with a 22 of 52.5 mm Hg, a PO2 of 74.7 mm Hg, and an oxygen saturation of 92%. Blood test results were as follows: leukocytosis, 14,240/mm[3] (neutrophil 88.6%); C-reactive protein, 12.64 mg/dL; erythrocyte sedimentation rate, 77 mm/h; aspartate aminotransferase, 24 IU/L; alanine aminotransferase, 24 IU/L; total bilirubin, 0.6 mg/dL; and alkaline phosphatase, 89 IU/L.An 81-year-old woman, with a past medical history of hypertension, visited the emergency department complaining of dyspnea. Her vital signs were as follows: pulse, 69 beats/min; blood pressure, 140/88 mm Hg; respiratory rate, 28 breaths/min; temperature, 35.2°C. On physical examination, she presented decreased breath sounds in the right lung. Initial arterial blood gas analysis with the patient breathing ambient air showed a pH of 7.26, a PCOA chest radiograph showed a right massive pleural effusion in the thorax and a mediastinal shift towards the left side. The initial contrast-enhanced chest CT scan showed a large abscess measuring 12.4 × 9.0 × 11.3 cm that occupied most of the right lobe of the liver in the hepatic medial (S4) segment and extended into the right pleural space, revealing a hepatopleural fistula  and normal erythrocyte sedimentation rate level (18 mm/h). She was transferred from the intensive care unit to the general ward.To treat the respiratory failure, an endotracheal intubation was performed and a 12-French chest tube for drainage was inserted into the right thorax. The drained pleural fluid was cloudy and yellow in color. A pleural fluid analysis revealed that the drained fluid was exudative in nature and the subsequent pleural fluid culture was positive for Fifteen days after hospitalization, a follow-up CT scan revealed that both the pleural empyema and liver abscess had markedly decreased Fig. . The cheThree days after drainage of the liver abscess, a chest radiograph showed that the right pleural effusion had decreased, and the patient's fever and dyspnea improved.3 As a result, cases of pleural empyema secondary to liver abscess have rarely been reported in the literature. Therefore, the present case of KPLA resulting in pleural empyema is a rare finding.Pyogenic liver abscesses seldom extend into the pleural space, because the diaphragm is a tough membranous barrier.,5,7,8  clearly identified a hepatopleural fistula tract between the pleural space and the liver abscess through a multiplanar reconstruction image. MDCT allows thinner collimation and enables the acquisition of high-resolution volume data sets. Multiplanar reconstruction facilitates a definitive diagnosis, as seen in our case.Only a few cases of pleural empyema secondary to KPLA have been reported,8 Table . The med,5 In our case report, although the woman had an abscess in both lobes, the main abscess with the hepatopleural fistula was in the right lobe of the liver. The other 2 cases which involved the left lobe resulted in pericarditis.,8 It is suggested that the liver abscess in the left lobe is more likely to cause pericarditis as well as empyema through transdiaphragmatic extension than the liver abscess in the right lobe. This is thought to be related to where the heart is located primarily on the left thorax. These findings suggest that the development of empyema and pericarditis depend on the predominant KPLA location.In the 2 cases with only pleural empyema Table , there w,5 In our case, Klebsiella pneumoniae was isolated only from the pleural fluid culture, and no microorganisms were isolated from the liver abscess. This might be due to the use of antibiotic treatment prior to the cultivation test.Both pleural fluid and liver abscess culture were positive in 2 of the patients with pleural empyema.,2 septic pulmonary embolism has also been reported.,2 Only a few cases of KPLA have been shown to directly invade the thoraces, as reported in our case. Recently, the capsular antigens K1 and K2, along with the presence of virulence gene, such as rmpA, have been regarded as important virulent factors of Klebsiella pneumoniae and contribute to the development of invasive disease. The K1 strain has been reported to be highly virulent on the account of hypermucoviscosity, which is related to high serum resistance, high-level resistance to phagocytosis, and complement deposition. Although these highly virulent strains of Klebsiella pneumoniae could be associated with local invasiveness, the exact mechanism of how this occurs is unknown. All previously reported cases,,5,7,8 including our case, did not serotype Klebsiella pneumoniae isolates. Further investigation of the virulence factors related to transdiaphragmatic invasion in patients with KPLA is required.In the patients with KPLA, the most common site of metastasis was the lung; The metastatic complications in patients with KPLA are usually severe, associated with a poor prognosis, and high mortality rate. Chang et al suggested that immediate drainage of the liver abscess can decrease the spread of metastatic infection, because it reduces the incidence of thrombophlebitis in the hepatic vein. The treatments for the KPLA related empyema,,5,7,8 as reported in Table ,8 thoracentesis in 1 case, additional surgical decortication and drainage of empyema in two cases,,5 percutaneous hepatic abscess drainage in 2 cases,,5 surgical abscess drainage in 1 case, and surgical hepatic abscess resection in 1 case. All patients were treated with antibiotics and underwent a drainage procedure. In the other cases, the liver abscess was drained first, but we performed a thoracostomy as an initial drainage procedure due to the patient's acute respiratory failure. Subsequently, both the empyema and the liver abscess decreased through thoracostomy and empirical antibiotics. However, both empyema and the liver abscess increased despite maintaining antibiotic treatment on follow-up CT. We speculated that the drainage of the liver abscess entered through the fistula tract during the thoracostomy, which was part of the empyema treatment. Percutaneous thoracostomy through the 7th intercostal space, with an excellent exposure of both the diaphragm and cavity of the hepatic abscess, has been described. In our case, only a thoracostomy provided effective pus drainage of both the thoracic cavity and the liver, which were communicated through the fistula tract identified on the multiplanar reconstruction CT imaging.The mortality rate of KPLA ranges from 3% to 17.1%.However, the empyema and liver abscess increased after removal of the chest tube. Finally, both liver abscess and empyema resolved through additional hepatic abscess drainage. Liver abscess and empyema recurrence may be due to insufficient drainage as a result of early removal of the chest tube.In conclusion, empyema is a rare but fatal complication secondary to KPLA. To our knowledge this case report is the first to describe pleural empyema and KPLA, which were identified through a hepatopleural fistula on MDCT. Both spaces were efficiently drained via only a thoracostomy and additional liver abscess drainage. If a patient with KPLA presents with dyspnea or pleural effusion, we should keep in mind that pleural empyema can be caused by a liver abscess. Early diagnostic evaluation, using MDCT, and immediate treatment, such as drainage combined with intravenous antibiotics can improve clinical outcome. Establishing standard treatment guideline and further investigation of the virulence factors in patients with empyema secondary to KPLA is required.Data curation: Eun Ji Lee, Jun Ho KimSupervision: Kyung Hee LeeWriting – original draft: Eun Ji Lee, Jun Ho KimWriting – review and editing: Yong Sun Jeon, Jung Soo Kim


