We report a case of conductive hearing loss caused by isolated congenital stapedial suprastructure fixation with normal footplate mobility. A 60-year-old woman visited the clinic for right-sided mixed hearing loss. Exploratory tympanotomy revealed a bony synostosis between the stapedial suprastructure and promontory, while all the ossicles were present and normally shaped. As the bony synostosis was separated, the stapes became mobile. This is the first report in the medical literature of this congenital ear anomaly. This case also illustrates that stapedial fixation can occur in the suprastructure as well as in the footplate; thus, one must be mindful of this when performing exploratory tympanotomy for stapes fixation. Congenital abnormalities of the ears are broadly classified into major and minor anomalies. While the major anomalies involve the external ears, the minor anomalies affect the middle ear structures only. Various minor anomalies have been reported, and several attempts have been made to classify them. The most widely accepted classification was proposed by Cremers and Teunissen based on the surgical findings of 104 minor anomalies, the largest number of cases reported to date . AccordiMany forms of minor anomalies exist. Here, we present a rare case of isolated stapedial suprastructure fixation with normal footplate mobility caused by bony synostosis between the posterior crura and promontory. This study was approved by the Institutional Review Board of the authors' institute (2021-02-023). To our knowledge, this is the first report of this entity in medical literature.A 60-year-old woman presented to our clinic for nonprogressive hearing loss in the right ear since childhood. There was no history of trauma, otitis, associated tinnitus, or vertigo. There was no history of previous ear surgery, and the family history was noncontributory. Examination revealed a normal-appearing external ear, external auditory canal, and tympanic membrane. A pure-tone audiogram showed a severe mixed hearing loss on the right, whereas the left ear showed a normal hearing level. Temporal bone computed tomography (CT) detected no abnormalities in the middle and inner ear.A clinical diagnosis of ossicular fixation was suspected, and an exploratory tympanotomy was conducted via the endaural approach under local anesthesia. No ossicles were absent or abnormally shaped . MalleusHowever, a pure-tone audiogram performed 2 months after surgery showed only slight changes Figures , and theThe present case demonstrated isolated congenital stapes ankylosis with suprastructure fixation. While all the middle ear structures were normally shaped, a bony synostosis between the posterior crura and promontory caused fixation. There have been a few presented cases of stapes ankylosis with stapes-promontory fixation, but the fixations were caused mainly by bony bridges between the structures , 5. OnlyA possible causal theory has been proposed based on stapedial development . During In cases of stapedial suprastructure fixation with a mobile footplate, division of the bony synostosis would be a treatment of choice for the correction of hearing. Indeed, there have been a few case reports of hearing improvement by simply disconnecting the bony bridge between the stapedial suprastructure and the surrounding structures , 3. In tThere have been several cases in which a clearly mobile stapes was confirmed during middle ear surgery, and temporary hearing gain was also achieved, but a bony fixation of the stapes necessitating a stapedectomy was found in revision surgery . It was This case report not only presents a new congenital middle ear anomaly but also suggests some important points when exploratory tympanotomy or any middle ear surgery for hearing correction are under consideration. In the present case, stapedial footplate fixation was strongly suspected before the first surgery for several reasons: (1) a Carhart notch in the audiogram, (2) normal shapes of middle ear structures on CT scan, (3) absence of previous ear surgery or infection history, and (4) the high incidence of stapedial footplate fixation among isolated middle ear anomalies . HoweverWe have presented a rare case of congenital stapedial suprastructure fixation with normal footplate mobility, which has never been reported in the literature. When performing exploratory tympanotomy for stapes fixation, one must keep in mind that fixation can occur in the suprastructure as well as in the stapedial footplate.


Neuroendocrine tumors (NETs) in the mediastinum are extremely rare. No uniform solution currently exists for the treatment of mediastinal NETs.We report the case of a 32-year-old man with symptoms of chest tightness, chest pain, cough, and panic.Computed tomography showed that the mediastinum and right lung occupied a space with uneven enhancement. A needle biopsy revealed mediastinal NETs. An atypical carcinoid was diagnosed using immunohistochemistry.The patient underwent 2 similar transarterial chemoembolizations of drug-eluting embolic microsphere procedures after 5 cycles of etoposide and cisplatin chemotherapy. The patient underwent successful surgical resection 2 months after the operation.The patient's quality of life was significantly improved, without chest tightness, chest pain, or other symptoms. At the 1-year follow-up, the patient had no tumor recurrence.For large mediastinal NETs with poor chemotherapy effects, surgical resection is safe and feasible after down-staging treatment via arterial chemoembolization of drug-eluting embolic microspheres. Here, a summary and analysis of clinical data is presented for a patient with a giant mediastinal NET that was successfully resected after the descending stage by drug-eluting embolic transcatheter arterial chemoembolization (DEE-TACE) had been performed. No tumor recurrence was observed at the 1-year follow-up after surgical resection.Neuroendocrine tumors (NETs) are thought to arise from cells throughout the diffuse endocrine system. NETs comprise a broad family of tumors, the most common of which are the gastrointestinal tract, lungs, thymus, and pancreas. Primary mediastinum is extremely rare.2A 32-year-old male patient was admitted with symptoms such as chest tightness, chest pain, cough, and panic. Computed tomography (CT) showed that the mediastinum and right lung occupied a space measuring approximately 11 cm × 17 cm, with uneven enhancement  catheter was used to examine the bronchial artery, internal thoracic artery, and phrenic artery for angiography Fig. . The resOne month after embolization, CT showed a considerable reduction in tumor size and blood supply. After a further round of DEE-TACE consolidation treatment had been completed, the CT scan showed a considerable reduction in tumor size 8.5 cm × 13 cm) and complete necrosis of the tumor  tumors that cannot be surgically removed and with locally advanced and distant metastases, a combination of systemic and local therapies should be adopted. In this case report, the tumor was considered to be at the G2 stage. An etoposide and cisplatin regimen was used for chemotherapy, but the effect was not good. Considering that the tumor was huge, surrounded by blood vessels, and difficult to surgically remove, embolization was decided to quickly reduce the tumor burden.NET mainly occurs in the stomach, intestine, and pancreas, but primary mediastinum is rare. Because the symptoms and signs of NET are not typical, their clinical manifestations are complex. Most patients have local spread or distant metastasis when diagnosed, and they lose the chance for radical surgery. Drug-eluting embolic microspheres can be loaded with chemotherapy drugs; therefore, they can embolize the tumor blood vessel bed and release drugs. According to the National Comprehensive Cancer Network guidelines for NETs, etoposide/cisplatin or etoposide/carboplatin are used as first-line drugs. There is no clear plan for second-line treatment, but fluorouracil or capecitabine combined with oxaliplatin or irinotecan can be considered. Furthermore, oxaliplatin is the only platinum drug that can be loaded with CalliSpheres microspheres. Microspheres that ranged from to 300 to 500 μm in diameter were chosen to avoid possible fistula or dangerous anastomosis.TACE has been previously used for the treatment of liver cancer, and this technology has been widely used in extrahepatic tumors.In conclusion, NETs originating from the mediastinum have rarely been reported. DEE-TACE downstaging treatment for mediastinal NETs may be safe and effective.Conceptualization: Zhen Li, Xinwei Han.Formal analysis: Zhen Li, Xinwei Han.Supervision: Zhen Li, Xinwei Han.Writing – original draft: Qi Yu, Zhen Li.Writing – review & editing: Zhen Li, Xinwei Han.


Dysembryoplastic neuroepithelial tumors (DNETs) are benign mixed glioneuronal neoplasms that frequently occur in children and young adults. We present the case of a 17-year-old male who arrived at the hospital following seizure-like activity. A magnetic resonance imaging (MRI) scan showed a 10 x 8 x 10 mm, oval-shaped, non-enhancing, well-defined mass within the right hippocampus. The patient underwent a transcortical approach via the middle temporal gyrus for resection of the mass; histopathological examination demonstrated the presence of round, uniform cells in an extensively myxoid background with diffuse reactivity to glial fibrillary acidic protein (GFAP). DNETs are considered benign, non-recurring lesions. Complete surgical resection is associated with a seizure-free outcome in 80% to 100% of cases. Dysembryoplastic neuroepithelial tumors (DNETs) are benign mixed glioneuronal neoplasms that frequently occur in children and young adults , especiaA 17-year-old adopted male with a history of bipolar and generalized anxiety disorders, on treatment with oxcarbazepine, presented to the hospital following a witnessed episode of generalized tonic-clonic seizures. He was reportedly confused for 20-30 minutes after regaining consciousness. He sustained right-sided head trauma with associated subconjunctival hemorrhage secondary to the fall and was transferred to an outside hospital where he had a second witnessed seizure. The patient was then transferred to our hospital for a higher level of care.Upon admission, he was oriented to time, place, and person. His blood pressure was 128/72 mmHg and oxygen saturation was 88% on room air. The rest of his vital signs were within normal limits. On neurologic examination, his speech was dysarthric, he had short-term memory loss, and a brisk right patellar reflex. The remainder of the initial examination was normal.Routine laboratory testing, including a complete blood count and a metabolic panel were normal. Ethanol, salicylate, and acetaminophen levels were negative. A serum oxcarbazepine level was 38 mcg/mL (reference range: negative).A brain magnetic resonance imaging (MRI) scan was obtained, which showed a 10 x 8 x 10 mm, oval-shaped, non-enhancing, well-defined mass within the body of the right hippocampus, causing superior displacement of the temporal horn of the lateral ventricle. A smaller second cystic lesion of 5 mm with similar characteristics was noted in the medial aspect of the inferior left temporal gyrus , suggesting DNET, World Health Organization (WHO) grade I Figure  as the dDNETs are low-grade, mixed neuronal-glial neoplasms that are common in the pediatric age group . They weDNETs usually manifest clinically as chronic, intractable, drug-resistant epilepsy before the age of 20 years and are considered the second most common type of pediatric epileptogenic tumors . These nThe tumors typically range from 10 to 25 mm in size and are usually located in the mesial temporal lobe, but frontal and parieto-occipital lobe lesions are also common [The absence of pathognomonic findings makes the DNET a challenging diagnosis. The differential diagnosis includes other glioneuronal or glial tumors such as gangliogliomas, oligodendrogliomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas . HoweverDNETs are considered benign, non-recurring mass lesions. They are one of the most common epileptogenic tumors in children and young adults. Usually, patients with DNETs present with chronic drug-resistant and partial complex seizures. One of the challenges for diagnosing DNETs is the absence of pathognomonic findings. This case report showed an atypical presentation due to the presence of asymmetric reflexes, which may be indicative of a possible mass lesion. Complete surgical resection is associated with a seizure-free outcome in 80% to 100% of cases, thus remaining the gold standard therapy.


