Ectopic insulinomas are extremely rare and challenging to diagnose for clinicians. Precise preoperative localization is essential to successful treatment.A 23-year-old man presented with a 1-year history of recurrent hypoglycemia.68Ga-Exendin-4 PET/CT and 68Ga-DOTATATE PET/CT which helped to make a conclusive diagnosis that the lesion in the gastric antrum was an ectopic insulinoma.Examinations in the local hospital did not reveal any pancreatic lesion. After admission, a fasting test and a 5-hour oral glucose tolerance test (OGTT) suggested a diagnosis of endogenous hyperinsulinemic hypoglycemia. Enhanced volume perfusion computed tomography (VPCT) revealed 2 nodules in the tail of the pancreas, a nodule in the gastric antrum, and a nodule in the hilum of the spleen. To differentiate which nodule was responsible for hypoglycemia, we performed The patient was cured with minimally invasive laparoscopic resection of the tumor.The symptoms were relieved and the blood glucose level remained normal after surgery.68Gallium-exendin-4 PET/CT is useful for precise localization and thereby successful treatment of insulinoma, especially for occult insulinomas and those derived from an ectopic pancreas.This case shows that  Biochemical diagnosis of insulinomas is well established when hypoglycemia, supported by the presence of Whipple's triad, is associated with endogenous hyperinsulinism. However, the localization diagnosis is the key to successful surgical treatment. If conventional imaging examinations failed to find a lesion in the pancreas, searching for the tumor was almost an impossible task for clinicians in the past. Recently, the advent of radionuclide-based molecular imaging probes for β-cells opens the possibility of accurate localization of both orthotopic and ectopic insulinomas. Here we described a young patient with hyperinsulinemic hypoglycemia whose conventional imaging examinations revealed multiple suspected lesions both inside and outside of the pancreas. Finally, a conclusive diagnosis of ectopic insulinoma in the gastric antrum was made with the help of 68Ga-Exendin-4 PET/CT. The patient was cured with minimally invasive laparoscopic resection of the tumor.Insulinomas are the most frequent functioning pancreatic neuroendocrine tumors (NETs) with an estimated annual incidence of 0.4 cases per 100000 population.2A 23-year-old man with recurrent palpitation and sweating after activities for more than 1 year was admitted to our hospital in September 2020. His symptoms could be improved after eating. 1 year ago, he was sent to the emergency department of a local hospital because of confusion and sweating, with his blood glucose determined as 2.8mmol/L. The upper abdominal MRI scan did not reveal abnormalities, and the Insulin autoantibody, Islet cell antibody, and glutamate decarboxylase antibody were negative. Oral glucose tolerance test (OGTT) showed delayed insulin secretion. He was diagnosed with prediabetes and discharged. Since then, he increased food intake and avoided exercises, and the symptoms did not recur. However, he gained 25 kg in 1 year. After admission, a fasting test was performed and was stopped after 4 hour because of hypoglycemic attack. Serum glucose level was 1.7 mmol/L, insulin 53.3 mU/L, C-peptide 7.88 ng/ml, and the insulin release index 1.74. During his hospitalization, the nadir of blood glucose levels was 1.6 mmol/L, insulin 72.9 mU/L, and C-peptide 8.79 ng/ml, and the insulin release index 2.53. The simultaneous cortisol level was 31.3 ug/dl (5–25), Adrenocorticotropic hormone 45.1 pg/ml (0–46), growth hormone 7.940 ng/ml (0.06–5.0). A 5 hour-OGTT (Table 68Ga-DOTATATE PET/CT.Pancreatic volume perfusion CT (VPCT) revealed a small nodule on the right side of the gastric antrum (maximum cross-section of approximately 21 mm × 16 mm)  neuroendocrine tumor (G2) with a distinct border surrounding exocrine pancreatic tissue Fig. . The immlts Fig.  showed tlts Fig.  the pati3 Ectopic insulinoma is extremely rare, accounting for about 1% to 2% of all insulinomas. There are only a few cases reported in the literature. The diagnosis of this disease can be quite challenging because of the lack of effective localization diagnostic methods. With the development and maturation of radionuclide-based molecular imaging probes for β-cells, reports of ectopic insulinomas increased significantly in the last decade. We searched the English literature of the past 40 years in PubMed and found 13 patients who had this disease. Their data were listed in Table Insulinoma is a functioning pancreatic neuroendocrine neoplasm composed of insulin-producing and proinsulin-producing cells, with uncontrolled insulin secretion causing a hypoglycemia syndrome. It is the most common functioning pancreatic neuroendocrine neoplasms with an estimated annual incidence of 0.4 cases per 100000 population.,4 The variability is dependent on tumor size, type of machine, protocols used, and the expertise of radiologists. In most of the cases (over 90%) the diameter of tumors is less than 2 cm, making it very difficult to accurately localize using MRI and CT prior to surgical excision. Moreover, multiple insulinomas, although rare (less than 10%) and often associated with MEN1, and ectopic insulinomas are even harder to accurately locate, and incomplete resection may cause symptom persistence. So like the patient in our study, quite a few patients suffered from a long history and repeated hospitalization because of the insensitivity of conventional localization examinations in the past. In the patient of our study, VPCT detected 2 nodules in the tail of the pancreas, a nodule in the gastric antrum, and a nodule in the hilum of the spleen. We first highly suspected that the 2 lesions in the tail of the pancreas where insulinomas located with a slight predominance were the cause of hypoglycemia. However, the nodule in the gastric antrum which was considered a NET by ultrasound endoscopy also showed early enhancement in the arterial phase in VPCT and had the same signal with normal pancreas in MRI, suggesting the possibility of ectopic insulinoma or a metastatic lesion, although insulinomas rarely spread to stomach. Besides, the nodule in the hilum of the spleen needed further examination. Therefore, multiple insulinomas, ectopic insulinoma, and metastatic insulinoma were suspected in this patient. No conclusive diagnosis could be made based on VPCT and MRI.Typical Whipple triad and endogenous hyperinsulinemic hypoglycemia lead to a suspected diagnosis of insulinoma. However, the difficulty of diagnosis of insulinomas lies in the localization of the tumors which is also the key to the success of surgical treatment. Insulinomas are evenly distributed in the pancreas or show a slight predominance in the head and tail regions. Searching for the tumors is very challenging for clinicians. A relatively high number of insulinomas could not be found by intraoperative inspection and palpation by the surgeon. The sensitivity of contrast CT and MRI, 2 traditional noninvasive imaging examinations used to locate insulinoma, has been reported to range between 33% to 64% and 40% to 90%, respectively.,6 Utilizing radionuclide-based exendin-4 (a stable synthetic GLP-1R agonist) imaging opened the possibility of highly specific imaging and accurate localization. 68Ga-labeled exendin-4 PET/CT, showed an excellent sensitivity of 95% to 98% and a distinct advantage over the traditional imaging modalities. In our patient, with the aid of 68Ga-Exendin-4 PET/CT, we could remove the suspect of the 2 nodules in the tail of the pancreas and the nodule in the hilum of the spleen and finally confirmed that the nodule in the gastric antrum with abnormally concentrated tracer was an ectopic insulinoma. 4 of the 13 cases of ectopic insulinomas we reviewed were diagnosed by 68Ga-Exendin-4 PET/CT, and the smallest tumor was 1.2 cm in diameter. This specific functional scan for insulinoma had high sensitivity for hidden small insulinoma, and helped to make a conclusive diagnosis when ectopic insulinoma was suspected.Glucagon Like Peptide 1 Receptor (GLP-1R) is overexpressed by up to 5 times in almost all benign insulinoma compared to normal human β-cells.68Ga-DOTATATE, a somatostatin analogue with highest affinity for somatostatin receptor 2. SSTs are overexpressed in neuroendocrine tumors, and SST PET has been suggested to be the preferred imaging modality for initial diagnosis of neuroendocrine tumors. Moreover, somatostatin imaging plays a role as a theranostic investigation to determine suitability for targeted radiotherapy with somatostatin analogs, which is known as peptide receptor radionuclide therapy. However, recent studies suggested that the expression of SSTs in benign insulinomas was less frequent and intense than GLP-1R, while most malignant insulinomas had high expression levels of SSTRs. Besides, the 18F-FDG PET/CT showed the SUV of the nodule in the gastric antrum was 5.4, and malignant lesion could not be excluded. Enhanced FDG uptake by high-grade tumors due to Warburg effect is a hallmark of in vivo tumor imaging with FDG PET/CT. The relationship of molecular markers with malignancy tendency in insulinomas was described as the “triple-flop” phenomenon, an increasing tendency toward malignancy during progression from GLP-1R avid (benign), to somatostatin receptor avid , to FDG avid  insulinomas. Because the 3 imaging examinations of our patient were all positive, regular follow-up is needed in case of recurrent or metastatic disease.In our patient, the tumor also took up high level of With regard to ectopic insulinoma, no precise definition was provided in the literature, the case reports roughly included several categories. The most recognized definition is an insulinoma found in an ectopic pancreas. An ectopic pancreas is a rare entity defined as the presence of pancreatic tissue without any anatomic or vascular continuity with the pancreas. It may occur at a variety of sites in the gastrointestinal tract having a propensity to affect the stomach and small intestine. Ectopic insulinomas within an ectopic pancreas were clearly described in 5 patients in our literature review. The most common site was duodenum and adjacent tissues, such as duodenal ligament, spleen, Treitz ligament and proximal jejunum. The tumor localization in this study was gastric antrum, which was also adjacent to duodenum. Other location, such as spleen, was also reported.,11 PP immunoreactivity were detected in 35% of cases, with a mean of 33% of all tumor cells; glucagons were found in 30% of cases and 30% of cells; somatostatin in 15% of cases and 20% of cells; serotonin in 20% of cases and 36% of cells; calcitonin in 20% of cases and 10% of cells; and insulin in 15% of cases and 2% of cells. No gastrin or vasoactive intestinal peptiepeptide immunoreactivity was detected. It was reported that 6 years after excision of a rectum G1 neuroendocrine tumor in a middle-aged woman, 5 pelvic lesions which might transit from rectum NET acquired the ability of insulin secretion and caused hypoglycemia. Both the rectum tumor and the 5 pelvic foci showed expression of insulin, while the former did not cause any symptom. How does non-functioning NETs acquire the ability of insulin secretion during the course of disease is unknown.The second category is non-functioning gastrointestinal neuroendocrine tumors that gradually achieve the ability of insulin secretion and cause hypoglycemia. Although without clinical syndrome, about 40% of non-functioning NETs produce multiple hormones. The hormones most often expressed are somatostatin, pancreatic polypeptide, and glucagon. In a series of 61 nonfunctioning tumors, Some studies clearly showed that non-islet-cell tumors were the origin of the hyperinsulinemia on the basis of the detection of proinsulin mRNA and insulin protein within the tumor cells. In this study, these tumors were not classified as an ectopic insulinoma.Some non-islet-cell tumors have been reported to secrete insulin and cause hyperinsulinemic hypoglycemia. Among them are bronchial carcinoid tumor, squamous-cell carcinoma of the cervix, neurofibrosarcoma, schwannoma, paraganglioma, small-cell carcinoma of the cervix, and gastrointestinal stromal tumor. The only malignancy criterion defined by World Health Organization (WHO) for insulinoma is the presence of metastases, being the liver the most frequent affected organ. Of the 13 ectopic insulinomas we summarized, 2 patients with liver metastases and lymph nodes metastases respectively could be confirmed as malignant insulinoma. However, because of the scarcity of cases, it was not reliable to conclude the malignant tendency of ectopic insulinomas. Most insulinomas are less than 2 cm in diameter, but more than half of the ectopic insulinomas we have reviewed from the literature were greater than 2 cm (7/13) in diameter. This may be related to the long course of disease (mean history: 29.7 months), delayed diagnosis, and the existence of exocrine pancreas.The majority of insulinomas are benign, only about 10% are malignant.68Ga-Exendin-4 PET/CT is essential to the localization diagnosis and precision surgery of this disease. 68Ga-DOTATATE PET/CT and 18F-FDG PET/CT are also helpful in characterizing the tumor and assessing the prognosis. Further investigation needs to focus on the biological behavior of ectopic insulinomas.In summary, we reported a case of ectopic insulinoma in gastric antrum. Precise preoperative localization and functional examinations with XZ, HJ, and FL collected, analyzed and interpreted the clinical data. HJ provided the pathology analysis. CF and JZ performed immunohistochemical and immunofluorescent staining of the tumor. ML reviewed the literature. XZ wrote the initial draft for this case. QH and ML critically revised the manuscript. All authors have read and approved the manuscript.Conceptualization: Ming Liu.Data curation: Xiaona Zhang, Hongwei Jia, Fengao Li, Chunyun Fang.Funding acquisition: Xiaona Zhang.Investigation: Hongwei Jia, Fengao Li, Qing He, Ming Liu.Methodology: Chunyun Fang, Jinyang Zhen.Supervision: Hongwei Jia, Qing He, Ming Liu.Writing – original draft: Xiaona Zhang.Writing – review & editing: Qing He, Ming Liu.