This is a rare case of an elderly woman diagnosed with acute myeloid leukaemia secondary to myelodysplastic syndrome who presented a spontaneous trans-iliac bone faecal fistula probably related to an incarcerated inguinal hernia and neutropaenia. As far as we know, this is the first described case of a trans-iliac bone faecal fistula. An 83-year-old white female was diagnosed with myelodysplastic syndrome (MDS). She had neglected an incarcerated right inguinal hernia for many years which was resistant to any attempt at manual reduction. In the following year, she presented recurrent episodes of abdominal pain, vomiting and fever, and was hospitalized several times for antibiotics due to a clinical diagnosis of neutropaenic enterocolitis. However, repeated abdominal ultrasonography never showed bowel wall thickening or perforation. When the MDS evolved into acute myeloid leukaemia (AML) the patient was admitted in poor condition, presenting fever, abdominal pain, vomiting, and a 10 cm diameter inflammatory swelling in her right gluteal region which was thought to be a cold abscess due to her neutropaenic status. A general surgeon was called to perform surgical drainage of the lesion.9/L, and leukocyte count of 1.7 × 109/L . The attempt to surgically drain the supposed abscess resulted in liquid faeces discharge through the cutaneous incision. Only then was the patient sent for CT which revealed a faecal fistula (Peripheral blood showed a haemoglobin concentration of 6.8 g/dL, platelet count of 69 × 10 fistula .The gluteal swelling was initially thought to be a cold abscess as a result of the patient’s neutropaenic status related to AML. Neutropaenic patients are immunosuppressed and lack the ability to mount an inflammatory response, and may not present the typical signs and symptoms of infection.She was admitted to hospital and cefepime, vancomycin, plus clindamycin were empirically initiated. After diagnosis of the trans-iliac bone faecal fistula by CT, a surgical gastroenterology evaluation was requested. The patient was managed conservatively due to her advanced age, poor condition, underlying haematological disease, and neutropaenia. Only a colostomy bag was placed on the lesion.Only palliative care was maintained. No chemotherapy was administered. Physiological intestinal transit and faecal fistula drainage persisted until death, which occurred 15 days later due to sepsis. Necropsy was not performed.1–3 Inguinal hernia can cause spontaneous faecal fistula4,5 as a rare complication.5,6 This kind of fistula seems to be very uncommon in neutropaenic patients although it has been previously described in a patient specific diagnosed with cyclic neutropaenia.7 A neutropaenic status, however, can predispose patients to neutropaenic enterocolitis which affects the colon, mainly the cecum, and/or the small bowel.8,9 Oedema, necrosis, haemorrhage and ulceration usually occur, and evolution to bowel perforation is also possible.9Spontaneous faecal fistulas are usually associated with previous abdominal surgery or trauma, inflammatory or infectious bowel diseases, diverticulitis, necrotizing pancreatitis, hernia mesh erosion, radiation, and several invasive medical procedures, among others.8,9 but repeated abdominal ultrasonography never confirmed this. The iliac bone was probably no obstacle to fistulization because of patient osteoporosis secondary to advanced age and bed confinement, added to possible bone weakness due to the underlying bone marrow disease.The patient presented spontaneous trans-iliac bone faecal fistula related to a neglected and chronically incarcerated inguinal hernia. Her neutropaenic status, which worsened when MDS transformed into AML, could also have played a role in the physiopathology of the fistula formation. She just presented findings which were clinically thought to be recurrent episodes of neutropaenic enterocolitisWe could not find any cases of faecal fistula due to an underlying disease or condition which have taken a trans-iliac bone route from the intestine to the site of faecal discharge. This case report is being published in view of its singularity.Patients suffering from immunosuppressive haematological diseases can present rare and unexpected complications.Their complications can be mostly atypical or subclinical, evolving differently from those of immunocompetent patients.A combination of findings including old age, bone frailty, chronic neutropaenic status and chronic partial obstacle to normal faecal flow by chronically incarcerated inguinal hernia may have contributed to this rare trans-iliac bone faecal fistula.


Idiopathic pneumoperitoneum is an extremely rare condition that can easily be misdiagnosed as an acute abdomen. Awareness of this phenomenon can help avoid unnecessary surgical intervention and the potential associated morbidities.A 76 year old man presented to hospital with dyspnoea and productive cough and was diagnosed with an infective exacerbation of COPD. He subsequently had a CTPA which showed a significant amount of free intraperitoneal gas in the upper abdomen. He was completely asymptomatic from this with no abdominal pain, distension, or significant rise in inflammatory markers. Of note, he had never had previous abdominal surgery or endoscopic procedures. He showed no signs of peritonism and was closely observed with serial abdominal examinations for 24 h. He subsequently tolerated oral intake and was discharged 8 days after admission.Spontaneous pneumoperitoneum can be due to a variety of intrathoracic, gynaecologic, intra-abdominal and iatrogenic causes. This is a rare case of asymptomatic idiopathic pneumoperitoneum where no clear aetiology or risk factors were found for his free intraperitoneal gas. To the best of our knowledge there have only been two published case reports in the English literature describing idiopathic pneumoperitoneum in a patient that was completely asymptomatic from it.As a general surgeon it is important to be aware of the non-surgical causes of pneumoperitoneum. Knowledge of this uncommon condition may help reduce the risk of unnecessary laparotomy. In 90% of cases it indicates a surgical emergency as a result of perforation of an intra-abdominal viscus requiring urgent surgical intervention . The rem2A 76-year-old man presented to ED with a two-week history of progressively worsening dyspnoea and productive cough on a background of mild chronic obstructive pulmonary disease (COPD). He had a history of small cell carcinoma of the lung, treated with chemotherapy and radiotherapy, and was in remission. Of note, he had no prior history of abdominal surgery or endoscopic procedures. His regular medications included salbutamol, tiotropium, aspirin, and thiamine. He did not complain of any abdominal pain or distension. He had a healthy appetite and was opening his bowels normally.9/L (>11 × 109/L) and CRP was 10 mg/L (>5.0 mg/L). ECG showed sinus tachycardia and his CXR did not show any obvious consolidation. He was treated as an infective exacerbation of COPD, with antibiotics, bronchodilators, and chest physiotherapy. He subsequently had a CTPA to rule out a pulmonary embolus which showed a moderate amount of intraperitoneal free gas in the upper abdomen  – all other vital signs were within normal limits and he was afebrile. His abdomen was soft and non-tender, with no apparent distension. There were no signs of peritonism. He had a reducible, non-tender left inguinal hernia. His WCC was 12 × 10 abdomen  and 2.FiDespite this finding, he was completely asymptomatic with no abdominal pain or distension throughout his hospital admission. His CTPA did not show a pneumothorax or pneumomediastinum, and his recent abdominal CT scans did not show signs of pneumatosis cystoides intestinalis as potential sources of the free air. The general surgical team reviewed the patient and diagnosed him with idiopathic pneumoperitoneum. He was treated conservatively with close observation and serial abdominal examination for the next 24 h. He was allowed oral intake with no clinical deterioration and was discharged on day 8 of his admission. The patient was seen one month later in the outpatient clinics and had remained asymptomatic.3Pneumoperitoneum is classically thought to represent a surgical emergency with gastrointestinal tract perforations accounting for over 90% of cases. There are, however, numerous other causes of pneumoperitoneum that do not require urgent surgical intervention . This caThe most common abdominal “non-surgical” cause of pneumoperitoneum is pneumatosis cystoides intestinalis (PCI) . This isTracking of air from the mediastinum into the retroperitoneum and peritoneal cavity is also a well-recognised phenomenon. Intrathoracic causes of pneumoperitoneum include thoracic trauma (such as barotrauma), pneumothorax, cardiopulmonary resuscitation, and invasive ventilation, particularly in cases with high peak inspiratory pressures. In such cases raised intrathoracic pressure leads to leakage of intrathoracic air through microscopic pleural and diaphragmatic defects, or retroperitoneal perivascular sheaths, and pneumomediastinum often co-exists ,13.The female genital tract can also provide a portal of entry for air into the peritoneal cavity in certain conditions. During laparoscopic investigation for infertility, dye insufflated via the external os can be seen emerging from the fimbrial ends of a patent salpinx . As a reThe most common iatrogenic cause of pneumoperitoneum is following abdominal surgical procedures (laparotomy and laparoscopy), and in the majority of cases the air is completely resorbed within two weeks after the procedure . There hFinally, there exists a group of patients with pneumoperitoneum who have no clear aetiology or demonstrable risk factors; these are true cases of idiopathic pneumoperitoneum . The maj4Idiopathic pneumoperitoneum is an extremely rare condition and can easily be misdiagnosed as an acute abdomen. It is a diagnosis of exclusion which should only be made once surgical and nonsurgical causes have been ruled out. We report a rare case of asymptomatic idiopathic pneumoperitoneum that was successfully managed conservatively with only serial abdominal examinations. It is hoped that greater awareness of this condition and a discerning approach to its management will help reduce the negative laparotomy rate in such patients.The authors report no declarations of interest.None.Ethical approval is not applicable.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Dr Masood Sidiqi and Tasfeen Billah  contributed in medical record review, literature search, and writing of the draft. Dr David Fletcher contributed towards review of the paper.N/A.Dr Masood Sidiqi.Not commissioned, externally peer-reviewed.


Fibroepithelial polyps are benign tumors of mesodermal origin that usually arise on the surface of the skin and to a lesser extent in the urinary tract; however, their presence on the penis is extremely unusual. We report the case of a 73-year-old male with an extremely large broad-based penile fibroepithelial polyp (FEP) involving the penile shaft and glans penis associated with chronic condom catheter use and that was treated with partial penectomy. A review of the literature is included to highlight the rarity of this case. To the best of our knowledge, this is the largest mass of its kind to be reported on the penis. Fibroepithelial polyps (FEPs) are benign neoplasms that are mesodermal in origin. Histologically, they have a characteristic squamous epithelial surface with an underlying fibrovascular stroma, and definitive histology is needed to rule out malignancy . We hereA 73-year-old male patient presented to our clinic complaining of a painless penile mass which has appeared 4 years ago and gradually been increasing in size. The patient has been known to have urinary urge incontinence for the last 17 years following spinal surgery. Since then, the patient has been using a condom catheter.As the growth of the mass accelerated over the last 4 months, it became harder for the patient to have good hygiene and the condom could not fit anymore for the huge mass, and so he started to design special plastic bags for that purpose. In addition, the mass itself was becoming a source of a very bad odor. The patient has been complaining of erectile dysfunction, stool incontinence, and bilateral lower limb weakness since the time of spine surgery. The patient has a good urinary stream and has neither dysuria nor hematuria. He reported neither the presence of similar skin lesions, previous penile surgeries (apart from circumcision during childhood), nor a history of recent travels. The patient denied using penile constrictive rings or vacuum devices. There was no history of trauma. He was not taking medication.On examination, the patient was having a huge (15 × 10 × 8 cm), nontender, “grape-like” penile firm mass originating from the ventral aspect of the penile shaft and glans penis Figures . The lesUpon presentation, the serum creatinine level was 2.3 mg/dl. Urine analysis and culture showed the presence of urinary tract infection. The nonenhanced urinary tract computed tomography scan showed bilateral mild hydroureteronephrosis down to the urinary bladder. Ultrasonography showed a postvoid residual volume of 350 cc. 18-French Foley's catheter was inserted easily and kept in situ. The patient was admitted to the hospital and managed with intravenous fluids and antibiotics. His creatinine became 1.18 mg/dl.Cystourethroscopy was performed and showed a normal-looking urethra, enlarged prostate, and severe trabeculations of the urinary bladder. At the same time, an incisional biopsy was performed which showed a benign fibroepithelial polyp with no evidence of malignancy. The situation was discussed with the patient and his family. Partial penectomy with excision of the whole mass was performed under general anesthesia. As the lesion was having a very broad base, it was not possible to excise the mass with preserving the penis. The postoperative course was uneventful. Follow-up for two years showed no recurrence.The final histopathology of the specimen confirmed the previous diagnosis of a benign fibroepithelial polyp with no evidence of malignancy. It showed a hypocellular collagenized, edematous, and vascular stroma. The stroma contained patchy perivascular lymphocytic aggregates. Furthermore, few smooth muscle cell strands were noted. The surface was covered by benign acanthotic stratified squamous epithelium Figures  and 4. SWe have performed a review of the English-written literature using PubMed and Scopus looking for these terms: “fibroepithelial polyp” and “penile fibroepithelial polyp”. The references were reviewed from the available papers and studied. At the end, 25 papers were selected.To the best of our knowledge, the literature describes only 25 cases of FEPs on the surface of the penis. The age reported in the studies ranged from 25 to 97 years, with the exception of 5 cases reported in children. Eleven of the adult cases, which constitute the majority, reported the long-term use of a condom catheter. 8 adult cases denied the use of the condom catheter. The remaining adult cases practiced male genital hanging kung fu , 3 or thIt should also be noted that 17 of the cases occurred on the glans penis, making it the most common site. Other sites of manifestation were either on the frenulum, ventral surface of the penis, or penoscrotal junction .The overall prognosis of penile FEPs is good, with only 1 of the 25 cases transforming into squamous cell carcinoma [FEPs are benign tumors that arise from the mesoderm . They moThe etiology of penile FEPs is generally unknown but has been strongly linked to chronic, improper use of condom catheters . It was Potentially, these FEPs could arise from the irritation of the glans , not onlIn relation to the histopathology, all pathology reports from the previously reported cases similarly stated the characteristic fibrovascular edematous stroma covered by keratinized squamous epithelium –17. AlsoIn this reported case, the chronic use of a condom catheter by the patient might have predisposed to the development of the giant mass. It is important to note that the poor quality of hygiene the patient maintained might have played a significant role as well.It is worth mentioning that upon the revision of the related literature, the current case we present has the largest mass recorded and at a maximum diameter of 15 cm. Partial penectomy was indicated in the case of our patient due to the replacement of the penile shaft and surrounding skin with the mass. Local complete excision of the mass with preserving the penis was impossible for us. We believe this procedure has never been performed in the case of a penile FEP according to the published data.Finally, the postoperative course of the patient was uneventful; hence, we conclude that wide local excision and partial penectomy can be a good and satisfactory treatment option in the case of large polyps such as this. Additionally, our patient might solidify the theory that chronic, improper use of a condom catheter may predispose to the development of penile FEP. The role of hygiene should be noted as well and mentioned to all patients using condom catheters.We underwent a review of the English-written literature using PubMed looking for the term: “penile fibroepithelial polyp”. Besides, we reviewed the references of the available articles.About 25 cases of a penile fibroepithelial polyp were reported in the literature .