Glomangioma is a benign tumor of mesenchymal origin, derived from the glomus body. It is responsible for the thermal regulation of the dermis. The occurrence of oncogenic osteomalacia related to glomangioma is rare. Only two cases have been reported thus far.A 32-year-old female, Brazilian, presented diffuse pain, during pregnancy, that developed progressively, limiting her mobility. Imaging showed a femoral neck fracture, and rheumatological laboratory examination showed hypophosphatemia. Also, the patient reported episodes of epistaxis during childhood and recurrence along with progressively right nasal obstruction. Endoscopic resection of the tumor was performed, and immunohistochemistry was conclusive for glomangioma. This case report describes the third case in which endonasal endoscopic surgery resulted in a favorable outcome.This case of glomangioma-induced oncogenic osteomalacia suggests that surgeons and clinicians should consider sinonasal tumors as a differential diagnosis of osteomalacia, and endonasal endoscopic surgery should be a possible curative resection. Glomangioma is a benign tumor of mesenchymal origin, derived from the glomus body, which is neural and muscular structure that helps to thermally regulate the dermis . It origIt is common in extremities such as the feet, toes, and palmar surface as well as the subungual and subcutaneous regions. Only 0.6% of all nonepithelial tumors of the nasal cavity, nasopharynx, and paranasal sinus result from glomangioma –4.About 500 cases of oncogenic osteomalacia (OO) have been described in the literature . OO rela2D In the right nasopharynx, somatostatin analog scintigraphy revealed an asymmetric increase in radiopharmaceutical concentration Fig. a. FurtheEndoscopic resection of tumor was performed with right maxillary, sphenoid, frontal, and ethmoid sinusotomy. Surgery occurred without complications. After surgery, the patient was administered antibiotics and nasal irrigation with saline solution, and did not exhibit nasal symptoms. Anatomopathological examination revealed fine-caliber ectatic vessels associated with elongated and spindle-shaped cells, arranged in diffuse beams and permeated Fig. . The immGlomangioma is a mesenchymal tumor that is derived from the glomus body and is present throughout the body. It represents hyperplasia or hamartomatous development of the glomus body. It is rare in the head and neck region. The first case of nasal glomangioma was reported in 1965. Since then, only 31 cases have been reported in the literature .Glomangioma presents as a reddish or purplish polypoid mass located in nasal cavity, paranasal sinuses, or nasal septum. It is less frequently present in ethmoid sinus and usually affects the elderly, although there have been reports in younger people, as in this case , 10. FemSymptoms include nasal obstruction, epistaxis, and pain . This paAbout 500 cases of OO have been described in the literature . It is rOsteomalacia is a disease of the bone metabolism that is characterized by defective mineralization of the bone matrix due to reduced phosphate levels. It presents with diffuse joint and bone pain, fractures, muscle wasting, limited mobility, weakness, and other nonspecific symptoms , 5, 8, 92D, which is required for enteral absorption of calcium and phosphate [In OO, fibroblast growth factor 23 (FGF23) is overexpressed by the tumor. It binds to the receptor on the proximal renal tubule and induces reduced expression of sodium phosphate co-carriers, leading to decreased renal phosphate reabsorption. It also inhibits the expression of 25-hydroxyvitamin D3 1-alpha hydroxylase, which results in inadequate production of 1,25(OH)hosphate , 9.Laboratory results of those with hypophosphatemia show normal or decreased calcium, reduction of 1,25-dihydroxyvitamin D3 with resistance to vitamin D supplementation and high alkaline phosphatase , 9.Due to slow and indolent growth, as in this case, the symptoms of a metabolic disease became more evident than the nasal ones. Most tumors are detected when they are relatively small. This patient presented with a tumor that was 1.5 × 2.0 × 5.1 cm in size. This filled about two-thirds of the nasal cavity, which differs from those previously described , 10, 11.The standard imaging examination for investigating OO and the tumor location is octreotide scintigraphy (Octreoscan). During this examination, the radiopharmaceutical binds to the somatostatin receptors that are expressed on the tumor surface , 8, 9.A complementary imaging examination that can be used is tomography with contrast-enhancing and magnetic resonance. This can be useful for therapeutic programming and defining the surgical approach .A definitive diagnosis was established by histopathological and immunohistochemistry . HistoloImmunohistochemistry positive for SMA defines the diagnosis as it is possible to distinguish from other nasal tumors such as hemangiopericytoma, olfactory neuroblastoma, nasal glioma, and embryonic rhabdomyosarcoma .The treatment is surgical and includes clinically normal tissue such as adjacent soft tissue. Most patients can be cured , 2, 10.In the described cases of OO-inducing mesenchymal sinus tumors, 12 of the 13 cases had been completely cured. We reported the third case in which endonasal endoscopic surgery resulted in a favorable outcome .Local recurrences have been described, although these have often been attributed to incomplete resection , 2, 10.2D [After exeresis, there is rapid clinical and laboratory recovery, with serum phosphorus returning to normal within the first 5 days in most patients. During this phase, the patient requires calcium supplementation to prevent hypocalcemia and hyperparathyroidism at a high level of 1,25(OH)The presentation of a nasal tumor associated with osteomalacia is challenging diagnostically owing to the tumors that cause this condition. It is particularly rare for glomangioma to also present in the sinonasal region.This case of glomangioma-induced osteomalacia suggests that surgeons and clinicians should consider sinonasal tumors as a differential diagnosis of osteomalacia.Complete remission of OO and the absence of tumor recurrence with endoscopic resection gives greater validation to this technique. This may be useful for planning the treatment approach in future cases.


This report involves a rare case of a 74-year-old man who presented with a progressively increasing swelling in the right groin, which represented a squamous cell bladder carcinoma herniating into the right inguinal canal. The manuscript discusses the role of multimodality imaging in bladder carcinoma presenting as an inguinoscrotal hernia. The patient subsequently underwent treatment with a chemotherapy regimen consisting of 5-fluorouracil and mitomycin, which was extrapolated from squamous cell carcinoma of the anal canal, and responded well. The most common risk factor is bladder outlet obstruction with chronic bladder distention and its contact with the inguinal canal. Risk is increased with advanced age, usually occurring after the fifth decade of life, probably related to decreased tone of the bladder wall; it tends to occur in obese men.2Urinary bladder hernia is uncommon, comprising less than 4% cases of all inguinal hernias.3 To the best of our knowledge, the available current literature does not mention squamous cell carcinoma (SCC) of the urinary bladder arising from the bladder hernia. In this article, we describe a rare case of an SCC of the urinary bladder presenting as an inguinoscrotal hernia.Urinary bladder malignancy within the inguinal hernia is extremely rare, with a few cases reported in the literature.A 74-year-old male, non-smoker with a past medical history of hypertension, benign prostate hypertrophy (BPH) and remote post-traumatic exploratory laparotomy presented to the emergency department with progressively increasing right groin pain and swelling. These symptoms had been present for 2 years, relieved by analgesic use, but had been exacerbated over the last 2 months. The patient had accompanying macroscopic haematuria and weight loss. Examination revealed a non-reducible, large painful mass palpable in the right groin.Computed tomography (CT) scan of the abdomen and pelvis demonstrated a 6.5 cm × 5.4 cm × 6.4 cm enhancing soft tissue mass in the right inguinal canal, inseparable from the anterolateral aspect of the urinary bladder with an accompanying hydrocoele . AdditioA cystoscopy was performed; however, the inguinal mass was not approachable. A trans-urethral biopsy of the enlarged prostate demonstrated moderately differentiated keratinising SCC with necrosis.A 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with CT (18F-FDG positron emission tomography [PET]/CT) scan demonstrated a hypermetabolic mass in the right inguinal region, with continuous extension into the urinary bladder lumen . HoweverTrans-urethral resection of the prostate demonstrated SCC that was unusual, but suggested submucosal infiltration of the primary bladder carcinoma. A modified treatment protocol extrapolated from treatment of SCC of the anal canal was utilised. The patient received radiation therapy and a chemotherapy regimen consisting of 5-fluorouricil and mitomycin.4 Three types of inguinal hernias of the urinary bladder have been described with regard to their relation to the peritoneum: (1) paraperitoneal hernia, in which the bladder remains extraperitoneal and medial to the peritoneal reflection, (2) intraperitoneal hernia, in which the bladder is completely covered by the peritoneum, and (3) extraperitoneal hernia, in which the peritoneum remains in the abdomen and the bladder alone herniates.5 Risk factors for inguinal bladder hernia include male gender, advanced age, chronic urinary obstruction and obesity.2 As with other hernias, bladder hernia occurs as a result of increased intra-abdominal pressure coupled with a loss of mechanical integrity of the muscles, tendons and other soft tissue structures.Most often, the urinary bladder herniates into the inguinal canal. The second most frequent location is through the femoral triangle, followed by less common sites including the obturator, perineal and umbilical regions.2 Although inconsistent, the classic Mery’s sign may be elicited, which is a two-step urination facilitated by applying pressure on the hernia, and disappearance of the hernia after