RB1 variants may vary during development. We describe an unexpected fatal case of trilateral retinoblastoma with an intracranial tumor in an unusual location and discuss genetic copy number analyses as a useful diagnostic tool with therapeutic potential.The clinical course of trilateral retinoblastoma can be unpredictable, and expressivity of germline  RB1 variants may vary during development.The clinical course of trilateral retinoblastoma can be unpredictable, and expressivity of germline  In high income countries, survival rate is >95%.RB1 gene, a first and second “hit” as described by Knudson in 1971 (Knudson's two‐hit hypothesis).RB1 variants arise in the developing retina, whereas in heritable retinoblastoma, a germline RB1 variant is present. The majority of heritable retinoblastoma patients have a de novo RB1 variant, as only 10% inherit the variant from a parent .Retinoblastoma arises from a cell that nearly always harbors pathogenic variants in both copies of the RB1 variant (hence heritable retinoblastoma), but some children with heritable retinoblastoma only develop unilateral disease, due to variation in penetrance and expressivity. Trilateral retinoblastoma refers to an intracranial tumor associated with heritable retinoblastoma and occurs in 3,5% of patients with heritable retinoblastoma.Retinoblastoma can affect one eye  or both eyes . Children with bilateral disease have a pathogenic germline In this case study of trilateral retinoblastoma, we describe a patient with an intracranial tumor in an unusual location and a challenging clinical investigation process involving extensive genetic testing.2A 15‐month‐old boy presented with deviation of the right eyeball that had been slowly progressing since onset one month earlier. There was no family history of retinoblastoma, and the patient had an unremarkable prior history with normal growth and developmental milestones. The diagnosis of retinoblastoma was made on the basis of ophthalmoscopy and ultrasound of the right eye that revealed large, calcified tumor masses. The anterior chamber was normal. A baseline brain MRI was performed and revealed no extraocular extensions or trilateral disease  in the cerebellopontine angle, subependymal tumor elements in the rim of the lateral ventricle anterior horns, several drop metastases in the thoracolumbar spinal canal, and enlarged lymph nodes on the left side of the neck  scan performed at one‐month follow‐up showed no pathological activity in residual lesions in the left cerebellopontine angle and the spinal canal. The three‐month follow‐up brain and spine MRI showed no changes.Five months after treatment was completed, the patient was admitted with sudden‐onset vomiting, headache, fatigue, and loss of balance. MRI showed multiple infra‐ and supratentorial processes bilaterally, and several small nodules in the ventricles. Aspergillosis was suspected, but biopsy showed malignant cells that were confirmed to be retinoblastoma Figure . The pat3RB1 was detected in DNA from the patient's blood. Quantitative multiplex PCR (QM‐PCR) confirmed that only one copy of exon 2 was present. For exon 1, three sets of non‐overlapping primers showed only one copy, the first primer being located at the position NM_000321.2(RB1): c.101. Analysis of the RB1 promoter and exon 3 revealed 2 copies.A heterozygous deletion of exon 2, and at least part of exon 1, of RB1 pathogenic variant was not identified after sequencing all RB1 coding sequence and flanking noncoding regions, methylation‐sensitive PCR analysis of the RB1 promoter,KIF14, DEK, E2F3, CDH11, and MYCN was performed using QM‐PCR.DEK and four copies of MYCN, confirming the presence of tumor DNA.It was suggested that the deletion might be a reduced‐expressivity variant, as the patient was only unilaterally affected at the time, and it is known that some exon 1 variants show reduced penetrance due to utilization of alternative transcription start sites.RB1 exons 1–2 . LOH is frequently the second mutational event leading to the development of a tumor in both retinoblastomas as well as in other cancers.KIF14, DEK, E2F3, CDH11, and MYCN genes showed differences in comparison with the intraocular tumor  or a metastasis. MLPA analysis of the intracranial tumor identified homozygous loss of 4RB1 gene that originated from a balanced chromosomal rearrangement in the mother. At age 4 years, the patient had no sign of recurrent eye disease, but had developed a large, symptomatic tumor located to the midline cerebellum. She died months later from disseminated disease with spinal involvement. Autopsy and thorough histopathological assessment were performed, and the cerebellar tumor was found to be a medulloblastoma that originated separately from the ocular tumors, based on histomorphological and immunocytochemical features.We describe a clinical case of trilateral retinoblastoma that is atypical in several aspects. Non‐pineal tumors in trilateral retinoblastoma usually occur synchronously, and almost exclusively in the supra‐ or parasellar regions.BRCA1 and BRCA2.E2F3 and MYCN,In addition to being a diagnostic tool, characterization of somatic alterations is the basis of personalized oncology therapies, developed to improve treatment outcomes and minimize long‐term sequelae by selectively targeting cancer cells. For some cancer types, targeted treatments are well established, such as the use of tyrosine kinase inhibitors in Philadelphia chromosome‐positive leukemiaRB1 variant may vary during development.More than 50% of trilateral retinoblastomas can be diagnosed by a baseline brain MRI in relation to the primary diagnosis of retinoblastoma,RB1 germline variant, and was expected to have a good prognosis. However, the patient developed aggressive tertiary disease that initially responded to treatment but recurred shortly after.Similarly, in a review by Yamanaka et al., no difference in latency period between intraocular and tertiary retinoblastoma of any location was found, when comparing patients according to laterality of their intraocular retinoblastoma.In conclusion, the clinical course of trilateral retinoblastoma can be unpredictable. Whether screening for trilateral retinoblastoma should be implemented is still a subject of debate, and no international consensus exists. Genetic analysis can be a useful diagnostic tool in challenging clinical investigations of trilateral retinoblastoma and has therapeutic potential.The authors declare no conflicts of interest.Saga Elise Eiset and Pernille Axél Gregersen drafted the manuscript. Mikkel Funding, Hilary Racher, Steffen Heegaard, Brenda Gallie, and Steen F. Urbak contributed to relevant parts of the manuscript. All authors reviewed and approved the final manuscript.None.Informed consent was obtained from both of the patient's parents for publication of this case report and clinical images. Written documentation of consent in accordance with the institution's patient consent policy is available upon request.


