Medulloblastoma presenting with diffuse leptomeningeal enhancement and no identified intra-parenchymal primary mass is extremely rare. A 14-year-old previously healthy boy presented with a three-week history of symptoms consistent with increased intracranial pressure (ICP). Magnetic resonance imaging (MRI) revealed diffuse leptomeningeal enhancement which prompted consideration of infectious, inflammatory, and neoplastic etiologies. The patient became rapidly unstable requiring the placement of an external ventricular drain (EVD) and induction of a phenobarbital coma for refractory seizures. The “sugar-coated” appearance of the abnormal enhancement and thickened tissues raised concern specifically for malignancy. The patient remained extremely unstable and ultimately required surgical decompression for increased ICP at which time a biopsy was obtained. Despite attempting bridging intra-ventricular chemotherapy, the patient, unfortunately, passed away, just 14 days from the initial presentation. Final pathology later confirmed the diagnosis of medulloblastoma. Awareness of medulloblastoma in the differential of diffuse leptomeningeal enhancement is crucial for early identification and treatment of this rare presentation. This case is the first pediatric report of primary leptomeningeal medulloblastoma without a primary mass involving the large cell/anaplastic variant. Medulloblastoma is a neuroepithelial malignancy of primitive neuroectodermal origin and is the most common malignant pediatric brain tumor ,2. It acWhile most cases present with a primary mass in the cerebellum or vermis with occasional dissemination into the subarachnoid space, there have been extremely rare cases presenting with leptomeningeal enhancement alone and no identified intra-parenchymal primary mass. Ferrara described the first documented case as “primary leptomeningeal medulloblastoma” . Early aDue to advances in molecular analysis, there are four defined subgroups of medulloblastoma with distinct molecular markers and varying prognoses for long-term survival. Current treatment regimens are based on subgroup and risk classification and typically include surgical resection of the primary mass in conjunction with radiation and chemotherapy. “Primary leptomeningeal medulloblastoma” is particularly challenging to manage not only because it is difficult to diagnose, but also because gross total resection is not an option, optimal treatment remains poorly defined, and disease progression is rapid with patient survival ranging from weeks to months from diagnosis ,3,5. EarA 14-year-old male presented to the emergency room with a three-week history of double vision, headaches, right-sided facial numbness, dysarthria, dizziness, and associated gait difficulties. An initial physical exam revealed an inability to adduct the right eye and a slight tongue deviation to the right. He was also noted to have had a 17-pound weight loss in the preceding months. Sedated brain MRI with and without contrast revealed extensive diffuse leptomeningeal enhancement, evidence of vasculitis, and secondary hydrocephalus with crowding of the posterior fossa Figures , 1B.2O, which decreased to 8-13 cm H2O after drainage through the EVD. EVD output became bloody two hours after placement and CT revealed a new right intra-parenchymal hemorrhage along the drain tract. Cerebrospinal fluid (CSF) cultures and gram stain were negative. Flow cytometry was negative for malignancy. CSF cytology demonstrated a few atypical and primitive-looking cells suspicious for a neoplastic lesion but indeterminate for malignancy. On hospital day 3, a complete MRI of the spine was performed which revealed diffuse leptomeningeal enhancement concerning for leptomeningeal carcinomatosis, infectious etiologies, or inflammatory processes  scan was discussed to potentially identify a primary mass and to possibly find a more accessible biopsy site, but the patient remained too clinically unstable to undergo prolonged imaging studies.On hospital day 8, the patient underwent a sub-occipital craniectomy for posterior fossa decompression at which time an S1 laminectomy was also performed for tumor biopsy. Bone marrow aspirate and biopsy were also performed. Bone marrow studies were negative for malignancy. Preliminary results from the tumor biopsy revealed a small blue cell tumor that was most likely a medulloblastoma. Pentobarbital infusion was able to be weaned and discontinued but unfortunately, seizures soon recurred and elevated ICPs again became an issue. Attempts to control the patient’s ongoing seizures and increased ICPs were largely unsuccessful, and he was too unstable to undergo emergent therapeutic radiation therapy. Intra-ventricular cytarabine was given on hospital days 10 through 12 in an attempt to control the disease. His family became increasingly concerned with the patient’s suffering given his rapid decline and uncertain neurologic prognosis. His parents made the decision to redirect goals of care to comfort care. The patient was extubated and peacefully passed away 14 days after the initial presentation.Final pathology from the biopsy later revealed medulloblastoma, large cell/anaplastic variant, WHO grade 4, and non-WNT/non-SHH subtype Figures -2D.Additional molecular analysis confirmed a p53 wildtype pattern, positive isochrome 17q, MYC amplification, and no MYCN amplification. The findings were consistent with Group 3 medulloblastoma.131I-burtomab. The disease burden resolved completely within one month and the patient had no evidence of recurrent disease radiologically at 17 months [Medulloblastoma with diffuse leptomeningeal enhancement and no primary tumor is extremely rare as only seven other cases have been reported -8. Of thIsolated diffuse leptomeningeal enhancement in the setting of increased ICP has a broad differential including infectious and inflammatory processes such as meningitis, autoimmune encephalitis, cerebral infarction, and meningeal carcinomatosis with a primary tumor outside of the CNS. Due to its rarity and its unique presentation with lack of a primary mass on imaging, primary leptomeningeal medulloblastoma is a challenging diagnosis to make. Primary leptomeningeal medulloblastoma should be considered in the differential diagnosis for presentations of diffuse leptomeningeal enhancement on MRI. This report describes a very rare presentation of medulloblastoma and is the first report of a pediatric patient with primary leptomeningeal medulloblastoma involving the large cell/anaplastic variant.


Thymic neoplasms are rarely seen among patients with multiple endocrine neoplasia type 1 (MEN1) and appear to be especially rare when pathological examination reveals a World Health Organization Type AB thymoma. In the case presented here, we report a 39-year-old woman with Type AB thymoma in MEN1. A 7.8-cm-sized mediastinal mass was diagnosed as a thymic neoplasm by computed tomography. In addition, pituitary tumor and hypercalcemia from parathyroid hyperplasia were found. Therefore, the patient was clinically diagnosed with MEN1 syndrome and underwent surgical resection of thymic tumor. At the 1-year follow-up, the patient appeared to be healthy without any sign of reoccurrence. Despite its rare occurrence, our case provides us with a new awareness that thymoma may coexist with MEN1. The prevalence of the disorder has been estimated at ~1–3 per 100 000. The tumor suppressor gene, MEN1, located within chromosome  control . Other l control . We descA 39-year-old Chinese female was diagnosed with a pituitary tumor at age 18 when she noticed aberrant growth development. Throughout the years, she developed PHPT, gastrinoma and bilateral adrenal hyperplasia. She was referred to our hospital for further evaluation to confirm the diagnosis of MEN1. Enhanced computed tomography (CT) demonstrated a 7.8-cm by 3.5-cm irregular anterior mediastinal solid-cystic mass within the thymic region . The leset al. reported a case of an aggressive thymoma (Type B3) in a patient with MEN1 syndrome [et al. reported a Type B3 thymoma with neuroendocrine differentiation in MEN1, and there were four previous cases in the literature which describe thymoma in MEN1 [We present an extremely rare case of a patient with WHO Type AB thymoma with MEN1. To the best of our knowledge, this finding is the first ever reported. Most of the neoplasms of the thymus gland which have been reported in MEN1 are thymic carcinomas. Only six cases of thymoma in MEN1 have been reported in the literature. Akua Graf syndrome . Tomita  in MEN1 .Clinically, the presence of thymoma in some MEN1 patients may go unreported until found on abnormal chest CT imaging. To date, the explanation of this rare combination of thymoma and MEN1 remains unclear.In summary, regular and thorough examinations of MEN1 patients are required and should be carried out to detect thymic tumors early because they can be a substantial cause of mortality. Further studies to elucidate the pathogenesis of thymic epithelial tumors in MEN1 and their clinical significance remain to be performed.Conception was by Z.F., and C.G.; data acquisition and analysis were done by Z.F., L.W. and J.W.; writing the article was done by Z.F. and L.W.; All authors read and approved the final article.