voiding.4The majority of patients with inguinal bladder hernia remain asymptomatic, thus making clinical diagnosis challenging, and dependent upon imaging findings. Symptomatic patients may present with swelling in the inguinal region, dysuria, haematuria and urinary obstruction.2 Benign prostatic hyperplasia, hydronephrosis, vesicoureteric reflux and scrotal abscesses may be associated with bladder hernia.2Potential complications of bladder hernia are obstructive uropathy, incarceration, strangulation, bladder infarction secondary to incarceration and iatrogenic injury damage during hernia repair.6 Predisposing factors include those that cause urothelial injury, such as indwelling catheters, chronic inflammation, calculi, smoking and urinary tract infections.2 Squamous cell carcinoma of the urinary bladder is also more common in countries where Schistosoma haematobium is endemic. In such endemic regions, patients with SCC of the bladder are 10–20 years younger at presentation than patients with transitional cell carcinoma.7 Additionally, SCC related to schistosomiasis is usually well differentiated, presenting as an exophytic, nodular and fungating mass with relatively low propensity for lymph nodal and distant metastases compared to SCC not related to schistosomiasis.7 This is believed to be caused by lymphatic and capillary fibrosis due to chronic parasite infection.Squamous cell carcinoma accounts for 3% – 5% of bladder malignancies in Western countries. Our index patient demonstrated a rare case of SCC within a herniated bladder that also infiltrated the prostate gland.in situ and metastases.8 When a trans-urethral resection of a bladder tumour (TURBT) is feasible, complete resection of the tumour is attempted, whereas radical cystectomy is the standard curative treatment for patients with muscle invasion.8 Patients with metastatic bladder cancer or unresectable muscle invasive tumours are treated with chemotherapy.8Accurate staging of bladder cancer is crucial to optimise and prognosticate the individual patient. Treatment of the bladder cancer depends on the presence or absence of muscle invasion, carcinoma 9Because of the paucity of specific symptoms, clinical diagnosis of bladder hernia is challenging. Imaging is important to diagnose a bladder hernia and its associated pathologies. It is important to diagnose the contents pre-operatively to avoid the risk of injury during surgical repair. Inguinoscrotal bladder hernias are associated with emergency complications such as obstructive uropathy and bladder infarction because of an incarcerated hernia that requires subtotal cystectomy.10 Computed tomography urography provides information about the anatomy, as well as physiological function, and is usually performed as a three-phase examination. Three phases of acquisition include non-contrast, nephrographic and excretory phases after intravenous administration of iodinated contrast material. The non-contrast phase can detect high-attenuation blood clots, calcifications and calculi. Acquisition of the urinary bladder in the nephrographic phase allows visualisation of an enhancing bladder tumour, which is better delineated against low-attenuation urine. At the delayed excretory phase, the tumour appears as a filling defect and the tumour may be a polypoidal, plaque-like, infiltrative or diffuse lesion.10 Delayed excretory phase scans are superior in delineating the inguinal bladder hernia, which is continuous with the urinary bladder lumen. Alternatively, a direct CT cystogram can be performed; however, it is invasive and requires catheterisation.Computed tomography is the most common imaging modality performed for the detection and staging of bladder tumours.11 Muscular invasion is seen as interruption of the normal hypointense band of the detrusor muscle by an intermediate to high signal tumour on T2-weighted images. Magnetic resonance imaging has a 91% sensitivity and 96% specificity in differentiating ≤ T1 tumours (non-muscle invasive) from ≥ T2 tumours (muscle invasive) prior to surgery.11 Newer functional MRI techniques, such as diffusion-weighted imaging (DWI) and dynamic contrast-enhanced (DCE) imaging, increase the accuracy of local staging.Magnetic resonance imaging provides superior contrast resolution compared to CT, making it possible to delineate the tumour from the normal detrusor muscle of the bladder wall. The detrusor muscle layer is seen as a hypointense band against hyperintense urine and perivesical fat on T2-weighted images.11 Staging of pulmonary, liver and lymph nodal metastasis is usually performed with CT. Detection of the primary bladder tumour on 18F-FDG PET/CT is often difficult because of the intense accumulation of excreted FDG tracer in the urine.12 Similar problems exist when evaluating tumour developing within a bladder diverticulum or bladder hernia. A careful inspection of the non-diagnostic CT images may detect extravesical extension of the tumour, which indicates T3b disease.12 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with CT is most useful in the evaluation of lymph node or distant metastasis, and recurrence. Increased FDG tracer activity within the adjacent organs, such as the prostate, vagina, cervix, uterus and rectum, may help in establishing T4a disease. Computed tomography and MRI are also useful tools in monitoring the effects of treatment.13Both CT and MRI have low sensitivity in detecting abdominal or pelvic lymph node metastasis. Pelvic nodes greater than 8 mm and abdominal nodes greater than 10 mm should be regarded as pathologically enlarged.In our index patient, a biopsy during cystoscopy was not amenable because of the size and position of the hernia. Instead, a trans-urethral resection of the prostate with a biopsy was performed, revealing metastases from the SCC of the bladder.To the best of our knowledge, SCC of the urinary bladder hernia has not been previously reported. Imaging plays a vital role in diagnosis and management of such unusual presentations. The role of multimodality imaging techniques, as well as imaging features, has been described in this article.


Choroid plexus carcinoma is an intraventricular neoplasm originating from the choroid plexus epithelium and is of rare occurrence in adults. However, owing to the low prevalence of choroid plexus carcinoma, there is very limited information about the disease entity and treatment. Here we report a rare case of choroid plexus carcinoma in an adult patient.A 46-year-old South Korean (East Asian) male presented with low back pain, headache, and diplopia. Magnetic resonance imaging demonstrated enhancing mass lesion in the left trigone, cerebellar with leptomeningeal spread. Surgery was performed via left parietal craniotomy, and the lesion was histologically confirmed to be choroid plexus carcinoma. The patient received adjuvant craniospinal irradiation for remnant mass and leptomeningeal spread. Magnetic resonance imaging performed immediately after completion of the treatment revealed a partial decrease in the size of the tumor. However, the patient expired died as a result of acute respiratory distress syndrome before follow-up of long-term outcome.Choroid plexus carcinoma with leptomeningeal spread in adults is very important for rapid diagnosis and treatment. In the case of the presence of leptomeningeal spread, craniospinal irradiation can be considered as a treatment method, but may have serious complications. Hence, the technique should be applied with care. Choroid plexus carcinoma (CPC) is rare intraventricular neoplasm originating from the choroid plexus epithelium. CPC is categorized as grade III according to the 2016 World Health Organization (WHO) classification, which is based on histological characteristics , and it is the most aggressive among choroid plexus tumors (CPTs) –8. Here,18F-fluorodeoxyglucose positron emission tomography scan showed no hypermetabolic lesions, thereby ruling out suspicious primary neoplasms. The patient underwent surgery via left occipital craniotomy. The tumor was a mildly firm, pinkish mass with calcified multilobulations , which waived the requirement for informed consent. A 46-year-old South Korean (East Asian) man visited the hospital with back pain that started 3 months back. The patient had several supportive therapies for sustained low back pain at another hospital, but the treatments were not effective. When the patient first visited the hospital, he complained of headache, diplopia, and sensory change of right arm and face in addition to low back pain. Multiple nodular mass lesions were observed around cauda equina on spine magnetic resonance imaging (MRI) taken at the other hospital. MRI with gadolinium enhancement revealed heterogeneous enhancing masses in the left cerebrum, bilateral cerebellum, and whole spinal canal, in particular diffuse wall enhancement in posterior and temporal horn of left lateral ventricle. Diffusion and perfusion MRI showed increased cerebral blood flow and high cellularity in the left cerebral mass lesions  scan with contrast enhancement at the supine position. Craniospinal irradiation (CSI) treatment was planned divided into three parts: brain, upper spine, and lower spine. To minimize bone marrow suppression, we proceeded to the intensity-modulated radiation therapy (IMRT) plan Fig. . After tChoroid plexus carcinoma is a very rare cancer with a poor prognosis that occurs most commonly in children, with and the incidence in adults being much less . The patet al. reported that CSI has better overall survival and progression-free survival rate than whole-brain radiation therapy (WBRT) or involved-field radiation therapy (IFRT), with statistical significance [et al. concluded that CSI could be more effective than chemotherapy when leptomeningeal seeding is present. Even in our case, radiation therapy was found to be effective for CPC, but it is contemplated that further consideration of the optimal radiation dose and treatment field will be necessary [A few studies have reported that radiation therapy leads to better clinical outcome in patients with CPC , 11–13. ificance . Furtherecessary .Although the best chemotherapy regimen for CPC is still unknown, a report revealed long-term outcomes while applying temozolomide (TMZ) in CPC patient with MGMT methylation, while another report showed no effect of TMZ in the absence of MGMT methylation , 5, 14.