The sphenoid bone is a complex structure in terms of its embryological origin. At birth, the sphenoid sinus is not pneumatised. Pneumatisation begins at around 4 months of age until the age of 12–14 years.If this process is absent or interrupted for reasons that are often unknown, it is called arrested pneumatisation.This report describes the case of a 15 year old patient, who consulted an ENT specialist for chronic headaches and hearing loss on the left side. Clinical ENT examination (including otoscopy) is normal. Tonal audiometry revealed a sensorineural hearing loss in the left ear. A CT scan of the petrous bone was normal but revealed a hypodense lesion in the left sphenoid bone. Lipoma was suggested. A brain MRI was performed in a clinic to better characterize the lesion. MR images showed a well-defined lesion with fatty content. The diagnosis was nasosinus fibrous dysplasia.In view of the diagnostic discrepancy, the patient was referred to our department for a specialist opinion. An additional brain scan revealed a non-eroded, non-expansive fatty density lesion with well-defined internal curvilinear calcification in the left sphenoid sinus location. Our final diagnosis was arrested pneumatisation.Most patients with arrested pneumatization of the skull base are asymptomatic. Sometimes it may be revealed by nonspecific signs and be confused with severe skull base disease, especially if the radiologist is not familiar with its existence or its typical features. •Arrested pneumatization is the absence or incomplete pneumatization of the sphenoidal sinus.•It is often discovered incidentally but can be revealed by non-specific symptoms such as headache, hearing loss.•It is a normal variant, recognition of its typical features on imaging is important for the radiologist .•No additional treatment or check-up is required. Indeed, it is formed by the fusion of different primordia whose embryonic origins are different the orbitosphenoid and the basi-post-sphenoid derive from the cephalic mesoderm whereas the alisphenoid and the basi-pre-sphenoid are from neural crest cell origin. The sphenoid bone has been linked with several developmental diseases. Sphenoid sinuses are two pneumatised structures located in the body of the sphenoid bone .Classically, the maxillary, sphenoid and frontal sinuses develop by expansion of certain ethmoidal cells in the neighbouring bones of mesenchymal origin that surround the ethmoid the ethmoid, namely the maxillary, frontal and sphenoidal bones .The normal process of pneumatization of the skull base and paranasal sinuses starts in utero and develops through young adulthood. It is known that the red bone marrow is replaced by the fatty marrow prior to the normal pneumatization process of the paranasal sinuses, including the sphenoid bones. The process of marrow conversion occurs before epithelialization and the formation of the respiratory mucosa in the aerated sinus. In the sphenoid, the fatty conversion usually begins at around four months of age, and ends at 10–14 years of age. The fatty marrow is replaced by a fully pneumatized sinus lined by respiratory epithelium .In rare cases for no specific reason, the pneumatization fails before the respiratory mucosa fully extends into the site of fatty marrow conversion. In this case, atypical fatty marrow remains permanently around the sphenoid sinus even in adult hood (imitating a lesion), thus called arrested pneumatisation .Individuals with arrested pneumatization of the skull base are usually asymptomatic and the variant is most often discovered incidentally when imaging is performed for a separate cause.Arrested pneumatization is usually mistaken for a tumour because the sphenoid bone is associated with several developmental diseases.The aim of this study was to highlight the imaging features of arrested pneumatization of the skull base to aid the clinician in recognizing this developmental variant which can be that can be confused with serious diseases of the skull base (tumours) and be symptomatic.Our work consists of a single case report and has been reported in line with the SCARE 2020 criteria .2The patient was 15 years old male, with no known pathological history or toxic habits. He consulted an Otorhinolaryngologist for chronic headaches and left-sided hearing loss. Physical examination was normal. Otoscopy was normal revealing a normal eardrum bilaterally. The tonal audiogram revealed a moderate sensorineural hearing loss of 46 dB in the left ear.Petrous bone CT scan was performed without any particular abnormality in the middle ear: the tympanic membrane and the ossicular chain are normal. However, a hypodense lesion in the sphenoid bone was noted. The initial diagnosis was a lipoma .Fig. 1CTTo better characterize the lesion radiologist then requested a brain MRI. The images show a well-defined, non-expanding lobulated lesion located at the site of pneumatisation of the left sphenoid sinus (arrows). The lesion shows increased signal on T1W and T2W images, which is consistent with the fat signal. The second diagnosis was nasosinus fibrous dysplasia .Fig. 2BrIn view the diagnoses discrepancy, the patient was referred to our department for a specialist opinion. An additional brain scan revealed. We completed the examination with a brain scan in order to explore the whole bone structure. The images show: a non-eroded, non-expansive fatty density lesion with a well-defined internal curvilinear calcification opposite the left sphenoid sinus. The lesion extended into the sphenopalatine cleft and pterygoid process, its margin was osteosclerotic, and the adjacent bone structure was intact and showed no mass effect . Our finSince this is a normal development variant, no additional examination or treatment was performed.In our case no other cause is found to explain his symptomatology. A clinical-radiological follow-up was instituted to ensure the benignity of the lesion.After one year of follow-up no aggravation of symptoms or complications were observed. The first radiological control showed a stable aspect of the lesion.3During normal development, a process known as red-to yellow marrow conversion takes place, in which the adipose tissue within the red marrow increases in relative percentage. Several studies indicate that pneumatization of the cranial bones, such as the paranasal sinuses and mastoid air cells, only occurs after red-to-yellow conversion. If the conversion process does not begin or is not completed, the pneumatization process is arrested and the area that is normally aerated is instead filled with yellow marrow ,7,9, henArrested pneumatization of the skull base is not well recognized by many clinicians and radiologists and can often be confused with other pathologies such as fibrous dysplasia, chordoma, chondrosarcoma, intraosseous lipoma, intraosseous hemangioma, hamartoma, ossifying fibroma, enchondroma, and fibrous osteoma .Unless complicated, Individuals with arrested pneumatization of the skull base are usually asymptomatic and the variant is most often discovered incidentally when imaging is performed for a separate cause. However in such cases of arrested pneumatization of sinus sphenoid, symptoms occurs mimicking serious disorders .Our patient is one of the rare cases of arrested pneumatization revealed by non-specific symptoms such as headache and hearing loss.Imaging perspective, a set of imaging criteria (Welker's criteria) have been proposed to establish the diagnosis: the lesion must be located at a site of normal pneumatisation or a site of recognized accessory pneumatisation; the lesion must be non-expansive; the lesion should have sclerotic, well-circumscribed margins; the lesion should show fatty content; on CT, internal curvilinear calcifications should be present - these should be morphologically different from the “ring and arc” type calcifications found with chondroid series tumours; any associated skull base foramina should retain their normal appearance ,9,12,13.Our case fulfills all radiologics criterias of an arrested pneumatisation of sphenoid sinus. In some cases the full range of radiological features are not found requiring regular follow-up.4Arrested pneumatization of the skull base is an anatomical variant that most commonly occurs in the sphenoid sinus. The diagnosis is made when the lesion is located in a site of normal pneumatization, especially the sinuses. If the lesion characteristics are complete the diagnosis of arrested pneumatization can be made with confidence, eliminating the need for additional interventions such as biopsy or surgery. Occasionally, the lesion may not fulfil all these radiological criteria, in which case serial imaging surveillance is required to establish benignity.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.The authors declared that this study has received no financial support.Harouna Maman Siradji: Corresponding author, writing the paper.Fadoul Achta: writing the paper.Aghrib Fatiha: writing the paper.Boussa Tressia: writing the paper.Belgadir Hasna: Correction of the paper.Elbenna Naima: Correction of the paper.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.1. Name of the registry: researchregistry2. Unique Identifying number or registration ID: 7110.3. Hyperlink to your specific registration (must be publicly accessible and will be checked):HAROUNA DJATAOU MAMAN SIRADJI.Authors of this article have no conflict or competing interests. All of the authors approved the final version of the manuscript.


We are presenting to you an magnetic resonance imaging (MRI) finding of a 27-year-old male who presented to us with complains of gradually progressing pain over the neck along with radiation to the upper extremity with more involvement of the right side than the left. He also complained of insidious onset of weakness in the upper extremity. Magnetic resonance imaging findings revealed T2 hyperintense mass (red arrow) in the intradural extramedullary mass involving the C3-C4 cervical vertebrae and the intervertebral disc in between, highly suggestive of neurofibroma. Spinal neurofibromas are frequently observed over the cervical cord and are usually asymptomatic. Our patient showed mild symptoms, probable reasons might be the slow growth rate of neurofibroma. He was referred for physiotherapy for further opinion. On physical examination, he had weakness in upper limb movement (MRC grade 2+) and weak grip strength. Surgery was the treatment of choice for him and he underwent surgical excision of it along with pre-operative physiotherapy management for 5 days. Post-surgery, there was an enormous improvement in his symptoms i.e. residual weakness persisting without any pain. He was prescribed strengthening and gripping exercises. Before discharge, he had a grade of 4+ on the MRC grading scale. Early medical and surgical management along with pre- and post-operative rehabilitation is the road to recovery in such patients.


