Schwannomas are solitary well-circumscribed encapsulated benign tumors that exhibit Schwann cell differentiation, and arise directly from myelinated peripheral or central nerves. Although they are usually asymptomatic and found incidentally, schwannomas can cause symptoms due to compression of nearby structures which, depending on the location, can make clinical presentations widely variable. Despite their rarity, schwannomas have been documented in a number of locations including the limbs, cerebellopontine angle, posterior mediastinum, and, far more infrequently, the lungs.18F]fluorodeoxyglucose positron emission tomography/computed tomography scan revealed a lobulated soft-tissue lesion measuring 23 mm × 23 mm in the lower lobe of the right lung. A computed tomography-guided core biopsy of the mass was performed, which revealed a benign spindle cell lesion based on histopathological examination and immunohistochemical staining. The mass was surgically resected via a right lower lobectomy, and subsequently confirmed to be an encapsulated neoplastic lesion composed of well-differentiated Schwann cells. There were no short- or long-term complications, morbidities, or recurrences based on 1-year follow-up.In this article, we report an incidental finding of an intrapulmonary schwannoma in a 59-year-old Pakistani woman who was grossly asymptomatic upon presentation to the cardiothoracic surgery clinic. An [This report underscores the predominantly asymptomatic nature of schwannomas and reemphasizes the efficacy of surgical resection as a safe and curative procedure for a tumor of this nature. Albeit very rare, intrapulmonary schwannomas can be considered a differential diagnosis when encountering solitary asymptomatic pulmonary nodules or masses.The online version contains supplementary material available at 10.1186/s13256-023-04234-z. Schwannomas, also known as neurilemmomas or neurinomas of Verocay, frequently appear as solitary well-circumscribed encapsulated benign tumors that exhibit Schwann cell differentiation and arise directly from myelinated peripheral or central nerves . The SchNF2 gene on chromosome 22, merlin, which is responsible for the regulation of several cell-signalling pathways by interacting with the actin cytoskeleton. As such, loss of merlin results in dysregulation of cell shape, cell growth, and cell-to-cell adhesion thereby leading to tumor formation fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) scan revealed a lobulated soft-tissue lesion measuring 23 mm × 23 mm  along the medial aspect of the anterior segment of the lower lobe of the right lung  of 2.7. No evidence of hypermetabolic nodal, pulmonary, hepatic, splenic, adrenal, or bony metastasis was observed.Radiological imaging (CXR) showed a nodular density in the right lower zone of the right lung measuring approximately 2.5 cm × 2 cm. A subsequently conducted high definition 8FfluorodA CT-guided core biopsy of the lesion was performed. Histopathological evaluation revealed a core of a spindle cell lesion with smaller cores of native unremarkable lung parenchyma. Significant cytological atypia or increased mitotic activity was not seen. Immunohistochemical staining revealed the cells to be S100 focal positive, SOX-10 negative, ASMA negative, Melan A negative, HMB-45 negative, STAT6 negative, and CD34 patchy positive. A diagnosis of a benign spindle cell lesion, likely of neural origin, was made. An elective surgical resection was planned for a month later.After standard preoperative optimization and preparation, a posterolateral thoracotomy with right lower lobectomy was performed under general anesthesia. The pleural cavity was entered through the fifth intercostal space. Enlarged station 9R and 10R lymph nodes and a mass in the lower lobe of the right lung were visualized, while no masses were observed in the middle or upper lobes. There were no pleural nodules or pleural effusion. The inferior pulmonary ligament was divided, and the inferior pulmonary vein was identified and divided between ties. The oblique fissure was incomplete, and was used to separate the upper, middle, and lower lobes of the lung through cautery. The pulmonary artery was identified at the confluence of the oblique and horizontal fissures, and the superior segmental and basilar branches were visualized. The superior segmental branch and the pulmonary artery distal to the take-off of the middle lobe branch were both divided between ties. The bronchus intermedius was dissected and the middle lobe bronchus was identified. The bronchus distal to the middle lobe take-off was dissected, and a TA-30 stapler was used to divide the superior segmental bronchus and the bronchus distal to the middle lobe take-off. The mass was then resected, and hemostasis was secured. The surgical wound was closed in layers after the placement of an extrapleural catheter and chest tubes, and the patient was shifted to the in-patient facility. The patient remained vitally and hemodynamically stable and was discharged 3 days after the operation. The patient was confirmed to be in her usual state of health upon 6-month and 1-year follow-up, with no late complications or recurrences.Histopathological examination of the resected lobe showed lung parenchyma infiltrated by an encapsulated neoplastic lesion composed of well-differentiated Schwann cells. The neoplastic cells showed a biphasic pattern with compact areas of spindle cells having moderate eosinophilic cytoplasm and normal chromatic elongated nuclei. Areas of occasional palisading (Verocay bodies) were also seen Fig. . These mSchwannomas present as well-circumscribed encapsulated masses and are solitary in up to 90% of cases . AlthougWhile the most common locations for a schwannoma are in the upper limb and the cranial vault  , the incmax of 2.7, which is concurrent with existing literature that suggests a value between 1.3 and 6 for benign neurogenic tumors [max value due to microvascular and cellular density, and a significant proportion of schwannomas being highly metabolic variants with an SUVmax of > 6 [Radiological examinations usually reveal a round, ovoid, or lobulated mass with soft-tissue density and well-demarcated borders , as was Histopathological examination of the biopsied schwannoma will reveal compact eosinophilic spindle cell areas (Antoni A areas) and hypocellular areas with an abundant extracellular matrix (Antoni B areas) , as in oGiven these tumors are loosely attached to their associated peripheral or central nerves , they caIn conclusion, this report highlights the rare case of an intrapulmonary schwannoma and underscores the predominantly asymptomatic nature of such tumors, which are often discovered incidentally during radiographic examinations performed for unrelated reasons. The successful surgical excision performed in this case demonstrated its efficacy as a safe and curative procedure, with no observed short-term or late-term complications or morbidities. These findings align with existing literature and emphasize that, albeit very rare, intrapulmonary schwannomas can be considered a differential diagnosis when encountering solitary asymptomatic pulmonary nodules or masses.Additional file 1. Completed SCARE Checklist for the Present Article.


Editorial on the Research TopicCase reports in pediatric orthopedics 2022Paediatric orthopaedics is a very fascinating branch of orthopaedics as a child's skeleton has great remodelling capabilities thanks to growth cartilage, but often this can be the cause of growth defects when damaged. Sometimes, the pathologies have a clinical manifestation that involves different systems, and often in these cases, they are rare pathologies with difficult diagnosis. The diagnosis in children is made more difficult by the complicated communication with the patients and treatment of the same pathology varies according to the patient's age. These situations are already present for well-known pathologies and become even more serious in the case of rare pathologies or for atypical manifestations where lack of diagnosis and treatment can lead to poor prognosis. The issues covered by these studies are different, all united by the fact of being unusual and rare. This topic aims to share a series of rare case reports to form an understanding of the possible expression of various conditions and their treatments for improving clinical practice, diagnostics, and therapeutics.Basiglini et al. showed that partial epiphysiodesis inserting the bone wax is possible in up to 30% of the cartilage area without osteotomy, because the cartilage around the lesion can react by hyperactivating and allow the recovery of the load axis. Ankle fractures account for about 5% of all paediatric fractures. While rare, loss of the medial malleolus is more common in children. Furthermore, it leads to a higher risk of complications such as medial physeal bar with varus angulation and leg length discrepancies (LLD) due to premature physis arrest. Medial malleolus contributes to maintaining normal tibiotalar joint characteristics, and fractures can significantly decrease contact area, increase contact pressure, and lead to ankle instability  in the cervical spine in a paediatric patient, reporting a treatment option that showed excellent results at 3 years of follow-up; in this case report, the age, site of the lesion, and histological variant were unique.Primary osseous spinal tumours are rare in children and young adults, accounting for only 1% of all spine and spinal cord tumours combined , but theFlorio et al. reported a successful reconstruction of the metatarsal bone with fibular graft for a case of giant cell tumour (GCT) marked by an unusual location and age group. GCT develops almost exclusively in the epiphysis of long bones, and the most common location is around the knee region; only four cases of GCT on metatarsal bone have been reported in the literature . This is the first case of non-traumatic massive heterotopic iliopsoas ossification described in the literature.Heterotopic ossification (HO) is a rare condition that is generally secondary to other conditions, particularly trauma (often repetitive microtrauma) or hereditary form; it is essential to make a differential diagnosis with oncological pathologies to better manage these conditions . This paQiu et al. reported a case report of KHE at a late age originating from the bone and limited inside the bone with bilateral involvement. It is important to note that when involved, only the bone without periosteal reaction KMP is not associated, and this can lead to a lack of diagnosis. This is the only case involving bilateral femur symmetry reported in the literature. In this case, sirolimus without prednisolone was effective. Given the rarity of the pathology, it may happen that despite the presence of KMP, there may be a delay in the diagnosis. In this topic, a case report of KHE with KMP in a 2-month patient has been reported; he showed symptoms from the first week of life and was treated first for urticaria, then cellulitis, and then necrotic fasciitis until the right diagnosis was made, at two months of age. He was treated with sirolimus (off-therapy because it is accepted from 2 years onwards) with excellent results 3 years after treatment.Kaposi haemangioendothelioma (KHE) is a rare vascular neoplasm that presents usually within the first year of life. There is often a delay in diagnosis, and this may lead to morbidity and mortality of up to 30%, mostly due to a life-threatening consumptive coagulopathy named the Kasabach–Merritt phenomenon (KMP), which occurs in 40%–70% of cases, although this is a complication. However, when present, it helps in the diagnosis . The authors reported a higher incidence of Crawford IV in boys and in the middle or distal part of the tibia; most patients were less than 3 years old, and the major surgical complications were ankle valgus and limb length discrepancy.Congenital bowing of the tibia is a rare condition (1/140.000–190.000) and is