Myofibrosarcomas are rare soft-tissue sarcomas mainly affecting the head and neck or extremities of adult patientsThe natural course of myofibroblastic malignancies follows a slowly-growing, infiltrative pattern of spread, but carries a risk of recurrence and metastasis, even after many years. The latter attribute can be explained by the lack of a capsuleBelow, we report a case of a mandibular myofibrosarcoma in an 11-year-old girl.An 11-year-old girl presented with a left-sided mandibular tumor. Initially, it had been enucleated transorally, before the patient was evaluated in our clinic. Histopathology was consistent with intermediate-to-high grade myofibrosarcoma. A CT scan one month post-operatively demonstrated a residual mass, measuring 3.7cm transverse × 1.9cm anteroposterior × 3.4cm cephalo-caudal, and involving the majority of the ramus.Segmental mandibulectomy was performed, encompassing the angle of the jaw. An osseThe pathology report was consistent with myofibrosarcoma. Six monMalignancies showing myofibroblastic differentiation are very rare and their biologic behavior is far from being elucidated. Myofibrosarcomas are soft-tissue sarcomas but a few of them are intraosseous, as the case with our patient.Myofibroblasts, which g11 cases of myofibrosarcoma of the head and neck have been reported in children. Myofibrosarcomas respond poorly to chemotherapy and radiation therapyIn the pediatric population, reconstruction following surgical resection is challenging, both in terms of donor site morbidity and rebuilding the growing facial skeleton. With regard to complications developing in the donor site, ankle instability is reported in 27% of children only, due to their greater ligamentous laxityThe female mandible has achieved 90% of the adult size at the age of 11Myofibrosarcoma is a rare and undefined entity, so that every new report of a case might contribute to understanding its natural course and management. In view of all the previous reports, the treatment of choice should be surgical resection. Here, we presented a case in a pediatric patient, trying to emphasize the importance of a comprehensive approach to a superior oncological outcome, as well as acceptable quality of life.


Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date. Agenesis of the dorsal pancreas is a rare congenital disorder that usually presents in early adulthood. The symptoms are non-specific and create a diagnostic dilemma. Less than a hundred have been described in the literature to date . It may A 55-year-old male patient presented to us with a two-month history of pain over the epigastrium and right hypochondrium. It was dull, intermittent, non-radiating, and had no identifiable aggravating or relieving factors. There was no history of vomiting, jaundice, or fever. There were no symptoms suggestive of steatorrhea. However, the patient had chronic constipation. He also had a history of recurrent urinary tract infections associated with burning micturition. He sought treatment at a private setup and was treated with oral medications. He could not provide any previous urine routine microscopy and culture reports. He was a known diabetic on oral hypoglycaemic agents for the past six years, which were started at a private clinic. He was taking glimepiride 1 mg once a day and metformin 500 mg twice a day. On presentation, the patient's blood pressure was 110/70 mmHg, pulse rate was 94 bpm, respiratory rate was 16 pm, saturation level was 97% on room air, and he was afebrile. His blood sugar level, as measured by a glucometer, was 330 mg/dl. On general examination, the patient was thin-built and had mild pallor. The abdomen examination was essentially normal. The rectal and proctoscopic examination revealed grade two internal hemorrhoids. Routine blood investigations, including amylase and lipase, were within normal limits except for glycosylated hemoglobin (HbA1c), which was 9.6% indicating poorly controlled diabetes. The patient was started on intermediate-acting insulin in the ward. The blood sugar levels were gradually brought under control with a daily requirement of 20 U of intermediate-acting insulin. Contrast-enhanced computed tomography of the abdomen was done, which demonstrated non-visualization of the neck, body, and tail of the pancreas suggesting dorsal pancreatic agenesis Figure . The visThe pancreas arises from the endoderm via the dorsal and ventral buds, which form as outpouchings from the foregut. The signaling pathways include the hedgehog, PDX1, and NOTCH genes. The dorsal bud develops more rapidly and forms the neck, body, and tail of the pancreas drained by the duct of Santorini. The ventral bud rotates clockwise to fuse with the dorsal segment, ultimately forming the head and uncinate process drained by the duct of Wirsung .Agenesis of the dorsal pancreas is an extremely rare disorder. We did the literature search on PubMed Central, Europe PMC, Scopus, and Google Scholar using the keywords: 'genesis of dorsal pancreas' and 'dorsal pancreatic agenesis'. A total of 57 publications were identified describing a total of 68 cases from 1913 to 2021 (Table Agenesis of the dorsal pancreas presents a diagnostic challenge as most of these patients present with non-specific abdominal symptoms. One of the major differential diagnoses to be ruled out is pancreas divisum. Cross-sectional imaging and endoscopic retrograde cholangiopancreatography will show the separation of the ventral and dorsal pancreatic ductal system. Atrophy of the body and tail of the pancreas occurs in chronic pancreatitis and may mimic agenesis. In such a scenario, there may be a history of previous abdominal pain radiating to the back associated with raised amylase and lipase levels. Agenesis of the dorsal pancreas has also been shown to have a syndromic association with polysplenia and the annular pancreas. Since presenting symptoms are non-specific, cross-sectional imaging and magnetic resonance cholangiopancreatography are the cornerstones for diagnosis. There is no specific treatment for this rare disorder. Patients need to be offered symptomatic management and diabetes control with insulin preparations. Steatorrhea due to exocrine insufficiency may be present, which can be managed by enzyme supplementation.


Neuroepithelial cysts are benign, congenital, ependymal-lined cysts that constitute an infrequent cause of neurological symptoms among intracranial cystic lesions. We present Indonesian patient who underwent brain MRI depicting neuroepithelial cysts and pathological examination confirmed. Surgery was performed and yielded a satisfying result. Awareness of the entity and specific imaging features is key to making the diagnosis of neuroepithelial cyst and commencing appropriate treatment. This report is expected to aid in the diagnosis of neuroepithelial cysts, which are rare, and reduce the occurrence of misdiagnosis. Neuroepithelial cysts are cavities with an ependymal or epithelial lining. They contain fluids of unidentified origin and develop in the central nervous system. Regarding ventricular or subarachnoid spaces, there is no clear communication between them. Moreover, the cysts are sporadic or evolve congenitally in the central nervous system and represent less than 0.4% of all cyst-like lesions. Those indications are commonly found in East Java The profound literature has described this mass as an ependymal cyst, neuroepithelial cyst, glioependymal cyst, and neuroglial cyst. These descriptors indicate the complexity of this disease's terminology A 29-year-old male patient presented with blurred vision for the past 2 years. He complained of weakness on the left side of the body, slurred speech, and a skewed face 1 year prior to hospitalization. The symptoms worsened in severity over time and persisted during the course. Upon admission, he was unable to walk and had lost the vision in his left eye. No account of head trauma or oncologic records was reported.Neurological examination revealed left-sided motor weakness without sensory disturbances, increased left-sided deep tendon reflexes with pathological reflexes, and normal cerebellar functions. Moreover, on ophthalmological examination, the light perception in the left eye was remarkably decreased. Fundoscopy detected right eye papilledema and left optic papilla atrophy. MRI examination of the head revealed a large, well-demarcated, intraparenchymal cystic lesion measuring 8.7 × 7.8 × 7.8 cm in the right frontoparietal lobe. It produced a significant mass effect on the surrounding parenchyma and resulted in noncommunicating hydrocephalus . The chaA mini craniotomy was conducted in the left frontal lobe to aspirate the cysts with insertion of an Ommaya reservoir. The cysts contained clear fluid without signs of recent or old hemorrhage. Histopathological examination was requested for the surgery afterward . The proNeuroepithelial cysts, also known as glioependymal cysts, originate from the area surrounding the craniospinal axis . The cysts develop from ectopic ependymal cells In contrast, the infratentorial GEC produces more unusual symptoms, such as hemifacial spasm, dizziness, and diplopia Both MRI and CT play an essential role in establishing a diagnosis, although MRI is the preferred modality for identifying neuroepithelial cysts. The cyst has smooth, rounded borders and is composed of thin, unilocular walls that contain CSF-like fluid that is identifiable on CT or MRI. CT scans generate a cystic focus with unenhanced low-density fluid, and MRI shows a cystic-filled lesion with fluid that is hypointense on T1-weighted and hyperintense on T2-weighted images. Moreover, the contents of the cyst show no restriction in the diffusion-weighted sequence, which is comparable to CSF and no surrounding edema. The mass effect caused by these cysts is minimal, even in prominent lesions, indicating their marginal growth, especially in adults. There is no enhancement after contrast media injection In this case, three differential diagnoses were reported: porencephalic, arachnoid, and epidermoid cysts. Porencephalic cysts circulate inside the white matter, replicate the CSF, and exert no mass effect on the neighboring tissue. Moreover, there is no enhancement with contrast administration that connects them to the ventricular system or subarachnoid space with adjacent gliosis. Arachnoid cysts exist as a singular, extra-axial cyst with a well-demarcated margin and share similar intensity to CSF. They dislodge the surrounding parenchyma, dent the skull bone, and exhibit no restriction on DWI sequence and are unenhanced on contrast administration. For this reason, most arachnoid cysts originate from above the tentorium and have no ventricular attachments. Epidermoid cysts contain a lobular cavity filled with CSF-like substance and exhibit gradual expansion features. Subsequently, they produce mass effect, invading the surrounding anatomy and wrapping nearby nerves and vessels. As demonstrated in fluid-attenuated inversion recovery (FLAIR) sequences, an epidermoid cyst covers the area of the heterogeneous regions of hyperintensity. In contrast, the emergence of CSF and hyperintensity on diffusion-weighted imaging sequence matters. Occasionally, there is calcification (10%-25%) and moderate peripheral enhancement These cavitating lesions are usually filled with clear, CSF-like fluid; however, several reports have indicated a variety of intracavitary contents that appeared xanthochromic, opalescent, milky, or turbid. Pathological examination reveals a partially ciliated cuboidal or columnar lining epithelium lying on the astroglial tissue bundles, interceded by the basal membrane. Surgical intervention is indicated based on the dependable location of the cyst and the subarachnoid or ventricular space approximation. The emergence of simple puncture and open or stereotactic drainage has resulted in a higher cyst recollection rate Several reports and literature discuss the radiological characteristics and management of such entities, investigating their sporadic occurrence and other benign, clinical, or subclinical intracranial cystic lesions. We observed one rare case of neuroepithelial cysts with clinical manifestations of visual and movement disorders. The findings of our study focused on the diagnosis of clinical and imaging features with histological evidence. Furthermore, we emphasized the algorithmic approach, based on the most expected anatomical sites, signs, symptoms, and radiological evidence. These approaches take into consideration the features of the lesions to narrow the differential diagnosis of intracranial cysts. Therefore, a systematic approach is key to evidence-based management of incidental benign variants  and avoiding unnecessary surgical interventions.Neuroglial cysts are congenital central nervous system lesions with challenging preoperative detection as this disorder correlates with many clinical and radiological characteristics of other anomalies. Nevertheless, histopathological studies remain the only method of producing a definitive diagnosis. The symptoms depend on the cyst's location and size; however, some patients may present with mild symptoms in cases of large lesions. In this case, surgery was the preferred modality for the symptomatic patient, and the location of the cyst determined the type of surgery, which also took into consideration the ventricular or subarachnoid spaces that needed to be approximated. Therefore, radiology has an essential role in diagnostics and may aid physicians with their diagnosis and surgical approach.Informed consent was obtained for publication of a case report. Written informed consent was obtained from the patient for the publication of this case report.