Cerebellar liponeurocytoma is a rare benign neoplasm of the central nervous system, which arises mainly in adult patients with only 3 cases reported in children. Due to its rarity, the diagnosis and treatment strategies for cerebellar liponeurocytoma remain unclear. The purpose of this study was to explore the epidemiology, clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in juveniles.A 5-year-old boy was admitted to the department of neurosurgery due to a 5-month history of headaches, nausea, vomiting, dizziness, dysphoria, as well as visual blurring associated with the peak of the headache. Magnetic resonance imaging showed a 4.9×5.4×6.2 cm mass located in the fourth ventricle and cerebellar vermis combined with hydrocephalus and periventricular edema. The mass was completely removed, and pathological examination indicated a cerebellar liponeurocytoma of the World Health Organization Grade II classification.The present study was the first to report a cerebellar liponeurocytoma with total tumor resection and adjuvant radiotherapy in a pediatric patient. Total tumor resection and postoperative radiotherapy together with close and long-term follow-up seem to be the optimal treatment strategy for juvenile patients. However, the side-effect of radiation needs to be considered. Cerebellar liponeurocytoma (cLNC) is a rare tumor of the central nervous system (CNS) that mostly affects the adult population and is mainly located in the posterior fossa of the brain, with the cerebellar hemisphere being the most common anatomical location, followed by the cerebellar vermis –3. In 19th ventricle and cerebellar vermis of a 5-year-old boy and summarized all the reported cLNCs developed in juveniles. In addition, we reviewed the available medical and scientific literature, and summarized the radiological and pathological features of this rare tumor entity, with epidemiology, diagnosis, surgery, adjuvant therapy, and prognosis being discussed in detail.When reviewing all publications involving cLNCs that were retrieved from PubMed, only 66 cases were found, and almost all of them were sporadic cases. cLNCs in children are extremely rare, with only 3 reported cases –13. We hA 5-year-old right-handed boy was admitted to the neurosurgery department with 5-month history of headache, nausea, vomiting, dizziness, and dysphoria, as well as visual blurring associated with the peak of the headache. In nearly a month, the boy’s clinical manifestations gradually worsened. He complained of being unable to maintain balance while walking or standing. The patient had no similar clinical history previously reported in his family. Moreover, neurological examination showed that the patient had bilateral grade II papilledema, gait ataxia, and was positive for the finger-nose and the Romberg test.th ventricle of the brain, combined with unclear edges and significant dilation of bilateral and 3rd ventricles. Magnetic resonance imaging  scan showed a heterogeneous dense large mass in the 4ing MRI; Figure 1A preoperative diagnosis of medulloblastoma was made and the surgery was performed under preoperative and intraoperative neuronavigation together with electrophysiological monitoring of cranial nerves VII, IX, X, XI, brainstem, somatosensory, and muscle-evoked potentials. The patient underwent midline suboccipital craniectomy, C1 posterior arch excision, and total tumor resection in a lateral prone posture. The tumor appeared pinkish, solid, and bloody. The interface between the tumor and healthy parenchyma was not as sharply defined.After combined diagnosis of two chief pathologists at our hospital and the Xuanwu Hospital Capital Medical University, China, the pathological diagnosis was cLNC, with WHO Grade II. Upon histopathological examination, hematoxylin and eosin-stained paraffin sections showed predominantly small to moderately, round or ovoid tumor cells, with low mitotic activity. These tumor cells grew in a sheet shape with clusters of fat cells were observed in the center   , left ceis (2/4) , and rigis (2/4) . The onsis (2/4) .The clinical presentation in children is not specific and depends on the tumor’s location and mass effect Table 1.On CT scans, cLNCs usually present as well-demarcated and heterogeneous hypodense or isodense solid masses, with or without the presence of microcysts in the tumor , 20, 21.Pathological examination is the most important tool for diagnosing cLNCs. We summarized the pathological characteristics of the four pediatric cases studied in Ki-67 is a nuclear protein that is associated with cellular proliferation . Higher th ventricle, which was preoperatively diagnosed in two cases of pediatric patients. Medulloblastoma always shows foamy histiocytes, primitive neuroectodermal cells, a high mitotic rate, and a high Ki-67 index , if the pathological examination presented a low Ki-67 index, and no atypical histopathological findings were observed, such as necrosis, high mitotic activity, and prominent vascular hyperplasia, periodical MRI surveillance for possible recurrence was among the primary treatment principle, and radiotherapy could be avoided , 16, 29.Given that there are only four known cases, the prognosis of children with cLNC is difficult to assess. Although the cLNCs were classified as WHO Grade II tumors, recurrence rates are extensively reported in adult cases, which is the reason for the WHO grade classification upgrade in 2007. In pediatric cases, only one case of recurrence was reported by Jouvet et al. with the main reason of ITR . ITR andIn summary, cLNC is rarely reported in the literature and, with only 3 known cases, is extremely rare in pediatric patients. Thus, more cases describing cLNC and long-term follow-up studies are warranted to fully understand cLNC in the juvenile population. Therefore, because of these limitations, our present case report might represent an additional reference among the few available that might serve as a potential guide for clinicians and clinical studies.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.The studies involving human participants were reviewed and approved by Ethics Committee of the First hospital of Jilin University. Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.CD, YJ and LZ made study design, data collection, data analysis and interpretation, and composed the manuscript and literature review. YL, CD and YW were the surgeon that performed the surgery and did data collection, data analysis, and interpretation. YS and YB made English and grammar corrections, critical revisions, and approved final version. YL had the acquisition, analysis or interpretation of data for the work, revising it critically for important intellectual content, final approval of the version to be published, and agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors agree to be accountable for the content of the work. All authors contributed to the article and approved the submitted version.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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


A 9-year-old male with history of mixed hearing loss presented with petrous apex lesion following episode of meningitis. Serial imaging revealed persistence of the lesion necessitating biopsy to rule out malignancy. Biopsy revealed inflammatory changes. The management of petrous apex lesions following meningitis can be conservative but repeat imaging is necessary to rule out progression and to rule out neoplastic process. We describe a case of petrous apicitis following bacterial meningitis in a child presenting with profound sensorineural hearing loss and progressive, bony erosions on imaging that prompted surgical intervention. Petrous apicitis is commonly associated with lesions of the fifth and sixth cranial nerves. However, sensorineural hearing loss and facial nerve paralysis are rare but important presenting symptoms for the clinician to remain aware of. Although petrous apicitis is traditionally described as an acute process, it has the potential to take an indolent course. These features, coupled with its capacity for bony destructive changes, may mimic a neoplastic process.A 9-year-old male was admitted to the pediatric intensive care unit with group A beta-hemolytic streptococcal meningitis and right-sided otorrhea. He had a history of recurrent acute otitis media and tympanostomy tubes, the most recent of which were placed 1 month prior to admission. At baseline, the patient had symmetric mild to moderate sensorineural hearing loss secondary to STRC-related non-syndromic deafness.On admission, audiogram revealed new onset profound, right-sided sensorineural hearing loss. On day two of admission, the patient developed right facial nerve weakness, House-Brackmann grade III. magnetic resonance imaging (MRI) taken on day three revealed non-coalescent mastoiditis. Management consisted of intravenous cefepime, metronidazole and vancomycin; ciprofloxacin-dexamethasone ear drops; and oral dexamethasone. He was discharged on Day 8 with a peripherally inserted central catheter (PICC) line and prescribed a 14-day total course of antibiotics, ciprofloxacin-dexamethasone ear drops and 9 days of oral steroids with a taper.Serial MRIs were conducted following discharge. MRI at 1 month showed a contrast-enhancing mass in the right internal auditory canal with enhancement of the right petrous apex and associated labyrinthitis. An MRI at 2 months showed persistence of the lesion, whereas computed tomography (CT) showed new osseous erosion and irregular lucency within the posterior cortex of the petrous apex  and 2. GAt surgery, the petrous apex was accessed using a translabyrinthine approach. Biopsies taken from the right vestibular nerve and the internal auditory canal showed no evidence of malignancy. Intraoperative findings included extensive scar tissue formation in internal auditory canal and labyrinthitis ossificans with a non-patent labyrinth. Gram stain, acid fast bacillus (AFB), fungal cultures and anaerobic cultures were negative. Clinically, the patient’s recovery was unremarkable.Petrous apicitis is suppurative inflammation of the petrous apex with parallel findings in the middle ear and mastoid . AlthougPetrous apicitis is classically associated with Gradenigo’s syndrome, first described in 1907 as otorrhea, ipsilateral sixth nerve palsy and retroorbital pain . This trProfound sensorineural hearing loss, as seen in our patient, is rarely described in older case reports as a prominent feature of petrous apicitis . In the Finally, although petrous apicitis is most commonly described in its acute form, it has the potential to manifest as a chronic, indolent process that mirrors the histopathologic changes seen in chronic middle ear disease , 10–12. Sensorineural hearing loss and facial nerve paralysis are important presenting symptoms of petrous apicitis. Furthermore, petrous apicitis has the potential to mimic a neoplastic process. These characteristics should be considered in the differential diagnosis of pediatric petrous apex lesions when the clinical presentation is appropriate.


Ileocecal valve atresia is the most uncommon yet remarkable form of the atresia found within the gastrointestinal system. We report a case on this rare entity with few cases documented in the literature to date. In our case, a one-day-old full-term male infant who developed the signs of intestinal obstruction was eventually taken for emergency laparotomy. The atretic area found intraoperatively was removed followed by the creation of an anastomosis. The patient recovered well postoperatively and continues regular pediatric follow-ups. Colonic atresia is a very rare form of intestinal obstruction in neonates, presenting with failure to pass meconium, vomiting and abdominal distension with an incidence rate of 1.8 to 15 per 100 live births in the general population .About half of the intestinal atresia have been documented in the jejunum and ileum . AlthougWe report a case of a one-day-old male infant diagnosed with intestinal atresia that involved the complete ileocecal junction, with no connection between the small and large intestine with the presence of an appendix. We further intend to discuss the reasons for the conclusions that were made intraoperatively and the potential outcome of the patient.A one-day-old male neonate presented with abdominal distension, failure to pass meconium, and bilious vomiting. He was born at 38 weeks gestation by normal vaginal delivery to a multiparous 29-year-old woman and weighed 2.550 Kilograms at birth. There was no remarkable family history.The prenatal ultrasound scan did not reveal any mass fetal anomaly and no evidence of polyhydramnios. An X-ray of the abdomen revealed dilated loops of the bowel with the presence of fluid on the right side and the absence of the bowel gas pattern in the lower abdomen Figure .Based on X-ray findings, small bowel obstruction was diagnosed, and he was immediately taken for emergency laparotomy.During surgery, the right transverse incision in the infraumbilical region was given. The bowel was explored, and it was seen that the terminal ileum was markedly dilated, and the cecum was small and collapsed. There was no connection between the terminal ileum and cecum. The appendix was seen attached to the cecum, which was removed. There was also a V-shaped defect within the mesentery supplying the ileocecal region. We removed the grossly distended last 5 cm of the distal ileum. We performed an end to side ileo ascending anastomosis after confirming the absence of a distal obstruction by irrigating the lumen of ascending colon with normal saline. The mesenteric defect was closed with absorbable sutures Figures -3.Histopathology of resected ileum showed bowel mucosa with vascular congestion, mild to moderate edematous changes of submucosa and serosa. The appendectomy specimen showed submucous serosal vascular congestion. He had an uneventful hospital course, and the incision was healing well.He passed meconium on postoperative day 2 and was restarted on oral feeding on a postoperative day 4. He was seen for his one-month follow-up, and examination showed his development on the growth chart was above the 25th percentile and was satisfactory.Ileocecal atresia is the most uncommon form of colonic atresia. The ileocecal valve creates a tonic pressure blocking the reflux of intestinal contents and also shows a response to various pharmacological and physiologic changes . AtresiaIn the cases documented in the literature, the neonates present mostly with the signs of intestinal obstruction. The clinical manifestations can include abdominal distension, bilious vomiting, and unable to pass meconium -9. InitiPre-operatively, the management comprises mainly supportive treatment with IV fluids and decompressive measures in case of obstruction. However, surgical procedure mainly depends on the intraoperative findings. In one of the case reports, the central part of the ileocecal valve was atretic with the valve repair . In our In conclusion, ileocecal valve atresia is a very rare anomaly with very few cases available in the literature. It commonly presents in neonates with signs of intestinal obstruction. Pre-operatively the diagnosis can be challenging, but imaging studies like plain X-ray abdomen, upper gastrointestinal contrast study, and contrast enema can help with the diagnosis. Intraoperatively, resection of the atretic colon along with ileocolic anastomosis with or without appendectomy has been the accepted management. We, therefore, intend to contribute it to medical literature along with a brief discussion on this rarest topic.


