Superficial angiomyofibroblastomas are mesenchymal tumours that occur in thegenital tract and are well described pathologically. This case report reviewsthe imaging appearances and highlights the MRI findings, which have not beenpreviously described. We describe the occurrence of this lesion in a vaginalcyst, which to the authors’ knowledge, has also not been previouslydescribed. The histological findings are also presented here. Mesenchymal tumours of the female genital tract are well described pathologically butthe imaging findings are less well documented. We present a case of an unusualmesenchymal tumour of the female genital tract, highlight the key sonographic andMRI findings and summarise the current diagnostic and management approach.A 50-year-old female presented with a history of prolonged menstrual bleeding andright iliac fossa discomfort. Her past medical history of note includedendometriosis, a partially septate uterus and two previous lower segment cesareansections. She had no allergies and denied any relevant drug history such astamoxifen or hormonal therapy use.Initial pelvic ultrasound demonstrated normal uterus and ovaries, but detected a 13mm vascular soft tissue nodule in a 28 × 20 × 25 mm cystic lesion inthe left posterior vaginal fornix .The difThe patient proceeded to have a non-contrast enhanced MR study. This confirmed awell-defined, predominantly cystic, 24 × 31 × 24 mm structure in theleft vaginal fornix of mildly hyperintense signal on T2 weighted (T2W) images,intermediate to high signal on T1 weighted (T1W) images with a mild increase insignal intensity on T1W fat-suppressed images. The internal solid component was ofintermediate signal with a hyperintense rim on T2W images and low signal on T1Wimages. There was no signal suppression on the Short T1 Inversion Recovery (STIR)images to suggest a fatty component .The lesThe patient underwent total laparoscopic hysterectomy and bilateralsalpingo-oophorectomy. The mixed solid cystic vaginal lesion was excised at the sametime.Gross examination revealed a nodular well-circumscribed lesion measuring 16 ×12 × 14 mm and a small tissue fragment measuring 5 × 6 × 2 mm.Both specimens were submitted to the laboratory labeled as vaginal cyst.Microscopically, the small tissue fragment was a strip of tissue lined by stratifiedsquamous epithelium with little underlying stroma. The nodule was awell-circumscribed lesion comprised of predominantly bland spindle cells with paleeosinophilic cytoplasm and ill-defined cell borders, set in loose focally oedematousstroma containing abundant thin-walled vascular channels . There wImmunohistochemical analysis showed the tumour cells were positive for ER, PgR, CD34,Desmin, CD99, BCL-2 and negative for H-caldesmon, SMA, c-Kit, S100 protein, MNF116,HMB45 and melan A. Less than 2% of cells were positive for Ki67.The uterus and fallopian tubes were normal and both ovaries displayed cystic changes,but there was no evidence of malignancy. The accompanying peritoneal washings werealso negative.A histological diagnosis of superficial myofibroblastoma of the lower female genitaltract was made.Sonographic surveillance over a period of 8 years has not demonstrated localrecurrence.2 The tumours have been described in patients with a wide age range1 and there is at least one reported case of the tumour presenting in pregnancy.3Superficial myofibroblastomas of the female genital tract are rare tumours but arewell documented histologically. They preferentially occur in the lower femalegenital tract, with majority of cases in the vagina, cervix or vulva.2,4 The tumours also have a very characteristic immunohistochemical profile andare positive for oestrogen and progesterone, CD34, vimentin and desmin,2 but do not express smooth muscle actin, which distinguishes them from leiomyomas.5The classical histological description is of a tumour with bland spindle cells thatare weakly eosinophilic, set in loose stroma with multiple vascular channels,oedematous foci and scattered inflammatory cells including lymphocytes and macrophages.1 they can recur locally with one series demonstrating recurrence after aperiod of 9 years,6 therefore long-term follow up is advised.Although these tumours do not have any malignant potential,7 Ganesan et al postulated that tamoxifen may drive growth through the estrogenreceptor or alternatively, patients on tamoxifen therapy are likely to be morevigilant as they are under surveillance for endometrial changes, so increasing thedetection rate.1The aetiology of the tumours remains unclear. Approximately 94% of thereported cases (32 of 35 in 2010) occurred in peri-menopausal or menopausal females,and 32% of patients (11 of 35) had a history of tamoxifen or HRT use.8Mesenchymal tumours in other locations have been linked to viral infections but todate, only one study has addressed this possibility in myofibroblastomas of thefemale genital tract and revealed no association to human papilloma virus, humanherpes virus 8 or Epstein Barr virus.The imaging features have not been previously described in the medical literature.Sonographically, the tumour in our case was a hyperechoic solid mass with intenseintralesional vascularity and demonstrated slow interval growth . The sof7 It also makes the potential diagnosis of a simple endometriotic cyst unlikelyas these have been shown to be purely cystic with diffuse internal echoes and scantyvascularity on ultrasound.9 The polypoidal, superficial cervical and vaginal subtypes of endometriosishave a more solid soft tissue component, but their immunohistochemical profilediffers from that of the superficial myofibroblastoma.10The finding of intralesional vascularity excludes lesions such as Bartholin cysts,rectoceles or urethral diverticula.11 These include aggressive angiomyxoma, angiomyofibroma, fibroblastoma andcellular angiofibromas, as well as the tumour described in our case, superficialmyofibroblastoma. The second subset of tumours that frequently occur in this regionbut also occur elsewhere includes fibroepithelial stromal polyps, leiomyomas andsuperficial angiomyxomas.7There is considerable overlap in the imaging and histological features of many of themesenchymal tumours of the genital tract. The majority of diagnostic strategies areaimed at distinguishing lesions on the benign end of the spectrum from the moreaggressive angiomyxoma subtype, which is invasive and warrants a differentmanagement strategy. There is a subset of tumours that are relatively site-specificand arise from the superficial tissues of the genital tract.11 Cellular angiofibromas, also known as angiomyofibroblastoma-like tumour,present as well-circumscribed tumours of inhomogeneous echotexture but arerelatively isoechoic to surrounding subcutaneous fat. On MRI, the tumours areisointense or hypointense to muscle on T1W images, heterogeneous intermediate tohigh signal on T2W images with scattered low signal areas depending on the fibroustissue component, and enhance following administration of Gadolinium.12 The majority of the more aggressive angiomyxoma subtypes are well-defined,hypoechoic, cystic or multiseptate vascular masses on ultrasound, of high T2 signalon MRI and have a swirled or lamellate appearance.14Angiomyofibroblastoma subtypes are well-defined, homogeneous vascular lesions ofmedium echogenicity on ultrasound and are homogeneously low intensity solid masseson T2W images.6 Our patient has been free of recurrence for 8 years.Ultimately, definitive diagnosis requires surgical excision and careful histologicalexamination. Long-term imaging follow up is recommended, as there has been at leastone case of recurrence after a period of 9 years.We have presented a unique case of superficial myofibroblastoma of the lower genitaltract and discussed the imaging findings and main differential diagnosis.Superficial myofibroblastomas are mesenchymal tumours that occurpreferentially in the female genital tract.The less aggressive spectrum of tumours appear to have a more solid,hyperechoic appearance on ultrasound and characteristic MR features whencompared to the more aggressive angiomyxoma subtypes which may behypoechoic, cystic or septated; however, definitive diagnosis based onimaging alone is not possible and surgical resection is required.These tumours may be locally aggressive and recur locally; therefore,follow-up imaging is advised, although there is no consensus about theoptimum length of time at present.The authors declare no conflicts of interest and have obtained writteninformed consent from all parties involved in this publication.