Biliary intraepithelial neoplasia (BilIN) is a precursor lesion of cholangiocarcinoma that has been rarely reported. The present study reports a 56-year-old male with low-grade BilIN of the bile ducts and the cystic duct margin. Stent exchange endoscopy demonstrated an irregular, intraductal mass extending along the common bile duct, common hepatic duct, and hepatic duct bifurcation. The peribiliary mass was found to abut the right portal vein, inferior vena cava, and pancreatic head, and replaced the right hepatic artery. In addition, there was evidence of gallbladder adenoma managed with cholecystectomy and a right-lobed liver lesion and cirrhosis, which prompted the discussion of prophylactic liver transplantation. We emphasize the radiological features of BilIN and associated pathological findings through multiple imaging modalities. Consideration of this diagnosis is indicated in western countries and requires timely management based on available guidelines. Biliary intraepithelial neoplasia (BilIN) is characterized as a spectrum of proliferative, flat, or micropapillary precursor lesions of bile duct adenocarcinoma . BilIN rBased on the 2010 World Health Organization classification, intraductal papillary neoplasm of the bile duct (IPNB) is defined as a papillary or villous neoplasm covering the fibrovascular stalks occurring in the bile ducts. Imaging features of IPNB vary according to the presence of intraductal lesions, degree of mucin production, and tumor location . While BDue to the poor prognosis of cholangiocarcinoma, it is critical to identify associated risk factors. In western countries, the major risk factors for biliary neoplasia are primary sclerosing cholangitis, Thorotrast deposition, abnormal choledochopancreatic junction, and choledochal cysts. In Southeast Asian countries, intrahepatic cholangiocarcinoma is more frequent than extrahepatic cholangiocarcinoma with predisposing factors of hepatolithiasis and parasitic infection . AdditioA majority of reported BilIN presents as lesions that do not form a mass or cause bile duct obstruction. However, when these tumors grow large enough, symptoms of biliary obstruction such as jaundice, intermittent pain, dyspepsia, weight loss, nausea, and vomiting become present . To our A 56-year-old male with no prior medical history and no history of smoking or heavy alcohol use presented with itching, very sporadic postprandial bloating, and light-colored stools. His most recent colonoscopy indicated no polyps or concerning lesions, but the presence of diverticulosis was noted. He denied changes to stool caliber, melena, hematochezia, fever, chills, night sweats, or any unplanned weight loss. Labs were significant for elevated total bilirubin, elevated alkaline phosphate, and abnormal liver function tests. The patient was negative for hepatitis A, B, and C, though there was an abnormal Epstein-Barr virus (EBV) result.He was referred for an abdominal ultrasound, which showed dilation of the common bile duct. He then underwent endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, and biopsy of proximal common bile duct intraductal stricturing lesion along with stent placement  at the confluence of the right and left hepatic ducts. The subsequent Whipple's procedure showed focal high-grade intraductal papillary neoplasm of the distal common bile duct to the proximal margin to the ampulla. There was no evidence of malignancy in the pancreatic head, duodenum, distal stomach, or lymph nodes.The association between BilIN, gallbladder adenoma, and cirrhosis is not well understood. Comparison of high-grade BilIN with ICPN of gallbladder suggests that conglomerated ICPNs may arise from high-grade BilINs with short papillary components . A priorDue to the poor prognosis of gallbladder cancer, every attempt must be made to identify asymptomatic stages and premalignant gallbladder polyps and adenoma. Gallbladder polyps larger than 1.5 cm, especially in solitary sessile hypoechogenic polyps, are associated with a risk of malignancy and require cholecystectomy when there are symptoms such as biliary-type pain and dyspepsia . IndicatPrior report of BilIN-2 in the common bile duct has been successfully treated with local excision with no evidence of recurrence during a three-year follow-up period; however, this treatment strategy may be limited to managing the less common extrahepatic BilIN . AnotherBilIN is a rare disease with few instances reported. This case study features a unique presentation of BilIN with imaging modalities highlighting an extrahepatic biliary mass. Understanding the natural history of the disease with possible progression to cholangiocarcinoma is valuable due to the poor prognosis associated with cholangiocarcinoma. The case provides insight into a rare disease with complex features, including low-grade IPNB with gallbladder adenoma and cirrhosis. Though the prevalence is low for a pathologic diagnosis of BilIN, there is still a need for further investigation to provide a greater understanding of disease progression and management.


We present a rare case of a 45‐year‐old man with abdominal pain and features suggestive of intestinal obstruction. The CT scan of the abdomen demonstrated a large mesenteric mass involving the distal segment of jejunum. Surgical excision of the lesion and histopathological examination revealed the diagnosis of cavernous hemangioma. Gastrointestinal hemangiomas are uncommon and the presentation of the patient depends on the location of the tumor. Diagnosis is usually established following surgery and histopathology. Surgical resection is usually curative. The most common sites are skin, face, scalp, and back with frequent presentations involving other locations like liver, spleen, brain, spinal cord, adrenal glands, and mediastinum. However, gastrointestinal (GI) hemangiomas are uncommon.A case of cavernous hemangioma being an unusual but potential cause of intestinal obstruction is hence presented, where the diagnosis was uncertain even after resection of the mass. Due to its rarity, diagnosis is often delayed. The definitive treatment entails resecting the mass. This case has been reported in line with the SCARE criteria.2A 45‐year‐old man presented in the emergency department with generalized abdominal pain for 4 days and multiple episodes of vomiting for 1 day. He reported not having any bowel movement for 3 days, which was followed by four episodes of watery stool on the day of presentation. There was no history of melena or hematochezia. The patient had a smoking history of 3 pack‐years. His previous medical, surgical, and medication history were unremarkable.On examination, the patient's blood pressure was 110/80 mm Hg with the heart rate of 88 beats per minute. The abdomen was uniformly distended and tenderness was elicited on deep palpation with no obvious mass. There was resonance on percussion and the bowel sounds were hyperactive. The hernial orifices were intact and the scrotal examination was unremarkable. The digital rectal examination showed an empty rectum. The rest of the systemic examination was unremarkable.3 and C‐reactive protein (CRP) of 27.2 mg/L. The radiographic examination of the abdomen demonstrated distended jejunal loops  are useful modalities for diagnosis. CT scan usually demonstrates non‐homogeneous enhancement of lesion with transmural thickening of walls of the intestine involved.The treatment of GI hemangiomas is surgical excision and likewise, in our patient, exploratory laparotomy with excision of the mesentery and affected bowel loop was performed. However, if a diagnosis had been made preoperatively, laparoscopic excision of the affected part could also have been performed.Mesenteric hemangiomas involving the bowel, despite being extremely rare, should be taken into account as potential diagnoses in patients presenting with features of intestinal obstruction. Sometimes, waiting for a definitive diagnosis can be lethal for patients' outcome, thus mandating an early intervention.ST, UK, and BDJ were entirely involved in the management of the patient and conception of the study. ST, AB, and BP prepared the manuscript. All authors were involved in critical revision of the manuscript.None.None.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


An arteriovenous malformation (AVM) is a very rare differential diagnosis of a posterior Mediastinum mass. We report a patient with an AVM mimicking a mediastinal tumour and describe the radiological, pathological findings and the treatment options. Rarity, unclear etiology  variable clinical presentation (infectious/ compressive symptoms) and non-specific findings on diagnostic imaging post a diagnostic challenge. We describe a case of a posterior mediastinum AVM in a 20-year-old man, which was treated as tuberculosis till further diagnosis was made.Arteriovenous malformations (AVMs) in the thoracic cavity are extremely rare, with evidence limited to a few case reports. This abnormal communication between arteries and veins can masquerade as infections like tuberculosis, neurogenic tumours or lymphomas.A 20-year-old gentleman, presented elsewhere with complaints of fever along with cough and mucoid expectoration for three weeks and significant loss of weight. A contrast enhanced CT (CECT) scan showed consolidation of the right middle lobe and medial Basal Segment of right lower lobe with a few scattered nodules. In View of typical radiological features (Right middle lobe Syndrome) and strongly positive Mantoux test (sputum negative for AFB), he was started on Anti Tubercular treatment with a 4 drug regime.He was subsequently referred to our centre and was revaluated. On physical examination, the patient’s chest was clear on percussion and auscultation, but a