considered the precursor of congenital pseudarthrosis of the tibia; little is known about its clinical features. While the diagnosis is simple, the treatment, which varies from conservative to surgical, is not. Surgical treatment could consist of different methods, none of which have shown superiority over the other, with a 50% amputation incidence. Dirani et al. reported the first clinical description of a patient with a splice variant in intron 34 in the COL1A1.Osteogenesis imperfecta is an autosomal dominant congenital pathology characterised by bone fragility and associated with other clinical signs. Many gene variants determine a different phenotypic expression. In their study, Caredda et al. showed how conservative treatment in the case of lumbar hemivertebra is a good choice; it can improve the Cobb's value of the curve and maintain the long-term correction. Conservative treatment in selected patients can also change the natural history of congenital scoliosis due to the failure of formation, which permits the hypertrophy of adjacent vertebrae, thus preventing its deformation. However, the treatment should be implemented as early as possible without waiting for the evolution of the curve, in contrary to what occurs in lower idiopathic scoliosis.Scoliosis is the most common congenital pathology, which can be caused by vertebra segmentation or formation defects. Bandinelli et al. reported the only case known in the literature of SLD in a paediatric patient that was not secondary to trauma or other conditions. The unique symptom was a wrist extension deficit. The only alteration found in the patient was generalised hyperlaxity. Thanks to the correct diagnosis and treatment, the patient showed a complete functional recovery after 12 months. It is important to know that a trauma-free SLD can exist, probably due to hyperlaxity, which allows to treat it adequately while avoiding long-term complications.Scapholunate dissociation (SLD) is a very rare condition, especially in children. When it occurs, in most cases it is post-traumatic or secondary to other conditions. If undiagnosed, it can lead to chronic pain, SL advanced collapse, and early osteoarthritis . In this


Maxillary sinus mucocele is a rare form of mucocele and are usually under diagnosed due to its vague symptomatic presentation. It is caused by obstruction of the natural ostium and accumulation of secretions inside the sinus cavities. It is a locally expansile lesion and symptoms are due to pressure on surrounding structures.A 45 -year- old female patient presented with swelling on the left side of the face for 6 months with left infra orbital pain. On examination the swelling was diffuse in the left side of cheek. CT scan showed a homogenous opacity completely filling the maxillary sinus with expansion of the walls which helped in diagnosing the condition. Endoscopic marsupialisation was done and the patient is under follow-up for more than a year with no recurrence.Maxillary sinus mucocele is an epithelium lined sac filled with mucous secretions. They are expansile and can cause bony erosion of surrounding anatomical structures. It is mainly differentiated radiologically by the presence of air in the sinus cavity. CT scan shows homogenous opacity completely filling the antrum with no air shadow. The walls may be thickened or thinned out. Endoscopic marsupialisation of the mucocele gives excellent results with minimal recurrence. Maxillary mucocele being a rare benign cystic lesion is mostly under diagnosed. Hence, proper clinical examination and radiological evaluation help in early diagnosis. Appropriate surgical management gives a good success rate with nil recurrence. Para nasal sinuses are empty spaces within the skull and are lined by ciliated columnar epithelium with numerous seromucinous glands. The secretions within the sinuses drain into the nose through the natural ostium. Mucocele are mucous containing cystic lesion which are formed due to blockage of the natural ostium with accumulation of secretions within the corresponding sinus cavities. When the secretions keep on accumulating over a period of time, it gradually enlarges which ultimately leads to erosion and the surrounding bone gets remodelled. They typically arise from chronic inflammation, scarring or surgical manipulation resulting in obstruction of the sinus ostia, although a compartment of a septated sinus can similarly become obstructed . MucocelThough mucocele being a benign lesion, its expansion into the surrounding anatomical structures can cause severe and permanent complications and damage if they left undiagnosed and untreated. Thus prompt diagnosis and suitable treatment methods are very important. Thus the current case study involves in reporting the unusual maxillary mucocele found in a 45-year-old female. The diagnosis treatment method followed for that particular patient is mentioned in detail for the future use.A 45-year-old female was presented with facial swelling on the left side of face for 6 months with pain in the left infra orbital margin for the past 2 months. Swelling was progressively increasing in size without pain initially. Patient started getting pain later which gradually worsened. Patient had no history of recurrent upper respiratory tract infection, fever and headache. There was no history of blurring of vision or diplopia. Patient has been on treatment with a GP for 2 months for swelling and pain but had no improvement. On clinical examination, the swelling was diffuse in the left side of cheek with normal skin. There was thinning of the infra orbital margin with tenderness in the medial aspect of the infra orbital rim. Diagnostic nasal endoscopy revealed a smooth bulge in the inferior and middle meatus. There was no evidence of infection or discharge in the middle meatus and the mucosa was normal. CT scan showed a homogenous opacity completely filling the maxillary sinus with expansion of the walls which is shown in The infra orbital margin was thinned out and elevated extending into the orbit. All other sinuses were normal. Visual acquity and field of vision were normal. Endoscopic marsupialisation of the mucocele was planned. Using nasal endoscope, a wide bore needle was inserted through the smooth bulge in the inferior meatus and 10 ml of straw-coloured fluid aspirated. The apiration of the contents of the mucocele is shown in Maxillary sinus mucocele is an epithelium lined sac filled with mucous secretions. It is very common in ethmoid and frontal sinuses and rare in sphenoid and maxillary sinus. It is mainly due to blockage of the natural ostium. Obstruction of the sinus ostium has been suggested as the primary etiologic factor. This may be due to a mass lesion, inflammation and fibrosis, osteoma, fibrous dysplasia, Paget's disease, malignancy, trauma, or previous surgery . In someMaxillary mucocele is a benign cystic lesion occurring due to blockage of natural ostium. It causes expansion of sinus walls with extension into the surrounding anatomical structures. Since it is a rare entity, it is usually missed out during diagnosis leading to unnecessary investigations and procedures. Clinical examination and radiological evaluation help in early diagnosis. Appropriate surgical management gives a good success rate with nil recurrence.


Esophageal duplication cysts are rare congenital tumors usually diagnosed and treated during childhood. Most of them are located in the mediastinum and appear as a mass besides the esophagus. Unfortunately, symptoms are non-specific and depend on the size and location of the mass; therefore, they can easily be missed. If symptoms appear, surgical resection is necessary to prevent troublesome complications.We present the case of a 60-year-old woman who presented with severe progressive dysphagia and epigastric pain. After further evaluation, a paraesophageal cystic mass was found, and surgery was required. Non-communicating esophageal duplication cyst was the final diagnosis.Esophageal duplication cysts are a rare pathology in adults; their symptoms will vary depending on their size and location. Preoperative diagnosis is difficult as symptoms are non-specific and can be missed. If severe dysphagia, pain, or any other complication appears, surgery should not be delayed. Esophageal duplication cyst is an unusual congenital disorder of the foregut; they are usually diagnosed during infancy and account for up to 10–15% of duplications of all foregut cysts . These cWe present the case of a 60-year-old patient who presented severe dysphagia, weight loss, and epigastric pain due to a large esophageal duplication cyst. After surgery, she underwent complete recovery.Patient is a 60-year-old woman without past medical history. She began to experience difficulty swallowing. The dysphagia was mild initially and did not affect her life, but as time passed, it worsened, first with solids and then with liquids. Due to this, she went to several physicians. Nonetheless, all endoscopies done at that time were normal; she grew tired of the doctors, and little by little, she changed her diet, she only ate liquids, and at one point, she couldn’t even swallow her own saliva.As a result, she suffered weight loss and was brought to a community hospital by her family.On clinical evaluation, an underweight patient (BMI 18.5) was encountered; she had mild epigastric pain; however, no tenderness was discovered; also, no masses or lymph nodes were found at that time.Esophageal malignancy and pseudoachalasia were included in the differential, and complementary exams were needed.A new upper endoscopy was done; nonetheless, it didn’t reveal any pathology; therefore, a contrast-enhanced computed tomography was requested, revealing a 7,9 × 9,5 × 7,1 cm subcarinal mediastinal mass with multiple heterogeneous densities; the mass had round and regular edges, shifting the esophagus and trachea, and heart towards the left. As a result, a cardiothoracic assessment was needed, and the patient was transferred to our hospital.  and account for approximately 10% of all mediastinal tumors in children :1 and ac. EsophagEsophageal duplication cysts are usually diagnosed up to 80%) during infancy, and only a few patients (less than 150 in the English literature) remain asymptomatic until adulthood , 4. A re0% duringPain, dysphagia, and reflux are the most common in adults, while airway obstruction and repeated pulmonary infection are more common in children , 4. AdulEsophageal duplications cysts share a common muscular wall with the native esophagus and are attached to it , 10. TwoEsophageal duplications cysts tend to appear in the right posterior-inferior mediastinum or lower-middle esophagus , 2. As iPreoperative diagnosis needs several imaging methods like CT or MRI , 7. NoneObservation may be possible in asymptomatic patients, especially in patients at high surgical risk; nonetheless, complete resection is mandatory if symptoms are present , 2, 13. Esophageal duplication cysts are still rare in adults and are frequently located in the distal esophagus; since larger cysts are more likely to cause symptoms, we should always keep this pathology among the differential diagnoses to achieve an accurate diagnosis and avoid cases such as our patient’s. Interestingly, this case also proves that the doctor–patient relationship is essential; if we can connect with our patients and investigate more about their pain instead of just reviewing exams, we can improve our practice and get to know the patients better.This report shows a case of an esophageal duplication cyst found in adulthood; the dysphagia and abdominal pain were progressive and eventually impaired the patient’s life. Although there are cases in which esophageal duplication cysts may go unnoticed, dysphagia is a symptom that should always be investigated to prevent events such as this. Esophageal duplication cysts are rare; however, this case proves that they can grow and produce serious complications, ones that should never go unnoticed.