Lumbosacral lipomas (LLs) may remain asymptomatic or lead to progressive neurological deterioration. However, sudden neurological deterioration is a rare and severe event. Herein, we report rare occurrences of sudden clinical deterioration in two previously asymptomatic children harbouring intradural LLs without dermal sinus tracts or signs of occult dysraphism. A review of the pertinent literature is also included.One child exhibited acute deterioration because of an epidural abscess associated with a filar lipoma without a sinus tract (probably caused by haematogenous spreading from a respiratory tract multiple infection), and the other child exhibited acute deterioration because of a very large, holocord syringomyelia-like cyst associated with a small conus lipoma. Both patients were 4 years old. In case #2, a previously undetected, severe tethered cord (conus at the S3-S4 level) was also present. A complete recovery was attained after an urgent surgical operation in both cases (in addition to targeted antibiotic therapy in case #1). All cases of deterioration in the literature were caused by abscess formation in dermal sinus tracts.Prophylactic surgery may be indicated even in asymptomatic children that have tethered cord and surgically favourable LLs , especially if the conditions are associated with progressive syringomyelia. Similarly, intradural dermal sinus tracts should be regarded as surgery-indicated, even if the conus is in its normal position and the patient is asymptomatic because there is a consistent risk of severe, infection-related complications. Finally, asymptomatic patients with filar LLs and a normally located conus can be candidates for surgery or an accurate clinical and radiological follow-up. Lumbosacral lipomas (LLs) usually accompany occult spinal dysraphism and are diagnosed in neonates/infants based on skin markers; they are occasionally diagnosed in children/young adults based on clinical deterioration , 2. LLs Herein, we report rare occurrences of sudden clinical deterioration in two previously asymptomatic children without signs of occult dysraphism. The goal is to describe a new possible clinical presentation of intradural LLs and to discuss the well-known dilemma of whether to operate on asymptomatic patients. In addition, the most recent and pertinent literature has been analysed.A 4-year-old boy was referred to our unit in August 2017 from another hospital because he developed sudden left lumbar cruralgia after a moderate back injury that occurred 2 weeks prior during a recreational activity. The child also had a fever, which started almost simultaneously with the head injury. The past medical history was unremarkable , with the exception of frequent episodes of respiratory tract infections.At the physical examination, the child was conscious and was complaining of lumbar pain radiating to the anterior thigh, palpation of the lumbar spine evoked the pain, no stiff neck was present, no skin markers were detected, and his body temperature was 38.5 °C. The neurological examination revealed no motor or sensory deficits, bladder disorders, or bowel disorders; however, the patient could not walk because of the intense pain. Once admitted, the child underwent spinal cord magnetic resonance imaging (MRI), which showed an intra- and extradural lesion extending from the lower L4 vertebra to the S2 vertebra, resulting in compression of the medullary conus and roots. The lesion appeared to be a fluid collection, with contrast enhancement, similar to an abscess Fig. . Blood lThe day after admission, a surgical excision of the lesion was performed through a L5-S1 laminectomy. A purulent collection that filled the epidural space was completely removed and sent for microbiological examination. At the S1 level, a partially collapsed lipoma of the filum that occupied the subdural space was progressively separated from the nerve roots under neurophysiological monitoring and excised by sectioning the terminal filum. The procedure was completed by duraplasty. The L5-S1 laminae were not replaced in order to leave the spinal cord decompressed. The regeneration properties of the bone at this age and the static behaviour of the sacral vertebrae are likely to close the bony gap, avoiding instability problems.A histological analysis of the surgical samples confirmed the diagnosis of a lipoma.Staphylococcus aureus, so a targeted antibiotic therapy was carried out for 4 weeks. Postoperative MRI (performed 1 month later) showed a normalization of the radiological picture  with severe deficits in dorsiflexion of the feet, a moderate deficit in trunk elevation, diffuse hypoesthesia of the lower limbs and urinary incontinence. The MRI scan of the spinal cord Fig.  documentThe child underwent surgery immediately after the MRI scan. Through an L5-S4 laminectomy, the dural sac was opened to explore and decompress the spinal cord. The small distal lipoma was dissected and removed, which immediately detethered the spinal cord.The diagnosis of a lipoma was confirmed by histological examination.The postoperative course was uneventful. The child showed a progressive improvement in both paraparesis and urinary incontinence, which were normalized after 3 weeks. The hypoesthesia significantly improved but still persisted in the 7-month follow-up. The postoperative MRI scan, performed 3 months after surgery, showed the detethering of the spinal cord and the significant reduction of the syringomyelia Fig. .Fig. 4MRThe management of asymptomatic children with LLs remains controversial. Some authors advocate routine surgery for all patients, regardless of the presence of symptoms or the patient’s age, to prevent neurological deterioration , 14. ForOn these grounds, it is clear that the main factor influencing the indication for surgery is neurological deterioration, which should be avoided in asymptomatic cases and reverted in symptomatic cases. The present experience raises the problem of the prevention of neurological deterioration in cases where it occurs suddenly and/or the lipoma could not be detected in advance. Actually, both of our patients showed a quickly deteriorating clinical course without any previous first signs. Instead, neurological deterioration usually occurs slowly and progressively, providing time to make the diagnosis or prepare a treatment. The main mechanism of deterioration is related to chronic ischaemic damage due to spinal cord tethering . The intIn case #1, the associated factor was a previous mild/moderate back injury. Therefore, one could speculate on the presence of a silent abscess (favoured by multiple respiratory infections) decompensated by the trauma. The abscess formation in an occult dysraphism is one of the most common precipitating factors reported in the literature (Table Staphylococcus aureus) secondary to dermal sinus tracts (with the inclusion of cysts in 50% of cases) have been reported in children; a good-to-excellent outcome was observed in only 60% of cases, and a significant mortality rate was reported (8%) [Independent of its causes, sudden neurological deterioration in patients with previously asymptomatic LL remains a rare but often severe and well-recognizable event. In case #2, the child experienced a quickly progressing paraparesis with bladder dysfunction. In cases of abscess formation, the clinical picture is even more severe because of the complications resulting from meningitis/myelitis, and the patients often show persistent deficits at a later follow-up Table . To dateted (8%) . A feverOn these grounds, two main issues seem to be relevant to minimizing the risk of neurological deterioration and improving its final outcome. The first issue is the diagnosis. To the best of our knowledge, we report here the first two cases of sudden deterioration in patients with truly “occult” LL , even though the presence of an abscessed epidural dermoid cyst cannot be theoretically excluded in case #1. Such a rare occurrence is the strength and, at the same time, the main limitation of this paper. As mentioned, the problem is not trivial because approximately one fourth of LL are without dural defects, though they can be associated with a tethered cord and/or syndrome . TherefoThe second issue is the management of asymptomatic patients. The management of children with complex LLs  and tethered cord remains inconclusive. Indeed, this point remains largely controversial, and it is very difficult to summarize the advantages and disadvantages of surgery from the literature due to the accumulation of heterogeneous data from case series concerning different types of treated lipomas, rates of preoperative symptomatic patients (progressive versus non-progressive), and outcomes of the different symptoms . Moreover, some authors are accustomed to operating on symptomatic patients only, while other authors include both asymptomatic and symptomatic patients in their series, preventing clear assessment of the rate of deterioration. Similarly, the surgical morbidity rate largely varies according to the type of the lipoma. It is widely accepted that lipomas with dural defects are burdened by a higher incidence of wound complications 10–20%), but these data are difficult to extrapolate from mixed series. On the other hand, the present experience reinforces the indication for surgery in asymptomatic children with noncomplex LLs and a tethered cord. An argument in favour of conservative management can be made based on the absence of symptoms in these instances. However, in our opinion, the low number surgical risks and the potentially severe complications in cases of sudden deterioration justify a more aggressive approach. Such an approach seems to be particularly appropriate when a progressive syringomyelia is evident on an MRI scan. Finally, intradural LLs with or without dermal sinus tracts and a normally located conus remain a matter of discussion. Because of the risk of deterioration even in cases of a simple lipoma of the filum  %, but th and the Sudden neurological deterioration is a rare but severe, adverse event that should be taken into consideration with LLs. The acute deterioration often results from infection-related complications of dermal sinus tracts, where the systemic component is predominant, and the neurological deficits can be absent. “True” sudden neurological deterioration remains very rare but raises the problem of prophylactic surgery in asymptomatic patients with radiological implications (a tethered cord and syringomyelia).


The nasopharyngeal angiofibroma (NA) is a benign tumor that originates from the pterygopalatine fossa and extends to the adjacent anatomical structures and affects frequently young individuals. The basic treatment for NA is surgical resection, but in some cases the tumor is surgically inaccessible.We describe the case of a 45-year-old male with respiratory difficulty after the appearance of a soft palate lesion. The clinical appearance of NA was not specific.There are still discussions about the best therapeutic strategy and controversies about performing incisional biopsy. To our knowledge, this is the first report of a NA in an adult patient in which the general health conditions prevented the therapeutic approach, besides the extension of the lesion and its complications.Our case shows that NA may reach high proportions and its involvement in older patients should be considered. In this report, we showed the limitation of the therapeutic strategy for advanced cases of NA. •A 45-year-old man with soft palate injury and respiratory distress.•Imaging examination showed involvement of the nasal cavity, nasopharynx and soft palate.•Initially, an intraoral incisional biopsy was performed under general anesthesia.•Surprisingly it was a nasopharyngeal angiofibroma.•General health status and extent of the lesion limited therapeutic intervention. The NA The diagnosis of NA is based on anamnesis, physical examination, and radiological and morpho-epidemiological findings, and diagnosing this condition is usually challenging . The iniWe report a case of NA in a man with an initial lesion in the hard palate, followed by respiratory difficulties. The present case has been reported in accordance with the SCARE criteria .2In March 2016, a 47-year-old male patient was admitted to the Oral and Maxillofacial Surgery and Traumatology service of a public hospital, referred by the medical team with the chief complaint of respiratory difficulty after the appearance of a soft palate lesion. On anamnesis, the patient reported rapid weight loss, drug use for more than 10 years, and the development of a hard palate lesion three years earlier, which was left untreated. In addition, the patient reported the development of a soft palate lesion one month earlier. Intraorally, an extensive mass of mucosa-like color was present in the palate region, which obstructed the oropharynx and consequently caused dyspnea.The sagittal computed tomography (CT) image reveals a soft tissue mass in the nasal cavity extending to the posterior portion of the nasopharynx and the lower part of the sphenoid sinus, with lowering of the entire musculature of the soft palate A. The coAfter the initial examinations, the patient was submitted to tracheostomy A and intThe patient was referred for excision of the lesion. However, due to the extension of the lesion and the patient's systemic conditions, the teams of different specialties decided not to perform surgical resection or radiotherapy, opting for embolization. However, as a technology procedure was not available at the time, the patient remained hospitalized awaiting treatment availability. Approximately one month after the incisional biopsy, bilateral swelling in the submandibular region and parapharyngeal regions was noted A and B. 3Diseases in the nasopharynx may be of different natures, such as benign or malignant neoplasms, and infectious or inflammatory diseases . The NA In the case presented, the clinical appearance of NA was not specific. Initially, the diagnostic hypothesis was malignant neoplasm . The NA manifests mainly in young men, probably due to increased circulating hormones ,5. The pImaging examinations have aided the clinical diagnosis of NA, whereas computed tomography assists in diagnosing bone changes, magnetic resonance imaging evaluates the tumor extension to soft tissues, and angiography estimates the vascular content of the lesion and arterial supply ,7,9,13. The pathological aspect of NA includes fibrous tissue composed of stellate and spindle cells, with varying degrees of vascularization ,16. The There is no consensus on the most appropriate therapeutic strategy for the treatment of NA in relation to morbidity and mortality ,13. In a4In summary, our case shows that NA may reach high proportions and its involvement in older patients should be considered, even though it is uncommon. In this report, we showed the limitation of the therapeutic strategy for advanced cases of NA in a compromised general health status. We reinforce the need for further unusual and complex cases to be reported, considering the rarity of this tumor.Ethical approval was not required and patient identifying knowledge was not presented in this report.There are no sponsors involved in the study.All authors contributed signiﬁcantly and in agreement with the content of the article. A.C. Vasconcelos, R.S. Caetano and J. Tramujas participated conducting the patient. S.A.J. de Freitas Filho, V.L. de Almeida and L.R. Paranhos participated in the process of writing of the paper. All authors have read and approved the final version of the manuscript.The authors declare that they have no conflicts of interest.Not applicable.Dr. Luiz Renato Paranhos.Written informed consent was obtained for publication of this case report.