significant bruit under the right scapular area was noted. A CECT scan demonstrated a non-enhancing posterior mediastinal lesion with few tiny internal calcific specks abutting the vertebral bodies without extension to neural foramina. A MRI revealed an 8x4.6x3.6 cm circumscribed mass lesion with lobulated margin in the right para-vertebral region (D4-D7) suggestive of a neurogenic tumour . A EUS gWith a presumptive diagnosis of neurogenic tumour of the posterior mediastinum he underwent a robot assisted excision of the posterior mediastinum mass. Intraoperative findings revealed a 6 x 4 cm mass with multiple dilated tortuous veins and 3rd, 4th, 5th right side nerve roots were seen traversing through the mass . Final h Presence of a well-defined lesion like in our case is atypical. These lesions can be incidentally detected on chest radiographs or can present with compressive symptoms or in rare scenario heart failure due to the ‘steal phenomenon’. Traumatic Etiology or iatrogenic injury to surrounding vessels is also described.Mediastinal AVM’s are a rare entity and are generally large, diffuse and ill-defined involving brachiocephalic, intercostal, and internal mammary arteries.Differential diagnosis of the lesion includes vascular mediastinal tumors like haemangioma, angiolipoma, and extra lobar pulmonary sequestration or non-vascular lesions like neurogenic tumours. Chronic infections like hydatid cysts and tuberculosis can also be considered in endemic areas. Complete excision can be difficult to the ill-defined nature and encroachment of vital mediastinal structures. Careful planning and preoperative embolization may reduce the intra operative bleeding. In our case we could achieve R0 resection with minimal invasive technique using a robot. We encountered no adhesions or any bleeding during surgery.Surgery with complete excision remains the cornerstone of treatment.AV malformations in the thorax are uncommon and can be symptomatic at presentation. Lesions that are diagnosed incidentally need to be treated early to avoid further progression. Surgery remains the main treatment of choice.Informed consent was obtained from the patient and the patient identity has been kept confidential in the manuscript.None.


Anatomical diversity is rather typical in the biliary region. However, it has only sometimes been documented that the arteries of a hepatobiliary origin compressed the extrahepatic bile duct. Biliary obstruction may be caused by a myriad of benign and malignant diseases. Right hepatic artery syndrome (RHAS) is described as the consequence of right hepatic artery compression of the extrahepatic bile duct. We report a case of a 22-year-old male who presented with a complaint of abdominal pain and was later admitted as a case of acute calculous cholecystitis with obstructive jaundice. Abdominal ultrasound showed a picture of the so-called Mirizzi. However, A magnetic resonance cholangiopancreatography showed a picture of RHAS, so the patient needed endoscopic retrograde cholangiopancreatography to decompress the biliary system which was later performed successfully followed by cholecystectomy. The diagnosis of RHAS is well established in the literature, and it depends on the facility of the institute, cholecystectomy versus hepaticojejunostomy or endoscopic treatment alone are the management options that have been utilized to manage such cases. The liver has a dual blood supply, receiving blood from the portal vein and the hepatic artery. The right lobe of the liver is supplied by a typical or normal right hepatic artery, which emerges from the proper hepatic artery. The origin and course of the right hepatic artery are prone to anatomical variations . The hepA relatively rare complication of acute cholecystitis is the development of a hepatic artery pseudoaneurysm; nevertheless, a pseudoaneurysm causing gallbladder neck compression and intrahepatic duct dilation cause a presentation similar to Mirizzi syndrome which is mostly unreported . MirizziThis is a case of a 22-year-old male not known to have a chronic medical illness and no significant medical or allergic history except surgical background of sleeve gastrectomy two years ago where he lost around 65 kg. The patient had a positive paternal history of pancreatic cancer. He complained of abdominal pain which lasted for 10 days on and off, associated with darkened urine, constipation, and anorexia. There is no history of nausea, vomiting, fever, or itching. Generally, the patient was not distressed but was deeply jaundiced. The patient was admitted with a case of acute calculous cholecystitis with obstructive jaundice. Upon examination, the patient was vitally stable with a blood pressure of 123/87 mmHg, a temperature of 36.8°C, a heart rate of 53 per minute, a respiratory rate of 20 per minute, and an oxygen saturation of 100%. Chest examination revealed equal bilateral air entry. Furthermore, his abdominal examination revealed right upper quadrant tenderness and a positive Murphy sign. A previous laparoscopic surgical scar was present. Laboratory investigations of the patient are depicted in Table An abdominal ultrasound showed that the liver was of normal size. However, coarse parenchymal echotexture was noted, which could be correlated with a hepatitis profile. The gallbladder showed multiple mobile tiny gallbladder stones, with minimal thickening of the gallbladder wall reaching up to 5 mm; however, no pericholecystic free fluid was seen. The common bile duct diameter appeared prominent, measuring 5 mm, with minimal proximal intrahepatic biliary duct dilatation. Further evaluation by magnetic resonance cholangiopancreatography was recommended. No common bile duct stone was detected at the visualized part of the proximal common bile duct Figures , 1B.MRCP showed that the gallbladder was distended, containing multiple small calculi along with diffuse gallbladder wall thickening measuring up to 8 mm with a streak of pericholecystic free fluid extending into the subhepatic space in keeping with acute calcular cholecystitis. A small cystic duct stone was seen causing the obstruction. There was a trifurcation anomaly of the intrahepatic biliary tree, intrahepatic biliary channels showing dilatation, and a slightly beaded appearance, which could be related to cholangitis Figure .Furthermore, there is an abrupt cut-off at the level of the proximal common hepatic duct beyond which the distal common hepatic duct and proximal common bile duct were not visualized/collapsed. A prominent vessel was seen crossing at the site of the cut-off which could be responsible for the focal narrowing along the retrograde intrahepatic dilatation as well as the focal structuring related to the cholangitis Figure . MoreoveUpon admission, the patient was kept NPO with intravenous fluid and analgesia along with the antibiotic for ultrasound and magnetic resonance cholangiopancreatography. The gastroenterology team was involved from the time of admission. The findings of the ultrasound and magnetic resonance cholangiopancreatography showed a picture of Mirizzi syndrome and the possible need for endoscopic retrograde cholangiopancreatography to decompress the biliary system which was performed in another hospital where the patient was accepted. The procedure was done without any complications and was successful as the patient’s symptoms improved.Jaundice or the development of gallstones has been attributed to anatomical variations of the hepatic artery. Bove et al. reported three cases of intrahepatic stones caused by the right hepatic artery compressing on the proximal common bile duct. In order to remove intrahepatic stones, the three patients had endoscopic retrograde cholangiopancreatography and biliary sphincterotomy. After the stones were removed, cholangiography revealed common bile duct compression immediately below the main hepatic confluence, which was later proven to be owing to right hepatic artery compression on MRI . ContradMirizzi syndrome and other chronic complications of symptomatic gallstone diseases are uncommon. When detected during a laparoscopic cholecystectomy, this condition's relevance and consequences are related to its associated and potentially serious surgical sequelae, such as bile duct injury, and its current therapy. A pressure ulcer caused by an impacted gallstone at the gallbladder infundibulum, followed by an inflammatory response that initially blocked the bile duct externally before eroding into the duct and developing into a cholecystocholedochal or cholecystohepatic fistula, has been used to explain the pathophysiological process leading to the subtypes of Mirizzi syndrome . A proloCui et al. described that the diagnostic sensitivity of Mirizzi syndrome may be increased by combining choledochoscope surgery with ultrasound, magnetic resonance cholangiopancreatography, and endoscopic retrograde cholangiopancreatography. It is effective to confirm Mirizzi syndrome during surgery using an intraoperative choledochoscope. The current gold standard for treating Mirizzi syndrome patients is open surgery. Patients should be carefully chosen for laparoscopic surgery, which should be restricted to those with type I Mirizzi syndrome . For theMirizzi syndrome accompanied by RHAS is a rare presentation. Abdominal ultrasound might not be the optimal tool for diagnosis as it might show signs of inflammatory hepatitis which can be misguiding in young adults. The magnetic resonance imaging is the golden standard to visualize the biliary tree. Surgery is the treatment choice in such cases as cholecystectomy was performed and the patient improved afterward. Further research focusing on Mirizzi syndrome can be beneficial in understanding the clinical characteristics of the condition.