The mural thrombus in the ascending aorta is rare, most of which are associated with aneurysm or atherosclerotic lesions, with high risks of causing catastrophic thrombotic events. A mural thrombus in the non-aneurysmal and non-atherosclerotic ascending aorta is exceptionally uncommon.We reported a large mural thrombus in normal ascending aorta of an asymptomatic patient. Preoperative imaging confirmed the presence of the sessile thrombus located at the left anterior wall of ascending aorta. Given that it had the potential to cause fatal thrombotic complications, surgical removal and segment of ascending aorta replacement were executed. The patient had an uneventful recovery and discharged 14 days after surgery.Anticoagulant is the therapeutic cornerstone of ascending aortic thrombus, but surgery should be performed aggressively when the thrombus is large or floating to avoid severe embolic complications or recurrence. The mural thrombus in the ascending aorta(AA) is rare, most of which are associated with aneurysm or atherosclerotic lesions, with high risks of causing catastrophic thrombotic events, such as ischemic stroke, acute myocardial infarction and peripheral arterial embolism. A mural thrombus in the non-aneurysmal and non-atherosclerotic AA is exceptionally uncommon. Herein, we report an asymptomatic large mural thrombus in normal AA which was successfully removed by surgery.A 59-year-old man complaining about right lower limb pain and swelling was transferred to our institution for an ascending aortic mass which had been found incidentally in local hospital. Extremity ultrasound confirmed deep vein thrombosis in his right lower limb, which was responsible for his suffering. He had a past history of massive pulmonary embolisms, which were reduced obviously after 5-month course of oral anticoagulant. Except smoking, he had no other risk factors like hypertension, diabetes mellitus and dyslipidemia and he took no medication at that time. The thoracic contrast-enhanced computed tomography(CT) showed a low-density defect with no enhancement in the left anterior wall of AAFig. A and theGiven that the large thrombus in the AA with high risks of fatal thrombotic complications, surgical removal was executed. The surgery was performed through a median sternotomy and then cardiopulmonary bypass was established by cannulation of the right axillary artery and right atrium. After AA cross clamped, it was incised under cardiac arrest and a sessile mass located on the left anterior wall was identified Fig. A. The maMural thrombi in the non-aneurysmal and non-atherosclerotic AA are extremely rare. Although some of those have been reported without significant signs of aortic atherosclerosis on preoperative imaging, they have been found attached at atherosclerotic lesions after aortotomy , which wAs for diagnostic tools, contrast-enhanced CT should be considered as the first choice because it can clearly show whether the aorta has dissection, hematoma or atherosclerosis, and whether the mass displays enhancement. Moreover, the size and location of the mass can be visually seen on reconstructive images. In our case, since the asymptomatic patient was incidentally detected a sessile mass in his non-aneurysmal and non-atherosclerotic AA, the possibility of ascending aortic tumor(e.g. sarcoma) should be taken into account. Thus, we applied PET to exclude the malignancy and evaluate the necessity of surgery. Besides, although transesophageal echocardiography is effective for evaluating the ascending aortic thrombus, this invasive procedure is very uncomfortable for patients and related to possible complications such as gastrointestinal perforation and aspiration pneumonia , which iAt present, there is no corresponding guidelines or consensus on the treatment of the ascending aortic thrombus. There are two main treatment approaches: surgery and conservative therapy with anticoagulant. Endovascular treatment and thrombus aspiration have been also applied in recent years. Anticoagulant should be regarded as the therapeutic cornerstone whether surgery or not, especially appropriate for the patients with high risks of surgical intervention. Some researchers have reported good outcomes of anticoagulant therapy alone . HoweverWe presented a large and sessile mural thrombus in the normal AA of an asymptomatic patient. Ascending aortic thrombus should be taken into consideration when meet with a relatively young patient showing clinical manifestations of arterial embolism. Although anticoagulant therapy is generally recommended, we advocate that surgery should be performed aggressively when the thrombus is large or floating to avoid severe embolic complications or recurrence.


Fallopian tube torsion secondary to paraovarian or paratubal cyst is a rare gynecological cause of acute abdomen. The condition has no distinctive signs and symptoms. There are no characteristic features on radiological imaging, making preoperative diagnosis very difficult. Paraovarian cysts that are less than 4 cm in size are mostly asymptomatic and found incidentally during a pelvic examination or radiological imaging. It seldom leads to any complications like fallopian tube torsion hemorrhage or rupture. We report a case of an adolescent girl, who presented with severe abdominal pain. Transabdominal ultrasound was suggestive of a cystic structure less than 4 cm in size in the left adnexa. Doppler ultrasound showed normal blood flow to both ovaries. Diagnostic laparoscopy was performed, which revealed a twisted left-sided fallopian tube with a fimbrial paraovarian cyst. Detorsion and paraovarian cystectomy was performed. Although paraovarian cysts are mostly asymptomatic, those arising near the fimbrial end can lead to torsion of the fallopian tube, therefore it should always be considered a possible cause of acute abdomen in adolescent girls with adnexal cysts on ultrasound. Timely surgical intervention can prevent complications such as fallopian tube necrosis, gangrene, removal of the tube, and its long-term implications especially in women of the reproductive age group. Paraovarian cysts (POCs) also referred to as paratubal cyst or hydatid cyst of Morgagni, represent approximately 10% of adnexal masses . POCs caA 19-year-old virgo intacta presented in the gynecology outpatient department complaining of severe dysmenorrhea of less than 24 hours duration and her menstrual cycle had started the same day. Pain located in the lower abdomen was acute in onset. It was associated with nausea and three to four episodes of vomiting. She had no urinary or bowel symptoms. The menstrual flow was normal and she had always had a regular menstrual cycle with no dysmenorrhea in the past. On examination, she appeared dehydrated, her pulse was 78 beats per minute but feeble, blood pressure was 110/70 mmHg and temperature was 36.5 degrees celsius. On abdominal examination, she had tenderness on deep palpation in the hypogastrium. There was no rebound tenderness, guarding or abdominal rigidity, and gut sounds were audible. On admission, urinalysis showed signs of infection with numerous pus cells, bacteria, nitrite and ketones were positive. However, other tests such as blood leucocyte count, hemoglobin, C-reactive protein levels were within normal limits.Pelvic ultrasound showed a normal size anteverted uterus measuring 73 x 40 x 42 mm in size, with an endometrial thickness of 5 mm. There was a left POC measuring 30 mm in size and both ovaries were of normal size, which was separately visualized. Doppler study showed normal color and spectral flow in both ovaries making the possibility of ovarian torsion less likely Figure .A CT scan pelvis with contrast was carried out which showed a well-defined unilocular POC of 34 mm in size in the left adnexa, with normal-looking ovaries and other pelvic organs including the appendix Figure .The empirical intravenous antibiotic was started in view of urinary tract infection, after sending urine for culture and sensitivity. Despite giving painkillers according to the WHO pain ladder, the pain persisted along with retching and vomiting six hours after the admission. A working diagnosis of POC accident  was made and laparoscopy was performed. Laparoscopic findings were consistent with torsion of left fallopian tube with a unilocular fimbrial POC 4 cm in size approximately Figure .The fallopian tube was twisted once, it was detorted and a linear incision along the wall of the cyst was made with Ligasure® Figure . The POCFallopian tube torsion is a rare cause of severe pelvic pain and it is very difficult to diagnose preoperatively because of its less distinctive signs and symptoms. Unfortunately, there are no characteristic laboratory findings or radiological features that can help in differentiating this condition from others. Its incidence is estimated at one in 1,500,000 . The avaThe differential diagnosis of fallopian tube torsion includes acute appendicitis, ectopic pregnancy, pelvic inflammatory disease, twisted ovarian cyst, ovarian cyst rupture, ovarian cyst hemorrhage, hydrosalpinx, degenerative leiomyoma and acute diverticulitis . Many reThere are no guidelines available for the management of fallopian tube torsion hitherto. However, due to its presentation as acute pelvic or abdominal pain, the definitive diagnosis mostly is made with diagnostic laparoscopy or exploratory laparotomy. Laparoscopy, being the gold standard, is preferable where expertise is available, and the patient is hemodynamically stable. An additional factor is that the size of the cyst should be operable, according to the Royal College of Obstetricians and Gynecologists (RCOG) guidelines on the management of ovarian cysts in premenopausal women, cysts greater than 7 cm in size leads to increased chances of cyst rupture at the time of surgery . The benFallopian tube torsion and fimbrial POCs may be a rare entity but it is one of the most important causes of acute abdominal or pelvic pain in women in the reproductive age group, especially with evidence of adnexal cyst on pelvic ultrasound.Although less information is available so far regarding the long-term implications of salpingectomy on future fecundity, early surgical intervention in the form of detorsion of the fallopian tube and removal of the POC can prevent complications like necrosis, gangrene, rupture and the need for removal of the affected fallopian tube.


Echinococcus spp. larvae, is rare, but its treatment remains a significant medical challenge. Approximately 70% of patients with spinal hydatidosis have lesions in their liver, 0–15% have lung involvement, and only 0.5–2% have bone involvement.Spinal hydatidosis, a zoonotic disease caused by infection withHere we report a 38-year-old Iranian man with spinal hydatidosis, who had a history of eight times surgery in over of 26 years due to hydatid cyst in the liver, lungs, and chest wall. At the most recent admission to hospital he presented with chest pain, paraplegia, and urinary incontinence. Magnetic resonance imaging revealed thoracic spinal hydatid disease. He underwent surgery, and the hydatid cysts were completely removed. Lower extremity forces recovered dramatically and completely within 4 weeks.Spinal hydatidosis is a rare disease, but it is associated with a high degree of morbidity, mortality, and poor prognosis. Because of the infiltrative nature of hydatid disease, surgery alone is rarely curative. The current case study demonstrates the importance of a suitable surgical approach, adequate intraoperative prophylaxis to prevent cyst rupture, and prolonged complete paraplegia. Echinococcus granulosus sensu stricto is a parasitic zoonotic disease which mainly occurs in pastoral areas worldwide [Human cystic echinococcosis (CE), caused by the larval form of orldwide –3. Lesioorldwide . Bone inA 38-year-old Iranian man, residing in a rural area, was admitted to our surgery unit with a history of back pain, chest pain, paraplegia and urinary incontinence within the last 45 days. The patient had a history of hydatid cyst(s) in the liver, lungs, and chest wall. The first surgery occurred 26 years previously when he was 12 years old and underwent thoracotomy for two hydatid cysts in the left lung. Subsequently, in April 1999, the patient was diagnosed with a hydatid cyst in his left lung and underwent surgery again. Nine years later, after confirmation of a hydatid cyst in his left lung, he underwent thoracotomy. In May 2011, computerized tomography (CT) revealed multiple cysts located behind the left lung and the fourth rib (R4), leading to the diagnosis of paraspinal hydatidosis and a second thoracotomy. In May 2014, his imaging results demonstrated the presence of four hydatid cysts in the left hemithorax. Total cystectomy was performed for one cyst under the latissimus dorsi, and two cysts behind the third rib (R3) and one cyst behind his left clavicle were drained. In the same year, one hydatid cyst was detected in his liver, and hepatic resection was performed. In March 2018, imaging results demonstrated the presence of multiple cystic lesions under R3, R3 and the fifth rib (R5). Thoracotomy was once again performed, and the cystic lesions and the necks of R3, R4, and R5 were removed. After surgery, albendazole therapy (400 mg/kg) was initiated and continued.In December 2019 the patient was referred to our surgery unit with progressive weakness. Spinal magnetic resonance imaging (MRI) showed multiple spinal epidural cystic lesions at the level of the third to fourth thoracic vertebrae (T3–T4)  is the gold standard treatment in spinal hydatidosis . SeveralPrimary hydatid cysts usually contain daughter cysts, and the rupture of these daughter cysts can lead to secondary cysts . The risBecause of the invasive nature of hydatid disease, surgery alone is rarely curative. Therefore, a correct preoperative diagnosis, choosing the suitable surgical technique, considering the infiltrative nature of the cyst, intraoperative prophylaxis to reduce spillage, and posterior surgical approach for treatment are of crucial importance in preventing recurrence.


