Posterior fossa atypical teratoid rhabdoid tumor (ATRT) is a rare childhood tumor usually associated with a dismal prognosis. Although upfront surgical gross total resection (GTR) has classically been the first line of treatment, new multimodal treatments, including two-stage surgery, are showing promising results in terms of overall survival (OS) and complication rate. We present a case of a 9-month-old child treated with two-staged surgery and chemotherapy. When deemed risky, multimodal treatments, including staged surgeries, can be a safe alternative to reduce surgical mortality and morbidity. At 23 months old, the patient had normal global development and no major impact on quality of life. We, therefore, discuss the most recent advancements from a treatment perspective, including molecular targeting.  Atypical teratoid rhabdoid tumors (ATRTs) are aggressive embryonal tumors with a standardized incidence of 0.15 per 100.000 inhabitants in Europe and of 75 cases per year in the US. It typically affects children younger than 3 years old, with a median age at diagnosis between 16 and 30 months ,3,4.First described in 1987 by Lefkowitz, it was initially identified as a primitive neuroectodermal tumor (PNET) .SMARCB1 (INI1) gene alteration on the loss of the long arm of chromosome 22 in 95% of all cases, and SMARCA4 (BRG1) gene alteration, located on chromosome 19p13.2, in the remaining population [Molecular analysis and technologies have allowed for a better understanding of this severe pathology, such as pulation .Nowadays, to diagnose this entity, INI1 or BRG1 inactivation must be found: these aberrations lead to tumorigenesis .New molecular discoveries have led to the current classification proposed in 2020 that divides ATRT into three main molecular subgroups, notably ATRT-TYR, ATRT-SHH, and ATRT-MYC ,10,11,12SMARCB1 is inactivated most often by heterozygous mutations completed by whole or partial chromosome 22 loss removing the second allele. It shows overexpression of tyrosinase [ATRT-TYR typically arises in the posterior fossa, in which rosinase ;SMARCB1 point mutations and overexpression of proteins in the SHH and Notch signaling pathways;ATRT-SHH (Sonic Hedgehog) occurs as either supra- (type 1) or infratentorial (type 2), with frequent compound heterozygous SMARCB1 deletions and MYC and HOX genes overexpression.ATRT-MYC is more commonly supratentorial with The introduction of this classification aims to unify the current knowledge and future research on targeted therapies for ATRT ,14.ATRT treatment still relies on surgery  with preserved neurological function) in order to time adjuvant treatments, especially radiotherapy that can lead to harmful effects on brain development ,15,16,17Even with trimodal treatment  depending on central nervous system (CNS) dissemination, metastatic (M+) ATRT OS at 3 years remains at 25% .Postoperative morbidity can be particularly high in posterior fossa ATRT, including hydrocephalus (HCP) in up to 80% of all cases, pseudomeningocele in up to 25%, as well as posterior fossa syndrome, meningitis, and lower cranial nerve palsies ,20,21.Tailored multistaged and multimodal treatments appear to be a safe strategy to control the disease and preserve the functional outcome.In this paper, we present a case of a 9-month-old female child with a posterior fossa ATRT treated with a partial debulking, neoadjuvant chemotherapy, and subsequent resective surgery. Secondly, we present a narrative review of the most recent relevant literature. Demographic, clinical, radiological, and surgical information on the patient was retrospectively collected at Geneva University Hospitals. Written informed consent was obtained from the parents.The detailed information about the pathologic characteristics was obtained from Geneva University Hospitals. We conducted restricted research using the keywords “Posterior Fossa” AND/OR “ATRT”, “EU-RHAB” AND/OR “Posterior Fossa”, “MULTISTAGE SURGERY” AND/OR “ATRT” in November 2022 in the following databases: PubMed/Medline and Google Scholar. The CARE guidelines were followed. The first author independently screened all titles and abstracts, and full-text copies of all relevant articles were obtained without the exclusion of pertinent studies. Research was collected from 1980 on in order to avoid obsolete pathological and molecular data. In addition, CNS ATRT was recognized as a separate entity and added to the World Health Organization (WHO) tumor classification of tumors in 2000 as a grade IV embryonal tumor . We exclThe child was 9 months old with eutocic delivery at 40 gestation weeks, and, according to the parents started to develop ataxia and hypotonia at 6 months old. She developed repeated morning and orthostatic vomiting. The head circumference was at the 98th percentile (46.5 cm), and a cerebral ultrasound (US) showed enlarged lateral ventricles .Clinical examination did not show any anterior fontanel bulging nor trismus. Whole brain and spine magnetic resonance imaging (MRI) was performed, showing a heterogeneous cystic-hemorrhagic lesion, partially calcified, in the posterior fossa, Measuring 46 × 34 × 49 mm and obstructing the IV ventricle and right Luschka foramen .In spectroscopy sequences an elevated choline peak and low N-Acetylaspartate (NAA) and creatine, were found, suggesting an ATRT . No leptMultidisciplinary consensus opted for upfront GTR. At the time of the first surgery, the child weighed 8 kg and measured 76 cm. Surgery, performed by an experienced senior microneurosurgeon, had to be aborted due to extreme blood loss from an intrinsically highly vascular tumor, and an external ventricular drainage (EVD) was left in place. A 460 mL blood transfusion, composed of red blood cells (RBC) and platelets (PLT), was given. After uneventful postoperative surveillance in the pediatric intensive care unit (PICU), the EVD was converted into a ventriculoperitoneal shunt (VPS) since the obstructive HCP had not been resolved. Moreover, foreseeing the need for an intrathecal treatment, and to avoid any manipulation of the VPS, an additional intraventricular Ommaya reservoir was left in place.Neuropathological analysis found a highly vascularized, densely cellular tumor showing a predominantly papillary architecture with solid areas A,B. TumoThe multidisciplinary pediatric CNS tumor board opted for chemotherapy following SIOPE ATRT01 (EudraCT Number: 2018-003335-29)/EU-RHAB Registry protocol . As induAt 6 weeks after starting induction chemotherapy (after 3 courses of induction chemotherapy), craniospinal MRI showed a partial response to the treatment, with a >50% volume shrinking and no leptomeningeal dissemination, as assessed by an experienced senior neuroradiologist consultant . ConsideThe second-look surgery was uneventful. The child weighed 9 kg and was 76 cm tall. A millimetric residual tissue was left in place, infiltrating the floor of the IVth ventricle at the bulbo-pontine junction on the right. Blood loss was estimated to be around 50 mL, whilst the patient was transfused with 2 RBC and of 1 PLT concentrate. A new postoperative MRI confirmed a millimetric enhancement at the right bulbo-pontine junction .Then 3 additional courses of chemotherapy were applied (with stem cell apheresis). Thereafter, 3 courses of high-dose chemotherapy, at least 28 days apart, each consisting of carboplatin, thiotepa, and autologous stem cell reinfusion on day 0 were performed.During the follow-up, the patient status was assessed by different consultants, including a pediatric neurosurgeon, pediatric oncologists and pediatric neuroradiologists. All treatment decisions were based on a consensus from the pediatric tumor board. Until now, overall follow-up lasted 14 months. At the last follow-up at 23 months old, the patient did not show any sign of recurrence, with stable enhancing tissue on the floor of the IVth ventricle. Clinical examination was normal, as was global development (as stated by a consultant infantile neuropsychiatrist) . No adveATRT is an aggressive tumor with poor prognosis and its outcome depends on age, location, and biological and histological characteristics . Considering its rare occurrence, posterior fossa ATRT remains difficult to cure, and even with multimodal treatments, the prognosis remains dismal, with a mean survival between 6 and 11 months .In the recent literature, the mean OS for ATRT patients does not exceed 12 months, and available series consider mixed cases of supra- and infra-tentorial cases . SimilarHistorically, the mortality rate in infants undergoing surgery for primary brain tumors in the first two years of life has undoubtedly exceeded that for older children, ranging from 7.3% to 33% depending on the nature of the tumor, tumor size, age at surgery, surgical blood loss, and actual definition of surgery-related death ,37,38,39When upfront GTR is deemed to be high risk, partial removal and a multistage approach can be offered as safe alternatives. Multi-stage approaches were first described for anatomically challenging complex lesions, mostly involving tumor removal and/or vascular exclusion for arteriovenous malformation (AVM) .Dandy, in 1925, described a vestibular schwannoma operated on in a two-stage surgery; Spetzler and his team described, in 1986, an AVM surgical resection anticipated by embolization ,42,43. Multi-staged surgery approaches have evolved into multistage treatment involving non-surgical treatments such as radiosurgery, chemotherapy, and targeted therapy.Until now, the only case reported in literature concerning a two-staged surgical treatment of a supra- and infratentorial ATRT tumor was described by Foreman et al. in 2016, in order to minimize surgical morbidity and the risk of postoperative complication .Considering ATRT’s anatomical and pathological complexity, clinical decisions should take into account the family’s expectations, molecular profile, long-term sequelae, and expert network pathology centralization. Together with the safest achievable GTR, methylation profiling data and tailored treatment are considered key factors to achieve longer OS, longer progression-free survival (PFS), and acceptable overall quality of life .Initial attempts to unify and categorize ATRT by immunohistochemistry were made by Torchia et al., who created two subgroups based on the existence (Group 1) or absence of ASCL1 overexpression (Group 2) . This clDifferent MRI studies are demonstrating correlations between specific radiological findings and the three different molecular subgroups .Following this taxonomy, a St. Jude institute study group compared two multi-institutional clinical trials with the aim of reporting molecular groups’ relevance to clinicopathologic features and the survival of children affected by ATRT.In their series, they profiled 64 ATRT cases as 21 TYR 33%), 30 SHH (47%), and 13 MYC (20%): the median age at diagnosis was lower in the SHH subtype and higher in the MYC subtype. They demonstrated that among infants, ATRT-TYR had the best OS, whilst ATRT-SHH was associated with metastasis and consequent inferior outcomes [3%, 30 SHIn the case of SMARCB1 mutations, there is no direct target, whilst other upregulated pathways can be sensitive to different molecules . Small mMoreover, ATRT-SHH was found to be sensitive to histone methyltransferase G9a (UNC0638), whilst ATRT-TYR and ATRT-MYC were found to be sensitive to PDGFRB enhancers (nilotinib and dasatinib) and to bone morphogenetic protein (BMP) ,49,50.Some of the new molecular inhibitors can be difficult to offer in the pediatric population: new types of orthotopic xenograft models have been created to study in vitro tumors’ responses to new potential treatments . Other pImprovements in molecular targeted therapy will include molecules able to simultaneously distinguish tumor cells from normal cells, as well as molecules able to avoid escaping mechanisms and to cross the BBB ,54.Other new cell engineering techniques are being deployed in clinical trials, such as chimeric antigen-receptor T-cells (CAR-T), which targets B7-H3 in ATRT-MYC in mice models and is showing promising results . These new engineering techniques seem to open new pathways to effective treatments implementing OS, but specific attention should be paid to short- and long-term direct drug toxicity, as well as neurological and reproductive sequelae ,57.Tumoral heterogeneity still remains a pivotal point in effective treatment . FinallyConsidering the rarity of this pathology, shared expertise has allowed research to widen and improve ATRT knowledge: the EU-RHAB Registry identified clinical factors influencing positive prognosis  or negatively  ,59. MultLastly, this global expertise should be shared as much as possible with children’s families: while in the past clinical decisions were often made solely by the surgeon/oncologist and accepted by the family, nowadays, the decision process is shifting towards an open discussion about risks and actual expectations before a common decision is made with the patient and his/her family .Pediatric posterior fossa ATRT is an heterogenous disease with unfavorable survival. GTR contributes in a relevant manner to the local control. When deemed risky, multimodal treatments, including staged surgeries, can be a safe alternative to reduce surgical mortality and morbidity, and to guarantee a good quality of life. New surgical technologies and targeted molecular therapies show promising results and could be integrated in such a multimodal approach after proving their efficacy. Further efforts should be put into multicentric studies.