Liver cancer subtypes differ in prognosis and genetic alterations. An accurate diagnosis made on time is the key aspect of clinical decision-making. Hence, a correct diagnosis is of pivotal importance for individual patients. In this study, we identified the most relevant clinical, radiological, and histological parameters for an improved subtype diagnosis of intrahepatic cholangiocarcinoma. As a result of our study, the radiologist should consider factors such as growth pattern, location, and contrast agent behavior. For the pathologist, precursor lesions, mucin secretion, and a periductal-infiltrating growth are of utmost importance, while immunohistochemical analyses are essential for exclusion of extrahepatic malignancies, but have so far only value for iCCA subtype analysis in the context with other parameters.Intrahepatic cholangiocarcinomas (iCCAs) may be subdivided into large and small duct types that differ in etiology, molecular alterations, therapy, and prognosis. Therefore, the optimal iCCA subtyping is crucial for the best possible patient outcome. In our study, we analyzed 148 small and 84 large duct iCCAs regarding their clinical, radiological, histological, and immunohistochemical features. Only 8% of small duct iCCAs, but 27% of large duct iCCAs, presented with initial jaundice. Ductal tumor growth pattern and biliary obstruction were significant radiological findings in 33% and 48% of large duct iCCAs, respectively. Biliary epithelial neoplasia and intraductal papillary neoplasms of the bile duct were detected exclusively in large duct type iCCAs. Other distinctive histological features were mucin formation and periductal-infiltrating growth pattern. Immunohistochemical staining against CK20, CA19-9, EMA, CD56, N-cadherin, and CRP could help distinguish between the subtypes. To summarize, correct subtyping of iCCA requires an interplay of several factors. While the diagnosis of a precursor lesion, evidence of mucin, or a periductal-infiltrating growth pattern indicates the diagnosis of a large duct type, in their absence, several other criteria of diagnosis need to be combined. IDH1-/2-mutations and FGFR2 gene fusions [Intrahepatic cholangiocarcinoma (iCCA) constitutes the second most common primary malignancy of the liver, with a poor median survival of only 13 months. Globally, many countries have experienced substantial increases in mortality in the past decades . Even if fusions ,9. Overa fusions . It has  fusions . As aptl fusions .This study aimed to unravel the best possible strategy to optimally subtype iCCAs as early as possible. The staging includes imaging techniques, which are often carried out before a biopsy is taken. For this purpose, we have characterized a large cohort of iCCAs. We comparatively analyzed iCCAs concerning their clinical parameters, radiological findings, and histomorphological and immunohistochemical results. For the best possible patient outcome, we will elucidate the most crucial factors which need to be addressed in imaging and histology. This manuscript may thus improve iCCA subtyping in clinical practice and help standardize the radiological and histopathological findings.In order to unravel subtype-specific features of iCCAs, we established a large and clinicopathologically well-characterized cohort of 232 operation specimens of iCCAs, with 148 small duct iCCAs and 84 large duct iCCAs, diagnosed according to the current WHO classification see .Both subtype groups were comparable in terms of age and gender distribution as well as body mass index (BMI) and physical status  . Large ds = 0.468, p < 0.001), hypovascular  and rim-enhanced  arterial enhancement pattern. For this reason, we excluded the parameters bile duct obstruction and contrast agent behavior from the regression analysis to avoid misinterpretation by multicollinearity. Other observed variables could not distinguish between both iCCA subtypes , invasion of visceral peritoneum and pT3-stage , as well as invasion of extrahepatic structures and pT4 stage  were associated, respectively.Concerning radiology, cross-sectional imaging procedures from 191 of the 232 patients were available in sufficient quality, including 146 computed tomography (CT) and 45 magnetic resonance imaging (MRI) scans. While small duct iCCAs were on average slightly larger and less frequently located near the portal bifurcation, the distance to the serosa did not show a significant difference in the comparative analysis . Large dsubtypes . As expeS = 0.706, p < 0.001), the PI growth pattern was not an independent variable. Consequently, the growth pattern was not included in the regression analysis. Nevertheless, univariate analysis of growth patterns showed the third-highest effect size after the detection of precursor lesions and the production of mucin. A graphical representation of the difference in growth patterns is illustrated in Small and large duct iCCAs differed significantly in several histopathological characteristics see . BiliaryIn immunohistochemical analysis, the antibodies CA19-9, EMA, CD56, N-cadherin, and CRP showed potential in the differential diagnosis of iCCA subtypes, while CK7 and S100 did not . CK20 waSurvival probability of the small duct iCCAs was better than that of the large duct iCCAs. However, in contrast to previous findings , this reIn our comprehensive study including 232 patients with iCCA, we unraveled the most important clinical, radiological, histological, and immunohistochemical features for the differential diagnosis of small and large duct iCCAs.Clinical indicators of iCCA are limited, and rarely reliable indicators to establish the iCCA subtype diagnosis. Initial presentation with jaundice favors the diagnosis of a centrally located tumor, yet may not distinguish iCCA from perihilar cholangiocarcinoma (pCCA) or benign diseases. When compared to small duct iCCAs, large duct iCCAs more often required larger and more invasive surgery involving the hilar region.Imaging techniques, especially CT and MRI, naturally play a pivotal role in the management of iCCAs. Various radiologic approaches have been proposed to distinguish iCCA subtypes. However, subtype comparisons are complicated by the fact that some even recently published studies only distinguish between mass-forming iCCAs and non-mass-forming iCCAs without considering the final histopathological subtype ,15,16,17Taking into account the frequency of liver lesions, studies involving comprehensive clinicopathologic investigations on the differential diagnoses are of special importance. Apart from the frequent benign liver hemangioma, rare cases of hepatocellular adenoma and focal nodular hyperplasia, primary and secondary malignancies need to be ruled out. In addition to iCCAs, also hepatocellular carcinomas (HCCs) are primary liver carcinomas with characteristic clinical and radiologic features distinguishing both entities. The hepatocellular origin of a tumor may already be distinguished conventionally and morphologically by routine histopathology together with typical radiologic features and clinical context, as HCCs are most frequently associated with liver cirrhosis. In particularly difficult cases, as for certain types of HCCs as well as combined hepatocellular-cholangiocarcinoma (HCC-CCC), the diagnosis needs further immunohistochemical analyses such as Hepatocyte paraffin 1 (HepPar1) . So far,Immunohistochemistry should be used in unclear cases, especially beforehand, when a metastatic event must be differentiated from a primary liver tumor. However, immunohistochemical analysis may be used in iCCA subtype analysis. This has been shown several times for several different markers. In a recent study involving a small cohort, CRP and N-cadherin-positivity as well as S100-negativity supported the diagnosis of small duct iCCAs . We wereThe present study has several limitations. First, the retrospective data acquisition of a pure operation cohort may have inadvertently led to a selection bias. However, this cannot be entirely avoided due to the rarity of iCCAs, so we collected a fairly large patient cohort to obtain the most comprehensive evaluation possible. Second, in contrast to the radiologist, the pathologist was not blinded by the subtype. Since the histological parameters were evaluated on the whole slides of the respective cases, the individual data may have been subconsciously interpreted. Nevertheless, since the immunohistochemical analyses were carried out at the TMA, blinding was guaranteed. Third, we compared MRI and CT images, which may have affected the results. CT is considered the standard imaging method for the assessment of liver masses, but is only of limited use for determining the exact tumor extent along bile ducts, and hence, the resectability . Due to Small and large duct iCCAs are two biologically different tumor entities, and their correct classification is therefore of utmost importance for prognosis and subsequent therapeutic decisions.In our analyses, growth pattern and presence of biliary obstruction were important for radiologic iCCA subtype diagnosis. For the final histopathologic diagnosis, a periductal-infiltrating growth pattern, mucin production, and precursor lesions such as BilIn and IPNB are the main factors that support the diagnosis of large duct iCCAs. If these factors are not present, the diagnosis of a large duct iCCA should only be made by combining various other factors, possibly including radiological and immunohistochemical analyses.The present retrospective cohort study includes 232 patients diagnosed with iCCA between January 2006 and August 2021 at the University Medical Center Mainz, Institute of Pathology. The iCCA subtypes were diagnosed based on typical macroscopic growth and localization as well as combination of histopathological parameters. Detailed clinical course data are continuously maintained in an existing relational Access database . For the current analysis, we retrieved baseline characteristics, survival data, concomitant diseases, and surgical characteristics. We assessed complication rates according to the Dindo–Clavien classification, with major complications defined to be requiring at least surgical, endoscopic, or radiologic intervention .