Pleuropulmonary blastoma (PPB) is a tumor occurring almost exclusively in infants and young children. This is the most common primary-lung malignancy in childhood. There is age-associated progression through a distinctive sequence of pathologic changes, from a purely multicystic lesion type I to a high-grade sarcoma type II and III. While complete resection is the cornerstone treatment for type I PPB, aggressive chemotherapy with a less favorable prognosis is associated with type II and III. DICER1 germline mutation is positive in 70% of children with PPB. Diagnosis is challenging, as it resembles congenital pulmonary airway malformation (CPAM) in imaging. Although PPB is an extremely rare malignancy, over the past five years we have encountered several children diagnosed with PPB in our medical center. Herein, we present some of these children and discuss diagnostic, ethical, and therapeutic challenges. Pleuropulmonary blastoma (PPB) the most common primary lung malignancy in childhood . First rPPB is unique among developmental malignancies of childhood in its age-associated progression through a distinctive sequence of pathologic changes from a purely multicystic lesion to a high-grade sarcoma. Type I PPB is composed of air-filled cysts with primitive mesenchymal cells beneath an intact, benign-appearing epithelium. These cysts can progress into type II PPB, in which the mesenchymal cells overgrow the septa, producing a cystic and solid neoplasm. Type III PPB is an exclusively solid sarcomatous neoplasm ,3.There is strong evidence for progression from type I to type II and type III PPB. Type Ir (regressed) PPB is a purely cystic lesion, microscopically devoid of subepithelial septal primitive cells, and it is considered to have a lower risk for neoplastic progression ,5. The pType I PPB may present with respiratory symptoms such as cough, dyspnea, chest pain or abdominal pain. Chest X-rays may show signs of pneumonia, and some cases may present with difficulty in breathing, from a pneumothorax or a large lung cyst, or a PPB can also present as an incidental radiologic finding of a lung cyst. The purely cystic type I PPB can be easily mistaken for other congenital lung malformations such as congenital pulmonary airway malformation (CPAM), as CPAM origins can be from the acinar structures of the lungs, thus creating a multiseptated cystic lesion similar to type I PPB. The diagnosis of type I PPB should be considered in the evaluation of any multicystic specimen of peripheral lung from young children. The final diagnosis requires meticulous pathological examination.Given the rarity and clinical heterogeneity, it is still unclear what is the optimal therapeutic approach. The treatment for type I and type Ir PPB evolved over time; most cases are treated with surgical resection alone, while in some cases adjuvant chemotherapy is added. Most type Ir PPBs are followed clinically after complete surgical resection, due to the low risk of tumor progression. Cases of type I PPB are treated with oncologic surgery alone. In cases of suspected incomplete resection or unresectable lesion, chemotherapy is added . A recenDICER1 variants as the first known genetic causes of PPB familial syndrome -fluoro-2-deoxy-d-glucose positron emission tomography-computed tomography showed no metastases : complex cystic lesion with aberrant vessels within the cyst septa located in the right upper lobe a. We susSix-year-old boy (older brother of patient no 3): two purely cystic lesions, one in the right middle lobe (diameter of 4 cm) and a smaller cyst in the left lower lobe b,c.Fifteen-month-old boy (younger brother of patient no 3): two purely cystic lesions, one in the right lower lobe (diameter of 0.6 cm) and a second cyst (diameter of 1 cm) in the left lower lobe d.We consulted the IPPBR team about the other three siblings as well; they considered the lesions to be a type I PPB, and resection was suggested.The parents were given the complete information about the findings and the consultations. They decided to proceed with a more conservative approach, with clinical and radiological follow up only. Two years later, the children are healthy, and the radiological findings are stable.DICER1-germline-positive patients. We summarize some of the challenges we dealt with while treating these patients.We presented six children with either proven PPB or DICER1-related cystic lung disease suspected to be PPB, all treated in our hospital within the last 5 years. This cluster of patients raised our awareness about this rare tumor and the diagnostic and management challenges it brings. Timely diagnosis and resection of type I PPB is crucial, but this entity is elusive, masquerading as a lung malformation, or growing silently in Can we differentiate CPAM from type 1 PPB, based on clinical aspects and imaging?Differentiating the entities may influence management. There are different approaches for the management of CPAM. Some centers advocate an aggressive approach by resection of all lesions, in fear of future progression to malignant PPB. Others choose to follow up, advocating that PPB is a rare entity and CPAM 1–3 are benign lesions that will not progress to PPB . The reap < 0.01) [Imaging alone cannot differentiate CPAM type 4 from type I PPB ,17,18. I < 0.01) .What are the differences between CPAM type 4 and type I PPB? Dehner et al. stated that CPAM type 4 and type I PPB are in fact synonymous, and, due to the concern of progression from type I PPB to the malignant type II and III PPB, it would be wiser to define these lesions as type I PPB rather than CPAM 4, and thus promote a more aggressive approach toward oncologic resection and genetic counselling .In Feinberg A. et al., the IPPBR team summarized their experience with the goal of answering this question. Overall, 112 cases of type I PPB gathered in the registry between 2002 and 2013 and 103 cases of CPAM that were resected in the same institution during the same period were reviewed. Features favoring CPAM were prenatal diagnosis, and CTA findings of a systemic feeding vessel and hyperinflated lung. Features that raised the suspicion of PPB were multi-segmental or bilateral lesions and the absence of prenatal ultrasonography findings .DICER1 germline mutation further confirmed our concern. We consulted the IPPBR, which supported our diagnosis of type I PPB. In the family with four children with DICER1 (patients 3–6), two of the three asymptomatic siblings have bilateral cystic lesions, and thus they are highly likely to having type I PPB.In our first two patients, the suspicion of type I PPB was high, due to the early symptomatic cystic lung lesion and normal prenatal follow up. In the recent IPPBR report, 85% of type I PPB cases were symptomatic at presentation, 32% with pneumothorax . The posDICER1 germline testing? Any congenital cystic lesion? Only in cases that are highly suspicious? Only in pathology-determined PPB?What are the indications for In 2016, the IPPBR convened an international DICER1 symposium and published a consensus paper on the indications for testing and the surveillance advised for DICER1-positive children [DICER1-germline-positive children are age based, with at least one chest CT performed in all cases [Recommendation for screening in ll cases . These iBased on the IPPBR recommendations, we tested and diagnosed the siblings of patient number 3, who presented with malignant PPB: all three siblings had CT findings suggestive of PPB. The family decided to continue with clinical and radiological follow up instead of resecting all lesions. This family evoked an ethical question regarding the indications for testing an asymptomatic sibling. The parents of patient no 3 stated during the follow up, post factum, that they preferred not to know the child’s DICER1 status. With increased awareness of the advantages of early detection of type I PPB, and the possible prevention of progression through early intervention, more families will eventually choose to know the DICER1 status. These four DICER1 siblings bring us to the next challenge.When can we safely say that a type I PPB lesion has regressed to Type Ir PPB?We know that pathology alone can provide the final discrimination between type I and Ir PPB, but in our DICER1 family, pathology was not available for the three siblings of patient no 3. The children differ in age and appearance of the lesions. The oldest sibling was 9 years old when diagnosed, and had a multiseptated single cyst, while the two younger brothers had multifocal simple cysts. Over 2 years of CT follow up, we saw no change in the appearance of the lesions in all three children (patients Nos. 4–6). Does it give us any reassurance? We know that the median age for diagnosis of type I PPB is 7 months, for type II PPB 35 months and for type Ir PPB 2.6 years , and thaThe IPPBR reported two cases of progression in type Ir PPB, both occurring within 2 years. They suggested 2 years of follow up for resected type Ir PPB. In type I PPB recurrence occurred more frequently and within 63 months, so they advised a longer follow up for type I PPB, of at least 6 years . We inteDICER1-germline-positive, asymptomatic child. Oncologic resection versus close radiologic and clinical follow-up: pros and cons.Multifocal cystic lesions in a Multifocal cysts are more difficult to manage. On the one hand, multifocal cysts have an increased risk of progression compared to a single cyst, while on the other hand multiple surgeries might increase surgical complications and decrease lung function. In the last IPPBR report , 25% of The role of chemotherapy in cases of suspected incomplete resection of type 1 PPB.PPB Ttpes II and III are aggressive malignant diseases and chemotherapy is mandatory. The primary treatment for type I and Ir PPB is surgery with complete resection. The reasoning for adjuvant chemotherapy in type IPPB and even type Ir PPB is to prevent recurrence or progression of any residual disease (microscopic or macroscopic) to the more aggressive tumor type II and type III PPB. In the recent report by the IPPBR , the groIs the prevalence of Type I PPB increasing in recent years, or have we misdiagnosed some of them as CPAM?Within five years we diagnosed six patients with DICER1 due to high level of suspicion, five of them were diagnosed with type I PPB. We consulted the IPPBR team in all our cases and learnt that there is a need for a high level of suspicion and experienced teamwork in order not to miss the type I PPB patients and to be able to treat them accordingly. It is possible that the prevalence of type I PPB is on the rise, due to the fact we might have misread some of our previous cases as CPAM. In fact, to emphasize the importance of a central pathology review for rare tumors such as PPB, it was reported that almost 20% of the pathology specimens submitted for review by IPPBR had another initial diagnosis. Thus, it may be challenging to identify a lesion as PPB, even for experienced pathologists .PPB is a rare tumor, with predicted progression from the benign type I PPB to malignant tumor. Timely diagnosis and complete resection of type I PPB correlates with the best prognosis. Symptomatic cystic lung lesion in infants, unidentified during pregnancy, especially when septated or multifocal, should raise the suspicion of type I PPB rather than CPAM, and induce testing for DICERI mutation and early complete resection. The management of multifocal cysts, the role of chemotherapy and management of older asymptomatic DICER1 children is complicated, and should sometimes be individualized. Consultation with the IPPBR team is recommended.