Differentiated thyroid carcinoma with an extensive intravenous tumor thrombus which is extending into internal jugular vein (IJV), superior vena cava (SVC) and right atrium (RA) is a rare clinical finding. We report a multimodal staged surgical approach for this life threatening complicated case.A 52 year old female, presented with diffuse thyroid swelling, FNAC revealed it as follicular thyroid neoplasm. Computed tomography (CT) scan showed tumor thrombus extending into IJV, SVC and right atrium (RA). We planned complete resection of tumor in two stage operation. Initially in first stage, cardio-thoracic surgery was done to remove SVC and RA thrombus to eliminate the immediate risk of pulmonary embolism. In the second stage, neck surgery was performed to resect thyroid tumor and to perform extensive thrombectomy in the cervical veins. This patient has been followed for one year after successful surgery without recurrence.Venous involvement by follicular thyroid carcinoma reaching to RA is a rare life threatening condition. Though there is no standard guidelines available, treatment strategies should be discussed and planned among multidisciplinary team. Intraluminal extension is not a contraindication for aggressive surgical management. It will avoid fatal pulmonary embolism, as well as improve overall survival of the patient.Invasion of the great vessels by thyroid carcinoma is usually associated with early relapse and poor prognosis, but if tumor in the blood vessel is resected completely, a better prognosis is possible. Two staged surgical approach is safe and gives a predictable outcome. Here we2A 52-year-old female was referred to us with diffuse thyroid swelling since 8 months. The patient was not having dysphagia, dyspnoea or hoarseness of voice, with no significant medical illness in the past. Pertinent physical examination revealed a large lobulated mass on anterior aspect of the neck with separate fusiform swelling laterally in the right side of neck; initially it appeared to be a nodal metastasis. Her pulse was 88 beats/min, blood pressure was 136/78 mmHg and normal heart sound with no audible murmur. Fine-needle aspiration cytological examination revealed clusters of atypical follicular cells. The serum thyroglobulin level was elevated to 23,500 ng/ml. Axial post contrast computed tomography (CT) scan images of the neck demonstrated diffuse large thyroid growth involving both lobes and intra-venular extension of the malignant thrombus into the IJV, left innominate vein, SVC and reaching to the RA, occupying 80% of the total RA volume with suspicious adherence B. Though2D echo-cardiography for cardiac evaluation showed tumor thrombus formation as mentioned above. Surprisingly rests of the cardiac functions were normal.2.1Stage 1, Median sternotomy for removal of RA & SVC Thrombus. Stage 2, Total thyroidectomy and extensive thrombectomy of neck veins.Only few cases of thyroid carcinoma with an extensive tumor thrombus in the atrium have been reported in the literature. Yamagami et al. described a single staged approach in a similar case of thyroid carcinoma with RA thrombus . We antiStage 1:- To avoid the sudden death due to pulmonary embolism, it was decided to resect the intracardiac extension of the tumor before going to definitive neck surgery. Median sternotomy was performed and pericardium was opened. Patient was given heparin sodium 2 mg/kg before handling great vessels. Aortic cannulation was performed for cardio-pulmonary bypass purpose. Inferior vena cava (IVC) and right innominate vein was also cannulated with minimal manipulation of RA. Cardiopulmonary bypass was established under normothermia with IVC drain and ascending aortic perfusion. Patient’s body temperature was reduced to 22ºC and heart beating was arrested with cardioplegia. Cardiopulmonary bypass was stopped and Total circulatory arrest achieved. Right atriotomy was performed .2.2Cardiothoracic surgery part was done first followed by 4 days recovery in intensive care unit. Neck surgery was performed after 10 days of first surgery. Patient recovered without any complication and discharged from the hospital 7 days after the second surgery, total hospital stay was around 17 days.Patient followed up closely, and after 1 month, radio-Iodine isotope-scan followed by Radioactive-iodine-131 (RAI-131) ablation with 100 mCi dose was done. Patient was monitored with serum thyroglobulin levels every third month. Patient is asymptomatic with no evidence of recurrence on RAI scan at the end of 12 months follow-up.3Differentiated carcinoma of thyroid with haematogenus spread extending into the IJV is well reported entity , but sprInvasion of the great vessels by thyroid carcinoma is usually associated with early relapse and poor prognosis , but if Similar cases receiving only palliative treatment due to difficult surgical approach resulted in poor outcome ,17. TherTreatment strategies should be discussed among multi-disciplinary team . In the 4Tumor thrombus usually occurs in the advanced stage of the differentiated thyroid cancer and implies that such tumors behavior is aggressive with poor prognosis. Preoperative evaluation by appropriate imaging and collaborative multi-modality approach for treatment plan is recommended. In absence of distant metastasis, median sternotomy for tumor thrombus removal and radical surgery in the neck for thyroid cancer would be the treatment of choice. Surgical treatment is definitely useful to avoid sudden fatal tumor embolism and also to improve survival. In our case, staged surgical approach was found to be safe and effective. It avoided operative complexity and perioperative morbidity with better post operative outcome.No funding and sponsor associated with this case report.No ethical approval required to publish this case.Informed consent from the patient have been taken before sending for publication.1. Dr. Prashant Lad- Cntributed to operation, follow up and writing manuscript2. Dr. Jateendar Kumar- Cntributed to operation, follow up and writing manuscript3. Dr. Jagadish Sarvadnya - Cntributed to operation, follow up and writing manuscript4. Dr. Abhijith Powar - Cntributed to operation, follow up and writing manuscriptNone.Dr. Prashant P. Lad.Not commissioned, externally peer-reviewed.I have no conflicts of interest to disclose concerning this case report.


Posterior fossa craniotomy with stereotactic biopsy confirmed a locally invasive diffuse large B-cell lymphoma (DLBCL). Primary central nervous system lymphoma (PCNSL) arising from the 4th ventricle is a rare extranodal manifestation of non-Hodgkin lymphoma (NHL), with few cases documented in the literature. Review of available cases lends support that lymphoma arising from the 4th ventricle has a variable clinical presentation, occurs most commonly in immunocompetent males, and should be on the differential of any immunocompetent adult presenting with a posterior fossa mass. Optimal treatment modalities are based largely on phase 2 clinical trials, and recommended guidelines regardless of anatomic location should be adhered to.A 65-year-old male with a history of ischemic strokes, seizures, and subarachnoid hemorrhage presented with a 4-week history of progressive diplopia, vertigo, nausea, and vomiting. Magnetic resonance imaging (MRI) revealed a 2.5 × 1.8 × 1.7 cm posterior fossa mass arising from the roof of the 4 These symptoms were initially intermittent but had become unremitting during his initial presentation. The patient denied focal neurologic deficits, ataxia, hallucinations, headaches, fevers, chills, or night sweats. The patient underwent an MRI and magnetic resonance venography (MRV) upon seizure onset that revealed 2 areas of chronic hemorrhage but was otherwise unremarkable .On admission, vital signs were stable. Physical exam demonstrated rightward horizontal nystagmus, 20/40 visual acuity bilaterally, and subtle bilateral dysmetria on finger-to-nose test. A complete neurologic exam was otherwise normal. Labs were unremarkable.th ventricle , with plans for subsequent treatment with temozolomide and whole-brain radiation. After receiving his first dose of rituximab and methotrexate, he noted significant improvement in his symptoms. After his second cycle of Hyper-CVAD, repeat imaging showed resolution of the masses; he has been on single agent ibrutinib as maintenance therapy since and without recurrence for 10 months.th ventricular PCNSL indicates all patients were immunocompetent, had a median age of onset at 61-years-old, and had a male-to-female predominance (3.25:1) and intracranial metastatic disease in 35.3% of cases, with 50% of these documenting metastases to other ventricles , altered mental status (32-43%), symptoms related to elevated intracranial pressure  (32-33%), and seizures (11-14%) , 24. Imantricles  7–22].0%, alter.0%, alteth ventricular mass. Imaging may allude towards a PCNSL. CT may demonstrate hyper or isoattenuated lesions with marked contrast enhancement in immunocompetent PCNSL, whereas MRI may demonstrate isodense to hyperintense enhancement on T2-weighted imaging, with homogenous enhancement on postcontrast imaging [IGH, BCL6, and MYC compared to systemic lymphomas [PCNSL should be considered in any adult presenting with a 4 imaging , 26. In  imaging . Microsc imaging . The maj imaging . Immunoh imaging . PCNSL cymphomas .While treatment of PCNSL has evolved in the past decade, many recommendations are derived from phase II clinical trials. In general, high-dose methotrexate (HD-MTX) and rituximab are recommended for initial induction agents due to improved overall response rates (ORR) and progression-free survival (PFS) . Recentlth ventricular PCNSL, 35.3% of patients received ITC [th ventricular PCNSL had gross subtotal or total resection [Both intrathecal chemotherapy (ITC) and surgical resection have been studied for the treatment of PCNSL, though routine use of either modality remains controversial. Despite the concern that CSF can harbor lymphoma cells and contribute to treatment failure or disease relapse , there hived ITC , 14, 16,esection –17, 22. th ventricle is a rare occurrence that is seldom described in the literature. This case provides further evidence that PCNSL is often a malignancy of immunocompetent males. Due to its uncommon occurrence, it is unknown if commonly utilized treatment modalities that are employed in other anatomic variants of PCNSL will have similar impact on overall survival and progression-free survival. While surgical resection has been used in over half of 4th ventricular PCNSL, it is unknown how this impacted OS or PFS. Further research is needed to determine the optimal treatment for the many variants of PCNSL, which may vary by location or tumor subtype.PCNSL arising from the 4