®, Medtron, Germany). The patients underwent an abdominal CT scan with an arterial phase tube voltage of 80 kV or 100 kV and a venous phase tube voltage of 120 kV. Tube current was modulated between 120 and 800 mA. Axial images were acquired with a slice thickness of 0.625 mm. During post-processing, images with a slice thickness of 1 mm, 3 mm, and 5 mm were reconstructed in the axial orientation and in sagittal and coronal views. The MRI was performed with different scanners: 1.5 T Sonata®, 1.5 T Avanto®, 3 T Trio®, 3 T Skyra® or 3 T Prisma ® . All patients underwent a similar imaging protocol comprising of four dynamic, contrast-enhanced, T1-weighted fat-saturated, three-dimensional acquisitions and a delayed T1-weighted fat-saturated transversal acquisition. The contrast agents were Magnevist® , Dotarem®  or Primovist® , which were applied with a body weight-adapted dosage (1.5 mL/kg body weight) and injection rate of 4 mL/s using an automated injector . We accepted externally produced CT or MRI if it was of sufficient quality. The radiologists were blinded from the histological subtype of iCCA. In patients with available CT images, the average Hounsfield units (HU) of liver and tumor tissue were measured in a central region of interest. Tumor density was standardized relative to background liver density, both measured in HU during the late arterial phase. We classified the growth pattern as either mass-forming or ductal-growing. The ductal-growing pattern was defined by a growth pattern at or around existing bile ducts. The relationship between the portal vein and hepatic artery was also assessed and categorized as follows: no contact, invasion, and entrapment. The minimal tumor-free distance between tumor and serosa as well as the portal bifurcation was measured.Preoperative imaging data were available from 191 out of 232 patients. Contrast-enhanced CT and MRI were performed in 146, and 45 cases, respectively. CT studies were routinely performed using a 256-, 64-, and 16-slice CT scanner . Iomeprol  was used as a contrast medium with a body weight-adapted dosage (1.5 mL/kg body weight) and injection rate of 4 mL/s using an automated injector  whole-slide tissue samples were histologically reevaluated for the presence of given histological parameters and classified into small- and large duct iCCAs according to the WHO classification of tumors of the digestive system  . The diaPrecursor lesions were classified accordingly. Depending on tumor location, as previously stated, we subdivided perihilar cholangiocarcinoma (pCCA) from iCCA . pCCA waImmunostaining was performed on TMA blocks sliced into 2–4 µm thick sections, deparaffinized, and rehydrated with decreasing concentrations of ethanol. Antigen retrieval and staining were conducted according to the manufacturer’s instructions  correlation coefficient for ordinal data. For multivariate analysis, the R environment for statistical computing ) and the Bias-Reduced Logistic Regression Statistics Software  in Free Statistics Software (v1.2.1), Office for Research Development and Education, (http://www.wessa.net/rwasp_logisticregression.wasp/)) (accessed on 2 May 2022) as proposed by Firth was used [p-value of ≤0.05 was considered statistically significant. Venn diagram has been visualized using the Venny Software ) [Survival analyses were plotted by the Kaplan–Meier method and compared by the log-rank test. To compare the distribution of categorical variables, we conducted chi-square tests using SPSS 27 Software . In addition, Cramér’s V was calculated and interpreted as follows: 0.1–0.3: small, 0.3–0.5: moderate, and >0.5: large effect size . We calcwas used ,43,44. Ay 2022)) .


Amygdaloid cysts are benign dysembryological cystic tumors that develop in the antero-lateral part of the neck; they represent 2% of laterocervical tumors of the neck; they are among the most frequent gill anomalies; they represent 6.1% to 85.2% of second cleft anomalies. They are due to the persistence of the cervical sinus during the differentiation of the branchial apparatus. They are manifested by a laterocervical swelling located at the anterior edge of the sterno-cleido-mastoid muscle. Their cystic nature is confirmed by ultrasound and CT. Treatment consists of surgical excision.We report the case of a 33-year-old man who consulted for a huge right laterocervical swelling that had been evolving for 16 months without any other associated symptoms. An exploratory cervicotomy with an anatomo-pathological study was performed, and the histological diagnosis retained was an amygdaloid cyst without signs of malignancy.The objective of this work is to analyze the anatomo-clinical characteristics and discuss the methods of management and the therapeutic indications of this affection. Amygdaloid cysts are among the most common gill anomalies, accounting for 6.1% to 85.2% of second cleft anomalies. They are due to the persistence of the cervical sinus during the differentiation of the branchial apparatus. The usual site is the middle third of the anterior border of the sterno-cleido-mastoid muscle, but they can be located at any point from the middle constrictor muscle of the pharynx to the supraclavicular region. We report this rare case of a huge laterocervical amygdaloid cyst [A 33-year-old patient with chronic smoking and occasional alcoholism and no other notable pathological history presented for 16 months with a right laterocervical swelling that gradually increases in volume without associated otological or rhinological signs; clinical examination revealed an enormous right laterocervical tumefaction stretching from the tip of the mastoid above to the supraclavicular region below, painless, slightly mobile, non-pulsatile, measuring almost 70 mm in long axis, of renitent consistency, its full limit was impossible to specify, the adjacent skin was healthy. Examination of the oropharynx, nasopharynx, and pharyngolarynx was normal. Contrast-enhanced computed tomography objectified the presence of Figure The cervico-facial MRI found a cystic mass under the right angulo-mandibular wall with a thick wall in its upper part of the same dimensions associated with lymph nodes in the territories of IIa and IIb, suggesting a cyst in the second branchial cleft Figure .The PET scanner Figure  shows a Histological examination confirmed the diagnosis of an amygdaloid cyst without signs of malignancy Figure .Cysts and fistulas of the face and neck are uncommon and poorly understood congenital malformations. The ENT specialist must recognize these lesions early to allow for appropriate management ,2. TonsiClinically, a cervical sinus cyst presents as an oval kidney swelling, mobile with respect to the superficial plane, usually located near the carotid bifurcation in a subhyoid position ,5. The lCT or MRI are particularly indicated to differentiate the lesion from other parapharyngeal tumors: hemangioma, lymphangioma, metastatic adenopathy, whose distinction with a degenerated amygdaloid cyst or an intracystic metastasis is very difficult and the confirmation remains anatomopathological after a surgical removal ,2. MagneHistologically, the tonsillar cyst is lined by an epithelium that is most often squamous , but it Amygdaloid cysts are benign dysembryologic cystic tumors developing in the anterolateral portion of the neck. They mostly result from branchial cleft abnormalities. They are due to the persistence of the cervical sinus during the differentiation of the branchial apparatus. They manifest as a laterocervical swelling in the anterior edge of the sternocleidomastoid muscle. Their diagnosis is suspected based on physical examination oriented by imaging data and confirmed by an anatomopathological examination. Therapeutic management is always surgical and should be performed as early as possible to limit the risk of inflammatory changes related to infectious episodes.