A cutaneous sinus tract of dental origin may easily be misdiagnosed and incorrectly treated. This paper reported a case of a 20‐year‐old male patient referred for a productive cutaneous sinus tract misdiagnosed by medical doctors for more than 4 years. The clinical and radiographic examinations confirmed the odontogenic origin related to a mandibular first right molar presenting an infected radicular cyst. Surgical treatment was performed leading to a significant healing of the sinus tract. Cutaneous sinus tracts of dental origin may easily be misdiagnosed. early and correct diagnosis of periapical root infections as well as their treatment can spare patients numerous other unnecessary treatments. Necrotic affected teeth exhibit apical periodontitis due to spread of infection into the periarticular area. The infection follows the path of least resistance between facial spaces, desiccate, and breakthrough the skin to form draining sinus tracts.A review of several reported cases showed that patients may undergo multiple unnecessary attempts of surgical excisions, drainage, biopsies, and long‐term use of antibiotics with no remission, due to improper diagnosis and the lack of treatment of the infectious dental origin.The aim of this paper was to present a case of a misdiagnosed cutaneous sinus tract related to an infected radicular cyst which was inappropriately treated for 4 years without any remission, and to highlight the importance of considering chronic dental infections as part of differential diagnosis for any orofacial skin lesion.2A healthy 20‐year‐old male patient was referred by a general practitioner to the department of dentistry at the university hospital Sahloul for cutaneous productive sinus tract mimicking acne.The patient reported a 4 years history of a drained and recurrent abscess in his right cheek that has previously been treated with different therapeutic procedure such as topical antibiotics and steroids  prescribed by his dermatologist; however, no response was noted.Besides, the patient did not report any history of dental pain or infection.The extra‐oral examination showed the persistence of a cutaneous fistula with a crusted appearance located on the lower part of right cheek measuring about 5 mm in diameter Figure . Gentle Palpation showed the presence of a cord‐like tissue that linked to fistula to the mandibular vestibular bone.The endobuccal examination revealed a poor oral hygiene and a decayed mandibular first molar (tooth 46) Figure .Pulp testing of the tooth 46 was negative while percussion, and periodontal probing revealed normal responses. No sign of mobility was present. Neighboring and contralateral teeth were also tested and were all within normal.Panoramic radiograph revealed a large radiolucency of 2 cm diameter with a well‐defined cortical border in relation with the roots of the tooth 46 Figure . A radioComputed tomography (CT) scan was indicated, and the axial slices confirmed the periapical lesion and showed a mixed radiolucent‐radiopaque appearance of the lesion, located on the apices of the tooth 46. It also revealed a local perforation on the buccal alveolar table in front of the same tooth  due to the radiographic appearance of the lesion.The extraction of the tooth 46 and the radicular cyst enucleation under local anesthesia was indicated. Thus, a mucoperiosteal flap was elected and revealed a cord‐like tract attached to the vestibular bone in the periapical region. The tooth 46 was extracted followed by the excision of the radicular cyst Figure . A meticAs for the tooth 47, pulp testing was positive, thus only control sessions were scheduled and the endodontic treatment was postponed.Histopathological examination of the lesion confirmed the diagnosis of odontogenic radicular cyst.Follow‐up was marked by the progressive healing to the cutaneous tract. At 1 month follow‐up, the lesion almost totally disappeared. Figure .3Cutaneous fistula of dental origin are uncommon lesions, but have been well documented in the literature. However, misdiagnosis and inappropriate treatment often arise.Due to their location on the head‐and‐neck region, odontogenic cutaneous fistulas are the interest of several medical specialties.Besides, in most cases, cutaneous sinus tracts of dental origin may not have any apparent dental symptoms and may progress over a long period of time without alarming the patient.The odontogenic cutaneous sinus tract on the oro‐cervicofacial region often develops as a result of chronic apical lesion caused by pulp degeneration or necrosis. The apical infection may spread through the narrow space and then perforate the cortical alveolar bone. In soft tissue, the infection may spread through the path of least resistance between facial spaces and finally perforate a mucosal or cutaneous surface.In this fact, when diagnosing and treating sinus tracts of unknown etiology in the facial and cervical area, dermatologist or plastic surgeon should always refer patients to the dentists to eliminate a possible dental infection.Such diagnostic and therapeutic misadventures highlight the importance of collaboration between medical and dental practitioners in the management of patients with head‐and‐neck lesions.Early diagnosis and appropriate treatment are essential. A proper diagnosis should include medical history of the patient, inspection, and palpation of the cutaneous lesion.Clinically, a cutaneous dental fistula has nonspecific skin manifestations and may resemble a pimple, ulcer, nodule, draining lesion, or indurated cystic area with purulent dischargeSamir et al described a classic cutaneous fistula with dental origin as an erythematous nodule of diameter up to 20 mm with or without drainage presenting skin retraction after healing.In this case, patient consulted for a suppurative cutaneous sinus tract with depression aspect in the right cheek with local alopecia of the whole area.Besides, intraoral examination may reveal one or several decayed teeth, a healthy‐looking tooth with an intact crown but an endodontic infection, or injured tooth.Pulp vitality test, percussion, and periodontal probing should be performed on the suspect tooth and adjacent teeth.Radiographic examinations, conventional or advanced imaging, should be indicated to identify a radiolucency at the periapical region of the suspected teeth.The indication of advanced 3D imaging is necessary, and patients should be evaluated using panoramic radiograph and cone‐beam computed tomography (CBCT) to evaluate the extend of the lesion and eventually confirm the causal tooth.In the present case, the tooth 46 was severely decayed with an infra gingival and juxta‐osseous dental tissue destruction, pulp testing was negative, but surprisingly the patient did not report any episodes of pain or discomfort.CBCT showed the existence of local perforation of the vestibular alveolar bone and a local bone sclerosis, which was also revealed by the conventional 2D radiography and which confirmed the chronic progressive evolution of the lesion.A possible diagnosis of infected radicular cyst was made. Differential diagnosis included infected cemento‐osseous dysplasia (COD), due to the radiographic findings which revealed mixed radiolucent‐radiopaque appearance of the lesion, located on the apices of the tooth 46.In some cases, the insertion of a probe or an endodontics gutta‐percha along the sinus tract is helpful for the ascertainment of the causal tooth,The differential diagnosis includes traumatic lesions, fungal and bacterial infections, pyogenic and foreign body granulomas, basal cell carcinomas, local skin infections such as carbuncle and infected epidermoid cysts, chronic tuberculosis lesion, osteomyelitis, actinomycosis, and gumma of tertiary syphilis. Rare entities to be included in the differential diagnosis are developmental defects of thyroglossal duct origin or branchial cleft, salivary gland and duct fistula, dacryocystitis, and suppurative lymphadenitis.The treatment of odontogenic cutaneous sinus tracts requires the elimination of the infection origin. Systemic antibiotic therapy was reported to result in a temporary reduction of the drainage and an apparent healing.However, the extraction, when indicated, or the conventional root‐canal treatment—when possible—is the treatment of choice.Antibiotics may be recommended as an adjunct to treatment in the setting of diabetes, immunosuppression, or systemic signs of infection such as fever, in fact systemic antibiotic administration is not indicated in patients with a cutaneous odontogenic sinus tract who have a competent immune system.After the eradication of the original source of infection, the sinus tract regularly disappears within 7–14 days after root‐canal treatment.In this case, the closure of sinus tract and the healing were seen 1 month later.Root‐canal therapy is the treatment of choice if the tooth is restorable. Once the tooth is treated, the need for surgical excision is controversial. Some reports have indicated a complete excision of the sinus tract lining, while others have suggested that surgical treatment and antibiotic therapy are not necessary after dental treatment.Root‐canal irrigation is a critical step on the success of root‐canal therapy because of the bactericidal action and elimination of necrotic tissue by the sodium hypochlorite. This step is usually followed by an intracanal medication with calcium hydroxide for its antimicrobial effects due to its high alkalinity; it has a destructive effect on cell membranes and protein structures, and stimulation of osseous repairClinical and radiological follow‐up should be regularly performed to detect the absence of healing and the persistence of periapical lesion.In this report, the extraction was indicated since that the tooth 46 was non‐restorable; thus, the extraction was associated with the enucleation of the radicular cyst and the simultaneous surgical excision of the sinus.The antibiotic indication, in this case, is due to the heaviness of surgery following cyst enucleation.As for the tooth 47, the radiographic investigations showed root resorption especially of the mesial one, but since pulp testing was positive, the endodontic treatment was postponed and control sessions were scheduled. The endodontic treatment should be performed as soon as any signs of infection or pulp necrosis are found.Finally, in some cases, plastic surgery may be needed at a later step if healing results in cutaneous retraction are disappointing.4This report highlights the importance of considering dental infection as a primary etiology in chronic cutaneous oro‐cervico‐facial sinus tracts.Early correct diagnosis based on radiographic evidence of a periapical root infection and treatment of these lesions can spare patient numerous unnecessary and ineffective antibiotic therapy or plastic surgical treatment, reducing the possibility of further complications.Authors declare no conflicts of interest.Latifa Hammouda and Dorsaf Touil have participated equally to the writing of the manuscript and have made the design, as well as the acquisition of data. Amira Kikly and Karim Jelassi have been involved in drafting the manuscript. Douki Nabiha contributed by revising critically for important intellectual content and has given final approval of the version to be published. All authors discussed the results and contributed to the final manuscript.A written consent has been obtained from the patient to write and to publish this report.