Extra osseous Ewing sarcoma (ES), an uncommon malignant neoplasm, accounts for about 15% of Ewing sarcoma, which mainly affects paravertebral region, lower extremity, chest wall, retroperitoneum, pelvis, and hip. Here is a 54-year-old woman of primary vaginal Ewing sarcoma with uterine fibroid, which has been fewly known or reported.The patient was admitted to our hospital because of vaginal pain. Her uterus showed as parallel position and enlarged as about 3 months of pregnancy size.Magnetic resonance imaging (MRI) and ultrasonography (US) demonstrated 2 heterogeneous masses in the vagina and uterus, respectively. Ultrasound-guided puncture biopsy revealed a malignant tumor in the right lateral vaginal wall.The patient was treated by hysterectomy, bilateral salpingo-oophorectomy, and tumors excision, with the subsequent treatment of chemotherapy.The patient recovered well without local recurrence for >1 year.Primary vaginal Ewing sarcoma is extremely rare. The treatments of uterine fibroid include uterine artery embolization and surgical options, While wide local excision followed by adjuvant chemotherapy and/or radiotherapy should be recommended for the vaginal ES. A 48-year literature review from 1970 to 2018 revealed that <30 cases of primary vaginal Ewing's sarcoma have been previously reported, from which few had discussed the imaging findings about primary vaginal Ewing sarcoma.Vaginal ES and uterine fibroid have certain manifestations and imaging features in MRI and US, but not specific.In addition, transvaginal ultrasound-guided puncture biopsy from the vaginal mass revealed the diagnosis of Ewing sarcoma.1 Extraosseous Ewing sarcoma (EES) grows rapidly, with round-cell malignancy of uncharacterized mesenchymal cell origin. Virtually, all Ewing sarcomas share a common chromosomal translocation of the long arm of chromosome 11 and 22. The translocation of the EWSR1 gene on to chromosome 22p12 next to the FLI1 gene may cause upregulation of insulin-like growth factor 1, playing a key role in cellular proliferation. The common translocation and a strong membranous expression of CD99 could unify the diagnosis of extraosseous Ewing sarcoma. A 48-year literature review from 1970 to 2018 revealed that <30 cases of primary vaginal Ewing's sarcoma had been previously reported,,2,4 and few literatures had discussed the imaging findings of primary vaginal Ewing sarcoma. In addition, uterine fibroid is the most commonly benign tumor in female pelvis, and occurs in about 20% to 50% of women around the world, from which black women of reproductive age have the highest incidence.–7 We present a rare case of a 54-year-old woman with primary vaginal Ewing sarcoma accompanying with uterine fibroid. Lesions were detected by ultrasonography (US) and magnetic resonance imaging (MRI) examination.As a highly malignant neoplasm of bones, Ewing sarcoma (ES) usually occurs during childhood, and nearly 15% of ES are extraosseous.2A 54-year-old woman was admitted to our hospital for the pain in the vagina. She had been with the symptom for about 4 days. Upon gynecological examination, the vaginal tumor had the following characteristics: painful mass, indurated and immobile, with the size of 6.5 × 6.0 × 4.0 cm. The mass lesion arised in the right lateral vaginal wall, extending to labia majora. Her uterus showed as parallel position and enlarged as about 3 months of pregnancy size. Cervix and all the vaginal fornices were free from mass.MRI images revealed a 7.5 × 4.9 cm solid and cystic mass, which was identified in the ischiorectal fossa adjacent to the right lateral vaginal wall. The mass had obscure margin, while lesion's edge showed both restricted diffusion and marked enhancement. However, the location of the mass was considered to be indeterminate on MRI. Additionally, the uterine mass, measured 6.7 × 4.8 cm, had smooth and well-defined margin, but with inhomogeneous enhancement Fig. A–E.US images showed a hypoechoic or anechoic mass in the right lateral vaginal wall, measured 6.72 × 4.66 cm, with no obvious blood flow signal Fig. . CT scan2, Etoposide 62.8 mg/m2). After 6 weeks of combination chemotherapy, there was a complete remission of the tumor by response evaluation criteria in solid tumors (RECIST) criteria. For >1-year follow-up, the ultrasound and other clinical examination demonstrated that the patient remained free of local recurrence and metastasis.Final surgical pathology of the 2 masses was confirmed as vaginal ES and uterine fibroid, respectively , cystic, calcified, myxomatous, lipomatous, and sarcomatous, and the degenerations are rarely pure occurring in fibroid.Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered as single tumor category known as Ewing family of tumors (EFTs), There have been less case reports describing the imaging appearance of vaginal ES. On MRI examination, the tumors may be T2 isointense to hyperintense, T1 isointense to muscle, with heterogeneous contrast enhancement, while the tumor could demonstrate a heterogeneous ultrasonic appearance with few Doppler color flow. During ultrasound examination, fibroids usually appear as well-defined, solid, concentric, hypoechoic masses, with a variable amount of acoustic shadowing. MRI and computer tomography might be helpful in the diagnosis of uterine fibroid. A nondegenerated fibroid is usually as isointense to myometrium on T1 and hypointense on T2 sequences, and shows avid enhancement in MRI scans. Furthermore, different forms of degenerations may occur simultaneously in a given fibroid, thus the categorization on imaging may be based on the predominant imaging feature.The imaging findings of extraosseous ES is usually presented as a hypervascular soft tissue mass with areas of necrosis or hemorrhage. The tumors may also invade adjacent bones, but do not affect the bone marrow. Calcifications could appear in up to 25% of cases.Our case demonstrates a solid and cystic mass with obscure margin of the vaginal ES, while lesion's edge showed both restricted diffusion and marked enhancement in MRI images. Similar heterogeneous characteristics were seen on US, which manifested as a hypoechoic or anechoic mass, with no obvious blood flow signal inside. The MRI and US features could be interpreted by extensive area of necrosis or hemorrhage, which are the pathological features of vaginal ES. On MRI, the uterine fibroid of our case had smooth, well-defined margin, and heterogeneous enhancement, with a little contrast agent entering the mass, which may be owing to tumor degeneration.