Astrocytic tumors are primary central nervous system tumors. They are the most common tumors arising from glial cells. In the new WHO classification 2021, adult-type diffuse astrocytic gliomas subdivide into isocitrate dehydrogenase (IDH)-mutant astrocytoma, IDH-mutant and 1p/19q-codeleted oligodendroglioma, and IDH-wildtype glioblastoma. The T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign describes the MRI appearance of IDH-mutant astrocytoma, it is considered a highly specific radiogenomic signature for diffuse astrocytoma, as opposed to other lower-grade. MRI is the first and most accurate diagnostic tool for low-grade gliomas (LGGs). It is particularly helpful in distinguishing a diffuse astrocytoma from an oligodendroglioma that will not demonstrate T2-FLAIR mismatch. The tumor displays a hyperintense signal on T2-weighted images and a hypointense signal on T2-weighted FLAIR images, which distinguishes it from other types of diffuse gliomas. We report a case of a 29-year-old female patient who was diagnosed with IDH-mutant 1p/19q-non-codeleted diffuse astrocytoma based on MRI T-2 FLAIR mismatch sign, which is confirmed by the molecular analysis in the pathology lab. Our aim of this report is to confirm the power of the MRI findings in the diagnosis of glioma genotypes and to assess neurosurgeons in the preoperative surgical planning. Low-grade gliomas (LGGs) are tumors arising from the supporting glial cells of the central nervous system, accounting for 15% of all primary brain tumors. These brain tumors have been graded within type from Grades 2 to 4 according to the 2021 WHO classification .Isocitrate dehydrogenase (IDH)-mutant diffuse astrocytoma is a subtype of LGG that primarily affects young adults with a mean age of 39.4 years and standard deviation of 17.3. Patients with this condition usually present with seizures and worsening headaches. The temporal and frontal lobes are predominantly involved, and the tumor resection significantly improves overall survival. Patients with IDH-mutant diffuse astrocytoma have a 3.9 to 10.8 years of median survival time [The “T2-fluid attenuated inversion recovery (FLAIR) mismatch” sign can identify a lesion, which appears on T2- weighted images as a high signal and on T2- weighted FLAIR sequences a low signal, except for a high signal in the peripheral rim . It is aDiffuse LGGs are diagnosed based on the histopathological and molecular features, such as IDH 1 and IDH 2 gene mutations and the codeletion of short and long arms of chromosomes 1 and 19, as per the WHO 2021 classification [The treatment of IDH-mutant diffuse astrocytoma is highly dependent on the T2-FLAIR mismatch sign, and a 2021 meta-analysis showed a pooled specificity of 100% for tumors with an IDH-mutant, 1p and 19q non-codeleted variant, which makes it reliable in the diagnosis of IDH-mutant diffuse astrocytoma and in distinguishing it from other diffuse gliomas, although the pooled sensitivity was 42% .Here, we present a case study of a patient with IDH-mutant 1p and 19q-non-codeleted diffuse astrocytoma that was diagnosed pre-operatively with the help of MRI.A 29-year-old Saudi Arabian woman, newly diagnosed with an undifferentiated connective tissue disease based on positive antinuclear antibodies, anti-Ro/SSA, and anti-La/SSB. However, she had negative double stranded DNA and rheumatoid factor. She was on hydroxychloroquine 200 mg and regularly follows up with the rheumatology clinic.She was admitted for involuntary jerky movements on her left side of body while sleeping, in addition to headache, numbness and blurred vision for one year. Upon examination, her cranial nerves were intact, power was five out of five on all limbs, sensations were intact, and gait was normal.The non-contrast MRI studies revealed a left temporal cortical and subcortical lesion with a homogenous hyperintense signal on T2 weighted imaging in the central aspect of the lesion. No peritumoral edema or mass effect was observed Figure .On FLAIR images, the left temporal cortical and subcortical lesions had central suppression of the central aspect of the lesion and a high-signal peripheral rim Figure .The lesion was well-defined, with no internal or peripheral enhancement. No reaction to the adjacent bone or meningeal enhancement was observed Figure .Two weeks prior to surgery hydroxychloroquine was stopped and she underwent left temporal craniotomy and excision of the low-grade lesion, and a sample was taken for histopathology and molecular analysis.The sample confirmed the cellular infiltration of the LGG. It exhibited round nuclear contours and distinct cellular borders based against the background of scattered capillary-sized vessels, similar to ‘chicken wire appearance’. The tumor cells were positive for glial fibrillary acidic protein (GFAP) and synaptophysin and exhibited a focal to patchy reactivity pattern suggestive of p53 among scattered neurons. The Ki-67 proliferative index was low . These features are consistent with those of WHO grade 2 gliomas. Genetic analysis was positive for the IDH 1 R132H gene mutation and negative for the 1p and 19q codeletion.Postoperatively, the patient was admitted to an intensive care unit for observation, which was uneventful. Day one post-operative MRI showed early postsurgical changes after gross resection of the left temporal mass, and no residual tumor was identified. She was shifted to the ward and kept under neuro-observation, where in the course of her stay the jerky involuntary movements stopped, her headache improved, and daily neurological examination showed intact cranial nerves, coherent speech, no focal neurological lesion signs, she had good power of limbs bilaterally and her pupils were equal and reactive to light. The patient was discharged home and continued hydroxychloroquine.She received one month of daily 100 mg of temozolomide (TMZ) and radiotherapy of 5,400 centigray (cGy) in 30 fractions to the left temporal lobe by the oncologists. During her 10th month follow up, MRI showed stable surgical changes in the left temporal lobe with no evidence of tumor recurrence (Figure The diagnosis of LGGs is made using imaging, histopathology, and molecular diagnostic methods. The current diagnostic imaging modality of choice is MRI, which indicates a homogeneous tumor with hypointense signal on T1-weighted sequences and hyperintense signal on T2-weighted and FLAIR sequences . Across The clinical implications of T2 mismatch sign in the setting of LGGs have been limited. The mismatch sign under strict criteria is able to diagnose IDH mutant non-codeleted astrocytoma preoperatively in adults with suspicious intracranial masses suggestive of diffuse gliomas. In addition, the T2 mismatch sign can be used as a predictive tool for the IDH status regardless of the 1p/19q status, where IDH wild type LGGs can be excluded with high confidence . In thisVarious studies differ in the significance of the mismatch alongside other parameters. Kapsalaki et al. reported that implementing additional co-registration imaging parameters have no significance in improving the diagnostic accuracy of the mismatch sign . AnotherThe IDH 1 mutation in LGG has recently been identified as a common early mutation, which is rarely observed in primary glioblastoma. Several studies have reported the role of IDH 1 mutations in the malignant progression of LGG. This indicates that a diffuse glioma has less aggressive behavior, favorable symptoms, and is less likely to undergo malignant transformation .Generally, the main treatment options of LGGs are surgical resection, followed by radiation and chemotherapy . Huang eThe T2 flair mismatch is a highly specific non-invasive radiological marker for diagnosing IDH-mutant non-codeleted astrocytoma in adult patients, particularly younger than 40 years old. It also able to predict the IDH status and exclude IDH wild. This non-invasive MRI finding can aid in the treatment decision processes in a significant proportion of patients presenting with non-enhancing, LGG-like lesions.


The Ewing's sarcoma Family of Tumors (EFT) includes classic Ewing's Sarcoma (ES) of bone, Extraskeletal Ewing's Sarcoma (EES) and malignant peripheral primitive Neuroectodermal Tumor (pNET) of bone and soft tissue.ES is an aggressive tumor with a high incidence of local recurrence and distant metastasis, which is more common in males respect to females, particularly in the first 2–3 decades of life.A 16-year-old male patient presented to our institute in June 2016 with a history of 6 months of odynophagia. Laringoscopy revealed a red mass located in the right part of base of tongue extending to homolateral glossoepiglottic vallecula with partial obstruction of airway. No enlarged lymph nodes were palpated.Total body contrast-enhanced Computed Tomographies (CT) scan revealed a 3–3.2 cm isointense mass located in the right part of tongue base extended to soft tissue with soft tissue surrounding and with a very poor contrast enhancement. No information of the nature of the mass was achieved by imaging. No neck lymphadenopathy and metastasis were evidenced . Due to Intraoperative frozen histology sections was performed but not diriment and therefore anatomopathologist asked for a larger amount of tissue. We decided to remove completely the mass performing partial glossectomy .Figure 2The margin of the resection, examinated with introperative histology, were free of any disease.Histological examination revealed a Extraskeletal Ewing's Sarcoma (EES). The microscopic examination of the specimen revealed a population of epithelioid cells with round nuclei and scant cytoplasm, a high number of mitotic figures and focal necrosis. The tumor cells were immunohistochemically positive for CD99 and Bcl-2 and negative for leukocyte common antigen (CD45), S-100, cytokeratin AE1/AE3 and desmin. Furthermore, interphase Fluorescence In Situ Hybridization (FISH) revealed a t translocation .Figure 3The patient was discharged after 2 days and recovered after 15 days.The patient began chemotherapy with ifosfamide, etoposide, vincristine, doxorubicin, cyclophosphamide ×4 cycles each. There has been complete response to therapy with no evidence of recurrence on CT imaging after 4 months.Ewing Sarcoma (ES) was first described in 1921 by James Ewing. It's a highly malignant bone tumor that involves children and young adults. Currently these tumors are classified as ES Family of Tumors (EFT) and include Ewing Sarcoma (ES) Extraskeletal Ewing Sarcoma (EES) and Primitive Neuroectodermal Tumor (PNET), which shows more neural differentiation.The imaging findings are nonspecific in EES, and can help only in localization, extension of the tumor and involvement of surrounding anatomical structures. The reported CT findings in ESS is mostly a mild heterogeneously enhancing mass. On MRI has an isointense signal in T1 if compared to muscle, a hyperintense signal in T2 weighted images and a low and heterogeneous enhancement.Our case showed a mild contrast enhanced mass without bony structure, vascular structure and lymphonodes of the neck involvement. Imaging findings were not suggestive for any diagnosis so we decided to perform a biopsy in order to plan further treatment.The diagnosis of EES is histological with a typical immunohistochemical pattern.Histologically ESS consists of a monotonous population of small, round, hyperchromatic cells with scant cytoplasm. The malignant cells appear undifferentiated and frequently lack any clear morphologic features. Necrosis, Homer-Wright rosettes and a background with neurofibrillary material are variably present.Confirmation of histologic findings can be achieved through molecular and cytogenetic studies. The characteristic chromosomal translocation t is found in about 90% of ES, ESS and Pnet.EES are rare that and therefore