In the ovine reproductive management, a thorough breeding soundness evaluation including specific inspection of the male genital tract represents a valuable tool for assessing the reproductive potential of a selected subject and for diagnosing genital disorders. During examination, accurate inspection on penis and prepuce is necessary, since conditions affecting these structures may hamper regular coitus. Records from 1270 males undergoing breeding soundness evaluation (n = 1232) or admitted for genital disorders to the Section of Obstetrics and Gynaecology (n = 38) of the Department of Veterinary Medicine, were collected, and lesions of penis and prepuce were therefore classified. The data collected revealed that 47/1270 rams examined presented lesions of the penis and prepuce. The most frequent condition was urolithiasis accounting for over 2% of the cases, followed by lack of the urethral process (0.39% incidence), lack of the glans penis and hypospadias (0.23% cases). Moreover, most of the conditions (40%) were observed in animals less than 2 years old, suggesting the importance of a careful breeding soundness evaluation in animals at young age. Breeding soundness evaluation in rams plays an essential role in the management of sheep farming because it allows one to predict the reproductive potential of selected animals. Basically, it consists of general clinical examination, specific inspection of the reproductive tract, and semen evaluation. Although general and specific inspections alone are not able to recognize sub fertile animals, in most cases they are sufficient to detect disorders that could cause reproductive failure Boundy . SpecifiAnatomically, the prepuce is composed of a double fold of skin. It extends from the scrotum cranially, adhering to the ventral aspect of the abdominal wall. The most cranial portion (3-4 cm long) is free and ends with the preputial orifice, commonly rounded by hair.The penis in the ovine is a tubular fibro-elastic organ, around 40 cm long with an exiguous portion of erectile tissue. The body of the penis is characterized by a sigmoid flexure, slightly caudal to the scrotum, and the glans penis presents the urethral process in its left side . From January 2010 to December 2020, records from 1232 rams undergoing breeding soundness evaluation were collected. All animals were reared under traditional semi extensive systems and were examined 1 to 2 months before the beginning of the breeding season (April-May) by two operators of equal experience. Moreover, in the same years, records from 38 rams admitted for genital disorders to the Section of Obstetrics and Gynaecology of the Department of Veterinary Medicine were comprehended in the study. Each record included age, breed and identification number of the animal, date of examination, history, findings on general and genital examination, diagnosis of possible genital conditions and treatment. Lesions of penis and prepuce were included in the study.A complete general examination was performed on each animal focusing on body condition score, status of limbs, feet and teeth and evidence of systemic diseases.All males underwent careful clinical examination of the reproductive tract. The scrotum was inspected for symmetry, changes in colour of the skin, lesions, and dermatitis. The testes, epididymis, and spermatic cords were examined by palpation to assess size, shape, consistency and free movement inside the scrotum.The internal mucosa and the external skin of the prepuce were evaluated, and a special attention was paid to the preputial orifice. The presence of signs of injuries, foreign bodies, ulcers, abnormal or blood discharge was recorded. Afterwards, examination of the penis was performed by externalization. Briefly, the ram was placed in a sitting position as vertically as possible; the sigmoid flexure was grasped firmly between the index finger and the thumb, pushing upwards towards the prepuce orifice and, at the same time, pushing the prepuce skin downwards. When the penis was extruded, particular attention was paid to any difficulty in the passage through the preputial orifice. The glans and the urethral process were assessed for alterations . Any abnormal condition of prepuce and penis was recorded and described.Lesions of the penis and prepuce were recorded on 47 out of 1270 rams  of total cases. An older ram of 5–6 years of age, undergoing a routine breeding soundness evaluation, was diagnosed with a preputial papilloma. Papillomas were found also in the scrotum, in the lower left eyelid and in the skin of the back. Another two rams were admitted to the Section of Obstetrics for an abscess and a traumatic injury. In the first case, the prepuce appeared swollen, hot, and painful and penis exteriorization was not possible. Bacterial isolation was not performed. The traumatic injury was characterised by a laceration involving all layers of the skin of the preputial orifice.Injuries of the glans penis have been found in two animals. One was an injury caused by a penetrating foreign body on the dorsal aspect of the glans while the other was a laceration.A ram of 3–4 years of age, undergoing a routine breeding soundness evaluation, was diagnosed with the complete absence of the glans penis. In the anamnesis, no data regarding the mating efficiency and fertility were provided. During examination, complete exteriorisation of the penis was not possible due to adhesions. The apical portion of the penis was rounded and characterised by tight tissue. On its ventral aspect, it presented a groove containing the urethral opening, as pointed out by the insertion of a urinary catheter Fig. .The absence of the urethral process was observed in 5/1270 cases (0.39% incidence). The restraint of the animal during shearing with consequent accidental amputation of the process (as reported by the owners) was the cause in 3 out of 5 cases). In one subject, retroflexion of the urethral process was described. Despite a normal libido, the ram was hypofertile, reluctant to mount and had urine retention in the preputial cavity. Following examination, a balanoposthitis and a completely bent urethral process were observed. The urethral process appeared retroflexed at around 1 cm from the tip, and adhesions kept the bent portion attached to the main part of the process . All rams were admitted to our section with an history of inappetence, stranguria and ematuria. Three of them also presented light preputial swelling. Eight subjects affected by urinary obstruction also presented pressure necrosis of the urethral process and penis at the presumptive obstruction site and downstream  Urolithiasis affected more frequently young animals: 23/28 were less than 2 years old. On the 28 cases, 16 males, including those affected by necrosis of the penis, were euthanised because owners declined treatment and only four of them gave consent for necropsy. Uroliths were found in the bladder (4/4), in the sigmoid flexure (3/4), in the penile urethra (2/4) and in the urethral process (3/4) and composition analyses revealed all uroliths to be composed of struvite.The majority of conditions affecting the penis and the prepuce can be easily detected by an accurate clinical examination.Among the congenital disorders of the penis and prepuce, the only one reported in this study was hypospadias, affecting three young rams of less than 1 year old. In two of them a split scrotum was also present. These are not an uncommon finding, since hypospadias is often associated with other abnormalities of the reproductive tract  affecting more frequently young animals (less than 2 years old of age). This was not surprising since age is considered a risk factor due to the smaller diameter of the urethral lumen (Riedi et al. In the present retrospective study, animals younger than 1 year represent 26.5% of the investigated population. In the same age group, 40.4% percent of all lesions were found. These data strongly suggests that an early breeding soundness evaluation, performed in pubertal animals, would support the optimization of selection programs, leading to early diagnosis and treatment of genital diseases or replacement of the subject.


Due to the location of paragangliomas (PGLs) behind the retrohepatic segment of inferior vena cava (IVC), it is difficult to expose and resect the tumor.131I-MIBG) imaging revealed that the tumor was PGL. A three-dimensional printing was performed to visualize the tumor. The laparoscpic surgery for the PGL behind the retrohepatic segment of IVC was performed and the tumor was resected completely without causing any tissues injury. The pathologic diagnosis was PGL and the patient was able to recover well.A tumor measuring 50×45×62cm behind the retrohepatic portion of IVC was found in a 51-year-old female with hypertention and diabetes mellitus. Although the test for catecholamines revealed no signs of disease, the enhanced computed tomography (CT) scan, somatostatin receptor imaging and iodine-131-labeled metaiiodo-benzylguanidine (This case demonstrates that laparoscopic surgery may be helpful in tumor accessibility, and could be used in the appropriate cases to remove PGLs that are located behind the retrohepatic segment of the IVC. Paraganglioma/pheochromocytoma (PGL/PHEO) is a tumor originating in the chromaffin tissue of the neuroectoderm, which mainly secretes catecholamines . The claPGLs could be divided into parasympathetic PGLs  and sympathetic PGLs  depending on whether the tumor comes from the sympathetic or parasympathetic ganglia . SurgicaHowever, the locations of some PGLs are very special, which brings great difficulties to surgical resection. There have been few reports of surgical treatment of PGLs behind inferior vena cava (IVC), but the locations are usually low  –12. HereA 51-year-old middle-aged woman was hospitalized due to hypertension for 5 years. Blood pressure fluctuated between 150-160/100-110mmHg and could be controlled by medication. There were no accompanying symptoms of headache, dizziness, palpitation, and hyperhidrosis. The general condition was good.The patient was diagnosed with diabetes mellitus nine years ago. fasting blood glucose (FBG) and postprandial blood glucose (PBG) fluctuated between 6-7mmol/L and 7-10mmol/L, respectively. She also had hyperlipidaemia for two years. The patient had a history of tonsillectomy. The patient had no special personal or family history.131I-MIBG) imaging were also performed, and the solid-cystic mass showed increased radioactive uptake in the solid portion. To reproduce the tumor morphology more intuitively, our hospital carried out three dimensional (3D) printing based on imaging data  and metanephrine , and 24-hour urine catecholamine levels were as follows: Dopamine 310.6 μg/24 h , norepinephrine 2.2 μg/24 h , and epinephrine 31.9 μg/24 h . The hemoglobin level declined (90g/L). Contrast-enhanced computed tomography (CT) of the abdomen and pelvis showed the following findings Figure 1131I-MIBG imaging supported the clinical diagnosis of PGL. The patient did not undergo germline mutational testing. The patient was treated for anemia, and the hemoglobin level was raised to 118g/L before surgery. The patient was also given medication (phenoxybenzamine) for 3 months prior to surgery. After consultation with liver surgery, vascular surgery, thoracic surgery, anesthesiology, and intensive care unit departments, our department and liver surgeons developed a special surgical plan for this case: Laparoscopic resection of the PGL.Although the patient had no typical features or elevated catecholamines, the results of somatostatin receptor imaging and The patient was placed in the supine position. The intraperitoneal route was adopted, and laparoscopic puncture holes were chosen as: 1) parumbilical, 2) 5cm above umbilicus, 3) 2cm inside the right anterior superior spine, 4) below the xiphoid, 5) subcostal boundary of the right midclavicular line. The right lateral peritoneum was opened, and the ascending colon was isolated to expose the tumor area. The tumor was completely covered by IVC 3. Then The pathological diagnosis of the tumor was paraganglioma without adrenal involvement. Immunohistochemical analyses were as follows: soluble protein-100 (+), synaptophysin (+), chromogranin A (+), cytokeratin [CK (AE1/AE3)] (-), Ki-67 (index 2%), melanoma antigen (-), α-inhibin (-), succinate dehydrogenase B (+), O-6-methylguanine-DNA methyltransferase (-) Figure 3PGLs may involve or invade IVC . While sOf the eleven cases reviewed, two underwent laparoscopic surgery and one underwent robotic-assisted surgery. Nozaki et al. found that the laparoscopic approach provided excellent exposure, allowing proper identification of the tumor’s origin and its relationship to surrounding structures . The robWe report a rare case of PGL that lies behind the retrohepatic segment of the IVC. The PGL was successfully resected by laparoscopy without IVC injury or partial liver resection. This case demonstrates that laparoscopic surgery may be helpful in tumor accessibility, and may be tried for resection of PGLs behind the retrohepatic segment of the IVC in appropriate cases.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.WW participated in the writing of the manuscript. JW contributed to the editing of the manuscript. JD, HL and ZJ served as scientific advisors. All authors contributed to the article and approved the submitted version.