An osteoid osteoma is a benign bone neoplasm with limited growth potential, characterized by significant nocturnal pain that usually responds to non-steroidal anti-inflammatory drugs (NSAIDs). The tumor may occur in any part of the skeleton, most commonly in the lower extremities and vertebrae.A 46-year-old female was diagnosed with a rare case of an intra-articular variant of osteoid osteoma, involving the articular eminence and glenoid fossa of the temporo-mandibular joint (TMJ).The tumor presented as a painful progressive swelling in the right pre-auricular area that had lasted for more than 2 years and which had been previously treated as a TMJ disorder. Computed tomography revealed a well-defined heterogeneous nidus involving the articular eminence of the TMJ. Surgical excision was performed and histological examination confirmed the diagnosis. In this article, in addition to describing the case, all cases of osteoid osteoma of the jaws and TMJ reported in the English-language literature are reviewed.Osteoid osteoma involving the TMJ is a rare presentation and may exhibit ambiguous symptoms, which could lead to misdiagnosis and delayed treatment. This tuHere, we present a case of osteoid osteoma of the temporo-mandibular joint (TMJ) involving the articular eminence, which, to our knowledge, has been rarely reported in the literature ,8.This work has been reported in line with the SCARE criteria .2A 46-year-old female presented to the morning consultation clinic of the Oral and Maxillofacial Surgery Department at our institute, complaining of progressive swelling and pain in her right pre-auricular area which had persisted for more than 2 years. The patient reported a history of traumatic incidents with a hard object in the same area, several months prior to the onset of symptoms. The intensity of pain had started to progressively increase, although it was partially responding to NSAIDs. The patient had undergone a TMJ washing procedure and had received an occlusal splint, but this had not alleviated the complaint.A physical examination revealed a 4 × 2.5 cm swelling with a faded border in the patient’s right TMJ region. The overlying skin was normal in color . The sweHistological examination demonstrated anastomosing trabeculae of osteoid and woven bone, lined by a rim of benign plumped osteoblasts in a loose fibrovascular stroma, and numerous multi-nucleated giant cells embedded in vascularized, fibrous connective tissue stroma . One wee3Osteoid osteoma is the third most-common benign bone tumor . AlthougWe conducted a search of the English-language literature held in the PubMed and ScienceDirect databases using the main search term “osteoid osteoma” and the additional search terms “maxillofacial”, “mandible”, “mandibular”, “TMJ”, “articular eminence”, “glenoid fossa”, “condyle”, “condylar”, and “jaw”. Following a detailed review of the literature, 27 reports of 29 cases of osteoid osteoma involving both the jaw and the TMJ were found and selected for this review , and areThe criteria for selection included initially the authors' designation of bone tumors involving the jawbones or the articular surfaces of the TMJ, that histologically consistent with osteoid osteoma, the typical feature of small sized painful lesions, and a radiographic picture of a nidus containing variable degrees of opacities that surrounded mostly by bony sclerosis.We found four cases (13.8%) of osteoid osteoma involving the maxilla; twenty-two cases (75.9%) which occurred in the mandible ; and only three cases (10.3%) that involved the articular eminence of the TMJ. The patients’ ages ranged from 4 to 77 years, and the majority of lesions occurred during patients’ second or third decades of life.The pathogenesis of osteoid osteoma remains controversial. Jaffe originally identified osteoid osteoma as a benign neoplasm . Some auTypically, osteoid osteoma is characterized by severe pain that gets worse at night and can be relieved by the use of NSAIDs .Some researchers have demonstrated high levels of inflammatory mediators in the nidus of these lesions, specifically prostaglandin E2 and prostacyclin, elucidating the characteristic nociception and rationale for the use of NSAIDs ,35. HoweGenerally, the intra-articular lesion is manifested as a diffuse joint pain that typically associated with a swelling and tenderness of the involved area, symptoms that might be falsely attributed to a more common intra-articular derangement, resulting in a possible delayed of diagnosis ,7. In acThe typical radiographic feature of osteoid osteoma is that of a nidus, with a variable degree of internal calcification and surrounding sclerosis . In the Histopathological findings showed that the nidus of the lesion consists of anastomosing trabeculae of woven bone with various degrees of mineralization, uniformly scattered within a loose vascular connective tissue, rimed by prominent osteoblasts, and often accompanied by numerous osteoclast-like giant cells. Those findings are consistent with the reported detailed histopathological studies of osteoid osteoma, which further demonstrated the prominent demarcation between the nidus and the surrounding extensive sclerosis of medullary bones .In differential diagnosis, considerations exist in differentiating between closely related benign osteoblastic tumours, osteoid osteoma and osteoblastoma. In the current WHO classification of bone and soft tissue tumors, osteoid osteoma and osteoblastoma are recognized as separate entities that share common features . Both siSurgical intervention remains the most commonly recommended treatment option for osteoid osteoma ,8,19,22.4Intra-articular Osteoid osteoma is a rare site of occurance, and usually exhibits nonspecific symptoms compared to those in other locations, leading to misdiagnosis and delayed treatment. In the TMJ, in the majority of reported cases, the lesion involved the condylar head among other articular surfaces. In this article, we have reported a case of intra-articular osteoid osteoma involving the articular eminence and glenoid fossa of the TMJ, which, to our knowledge, has been only reported few times in the literature. CT images were helpful in displaying the typical radiographic features and localization of the lesion. Surgical intervention was performed to excise the lesion, which resulted in the complete relief of symptoms and uneventful healing of the area. Histological examination confirmed the diagnosis of osteoid osteoma.All authors declare that there is no any sources of funding.Ethnical approval is exempt from my institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.HAA: Conceptualizing and writing of the paper.SAI and HAA: Assistant to writing of the manuscript and involved in care of patient.ASAA: Editing of manuscript.SAI and HAA: Main surgeon involved in care of patient and final editor of manuscript.NA.Ahmed AlsheweredNot commissioned, externally peer-reviewed.All authors declare that there is no any conflicts of interest.


A 6-year-old neutered male Yorkshire Terrier presented with recurrent pericardial effusion. Although clinical examinations including computed tomography were inconclusive, an exploratory thoracotomy revealed multiple small nodules and plaques on the inner surface of the pericardial sac (Day 1). A subtotal pericardiectomy was performed to prevent cardiac tamponade due to the increasing pericardial effusion, and the resected section of the pericardium was histopathologically diagnosed with mesothelioma. After surgery, chemotherapy with intrathoracic carboplatin was commenced. During the course of the treatment, a detailed follow-up ultrasonographic scan was performed to detect early lesions disseminated on the pleura, originating from the primary pericardial mesothelioma. On Day 101, the minute pleural nodules, which were disseminated lesions as predicted, were successfully imaged by ultrasonography. As the clinical stage advanced, the nodules were observed to gradually increase in size and number, implying tumor progression. These observations highlight the feasibility of ultrasonography in detecting minute disseminated lesions at an early stage, monitoring tumor progression, and thereby, predicting the prognosis of canine pericardial mesothelioma. Mesothelioma is a tumor of mesodermal origin that arises from the mesothelium, a monolayer of flattened epithelial cells lining the surface of the pericardial, pleural, and peritoneal cavities including the tunica vaginalis of the testes –3. This A neutered, male, 6-year-old Yorkshire Terrier weighing 1.76 kg was referred to the Animal Medical Center (AMC) at the Tokyo University of Agriculture and Technology with pericardial effusion of modified transudate. Recurrent effusion could not be controlled with standard treatment including antibiotics and steroids at a primary veterinary clinic.4 μl; reference range: 20–50 × 104 μl), and the plasma biochemical profile revealed mild azotemia  and moderately elevated levels of alanine transaminase . White blood cell count, plasma C-reactive protein, and albumin were within respective reference ranges. Serum cardiac troponin I  was also within normal range. From these results, inflammatory diseases, hypoalbuminemia, and uremia were unlikely to be the cause for effusion. The non-specific results of the blood tests warranted a more detailed examination by diagnostic imaging to search for the presence of any cardiovascular disorders or neoplastic diseases.At AMC, in order to detect the underlying cause of fluid production, detailed examinations, including physical examination, sphygmomanometry, complete blood count (CBC), plasma biochemical analysis, as well as radiography, and ultrasonography were performed. Physical examination was unremarkable. Blood pressure was relatively high on sphygmomanometry   imaging was performed under general anesthesia to exclude the possibility of underlying neoplastic disease within the overall pleural cavity. While CT images confirmed bilateral small to moderate fluid accumulation within the thoracic and pericardial cavities, there was no confirmatory evidence of malignancy as reported previously . For theDuring surgery, in addition to serosanguinous pleural effusion; multiple white, minute-to-small nodules and plaque lesions were observed on the inner surface of the pericardial sac . The out2, and gemcitabine at a dose of 2 mg/kg weekly was administered for 6 months. The dog died of progressive respiratory distress seven and a half months after the surgery; an autopsy was not performed as the owner opted against it.For a precise follow-up examination, especially for the evaluation of size and number of nodules, scanning was always performed in the standing position, and the scanning window was set on the right ventral region of the second intercostal space, where the minute lesion was initially identified. As the disease progressed, the nodules gradually increased in size and number regardless of the intermittent resolution of the pleural effusion by chemotherapy. At the advanced stage, the nodules coalesced with each other and covered the surface of the pleura, leading to pleural thickening . ScanninIn the management of thoracic mesothelioma, the evaluation of therapeutic effects in controlling the pleural and pericardial effusion as well as tumor progression is important. While the effusion may be easily detected by ultrasonography, precise assessment of tumor progression, particularly at an early stage, has never been conducted by ultrasonography, since the imaging modality often fails to identify minute lesions that grow and spread invasively along the thin layer of the mesothelium in the body cavity , 3, 5. ITo detect the minute tumor lesions on the parietal pleura, the presence of pleural effusion was important, to create an echo-free space between the parietal and visceral pleurae, thus providing a good contrast to the mass against the surrounding area. However, in the absence of pleural effusion, with the parietal and visceral pleurae in close contact, detection of the early minute lesions on the pleura was not possible due to the strong reflection on the surface of the lung rich in air. For the evaluation of tumor progression, detailed scanning of the parietal pleura by ultrasonography before the complete drainage of the fluid could improve the chances of detecting early minute lesions.In this case, the disseminated tumor on the parietal pleura exhibited a characteristic nodular appearance with dome-like or plaque-like shapes with homogeneous, low echogenicity on the inside. Interestingly, this appearance on ultrasonography was similar to that of human mesothelioma of the tunica vaginalis testis, where the hydrocele showed a good contrast against the small nodules attached to the surface of the tunica vaginalis . In contIn conclusion, this report highlights the feasibility of using ultrasonography in assessment of tumor progression in a case of canine pericardial mesothelioma. Considering that precise evaluation of tumor progression is of vital significance in formulating the therapeutic options, as well as for the prediction of prognosis, non-invasive ultrasonography could be a promising modality to serve the purpose.This study was carried out in accordance with the recommendations of the Guidelines by the Clinical Research Ethics Committee in the Tokyo University of Agriculture and Technology.Written informed consent for the publication of this case report was obtained from the owner. The report was written retrospectively on a patient treated with appropriate standards of care at a veterinary teaching hospital.RN contributed to writing the manuscript and literature review as well as collecting, interpreting, and describing the imaging findings. YN, SC, and HX assisted by performing the ultrasonography on the patient and collecting the data. T. Usui and KS contributed by collecting and evaluating the pathological data. TF contributed to the molecular pathological discussion and grant-related works. RF performed the described surgery on the patient and confirmed the macroscopic findings. T. Uchide contributed to writing the manuscript along with interpreting, and describing the imaging findings as the professional examiner who performed the ultrasonography on the patient.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