Intramuscular hemangiomas are rare benign tumors that are difficult to diagnose. We report a successful case of intramuscular hemangioma excision involving the external oblique muscles. The mass was excised successfully, and histopathology confirmed the diagnosis of intramuscular hemangioma with a negative margin and no malignancy. Intramuscular hemangiomas are rare benign tumors that are difficult to diagnose. We report a patient with a 3‐year history of a mass that was excised successfully. Histopathology confirmed the diagnosis of an intramuscular hemangioma. A literature review was performed using PubMed and Google Scholar search engines using the keywords “hemangioma,” “abdominal hemangioma,” “hemangioma and rectus,” and “intramuscular hemangioma.” No statistical analysis was required for this case report.33.1The patient was a 21‐year‐old man with no known medical illness other than a torn external adductor muscle in his left thigh 2 years previously that was treated conservatively. The patient had no surgical history. He presented to the general surgery clinic with a history of a mass that was neither growing nor painful and had been present for the past 3 years. Examination showed a soft, compressible, nonpulsatile mass with indistinct borders on the right flank.3.2Ultrasound (US) of the soft tissue Figure  revealedAbdominal MRI with contrast Figure  showed aA CT‐guided biopsy Figure  suggesteThe patient was informed of the results, and consent for surgery was obtained with an explanation of the risks and benefits of surgery as well as alternative treatments. It was explained that in case of muscle invasion and/or features of neoplastic lesions, a compartmental excision of the abdominal wall with reconstruction with alloplastic material might be needed.3.3The patient was placed in a supine position under general anesthesia. After preparing the abdomen and draping in the usual manner, we observed a lateral bulge on the right side of the abdominal wall, which the surgeon marked preoperatively. MRI showed that the mass extended from the lower edge of the costal margin to a few centimeters above the inguinal ligament (15 cm). The mass lay immediately below the external oblique muscle and aponeurosis. We decided to perform an oblique flank incision because the medial extension was only to the medial border of the rectus muscle.A 10‐cm incision was made. The fascia of Scarpa was incised and lateralized with skin elevation. The external oblique muscle and aponeurosis were then incised in the direction of the fibers. Minor bleeding was encountered in the highly vascularized tumor below the muscle. The external oblique was mobilized medially until the rectus muscle was observed. The rectus muscle was not invaded, and the mass was easily dissected.A ligature bipolar device was used for dissection and coagulation. The entire medial border of the tumor was easily removed. The caudal side was thin (spike‐like) and could be exposed and mobilized. The lateral side was somewhat difficult to distinguish and dissect from the internal oblique superficial fibers. We decided to remove some of the superficial fibers and dissect them anatomically. The upper border was freed from the lower edge of the costal margin.The specimen was retrieved and marked for histopathological examination .In our study, US of the mass showed a large echogenic solid lesion in the anterior abdominal wall on the right side. This was followed by MRI with contrast showing a large intramuscular mass in the right anterior abdominal wall composed predominantly of adipose tissue with internal linear and nodular high T2 components showing minimal diffusion restriction and enhancement, which are highly suspicious for well‐differentiated liposarcomas rather than lipomas. Therefore, CT‐guided biopsy was recommended to confirm the diagnosis of intramuscular hemangioma.Spontaneous resolution is unusual and may cause local destruction over time owing to the effect of pressure on nearby structures.5Intramuscular hemangioma is difficult to diagnose and its presentation is frequently unusual. We demonstrated that proper planning and imaging modalities are needed to successfully diagnose and manage intramuscular hemangiomas. Wide excision of the mass is required to decrease the risk of malignancy.No conflict of interest.The author confirms sole responsibility for the following: study conception and design, data collection, analysis and interpretation of results, and manuscript preparation.Written consent has been obtained from the patient.


Intestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. In pediatric age it is often associated with genetic syndromes such as Neurofibromatosis 1 (NF1), multiple endocrine neoplasia type 2B (MEN2B) and Cowden syndrome (PTEN mutation), and ganglioneuromas (GNs) may be sometimes the first sign of the disease. Isolated GNs are rare and sporadic. Clinical symptom vary and depend on the size and on the location of the GNs. This disorder affects intestinal motility and it, consequently, causes changes in bowel habits, abdominal pain, occlusive symptoms and rarely lower gastrointestinal bleeding secondary to ulceration of the intestinal mucosa. On the other hand, patients can remain asymptomatic for many years.We describe a 9-year-old boy referred to our emergency department for right lower quadrant abdominal pain. No familial history for gastrointestinal disorders. No history of fever or weight loss. At physical examination, he had diffused abdominal pain. Abdominal ultrasonography showed a hypoechoic formation measuring 41.8 mm by 35 mm in the right lower quadrant of the abdomen. Routine blood tests were normal, but fecal occult blood test was positive. Abdominal TC confirmed the hypodense formation, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum-last ileal loop. Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop. Laparoscopic resection of the polypoid lesion was performed. Histological diagnosis of the large neoplasm observed in the terminal ileum was diffuse ganglioneuromatosis. NF1, RET and PTEN gene tests resulted negative for specific mutations. At the 1 year follow-up, the patient presented good general condition and blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative.Only few cases are reported in literature of IG in pediatric age. Although rare, the present case suggests that this disorder must be taken in consideration in every patient with GI symptoms such as abdominal pain, constipation, lower intestinal bleeding, in order to avoid a delayed diagnosis.The online version contains supplementary material available at 10.1186/s13052-021-01024-5. Intestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. Ganglioneuromas (GNs) are hamartomatous tumors that originate in sympathetic ganglia and adrenal glands. More rarely, they occur in the gastrointestinal tract, originating in enteric nervous system cells . GNs areA 9-year-old boy was referred to our emergency department for acute and invalidant right lower quadrant abdominal pain. Family and physiological history were unremarkable. Because of the presence of lower gastrointestinal bleeding at 6 months of age, he started dietetic therapy with extensively hydrolyzed formula with good results until 18 months when he started a free diet. He had no history of fever, poor appetite, weight loss or constipation and diarrhea. Physical examination and vital parameters were normal, no lymphadenopathy or organomegaly. His abdomen was tender but he presented diffuse pain on abdominal palpation. For this reason, an abdominal ultrasonography was performed and it showed, in the right lower quadrant of the abdomen, edema of mesenteric tissues and of the last ileal loop, which was collapsed. Moreover, it revealed, an hypoechoic cecal polypoid lesion (41.8 mm by 35 mm) with intralesional vascularization , fecal occult blood tests (positive for blood), fecal calprotectin (200 μg/g) and an abdomen TC and colonoscopy were performed.The abdomen TC revealed an hypodense formation of the last ileal loop, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum. This formation presented inhomogeneous enhancement after contrast medium, highlighting numerous vascular poles. Inhomogeneous thickening of the contiguous loose cells was associated. The CT finding appeared suggestive of an expansive polypoid-like lesion on an invaginated loop Fig.  c and d.Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop Fig.  a, b, c.Microscopic examination revealed a transmural vaguely nodular proliferation, poorly demarcated, made of confluent mature ganglion cells and spindle cells with schwannian features. No mitosis were observed. Immunohistochemistry showed a strong staining of the ganglion cells for GFAP and S-100 and of spindle cells for S100. The neoplastic proliferation affected all layers from mucosa to muscularis propria effacing intestinal wall architecture. In the mucosa it caused expansion of lamina propria among crypts and was associated with focal pseudopyloric metaplasia. There wasn’t ulceration. Interspersed among neoplastic cells there were several eosinophils. Fig.  d and e.Specific systemic disorders associated with diffuse ganglioneuromatosis presenting characteristic clinical stigmata such as, mucosal neuromas and marfanoid habitus in MEN2B, cafe’-au-lait spots and cutaneous neurofibromas in NF1, or trichilemmomas and “cobblestone” tongue lesions as in Cowden syndrome, were absent in the present case. However, we referred the patient for genetic analysis of NF1, RET and PTEN genes and all resulted negative for specific mutations. At the last follow-up after one year, the patient appeared in good general condition. General physical examinations, blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative.Intestinal GNs is a rare form of benign neurogenic tumor characterized by hyperplasia of ganglion cell nerve fibers and supporting cells .According to WHO 2019 classification, intestinal ganglioneuroma can be broadly divided in 3 groups: solitary ganglioneuroma, a single mucosal polyp < 1–2 cm; ganglioneuromatous polyposis and diffuse ganglioneuromatosis. When presenting as a polyp, ganglioneuroma may histologically be reminiscent of juvenile polyp for cystic dilatation of glands.In this context, an accurate differential diagnosis appears fundamental. About the 70% of colonic polyps is represented by juvenile solitary polyps, not related to malignant transformation.This are reported meanly in children between 2 and 5 year of age with incidence from 0.08 to 3.7% . On the Polypoid GNs are usually adenomatous or hyperplastic small (< 2) juvenile tumors and can be either sessile or pedunculated in their morphology. Polypoid GNs can be solitary or come in small groups.In ganglioneuromatous polyposis, there are usually a large number of polyps – groups of 20 and above are common – which can be sessile or pedunculated. They range from 1 mm to 2.2 cm in size and are found in the mucosa and/or submucosa. Such polyps can be filiform projections containing ganglion cells, or they can be impossible to distinguish from polypoid GNs in histological terms, and they have been linked to cutaneous lipomas. The diffuse ganglioneuromatosis lesions can present tissue that is nodular and either mucosal or transmural, involving the mesenteric plexus. Such lesions can be much larger, reaching sizes of 17 cm. They present as projections or proliferations; either fusiform hyperplastic projections, or irregular transmural proliferations that merge with the myenteric plexus .Clinically, symptoms of GNs depend on the type of lesion, size and location. Polypoid GNs are typically asymptomatic or may present with abdominal pain, bleeding, obstruction, ileus, and constipation. Since the first report of GNs in 2000, only a few cases have been reported in the medical literature. Herein, we