She was an active smoker of 20 cigarettes a day and had an alcohol intake of 16 units/week. She had no significant family history of cardiovascular disease and did not take any regular medications. On admission to the emergency department, clinical examination was normal; however, serial 12-lead ECG showed transient inferior T-wave inversion. Routine bloods including full blood count, renal function, and liver function were normal. Troponin levels were mildly elevated and dynamic, and C-reactive protein was significantly elevated at 61 mg/L . A computed tomography aortogram was performed, which showed no aortic dissection and flagged no other pathology of relevance.A 45-year-old woman was admitted to a tertiary hospital with acute chest pain radiating to the abdomen. She had a history of mild hypertension and an elevated body mass index of 32 kg/mVideo S1). She was treated medically for a presumptive diagnosis of myocardial infarction with nonobstructed coronary arteries (MINOCA) with aspirin and statin, and was referred for an inpatient cardiovascular magnetic resonance (CMR) for further assessment.In view of the cardiovascular risk factors, dynamic troponin, and ECG changes, she underwent invasive coronary angiography. This showed a nonobstructive lesion in the left anterior descending artery with no other major abnormalities (CMR at 1.5T showed normal biventricular size and function with normal left ventricular ejection fraction of 58% and no regional wall motion abnormalities. On T1 and T2 mapping, there was no evidence of edema or diffuse myocardial fibrosis. Late gadolinium enhancement revealed no myocardial infarction or focal fibrosis. Unexpectedly, on the T1 maps, an enlarged and distended gallbladder filled by an unusual fluid was noted raising the suspicion of an exudative process (Figure She was reviewed by the gastrointestinal surgical team and proceeded to have further imaging including an ultrasound scan of the liver and magnetic resonance cholangiopancreatography, confirming an obstructing gallstone in the cystic duct with resulting gallbladder distension. The following day, she underwent urgent laparoscopic cholecystectomy with removal of a distended gallbladder Figure . Histopa1 Furthermore, our case highlights the additional utility of CMR in the identification of extracardiac causes of chest pain.CMR is recommended by contemporary clinical practice guidelines in patients with MINOCA to confirm the presence of an ischemic pattern and to exclude other nonischemic cardiac causes, having both a diagnostic and prognostic value.2 Even though the majority of extracardiac findings are likely to be benign, this was not the case for our patient who would have been discharged by the cardiology team if the pathology was not identified. This would have resulted in a delay in the diagnosis of acute cholecystitis leading to worse clinical outcomes, including possible severe sepsis, gallbladder perforation, and presentation as acute abdominal syndrome.3Recent research has shown that incidental extracardiac findings are seen in up to 35% of CMR studies, with major extracardiac findings in up to 12% and extracardiac findings changing management in 1% of patients.4 To the best of our knowledge, parametric mapping has not been previously shown to diagnose acute cholecystitis, highlighting the possible diverse application of the technique. Caution should be applied in the interpretation of the mapping images for extracardiac findings, and integration with other imaging modalities should be sought.While T1 mapping is usually utilized for myocardial assessment, more recently, a role has been shown in the assessment of pleural and pericardial effusions.The authors would like to thank Professor Ameet Patel and Francesca Forbes-Boulter for their help in this case.G. Sen is funded by the British Heart Foundation grant FS/CRTF/22/24368.None.Video S1


Echinococcus granulosus causes hydatid disease. The most affected organ is the liver which is followed by the lungs. The pleural cavity being the primary location of hydatid cysts is rare and should be discussed further. This paper documents a rare case of primary pleural hydatidosis which can present with a merely isolated cough followed by dyspnea. The diagnosis and surgical treatment along with post-operative medications are vital in this case.We present a case of a 45-year-old who suffered from a cough for more than one week which did not subside after taking medications. This symptom was followed by dyspnoea for which an X-ray was done which showed left-sided pleural effusion, a complication of pleural hydatidosis. Computed tomography showed multiple cysts in the pleural cavity which confirmed the diagnosis of primary pleural hydatidosis as the cysts were not present in any other sites. Blood work revealed eosinophilia which is significant in parasitic diseases. A left posterolateral thoracotomy was performed, and the cysts were surgically removed. Additionally, empyemectomy and pleurectomy were done. The patient was then treated with anti-parasitic therapy and was advised to get X-rays during the follow-up visits. The X-rays were normal and indicated that there was no disease recurrence.Echinococcus granulosus is a parasitic worm that causes hydatid disease. The primary location is the liver. A diagnosis of intrathoracic but extrapulmonary disease, which involves the presence of hydatid cysts in the pleura, heart, pericardium, mediastinum, chest wall, and diaphragm, is difficult in individuals lacking a primary cyst in a common location .This case implies the significance of a cough of more than a week that is not relieved by medications. This should be carefully evaluated and followed in cases that have a rare diagnosis requiring surgery. A diagnosis of primary pleural hydatidosis with left-sided pleural effusion and atelectasis with mediastinal shift to the right side was made which was treated with a surgical procedure. •Primary pleural hydatidosis can present with merely an isolated cough.•Pleural effusion is one of the complications of ruptured pleural hydatid cysts.•Posterolateral thoracotomy is done to remove the hydatid cysts.•Pleurectomy and empyemectomy are done in infection-induced pleural effusion. The parasites that form hydatid cysts are members of the genus Echinococcus. They are closely related cestodes that have adapted to a diverse range of host populations connected by predator and prey interactions .Hydatid disease caused by Echinococcus granulosus typically requires two hosts: one intermediate host  and one definitive host  ,3,8.About 60–80 % of hydatid cysts are found in the liver. The second most common location is the lung which has been seen in 10–30 % of cases of hydatidosis. A hydatid cyst in the pleural cavity as the primary location is very rare .One of the complications of a ruptured pleural hydatid cyst is a massive pleural effusion associated with atelectasis and mediastinal shift to the opposite side of the chest .We present a rare case of a patient who came with a mere presentation of an isolated cough for more than 1 week. This cough was not relieved by any medications. It was followed by a short presentation of dyspnoea for which an X-ray and a Computed tomography were done and a diagnosis of primary pleural hydatidosis was made with a complication of pleural effusion. He was taken to the operation room for surgical removal of the hydatid cysts from the pleural cavity.2A 45-year-old male presented to the emergency department complaining of a cough for more than a week. This cough was not relieved by any medications. It was followed by dyspnoea for 2 days. From the patient's history, we found that he is a schoolteacher from a middle-class family. The living conditions of the family are at subpar levels. He is a non-smoker and an occasional alcohol consumer. There is no evidence of contact with domestic animals like dogs, sheep, or cows. The patient lives with his wife and two children in his home with no domestic animals as pets or livestock. Contamination of food and water are a few environmental risk factors that the patient has.On physical examination, the patient had a normal heart rate, blood pressure, oxygen saturation, and temperature. His respiratory rate was significant for tachypnoea with 25 cycles per minute. On clinical examination, breath sounds were decreased on the left side, also vocal fremitus was found to be decreased, along with dullness on percussion on the left side of the chest. The right side of the chest showed normal clinical examination. Laboratory findings were significant for eosinophilia (12 %). A routine chest X-ray revealed rounded opacities and a large left-sided pleural effusion with a right-sided mediastinal shift. It did not reveal consolidation of the upper zone with ipsilateral hilar enlargement or any features suggestive of pulmonary tuberculosis.The patient was hospitalized urgently and was referred to the Respiratory Medicine Department for further evaluation and management. The patient was scheduled for a pleural drainage procedure. A pleural drainage was inserted in the 5th intercostal space anterior to the mid-axillary line on the left side of the chest. 2600 ml of cloudy yellowish fluid was drained within 24 h. A differential diagnosis of tuberculosis, malignancy, and parapneumonia was considered based on the imaging which showed large left-sided pleural effusion and exudative pleural effusion on seeing the cloudy yellowish fluid on drainage. The fluid drained was sent for protein, lactate dehydrogenase, Gram stain, cytology, and microbiological culture. The pleural fluid analysis confirmed an exudative pleural effusion.. He completed the pre-anesthetic check-ups and was posted for surgery by the Chief Surgeon.Computed tomography was done to confirm the suspicion of hydatid disease. The CT revealed multiple small cysts which are round in shape filling up the entire left pleural cavity. Left-sided pleural effusion was associated with left lung atelectasis and right mediastinal shift. Abdominal CT was done which showed no lesion in the liver suggestive of the absence of a primary foci of hydatidosis in the liver. The patient was referred to the Surgery Department for further management of the case. Preoperative antihelminthic treatment with Albendazole 15 mg/kg/day for 6 days was completedThe patient was taken to the operation room for a left-sided posterolateral thoracotomy in the 5th intercostal space. The pleural space was then irrigated with hypertonic saline to kill the scolices. Multiple daughter cysts were removed, and a thorough inspection showed no other cysts in the pleural space, lung, diaphragm, or mediastinum .Fig. 1–3A pleurectomy and an empymectomy were done in view of infection-induced pleural effusion. After the procedure was done, the collapsed lung was expanded fully, and no air leakage was found. Additionally, there was no sign of parenchyma damage to the lung. It was followed by the closure of the thoracotomy. The patient was shifted to the Intensive Care Unit and was then discharged when he was hemodynamically stable and symptomatically better with 10 mg/kg/day of oral Albendazole for 1 year. The patient was asymptomatic and showed normal chest X-rays in the follow-up visits done monthly and yearly.3Echinococcus granulosus is a parasitic worm that in adult form resides in the jejunum of dogs and other canines and lays eggs that are expelled in the stool .The intermediate hosts  ingest the eggs, which then release embryos that enter the portal circulation. These embryos mostly reside in the liver but also tend to spread to different organs in the body. A diagnosis of intrathoracic but extrapulmonary disease, which involves the presence of hydatid cysts