–17 On MRI, the proposed diagnostic imaging criteria for extraskeletal ES relied on the absence of adjacent bony invasion. Our case had no bone invasion, but the location of vaginal mass was considered to be indeterminate on MRI. In contrast, US could present the accurate location of the vaginal tumor. In comparison with MRI, US has several advantages, as it is widely available, well tolerated for patients, and it could be combined with a ultrasound-guided puncture biopsy, which is a safe, fast, and reliable method for pathological diagnosis.Differential diagnosis for a patient with an aggressive pelvic (extraovarian) lesion should include rhabdomyosarcoma, extraskeletal ES, synovial sarcoma, malignant melanoma, and some less frequently carcinoma. A standard treatment guidelines available for vaginal ES is lacking due to rarity of its occurrence.,20 Wide local excision followed by adjuvant chemotherapy and/or radiotherapy is recommended. The outcome for the tumor is primarily dependent on the presence of distant metastases. In the absence of detectable metastases, 70% of children and adolescents are cured with a combination of intensive, multiagent chemotherapy with surgery and/or radiotherapy.,21 As to the present case, the patient was treated by hysterectomy, bilateral salpingo-oophorectomy and tumors excision, with the subsequent treatment of chemotherapy. The patient had remained free of local recurrence and metastasis for >1-year. Given the possibility of local recurrence, it is essential to have continuous close follow-up of the patient.Most uterine fibroids do not require treatment unless they are causing symptoms. After menopause, fibroids shrink, and it is unusual for them to cause problems. In those who have symptoms, uterine artery embolization and surgical options have similar outcomes with respect to satisfaction.In conclusion, we report an extremely rare case of vaginal ES with uterine fibroid. Imaging is crucial for defining the extent of disease, evaluating response to treatment, and detecting recurrent/metastatic disease. Vaginal ES and uterine fibroid have certain imaging features in MRI and US, but not specific, while ultrasound-guided puncture biopsy plays a key role in confirming the diagnosis of vaginal ES.The protocol for the present retrospective study was approved by the Ethics Committee of Hubei Cancer Hospital, and informed consent was obtained from the patient.Conceptualization: Gaolong Weng, Fang Li.Investigation: Shue Zeng, Hongbin Wang.Writing – original draft: Maolin Xu, Fang Li.Writing – review & editing: Yulin Liu, Shue Zeng.


Even routine diagnoses, such as septal deviation, which most people do not think need imaging, require careful examination because rare diagnoses such as lipoma may occur in the nose. Careful examination and imaging lead to the best treatment. Even routine diagnoses, such as septal deviation, which most people do not think need imaging, require careful examination because rare diagnoses such as lipoma may occur in the nose. Careful examination and imaging lead to the best treatment. Computed tomography (CT) without contrast revealed unusual septal thickness, and subsequent magnetic resonance imaging showed an amorphous heterogenous fat content mass. The mass was excised using submucoperichondrial approach with no complication.The lipoma is the most common soft tissue neoplasm in adults. Its highest incidence is among those aged 40‐50 years and is slightly more prevalent in males. It accounts for differential diagnosis of nasopharynx mass as a benign one that, in most cases, is located in the upper back, shoulders, arms, buttocks, and upper thigh.2A 22‐year‐old man presented to our hospital with left‐sided nasal obstruction for more than 2 years. The patient's medical history, including the previous history of allergy or surgery, was nonspecific. In physical examination, left‐sided septal bulging, which was soft and compressible, was observed Figure . A more 3Lipoma is a benign slow‐growing neoplasm that mostly consists of mature adapoid cells. Thirteen percent of lipomas occur in head and neck, which the posterior neck, chicks, tongue, floor of the mouth, and buccal sulcus are the most common.There have been reports of pediatric nasal lipoma and their associations with midline facial defects and different syndromes.In our study, nasal septal lipoma was reported in a 22‐year‐old man. Tumor removal was done using the mucoperichondrial flap. Differential diagnoses of nasal lipoma are dermoid cysts, teratoma, glioma, encephalocele, and meningomyelocele, which all of them may resemble a deviation if the septum is examined in less than a meticulous way4Lipoma of the nasal septum is a rare presentation. We reported a case of nasal septal lipoma, presented with a nasal obstruction that could easily pass as a septal deviation without a conscientious examination. Performing imaging studies before septoplasty in cases of nasal obstruction with questionable physical examination could lead to a precise diagnosis.Authors declare no conflict of interest.HJ, MR, and FDF: conceptualized and designed the work. HJ, FDF, MDF, DJ, and MR: critically revised the article. All authors: approved the final version and have the agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.This study protocol was approved by the local ethics committee of the Iran University of Medical Sciences. Informed consent was obtained from the patient before the study.