Goldenhar syndrome is a rare congenital disorder that affects the development of the craniofacial region, spine, and ears. It is characterized by a wide range of symptoms that can vary in severity and may include facial asymmetry, microtia or anotia, cleft lip or palate, vertebral anomalies, and eye abnormalities. Although the cause of Goldenhar syndrome is not fully understood, it is thought to be related to disruptions in the early embryonic development of the affected tissues. The diagnosis is typically made based on physical examination and imaging studies, and management may involve a multidisciplinary team of healthcare professionals, including geneticists, audiologists, and plastic surgeons. Treatment options depend on the specific symptoms and may include surgery, hearing aids, and speech therapy. While Goldenhar syndrome can have significant physical and functional implications for affected individuals, early detection and appropriate management can help improve outcomes and quality of life. Goldenhar syndrome (GS), also known as oculo-auriculo-vertebral dysplasia, is a rare multifactorial condition characterized by a defect in the development of structures derived from the first and second branchial arches . The firMost cases of this congenital condition are diagnosed at birth due to the presence of classical features. However, in some cases, patients may present atypically. Developmental delays and intellectual disability are uncommon features of GS and are seen in 5-15% of cases . The undIn this report, we present the case of an eight-year-old female diagnosed with GS at birth; however, she did not have the typical unilateral asymmetric craniofacial or vertebral abnormalities. Instead, she had ocular defects and developmental delays in the gross, fine, and speech milestones.An eight-year-old female diagnosed with GS at birth, born from a non-consanguineous marriage, was brought to the child psychiatry department of a tertiary care hospital with complaints of delay in developmental milestones at the age of four. The child was born at term by cesarean section, weighing 2.9 kg. She had no history of neonatal jaundice or any other cardiopulmonary complications at birth. The mother also had no antenatal complications during her pregnancy. The child’s parents were healthy, and there was no family history of genetic disorders. Her birth features included bilateral upper eyelid colobomas (left larger than right), bilateral limb dermoids and lipodermoids, and preauricular skin tags. She also had incomplete eye closure, and the globe was visible in both eyes at the time of birth. She underwent corrective surgery, which included bilateral upper lid coloboma repair with eyelid reconstruction and the excision of pre-auricular tags under general anesthesia at three months of age. After canthotomy, superior cantholysis, and direct suturing, good correction of the coloboma was achieved in the right upper lid. The larger coloboma on the left side necessitated a modified Hughes sliding flap for posterior lamina reconstruction. The skin was obtained from a pre-auricular tag after soft tissue debunking; a full-thickness graft was used for anterior reconstruction. The patient had asymmetrically shaped ears, with the right ear lobule larger than the left. She did not have any hearing loss. Genetic testing was done to rule out any chromosomal abnormalities. Hence, based on her clinical presentation, especially ocular findings, bilateral limb dermoid and lipodermoids, and preauricular skin tags, a diagnosis of GS was made. The patient had no other features typical of GS such as unilateral asymmetric facial defects, ear defects, mandibular defects, or vertebral abnormalities.At the age of three months, the patient had a few seizures, and electroencephalography (EEG) was done. The child was sedated and asleep during most of the EEG recording, and her sleep patterns were symmetrically distributed. The EEG revealed an elliptic waveform at 8 Hz over the posterior head region. She was diagnosed with a seizure disorder. The patient was prescribed Levicetiracetam, and the frequency of her seizure episodes gradually decreased. The child had not experienced any episodes in the past 10 months. However, in the later course of her development, the patient showed delays in developmental milestones. She experienced a global delay in gross motor, fine motor, social skills, and language.The patient was brought to the child psychiatry department of a tertiary care hospital at the age of four with complaints of delays in milestones. At the age of four, MRI was done due to delayed milestones and frequent seizure episodes, revealing a focal area of thinning of the posterior body of the corpus callosum (CC) Figure .There was a symmetric gliotic signal with a T2 fluid-attenuated inversion recovery (FLAIR) hyperintense signal and a T1 hypointense signal in both the centrum semiovale and the posterior corona radiata and peri-trigonal white matter (Figure The angular margins of the trigones of the lateral ventricles were noted. The mild gliotic signal in the right dentate nucleus was greater than that in the left dentate nucleus. A thinned-out posterior corpus callosum was suspected to be causing a delay in cognitive and speech development. No radiological abnormalities were found on the cervical and spinal X-rays. The patient had two siblings with normal development. Currently, the child is receiving treatment for a delay in speech, gross, and fine motor skills. It includes speech and language therapy along with occupational therapy. She is also on quetiapine for her mood changes, in addition to the antiepileptics. No significant improvement in speech has been observed with speech and language training. She hoots and makes sounds but cannot say or write words. Her vocabulary is limited to single words in the native language, indicating the names of her mother and father, which are also not proper or complete. Gross motor skills have shown improvement with occupational therapy, which is mostly activity-oriented. The child is presently attending a school for special children affiliated with the psychiatry department of the hospital. However, she still lacks social cues and finds it difficult to be around her peers at the special school as well.The branchial arches form within the fourth week of development and are responsible for developing many craniofacial structures . The firGS is characterized by the classic triad of mandibular hypoplasia, ocular and auricular malformations, and vertebral abnormalities . HoweverThe CC is a thick bundle of nerve fibers that connects the two cerebral hemispheres, allowing them to communicate and coordinate appropriate bodily functions . There iIn this case, thinning of the CC was possibly due to cerebral gliosis. Gliosis occurs when there is an injury to the central nervous system due to infarcts, infections, toxins, or neoplasms, which may lead to scarring and cerebral dysfunction . On MRI,The diagnosis of GS is clinical. Imaging tests such as a CT scan, an MRI, an ultrasound, an echocardiogram, and an EEG can be conducted to assess the severity of symptoms . In thisTreatment of GS is symptomatic and may include a combination of supportive, medical, and surgical modes of therapy. Feeding assistance and hearing aids and glasses for visual and hearing impairments are commonly used . PhysicaIt is essential for physicians to note that while most patients present classically, a few cases are atypical; therefore, a high index of clinical suspicion is required. This will allow for early diagnosis and management of GS and prevent a decline in the child’s quality of life.GS is a rare multifactorial condition possibly caused by defective cellular migration and is characterized by the abnormal development of the structures derived from the first and second branchial arches. It is classically characterized by the triad of mandibular hypoplasia, ocular and auricular malformations, and vertebral abnormalities. The effects of GS on the brain are not well known; however, there is vast neurological involvement associated with this syndrome that requires further research. In this report, it is suspected that the global developmental delay and seizures were caused by focal thinning of the CC and surrounding cerebral hemispheres due to underlying gliosis. Physicians should be aware of this possible association and implement diagnostic tests such as MRI when presented with children with developmental delay and seizures in the setting of GS. Early diagnosis and intervention with symptomatic treatment and psychosocial support will improve the quality of life for children as they age and adjust to functioning in society.