A seven-year-old Indian boy presented with a reddish mass over the outer part of right eye; the mass had been present for two months. It was associated with pain, discharge, and intermittent double vision. There was no history of ocular trauma, surgery, or any similar complaints. The patient was afebrile with no swelling over any other parts of the body. The ophthalmic examination revealed stretched right upper and lower eyelids, temporal displacement of the lateral canthus, and matting of the eyelashes. A well-defined, reddish yellow, oval mass measuring 15 The B-scan ultrasonography of the right orbit revealed a highly echogenic structure within a hypoechoic cystic lesion inside the lateral rectus muscle belly [Figure 2(a)]. The axial scans of computed tomography (CT) showed a hyperdense spec inside a hypodense lesion over the anterolateral part of the right globe [Figure 2(b)] and a bulky lateral rectus belly containing another heterogenous lesion [Figure 2(c)]. The brain CT showed a healed granuloma in the left parietal lobe [Figure 2(d)]. The history, clinical features, and radiological features were suggestive of right twin-orbital myocysticercosis with healed neurocysticercosis. On retrospective history, there was a contributory history of frequent consumption of smoked pork. There was no history of headache,     vomiting, or seizure, and neurology clearance was obtained before prescribing oral albendazole (15 mg/kg/day in two divided doses) and oral steroids (1 mg/kg/day). The enzyme-linked immune sorbent assay (ELISA) for cysticercosis and stool examination was negative.To prevent the spontaneous rupture and extrusion of the large, anterior, subconjunctival cyst, an “in-toto” surgical excision of the cyst was performed under general anesthesia. Intraoperatively, the lateral rectus muscle was hooked, and a gentle and blunt dissection was performed to remove the cyst in-toto [Figure 3(a)]. A reddish-yellow cystic mass (18 Taenia solium) into ophthalmic tissues is called ocular or adnexal cysticercosis. The larvae spread to ophthalmic tissues via the hematogenous route, and the involvement of extraocular muscles is known as orbital myocysticercosis. In adults, the orbital myocysticercosis may have an acute presentation akin to idiopathic orbital inflammatory disease (IOID), while in the younger age, it may present as an anteriorly prolapsed subconjunctival cyst.,2,3 In the latter scenario, there can be another “twin-cyst” or a continuation of posterior cyst in the associated rectus belly. Orbital imaging  may reveal the internal contents, extent, and association of cystic lesion inside the extraocular muscles or orbit.The infiltration of cysticerci (larvae of ,2,34]4] Traditionally, medical management is the first line of treatment for orbital myocysticercosis, but an anterior subconjunctival cyst, amenable to surgical excision, can be removed, preventing spontaneous inflammatory rupture of the cyst. Acute painful orbital conditions should be included in the differential diagnosis of the orbital myocysticercosis. Our case depicts a classical presentation of orbital myocysticercosis with a radiologically detected posterior “twin-cyst” and its successful surgical management. The authors also want to emphasize on the therapeutic medical management of orbital myocysticercosis in case of posteriorly placed orbital cysts.The extraocular muscles have a rich vascular supply favoring the lodgment of cysticercosis larvae.Sri Kanchi Sankara Health & Educational Foundation,India.There are no conflicts of interest.


Hepatic sclerosing hemangiomas are rare tumors whose appearance on imaging is similar to that of malignant tumors. The resulting difficulties in preoperative diagnosis frequently lead to surgical resection in order to accurately identify the tumor.A 68-year-old man was diagnosed with multiple gastric carcinoid tumors  after total gastrectomy with D2 lymph node dissection. Enhanced computed tomography of the abdomen 2 years after the initial surgery revealed a novel solid lesion with ring enhancement measuring 22 × 15 mm at S5 of the liver. Metachronous liver metastasis from multiple gastric carcinoid tumors was suspected, and partial hepatectomy of S5 was successfully performed. Histopathological diagnosis was sclerosing hemangioma with no malignant findings.Ours is the first reported case of a newly appearing sclerosing hemangioma excised and diagnosed histopathologically. Hepatic sclerosing hemangiomas are rarely observed, but they are often resected under a preoperative diagnosis of malignant lesion due to the similarity on imaging studies.Surgeons must consider that a hepatic tumor may be a sclerosing hemangioma, particularly when the lesion appears to be ring enhanced. They are rarely resected because of the characteristics on imaging studies. However, these lesions may alter into hepatic sclerosing hemangiomas when the hemangioma is denatured and replaced by connective tissue . On imag2A 68-year-old man underwent total gastrectomy with D2 lymph node dissection and cholecystectomy 2 years ago under the diagnosis of gastric carcinoid tumors. The pathological diagnosis was multiple gastric carcinoid tumors with lymph node metastasis  and normal gall bladder . The patLaparotomy was performed, and we confirmed that no other metastatic lesion was present by intraoperative exploration using ultrasonography. Following the ultrasonography-guided marking on the liver surface, S5 partial hepatectomy was successfully performed under total hepatic ischemia with the Pringle maneuver. The total ischemic time was 135 min, the operation time was 306 min, and the bleeding was 1320 ml. The resected specimen showed a 15 × 11 × 19 mm well-marginated nodular lesion with a mixture of whitish and reddish regions . HistopaThe postoperative course was uneventful, and the patient was discharged on postoperative day 19.3Hepatic hemangiomas are frequently observed benign lesions among the tumorous lesions occurring in the liver. They are classified into capillary hemangiomas, cavernous hemangiomas, and sclerosing hemangiomas . Small cGenerally, follow-up or surgical resection is the management techniques of choice for hepatic hemangiomas. Sakamoto et al. analyzed 510 cases of surgical resection of hepatic hemangiomas and stated that surgical indication can be stratified according to the diameter of the hemangioma [The most frequent distant metastatic organ from gastric carcinoid is liver. Treatment strategy depends on the intrahepatic distribution of the metastatic lesion. According to the ENETS Consensus Guideline, anatomical hepatectomy is recommended for the cases confined to one lobe or two adjacent segments . For casIn our case, we chose to resect the newly appeared liver mass during the postoperative follow-up after the multiple gastric carcinoid resections. To the best of our knowledge, there has been no such report previously. Hepatic sclerosing hemangiomas are difficult to diagnose because they shows findings similar to malignant lesions. Therefore, malignancy must be included in the differential diagnosis of hepatic lesions.The authors  declare no conflicts of interests or disclosures.This work received no funding.This study is exempt from ethical approval in our institution.Consent obtained.RK is the primary investigator and contributed to conceptualization, data collection and drafting the manuscript. NM, YM, TS, TH supervised and checked the manuscript. All authors have read and approved this manuscript for publicationNARyota KoyamaTomonori HamadaNot commissioned, externally peer-reviewed


Adenomatoid odontogenic tumor (AOT) is a relatively uncommon benign neoplasm of odontogenic epithelial origin, accounting for less than 5% of odontogenic tumors.  The reported case describes morphological characteristics, clinical course, radiographic and histopathological features, and surgical therapy of an extrafollicular variant of AOT in the maxillary posterior region. An asymptomatic swelling on the left side in the posterior region of the maxilla, gradually increased since approximately 12 months, developed in a 16-year-old Caucasian female patient. Radiographic images revealed a well-defined, unilocular radiolucency, with some small foci of radiopacity inside, and root resorption of the first and second molars. On the base of the histological examination of the specimen retrieved by incisional biopsy, the diagnosis of AOT was made, and the conservative surgical enucleation of the lesion was performed.  The present case was reported in agreement with an extensive review, in which it was recommended to discontinue reporting classic follicular cases because their clinicopathological profile was well-known, but to continue reporting well-documented cases of the extrafollicular variant, with indication of the exact position.  