Ewing's sarcoma is an uncommon malignancy primarily affecting bone tissue and usually occurs in adolescents and young adults. This paper reports a rare case of extra-skeletalEwing’s sarcoma of the oral cavity soft tissue. In the clinical examination, a mass of 1.5×1.5cm in diameter was observed in the right mandibular vestibule.Radiographic examination revealed no involvement of mandible. Microscopically, the tumor was composed of malignant small round cell tumor that exhibited immunoreactivityfor CD99. Enucleation surgery under local anesthesia was performed for the patient and prognosis was excellent. The patient remained symptom-free after 13 months of follow-up. Ewing’s sarcoma (ES) which is first described by James Ewing in 1921 is a malignancy primarily affecting skeletal system and it is commonly occurring in adolescents and young adults  . ES ariBased on the results of previous studies, the incidence of ES in the head and neck region is uncommon, when it occurs; it generally involves the mandible and less frequently the maxilla  - 6. ThA 27-year-old female with 10-month history of a gradually increasing intraoral swelling visited the oral and maxillofacial surgery ward of private hospital, Tehran, Iran. According to the patient's remark, the swelling has become firm in the last two months. There was no relevant medical or family history or a history of trauma, smoking, alcohol, addiction, and medication usage. On extraoral examination, there was no swelling and asymmetry. In addition, there was no lymphadenopathy.Intraorally, a removable pinkish-red dome shape exophytic lesion measuring 1.5×1.5cm in the right mandibular vestibule, in the region of right first and second premolar was noticed . On palpation, the swelling was non-tender, rubbery in consistency with telangiectatic vessel on its surface and no ulceration was noted.Clinically the lesion resembled to benign salivary gland tumor. Panoramic radiograph showed no involvement of mandible. Aspiration was performedand the result was negative. The mass was enucleated under local anesthesia via vestibular incision with maintenance branches of mental nerve. Tissue was sent for histopathological examination.Grossly, the tumor was well-circumscribed and encapsulated . The H &Considering that immunohistochemistry as the mainstay of diagnosis, and in order to rule out other malignancies, immunohistochemistry assessments were performed. The tumor cells showed membrane positivity for CD 99 and were negative for leukocyte common antigen (LCA) and P63. These features were conclusive of ES .In order to rule out metastatic origin of tumor, whole body scan and MRI with/ without gadolinium were carried out. A well-defined mass in subcutaneous regionover the right mandibular bone were observed. The tumor was high signal and heterogeneous on T2W images. On T1W images, the tumor was isointense to the musclesand on T1W post-contrast scans, heterogeneous enhancement was seen . In addiEES is a rare, rapidly growing malignant neoplasm that predominantly affects adolescents and young adults between 10 and 30 years of age, whereas the disease is distinctly uncommon in individuals before age 5 and after age 30  - 8. OvHigh-resolution computed tomography (CT) and MRI scans are not specific but useful in the diagnosis and treatment planning. In the present study, we preformed MRI with/without gadolinium and whole body scan in order to rule out metastasis. The MRI features revealed isointensity to the muscles on T1W and heterogeneous enhancement on T1W postcontrast scan. These findings were similar to previous studies  .Biopsy is necessary to make a definitive diagnosis, and the tumor must be distinguished from similar tumors and conditions  . For thDepending on the site of the tumor and its extension, treatment of EES includes multimodality approach consisting surgery, chemotherapy and radiotherapy. Overall, the prognosis of ES is poor  ; our paInformed consent was obtained for publishing her clinical photography and radiography.Although EES is quite rare, it should be take into consideration as differential diagnosis of intraoral masses. The present study highlights a rare case of EES in third decade of life and presented as mass in soft tissue of right mandibular vestibule with excellent prognosis and without recurrence.


Rathke’s cleft cysts (RCCs) are benign cystic lesions from the remnant cells of the craniopharyngeal duct within Rathke’s pouch. Anticipation of such condition will help planning the treatment course.We present a case of a 46-year-old male, referred to our tertiary center with a history of seizures, confusion and agitation for the past two weeks. After thorough investigations, he was found to have hyponatremia with low serum cortisol and hypothyroidism. After being sedated, intubated and stabilized, Magnetic Resonance Imaging (MRI) brain showed relatively medium to large sellar lesion which was highly suspicious of a craniopharyngioma (CP). However, after performing an endoscopic transphenoidal surgical resection of the lesion, clinical diagnosis supported RCC.The best treatment approach for CP is a complete surgical resection via a transphenoidal approach. Considering the fluid component of RCC, it may be treated with a more conservative surgery and does not necessitate a surgery as invasive as that of CP.Given the mixed solid and cystic nature of CPs, it is possible to confuse it with RCC and vice versa. The presented case highlights that Rathke’s cyst may be challenging in terms of pre-operative diagnosis, peri-surgical management and histopathological examination. At autopsy, they are found in 13%–33% of patients . Most RCThe case presented to our tertiary center highlights that Rathke’s cyst may be challenging in terms of pre-operative diagnosis, peri-surgical management and histopathological examination. The work has been reported in line with the SCARE guidelines .2A medically-free 46-year-old male was referred to our tertiary center with a history of a recent-onset generalized tonic clonic seizure two weeks prior to his presentation. He had five episodes of these generalized tonic clonic seizures, each lasting for approximately two minutes. One week later, he became confused as he could not recognize his family and became very agitated. His symptoms were not associated with any visual changes, fever, chills or neck stiffness. He had a positive history of on and off headache since he was 20 years old that did not require any medications. His past medical and surgical history, family history and drug history were all unremarkable.Upon examination, he was found to be drowsy, agitated and confused but was able to obey commands. His Glasgow coma scale was 15/15. His vital signs were all within normal ranges. The rest of the neurological and the respiratory examinations were found to be unremarkable. He was put on leviteracetam 500 mg orally twice per day for his seizures as well as haloperidol 5 mg IM four times a day as needed for his agitation.The patient was seen by endocrinology and was found to have a low adrenocorticotropic hormone (ACTH), a low PM cortisol, hyponatremia and a high thyroid stimulating hormone (TSH). Synacthen test was within normal range. He was put on hydrocortisone 20 mg q am and 10 mg q pm initially then decreased to 10 mg am and 5 mg pm as well as levothyroxine 75 mcg daily. Unfortunately, the patient started to develop extra-pyramidal symptoms presenting as abnormal movements in the lower limb and orofacial dyskinesia. It was believed to be caused by the haloperidol, which was therefore stopped. It was also believed that his agitation could be caused by leviteracetam, hence it was switch to sodium valproate 500 mg twice daily and clonazepam 1 mg once daily at bedtime.After being sedated and stabilized, on MRI image, he was found to