A sacrococcygeal teratoma (SCT) is a rare embryonal tumor that emerges in the sacrococcygeal area. It affects one in every 35 000–40 000 live births. Herein, we report a case of a substantial SCT in a neonate.A neonate girl from consanguineous parents was delivered by cesarean section with a large mass (18×17 cm) in the sacrococcygeal area. The baby’s birth weight was 5 kg, of which 2.5 belonged to the mass. The vital signs were within normal ranges and she had weak movement with bluish peripheral limbs. Oxygen saturation was around 85% for a short period after birth. According to the American Academy of Pediatric Surgical Section, the tumor was type I. After the fifth day of delivery, a complete resection was done through a chevron incision. The patient was put on ‘nil by mouth’ for about 24 h and given intravenous fluid.The histopathological examination of the surgical specimen confirmed extragonadal immature teratoma. The histological classification of SCT is divided into three types: malignant teratomas ; immature teratomas ; and mature teratomas (fully differentiated tissues).SCT has rarely been reported as a giant mass. Radiologic examinations in the early stages of pregnancy may be essential to the early diagnosis of the condition. HighlightsSacrococcygeal teratoma (SCT) is a rare form of embryonal tumor that emerges in the sacrococcygeal area.SCT affects about one in every 35,000–40,000 live births.This phenomenon is a very uncommon disease, and there are only a few reported cases in the literature.This study aims to report a rare case of a giant SCT in a neonate.1. Detecting first-trimester SCT relies on identifying a protruding mass in the caudal region. Some SCTs might be small during the first trimester, making them harder to detect early. Hence, only cases with large masses can be diagnosed in this period2. In addition, the routine sonographic examination in the second trimester aids in diagnosing the tumor to prevent perinatal and neonatal complications such as fetal hydrops, tumor rupture, high output cardiac failure, and placentomegaly, which are associated with increased mortality rate5. Clinical signs and symptoms such as anal displacement with the tightness of the anal canal and constipation can occur due to tumor compression on the bladder or rectum4. In 1973, based on the intrapelvic/intra-abdominal extension of the tumor and its external component, Altman classified SCT into four types, according to the American Academy of Pediatric Surgical Section (AAPSS)6. Herein, we report a case of giant SCT in a neonate. The report has been written according to the SCARE  guideline and the references have been confirmed to be eligible8.Sacrococcygeal teratoma (SCT) is a rare form of embryonal tumor that emerges in the sacrococcygeal area (SCA), containing tissues from all three germ layers . SCT affects one in every 35,000–40,000 live births with a female-to-male ratio of 3:1 to 4:12, without any chronic diseases.A neonate girl from consanguineous parents was delivered by cesarean section with a large mass 18×17 cm) in the SCA  and respiratory rate (50 breaths per minute) were normal, while she had an oxygen saturation of around 85% for a short period after birth. She had weak movement and bluish peripheral limbs.The mass weight was 2.5 kg, with a soft surface and variable consistency Fig. . After dThe baby was admitted to the neonatal intensive care unit (NICU). An incubator was used to prevent hypothermia, oxygen therapy by nasal cannula, and for further monitoring and management.After the fifth day of delivery, under general anesthesia, a complete resection through a chevron incision was done. Intraoperatively, a Hegar dilator was placed inside the rectum as a guide, and a small lower lateral rectal tear was sutured without postoperative complication Fig. . The patThe postoperative period was uneventful, and the baby was discharged home after 7 days. After 2 years of follow-up, no recurrence was demonstrated.9. According to the AAPSS, there are four types of SCT based on internal and external tumors. Type I is characterized by an externally visible mass; type II by an external mass with a considerable intrapelvic component; type III by an external mass with a pelvic mass; and type IV by an entirely internal mass. Prenatal U/S makes identifying types I and II easy, while type IV has a higher risk of malignancy. The present case was a neonate female with a type 1 SCT that could easily be diagnosed by U/S examination during the second trimester. Regular U/S is often used in the second trimester to make an antenatal diagnosis, while detection is possible in the first trimester. It is common for pregnant women, and also in our case, to skip regular U/S screenings in the first trimester, which causes challenges in detecting masses at an early stage3. It has been reported that the detection of SCT in the first trimester may go unnoticed due to its small size2. However, in the present case, due to the large size, it would have been noticed in the first trimester. However, we believe that even if we had detected it in the first trimester, nothing significant would have changed in the therapeutic plan. Due to cultural beliefs, it was impossible for parents to accept termination during that period.The SCT is an infrequent embryonal tumor in the SCA of neonates. It has a favorable prognosis depending on the date of diagnosis, the simplicity of coccygectomy, and the tumor’s risk for malignancy. The female neonate is mainly affected; however, it is unclear why women predominate11. The primary treatment for SCT is coccygectomy within a few days after delivery. The optimal time for surgery is between the infant’s birth and 2 months of age. The surgery time is essential since postponing is linked to a greater risk of malignancy and recurrence. Without coccyx excision, the recurrence rate reaches 37%10. Due to the above-mentioned risks, in the present case, a coccygectomy was performed on the fifth day after delivery.The Benachi classified the prognosis into three categories. Group A includes tumors smaller than 10 cm or avascular tumors moderately vascularized with gradual growth; group B includes tumors greater than 10 cm with rapid growth and marked vascularity linked to heart failure; and group C includes tumors of 10 cm or greater with gradual growth, cystic predominance, and moderately vascularization. The tumor of the present case was classified as group C3. During the perinatal period, increasing tumor size may cause obstetric complications such as a premature baby, tumor hemorrhage or rupture, or dystocia1. In the present case, although the mother was overweight with some blood pressure instability, no complications occurred during and after birth. Body structural anomalies may be found due to the tumor’s growth, such as cardiomegaly, anal stenosis, and hydronephrosis. The size of the tumor and the pressure it puts on nearby organs may be detected by MRI, which can also differentiate this condition from common differential diagnoses such as distal neural tube defects (myelomeningocele or myelocystocele)3.Infants with large or severe vascular tumors should have a cesarean delivery to limit the risk of bleeding3. Although the literature has reported excellent survival rates, the mortality rate for masses larger than 10 cm is 18%. Even in patients with an entirely removed coccyx, the probability of recurrence ranges from 11% to 22%, so close follow-up every 3–6 months with physical examinations such as a rectal exam, diagnostic imaging, and AFP are recommended for at least 3 years9. In our case, there has been no recurrence after 2 years of follow-up.Recently, there has been an agreement that coccygectomy is the best way to avoid a benign teratoma recurrence with a decrease in any harm to the tumor cyst wall. However, even after complete excision, an SCT may return years later, so patients must be under continual monitoring till adulthood12. The histological classification of SCT is divided into three types: malignant teratomas ; immature teratomas ; and mature teratomas . Histopathology results in our case confirmed an immature teratoma. The microscopy section in histopathology revealed mature tissues from all three germ layers. In the present case, AFP was elevated; however, MRI revealed that the mass was type I. According to the AAPSS. A complete resection of the mass was conducted through a chevron incision.High levels of tumor markers, such as AFP, are used to determine the probability of a malignant tumor. High levels of AFP may indicate malignancy. In newborn babies, these values are typically high and only return to normal levels around 9 months of age. Therefore, this may lead to misdiagnosing the condition as a malignant tumor. Due to this reason, an MRI was done for the present case to differentiate if the tumor was malignant or notSCT has rarely been reported as a giant mass. Radiologic examinations in the early stages of pregnancy may be essential to the early diagnosis of the condition. Despite the associated risks and complications, neonates with giant SCT can be delivered by cesarean section without significant complications.The ethical committee of the University of Sulaimani does not need ethical approval for case reports (until 3 cases) in our locality.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.The patient was completely satisfied with the management approach and did not have any complaints about it. Additionally, the patient praised the staff for their efforts during his treatment.None to be declared.W.N.S.: the pediatric surgeon who managed the case and was one of the major contributors to the study; K.M.H., B.A.M., and H.O.K.: conducted the literature review; S.M.A. and F.H.K.: involved in the manuscript drafting; B.A.A., S.H.M., Z.B.N., R.Q.S., and H.A.A.: involved in the critical review and improvement of the manuscript. All of the authors were involved in the final approval of the study.The authors declare that they have no conflicts of interest.http://www.researchregistry.comOur case does not match the criteria you have mentioned as it is a case report, so our study was not registered. If registration is still needed at the end, we will register the case at the following registry: Fahmi H. Kakamad.Not applicable.Not commissioned, externally peer-reviewed.