describe the clinicopathological features of this case for further understanding of the disease and review past cases in the literature Table .Table 1Indeed, Abraham et al. reported a case of a solitary colonic ganglioneuroma which presented with self-limited episodes of hematochezia due to the presence, on colonoscopy, of a 0.6 cm sessile polyp in the cecum, that produced a histological finding of ganglioneuroma .Moreover, Soccorso et al. described a case of a 5 year-old-girl, who referred to the emergency department, as in our case, with abdominal pain, bilious vomiting and bloody stools caused by colonic intussusception. This diagnosis was made with US scan and she recived immediately laparotomic surgical treatment. A GN centered in the muscularis propria was identified . MatthewRecently, another case of 11-year-old boy with isolated ileal ganglioneuromatosis has been described. His clinical symptoms were abdominal distension, vomiting, and severe constipation. The diagnosis required necessary histopathological findings such as an extensive proliferation of ganglion cells in the submucosa with numerous hypertrophied nerve bundles extended to the serosa. In this case surgical treatment has been chosen because it is well known that endoscopic treatment is often inconclusive [On the other side, MEN2B, NF1 and Cowden’s syndromes typically occur, associated with ganglioneuromatous polyposis and diffuse ganglioneuromatosis, rather than in association with isolated GNs.Gfroerer et al. described a case of an infant with intestinal ganglioneuromatosis associated with MEN2B and reviewed the literature focusing on gastrointestinal symptoms of MEN2B. The main symptom was severe constipation, leading to a colonoscopy. A rectal biopsy was performed, and a precocious diagnosis of GNs and subsequently of MEN2B syndrome at 6 months of age was made ..Nguyen et al., reported a case of isolated intestinal GN in association with MEN2B syndrome. In detail, the patient, at 10 years of age, presented melena and iron deficiency anemia that led to colonoscopy, which revealed three juvenile polyps that were removed, with subsequent improvement in the anemia. After 15 months, on a follow-up, colonoscopy showed a sessile polyp in the transverse colon, that presented histological characteristics of GN. Because of the presence of GN, the patient underwent a screening for MEN, that revealed a new polymorphism of the RET proto-oncogene .Furthermore, Thay et al. described in a patient suffering from NF1 a case of intestinal diffuse GN accidentally found during surgery to remove multiple malignant peripheral nerve sheath tumors arising in the retroperitoneum .Severe constipation has been described as predominant symptom also in a 6-years -old patient with diffuse ganglioneuromatosis associated to PTEN mutation. In this case it has been hypothesized that the GNs could be due to an altered proliferation of neural crest cells during embryonic growth of the enteric nervous system .Moreover, concerning the association between Cowden’s syndrome and GNs, Herranz Bachiller et al. reported a case of diffuse colonic ganglioneuromas which presented with rectal bleeding associated with esophagogastric polyposis and facial trichilemmomas. Genetic tests for MEN2B and PTEN were performed, that resulted negative for the former and pending for the latter. Clinical diagnosis of Cowden’s syndrome was made and treatment started, with close follow-up in order to prevent any incidence of the malignancy related to this disease , 18.Because GNs do not have endoscopic morphologically peculiar features, their diagnosis is usually made by microscopic and immunohistochemical examination of the biopsy that reveals immunoreactivity to S100 with the “comma-shaped” nuclei associated with aggregates of ganglion cells , 20.Moreover, in our case, abdominal ultrasound (US) has been a crucial examination because it showed immediately the presence of the polypoid lesion. In literature, various studies underline the importance of abdominal ultrasound in the possible detection of polypoid lesions localized in the colon if they present a diameter greater than 2 cm. Because children present small body habitus and less fat tissue in the abdominal wall and peritoneal cavity, US could be a potential useful tool in the diagnostic work-up for abdominal pain associated to alarm signs such as bleeding, obstruction and ileus . In addiUS cannot replace endoscopy in the diagnosis of colonic polyps, Moreover, this non-invasive instrumental examination could be useful also in patients with positive faecal tests, as in our case, because it could detect invaginated polypoid lesions that bleed because they are ulcerated ..This approach might be a good compromise in patient with moderate alarm signs olso in terms of cost/benefit perspective.Nowadays, no specific guidelines are available for treating GNs, and treatment of intestinal GNs depends on their size, location and associated symptoms. In the literature, some authors reported that solitary GNs were successfully treated by endoscopic complete resection , 23–25.Literature data about GNs in pediatric population are scarce. It is for this reason, our patient performed a follow-up with esophagogastroduodenoscopy, colonoscopy and MR-enterography but because GNs are a benign lesion with excellent prognosis, a follow-up with colonoscopy i unnecessary. In fact, Shekitka et al. reported a 8-year follow-up study of 28 patients with solitary GNs during which none of the patients developed NF1 disease, MEN2B, recurrence of GNs or other complications .In conclusion, in every patient with recurrent abdominal pain, not due to functional gastro-intestinal disorder, although rare, the diagnosis of GNs need to be considered. In presence of alarm signs an abdominal US could help to identify polypoids lesions located in the colon. So, appropriate laboratory examination followed by an abdominal US, could be the first steps in making an early diagnosis and performing a medical/surgical treatment of intestinal GNs.Additional file 1.


Sinonasal undifferentiated carcinoma (SNUC) is an extremely aggressive malignancy. Extension to the orbit and adjacent structures is common, but isolated visual loss as a presenting symptom is rare. We report a rare case of SNUC with bilateral visual loss as the initial manifestation. A 34-year-old gentleman was presented with acute onset loss of vision in both eyes for one week. It was followed by recurrent headaches and epistaxis. Visual acuity in the right eye was 2/60 and 3/60 in the left eye. Funduscopy showed a bilateral swollen disc. Neuroimaging revealed a large mass in the ethmoidal sinus extended laterally causing compression to recti muscles and the optic nerves. The histopathological examination of nasal tissue biopsy showed features of SNUC with bone and perineural invasion. A diagnosis of SNUC with bilateral compressive optic neuropathy was established. The patient underwent tumor debulking and base of skull reconstruction by the neurosurgical team. This was then followed by chemotherapy and radiotherapy. The patient’s right eye visual acuity initially improved to 6/9. However, his both eye vision developed into no light perception during treatment. In conclusion, SNUC is a highly aggressive tumor that may present with acute blindness. Early treatment may save a life, but the visual prognosis is guarded due to extensive optic nerve damage caused by tumor compression. Carcinomas of the nasal cavity and paranasal sinuses (PNS) account for 0.2%-0.8% of all malignant neoplasms . SinonasA 34-year-old gentleman was presented with acute loss of vision in both eyes and headache for one week. It was later followed by a few episodes of epistaxis. The patient had no nasal symptoms like anosmia, nasal congestion, or obstruction. There were also no ocular symptoms like proptosis, periorbital swelling, or diplopia. He did not have any fever, nausea, vomiting, or loss of weight and appetite. He was a non-smoker and had no family history of malignancy. He worked as navy personnel and denied any exposure to coal, coal, chromium, and nickel.On examination, the patient’s visual acuity in the right eye was 2/60 and 3/60 in the left eye. Optic nerve functions such as light brightness, red saturation, and color vision tests were reduced in both eyes. There were no limitations of extraocular muscle movement with no diplopia and proptosis. Fundoscopy showed a bilateral swollen disc with a normal macula reflex. As the patient had concomitant epistaxis, a rhinologist was consulted. The nasal-endoscopy assessment showed a polypoidal fleshy mass occupying both nasal cavities, obstructing the osteomeatal complex. Other cranial nerves and central nervous system examinations were normal. Systemic examination was unremarkable.Contrast-enhanced computed tomography (CECT) of the brain and PNS revealed a large lobular extra-axial lesion measuring 4.2 x 4.7 x 3.4 cm in the ethmoidal sinus with surrounding bony erosion. The mass extended to the extraconal compartments of both orbits with compression of both optic nerves and medial recti muscles, nasal cavity, sphenoid sinuses, and anterior base of the skull Figure . Biopsy 2 and 5-fluorouracil 750 mg/m2 on day 1 to day 5 of adjuvant were given for a total of three cycles. Just before his third cycle of chemotherapy, he complained of worsening right eye vision. CECT of the brain and orbit showed a significantly increasing size of the paranasal mass measuring 6.2 x 5.1 x 7.1 cm. The mass was invading the intraconal spaces bilaterally with compression to both optic nerves (Figure 2. During his recent follow-up, his visual acuity remained non-perceptive of light in both eyes with left eye proptosis. Fundoscopy showed bilateral optic atrophy. There was no other neurological problem.The patient underwent successful combined endoscopic and transcranial excision of the tumor. Debulking of the tumor was performed via bicoronal craniectomy with the base of skull reconstruction. Post-operative right eye vision improved to 6/9 but his left eye worsened to no light perception. Funduscopy showed resolving optic disc swelling in both eyes. He was planning for four cycles of chemotherapy. Intravenous cisplatin 75 mg/ms Figure . SubsequSinonasal undifferentiated carcinoma is a rare entity specific to the nasal cavities and paranasal sinus. It makes up <1% of all malignancies . A studyThe exact pathogenesis of SNUC is unknown. However, SNUC often originates from the Schneiderian epithelium, which lines the nasal cavity and the PNS . About 5The most common presenting symptoms of patients with SNUC include nasal obstruction, epistaxis, and facial pain, which were described in the previous studies ,12. A stDifferential diagnoses of bilateral optic disc swelling in young patients include infections, inflammatory, demyelinating, and neoplasm . In our SNUC is a rapidly growing and locally destructive lesion, which tends to extend orbitally and intracranially beyond usual anatomical limitations of the nasal cavity and PNS. Intensive multimodal treatment, including surgical resection and adjuvant therapy such as radiotherapy and chemotherapy treatment, is required in the treatment of SNUC. Surgery can be done preceding or after chemoradiation . RadicalThe overall five-year survival for SNUC is estimated at 22% and five-year distant metastasis-free survival is estimated at 35% . Some stSNUC may occur in young patients and usually presents with the advanced stage of the disease. Infiltration or direct compression of the optic nerve by the tumor may lead to a poor visual prognosis. Combined management by ophthalmology, rhinology, and neurosurgery teams is important. Although prognosis is guarded, timely treatment may halt disease progression, improve the survival rate, and reduce the risk of distant metastasis.