Venous malformations (VMs) may manifest clinically in a broad spectrum. Most VMs are sporadic with previous studies reporting less than 1.2% to be inherited. Conversely, multifocal lesions, such as glomuvenous malformations (GVMs), which have glomus cells in their vascular walls, have been reported to have a frequency of inheritance of 63.8%. Both VMs and GVMs may occur due to sporadic mutation and must be differentiated clinically because this will dictate their proper treatment. Sporadic GVMs involve skin and subcutis, with bluish-purple coloration, are painful to compression, and have no radiographic evidence of phleboliths. Previous studies have demonstrated that VMs are almost always associated with a single lesion that is nontender to compression and are often able to be diagnosed by the presence of phleboliths on radiographic imaging. We present a case of a 14-year-old right-hand-dominant male who presented with two distinct VMs on the dorsum of the right index finger at the proximal and middle phalanges. A previously biopsied lesion overlying the ipsilateral olecranon, which was reported as a possible glomus tumor versus vascular malformation, was present as well. Based on history, physical examination, multicentric presentation, and radiographic findings, the presumptive diagnosis was that the lesions were GVMs. However, after surgical excision and histopathologic examination, the lesions were determined to be VMs because of the absence of glomus cells. Due to the difference in treatment modalities for VMs and GVMs, the ability to accurately diagnose these lesions clinically is essential. This case represents an anomalous presentation of multiple venous malformations occurring in two distinct locations in a 14-year-old boy. Vascular anomalies are often classified into two distinct categories, tumors versus malformations, based upon clinical and histologic characteristics. While proliferating endothelium is suggestive of a vascular tumor, malformations are described as structural anomalies . A varieVMs are present at birth and described as blue, soft, compressible lesions that may contain palpable calcified phleboliths . Errors In contrast, GVMs are due to a loss of function mutation in the glomulin gene. GVMs are characterized by abnormal glomus cells along ectatic veins most commonly manifesting as multiple, small <5 cm) lesions involving the skin and subcutaneous tissue  cm lesio. While VDifferentiating between GVMs and VMs is of particular importance when it comes to treatment strategies and response. VMs are often difficult to excise completely due to the invasion of surrounding tissues and deeper structures, but they may be improved by external compression and are often shrinkable with sclerotherapy. There is no role for sclerotherapy or compression garments in GVMs; however, they are often easily resectable due to their more superficial nature .This report demonstrates an anomalous case of VMs in an adolescent boy presenting with multiple, sporadic VMs that were unable to be differentiated clinically.This article was previously presented as a virtual poster presentation at the Mountain West Society of Plastic Surgery Annual Meeting on March 6, 2021.An otherwise healthy, 14-year-old, right-hand-dominant male presented to our clinic for the evaluation and treatment of two distinct, blue, subcutaneous, non-tender, mobile, ovaloid nodules overlying the dorsal aspect of his right index finger Figure .He endorsed a history of multiple lesions being present in different locations since birth that had been followed up clinically into adolescence. Several of these lesions were noted to regress on their own, but others grew proportionally with the patient. The lesions that exhibited proportional growth with the patient included the two on his right index finger, as well as one overlying the ipsilateral olecranon Figure . The lesBased upon the superficial nature of the lesions, as well as clinical suspicion for a GVM, the patient was not considered a candidate for sclerotherapy. He underwent successful debulking of the right index finger lesions Figure , and surHistopathology demonstrated nodules composed of thin-walled vessels containing erythrocytes within the subcutis surrounded by uninvolved fibroadipose tissue Figure . A few sBased upon these results, the patient was diagnosed with a VM. Following surgery, the patient had an uneventful postoperative course with full range of motion in his index finger at the two-month follow-up Figure .VMs are present at birth but can be difficult to diagnose at the time until the child has grown up and reached adulthood. These lesions present clinically as blue, soft, compressible lesions that may contain hard calcifications, known as phleboliths, caused by the stagnation of blood in these lesions.Almost all VMs are sporadic, with one study reporting that 1.2% of VMs were inherited. GVMs, on the other hand, are typically familial, with one series reporting a frequency of inheritance at 63.8% . Until rVMs and GVMs are histologically and molecularly distinct vascular anomalies, and due to differences in treatment modalities, clinical criteria have been set forth to better distinguish between the lesions to direct management. The majority of VMs can be diagnosed clinically through patient history and physical exam . VMs areVMs are known to cause a chronic coagulopathy, and as such, are capable of causing thrombosis or bleeding. Due to this chronic coagulopathy, laboratory studies including D-dimer and fibrin levels can be useful when evaluating for a possible VM as these are often elevated . PreviouThe ability to differentiate between GVMs and VMs is paramount in planning successful treatment. While compression garments often improve the pain associated with VMs located in extremities, they can worsen the pain due to a GVM. Surgical excision of GVMs is generally quite successful, while complete excision of a VM is often difficult due to the extensive involvement of tissues and deep structures surrounding the lesion . The effThis case presented here demonstrates an anomalous presentation based on previously defined clinical criteria of a VM. This adolescent male presented with multiple lesions involving the right, dorsal index finger, as well as another overlying the right olecranon. Further evaluation with ultrasound and radiographic imaging suggested GVMs with slow-flow lesions and no phleboliths noted. Based on these suggested findings of GVMs, the patient was not considered a candidate for sclerotherapy. Surgical excision of these superficial lesions with no surrounding tissue involvement was easily accomplished, further suggestive of GVMs. Upon histopathological examination, however, no glomus cells were seen, and the lesions were determined to be VMs. As such, this case represents an unusual presentation of VMs occurring in a 14-year-old male with notable deviation from criteria previously put forth to distinguish between VMs and GVMs.Due to the wide variety of presentations and distinct differences in the management of VMs versus GVMs, clinical diagnosis is essential. VMs tend to be found as solitary lesions, that may involve deeper structures, and are not painful to compression. Phleboliths are often present on radiographic imaging. GVMs tend to occur as multiple lesions involving the superficial skin and subcutis that are painful to compression. Glomus cells are demonstrated on histopathology and there is no evidence of phleboliths on radiographic imaging. This case represents a mixed presentation and deviation from the clinical criteria previously used to differentiate a sporadic VM from a GVM.


Amyand’s hernia is an extremely rare condition in which the appendix is positioned in the inguinal hernia sac. Acute appendicitis is much less common in this situation, especially in the elderly population, and few reports are found in the literature. Whether the presence of the appendix within the inguinal sac increases the chance of developing appendicitis or the relationship is coincidental is unclear. Majority of cases reported are in the male pediatric population. The varying surgical approaches are entirely case-specific without much standardization. Perioperative planning remains difficult in all cases as this condition is exceedingly rare, especially outside of the pediatric population. We present such a case with the hope that we increase awareness about this uncommon condition, in which preoperative diagnosis and planning remains difficult. Inguinal hernias account for 75% of abdominal wall hernias. Incarceration of the appendix in the inguinal sac is known as Amyand’s hernia and can present with or without acute appendicitis. It was first described by Claudius Aymand in 1735 in an 11-year-old boy, and this condition remains rare with an incidence of around 0.5-1% . WhetherWe presented this rare case at the American College of Gastroenterology conference as a poster in October 2021.A 97-year-old male with a history of advanced dementia, benign prostatic hypertrophy, urinary stones, and 10-year history of bilateral non-reducible inguinal hernias extending down into his scrotum who presented to the emergency department (ED) with right-sided abdominal pain. In the ED, the patient was noted to be hemodynamically stable. Physical examination was notable for soft, non-distended abdomen with significant tenderness to palpation along the right lower quadrant, and two large bilateral inguinal hernias, and the patient was non-cooperative to reduction at the time. Lab work was without any notable leukocytosis or lactic acidosis. Computed tomography (CT) scan performed at the time demonstrated a large left-sided hernia containing the sigmoid colon without evidence of stranding, inflammation, or obstruction. The right side was also noted to have a large hernia with the cecum and appendix in the sac associated with stranding and some pericolonic fluid collection (Figure Amyand’s hernia is present three times more likely in the pediatric patient due to a patent processus vaginalis and appears more commonly in males . It typiWith the increased use of CT scans, this diagnosis is more frequently being made preoperatively, allowing for both safer preoperative planning and intraoperative decision-making . CT scanAmyand's hernia, although quite rare, should remain on the differential in patients presenting similarly to ours. While the patient will ultimately require surgical interventions, it is important to keep in mind the role of pre-operative imaging that may play a big role in management of such patients prior to their surgery and lead to improved preoperative planning and intraoperative decision-making. Given its rarity, it is difficult to standardize an approach applicable to most cases. Especially in the geriatric population, perioperative planning will aid in choosing the least invasive approach, which can optimize post-operative pain control and prevent delirium in this specific patient population. The use of perioperative imaging may be the key to personalizing each case to the best of the physician’s abilities. Early diagnosis and awareness of such condition will also aid in timely intervention with proper planning before complications may arise. We hope that by presenting this case we may allow everyone, including the emergency medicine physician and hospitalists, to keep this condition in mind with prompt surgical referral.