in the pleura, heart, pericardium, mediastinum, chest wall, and diaphragm, is difficult in individuals lacking a primary cyst in a common location ,5.Primary pleural hydatidosis is the presence of hydatid cysts primarily in the pleural cavity. Pleural effusion, pneumothorax, hydropneumothorax, empyema, pleural thickening, and collapsed lung can result from cysts at this location rupturing and releasing their contents into the pleural cavity. One of the complications which is rare is an anaphylactic shock due to the rupture of a pleural or a lung hydatid cyst .In our case, the cysts were found only in the pleural cavity for which left-sided posterolateral thoracotomy in the 5th intercostal space was done to kill the scolices and remove the cysts. Additionally, we drained the parasitic pleural effusion. Because we were unable to locate any extra pleural lesions, we concluded that the diagnosis was primary pleural hydatidosis, which is an incredibly uncommon clinical condition. Pleural hydatidosis can resemble the clinical symptoms of pleural effusions such as dyspnoea, chest discomfort, dry cough, and a mediastinal shift to the opposite side. However, in 15 % of the cases, it can be asymptomatic .In our case, the patient presented with a cough for more than 1 week which did not subside with medications. It was followed by shortness of breath. When evaluated, the X-ray showed a left-sided pleural effusion and a lung collapse on the same side of the effusion. Computed tomography showed multiple cysts in the entire left pleural area, causing a mediastinal shift to the right.Radiological imaging is an important modality for the diagnosis of pleural hydatidosis. Most often, a chest X-ray and a CT scan can assist in confirming the diagnosis and plan for surgery, which is the primary treatment for hydatid disease in addition to postoperative antiparasitic therapy .Skin tests like Casoni's test, indirect hemagglutination test, and complement fixation tests can be used for diagnostic purposes. We rarely use them routinely because of their false-positive results.When the pulmonary or primary pleural hydatidosis is bilateral, a median sternotomy with resection of cysts is the choice of surgery. Whereas a thoracotomy and resection of cysts on the same side of the disease are preferred in unilateral cases .Staged Thoracotomy was not preferred in our case as it is usually used in bilateral pleural hydatidosis cases. Video-assisted thoracoscopic surgery, a minimally invasive procedure was not used here as it was not feasible to remove such multiple cysts from the thoracic cavity and as the risk of rupture of the cysts was high.In our case, the patient was successfully treated with a left posterolateral thoracotomy followed by irrigation with a hypertonic solution. In addition to resection of all the cysts from the pleural cavity, pleural drainage, empyemectomy, and pleurectomy were done.A table comparing this case with the previously reported cases of primary pleural hydatidosis is added below to highlight the novelty of our case:4Primary pleural hydatidosis which is a very rare disease can have a mere presentation of cough followed by dyspnoea. In our case, the X-ray showed a left-sided pleural effusion, a complication of ruptured pleural hydatid cysts. Hydatid disease was confirmed on a CT scan. A left posterolateral thoracotomy was performed on the patient. A full recovery of the lung's size and functionality offered the prognosis of a very positive outcome. To identify recurrence, it is crucial to do a CXR on follow-up visits. It is also vital to provide anti-parasitic drugs.5Work has been reported in line with CARE criteria.Written informed consent was obtained from the patient for the publication of this case report and accompanying images.Not applicable.Not applicable.Marlyn and Dr.Preethi wrote the Manuscript,Dr.Tigran and Dr.Armen provided and cared for study patients. Foorquan and Dr.Natalie made the final draftMarlyn Susan George.N/A.The authors declare that they have no competing interests.


Langerhans cell histiocytosis (LCH) is a neoplastic transformation of myeloid precursors that commonly presents as an osteolytic lesion of the long or flat bones in children. Aneurysmal bone cysts (ABC) are benign neoplasms that frequently affect the metaphysis of long bones and the spine, often revealing a rapidly expansile lesion with fluid-fluid levels. LCH with secondary ABC-like changes is a rare condition that has only been reported five times, with two presentations in the cranium. The aim of this paper is to review the etiology, clinical and radiographic presentations, and treatment of this condition, as well as to present a novel case on the topic.We describe a 5-year-old boy with a rapidly growing head mass and eye pain resulting in a diagnosis of LCH with secondary ABC-like changes. Radiography demonstrated an expansile, lytic lesion of the left parietal bone with fluid-fluid levels. A confirmatory diagnosis was made through histopathology, demonstrating an inflammatory, histiocytic infiltrate staining positive for CD1a, CD68, CD207 (Langerin), and S-100. The lesion was surgically excised, and the patient recovered without any complications.We present a novel case of LCH with secondary ABC-like changes managed with surgical excision. While a radiographic workup with multiple imaging modalities is helpful for diagnosis, a thorough immunohistochemical analysis is essential as imaging characteristics are variable and nonspecific. Furthermore, surgical excision should be considered first-line treatment for lesions involving the skull in surgically accessible areas as it is curative, alleviates symptoms, and allows for histopathological diagnosis. Langerhans cell histiocytosis (LCH) is a neoplastic transformation of myeloid precursors characterized by the expression of CD1a, CD68, CD207 (Langerin), and S-100 . DespiteAneurysmal bone cysts (ABC) are a benign, clonal neoplasm involving translocations of the USP6 gene, a ubiquitin-specific protease involved in a variety of processes including protein stability and degradation, cell signaling, angiogenesis, and inflammation . ABCs arThe incidence of LCH and a secondary malignancy is approximately 2.6% in children . Past reThe objectives of this study are to present a case report and to review the existing literature on concomitant ABC and LCH. PubMed, Ovid, Embase, and Cochrane Central were queried from date of inception to May 2023.A 5-year-old boy of Hispanic background with no significant past medical history presented to the emergency department with a headache and three-month history of a painful, rapidly growing bump on the left side of the head. There was no history of trauma. Two weeks prior to presentation, the patient started feeling sporadic left eye pain without erythema, discharge, or vision changes. The patient’s parents also reported a recent bump on the right temple that self-resolved within three days. Physical examination revealed an immobile, spongy lesion on the left side of the head just superior to the pterion. The lesion was tender to palpation but there were no overlying skin changes or warmth. He had normal vital signs and a normal appearance other than an obvious skull deformity at the site of the lesion.An initial ultrasound showed a non-compressible fluid collection involving soft tissues of the left cranium. A subsequent cranial X-ray was unremarkable. A computed tomography (CT) scan without contrast revealed an expansile, lytic lesion of the left parietal bone, located just posterior to the coronal suture. Heterogeneous attenuation was observed throughout the lesion Fig. a. On T2-Given the patient’s significant pain and rapidly growing mass, the lesion was excised by craniectomy, and the skull repaired by titanium mesh cranioplasty Fig. d. On micLCH is a rare, neoplastic transformation of myeloid precursors that differentiate into CD1a + and CD207 + cells within lesions . Beyond Radiographic findings for LCH typically show a focal, osteolytic lesion that is sharp and round, aptly described as having a “punched-out” appearance . These lTreatment of LCH involves risk-stratifying patients based on the severity of LCH disease and organ involvement. Unifocal, single-system lesions typically require local therapy, surgery, or observation, while systemic involvement may require more aggressive treatment with medication and radiation therapy , 3. WithABC is a benign neoplasm that commonly presents as a local lesion in children and younger adults . It ofteRadiography is first-line imaging for a patient with suspected ABC. Imaging frequently shows a lytic, expansile lesion with internal septations and a sclerotic border. Fluid-fluid levels are also common but not required for diagnosis . CT and Treatment of ABC has traditionally involved curettage with or without adjunctive therapy. Wide-local excision may also be considered and less invasive treatments, such as sclerotherapy, arterial embolization, and monoclonal antibodies, have also been developed . DespiteThe presence of LCH with secondary ABC-like changes is rare. Only five cases have been reported in the literature with two localized to the cranium. As described in Table In this case report, we describe a 5-year-old with an unusual constellation of findings resulting in a diagnosis of LCH with secondary ABC-like changes. The patient presented with a rapidly growing mass on the head and intermittent eye pain without a history of trauma. Radiographic findings demonstrated an expansile, lytic lesion of the left parietal bone with heterogeneous attenuation and fluid-fluid levels. No dural invasion was noted. A confirmatory diagnosis was made through histopathology, demonstrating an inflammatory, histiocytic infiltrate with cells staining positive for CD1a, CD68, CD207 (Langerin), and S-100. Taken in conjunction with past reports of this condition, our case demonstrates the importance of a thorough radiographic and histological analysis for the accurate diagnosis of LCH with secondary ABC-like changes. Although radiography is the first line imaging modality, including an MRI and CT scan can help improve the sensitivity, specificity, and positive predictive value of this diagnosis . While t