The present case was reported in order to expand the knowledge about the clinical behavior and surgical treatment of the extrafollicular variant of AOT. Adenomatoid odontogenic tumor (AOT) is a relatively uncommon benign neoplasm of odontogenic epithelial origin, accounting for less than 5% of odontogenic tumors –3.The currently used name of “adenomatoid odontogenic tumor” (AOT) was proposed by Philipsen et al.  in 1969 Later in the WHO third edition 2005) of Head and Neck Tumors, AOT was defined as an odontogenic tumor “composed of odontogenic epithelium in a variety of histoarchitectural patterns, embedded in a mature connective tissue stroma, and characterized by slow but progressive growth”  of Head .The reported case describes morphological characteristics, clinical course, radiographic and histopathological features, and surgical therapy of an extrafollicular AOT, which developed in the maxillary posterior region of a 16-year-old Caucasian female patient.A young patient  was referred to the Oral Surgery Unit of the Policlinico Umberto I Hospital–Sapienza University of Rome with the chief complaint of asymptomatic swelling in the left side in the posterior region of the maxilla, gradually increased to the present size of 3.5 cm since approximately 12 months.Medical history and extraoral examination were noncontributory, and there was no regional lymphadenopathy.Intraoral examination revealed, in the buccal fold of the left maxillary posterior region, a swelling extending from behind the canine up to the tuberosity, covered by normal oral mucosa . On palpThe involved teeth were sound, positive at cold sensitivity test, and without mobility.Panoramic radiograph showed in the left maxillary posterior region a well-defined, unilocular radiolucency, root resorption of the first and second molars, and presence of an unerupted third molar .Panorex view of the Computed Tomography (CT) revealed a hypodense intrabony, unilocular lesion circumscribed by radiopaque border, extending from the mesial margin of the first premolar to the distal margin of the second molar and apicocoronally from the sinus floor to the alveolar ridge. The resorption of the first molar roots and the second molar mesial root and the unerupted third molar not related to the lesion were also detectable .In the axial view of the CT, a limited expansion and thinning of the buccal and palatal cortical plates, limited cortical perforation in the vestibular wall upper the first molar, and small foci of radiopacity near the mesial root of the first molar were observed .The resorption of the first molar roots was also evident in the coronal view of the CT .Based on the clinical and radiographic findings, different pathologic conditions, such as dentigerous cyst, calcifying odontogenic cyst, odontogenic keratocyst, central giant cell granuloma, unicystic ameloblastoma, calcifying epithelial odontogenic tumor, ameloblastic fibroma, and ameloblastic fibroodontoma, were considered, and the preventive histological diagnosis was needed for treatment planning.Incisional biopsy was performed under local anesthesia .Histological examination showed a nodular proliferation of polyhedral epithelial cells of probable odontogenic origin, organized either in small cystic spaces with intraluminal basophilic PAS-positive material or in syncytial and trabecular nests. The stroma was poorly cellular, either intensely eosinophilic or amorphous like dentin, with some calcifications . A diagnConservative surgical enucleation of the lesion, extraction of the first molar, and apicectomy of the involved teeth, previously endodontically-treated, were planned.The surgery was performed under general anesthesia. Surgical access was obtained through a full-thickness trapezoidal intrasulcular buccal flap, extending from the mesial aspect of the canine to the distal aspect of the second molar . The flaAfter the first molar extraction, ostectomy was performed under sterile saline irrigation with a round tungsten carbide burr mounted on a low-speed handpiece used with a tangential movement not to involve the underlying lesion. The fibrotic capsule surrounding the tumor was dissected from the bony wall, and the mass was completely enucleated Figures .Before flap repositioning, amputation of the resorbed mesial root of the second molar, and apicectomy of the premolars and the remaining roots of the second molar, thorough debridement of the bony cavity and trimming of the rough bony edges were carried out.A releasing periosteal incision was made at the bottom of the buccal flap to enhance its elasticity and to achieve tension-free primary closure, the tumor cavity was packed with absorbable gelatin sponge , and the interrupted sutures were placed  .The surgical specimen was submitted for histopathological examination, and the diagnosis confirmed the previous incisional biopsy .Healing was uneventful without any complications and follow-up was performed at 3, 6, and 12 months.One year after the surgery, clinical examination and radiographs showed restitutio ad integrum both of the bone and the soft tissues and no local recurrence .An extrafollicular case of AOT in the posterior maxillary region was described, as suggested in the review by Philipsen et al. . The autAOT is relatively rare, with only 1558 cases reported in a total of 436 publications retrieved in a recent updated analysis of the literature undertaken without time restrictions up to July 2018 [AOT affects more frequently the second and third decade of life range: 1-83 years), female patients , and maxilla over the mandible ratio: 2.1 : 1), with a preference for the anterior portion of the jaws [-83 years.1 : 1, wAOT has three distinct clinicopathological variants without any relevant clinical and radiographic differences between them: intraosseous follicular , related to retained tooth, most often a canine; intraosseous extrafollicular (24%), which is subdivided into E1 no relation to tooth structure either erupted or unerupted, E2 intraradicular adjacent roots, E3 superimposed on the root apex, and E4 superimposed on the midroot level; and peripheral (3%), in the soft tissue overlying the tooth-bearing area , 6, 8–10For the follicular variant, there is histologic and immunohistochemical evidence that it arises from the reduced enamel epithelium of the dental follicle, whereas origin of the extrafollicular variant is less clear , 12. PhiThe lesion ranges from 2 to 7 cm in size, has a slow growing pattern which can result in a painless expansion of the jaws, and often leads to the displacement of the adjacent teeth, whereas root resorption is more rare , 10. TheIn this report sex, age, and clinical and histopathologic appearance were consistent with the literature, but the location in the posterior region of the maxillary jaw and some features, such as root resorption, cortical perforation, and small radiopaque foci inside the radiolucent lesions, were less frequent.Conservative surgical approach with complete enucleation of the lesion is the treatment of choice, justified by the benign behavior, presence of clear demarcation, and low rates of recurrence , 14.In the present case, in addition to excision of the lesion, surgical management required the extraction of the first upper molar, mesial root amputation of the second molar, and apicectomy of the premolars and the remaining roots of the second molar.The present case was reported to increase the knowledge about morphological characteristics, clinical course, radiographic and histopathological features, and surgical therapy of an extrafollicular AOT arising in the posterior maxilla.


Review of the first documented case of aortic wall metastasis from a limb sarcoma.In a 56-year-old woman with a diagnosis of a high-grade limb fibrosarcoma, an aortic metastasis was revealed by a fast growing aneurysm of the descending thoracic aorta. This was managed with an endoprosthesis.The presence of an aneurysm in a patient with a sarcoma with a high potential for metastasis and poor cardiovascular risk factors should alert physicians. Soft-tissue sarcoma is a rare entity . Tumors spread primarily through the vascular system, and lungs are the most common site for distant metastasis. To our knowledge, an aortic metastasis of a sarcoma has not yet been described as primary intimal sarcomas are most commonly reported in the literature . In thisA 56-year-old nonsmoking woman consulted for a painful mass in her left thigh. Her past medical history was only significant for untreated autoimmune hepatitis. MRI of her left thigh showed a posterolateral muscular mass measuring five centimeters, with both necrotic and enhanced portions Fig. a. Histol® Bolton Medical). The presence of an atypical epigastric artery angiogram led to an artery biopsy during the procedure, which found no evidence of dysplasia. Despite negative blood samples taken near the aneurysm, the possibility of an infectious location was discussed due to the patient’s poor dental condition. Postoperative probabilistic antibiotic treatment was started with oxacillin and ofloxacin, and then amoxicillin.A thoracoabdominal CT scan was performed for tumor staging and found a 35-mm saccular aneurysm of the descending thoracic aorta Fig. b–d. The Surgical resection of the primary tumor was rejected due to histological confirmation of a right gluteal metastasis . Initially, the patient received conventional chemotherapy by doxorubicin and ifosfamide, but the treatment was quickly switched to cisplatin and paclitaxel due to tumor progression. The patient was then included in a clinical trial (NCT01308034 Study of Continuous Dosing of Sunitinib in Non GIST Sarcomas with Concomitant Radiotherapy) and treatment by sunitinib was introduced with concomitant radiotherapy. Due to adverse effects that led to a deep thrombocytopenia (50 G/L), sunitinib was stopped.Follow up CT scans Fig. a–c showeThe luminal-type intimal sarcoma (IS) is the most common sarcoma of the aorta . In the ) and malignant. This classification helps physicians adapt treatment according to the risk of tumor metastasis. There are no current guidelines regarding initial tumor staging, but the WHO clearly recommends multidisciplinary decision-making. Sarcomas spread primarily through the vascular system, and the lungs are the most common site for distant metastasis. Patients at high risk of metastasis usually have an initial chest CT scan; a thoracoabdominal CT scan may be also performed.Sarcoma survival rates are closely linked to their potential metastasis risk. Since 2002, the World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone divided these tumors into three risk groups: benign, intermediate . Primary aortic sarcomas are rare tumors, with approximately 30 cases reported in the literature .Typically, patients with aortic intimal sarcomas have symptoms caused by tumor emboli including pulseless extremities or abdominal pain, rather than symptoms directly related to the primary tumor . In our [18F]-2-Fluorodeoxyglucose (FDG) is the most commonly used tracer for PET imaging. This is a glucose analog that accumulates in cells and FDG uptake reflects tissue metabolic activity. In oncology, it is used to detect metastases by virtue of their metabolic differences compared to surrounding normal tissue. False positives commonly occur with active inflammatory or infectious lesions and false negatives occur with low metabolic activity tumors or small lesions (< 1 cm), due to limited resolution. In our case, PET-CT was used in conjunction with initial CT scan as a complementary diagnostic tool and showed intense FDG uptake around the aneurysm. However, in the case of rapidly evolving atherosclerotic aneurysms, FDG uptake can also be seen. An aortic metastatic localization was not initially considered to be the most likely diagnosis in our case. Follow-up CT scans and MRI findings found enhanced tissues around the aneurysm.The endovascular treatment of aortic aneurysms is now the gold standard to avoid the risk of rupture beyond 5 cm. In this case, simple monitoring was impossible in view of rapid progression, and the multidisciplinary team judged open surgery to be inappropriate and too invasive.To our knowledge, an aortic metastasis of a sarcoma has not yet been reported in the literature. Immunohistochemical tests are needed to discriminate primary intimal sarcomas from other aortic tumors. Indeed, intimal sarcomas do not express epithelial, smooth muscle, or schwannoma-like markers and are positive for mesenchymal marker, vimentin. In our case, pan cytokeratin and smooth muscle actin markers were positive, similar to the primitive limb sarcoma.Aortic metastases of limb sarcoma are rare. In the early stages, tumor aneurysms are very similar to atherosclerotic aneurysms on CT findings, which often lead to a delay in diagnosis. The atypical localization of the aneurysm, low cardiovascular risk factors, rapidly evolving aneurysm and enhanced tissues around the aorta are suspicious findings for aortic metastasis. FDG uptake around the aneurysm, although not specific, can contribute to the diagnosis.