A 61-year-old woman presented to the hospital with a clinical picture consistent with acute appendicitis. During surgery, the appendix was found to be gangrenous and involved the appendiceal base, so an ileocecectomy was performed. Pathology revealed an incidental neuroendocrine tumor of the terminal ileum involving five of nine lymph nodes. The patient later developed mesenteric venous thrombosis but was diagnosed and treated promptly, and she is now doing well. There have been previous reports of small bowel neuroendocrine tumor resulting in bowel ischemia, usually due to fibrosis which can result in obstruction of the mesenteric vessels. However, this is the first known report of a small bowel neuroendocrine tumor presenting with appendicitis, which most likely was from an ischemic etiology. This case also demonstrates the importance of a high index of suspicion for mesenteric ischemia in patients with small bowel neuroendocrine tumor who present with acute abdominal pain. Small bowel neuroendocrine tumors are rare, and symptoms are often vague . Unfortu2. She was a non-smoker and had a family history of breast cancer and heart disease. She was afebrile and hemodynamically stable. One examination, she had bilateral lower quadrant abdominal tenderness, right greater than left. Workup revealed a white blood cell count of 13.7 thousand and a lactic acid of 1.5 mmol/l. Computed tomography (CT) scan of the abdomen and pelvis revealed an inflamed appendix with mesenteric fat stranding, consistent with acute appendicitis.The patient is a 61-year-old female who presented to the hospital with a 2-day history of right lower quadrant abdominal pain. She had a history of hypertension, hypercholesterolemia and a hysterectomy but was otherwise healthy. Her body mass index was 31.6 kg/m2, and Ki67 proliferative index was 3%. Five of nine lymph nodes were positive for metastatic neuroendocrine tumor with extranodal extension of the tumor and perineural invasion. Surgical margins were clear. Tumor, node and metastasis (TNM) histopathologic stage was pT2, N1. The patient developed a postoperative ileus but this resolved and she was discharged on postoperative day 7.The patient was taken to the operating room for a laparoscopic appendectomy. During surgery, the appendix was noted to be gangrenous and perforated. Because the inflammation involved the entire appendix including the appendiceal base, decision was made to perform an ileocecectomy. Pathology revealed a gangrenous perforated appendix with periappendiceal abscess along with a well-differentiated, 1.2 cm neuroendocrine tumor of the terminal ileum  and 2. MTwo weeks after discharge, the patient returned with crampy abdominal pain. She was moderately tender on examination. White blood cell count was 15 000 and lactic acid was elevated at 3.5 mmol/l. CT scan of the abdomen and pelvis revealed an intraluminal filling defect of the superior mesenteric vein and portal vein not present on prior CT, consistent with acute venous thrombus . There wAfter discharge, the patient had a PET scan that was normal. Her urinary 5-HIAA was 2.1 mg/24 hours, and her chromogranin A levels have remained normal. She was not placed on any adjuvant treatment. It has now been 2 years since she first presented and she is doing quite well.Neuroendocrine tumors are a rare, heterogeneous group of neoplasms. The incidence is about one in 100 000, although this may be increasing . These tThe classic teaching regarding the pathophysiology of acute appendicitis has been an etiology of luminal obstruction, which leads to increased intraluminal pressure. This leads to progressive inflammation, dilation and subsequent perforation. However, recent studies have questioned whether this sequence is necessarily true in all cases. There could potentially be multiple possible etiologies for acute appendicitis, including ischemic and neuroimmune mechanisms . In our Mesenteric venous thrombosis has been reported in patients with neuroendocrine tumors, either from direct encasement of vessels which leads to venous stasis or due to the cytokine response that leads to fibrosis. In the literature, there have also been several reports of mesenteric venous thrombosis as a result of acute appendicitis itself , 8. It mThe symptoms of small bowel neuroendocrine tumors can be quite vague; this can lead to a delay in diagnosis, although many are found incidentally. A high index of suspicion for mesenteric ischemia should be held for patients with small bowel neuroendocrine tumor who present with acute abdominal pain.None declared.None.


Magnetic resonance imaging (MRI) provides high-contrast resolution and is the preferred diagnostic tool for neurological disease. However, long exam times discourage MRI in emergency settings, and high-field MRI scanners (1.5-3T) require dedicated imaging suites. New, portable low-field-strength MRI machines (0.064T) have lower resolution than fixed MRI, but do not require restrictive environments or intrahospital transport. We present a case of a 78-year-old male with altered mental status who underwent 0.064T portable MRI and fixed 3T MRI exams in the emergency department. Imaging showed no evidence of acute infarction or intracranial lesions. The 0.064T images were of poor quality relative to 3T sequences, but the results of the portable MRI agreed with the conventional 3T MRI and a computed tomography scan from the same day. The compatible imaging results suggest that portable, low-field MRI can aid in neurological diagnosis without transporting patients to the MRI suite. Further studies should expand this comparison between high- and low-field MRI to better characterize the role and clinical applications of point-of-care MRI. Magnetic resonance imaging (MRI) has high-contrast resolution and is the preferred imaging modality for neurological disease ,2. ThereIntrahospital transport increases the risk of patient harm and transmission of infection, resulting in additional costs ,4. The tThe recent development of low-strength magnetic field technology for MRI (0.064T) has enabled rapid neuroimaging at the point of care (POC). These portable machines, currently only available for brain imaging, have lower resolution than conventional, fixed MRI, but can be used in the emergency department (ED) and intensive care unit. They also have minimal risk of magnetic projectile accidents, requiring fewer safety precautions and less training for technicians ,13. The A 78-year-old man with end-stage renal disease presented to the ED with altered mental status. His daughter reported that he had two nights of hallucinations that progressed to whole-body shaking that morning. The patient’s baseline was alert and oriented to person, place, and time. He missed hemodialysis on the date of admission, but there were no other recent changes to his health. The patient presented with a heart rate of 75 beats per minute, blood pressure of 131/95 millimeters of mercury, respiratory rate of 18 breaths per minute, oral temperature of 36.4 degrees centigrade, and an oxygen saturation of 99% on room air. A right-sided facial droop was noted. No other significant findings were recorded from the physical or neurological exams. His speech was unaffected, and he had no other focal neurological findings. A CT of the head without intravenous (IV) contrast was performed 30 minutes after arrival in the ED. The exam was normal, with no intracranial hemorrhage, midline shift, herniation, or hydrocephalus. There was mild to moderate atrophy and white matter attenuation, as well as intracranial vascular calcifications and trace fluid in the left mastoid air cells. The paranasal sinuses were clear, with a normal appearance of the surrounding bone and soft tissue.After consultation with neurology, a portable MRI brain scan was ordered without IV contrast and performed 6 hours after arrival. The scan revealed scattered subcortical and deep T2/fluid-attenuated inversion recovery (FLAIR) hyperintense foci, along with prominent sulci and ventricles Figure . PerivenThe 3T scan was negative for acute infarction, midline shift, herniation, and hydrocephalus. T2/FLAIR hyperintensities in the central pons and white matter were noted, along with prominent ventricles Figure . MultiplAfter the CT and MRI results were negative for structural causes, the patient was admitted to a neurology stepdown unit the same night for encephalopathy.To our knowledge, this is the first report comparing fixed and portable MRI in an emergency setting in the same patient on the same day for a potential acute neurological event.Low-field MRI machines are compatible with nearby ferromagnetic materials and can operate on standard 120-volt electrical power, allowing use outside of the MRI suite. Metal objects and standard ventilators, pumps, and other life support devices are permitted close to the machine . This peWhile the 0.064T images were of poor quality relative to the 3T images, some of these were due to patient movement, rather than inherent differences in resolution. The patient was given sedation for the standard machine, but not for the POC MRI exam.The results of the POC MRI agreed with the fixed 3T MRI and CT scans. This suggests that the new machine can aid in neurological diagnosis, without the limitations of conventional scanners. Additional studies are necessary to determine the diagnostic capacity of portable MRI for patients with positive findings. The concordant results between 0.064T MRI, 3T MRI, and CT readings in this case support the use of POC MRI for the detection of neurological pathology in the ED. As low-field MRI machines provide low-resolution images, follow-up study with a 1.5-3T MRI may be required in the case of movement artifacts or nondiagnostic images. The lower cost of POC MRI could also enable imaging in clinics or under-resourced institutions. We plan a prospective case series at this academic medical center for a more representative comparison between high- and low-field MRI technology.


To describe a case of complete spontaneous regression of a middle ear melanoma.We present a case of a 68-year-old man with complaints of unilateral hearing loss and an ipsilateral facial nerve paresis. Radiological and histopathological examination revealed a cT4bN0M0 mucosal melanoma of the middle ear.The patient underwent a subtotal petrosectomy and postoperative radiotherapy.Computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography/computed tomography with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET-CT), and histopathological examination.After subtotal petrosectomy, histopathological examination of the resection specimen showed only fibrosis and a histiocytic and clonal T-cell infiltration, but no residual melanoma at the primary tumor site, consistent with spontaneous tumor regression. Follow-up MRI scanning 6 and 12 months after radiotherapy showed no signs of tumor recurrence.This case describes the concept of spontaneous regression of a mucosal melanoma of the middle ear. Spontaneous tumor regression at this location has not been described before. A 68-year-old male patient was referred to the department of otorhinolaryngology because of left-sided, progressive hearing loss. He was suspected of having an otitis media with effusion. However, routine clinical examination after 3 months showed an atypical granulomatous presentation of the tympanic membrane and therefore a biopsy was taken. Histopathological examination showed a highly atypical and mitotically active stromal melanocytic proliferation, positive for S100, Melan-A, and MITF  was almost in remission. The patient had no complaints of otalgia or otorrhea and there was no history of an ear trauma. He had a history of hypertension, atrial fibrillation, coronary artery disease, depression, and obstructive sleep apnea syndrome. There was no family history of melanoma. At physical examination, inspection of the facial musculature showed a mild degree of asymmetry of lip closure (HB II/VI). Otomicroscopy showed a mass at the level of a seemingly thickened (or absent) tympanic membrane with blue discoloration at the posterior upper and lower quadrant post-biopsy. This mass was non-pulsatile and the usual anatomical landmarks such as the annulus fibrosus tympanicus and the tympanic membrane could not be identified. The Rinne test using a 512-Hz tuning fork was negative on the left side and the Weber test was lateralized to the left ear. Further physical examination including nasal endoscopy and palpation of the neck showed no abnormalities. The audiometry examination showed a left-sided mixed hearing loss of 92 dB HL with maximum speech recognition of 88% at 110 dB SPL.Computed tomography (CT) and magnetic resonance imaging (MRI) revealed soft tissue invasion of the left mastoid bone and the middle ear cavity, completely embedding the ossicular chain Fig. . DehiscePositron emission tomography/computed tomography with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET-CT) demonstrated intense FDG-uptake at the left middle ear cavity indicative for a malignant tumor based on the histopathological diagnosis Fig. A. Only mThe tumor was staged according to the 8th TNM Classification for Mucosal Melanoma of the Head and Neck as a cT4bN0M0 malignant mucosal melanoma originating from the left middle ear cavity .One month after presentation at our outpatient clinic, a subtotal petrosectomy was performed. During surgery, suspicious brown-colored granulation tissue was obtained from the middle ear cavity and mastoid bone. Furthermore, several defects of the tegmen tympani were present at the level of the middle and posterior cranial fossa. The granulation tissue was removed from the mesotympanum, hypotympanum, sinus tympani, oval window, antrum, mastoid cavity, and perilabyrinthine cell tracts. Microscopically radical resection of the aberrant tissue was not possible due to the extensive spread. The middle ear cleft was obliterated with abdominal fat.+, partly CD4+, partly CD8+ T-cell infiltrate, and CD68+ histiocytic infiltrate was observed within a large area of fibrosis and dilated blood vessels. Additional melanocytic stainings  were all negative  and a FDG-PET-CT follow-up scan revealed minimal soft tissue induration at the petrosectomy site and mild FDG uptake with no evidence of residual tumor Fig. B. Since Malignant melanoma develops through the neoplastic transformation of pigment-producing cells, usually located in the epidermis and dermis . Accumul+ and CD8+ T-cells directed against melanoma cells  performed on tissue from this area showed a highly clonal T-cell response, further supporting the phenomenon of regression  have been reported in the literature ,10,14. O