The etiology of complicated pleural effusion can be vast. We present a unique case of an unsuspected metastatic endometrial stromal sarcoma (ESS) in an asymptomatic patient with an incidentally found complicated pleural effusion. A 69-year-old female with no pertinent past medical history was referred to pulmonology for an effusion noted on a routine chest X-ray. Her surgical history was significant for a hysterectomy. At the time of evaluation in the pulmonology clinic, the patient was asymptomatic with stable vital signs. Computed tomography of her chest showed a complex pleural effusion which was drained by cardiothoracic surgery. Fluid analysis results were positive for estrogen and progesterone receptor-positive mesenchymal tumor. Follow-up imaging was negative for any other metastasis. Appropriate management and drainage of this asymptomatic pleural effusion resulted in the diagnosis of a rare malignancy. Given the good clinical prognosis of mesenchymal tumors, the patient was appropriately treated and doing well.We present the case of a patient who was found to have a rare malignancy rather than a benign chronic pleural effusion, as previously suspected. This neoplasm represented a metastatic ESS, especially in this patient’s setting of a hysterectomy. The etiology of complicated pleural effusion can be vast. Malignant pleural effusions are the second most common cause of exudative effusions and are indicative of disseminated malignancy and poor clinical outcomes . As a reThis case was previously presented as an oral presentation during the annual Chest Conference in Nashville, Tennessee on October 19, 2022.A 69-year-old female presented to the pulmonary clinic for evaluation of an abnormal chest X-ray with an incidental pleural disease that was obtained as a health maintenance examination Figure .At the time of presentation, the patient was asymptomatic. Her vitals, physical examination, and laboratory findings were within normal limits. She had a non-contributory past medical history. She had a remote surgical history of hysterectomy for an unknown reason. The patient also denied any family history of endometrial and pulmonary malignancy. She denied any smoking history or exposure to secondhand smoke. A computed tomography (CT) of the chest was obtained and demonstrated a complex left pleural effusion Figure .Point-of-care ultrasound was consistent with an organized effusion, and thoracentesis was performed. Only 30 mL of fluid was obtained with fluid analysis resulting in an exudate with lymphocyte predominate. Because the effusion was well organized, cardiothoracic surgery was consulted for diagnosis and decortication.During surgery, a large complex mass surrounded by mucinous cysts was seen occupying the entire left lower lobe of the lung Figures , 4. The Genetic studies were negative for fusion genes. Cytology of the pleural fluid was also positive for atypical cells. On detailed analysis, she was found to have metastatic ESS. A postoperative positron emission tomography scan showed no definite evidence of either local or metastatic disease Figure . She wasWe presented the case of a 69-year-old patient with a history of hysterectomy who was found to have a rare malignancy rather than a benign chronic pleural effusion, as previously suspected. This metastatic ESS is especially intriguing in the setting of the patient’s history of hysterectomy. ESS are very rare malignant tumors that account for around 0.2% of all uterine malignancies . The annDue to the rare occurrence of metastatic ESS, clinical information regarding diagnosis, different presentations, and management is scarce. This unique case of metastatic ESS in an elderly asymptomatic patient highlights the importance of diagnostic studies for complicated pleural effusion. In case of complex, well-organized effusions or when there is a high clinical suspicion for malignancy, surgical interventions should be pursued in a timely manner as they can provide both diagnostic and therapeutic opportunities to improve clinical outcomes.


Cemento-ossifying fibroma (COF) is a benign odontogenic neoplasm. It is considered an ossifying fibroma with traces of interspersed cementum fragments. Here we present a case report of the occurrence of COF in the maxillary anterior region of an elderly woman. A 61-year-old female reported with a painless, progressive, slow-growing swelling on the upper front jaw region for the past five years. A single, localized, swelling on the anterior region of the maxilla which was non-tender and bony hard in consistency. Radiological examination consisting of orthopantomagram (OPG) and cone-beam computed tomography (CBCT) revealed increased thickness of bone over frontal, parietal and maxilla with alteration of trabecular pattern - cotton wool/ground glass. Serum alkaline phosphatase level was found to be 865 U/l, however, serum calcium level and other routine blood investigations (hemogram) were within normal limits. The above radiological and laboratory findings are more in favour of primary bone pathology and with a biopsy later correlating with histopathological findings; it was diagnosed as COF. Under conscious sedation, surgical excision of the bony mass was done along with extraction of associated teeth. The patient is currently on regular follow-up and planning for a dental prosthesis is in progress. Cemento-ossifying fibroma (COF) is a benign odontogenic neoplasm. It is an ossifying fibroma that shows the presence of cementum in it . The gloThe tumor has a probable origin from the cells of the periodontal ligament and this may account for the cementum tissue formation within the tumor . The cliHere we present a case report of the occurrence of COF in the maxillary anterior region of an elderly woman. Though reports of ossifying fibroma are already published, we could not find any literature correlating with alteration in serum biochemistry which was seen in our case. Consensus-based Clinical Case Reporting (CARE) Guideline was followed . InformePatient information and clinical findingsA 61-year-old female reported with a painless, progressive, slow-growing swelling on the upper front jaw region for the past five years. A brief timeline of the way the swelling progressed is given in Table Diagnostic assessmentRadiological Investigation and FindingsAn intraoral periapical radiograph of 11, 12, 13 showed coronal radiolucency involving enamel, dentin, and pulp in 12 with loss of crown tooth structure in 13. Following this a full skull cone-beam computed tomography (CBCT) of field of view (FOV) 16 X 17 cm was captured. The scan showed an increase in bone mass as evidenced by buccolingual widening of the alveolus in the maxilla Figure . The disHematological investigationsOn blood investigation it was found that the serum alkaline phosphatase level was 865 IU/L  with normal serum calcium level of 9.2g/dl . Apart from this routine hemogram revealed hemoglobin 12.4g/dl , red blood cell (RBC) count 4.73 million/cu.mm  and white blood cell (WBC) count 9765/microlitre . Other blood investigations were within normal limits. All other hematological parameters were within normal range.Excisional biopsyThe patient was referred to oral surgery postgraduate clinic and an excisional biopsy was performed under conscious sedation. Preemptive 2% lignocaine without adrenaline was also administered through infraorbital nerve block and supplementary anterior and middle superior nerve blocks. A crevicular incision was placed over the 13 to 23 region and mucoperiosteal flap was elevated and excision and curettage of the mass were performed followed by thorough debridement. This was followed by extraction of teeth associated with the lesion . Teeth that were infected root stumps  were also extracted along with teeth with poor prognosis . After achieving hemostasis the wound was closed with vicryl resorbable sutures. Postoperative antibiotics (amoxicillin 500 mg three times a day for five days and metronidazole 400 mg three times a day for five days), analgesics (paracetamol + aceclofenac three times a day for three days and two times a day for two days) and serratiopeptidase 5 mg two times a day for five days were prescribed. The patient was advised to be on a soft diet for five days and to do sponging with ice water from day two to day five of the postoperative phase. The biopsy findings were consistent and showed the presence of homogenous eosinophilic areas with scattered osteoblasts and irregular immature bone formation which were consistent with ossifying fibroma (Figure Follow-up and outcomesThe patient was reviewed after one week for the occurrence of any pain and subsequently followed up for a period of two months for the occurrence of swelling. The postoperative pain was subminimal for the first three days and gradually dissipated. Postoperative healing was adequate and the patient exhibited no adverse manifestations till date and the mass has not shown any recurrence as seen in a postoperative scan taken after a period of two months Figure , 7. The One of the most prevalent fibro-osseous lesions in the maxillofacial area is the ossifying fibroma, which often occurs in conjunction with fibrous dysplasia. They have distinct patterns of disease progression, but the various similarities in histomorphological and radiographic features pose difficulties in their classification and management. In the 2017 WHO classification, COF has been defined as a benign mesenchymal odontogenic tumor. This has been reaffirmed in the 2022 classification as well. Further in the 2022 classification it has been completely separated from the nonodontogenic juvenile, trabecular and psammatoid types .In the clinical setting, COF appears as a painless, slowly expanding mass in the mandible rather than the maxilla, often located apical to posterior teeth, and more prevalent in females than males . It is pCOF depends on the degree of mineralisation. It appears radiolucent in the initial stages; calcific flecks increase with maturity of the lesion and, finally, progress to a complete radiopaque mass . A few sHistologically, ossifying fibroma is composed of cellular fibrous connective tissue containing varying amounts of woven bone, lamellar bone, osteoid, round cementoid calcifications and irregularly shaped bone trabeculae. Owing to the presence of this cementum-like material, ossifying fibromas have also been called COF . The patIn this case there was a 20-fold increase in the concentration of alkaline phosphatase. Alkaline phosphatase is a hydrolase enzyme with a role in dephosphorylation that is distributed primarily in the liver and bone which together account for 91%. An increase in alkaline phosphatase in the bone implies that there is an increase in the activity of osteoblasts. An increase in the concentration of this enzyme is noted in bone carcinomas and Paget's disease where there is an increase in bone turnover . In a stTo date there are no studies in the literature which report an elevation of alkaline phosphatase in patients with cemento-ossifying fibroma. Normally an increase in alkaline phosphatase is seen in cases of lesions having an active osteoblastic activity. In case of Paget's disease, which is common in the current age and site of the case report, we anticipated to look for Paget's disease and hence had requested the test. But to our surprise we noted that there was an increase in alkaline phosphatase and histopathology showed ossifying fibroma.The differential diagnosis of COF includes other fibro-osseous lesions, the most common benign fibrous dysplasia. Fibrous dysplasia is more common in the maxilla and it tends to grow longitudinally with ill-defined margins and is self-limiting. COF can be differentiated from Pindborg's tumor, as the latter is mostly associated with an impacted tooth and presents with flecks of calcification .The treatment of COF varies according to the size of the lesion. The most recommended therapy for COF is curettage or enucleation. COF appears to be well demarcated from the surrounding bone and hence allows easy enucleation. Radical resection followed by bone grafting can be considered in cases of recurrence or aggressive nature of the lesion. However, in a study of 64 cases of COF a recurrence rate of 28% was reported in 18 patients treated by surgical curettage and/or enucleation , recurreCemento-ossifying fibroma is a benign odontogenic tumor where some difficulty persisted regarding its pathogenesis. Here we have a case report of an elderly female who had presented with slow-growing swelling for several years causing facial disfigurement and which required a surgical excision. This was among the few cases which had reported an elevation of alkaline phosphatase in ossifiying fibroma. Interestingly both cases had involvement of multiple craniofacial bones. Though there was no recurrence noted in the patient to date an early presentation of the patient would have spared the needless extraction of multiple teeth in them. From this case report we can understand that there are instances of elevation of alkaline phosphatase in ossifying fibroma and it might be prudent to do a study of alkaline phosphatase levels in patients with ossifying fibroma to understand the clinical significance of the parameter.