Emphysematous aortitis is a rare condition that manifests through the presence of air within or surrounding the inflamed aorta. Aortic inflammation may result from either an infective or a non-infective cause. Recognition of this rare condition is important as the resultant clinical deterioration may be rapid and lead to inevitable death. Cross-sectional imaging, with its relatively wide availability, proves an important tool in the diagnosis and subsequent management of this condition. We report two such cases encountered in our centre, with particular focus on the imaging findings on CT. However, certain conditions may predispose the aorta to infection; these include atherosclerotic disease, a pre-existing aneurysm, diabetes and other immune-susceptible states and the presence of medical devices or open surgery.3 A wide spectrum of causative organisms may be found including Staphylococcus aureus, Salmonella, Pneumococcus, Escherichia coli, Treponema pallidum, Candida, Aspergillus and Tuberculosis.1 Prompt diagnosis is critical in treatment planning and instituting subsequent therapies. We describe the CT imaging findings of two patients encountered in our centre.Aortitis refers to the inflammatory process involving the aortic wall, which can be either infective or non-infective in origin.9 l–1 with predominant neutrophilia. In view of the underlying AAA, a repeat CT was pursued. This revealed the presence of air surrounding the endovascular graft, with associated inflammatory changes and aortic wall thickening. The air was seen extending to the proximal common iliac artery . Initial CT angiography of the aorta revealed an infrarenal AAA measuring 6.3 cm × 7.0 cm × 20.0 cm, which extended to the level of the right common iliac artery. There were scattered wall calcifications. The thoracic aorta was normal. After discussing with the patient and family members, a decision was made to repair the AAA via an endovascular approach. A bifurcated endovascular stent graft was deployed via a percutaneous femoral route. The procedure was successful. However, 3 months post procedure, the patient came back with complaints of fever of 39°C, associated with chills, loss of appetite and back pain. He reported no vomiting or diarrhoea. Full blood count revealed a total white cell count of 15 × 10c artery . Medical9 l–1 with predominant neutrophilia. A multiphasic CT hepatobiliary system was initially planned, with a provisional diagnosis of hepatobiliary pathology. During the image acquisition, incidental finding of an infrarenal AAA was noted. The AAA measured 3.4  cm × 3.4 cm × 2.7 cm with protruding mural thrombus. No clear fat plane was identified, and multiple air pockets were seen. Retroperitoneal and para-aortic collections were present, with no bony destruction  was what likely happened in our first case, whereas a haematogenous spread from a distant source was likely the source in the second case, taking into account the history and underlying conditions.Under normal circumstances, the aorta is resistant towards infection. However, certain conditions may predispose it to infection, including atherosclerotic disease, an underlying aneurysm, patients who are immune-compromised and the presence of a medical device or open surgery. A few postulations of the pathophysiology are (1) direct extension of a local infection, (2) traumatic contamination, (3) septic embolism from endocarditis and (4) haematogenous spread from a distant source of bacteremia.2 Periaortic gas and vessel wall emphysema indicate more advanced disease,10 and therefore a poor prognosis, as noted above. With the advent of antibiotics, most of these signs are not usually seen.12Cross-sectional imaging proves to be an indispensable tool in the diagnosis and further management of this condition. CT is widely available and has multiplanar as well as post processing capabilities. Patients with this condition are often systemically unwell; thus a short image acquisition time is preferred. Among the signs of infective aortitis on CT are aortic wall thickening, periaortic fluid, a rapidly enlarging aneurysm, gas formation and air pockets and vertebral body destruction. Periaortic asymmetric fat stranding or a periaortic soft tissue mass are present in 48% of cases, and may be the only early signs of infection.12 In the later stages, with gas formation surrounding the infective process, CT is a better option.The role of MR, despite being able to provide excellent soft tissue contrast and not involving ionizing radiation, is limited in the acute setting, which is how these patients usually present. The longer acquisition time, the relative preponderance to artefacts in relation to CT, and limited access make MR a less preferred cross-sectional imaging modality compared to CT. However, if access to MR and patient cooperation are not limiting factors, MR may be best to demonstrate aortic wall oedema, particularly in the early stages and when aortitis is highly suspected.1 as was the case with our second patient. He was initially investigated for hepatobiliary pathology, only to be found to have emphysematous aortitis. The diagnosis in the first patient was aided by the knowledge that he had a previous endovascular procedure prior to presentation. However, the patient still succumbed as emphysematous aortitis is known to be a life-threatening complication after endovascular aortic repairs.13Accurate clinical diagnosis remains a challenge to treating physicians, as often these patients present with non-specific clinical signs and symptoms,2 The goal of surgery is diagnostic confirmation, control of sepsis, control of haemorrhage in cases where rupture has occurred and reconstruction of the aortic vasculature.12The mainstay of treatment involves medical therapy including appropriate antibiotics, preferably according to the sensitivity of the pathogen isolated from blood cultures. This is followed by surgical resection of the infected aortic segment with in situ or extra-anatomic reconstruction.14 Vigilance and early recognition enable prompt diagnosis and treatment and may potentially prove to be life saving.The diagnosis of “emphysematous aortitis” is a loosely used term to denote the presence of air in association with aortitis. A multipronged approach that includes clinical, laboratory and radiological findings is needed in guiding the approach to patient treatment strategies. CT is an excellent tool in recognition and diagnosis of this condition. Conforming to the current European Society of Cardiology guidelines on the management and treatment of aortic diseases, CT and if permissible MR, should be used as first-line imaging techniques in suspected aortic infections.Emphysematous aortitis is a life-threatening condition, albeit rare these days; early recognition followed by appropriate management may be life saving.Certain conditions predispose the aorta to infection; recognizing these allows early and accurate diagnosis.Clinical presentation alone may be vague and fail to lead to an accurate diagnosis. Hence, the role of cross-sectional imaging  is of utmost importance.Certain CT features, when recognized, enable the treating physician to diagnose and institute appropriate treatment/ operative intervention.Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Shortly after an operation infection, intraabdominal abscess, seroma, haemorrhage or development of paralytic ileus may occur. Postoperative adhesions, nonspecific abdominal pain without signs of obstruction, incisional hernia and appendicitis or mucocele in appendiceal stump present common late complications. We present a unique case of a late complication after appendectomy.The case report describes a unique expansion in the ascending colon of a young athlete with long-lasting abdominal pain in the lower right quadrant. Colonoscopy showed a lesion in a wall of the ascending colon. Computed tomography (CT) confirmed a cystoid formation of high content density in a wall of the caecum. A right hemicolectomy was performed. Histology showed a lesion located in the submucosa with intestinal lining and stroma rich in lymphoid cells. These are the typical attributes of the wall of the appendix. Other parts of the wall were not demonstrated, and there was no communication with the lumen of the native bowel.A submucosal cavity filled with acellular matter, which were probably disintegrated epithelioid structures, and calcifications were found in the ascending colon, while no intestinal cell atypia or dysplasia was found. The case cannot be classified under any previously presented diagnosis. Shortly after operation, complications occur in 11–16 % of cases and include wound infection, intraabdominal abscess, seroma, bleeding or development of paralytic ileus . ResiduaThe case report describes a unique complication, which is previously absent from the literature.Our work has been reported in line with the SCARE criteria. 2A 23-year-old male, with a history of appendicitis 9 years ago and a previous operation for an inguinal hernia was admitted to our teaching hospital due to long-term intermittent abdominal pain in the right lower quadrant. The pain was aggravated by intensive training. Discomfort graduated to changes in bowel habit, such as constipation, flatulence, dyspepsia with nausea and impaired digestion. Over the previous 6-month period, a weight loss of 5 kg occurred.A physical examination showed only a smooth scar after appendectomy, with tenderness in the right lower abdominal quadrant during deep palpation but without palpable resistance. A colonoscopy, performed at the district hospital, revealed a submucosal cylindrical formation in the wall of the ascending colon protruding into the lumen . There wA CT scan was performed in arterial, venous and late phase after the administration of iodinated contrast medium in the peripheral vein . The scaBased on a multidisciplinary consensus, the patient was indicated for a laparoscopically assisted right hemicolectomy under general anaesthesia. A surgical team, specialized in laparoscopic abdominal operation, performed the hemicolectomy 3 months after the expansion was diagnosed. During the operation, there were no pathological findings for other abdominal organs. There were adhesions to the scar after appendectomy, which were disrupted. The formation in the ascending colon was not found after mobilization of the colon from the parietal peritoneum; therefore, a perioperative colonoscopy was performed. The colonoscopy confirmed that the formation in the caecum extended to the ascending colon. After the examination, the laparoscopically assisted right hemicolectomy and mesenteric lymphadenectomy were performed. Approximately 13.5 cm of colon without appendix and 6 cm of terminal ileum were removed . During Histologically, there was a submucosal cavity lined by intestinal mucosa with stroma rich in lymphoid tissue, while no other layer of the wall was found. There was no communication with native bowel lumen . The cav9/L and a CRP (C-reactive protein) level up to 207 mg/L, probably due to the reaction to an operation and systemic inflammatory reaction, the next day a laboratory examination showed no disturbances. In few days the patient made an uneventful recovery and was discharged home within a week. Three weeks after operation, the patient was without any difficulties. Patient was seen regularly for 3 months, without any complication, he was forwarded to follow-up at a district hospital.Shortly after operation a laboratory examination showed an elevation of the white cell count to 21 × 103Considering the history, age, location of the lesion, clinical condition, CT findings and after correlation with the macroscopic and histological findings, the residual epithelium of the appendix with cystic transformation appeared as the most likely diagnosis. Appendiceal tissue was probably dislocated to the submucosa of the caecum during the appendectomy. The cavity was forming for 9 years, filling with disintegrating epithelial structures, and over the years it grew to a size that caused clinically significant difficulties. Small calcifications are typical of the disintegrating epithelial cells. In the available literature, similar complications of appendectomy have not yet been described. In differential diagnosis, the cystic lesions, congenital abnormalities, tumours and postoperative complications were considered. Particularly, we tend to exclude mostly duplications, congenital or acquired diverticulum and appendiceal mucocele.Gastrointestinal duplications are rare congenital anomalies located anywhere within the gastrointestinal tract. The most common location is the terminal ileum.There are three criteria required for diagnosis of duplication, which are as follows:first, a connection to the gastrointestinal tract, which commonly means either the serous or muscle wall layer;second, an alimentary tract-type mucosal epithelial lining;third, contains all 3 layers of bowel wall, including the muscularis propria.Imaging methods, such as CT, ultrasonography (USG) or magnetic resonance (MR), are a very important part of the diagnostic process. In CT examination, spherical or tubular duplication cysts are distinguished, they do not infiltrate adjacent structures or organs, and the wall might be denser, but the content density reaches 0–20 HU, with no significant contrast enhancement. A rare case appeared in the literature , whereinDuplications are a rare diagnosis. The duplications, although benign, have the potential to become malignant in up to 23% of cases; therefore, resection is recommended as a method of treatment .The presence of the muscularis propria in the wall and a communication with the alimentary tract are the attributes of either congenital or acquired diverticulum. The muscularis propria was not proven in the presented case, and therefore the diagnosis of diverticulum was excluded. Communication with the alimentary tract was not proven either.Appendiceal mucocele is a rare acquired condition found in the stump after less than 1% of appendectomies . We wereIn the case of our patient, the high-density content was caused by a large number of calcifications, which usually evolve during long-term congestion. The cavity was located intimately to the stump after appendectomy, and it reached the hepatic flexure as it grew. Since the muscularis propria was missing, we conclude that the original appendiceal mucosa cells transformed cystically after being dislocated to the submucosa of the caecum during surgery.4The described late complication after appendectomy is rare and according to our knowledge it has not yet been published. A possible descriptive term might be a submucosal mucoid.We declare that we have no competing interests.National Sustainability Program I (NPU I) Nr. LO1503 provided by the Ministry of Education Youth and Sports of the Czech Republic and grant SVV 260392 provided by Charles University and by the project of the Ministry of Health of the Czech Republic - Conceptual Development of Research Institutions00669806 – FN Plzen, by the project of the Charles University Prague Progress Q39, by the project Centrum of clinical and experimental liver surgery “UNCE/MED/006 of the Charles University and by the project CZ.1.05/2.1.00/03.0076 from European Regional Development Fund.This study was supported by the The role of the donators was funding the grammar proof-reading by American Journal Experts and the publication related costs.Ethical approval has been exempted by our institution. No identifiable patient’s parts were seen and all of the data were collected from clinical records and imaging systems for routine perioperative planning.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Kristýna Bajcurová: Writing – Original Draft, Resources, Visualization. Petr Novák: Resources, Writing – Review and Editing. Jan Geiger: Resources. Eva Korčáková: Resources, Writing – Review and Editing, Supervision. Hynek Mírka – Writing – Review and Editing, Supervision. Petr Rajal: Resources. Marcela Podolcová – Resources. Ondřej Daum: Resources, Writing – Review and Editing.All authors read and approved the final manuscript.Not applicable.Kristyna BajcurovaNot commissioned, externally peer-reviewed.