have a sellar/supra-sellar lesion measuring approximately 3.04 × 2.21 cm, predominantly cystic in nature with prominent calcification of the anterior wall. On T1 imaging, it was found to have a hyperintense signal. There was resultant expansion of the sella and scalloping of its walls as well as elevation of the optic pathway. The lesion was closely related to the anterior cerebral arteries, supra-clinoid internal carotid arteries and the basilar artery A and B. Pre-operatively, the patient required to have a stress dose of hydrocortisone 100 mg IV. Surgical transphenoidal approach was done for complete resection of the CP. The solid component of the lesion was sent for frozen section and the fluid was sent for cytology. The patient was shifted to the ICU in a stable condition. There were no surgical complications.Cystic fluid cytology confirmed cytomorphological features consistent with cyst contents and negative for malignant cells. Histopathological examination of the supra-sellar lesion revealed microscopic focus of glandular tissue suggestive of salivary origin within a background of vascularized connective tissue. The supra-sellar tumor also revealed dense fibrous tissue with calcification, old hemorrhage, cholesterol clefts and hemosiderin laden macrophages.Upon endocrinology, otolaryngology and neurosurgery clinic follow ups, the patient was found to be stable, seizure free, and without active complaints. He was still found to have panhypopituitarism and was maintained on hydrocortisone and levothyroxine. Post-operative EEG was normal. Post-operative MRI, done 9 months later, revealed residual calcified component at the anterior surgical margin, asymmetric to the right side in the supra-sellar region (with resultant elevation of the optic chiasm) and symmetric to the left side in the sellar region. There was no acute infarct, hydrocephalus or herniation syndrome C.3Rathke’s cleft cysts (RCC) are uncommon benign cystic lesions of epithelial remnants of the corresponding Rathke’s pouch with undetermined pathogenesis . The incCP is a benign tumor of central nervous system (CNS), which accounts for less than 1% of all CNS tumors. Although it’s considered a benign tumor, CP of sellar and suprasellar regions frequently causes invasion to neurovascular structures including pituitary gland and optic apparatus .The patient reported in this case had typical history of headache followed by a recent onset of seizures and endocrinological disturbances. However, he did not have visual manifestations. His rapid deterioration of symptoms might be related to the enlarging cyst size that might reach up to 4 cm with accumulating fluid and gelatinous material ,10. ManyRadiological examination aids in the diagnosis of RCC, where a well-demarcated cystic lesion is usually identified with variable intensity by MRI depending on the cyst contents . HoweverIn terms of the management, the recommended approach is trans-sphenoidal for drainage and biopsy of RCC. We have used the same surgical modality for our patient aiming at complete excisional biopsy . It is bSurgical complications have been reported and include: meningitis (due to leakage of the cystic fluid into the subarachnoid space), CSF rhinorrhea, abscess formation and diabetes insipidus ,17. In tIn conclusion, it is of importance to be familiar with the diagnosis of RCC and CP, and for them to be kept in the differential diagnosis of sellar and supra-sellar lesions in order to allow proper surgical planning, to prevent neurological and/or ophthalmological morbidity and avoid possible complications. The final histopathological diagnosis is challenging, as in this reported case. Therefore, clinical diagnosis can be established in similar cases.The authors whose names are listed certify that they have no affiliations with or involvement in any organization or entity with any financial interest, or non-financial interest in the subject matter or materials discussed in this manuscript.This case report has been approved by the HEC/IRB at King Faisal Specialist Hospital and Research Center according to the declaration of Helsinki.Informed consent was obtained by the patient for the publication of this case report and images.1.Faisal A. Farrrash: Substantial contributions to the conception or design of the work. Final approval of the version to be published.2.Maher Hassounah: Substantial contributions to the conception or design of the work; or the acquisition.3.Hala A. Helmi: Drafting the work or revising it critically for important intellectual content.4.Eyas Othman : Substantial contributions to the conception or design of the work.5.Naif H. Alotaibi: Substantial contributions to the conception or design of the work.NA.Dr. Faisal Farrash.Not commissioned, externally peer-reviewed.The authors have no potential conflict of interest to declare.


Splenic injuries after colonoscopy are an uncommon complication, which can lead to potentially unfortunate outcomes. Their management depends on the type of the splenic damage . We describe the case of a woman who visited the Emergency Department due to abdominal pain and pre-syncopal condition, which had occurred 12 hours after she underwent a colonoscopy. An abdominal computed tomography scan showed a splenic hematoma and a hemoperitoneum. An emergency splenectomy was performed successfully. Emergency physicians, who are at the forefront of diagnosing and treating patients, should consider this post-endoscopic complication in order to implement a prompt treatment. Splenic rupture is a rare complication that may occur following colonoscopy. The first description of this condition, due to an endoscopic procedure, dates back to 1974 -4. The m3; neutrophils 85.1%) and plasma D-Dimer 359 (NR: 2-200 ng/ml) associated with anemia (hemoglobin 10.7 g/dl) were present. Renal, bilio-hepatic, and pancreatic functions were normal. Physical examination revealed signs of peritoneal reaction to palpation. At this point, it was decided to perform an abdominal focused assessment with sonography (FAST), which demonstrated a hemoperitoneum. A subsequent computed tomography (CT) with intravenous (IV) contrast highlighted, in addition to the hemoperitoneum (yellow arrows), the presence of a hematoma due to the rupture of the spleen (white arrow), as shown in A 75-year-old woman was admitted to the Emergency Department due to abdominal pain associated to a pre-syncopal condition. Twelve hours earlier, she had performed a complete colonoscopy as a post-polypectomy surveillance at another hospital. The patient reported an open-cholecystectomy, denied any kind of trauma in the previous days and mentioned that she was suffering from Hashimoto's thyroiditis and arterial hypertension, for which she was regularly in treatment with levothyroxine sodium and calcium channel blocker. No anticoagulant/antiplatelet agents were taken. On admission, the oral temperature was 38.6 °C, blood pressure 105/55 mmHg, pulse rate was 98/minute and the respiratory rate 17/minute. Increases in white blood cell count  and a copious intra-abdominal hemoperitoneum with perihepatic fluid collection (yellow arrows), in both arterial  and portIn a 2016 review, Jehangir A et al. identified 172 cases of splenic rupture and noted an incidence of 1–21/100.000 for this complication, pointing out that it had a higher predominance among females (71.5% of cases), with a mean age of 63 years, and a previous history of abdominal surgery in 50.8–65% of cases . As alreSplenic rupture may be a dangerous complication of colonoscopy. FAST abdominal sonography performed in the emergency room certainly represents an excellent first diagnostic examination in suspected cases. Nevertheless, abdominal CT scan represents the gold standard exam for detecting splenic rupture and splenectomy is required in most of cases.


Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors with distinct morphological and biological manifestations, the liver being the main organ affected by its metastases. However, primary hepatic involvement is quite rare. Hepatic NENs can have a variety of radiological presentation forms and can therefore mimic other lesions, making their diagnosis challenging. Nonetheless, certain imaging aspects allow NENs to be included among the main differential diagnoses of hepatic lesions and can guide the search for an extrahepatic primary site when the probable diagnosis is metastases. They can be associated with genetic factors , although most NENs are sporadic tumors. In recent years, mainly due to advances in diagnostic methods, there has been an increase in the incidence of NENs in the United States, where it rose from 1.52 cases/100,000 population in 1973 to 7.41 cases/100,000 population in 2012.Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors with diverse morphological and biological manifestations that may appear in any tissue, the gastrointestinal (GI) tract being its most common primary site, accounting for 66-74% of cases. It is also an important biomarker for patient follow-up.The diagnostic workup of an NEN includes hormone tests, imaging tests, and pathology studies. In patients with symptoms related to hormone production, biochemical tests should be requested to investigate the corresponding syndrome. For example, a 5-hydroxyindoleacetic acid test in a 24-h urine sample should be performed for carcinoid syndrome (serotonin hypersecretion). The diagnostic biomarker of choice for symptomatic and asymptomatic cases is chromogranin A because it is the most sensitive.Collecting samples for anatomic pathology studies is of great importance for making the definitive diagnosis of an NEN. The access route will depend on the site affected. For example, endoscopic ultrasound is used for pancreatic neoplasms whereas percutaneous access is used when there is liver involvement. In the latter case, ultrasound-guided percutaneous access is preferred, although computed tomography (CT) guidance can be used in more difficult cases18F-fluorodeoxyglucose (18F-FDG) or 68gallium DOTATATE. In cases of NENs, CT and MRI are used for locating and staging the disease, defining the extent of the primary lesion, and determining whether or not there are any metastases,5. In most cases, regardless of their location, the lesions are hypervascular, hence the importance of the arterial phase in imaging protocols. However, depending on the type, size, and location of the tumor, the portal and late phases are also key for a more effective analysis. On MRI scans, in addition to a hypervascular enhancement pattern, lesions usually present a hyperintense signal on T2-weighted sequences and significantly restricted diffusion on diffusion-weighted imaging (DWI). The DWI technique, combined with the use of a hepatobiliary-specific contrast agent, has increased the sensitivity of MRI in detecting liver metastases, including those originating from NENs -6.The radiological methods used for investigating NENs include CT, magnetic resonance imaging (MRI), and nuclear medicine tests, especially positron emission tomography/CT (PET/CT) with 18F-FDG radiotracer is mainly used to visualize the metabolic activity of poorly differentiated NENs, whereas 68gallium DOTATATE is used for well-differentiated tumors.Images obtained with PET/CT are used for detecting and staging tumors, as well as for informing and monitoring the treatment of patients with an NEN. In addition to defining the location of the lesion, PET/CT scans provide us with physiological information represented by increased glucose metabolism or expression of somatostatin receptors, depending on the degree of tumor differentiation. The . Primary hepatic NENs (PHNENs) are extremely rare, only approximately 150 cases having been reported to date (in the English-language literature). Diagnosing a hepatic NEN is challenging because its symptoms are unremarkable, indolent, and often nonspecific, as well as because its radiological findings are very similar to those of other liver lesions and their various forms of presentation.Approximately 15% of patients with an NEN have metastases. Most (46-93%) of the metastases from an NEN affect the liver, whereas the pancreas and small intestine are the most common primary sites,8,9.There are currently no definitive guidelines for the treatment of PHNENs and hepatic neuroendocrine metastases. The treatment plan is individualized, taking into account the site, stage, and degree of differentiation of the tumor, as well as the age, comorbidities, and symptoms of the patient, in a multidisciplinary approach in which surgery, chemotherapy, radiation therapy, transcatheter arterial chemoembolization, and the use of somatostatin analogues are all available options.Given the wide range of types and clinical characteristics of NENs, there is currently no consensus on how the affected patients should be monitored after treatment. Nevertheless, one could say that the follow-up of patients with liver metastases is individualized on the basis of their clinical status (including symptoms) and degree of tumor differentiation, making use of a combination of laboratory tests and tumor markers (including chromogranin A), as well as  imaging findings.In patients with a suspected PHNEN, one should try to confirm that there is no primary extrahepatic site. To that end, complementary tests, such as endoscopy, colonoscopy, and bronchoscopy, may be neededThe objective of this study was to illustrate the common and uncommon presentations of PHNENs and hepatic neuroendocrine metastases. We hope that the information provided will facilitate their diagnosis.. This type of neoplasm can occur in patients of any age, although it is mainly reported in adults (40-50 years of age), with similar distribution between men and women. There are no known risk factors for PHNEN, and the associated mortality rate is estimated to be 25%. In most cases (80%), it is not accompanied by metastases at the time of diagnosis.Only 0.3% of all NENs are PHNENs. Although the histogenesis of a PHNEN is unknown, it is thought to originate from ectopic pancreatic or adrenal cells in the liver, neuroendocrine tissue in the intrahepatic biliary epithelium, or chronic inflammation in the biliary tract causing intestinal metaplasia. Establishing a diagnosis can be challenging because the radiological findings are quite similar to those of other liver lesions such as hepatocellular carcinoma, cholangiocarcinoma, metastatic liver disease, and hydatid cyst. One of the diagnostic criteria for a PHNEN is the absence of lesions at other sites commonly affected by this type of tumor, such as the small intestine, the pancreas, and the lungs. Therefore, when an NEN is found in the liver, looking for an extrahepatic primary site is paramount.Because PHNENs are slow-growing tumors, with clinical manifestations that can be nonspecific or even absent, they are usually detected because of the appearance of symptoms caused by the mass effect in the liver and adjacent organs, such as jaundice, palpable mass, and abdominal distention, and pain. Approximately 5% of patients present with the classic carcinoid syndrome,14, as depicted in The radiological characteristics of PHNENs are not well known, probably because of the low number of cases reported. The lesions are usually single and heterogeneous, showing a hypervascular enhancement pattern that is more pronounced in the periphery and late enhancement in the center, with or without cystic areas (corresponding to necrosis) and a fluid-fluid level. The occurrence of metastasis depends mainly on the extent, degree of differentiation, and proliferative activity of the primary lesion. Liver metastases constitute the most important predictor of mortality in patients with an NEN. Among patients with an NEN, the five-year survival rate is significantly lower for those with liver metastases than for those without,15-30-40% vs. 75-99%.Metastases from an NEN account for approximately 10% of all liver metastases. The liver is the organ most commonly affected by NEN metastases. Just over half of NENs of the GI tract show liver involvement at the time of diagnosis, and metastasis occurs, on average, seven years after the appearance of the primary lesion. The small intestine  is the most common primary site.Although NENs of the GI tract have a slow progression, syndromic patients may present with symptoms related to hormone production and hypersecretion. In patients with nonfunctioning tumors, symptoms depend on tumor size and metastasis location, the most common clinical manifestation being nonspecific abdominal pain and the second most common being weight loss  or a single mass with cystic cavities and a fluid-fluid level (in cases of primary disease). The role of radiologists goes beyond diagnosis to include participation in the management, treatment, and follow-up of these neoplasms.