Morel-Lavallée lesions are posttraumatic, closed degloving injuries in which the skin and subcutaneous tissue are separated abruptly from superficial underlying fascia. This condition leads to an effusion containing hemolymph and necrotic fat. Magnetic resonance imaging, when available, is the modality of choice in the evaluation of Morel-Lavallée lesion. Early diagnosis and management is essential as any delay in diagnosis or missed lesion will lead to the effusion becoming infected or leading to extensive skin necrosis. We present a condition of a Morel-Lavallée lesion involving the scalp and complicated by conjunctival chemosis.We report on a 3-year-old black African girl who presented a fluctuant swelling of entire scalp, extending to upper part of the face on the seventh day after a forehead trauma due to falling on a rock while playing. Skull x-ray revealed soft-tissue swelling, giving an impression of large fluid collection in the deep subcutaneous tissues with no bone fracture. A diagnosis of Morel-Lavallée lesion of the scalp complicated by conjunctival chemosis was made. The patient was managed with percutaneous drainage and compression bandage. The patient improved well and was subsequently discharged without any vision impairment. There was no recurrence of the lesion on follow-up.The Morel-Lavallée lesion of the scalp complicated with conjunctival chemosis is a rare presentation of this condition. Prompt diagnosis and management are crucial for preventing complications. Image-guided diagnosis and treatment still remain a challenge in the setting of low-resource health facilities. Morel-Lavallée lesion (MLL), first described in 1853, is a closed degloving soft tissue injury, a result of abrupt separation of skin and subcutaneous tissue from the underlying fascia . InitialHere we report a rare presentation of MLL involving the scalp complicated by conjunctival chemosis in a 3-year-old girl. To our knowledge, this is the first case of MLL with eye involvement reported in the literature.3]. The diagnosis of Morel-Lavallée lesion with conjunctival chemosis was established. The patient received two pints of packed red blood cell (PRBC) transfusion and ceftriaxone 350 mg intravenously twice a day. She was also managed with percutaneous drainage and compression bandage  or anechoic (lymph-predominant) images depending on the age and predominant content of the lesion. A heterogeneous image with irregular margins and lobular shape may be seen in lesions less than 1 month old. For lesions older than 1 month, a homogeneous image with smooth margins and flat or fusiform shape may be seen. MRI scan shows soft-tissue contrast enhancement , 10. HenIn this case, the conjunctival chemosis and blurred vision may be explained by the conjunctival and probably corneal infiltration of swelling and the inflammatory reaction. To the best of our knowledge, this is the first case of MLL of scalp with eye involvement reported in the literature.This case was managed with percutaneous drainage and compression bandage. An antibiotic therapy was added to this approach. Currently, there is no accepted management approach for MLL. A variety of conservative and surgical options exist. This may be a stage-based or an individualized management approach. Objectives of management include drainage of the hemolymphatic liquid for resolving the contour deformity and allowing the apposition of lesion walls, debridement for removing necrotic tissues or mature capsule in chronic lesions, and meticulous dead space management to prevent recurrence by facilitating long-term adhesion of lesion walls either by fibrosis or using surgical apposition techniques , 12. In Recently, a new management algorithm was proposed and appeared to be a rational approach . It recoIn conclusion, MLL is a rare condition found after a trauma and can lead to infection, skin necrosis, or chronic encapsulated hemolymphatic liquid effusion. This is a rare presentation of MLL with conjunctival chemosis. Diagnosis of MLL can be made on the basis of clinical and radiological examination. Its diagnosis and treatment are often delayed because they involve internal degloving without surface penetration. Image-guided diagnosis and management is a challenge for health facilities in poor settings.


Leiomyosarcoma is an uncommon soft tissue sarcoma that composed of malignant mesenchymal cells with distinct features of the smooth muscle lineage. Typically affects the uterus and gastrointestinal tract, it can rarely be seen in large blood vessels, lymphatic and glandular duts, the mesentery, the omentum, retroperitoneum, and limbs. Occurrence is particularly rare in the limb region. Retrospective study based on patient records and postoperative pathological histological features. Four patients with limb leiomyosarcoma that were operated between 2016 and 2020 were included, three of them arising in the subcutis of the thigh region and one in cubitus. Extend resection with satisfactory outcomes is reported. Pathological examination showed that masses were composed of a fascicular arrangement of hyperchromatic spindle-shaped cells, characterized by the proliferation of epithelioid cells with eosinophilic cytoplasm for epithelioid leiomyosarcoma. Leiomyosarcomas that arise in the soft tissue, although rare, should be differentiated from other lesions, such as neurilemoma, neurofibroma, liomyoma,lipomyoma, synoviosarcoma, rhabdomyosarcoma, malignant fibrous histiotoma, and malignant neurinoma. Leiomyosarcoma is an uncommon soft tissue sarcoma composed of malignant mesenchymal cells showing distinct features of a smooth muscle lineage. It usually affects the uterus and gastrointestinal tract but that may be also found in less common sites including large blood vessels, lymphatic and glandular duts, the mesentery, onmentum, and retroperitoneum as well as the limbs. However, the limb region is a rare location . LeiomyoFour patients with limb leiomyosarcomas based on patient records and postoperative pathological histological features were included in this retrospective study between June 2016 and June 2020.There were 3 males and 1 female with an average age of 41.5 years (21–70 years). All the cases whose primary symptom was asymptomatic except slowly enlarging painless soft tissue masses. The course of the disease was 0.5 year to 2years.Three of them arising in the subcutis of the thigh region, one in cubitus. The masses range from 2 to 8 cm in diameter. Gross total extended resections with 3-5 cm of normal tissue around the tumor and wide negative margins by intraoperative frozen sections were given for all patients. All the patients were primary tumors. And one patient with pulmonary metastasis later was managed with chemotherapy. Follow-up times are from 12 to 36 months . All patThe postoperative course was uneventful, and functional exercises were progressively started with the help of rehabilitation therapist. Three of the patients had an uncomplicated recovery and showed no evidence of recurrence or metastases. The patient with pulmonary metastasis survived with 2 years of postoperation . TypicalMost leiomyosarcomas (LMS) are located in the mesentery, omentum majus, and retroperitoneum, with rare reports in the somatic soft tissue. LMS account for about 10%-15% of limb sarcomas, with predilection for the lower limbs, especially the proximal limb. This may be related to the abundant vasalium in the proximal limb . The incCausal factors have been identified for the etiology. A high rate of LMS in somatic soft tissues has been reported in acquired immunodeficiency syndrome (AIDS) and after kidney or liver transplantation. Smooth muscle cells infected by Epstein-Barr virus may be associated with tumor pathogenesis . HematogIconography examination plays an important role in diagnosing LMS of the soft tissue. MRI images show irregular masses with poor defined boundaries and no obvious capsule surrounding the lesion. Small areas of necrotic cystic degeneration can also be seen within the tumor. Most lesions show nonuniform equisignal on T1WI and patchy high signal on T2WI. Lesions show significantly inhomogeneous enhancement, with a patchy area of low signal and no obvious enhancement in the center of the lesion . CT imagAlthough imaging examination help for LMS identification in the soft tissue, definite diagnosis depends on postoperative pathological histological features. ELMS of the soft tissue composed of cells showing distinct features of the smooth muscle lineage, which have been characterized as intersecting, sharply marginated fascicles of spindle cells with abundant eosinophilic cytoplasm, and elongated and hyperchromatic nuclei . ProlifeLMS of the soft tissue should be differentiated from benign tumors such as neurilemomas and neurofibromas. If a benign tumor is misdiagnosed preoperatively, local recurrence can occur due to incomplete resection. This can be found to grow along the peripheral nerve and the well-defined margins of benign neurogenic tumors and moderately to significantly enhanced and capsular for the parenchyma of the LMS of the soft tissue, which may help to differentiated from benign tumors .Secondly, there is need to exclude important likely differential diagnosis of LMS in the soft tissue such as synoviosarcoma, rhabdomyosarcoma, malignant fibrous histiotoma, and malignant neurinoma. Synoviosarcoma mainly occurs in young adults (20 to 40 year olds). Localization of synoviosarcoma is mostly around the joints and is closely related to the joint capsule, peritendineum, and adjacent bone. The incidence of bone destruction is greater than 20%, which is higher than LMS in the soft tissue . RhabdomBesides, LMS should be differentiated from some other tumors of the limb region. Among the tumors of the limb region that can be confused with LMS are liomyoma and lipomyoma. We can find the difference between the LMS and liomyoma from the invasion, cell atypia, nuclear division, and focal necrosis. Lipomyoma is a common lobulated soft tissue benign tumor composed of mature adipocyte showing distinct features of the mesenchymal tissue.In conclusion, we describe four cases of LMS arising from the limb region. There are no clear diagnostic criteria for LMS, and diagnosis should be made from the microscopical and histopathological features as well as imaging examinations. LMS should be considered in the differential diagnosis of some lesions including neurilemoma, neurofibroma, liomyoma, lipomyoma, synoviosarcoma, rhabdomyosarcoma, malignant fibrous histiotoma, and malignant neurinoma. We emphasize the potential for malignant transformation of ELMS, highlighting the need for prompt extended resection and close follow-up in their management.