Anastomotic hemangioma is a rare subtype of capillary hemangioma primarily found in the genitourinary tract. We present a case of a patient with an anastomotic hemangioma located in the retroperitoneal space; then, we explore and summarize the imaging features from previously reported cases for accurate diagnosis.A 57-year-old woman complained of left lower back pain. Contrast-enhanced ultrasound revealed a hypoechoic mass with “slow-in and slow-out” enhancement. Abdominal CT scan displayed a well-defined, round soft tissue mass in the right retroperitoneal region with obvious enhancement. MRI indicated low signal on T1-weighted imaging, high signal on T2-weighted imaging and diffusion-weighted imaging, and progressive enhancement after enhancement. Surgical removal of the tumor was performed. Histopathological examination exhibited a distinct tumor border with interconnected blood vessels and a cavity lined by a single layer of cubic endothelial cells. Immunohistochemistry confirmed the presence of CD31[+] and CD34[+]. The final pathological diagnosis was anastomotic hemangioma. No recurrence was observed during a 40-month follow-up.Retroperitoneal anastomotic hemangioma is a rare and benign neoplasm that may be misdiagnosed as ectopic pheochromocytoma or angiosarcoma. This case report presents and analyzes the imaging characteristics of a series of retroperitoneal anastomotic hemangiomas, which can be valuable for future diagnoses and help prevent unnecessary surgeries. Anastomosing hemangioma (AH) is a rare but benign vascular tumor first documented by Montgomery and Epstein in 2009 . AH primA 57-year-old woman with left lower back pain for 1 day was admitted to our hospital. The patient had a history of hypertension and hepatitis B infection. Physical examination and laboratory tests, including routine blood and tumor markers, yielded normal results. The outpatient doctor considered that the lower back pain was caused by the kidney. A renal CT scan was performed, revealing a 4.1×3.3×4.8 cm soft tissue density mass in the inferior cavity space of the right kidney. The CT values in the plain scan, arterial phase, and venous phase ranged from approximately 13 to 25 HU, 26 to 174 HU, and 29 to 138 HU, respectively. Notably, the arterial phase demonstrated significant enhancement of the mass, which extended to the center during the venous phase  for the publication of any potentially identifiable images or data included in this article.LZ: Conceptualization, Funding acquisition, Writing – original draft, Writing – review & editing. JW: Data curation, Resources, Writing – original draft.


Pseudoaneurysm of the palmar arch is a rare entity. Diagnosis is dependent on high clinical suspicion. We present a case referred to the emergency department, with a history of glass penetrating trauma to the palmar surface with a pulsatile mass and jet bleeding. Doppler ultrasound evidenced a partially thrombosed pseudoaneurysm. A CT angiography examination showed a saccular formation arising from the superficial palmar arch. A conventional surgical approach was indicated. A clinical suspicion must be ventured to arrive at the correct diagnosis. Imaging modalities are needed to identify the pseudoaneurysm and plan the treatment course. Nonetheless, the sequence of diagnosis is individual, because further evaluation with different imaging methods may not change the rationale for the intervention. In our experience, conventional surgical removal is preferable, due to its safety and well-established outcomes. At that time, plain radiographic images showed no evidence of foreign body, bone injury, or any other abnormality. The patient was initially treated with a primary suture in a primary hospital. He developed severe pain, limited movement of the 1st and 2nd ipsilateral fingers, and jet bleeding at the site of previous suturing for two days. On physical examination, a pulsatile hematoma was observed in the left palmar region at the site of the previous suture, approximately 20 mm in diameter, with radial and ulnar pulses present, normal sensation, and capillary refill in his fingers. Allen’s test was performed, showing deep palmar arch patency.A Doppler ultrasound of the left hand was requested . A pseudA conventional surgical approach was indicated. The patient underwent local anesthesia and sedation, followed by a semicircular incision in the palm of the left hand and dissection of the pseudoaneurysm, with thrombus removal  and 3B. Written informed consent was obtained from the patient and the study was approved by the Hospital Medical Care ethics committee, with reference number 4.451.766.6 The SPA is commonly formed by collateral circulation between the ulnar artery (UA) and the superficial branch from the radial artery (RA), while the UA is the main contributor to the SPA7  and the deep palmar arch (DPA).the SPA7 . The SPA8 This entity is most frequently reported after penetrating trauma to the artery due to disruption of the arterial wall resulting in bleeding and formation of an adjacent hematoma, which is later surrounded by fibrous tissue, creating a continuous pocket with the artery. Compared with the native vessel, the pseudoaneurysm is more susceptible to rupture, although rupture is uncommon.2 Regardless of type, pseudoaneurysms are very rare in adults, and extremely rare in the pediatric population.9 They mostly occur as a result of penetrating hand trauma, repeated blunt trauma, infections, connective tissue diseases, vasculitis, and even congenital defects.9Pseudoaneurysms of the superficial palmar arch are rare. There are only a few cases described in the literature.10 Complications can include infection, arterial occlusion, nerve compression, abscess formation, and rarely, bone erosion.11 In this case, jet bleeding was present 10 days after trauma, which is an uncommon presentation.Clinical presentation can include local pain, swelling, and a pulsatile mass in the palm near the site of injury, although hemorrhage is a rare presentation.1 In a post-traumatic scenario, foreign body retention and abscess formation should be taken into consideration, but pulsation of the mass should raise suspicion of the presence of a false aneurysm, especially if it arises progressively.4Due to its rarity, differential diagnosis can be challenging. Lipomas, incision cysts, and fibromas must be excluded to arrive at the correct diagnosis.5 CT angiography can also be used, although magnetic resonance angiography is useful and does not need any contrast medium. These imaging methods are effective for assessment of the vascular anatomy, characteristics, and location of the pseudoaneurysm. However, the diagnostic sequence is individual, because further evaluation with different imaging methods may not change the rationale for intervention. Bouvet at al. proposed an imaging algorithm for suspected hand aneurysm.8 After an aneurysm is suspected, the first method should be Doppler ultrasound, which provides quick access, with reproducible technique, for positive diagnosis of aneurysm or pseudoaneurysm and for differential diagnosis.8 If the suspicion is confirmed, the second step is to assess whether there is acute ischemia or not. In cases presenting with acute ischemia, arteriogram is the next diagnostic step, remaining the gold standard for confirmation of arterial pseudoaneurysm, especially when there are other vascular abnormalities. It is considered a fundamental step prior to surgery and an exhaustive arterial angiogram is recommended, exploring the proximal arteries in order to exclude other sites of atheromatous embolism.12 If there is no evidence of acute ischemia, CT/MR angiography should be performed.Imaging approaches to these lesions can be a matter of debate and there is a lack of studies with large samples. Ultrasonography is typically used as the initial diagnostic imaging modality and angiography is used to evaluate collateral blood flow.In this case, the patient underwent CT angiography because there was no sign of acute ischemia and patency of the deep palmar arch had been confirmed. Questions were raised about the sequence of imaging methods needed to evaluate the lesion, since further evaluation with different imaging modalities may not change the rationale for intervention and may not provide additional important anatomical details that CT angiography had not shown.11 Arteries have been repaired or resected, with or without bypass surgery.2 Reconstruction with end-to-end anastomosis using microsurgery has also been described.1 Restoration of the blood flow is preferable, especially in children.1 Uncomplicated, small, stable pseudoaneurysms can be managed conservatively with compression bandages and close follow-up.13 However, this could be dangerous due to the risk of distal embolization with subsequent occlusion of the run-off arteries. Ultrasound-guided thrombin injection has been found to be an effective, time-saving, and safe procedure for treatment of pseudoaneurysms,15 but it should be avoided as treatment for cases with arteriovenous fistula or when the pseudoaneurysm neck cannot be seen. Our patient was successfully treated with a conventional surgical approach with primary suture, the deep palmar arch was anatomically intact, and the damage to the superficial palmar arch was less than 50% of the vessel, with evidence of good perfusion.Surgical excision and vascular reconstruction are most often performed to ensure distal perfusion and avoid complications.Despite its rarity, pseudoaneurysm of the superficial palmar arch is a serious condition and can lead to severe symptoms and complications in patients after a palmar trauma. For correct diagnosis, suspicion of this condition should be investigated in steps to exclude differential diagnoses, proceeding with the correct imaging modalities. This is important, since there is no strong evidence in the literature on the diagnostic imaging sequence and the decision depends on the singularities of each case. We prefer conventional surgical treatment, due to its safety and well-recognized outcomes. However, other non-invasive treatments have been described in the literature with safety.