This is the first reported case of a primary intraosseous angioleiomyoma and the second case of a primary leiomyoma of the rib, irrespective of age. Angioleiomyomas mostly occur in patients of advanced age, in any part of the body, particularly the lower extremities and present as painful, slow-growing nodules in the dermis, subcutaneous fat or deep fascia. Other localizations, especially bone, are considered extremely rare, as well as their occurrence in paediatric patients.A 10-year-old girl was admitted to the orthopaedic surgery department for further assessment of a pain localized in the posterior part of the right hemithorax. After magnetic resonance imaging (MRI) and surgical biopsy, intraosseus angioleiomyoma of the fourth rib was diagnosed by histopathology examination. Atypical costal localization of this type of a benign tumour presents diagnostic difficulty, especially in children. The differential diagnoses included cartilaginous tumours, Ewing sarcoma, fibrous dysplasia, Langerhans cell histiocytosis, intraosseous haemangioma and metastatic tumours. We report a detailed diagnostic procedure including MRI, selective angiography and histopathologic examination.Diagnosis of intraosseous angioleiomyoma is difficult due to the extreme rarity of this tumour and absence of pathognomonic radiological signs. Although very rarely identified in bones and young age group, radiographers and reporting doctors should be aware of this possible angioleiomyoma presentation and supported by the provided detailed diagnostic information. Angioleiomyoma is a rare benign tumour, occurring in the soft tissues (dermis and subcutaneous fat) of the lower extremities in middle-aged women between fourth to sixth decade of life  It can aIn our paediatric patient case, primary intraosseus angioleiomyoma was localized in a rib and to our knowledge, this is the first reported case thus far with rib localization and the second case of a primary leiomyoma in the rib. We describe the differential diagnosis of angioleiomyoma at this atypical localization considering the common costal tumour pathologies in children, such as cartilaginous tumours, Ewing sarcoma, fibrous dysplasia, Langerhans cell histiocytosis, intraosseous haemangioma and metastatic tumours.A 10-year-old girl was admitted to the orthopaedic surgery department for further assessment of a pain localized in the posterior part of the right hemithorax. According to the physical examination, there was no evidence of a palpable chest wall mass, but the patient reported worsening of symptoms during palpation. Skin and subcutaneous tissue showed no swelling or discoloration. Laboratory values, including serum levels of tumour markers, were all within the normal reference ranges. The initial chest X-ray performed in another institution has been lost. We opted for MRI instead of the CT as the next diagnostic procedure in order to avoid additional exposure of the young patient to ionizing radiation.Magnetic resonance imaging (MRI) of the thorax revealed a spherical, lobulated tumour, located in the posterior arch of the right fourth rib and the adjacent chest wall, 10 mm from its costovertebral junction. The lesion measured 30 × 50 × 20 mm in all three diameters and showed heterogeneous signal intensity. It was mostly hyperintense relative to the muscle on non-contrast T1-weighted (T1W) fast spin echo (FSE) images  and MRI are the diagnostic modalities of choice. Whereas CT provides high diagnostic sensitivity for bone and lung parenchyma, MRI is more optimal for assessment of soft tissue propagation. Because of its non-invasiveness, chest MRI has lately become the first step in the diagnostics sequencing of the chest wall tumour assessment for pediatric population. Angioleiomyoma MRI presents as a well-demarcated and strongly enhancing mass with an isointense to slightly hyperintense signal compared to muscle on T1W images and hyperintense signals on T2W images . MRI feao et al. . Accordio et al. . Taken tGanyusufoglu et al. [The only case of leiomyoma in the rib was published by u et al. . They shAtypical costal localization of angioleiomyoma created a diagnostic challenge, considering that the rib is the common site of several tumours in children, such as cartilaginous tumour, Ewing sarcoma, fibrous dysplasia, Langerhans cell histiocytosis, intraosseous haemangioma and metastatic tumours .Table Enchondroma was also taken into consideration as a differential diagnosis, having in mind that it is the second most frequent tumour of the ribs  and thatIn addition to the most frequent benign rib tumours in children, the most frequent malignant tumours with the same localization in children were also taken into consideration – Ewing sarcoma and metastatic tumours. Ewing sarcoma is the second most frequent malignant bone tumour in children, with a peak incidence in the second decade of life , 19. In Langerhans cell histiocytosis matched our case by several MRI characteristics: a demarcated lesion with hyperintense appearance on fluid-sensitive sequences and diffuse contrast enhancement. However, the predominantly hyperintense lesion on T1W in our patient diverged from the typical hypo- to isointense Langerhans cell histiocytosis appearance on T1W .Tew et al. [Because of the highly suspected vascular tumour, intraosseous hemangioma was also included in the differential diagnosis. Hemangiomas with bone localization are rare and include less than 1% of all bone tumours . Our casw et al. . They rew et al. .Diagnosis of intraosseous leiomyoma is difficult not only due to the extreme rarity of a tumour but also the absence of pathognomonic radiological signs. The exact diagnosis is thus only achieved by histopathological examination and immunostaining . DiffereIn conclusion, this case emphasizes that atypical intraosseous angioleiomyoma, especially in the ribs of paediatric patients, could mislead the referring physician to more common pathologies in this region. Besides its extreme rarity, diagnosis of intraosseous angioleiomyoma is further complicated by the absence of any pathognomonic radiological signs. Its diagnosis is thus reliably achieved by histopathological examination combined with immunostaining. We expect that the detailed description of this unusual presentation of angioleiomyoma would provide a valuable resource to facilitate diagnosis of such untypical presentations in the future.


Acute Esophageal Necrosis Syndrome (AENS) is a rare and unknown clinical entity, defined as a diffuse circumferential black-appearing friable esophageal mucosa going from the distal esophageal mucosa to the gastroesophageal (GE) junction. Esophagogastroduodenoscopy (EGD) remains the gold standard in making diagnosis.We report here the case of a 45-year-old man with necrosis of the esophagus treated conservatively. Regression of the lesion but persistence of ulcerations were seen on the endoscopic follow-up. Distal esophageal stenosis was then diagnosed and treated by endoscopic dilation.Diagnosis of AENS must be considered when an old patient, with multiple comorbidities, presents an upper digestive hemorrhage. Upper endoscopy is mandatory. Treatment is in most of the cases conservative. Esophageal stenosis is a frequent complication.Although AENS is a rare clinical entity, it should not be dismissed by doctors, avoiding useless surgical management. This pathology remains nevertheless associated with a considerable mortality rate. To date29/L and a CRP measured at 128.0 mg/L. An emergency EGD was then performed and full necrosis of the lower esophagus was diagnosed (A 45-year-old man, with history of biliopancreatic diversion for alcoholic chronic pancreatitis, was referred to the emergency unit of Pasteur Hospital in Nice for a 72-h history of tiredness, dysphagia and hematemesis. There was no history of peptic ulcer disease or esophageal reflux and he denied ingestion of any caustic agents. On admission, there was no clinical sign of anemia and vital signs were in normal ranges, the patient was afebrile. Blood sample showed an inflammation syndrome with a white blood cell count at 10.8 × 10iagnosed . No bleeiagnosed . An endo3AENS is a rare clinical entity and thus has little mention in literature. This syndrome concerns more often elderly men, with a mean age of 67 years old ,3. The mIn conclusion, AENS is a rare but very serious disease, with specific etiology remaining unknown and considered multifactorial. Diagnosis of AENS must be considered when an old patient, with multiple comorbidities, presents an upper digestive hemorrhage. Early endoscopic diagnosis is essential in order to allow fast and effective therapeutic care. In absence of complications, treatment is conservative involving proton pump inhibitors along management of all comorbidities. Finally, this entity is associated with a considerable rate of mortality.No.Ethical approval has been exempted.No ethnical approval.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Maubert Alexandre: Conceptualization, Redactor.Frey Sebastien: Redactor, English Translator.Rahili Amine: Investigator, Supervisor.Filippi Jérome: Investigator.Benizri Emmanuel: Supervisor.No.Maubert Alexandre.Not commissioned, externally peer-reviewed.None.