Herein, we report a case of tuberculous severe acute colitis. It is a rare and life-threatening condition. Our literature review found only five published cases. It occurs generally in immunocompromised patients. Extended colonic inflammation seems to be the main predictive factor of death. Moreover, an early diagnosis and rapid onset of antituberculous treatment are mandatory to save the patient's life.Herein, we present a case of tuberculous severe acute colitis with a review of the reported cases. The patient presented with a severe and idiopathic acute colitis. He was put on broad-spectrum antibiotics and intravenous corticosteroids. At day two, he developed septic shock and colic perforation. Colectomy was performed. Microbiology investigation and pathology examination confirmed tuberculous colitis.Tuberculous severe acute colitis occurs generally in immunocompromised patients. Extended colic inflammation seems to be the main predictive factor of death. Moreover, an early diagnosis and rapid onset of antituberculous treatment are mandatory to save the patient's life. However, diagnosis is difficult as symptoms aren't specific. Microbiology and pathology were compulsory to retain colic tuberculosis in all the reported cases.Tuberculous severe acute colitis is a challenging and life-threatening condition. It usually occurs in immunocompromised patients. Abdominal CT-scan may evoke the diagnosis. Microbiology and pathology are mandatory to retain the diagnosis. Early diagnosis and onset of antituberculous treatment are compulsory to save the patient's life. •Diagnosis of tuberculous severe acute colitis is difficult.•It usually occurs in immunocompromised patients.•Surgery is mandatory if perforation occurs.•Early diagnosis and onset of antituberculous treatment are compulsory to save the patient's life. Haemoglobin was at 9 g/dL. Liver and renal functions were normal. Chest X-ray was normal. Colic diameter was normal on abdominal X-ray. On the emergency CT-scan, the colic and upper rectal walls were thickened and intensively enhanced after contrast fluid injection. There were also multiple necrotic lymph nodes and mesenteric hyperemia. There wasn't any intra-abdominal fluid or pneumoperitoneum nor sclerolipomatosis was found. Rectoscopy revealed large and deep ulcerations of the upper rectum with mucosal friability and bleeding and loss of the typical vascular pattern. Biopsies weren't possible to avoid iatrogenic colic perforation. The patient was put on broad-spectrum antibiotics and intravenous corticosteroids. At day two, he developed a septic shock and abdominal guarding. So, we decided to operate. We conducted a mid-line laparotomy and discovered a widespread purulent peritonitis with pseudomembranes. The colon and rectum were thickened, and the colon was perforated at the splenic fixture (A 45-year-old man with no past medical history presented to our emergency department for acute abdominal pain, fever at 38,9 °C, chills and bloody diarrhea that lasted for two days. Before admission, he never experienced diarrhea nor bloody stools. He reported a recent weight loss of 7 kg in the last two weeks. Heart rate was at 100 beats/minute, blood pressure was at 110/70 mmHg, respiratory rate was at 20/minute. Abdominal examination found a diffuse tenderness without guarding. C reactive protein was at 40 mg/L. WBC was at 10200 cells/mm fixture . There a fixture . Mycobac3In this paper, we presented an uncommon presentation of tuberculosis: severe acute and isolated colitis. Tuberculosis affects the digestive tract in 2% of cases . UsuallyBased on our review of published cases, we found that this condition is most common in immunocompromised patients. Extended colic inflammation seems to be the main predictive factor of death. Moreover, an early diagnosis and rapid onset of antituberculous treatment are mandatory to save the patient's life. However, diagnosis is difficult as symptoms aren't specific. Microbiology and pathology were compulsory to retain colic tuberculosis in all the reported cases. Presenting with an acute and idiopathic severe colitis, our patient was given intravenous corticosteroids. This contributed considerably to septic shock and colic perforation.Retrospectively, we think that tuberculosis could have been evoked in our case. Indeed, abdominal CT-scan revealed multiple necrotic lymph nodes as usually seen in intestinal tuberculosis. Furthermore, the surgical specimen contained a necrotic cheese-like material in the center, which was suggestive of tuberculosis.4Tuberculous severe acute colitis is a challenging and life-threatening condition. It usually occurs in immunocompromised patients. Abdominal CT-scan might evoke the diagnosis. Microbiology and pathology are mandatory to retain the diagnosis. Early diagnosis and onset of antituberculous treatment are compulsory to save the patient's life.No source has funded this manuscript.All the authors have read and complied with the policy of the journal on ethical consent.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.Not commissioned, externally peer-reviewed.Haddad Anis: Writing – original draft.Sebai Amine: Writing – original draft.Chelly Beya: Writing – original draft; Data interpretation of the pathological findings.Maghrebi Houcine: Study concept and design.Chaker Youssef: Methodology.Montasser Kacem: Supervision.Not appliable.Sebai Amine.The authors declare that they have no conflict of interest.


Yolk sac tumor (YST) is the most common prepubertal testicular tumor. It is considered a subtype of non-seminoma germ cell tumor (NSGCT) that is presumed to have an aggressive behavior with high malignant potential, thus requiring multimodality treatment with resection and chemotherapy. Treatment is curative for the majority of patients, even the ones with relapse after a few years. Here, we describe for the first time an atypical case of YST recurrence 17 years after primary treatment of YST. This is a case of YST in a 32-year-old man who presented with a large cerebellar mass consistent with YST recurrence after being in remission for 17 years. He underwent suboccipital craniotomy and complete excision of the tumor, as evident on postoperative MRI with a plan for stereotactic radiosurgery with dose and fractionation determined by MRI at four weeks postoperatively. However, the four-week MRI postoperatively revealed a large mass that was engulfing the prior resection cavity, indicative of unusual rapid tumor recurrence despite evidence of complete resection. The highly aggressive nature of this tumor should prompt clinicians to consider chemotherapy and radiation earlier than four weeks postoperatively. Testicular germ cell tumors (GCTs) consist of seminoma and non-seminoma germ cell tumors (NSGCT). Endodermal sinus tumors or yolk sac tumors (YSTs), a type of NSGCT, are rare in adult patients, but they are the most common pediatric testicular tumors . MicroscA 32-year-old man presented to the emergency department with a two-day history of worsening headaches, dizziness, some vision changes, dysarthria, and memory disturbances. On admission, motor examination revealed right upper extremity dysmetria with no weakness or numbness and no cranial nerve palsies. He had a past medical history of YST of the right testes diagnosed at the age of 15. He was treated with right orchiectomy and retroperitoneal lymph node dissection (RPLND) as well as four cycles of BEP  and was in remission for 17 years. He presented to the emergency department five months prior to this admission with acute hypoxic respiratory distress and was then diagnosed with YST metastasis to the left hilar/posterior mediastinal and spleen. Subsequently, he was treated with four cycles of VIP  with ~30% treatment response consistent with RECIST , partial response and normalized alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (ß-hCG). On this admission, a computed tomography (CT) scan of the head without contrast revealed a large right cerebellar mass Figure . MagnetiThe patient underwent right frontal external ventricular drain (EVD) placement and a suboccipital craniotomy for transcerebellar tumor resection. The tumor was noted as very friable and was easily suctioned from the surrounding parenchyma. The friable tumor was completely removed with no intraoperative nor postoperative complications. Postoperative MRI with and without gadolinium of the brain revealed no evidence of enhancement to suggest residual disease Figure -2D. Pathological examination of the tumor specimen confirmed a metastatic malignant germ cell tumor consistent with his known prior primary diagnosis of YST. Postoperatively, AFP was elevated at 68.3 ng/mL, and ß-hCG was within the normal range, findings consistent with a YST. The patient was evaluated for the metastatic source of the brain lesion with full-body CT, which revealed a splenic hypodense lesion, measuring 1.8 cm previously noted to be 3.2 cm three months prior to the operation. The previously found left hilar mass noted five months prior was unchanged in size. There were no new intra-abdominal/pelvic metastases nor lymphadenopathy. The EVD was removed postoperatively, and he was subsequently discharged home after an uneventful postoperative course.He was evaluated three weeks later as an outpatient for consideration of radiation therapy. He stated he was recovering well and denied vision changes, focal weakness, focal sensory loss, pain, and bowel/bladder incontinence. Postoperative stereotactic radiosurgery was recommended with dose and fractionation determined after MRI, but MRI at four weeks postoperatively showed a large heterogeneously enhancing solid and cystic mass in the right cerebellar hemisphere engulfing the prior resection cavity. Figure  A repeatPostoperative MRI of the neuroaxis revealed new metastasis in the cervical spine and an enhancing lesion within the conus medullaris, findings reflective of drop metastasis. Due to the rapid development of his tumor, he began radiation therapy one week postoperatively, a total dose of 40 Gy of focal external beam radiation to the cerebellum and spinal lesions in 10 fractions (400 cGy/fraction), over 14 days using 6 MV photons via intensity-modulated radiation therapy. He tolerated the radiation well with no side effects but was subsequently admitted six weeks postoperatively for back pain and a urinary tract infection. An updated MRI of the cervical and lumbar spine revealed significant progression of the disease with a large conglomerate of metastasis at T12-L1 vertebra. On admission, his serum AFP was 421 ng/mL. The patient’s Karnofsky performance score continued to decline due to progressive bilateral lower extremities and left upper extremity weakness. He was discharged home with plans for hospice care and palliative spine radiation therapy as an outpatient and passed away two months later.The rate of NSGCT late relapse (> two years) after successful primary treatment is estimated to be 1-3% . HoweverThe optimal management of patients, especially in the pediatric population with YST of the testis, remains controversial. The major controversies are the use of chemotherapy [BEP/etoposide and cisplatin (EP)] with or without RPLND as well as radiation therapy. The present case highlights the importance of long, even lifelong follow-up, although RPLND has been shown to be effective in preventing metastasis of YST in some cases -8. This Though rare, intracranial YST metastasis has an abysmal prognosis. Therefore, a combination of surgical resection, chemotherapy, and radiotherapy is often recommended for the management. The presented case showed that YST brain metastasis could have an unusually rapid recurrence despite a gross total resection. Due to its high propensity to recur and metastasize to the spine, if feasible, the craniotomy for complete resection of the tumor should be followed urgently with radiation therapy and chemotherapy before four weeks postoperatively. This approach in special cases with high metastatic potential may be more effective at preventing cerebrospinal fluid (CSF) dissemination, further metastasis, and multifocal disease. Nonetheless, given the highly aggressive potential of YST with central nervous system (CNS) presentation, the most effective approach is long-term and regular surveillance to diagnose possible YST recurrence as early as possible before evidence of CNS metastasis.