Soft-tissue sarcomas (STS) comprise a heterogeneous category of malignant tumors originating from mesenchymal tissue. Spindle cell sarcoma, characterized by its infrequent occurrence, poses diagnostic and therapeutic complexities owing to its rarity. We present a case of an 80-year-old male with a diagnosis of spindle cell sarcoma in the retroperitoneal space. The patient underwent midline exploratory laparotomy for tumor excision and was planned for postoperative chemotherapy. Unfortunately, the tumor recurred aggressively, leading to a fatal outcome. This case highlights the uncommon occurrence of retroperitoneal spindle cell sarcoma (RPSCS) and the importance of accurate diagnosis, appropriate surgical management, and adjuvant therapy. Less than 1% of all adult malignancies are sarcomas, a rare type of malignant tumor originating from the mesenchymal tissue . SarcomaOne of the unusual types of undifferentiated STS is spindle cell sarcoma. Only a few cases have been documented in the medical literature due to their rarity . SpindleAn 80-year-old male presented to the surgical outpatient department with the complaint of a mass in the left hypochondrium and intermittent constipation. The patient had first observed the mass two months ago. Mass had gradually increased in size. It was non-tender, extending from the left hypochondrium to the umbilical region. Umbilicus was inverted, and there were visible veins on the mass and the adjacent quadrants as well. Intermittent constipation was associated with the abdominal mass. His history was remarkable. He had been treated for a left-sided direct inguinal hernia five years ago. He had a left-sided orchidectomy due to an extra-testicular atypical lipomatous tumor three years ago.The patient was vitally stable. A general physical examination revealed that the patient was blind in the left eye, secondary to a road traffic accident. No remarkable findings in the examination of other systems, namely nervous, respiratory, and cardiac, were found. A complete blood workup was done. No significant finding was present. Ultrasound of the abdomen and pelvis pointed toward a retroperitoneal mass. Based on this finding, a computed tomography (CT) scan of the chest, abdomen, and pelvis was performed. Along with it, levels of serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (hCG) were also measured. Both were within normal ranges (1.42 IU/mL and <2ng/ml). CT scan showed a large complex abdominal mass occupying most of the abdominal cavity, measuring 28.5 × 20.0 cm, having internal soft tissue density and fat density mass. The lesion was found pushing the left kidney superiorly, along with compression of descending colon. No remarkable finding in the chest or pelvic region was found. There was no lymphadenopathy. Furthermore, a core biopsy of the lesion was performed. Section revealed tissue cores with spindle cell neoplasm, composed of sheets and fascicles of moderately pleomorphic spindle cells with 15 mitoses per 10 high power fields. Immunohistochemical staining showed CD34 and S100 positive. Hence a diagnosis of spindle cell sarcoma was established. Based on the complete workup and established diagnosis of spindle cell sarcoma, midline exploratory laparotomy was performed. A mass measuring 21 × 20 cm was excised after separation from surrounding structures. Mass was 15 kg in weight, measured after the operation. The post-operative recovery of the patient was uneventful. Histopathology of the excised lesion confirmed the results of initial investigations (core biopsy). The patient was explained about the nature of his illness and the importance of regular follow-ups. The patient was directed to the oncology department for postoperative chemotherapy. However, the patient failed to comply with the surgeons’ advice even after repeated explanations and did not get an oncology consult.The patient presented again four months later with similar complaints and additional complaints of urinary obstruction and lower back pain. Mass had recurred in the abdomen, and it was in the left hypochondrium. Mass had grown aggressively this time. Mass had started growing for one month (that is three months after the first laparotomy). CT scan showed 210 × 145 × 200 mm hypodense mass in the left hypochondrium and pelvis. It had a bilobed appearance with well-defined margins having internal necrosis and a solid component with no calcific or fatty component, causing compression over the left ureter leading to significant hydronephroureter. Details can be seen in Figure However, the patient’s vitals started crashing intraoperatively. A mass compromising of 10 kg was excised. The patient was shifted to intensive care unit (ICU) post-operatively. Unfortunately, the patient’s vitals did not recover, and the patient expired within 48 hours of the surgery.To the best of our knowledge, limbs are where soft tissue tumors typically manifest. Abdominal soft tissue tumors are, therefore, uncommon, particularly retroperitoneal spindle cell tumors . People Due to their rarity, sarcomas sometimes undergo delayed or incorrect diagnoses, particularly in settings with limited facilities. A patient's history, imaging results, and biopsy are all part of the evaluation of a suspected soft-tissue sarcoma patient. In the case being presented, a CT scan and biopsy helped immensely in directing surgeons toward a definitive diagnosis. The key modality for evaluating soft-tissue masses in the extremities, trunk, head, and neck is magnetic resonance imaging (MRI) .The recurrence of spindle cell sarcoma can vary depending on several variables, such as the location and stage of the tumor, the efficacy of treatment, and unique patient characteristics. Local recurrence following surgery was documented in two distinct investigations by Swamsura et al. and Diageler et al., with median follow-up times of 19 months and 15.7 months, respectively ,9. SurgeSTS must be viewed as a single entity to assist clinical research on these incredibly rare disorders. Due to the diverse natural history and biological behavior exhibited by STS, establishing management guidelines that adequately address the unique clinical presentations of individual patients is exceedingly challenging. With more than 50 distinct histologic subtypes, physicians must tailor guidelines to align with the specific clinical characteristics of each patient's disease. Nonetheless, certain fundamental principles form the basis for a multimodal approach to managing STS, which should be applied within the framework of a highly personalized assessment of both the patient and the tumor.Publication of this case report on spindle cell sarcoma is essential to add to the limited body of knowledge on this uncommon and heterogeneous disease, providing valuable insights into its clinical presentation, management strategies, and potential therapeutic approaches, ultimately enhancing and improving the care for patients with spindle cell sarcoma.


Bowel perforation is an emergency condition that requires critical thinking and readily intervention; nevertheless, on occasions, its presentation can be challenging to diagnose. Several etiologies could cause bowel perforation, including obstruction, mass, inflammation, ischemia, etc. On rare occasions, a foreign body could be the cause of perforation, which mandates a detailed history and focused review of the images when the patient’s condition allows. We report a case of ileal perforation caused by an ingested wooden toothpick that was suspected on the CT images, which the patient has no memory of ingesting.  Foreign body (FB) ingestion is a common emergency presentation, with a higher incidence observed in the pediatric and geriatric age groups. While most cases of FB ingestion pass through the gastrointestinal (GI) tract without causing any noticeable symptoms, there are situations where it becomes a surgical emergency and requires intervention. Bowel perforation, although rare, can occur in <1% of cases, with sharp objects being the main culprits in such instances.The diagnosis of FB ingestion can be challenging, especially when the object is not radiopaque or when it becomes surrounded by inflammatory processes that hinder visualization on imaging studies. This can make it difficult to determine the presence and location of the FB. In such cases, diagnostic laparoscopy is an excellent surgical option. It allows for the direct visualization of the GI tract and the identification of the etiology behind the patient’s symptoms. This approach enables surgeons to accurately diagnose and manage the condition, offering a more targeted and effective treatment plan. If an FB is identified during the procedure, surgical intervention can be performed to remove it. Laparoscopy offers several advantages over open surgery, including smaller incisions, reduced postoperative pain, shorter hospital stays, and faster recovery times.In this case report, we present a unique and uncommon occurrence of FB ingestion that posed a diagnostic challenge but was ultimately managed successfully after careful evaluation and consideration.A 36-year-old female, not known to have a medical illness, underwent laparoscopic Sleeve Gastrectomy 3 years ago and laparoscopic cholecystectomy 1 year ago. She was presented to the emergency department on 2 June 2023 with a 1-day history of sudden, severe, left-lower-quadrant (LLQ) pain that was nonradiating, scaled 7/10, and associated with nausea and vomiting. She denies a history of diarrhea, constipation, fever, melena, or hematochezia. On clinical examination, the patient’s vitals were within normal ranges.3. Contrast-enhanced CT of the abdomen (The abdominal exam showed severe tenderness in LLQ + rebound tenderness. The white blood cell count was 9000/mm abdomen  and 2 shMore history was obtained from the patient, specifically focusing on FB ingestion; she denied intentional ingestion of FBs or eating fish any time before the initiation of her symptoms. Obstetrics and gynecology service was consulted regarding the right adnexal mass on CT in the same admission, and ultrasound was performed and it showed neither adnexal mass nor ovarian abnormalities.During her hospital stay, the patient showed no improvement regarding abdominal pain, nausea, or abdominal exam. Therefore, she was offered a laparoscopic exploration to verify the diagnosis of a FB to which she agreed. She was taken to the operating room, and the exploration showed an inflammatory process in the LLQ  with theThe patient recovered without complications and was discharged home on Postoperative Day 2. Upon follow-up in the clinic, the patient reported the resolution of all her symptoms and was discharged from the clinic.In this rare presentation of ingested FB, the diagnosis was quite challenging, given the fact that most of the patients in their full state of mind deny ingestion of FB, as its ingestion in this age group is improbable, and possibly an adult individual would memorize or notice ingesting a FB.FB ingestion is an emergency condition that requires prompt attention. The most affected populations are the pediatric, geriatric, alcoholic, psychiatric, and patients with dentures , 2. TypeThe most common sites of perforation account for the duodenum and the sigmoid colon; other sites include the esophagus, stomach, and small bowel . The mosAn upper GI endoscopy is an effective choice for patients who report ingesting FB, and the diagnostic modalities locate it in the proximal GI tract, and the gastroenterologist shall determine accessibility . The sucDiagnostic laparoscopy is feasible and effective in diagnosing and managing FB complications. Furthermore, it can be utilized to establish a diagnosis and treat the cause of the patient’s symptoms and any present complications when the diagnosis is in doubt .FB ingestion is a rare presentation; yet, serious complications could happen and be fatal, and a prompt intervention when complications occur is mandated to decrease the morbidity and mortality of the patient. A high index of suspicion, especially in the extremes of ages, and detailed history along with thorough investigation is crucial to reach a diagnosis. Endoscopy has a rule in the proximal GI tract FB; surgery whether laparoscopic or open is mandated in case of the presence of complications.