The baby wasdelivered via elective caesarean section at 36+3 weeks, and surgical excision of the10x10x5 cm3 mass was performed successfully on the third day of life. Despite asurgical site infection, the patient had a favourable outcome with normal vital signs,bowel, bladder, and lower extremity functions upon discharge. Early diagnosis and promptmanagement of sacrococcygeal teratoma in newborns is vital for optimal outcomes, providingvaluable insights and guidance to medical practitioners.Sacrococcygeal teratomas are common tumours in neonates and infants, primarily affectingfemales. A 35-year-old primigravida presented with a large sacrococcygeal teratoma thatwas detected during the 30 It exhibited multiple cystic areas with internalvascularity which was an incidental finding . At 31 weeks of gestation, fetal magnetic resonance imaging(MRI) revealed a single intrauterine pregnancy with a large, complex cystic lesionexhibiting an exophytic component in the sacrococcygeal region. A small presacral componentwas also present, consistent with a sacrococcygeal teratoma (type I). There was nosignificant family history of congenital birth defects or genetic disorders.A follow-up ultrasound performed at 34 weeks gestation, revealed an increase in the size ofthe mass. After a perinatal consultation at 36+3 weeks of gestation, the baby was deliveredvia elective caesarean section. The birth weight of the baby was 3370 gm, and the APGARscores were normal. Immediately after birth, the baby was transferred to the neonatalintensive care unit (NICU) for observation .After the baby's birth, a neurosurgical consultation was conducted to plan theexcision of the sacrococcygeal mass and cover the defect. A pre-operative pediatricechocardiography revealed a patent foramen ovale with mild pulmonary artery hypertension.Abdominal ultrasonography (USG) showed normal results. A whole neuraxis MRI was performed,which revealed a multiloculated solid cystic mass arising from the lower vertebral endinvolving sacrococcygeal region with predominantly extra fetal portion, the small presacralcomponent containing multiple cysts, and fat component without restricted diffusion .On the third day, excision of the sacrococcygeal mass was performed under generalanaesthesia in a prone position which lasted for approximately four hours. Caudal analgesiawas not administered due to the presence of the mass. Pain management was done withparacetamol and fentanyl. Additionally, neonatal considerations such as fluid management andtemperature were closely monitored. The total blood loss amounted to approximately 50 to 70ml, and 40 ml of blood was transfused intraoperatively to compensate. After the successfulsurgery to remove the sacrococcygeal mass, the excised tissue was carefully collected andsent for a detailed histopathological analysis to evaluate the treatment plan for thepatient .Microscopically, the mass exhibited various tissues and inflammatory infiltrates, but nosigns of malignancy were observed.After the operation, the baby was intubated and transferred to the NICU. However, she wassuccessfully extubated on the same day. She received respiratory support through RAM-CPAP(respiratory assist module-continuous positive airway pressure). Her vital signs were normalduring her NICU stay, and her surgical wound was regularly dressed. She was transferred fromthe NICU to the nursery on the third day after the operation.On the seventh postoperative day, the neonate developed a surgical site wound infection,accompanied by wound gaping. However, there were no neurological deficits, bowel or bladderincontinence in the neonate. Regular dressing was performed, and it was advised to place thebaby in the prone and lateral positions to facilitate healing. Despite following the advice,the wound gap persisted. To address this issue, a secondary closure of the wound wasperformed on the 19th day of life under general anaesthesia .2Sacrococcygeal teratomas, rare extragonadal neoplasms arising in the presacral area, arethe most common germ cell malignancy in newborns and young children. Female preponderancehas been noted in various literatures with the male:female ratio of 3.4:1. Typicallypresented as large cysts or solid masses along the body's midline, these tumoursconsist of tissues derived from two or more primitive germ cells. Although predominantlybenign and cystic, sacrococcygeal teratomas have a minimal 1-2% risk of malignanttransformation in adulthood.3 The use of CT (computed tomography) scansand/or MRI (magnetic resonance imaging) is invaluable for diagnosing these lesions anddetermining their origin, extent, and relationship to the pelvic and abdominal organs.Altman's classification categorises sacrococcygeal teratomas (SCTs) into four types:type I, an external mass with a small presacral component; type II, an external mass with anintrapelvic component; type III, an external mass with both pelvic and abdominal components;and type IV, an internal mass located within the pelvis and abdomen.4 In cases where histopathological examination reveals malignantdifferentiation, the risk of recurrence can be reduced by administering postoperativechemotherapy and radiotherapy. In our case, the histopathological examination shows a benigntumour, hence no chemotherapy and radiotherapy were advised postoperatively.The neonatal period has been considered the ideal period of surgical resection and notexceeding two months of age. A delay in surgery has been shown to be associated with ahigher rate of recurrence or malignancy. Surgical excision is considered curative when themass is removed along with the coccyx, and confirmation of the teratoma's benignnature is provided by the histopathological report. Pathologically, teratomas are classifiedas either mature and well-differentiated or immature and poorly differentiated, with thelatter having a higher tendency for malignant transformation.5 When hydropsdevelops, the mortality rate reaches almost 100 percent. In our case, no associatedanomalies were detected in the abdominal and pelvic ultrasound and echocardiogram. Specificconcerns for these patients include their surgical positioning, blood and fluid loss, andtemperature regulation. Maintaining the patient in a prone position is also necessary toprevent wound dehiscence and facilitate wound care in the postoperative period. Thesepatients experience significant blood loss and hypovolemic shock due to the large pelvicvenous bed, intra-tumour arteriovenous fistula, and associated coagulopathy. To preventexcessive blood loss, ligating the median sacral vessels early is recommended.Providing anaesthesia to neonates undergoing surgery to remove a sacral teratoma is achallenging task. Several associated abnormalities include hydrocephalus, spina bifida,transposition of great vessels, and cleft lip and palate. Fetal death is often caused by thetumour's arteriovenous shunting, leading to cardiac failure and hydrops, which mayrequire premature delivery.6 Surgical procedures carry the risk of complications such as damage tothe pelvic nerve, rectum, and bladder, which can lead to bowel and bladder dysfunctionduring later stages of treatment.7 Therehave been reported cases of venous air embolism and cardiac arrest during the surgicalprocedure.8 A caudal block iscontraindicated in the presence of a sacrococcygeal teratoma or sacral agenesis. Twoinstances are documented where the optimal use of ultrasound helped place a sacralintervertebral catheter in two neonates. Long-term follow-up is necessary not only formonitoring tumour recurrence but also for diagnosing and treating potential secondaryurinary and/or faecal incontinence.Alternative approaches to managing SCT include techniques like radiofrequency thermalablation to disrupt the tumour's blood supply and tumour embolization. However, astudy has reported unsuccessful attempts to reduce blood flow through the SCT usingembolization, balloon occlusion, and sclerosis.9, and in our case, the baby developed a surgical site woundinfection and wound separation, requiring secondary suturing closure at 19 days of life.According to a review, the perioperative death rate was reported as 5.6 percent, with causesof death attributed to haemorrhage, prematurity, birth asphyxia, or tumour rupture. Aftersurgical resection, recurrence rates for both benign and malignant SCTs have been reportedto range from 7.5 to 22 percent.10 Ifthe coccyx is involved, the likelihood of recurrence is higher. Surgical proceduresinvolving the presacral area have been associated with a higher incidence of lower extremityweakness, paralysis, and complications related to bowel and bladder function.7Wound infection is a commonly observed postoperative complicationThe management of sacrococcygeal masses can pose challenges in terms of anaesthesia andsurgical view, particularly due to associated anomalies and potential blood loss. In ourcase, the mass was an incidental finding during regular follow-up. The successful managementof this case highlights the importance of a multidisciplinary team approach.


Fetus in fetu (FIF) is a rare congenital anomaly with two controversial theories regarding its embryogenesis. Although it is an extremely rare condition, it should be kept in mind as a differential diagnosis while evaluating children with abdominal calcification. Radiological findings on postnatal days 2 and 5 of a neonate with an antenatal scan showing an abdominal mass in the fetus are described here. Ultrasonography and magnetic resonance imaging (MRI) revealed the mass in which the contents favored a diagnosis of the FIF. Characteristic features of FIF on MRI have been less explored and knowledge regarding the same will be of immense help to the radiologist. Complete surgical excision followed by histopathology confirmed the diagnosis. Fetus in fetu (FIF) is a congenital anomaly that is extremely rare in occurrence. Exact embryogenesis is unknown and controversial. However, two theories have been proposed. One describes FIF as a variant in the spectrum of monozygotic twinning and the other labels it as a highly differentiated teratoma. It should be differentiated from a teratoma because of the latter’s malignant potential (10%) whereas FIF is a benign condition. Preoperative diagnosis is based on radiologic investigations such as plain radiography, computed tomography (CT), ultrasonography (USG), and magnetic resonance imaging (MRI). Treatment is complete excision with the surrounding sac.A 28-year-old mother at 39 weeks presented to the casualty for the first time. A term male baby weighing 3.12 kg was born by emergency cesarean section. The baby cried immediately after birth and direct breastfeeding was initiated with good suck. A third-trimester antenatal scan showed a complex cystic left suprarenal lesion in the fetus. No family history of twinning was present. On physical examination of the newborn, a 5×5 cm well-defined, non-tender, and firm mass was noted in the left hypochondrium. Complete blood count, kidney function test, serum beta-human chorionic gonadotrophin, serum alpha-fetoprotein, serum carcinoembryonic antigen, and catecholamine levels were within normal limits.On abdominal ultrasound taken on postnatal day 2, there was evidence of a focal, well-circumscribed heterogeneous complex solid cystic lesion measuring 5.5×4.2 cm in the left supra renal region within the retroperitoneal space. Osseous elements and linear echogenic areas with posterior acoustic shadowing were noted within the mass resembling limb bone Figure .MRI of the abdomen was done on postnatal day 5 with a 1.5 T scanner and the protocol used an axial and coronal T2-weighted Half-Fourier acquisition single-shot turbo SE (HASTE) sequence, axial 2-D fast low angle shot (FLASH) plus fat suppression sequence, axial, sagittal and coronal true Fast sequence (TRUFI). MRI done on postnatal day 5 revealed a well-defined lobulated mass with mixed high and low intensities of approximate size 51×38×49 mm in the left retroperitoneum in the suprarenal region which was predominantly cystic. T2 hypointense areas resembling fetal parts such as limbs, head, rib-like bones, and abdomino-thoracic cavity were seen within the lesion. The mass was seen abutting the left kidney and spleen and reaching up to the subphrenic region. There were no features of invasion of other structures as the fat planes between the mass and surrounding structures were well maintained Figures -2d. A diElective laparotomy followed by histopathology confirmed the imaging findings. On laparotomy, a left upper quadrant retroperitoneal mass of size 5×5 cm with varying consistency and covered with a sac with feeding vessels from the abdominal aorta was seen. The mass was completely excised. On opening the sac, a malformed fetus was noted within.A gross examination of the mass showed a fetus with an attached umbilical cord and placenta aggregate weighing 37g. The fetus measured 5×3.5×2cm, the attached umbilical cord measured 3.5×0.5cm, and the attached placenta measured 4×3.5×2cm. The outer surface of the fetus was pale white, glistening, and showed 4 primitive limbs with digits. Cutting through the fetus showed a cavity with multiple nodular tissue, bone, and cartilage. The cut section of the already opened umbilical cord showed one blood vessel measuring 2.5×0.3cm (Figure Microscopic examination of the specimen showed a partially organized collection of fetal tissue like glial-neuronal and choroid tissue, skin with hair follicles and dermal mesenchyme, cartilage, bone, muscle, intestine, liver, adipose tissue, vessels, and ganglion. The umbilical cord showed a single vessel with a covering of Wharton jelly and fibro collagenous tissues. Placental tissue with many congested blood vessels was also noted Figures -4d.FIF is an extremely rare entity wherein a parasitic twin grows in the body of its partner in a monozygotic diamniotic twin pregnancy. It occurs in one in 500,000 live births  and arouIt is commonly located in the retroperitoneum with other rare, reported locations being cerebral ventricles , pelvis There exists an ongoing controversy as to whether FIF is a separate entity or a highly differentiated teratoma. The presence of vertebral bodies and limbs can be used to differentiate FIF from a teratoma . The preImaging plays a very important diagnosis in diagnosing FIF. There are multiple case reports describing the diagnosis of FIF upon identification of vertebral column in CT in literature; however, reports containing ultrasound and MRI findings are sparse ,12,15-17Sonography usually shows a complex amorphous mixed echogenic mass with long bones seen as linear hyperechoic areas with distal acoustic shadowing. MRI has distinct advantages over CT scans such as lack of ionizing radiation, detection of insufficiently calcified vertebral column which may be missed on CT, absence of bony artifacts, and the need to use iodinated contrast media. Parashari et al. described a case of FIF showing a well-formed femur-like long bone and a fetiform solid component containing areas of skeletal elements resembling a vertebral axis within the mass . Lu et aTreatment of FIF is complete excision with all of its surrounding membranes. The possibility of malignant transformation is more in cases with raised CEA levels and the presence of residual tissue of FIF remaining after surgery. Such patients should be on regular follow up. The prognosis of FIF is good since only a single case of malignant recurrence reported as of now.In summary, FIF is a pathologic condition that must be differentiated from a teratoma. Abnormal embryogenesis in a monozygotic dichorionic pregnancy is the more preferred theory of etiology in literature than it being a highly differentiated teratoma. However further studies on the possible association between FIF and teratoma are warranted to establish the true nature of the disease. With advancements in cross-sectional imaging, it is now possible to accurately diagnose FIF preoperatively. As FIF is mostly a pediatric condition, future efforts should be aimed at the usage of modalities like ultrasound and MRI which do not use ionizing radiation to diagnose FIF preoperatively.