Epithelioid sarcomas (ESs) of the vulva are extremely rare soft tissue tumors characterized by an aggressive clinical course and a poor prognosis. The proximal type of ES occurs in the trunk and external genital area and has higher recurrence and distant metastasis rates than the distal type, which is found in the upper and lower extremities. We describe a case of a vulvar ES in a 24-year-old patient who was referred from the department of plastic surgery with protruding mushroom-like lesions in multiple areas, including the lower abdomen, whole vulva, anus, and both inguinal lesions. A biopsy of the lesions confirmed a proximal-type ES. Computed tomography and magnetic resonance imaging revealed multiple metastatic lesions in several regions, including the perineum, vagina, and inguinal regions; nodal metastases in the left external iliac and right inguinal region; and distant metastases in the lungs, pleura of the left lung, bones, and soft tissue. The patient underwent active palliative radiotherapy, followed by chemotherapy, and showed a partial response to treatment. Nineteen months after the initial diagnosis, the patient expired due to cancer progression and pneumothorax. ESs are a very rare kind of tumor, which is characterized by aggressive biological behavior and a poor prognosis . Their n2In July 2013, a 24-year-old Korean woman referred to the Department of Plastic Surgery of Dong San Medical Center, Daegu, Korea because of a palpable mass in her lower abdomen. Due to the abdominal wall mass and a history of vaginal bleeding since October 2012, the patient was referred to the Department of Obstetrics and Gynecology of Dong San Medical Center. The patient was a nulligravida and had no history of illnesses and no personal or family history of malignancies. A detailed physical examination revealed protruding mushroom-like masses in multiple areas, including her lower abdomen, whole vulva, anus, and both inguinal lesions. The primary lesion comprised a large area of necrotic tissue with exposed bone in the entire vulvar region . Punch bMagnetic resonance imaging of the pelvis showed a large, irregularly shaped infiltrating mass with multifocal intratumoral necrosis on both sides of the vulvar and perineal areas, mainly located in the subcutaneous layer. The size of the main mass was at least 12 × 7 × 8 cm. The mass extended to the lower vagina and the left pelvic wall near the obturator internus area a. MultipAccording to the histological features, the differential diagnoses were malignant melanoma, epithelioid leiomyosarcoma, angiomatoid tumor, rhabdomyosarcoma, metastatic carcinoma, and proximal-type ES. The immunohistochemistry (IHC) results were diffuse and strong positive for epithelial markers of cytokeratin a, epithe2 on Days 1 to 3). Follow-up imaging studies revealed a good response, with partial resolution of the vulvar lesions and complete resolution of pelvic bone metastasis (2), vincristine (1.5 mg/m2), and dacarbazine (250 mg/m2) on Days 1 to 5, new metastatic lesions were detected in the brain, left mediastinum, neck, and base of the skull. Consequently, radiotherapy was initiated. Nineteen months after the initial diagnosis, the patient expired due to cancer progression and pneumothorax.The patient’s treatment started with palliative radiation therapy  of the vulva and abdominal mass for two weeks, followed by two cycles of adjuvant chemotherapy consisting of ifosfamide  of INI1  is observed in malignant rhabdoid tumors in infancy and in atypical teratoid/rhabdoid tumors . PreviouIn a previous study that included 37 ES patients, the initial treatment in all cases was a surgical approach, ranging from local excision to radical vulvectomy, with or without node dissection. The latter was possible due to the absence of distant metastasis. Many studies have reported wide local excisions, with surgical margins of at least 2 cm, rather than radical vulvectomies . HoweverThe role of adjuvant radiotherapy and/or chemotherapy in ES treatment remains unclear. Due to the poor prognosis owing to the aggressive nature of ESs, there are currently no accepted standardized treatment guidelines. A previous study found that despite palliative chemotherapy, the prognosis of ES patients with metastasis was poor, with one- and five-year survival rates of 42% and 0%, respectively .Radiotherapy is known to be less effective against sarcomas. However, in our case, radiotherapy was the most effective treatment in reducing the size of the main lesion in the vulva. Combination chemotherapy including ifosfamide elicited a partial response. Ifosfamide and anthracyclines are known the only active agents in advanced soft tissue sarcomas . In thisIn our case, the patient died following disease progression. Palliative radiotherapy followed by chemotherapy resulted in an overall survival of 19 months. Few cases of advanced vulvar ESs with metastases to other organs at the time of the initial diagnosis have been reported. Due to aggressive disease progression without proper treatment, cases of sudden death within a day of the diagnosis have been reported .Most cases of vulvar ESs are detected at an early stage, without regional and/or distant metastasis, and may or may not require adjuvant therapy. Our case is interesting because it is the first case of a vulvar ES not only with multiple distant metastases but also with an extremely large lesion at the time of the initial diagnosis. This case also demonstrates the use of possible treatment options, including radiotherapy and target therapy, in an ES with distant metastasis. Patients with metastatic or advanced ESs may benefit from multimodal approaches. Depending on the number and location of metastatic lesions, it is necessary to consider various treatment approaches. In selected patients, not only surgery and systemic chemotherapy but also radiotherapy and/or target therapy should be considered to achieve optimal results and prolong overall survival.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Herein we describe a rare case of a 59-year-old male who was diagnosed with giant hyperplastic polyps after referring with symptoms of abdominal pain and vomiting, and associated red-flag symptoms of unintentional weight loss and early satiety. Gastroscopy is a common procedure indicated in many conditions that is performed in endoscopy units and allows for direct visualization of the upper gastrointestinal tract to the proximal duodenum . IncidenA 59-year-old male was referred to the gastroenterology department by his general practitioner with symptoms of upper abdominal pain and vomiting associated with early satiety, poor appetite, and a 5-kg weight loss over a period of 6 weeks. During this time, he also noted occasional black stools, but these were intermittent enough that he was not duly concerned and did not present acutely to the hospital. The patient described abdominal pain in the left upper quadrant, consistently present as a dull ache with waves of sharp exacerbations. His vomiting was worse in the mornings, and he denied any hematemesis. The patient’s medical history was significant for ischemic heart disease, for which he takes aspirin; gout, with no recent need for steroids or NSAIDs; and previous atrial fibrillation, for which he was electrically cardioverted and did not require ongoing anticoagulation. His social history was significant for an excessive alcohol intake of 6-8 standard drinks per night, which he had been consuming for about 5 years. He is a non-smoker. There is no family history of any malignancy. Investigations revealed microcytic anemia with hemoglobin of 80 g/L and concurrent iron deficiency with a ferritin of 21 ug/L; the anemia has since normalized. His liver function tests were abnormal with an elevated ALP and GGT of 256 and 612, respectively. Ultrasonic examination demonstrated heterogeneity consistent with steatosis with a normal contour and no liver lesions. The patient came forward for a gastroscopy, the findings of which demonstrated a large pre-pyloric mass that was semi-circumferential, about 6-8 cm in length, and extended from the pylorus to involve most of one half of the gastric antrum. There was associated reflux esophagitis. Representative endoscopic pictures are shown in An urgent CT of his chest, abdomen, and pelvis demonstrated a gastric antral mass. Associated with this was minor, sub-centimeter, lymphadenopathy of the mediastinum, right hilum, and upper abdomen in the left gastric artery lymphatic basin. Helicobacter pylori was not present. Initial biopsies of the lesion showed moderate non-specific mixed inflammatory changes with granulation tissue. The epithelium was reactive with atypia but no dysplasia. Given the high suspicion of malignancy based on the endoscopic appearance, the radiology, and presumed non-representative biopsies, the patient underwent repeat gastroscopy. The repeat gastroscopy findings were that of prominent, nodular mucosa in the gastric antrum. The largest area of irregular mucosa again was noted to extend from the gastric antrum to the pylorus on the lesser curve aspect and was estimated endoscopically to be 6 cm in length and 3 cm in width. Further biopsies were taken.Gastric hyperplastic polyps represent the second most common type of gastric polyps after fundic gland polyps . EndoscoThe majority of gastric hyperplastic polyps are found incidentally, as usually they are asymptomatic. However, giant gastric hyperplastic polyps may present with a variety of non-specific symptoms related to the upper gastrointestinal tract, including bleeding or anemia, dyspepsia, or even gastric outlet obstruction ,6. In th7 it is suggested both adenomatous and hyperplastic polyps have malignant potential and are precursors of early gastric cancer. Polyp size has been regarded as an important factor with a correlation of the larger the lesion, the higher the chance for malignant transformation. Based on this, our patient with a polyp of such a large size would be at higher risk of developing dysplasia and of subsequent malignant transformation. Therefore, the decision was made to proceed to polypectomy with a follow-up surveillance gastroscopy at 1 year to assess.When examined histologically, gastric hyperplastic polyps consist of the foveolar cells that line the gastric mucosa; these cells branch and become stretched and abnormally edematous and vascular ,7. OwingThe evidence to support our decision came from studies that recommend polypectomy on all polyps >2cm, as biopsy alone may miss foci of dysplasia and underestimate the malignant potential .Helicobacter pylori, and our patient was negative for this. It is recommended to perform a biopsy of the surrounding flat mucosa to assess for infection and treat as appropriate, with around 80% of polyps regressing from treatment of Helicobacter alone (Association is noted with er alone . Biopsy er alone . These h