This is the first report on a localized pigmented villo-nodular synovitis (PVNS or TSGCT) occurring in the trochanteric bursa. Bursal involvement in PVNS is extremely rare. Most often PVNS occurs either as a localized or diffuse lesion in a major synovial joint, such as the knee, ankle joint or hip joint. In principle, all synovial structures can be involved. The case reported here is remarkable regarding the long period between the occurrence of the first symptoms and the final diagnosis as well as the age of the female patient (75 yrs).Therapeutically a complete resection was performed in order to avoid recurrence. More then three years later the patient did well and there has been no evidence of recurrence yet. A 75-year-old female had been suffering from a fluctuating soft tissue mass on her lateral proximal thigh for seven years. Prior to the symptoms she had fallen on her left trochanter. Primary treatment was done symptomatically. Due to the long-lasting symptoms a MRI was finally performed exhibiting the findings mentioned below. The patient was admitted without any clinical signs of infection, an almost normal gait-pattern and a free range of motion of the ipsilateral hip joint. White blood cells (WBC) and C-reactive protein (CRP) were within normal range. Lateral to the greater trochanter in the bursa trochanterica there was evidence of fluid showing homogeneous hyperintensity without sedimentation in fluid-sensitive sequences. Proton-weighted imaging revealed two nodular wall extensions in teardrop shape extending craniolaterally. These extensions measured a maximum of 1 cm at the base. In addition, at the roof of the fluid-filled cavity there was a space measuring approximately 1.5 x 0.5 cm. In the native fat-suppressed T1 turbospinecho sequence, this space-occupying lesion was partially hyperintense. This was considered to be hemosiderin. After application of gadolinium, there was a vigorous enhancement of the lining of the height. In addition, there was diffuse enhancement of the muscles in the immediate vicinity of the bursa. The hemorrhaged mass showed no enhancement of the contrast medium. In summary, the recent MRI exhibited an enlarged trochanteric bursitis with some intrabursal soft tissue formation suspicious for pigmented villo-nodular synovitis (PVNS) of the bursa trochanteric (PVNSBT) Figure 1 . The enlarged trochanteric bursa exhibited several lacunae with a thickened synovial layer in terms of an unspecific synovitis and parts showing villo-nodular yellowish to brownish synovitic changes to the extensions already visable in the MRI. Furthermore, the medial glutaeus muscle exhibited degenerative lipomatous changes, probably as a sequela of her previous trauma. Therapeutically a complete resection of the trochantic bursa was performed to ensure total resection of PVNSBT tissue. Histologically PVNS of the bursa was confirmed without any signs of malignancy. Postoperatively the patient recovered well. At final follow-up three and a half years postoperatively, no signs of recurrence could be detected with a follow-up MRI. Clinically the patient exhibited a slight limping owing to the weakness of the medial glutaeus muscle. Pigmented villo-nodular synovitis (PVNS) and/or giant-cell tumors of tendon sheaths / tenosynovial giant cell tumors (TSGCT) are rare diseases of synovial tissue occurring most often in synovial joints such as knee and ankle joints , 2], , , 5]. . 5]. Apart from synovial joints in principle all synovial structures  such as The incidence is reported to be about 1–2/million , 12], , . A more Jaffe et al.  were, toPVNS is mostly an intraarticular process but may also expand towards the extraarticular vicinity of the involved joint. Other joints such as shoulder and elbow joint, the wrist, fingers, subtarsal joint, other joints of the foot, the temporo-mandibular and the infratemporal fossa , 16], , , 18], ,  are rareDue to the osteolytic capability of giant-cells in PVNS the tumor can invade bone and produce osteolytic lacunae in these bones , 26], , , 28] or or28] orRarely, a multilocular articular occurrence is reported , 32]. I. I32]. IEven in metastasing cases the primary lesions may normally exhibit no signs of malignancy. Benign but aggressive PVNS is mostly seen in adults aged between 20 and 50 years, but even in children this lesion has been found , 38], , , 40]. I. I40]. IThe occurence in bursa has been described only once so far . Only caApart from the hip joint there is only one report of PVNSB involving the iliopectineal bursa in the region of the hip . Regarding the diagnostic procedure, the most important issue seems to be to remember such a rare disease. In addition, this case showed two specialities: the age of the patient was very high and the bursa trochanteric, normally a very small bursa, was extremely enlarged and contained “normal” infected synovial tissue parallel to PVNS synovitis with the typical macroscopic changes such as pigmentation, villi and noduli. The rarity of this location might have caused the long period between onset of symptoms and final diagnosis and immediate treatment. As for many other cases it is well-known that patients suffer for a long time before the final diagnosis is made as they experience unspecific symptoms such as tenderness, joint effusion, swelling and a limited range of motion. Regarding imaging procedures MRI has a central role in the identification of both the extra- and intraarticular tumors , 54]. P. P54]. PThe current treatment of choice for both the extraarticular diffuse and localized type of PVNS as well as for TSGCT is the complete surgical removal of the tumor masses if possible , 57], , . In primary circumscribed tumors of either location (intra- or extrarticular) additional adjuvant therapeutical procedures such as external radiation or radiosynovectomy with isotopes do not seem to be indicated, and neither are new medical targeted therapies such as imitanib or similar medications.In contrast, for the diffuse type an additional adjuvant therapy such as postoperative radiotherapy , 2], ,  or radioPVNS is a very rare disease of synovial joints or other synovial structures containing synivial tissue such as tendon sheaths, and involvement of bursa is extremely rare. We report the first case involving the trochanteric bursa in a 75-year-old woman, who had suffered for seven years before being diagnosed. This indicates that the awareness of such a bursal lesion is most important to shorten the time until the final diagnosis is made. The authors declare that they have no competing interests.


An 89-year-old male presented with syncope and worsening difficulty in breathing through the left nostril. Computed tomography demonstrated a tumor in the anterior ethmoid air cells and maxillary sinus, which extended into the frontal lobe. Magnetic resonance imaging similarly demonstrated an aggressive lesion. This mass was difficult to differentiate from more commonly seen lesions at this location such as an esthesioneuroblastoma or nasopharyngeal carcinoma. Direct visualization, biopsy, and subsequent pathologic analysis eventually confirmed the diagnosis of malignant Ewing sarcoma (EWS).Our case explores the radiological findings of EWS originating from the ethmoid sinus, compares EWS with other common carcinomas in the same location, confirms the diagnosis through pathological correlation, and investigates the prognosis and treatment of these lesions. This case highlights the importance of a multidisciplinary approach to diagnose EWS when it occurs in an atypical location. The clinical team relied on input from the radiology, surgery, ENT, neurology, and pathology departments to make an accurate diagnosis and plan treatment for this aggressive tumor. Ewing sarcoma (EWS) is a rare malignant bone tumor that primarily affects the pelvis and lower extremities of children and adolescents. Rarely, EWS can develop in the sinonasal tract. The incidence of adult-onset EWS in the sinonasal tract is difficult to accurately assess due to the rarity of this cancer in adults and within this location. Head and neck cases of EWS range from 3-9% of all EWS cases . A revieHistory and examinationAn 89-year-old man presented with syncope and progressive difficulty breathing through the left nostril. His past medical history was significant for coronary artery disease, benign prostate hyperplasia, and stage 3 chronic kidney disease.A review of systems was negative for nosebleeds or headaches. Physical exam demonstrated symmetric facial movement, negative sinus tenderness, intact extraocular eye movement without nystagmus, normal cranial nerve II-XII function, and no lymphadenopathy. Nasal and oral examination showed no masses or polyps, with moist mucous membranes.Imaging findingsThe patient underwent computed tomography (CT) of the head without contrast Figures , 1b. IniThe patient subsequently underwent magnetic resonance imaging (MRI) of the brain with contrast for further evaluation of the mass Figures -2c. TherHistological analysisThe patient was referred to the ear, nose, and throat service. Flexible fiberoptic laryngoscopy was performed, demonstrating a mass superior and medial to the middle turbinate with normal surrounding mucosa and no ulceration, correlating with the mass visualized on cross-sectional imaging. Biopsy samples were taken and sent for histological review.Differential diagnoses for the mass included esthesioneuroblastoma (ENB), nasopharyngeal carcinoma, angiofibroma, and squamous cell carcinoma. Left nasal biopsy showed undifferentiated small, round, blue cells with apoptotic bodies and mitotic figures Figures , 3b. ImmEwing sarcoma (EWS) is a primary bone and soft tissue tumor typically occurring in children and adolescents and usually originating within the medullary cavity of long or flat bones . Common In 85% of EWS cases, a t chromosomal translocation is found. The EWSR1 gene from chromosome 22 fuses with the FLI-1 gene on chromosome 11 . This fuA characteristic feature of Ewing sarcoma is the presence of a periosteal reaction, typically referred to as an “onion skin-like” or lamellated pattern of new bone formation. On CT imaging, Ewing sarcoma typically appears as an aggressive heterogeneous mass with poorly defined lucent lesions and margins sparing the cortex. On MRI, there is intermediate to high signal intensity on T2-weighted images and low to intermediate signal intensity on T1-weighted images . In contThe histological differences between EWS and the differential diagnoses in this case ultimately demonstrated the importance of multidisciplinary radiologic-pathologic teamwork. EWS typically demonstrates uniform, small, round cells with round nuclei and stippled chromatin. There is a high nuclear-to-cytoplasm ratio and mitotic figures are sparsely present. Additionally, the tumor cells contain abundant glycogen within the cytoplasm with minimal intervening stroma between cells. In the majority of cases, EWS stains positive for CD99, vimentin, FLI-1, and NKX2.2, the combination of which carries high specificity for the disease . In contTreatment for Ewing sarcoma also involves a multidisciplinary approach. Neoadjuvant chemotherapy of alternating vincristine, doxorubicin, cyclophosphamide (VDC), and ifosfamide and etoposide (IE) is considered the standard of care in the United States. Slightly different chemotherapy regimens have also proven successful, such as the exclusion of cyclophosphamide or just relying on VDC without the use of alternating IE, depending on the shared decision-making between the oncologist and patient. These chemotherapy agents are typically used in combination with targeted radiotherapy of the primary site. Patients who received radiation therapy for metastatic EWS have better three-year event-free survival than those who do not (35% versus 16%) . ExcisioSinonasal Ewing sarcoma represents an exceedingly rare diagnosis, particularly in older populations. This, in conjunction with the various other differentials most commonly seen within the sinonasal tract, makes diagnosis on cross-sectional imaging difficult. This case highlights the importance of maintaining an index of suspicion for EWS when considering the differential diagnosis of a highly aggressive appearing sinonasal mass. Additionally, this case demonstrates the radiologic-pathologic correlation required for a definitive diagnosis and for appropriate surgical and oncological treatment planning.