Nevus is a group of melanocytes that grow together to form a benign growth on the skin. It is often a black lesion that may protrude from the skin. Becker's nevus is a hyperpigmented lesion that presents congenitally or is acquired. The pigmentation and unusually high hair growth might grow darker with time. Becker's nevus, which can interchangeably be called Becker's melanosis, is a rare disease usually presented by men. The following case is of a 21-year-old male who presented with a hyperpigmented lesion on his right arm. The lesion started at age 16 and increased in size gradually; it involved the flexor surface of the right elbow joint and showed hypertrichosis with irregular margins. On examination, the top dermis contained melanophages, and the basal layer was hyperpigmented. Based on clinical appearance and examination, Becker's nevus was diagnosed. In 1949, Becker published the first description of Becker's nevus (BN) . It is fA 21-year-old healthy medical student from Delhi presented with an asymptomatic hyperpigmented patch on the flexor surface of the elbow of his right arm for the past six years. The patch was small at first and gradually progressed to increase in size before stopping to grow after a year. It is measured 5 x 3cm and is located on the flexor part of the elbow joint with irregular spread but sharp demarcation and even hyperpigmentation throughout (Figure Dermoscopy examination with a polarizing digital dermatoscope Figure  performeThe patient was counseled about the cosmetic hindrance posed by the lesion and was advised Q-switched laser treatment along with a follow-up; however, since the condition is benign and does not pose any threat, the patient decided not to pursue any line of treatment.There are various clinical manifestations of BN. Although an autosomal dominant inheritance mode with varying penetration in the skin has been proposed, the acquired disease is most frequently the cause. Research comprising over 19,000 French male recruits between the ages of 17 and 26 found a prevalence of 0.5%. Showing a 5:1 male-to-female ratio, BN is more prevalent in men. Increased androgen receptor density is proof that there is a disruption in the activity of androgen receptors in BN . In variBecker melanosis is another name for BN. It is a benign lesion that can appear congenitally or acquired with hypertrichosis or hairlessness lesions. It's a rare condition that primarily affects men. It is frequently pigmented, grows darker over time, and is frequently covered in profuse hair growth. BN is a rare clinical illness with a high level of anxiety that is primarily diagnosed clinically. Since the disease has received less investigation and writing as compared to other dermatological disorders such cases should be recorded regardless of the presentation being typical or atypical. An extensive amount of research must be performed on the disease regarding the atypical presentations and treatment modalities to ensure prompt diagnosis. As such, diagnosis is rare and needs to be considered. Patients must be reassured since the disease process is usually benign and the prognosis good.


Background: Paratesticular tumors (PTs) are very uncommon, accounting for almost 5% of intrascrotal tumors. Of these, adenomatoid tumors (ATs) represent about 30% and most frequently arise in the tail of the epididymis. Ultrasound (US) examination is the first-choice imaging method employed for the evaluation of the scrotum. Unfortunately, there are no specific US-imaging features useful for distinguishing an AT from a malignant lesion. To increase diagnostic accuracy and confidence, new sonographic techniques have incorporated real-time tissue elastography (RTE) under the assumption that malignant lesions are “harder” than benign lesions. Case report: In our paper, we describe a very rare case of a 60-year-old patient with a giant paratesticular mass mimicking malignancy when examined using RTE, i.e., it was stiffer than the surrounding tissue (a hard pattern), which, upon histologic examination, was identified as an AT. Discussion: Our case underscores that there is also a significant overlap between different types of scrotal lesions when RTE is used for examination. Thus, if a PT is found, the imaging approach should always be supplemented with more definitive diagnostic methods, such as FNAC or FNAB, which are the only diagnostic methods capable of leading to a certain diagnosis. Conclusions: Alongside underlining the importance of pre-operative imaging for making correct diagnoses and selecting the correct therapy, we wish to draw our readers’ attention to this report in order to demonstrate the clinical implications of a giant AT presenting as stiff lesions when examined using SE. Paratesticular tumors (PTs) are very uncommon, accounting for almost 5% of intrascrotal tumors . GeneralHigh-resolution Ultrasound (US) is the first-choice imaging method for the evaluation of the scrotum due to its excellent spatial resolution, real-time correlation with physical examination, low-cost, and readily available nature and the fact that it only exposes patients to non-ionizing radiation ,3. Once Real-time tissue elastography (RTE), an approach that has only recently introduced for diagnostic use, can improve sensitivity and specificity with respect to differentiating scrotal lesions before surgery by operating under the assumption that malignant lesions are “harder” than benign lesions ,8,9, butHerein, we present a very rare case of a patient with a giant paratesticular mass mimicking malignancy when examined using RTE, which, following the performance of a histopathological examination, was identified as an AT.A 60-year-old male presented to our attention with a mass similar in size to a large orange (approximately 5 cm) localized on the lower side of his left hemiscrotum that had been progressively increasing in size over the last 8–12 months. The patient also reported a history of recurrent left epididymitis five years prior to his presentation to our department.During a physical examination, it was observed that the left scrotum was significantly enlarged, and a large, non-reducible, and painless mass was palpated. The mass was well-marginated, smooth, and firm in consistency. No sign or symptoms suggestive of epididymo-orchitis were observed. There was no history of trauma or inguino-scrotal surgery, including vasectomy. Laboratory blood tests revealed no abnormalities. The results obtained from a seminogram and spermioculture were normal. Scrotal US examination was performed using a Logic E9 unit  with linear probes (ML 6–15-D—4.5–15 Mhz and 9L-D 3.0–8 Mhz). B-Mode US revealed a rounded, hyperechoic, and coarsely heterogeneous solid mass within the lower part of the left hemiscrotum measuring 4.8 × 4.1 cm in size. The left testis was lifted by the mass, from which it was poorly dissociable at the lower pole; however, it was preserved in size and echostructure . The conCD was employed to determine the vascularization of the mass. To maximize sensitivity to slow flow velocities, CD was performed using the highest signal gain setting possible (without the appearance of background noise) and low pulse repetition frequencies, allowing for the demonstration of peripheral lesional vascularity . A gradeIn addition, real-time strain elastography (SE) was carried out as an additional diagnostic tool for evaluating tissue elasticities when conducting US. Specifically, SE was performed by employing repeated compressions and decompressions with the transducer using a freehand technique. The pressure applied was adjusted according to the visual indicator for compression presented on a video screen. Tissue elasticity was calculated in real-time, and stiffness of tissues was displayed as a color-coded overlay on the B-mode image. During SE imaging, it was observed that the lesion was stiffer than the surrounding tissue (constituting a hard pattern) and was larger in size on the elastogram than on the B-mode US image, showing an E/B ratio > 1. Then, SE images of the lesions were assigned an elastographic five-point color score according to the distribution and degree of strain suggested by Itoh et al. for breast disease ,8,9. As Due to the tumor’s size, Doppler vascularity, and RTE features, the treating physicians suggested conducting an in-depth diagnosis. However, although either contrast-enhanced ultrasound (CEUS) or fine-needle aspiration cytology (FNAC) or biopsy (FNAB) was recommended as the first step, the patient preferred to directly proceed to radical surgical excision because he had no fertility requirements. Therefore, a left orchiectomy was performed.The surgical specimens obtained included the left testis, measuring 4.9 × 2.5 cm, and a large, greyish-to-white soft tissue mass measuring 4.9 × 3.6 cm. All the specimens were sent for histopathological analysis, which demonstrated a tumor composed of cells that were arranged in cords and acini. Immunohistochemical tests showed the presence of WT1 gene expression and positivity for markers including calretinin, cytokeratin AE1/AE3, and EMA, thus revealing a mesothelial origin of the tumor akin to that of an AT. The final pathology report confirmed a completely removed AT of the left scrotum arising from the tail of the epididymis. The immediate postoperative course was uneventful, and the patient was discharged on postoperative day one.Paratesticular structures consist of the spermatic cord, the epididymis, the tunica vaginalis, and vestigial remnants . Each ofAn AT is rare benign tumor of the male genital tract. Godman and Ash first coined this term in 1945. Pathologically, it is defined as a hamartomatous tumor of mesothelial origin. It is a benign tumor, even if cellular atypia and local invasion have been occasionally observed . AT is tGenerally, ATs are diagnosed in men between 20 and 50 years old, although they may affect patients of any age. Associated trauma has been described in a few cases . ClinicaRadical orchiectomy is an effective oncological procedure for treating scrotal lesions, but it carries risks of psychological, cosmetic, hormonal, and fertility-related complications. Testis-sparing surgery might be a viable alternative in cases where the likelihood of malignancy is low. However, ATs are difficult to diagnose preoperatively; thus, many patients are subjected to unnecessary orchiectomy that could be avoided if the diagnosis of a tumor was timelier and more accurate (this is particularly relevant for young patients) . TherefoRecently, novel US techniques such as RTE have been suggested to be used as additional multiparametric imaging tools for the characterization of scrotal lesions ,8,9. To Our patient was investigated using SE, which showed that the entire PT lesion was blue, thus indicating a stiff lesion. When classified according to the Itoh-modified score, the lesion was stiffer than the surrounding tissues and considered to be larger when compared with conventional US (E/B ratio > 1); thus, it was categorized with a score of 5, which is suggestive of malignancy. In contrast, the final pathology report revealed an AT of the scrotum.Despite the extensiveness of our extensive literature review, only a single case of AT assessed using RTE was found. Even in this previous paper by Pitcher et al. , false-pThese two cases underscore that a stiff paratesticular lesion determined as such via SE (E/B ratio > 1) should not be the sole multiparametric US finding relied upon for diagnosis, especially when other B-Mode US or Doppler features are inconclusive or erroneously suggest malignancy and it is not possible to perform contrast-enhanced ultrasound with a contrast medium in a short time. In our opinion, if a PT is found, the imaging approach should always be supplemented with more definitive diagnostic methods such as FNAC or FNAB, with the two cited approaches being the only diagnostic methods capable of leading to a certain diagnosis . Our artAlthough US is the mainstay of scrotal imaging for the evaluation of both intra- and paratesticular lesions, a correct diagnosis may be challenging due to the absence of specific features. To increase the diagnostic accuracy of US with respect to differentiating scrotal lesions, RTE, operating under the assumption that malignant lesions are “harder” than benign lesions, has been employed. However, a significant overlap between different types of lesions also exists when using RTE for examination, as demonstrated in our case, and a certain diagnosis is often only possible only using cyto-histopathology.


Intradural extramedullary (IDEM) tumors are the most commonly observed intraspinal tumors, comprising over 60% of tumors found within the spinal canal, and the vast majority of these lesions are benign lesions. IDEM metastases are rare, but if they occur, they commonly manifest as leptomeningeal disease, secondary to drop lesions from intracranial metastases from adenocarcinomas of the lung, prostate cancer, breast cancer, melanoma, or rarely, as a result of lymphomas. The purely non-neurogenic origin of IDEM metastases is rare. Herein, we describe a patient with a previous history of treated colon cancer who presented with a progressive neurological deficit and whose imaging revealed multiple intradural, extramedullary and osseous lesions at the cervical and thoracolumbar spines. With the previous known primary and multiplicity of the lesions, an initial diagnosis of spinal metastasis was made, But it was proven to be schwannoma on histology. We emphasize the diagnostic dilemma in this case and the importance of detecting subtle imaging findings, which may be helpful to differentiate between metastatic disease and a second primary tumor. The patient was a 72-year-old gentleman with underlying dyslipidemia. He had a history of ascending colon adenocarcinoma, diagnosed six years ago. He underwent a right hemicolectomy, completed chemoradiotherapy in the same year, and has been in disease remission since then. He presented to us with a two-week history of progressive bilateral upper and lower limb weakness, predominantly affecting the upper limbs more on the right side. Later, he developed difficulty walking and a deterioration of his gait due to weakness. However, he was still able to move all four limbs and did not complain of any sphincter dysfunction or constitutional symptoms.Examination showed no evidence of neurocutaneous markers. Neurological evaluation revealed reduced motor function, mainly affecting the upper limbs. Muscle power at both upper limbs was graded 4/5 in all groups, except left elbow flexor, right wrist extensor and elbow extensor, which was a full 5/5. The lower limb muscle power was full except for bilateral hip flexion and right knee extension, where it was grade 4/5. Tendon reflexes were normal in both the upper and lower limbs. Sensory examination was normal, except for decreased pain sensation to pin-prick in both the upper and lower limb dermatomes.Routine investigations that included hematological and biochemical investigations were normal. The chest radiograph was normal, and the cervical radiograph showed spondylotic changes. Ultrasonography of the abdomen and bilateral lower limbs showed no mass or lower limb deep vein thrombosis.Whole-spine magnetic resonance imaging (MRI) with gadolinium was executed, and apart from degenerative changes of the spine, the MRI revealed several intradural extramedullary lesions at the cervical and thoracic spine, namely at C4–C5, T11, T12, and T12/L1 levels . These lOwing to the presence of cervical cord compression, which was causing progressive clinical symptoms, he was subjected to a C4–C5 laminectomy accomplished via a posterior approach. A dural opening revealed a firm tumor anterior to the dentate ligament, arising from the right C4 nerve rootlet. The tumor was excised in total, and the involved nerve rootlet was sacrificed. Intraoperative diagnoses of neurofibroma or schwannoma were then made.The postoperative condition was uneventful, with improvement of the upper limb power and intact upper and lower limb sensation. He was able to walk upon discharge. Contrast-enhanced computed tomography (CT) of the thorax, abdomen, and pelvis were carried out, showing degenerative changes at the cervical and lumbar spines as well as a well-defined lytic lesion with the corduroy sign at the T9 vertebral body suggestive of intraosseous hemangioma, consistent with the MR characteristics that are typical of hemangiomas. The other vertebral lesions seen within T2 and T10 were not appreciated on CT. There is no evidence to suggest recurrence of the colon carcinoma or distant metastasis.Microscopic examination of the specimen showed neoplastic cells with a biphasic pattern, composed of mainly compact hypercellular Antoni A areas and occasional myxoid hypocellular Antoni B areas. The cells were spindle-shaped, hyperchromatic nuclei, some with cytoplasmic nuclear inclusions, and inconspicuous nucleoli with ill-defined eosinophilic cytoplasm. Nuclear palisading (Verocay bodies) was easily seen within the hypercellular areas. There were also collections of hemosiderin-laden macrophages and hyalinized blood vessels seen. No necrosis or mitosis. There is no evidence of malignancy. Final histopathological features were consistent with schwannoma.18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) was performed upon follow-up to exclude metastasis, which revealed reduced FDG metabolism of the T9 vertebral body lesion corresponding to a hemangioma , and magnetic resonance imaging (MRI) as well as nuclear medicine imaging ]. Despite extensive imaging, diagnostic dilemma occasionally persists regarding the true nature of some bony lesions .Intradural extramedullary (IDEM) tumors are the most commonly observed intraspinal tumors, comprising over 60% of tumors found within the spinal canal . IDEM coThe commong differentials for IDEM consists of one of three subtypes: meningiomas, schwannomas, or neurofibromas . MeningiIDEM metastases of non-neurogenic origin are rare, representing less than 4% of all spinal metastases ,12. To oWhile IDEM metastases can occur at any level, the majority appear to occur in the lumbar spine. Lower CSF flow velocities in the lumbar spine may predispose this region to a higher probability of tumor cell settling ,17. ThisThe other diagnostic dilemma in this case was the presence of multiple osseous lesions. Although one of the osseous lesions showed typical hemangioma features, the rest of these lesions were indeterminate. The stereotypical hemangioma usually causes no problems because, on MRI, it appears hyperintense on T1WI and T2WI and enhances intensely on post contrast T1WI fat-suppressed images. These lesions are also usually clearly demarcated. However, some hemangiomas do not have these stereotypical characteristics. Atypical hemangiomas, which may vary in appearance, include those that are hypointense on T1WI but retain the typical characteristics on T2-weighted and fat-suppressed postcontrast images. Thus, these atypical features of hemangiomas cause difficulty in distinguishing benign lesions from metastases .18F-FDG PET/CT, which was performed upon follow-up, revealed reduced FDG metabolism of the T9 vertebral body lesion corresponding to a hemangioma. The rest of the spinal lesion revealed no hypermetabolic uptake to suggest metastasis. Thus, these small lesions at the T2 and T10 vertebral bodies may also represent atypical hemangiomas. The rest of the IDEM lesions also showed no hypermetabolic uptake to suggest metastasis. These characteristics, together with histopathological findings, led to the final diagnosis of multiple schwannoma.It is possible that newer imaging modalities, such as positron emission tomography, may also prove helpful in determining the nature of spinal cord tumors . SimilarThis case underscores the need to consider the diagnosis of sporadic benign IDEM lesions when evaluating patients with a known primary with multiple imaging abnormalities. IDEM metastasis is rare, even in patients with a known previous primary. The multiplicity of the lesions with multiple underlying bone lesions had imposed a diagnostic challenge in this case. Thus, a thorough and careful review of radiographic features with the detection of subtle imaging findings may be helpful to differentiate metastatic disease from a second primary tumor.