guidelines regarding therapy and surgery are still discussed.Complete surgical excision, when feasible, is regarded as the best modality of local control, given the higher risk of local recurrence when radiotherapy is used as the sole treatment of the primary tumor. Radiotherapy alone (in the range of 45–60 Gy) should be applied if complete surgical excision is impossible. Postoperative radiotherapy should be given in cases of inadequate surgical margins or in presence of metastases and therefore, in our case, it was not carried out. Combining surgery and chemotherapy, survival is ∼60% to 70% in localized tumor. All current trials employ 6–10 cycles of chemotherapy usually applied at 2 to 3 week intervals. Treatment duration is thus 10–12 months. Agents considered most active include doxorubicin, cyclophosphamide, ifosfamide, vincristine, dactinomycin, and etoposide.About surgical strategy, we decided to perform a Trans-Oral Robotic Surgery (TORS). TORS has been used with good results for benign pathologies such as the surgical treatment of sleep apnea (OSAS)Over the past 10 years, there have been increasing reports of the use of primary radiation or combined chemotherapy and radiation for tongue base neoplasms. The key factor driving this movement away from primary surgery was the reported morbidity of such surgical procedures.2 laser pharyngeal surgery reintroduced primary surgery as a means of treating base of tongue cancer, but this technique is technically challenging, has a steep learning curve, and a limited operative field of view because it is performed through a laryngoscope. Furthermore, the transoral laser surgery requires cutting directly through the tumor to determine extent of resection. Nonetheless, the transoral approach did reveal that 92% of patients achieved swallowing without permanent gastrostomy tube.Cervical incisions and dissections with mandibulotomy or pharyngotomy were typically required to remove base of tongue neoplasms even in the early stages. These approaches left the patient with various levels of significant speech and swallowing dysfunction as well as cosmetic deformity. The introduction of endoscopic laser microsurgery for tongue base cancer has been reported by Steiner et al.We believe that TORS for tongue base lesions has significant advantages over both classical open tongue base surgery and laser microsurgery. With respect to open approaches, it is well known and widely reported that open surgery of the tongue base has obvious negative impact on both functional and cosmetic outcomes. TORS eliminates risk of fistula and infections and the need for mandibulotomy with a lip split or visor flap or transpharyngeal approaches that adversely affect chewing, swallowing and speech function, and cosmesis. In addition, we believe that TORS tongue base resections can be performed safely without tracheostomy, which is typically used for open approaches.TORS offers several potential advantages over laser base of tongue surgery as well. Although the standard operating microscope used in laser procedures provides excellent direct visualization to an exposed area, it cannot view around corners or cannot be rotated along three-dimensional axes. The 0 or 30 degree angles and the maneuverability of the robotic endoscopes is a key issue in the ability to achieve en bloc resections of the tumors with negative margins like as in our case. Laser microsurgery is essentially one-handed surgery, whereas robotic surgery is two- or even four-handed (if the assistant is included), and tissue manipulation and retraction is comparable with open surgery. It is our impression that TORS offers a wider variety of technical options for hemostasis compared with transoral laser microsurgery. Hemostasis was easily managed in the live surgeries with either monopolar or bipolar cautery robotic instrumentation and the use of small-sized hemoclips.The patient has signed an informed consent allowing us to use his medical records.Extraskeletal Ewing's sarcoma are rare tumor but that can arise in the head and neck region. A combination of multiple diagnostic modalities, surgery and chemotherapy represent the correct treatment in these cases. In particular for the tumors of base of tongue, TORS offers a complete excision of tumor and therefore a best outcome.The authors declare no conflicts of interest.


A 48‐year‐old female patient presented with longstanding unrecognized celiac disease (CD), a family history of CD, and a short duration of alarming symptoms. The diagnostic evaluation revealed the concomitant presence of small and large bowel ulcers raised a dilemma about differential diagnosis in her case. Pathologic examination of tissue specimens from the jejunal ulcer led to the diagnosis of enteropathy‐associated T‐cell lymphoma. In recent years, the availability of modern cross‐sectional imaging and endoscopy modalities has dramatically improved the detection and characterization of small bowel lesions. Characterization of small bowel ulcers by endoscopy and radiology imaging in a patient with suspected complicated CD (CCD) needs to be made in conjunction with all clinical factors, as there is a wide overlap of the possible etiologic factors. Enteropathy‐associated T‐cell lymphoma is a highly aggressive T‐cell lymphoma with a poor prognosis, since early diagnosis and appropriate treatment may be delayed due to nonspecific clinical and endoscopic presentation. Therefore, it is crucial to timely recognize patients with suspected CCD and properly navigate diagnostic imaging tools, acquire adequate biopsy, and perform immunophenotyping to set early diagnosis in patients with diffuse intestinal ulcers and CD. Celiac disease (CD) is an autoimmune enteropathy caused by gluten exposure in genetically predisposed individuals, characterized by intraepithelial lymphocytosis, crypt hyperplasia, and villous atrophy of small bowel mucosa. There has been a substantial increase in the number of CD diagnoses in recent years, while many patients still remain undiagnosed.Although rare (around 1% of patients diagnosed with CD), the complications of CD include hyposplenism, refractory CD (RCD), intestinal lymphoma, small bowel adenocarcinoma, and ulcerative jejunoileitis.Duration and dose of gluten exposure appear to be risk factors for RCD and EATL. Patients with persistent villous atrophy have a significantly higher risk of lymphoproliferative malignancy compared to those with mucosal healing, so it is important to emphasize that in most cases, EATL does not develop from well‐treated CD but rather in the setting of type 2 RCD 2.Herein, we report a case of longstanding, unrecognized CD complicated with EATL in a 48‐year‐old female patient. We also discuss challenges in the diagnostic assessment of patients with suspected CD complications.9/L, reference range 158–424 × 109/L), normal values of inflammatory parameters , and positive serology for CD . Esophagogastroduodenoscopy revealed scalloping of folds and fissuring of intervening duodenal mucosa , so she was admitted to our gastroenterology department for re‐evaluation. Laboratory workup revealed increased inflammatory parameters  and hypoalbuminemia , with negative blood cultures. Computerized tomography revealed multiplied and enlarged mesenteric lymph nodes. Lower endoscopy showed multiple ulcerations in all segments of the large bowel with normal surrounding mucosa  as the first presentation of CD was established. The patient was treated with chemotherapy but during the further clinical course, she developed lethal complications and died 6 months after establishing the diagnosis of EATL.The prevalence of CD is increasing in Western countries for reasons that are currently unknown.Our patient with longstanding unrecognized silent CD and strong family history presented with a short duration of alarming symptoms that were recognized as signs of complicated disease and prompted further evaluation. During the endoscopic diagnostic evaluation, ileocolonoscopy revealed multiple colon ulcers and histology showed nonspecific findings.At this point, we considered several small bowel imaging procedures that would be valuable in this setting. According to recent guidelines, capsule endoscopy is considered a first‐line imaging procedure for small bowel investigation.As radiologic techniques allow visualization of the entire small bowel, computerized tomography or magnetic resonance enterography may have been the best option for our patient but due to the high cost and lack of expertise, these are not readily available in all centers, including our hospital. Furthermore, positron emission tomography scan has been shown to be effective in the detection of CD‐associated lymphoproliferative disorders such as EATL, and could therefore be suggested in addition to other cross‐sectional imaging techniques in this setting.So, in order to localize the lesion in the small bowel in our patient, we decided to perform SBFT which revealed an ulcer in the mid‐small intestine. The newer, improved cross‐sectional imaging techniques have mostly replaced fluoroscopic examination as a primary tool for small bowel evaluation. However, some studies suggest that SBFT continues to be used in the setting of inflammatory bowel disease and other disorders.The results of recent studies suggest that the endoscopic approach has advantages over cross‐sectional imaging investigations in patients with suspected complicated CD (CCD),In our patient, an orthograde single balloon enteroscopy was subsequently performed and revealed jejunal ulcers. Histology was compatible with the diagnosis of EATL, a rare and aggressive complication of CD.Small and large bowel ulcers in a patient with positive serology and a family history of CD raise a strong suspicion of a complication of untreated CD, although other differentially diagnostic possibilities must be considered.Three main points of clinical importance are highlighted in this case report. First, early family screening and initiation of GFD are essential for the prevention of neoplastic complications such as EATL. Identification of the subgroup of CD patients at risk deserving an early diagnostic approach is mandatory and even more urgent given the availability of high‐performing imaging tools for small bowel. Second, differentiation of small intestine ulcerations based only on the endoscopic image is not possible as there is a wide overlap, so it is essential to take clinical context into account. Histology is crucial as it can provide diagnostic confirmation. Third, although suitable accuracy for small bowel tumors has been described for radiologic imaging, endoscopy appears to be the most appropriate approach when suspecting early‐stage small bowel tumors.The authors declare no conflict of interest.The corresponding author and the co‐authors are going to overtake the costs.Since the patient died, the Helsinki Declaration is not applicable. The relatives agree with publishing the case report.Video 1. Single‐balloon enteroscopy showing large ulceration in the proximal jejunum.Click here for additional data file.


Although Bochdalek hernias are uncommon in adults and difficult to diagnose, such congenital diaphragmatic hernias (CDHs) are some of the most serious malformations among newborns. In particular, CDHs are accompanied by high mortality and marked disability later in life due to concomitant morbidity  and require long-lasting neonatal treatment in an intensive care unit. Late-diagnosed CDHs are extremely rare and always show small defects that justify a better prognosis than CDHs with large defects. In most cases, such CDHs go undiagnosed due to their mild, delayed symptoms. In the case described here, an 18-year-old Asian male presented with abdominal pain and a bowel contour in the epigastric region and left upper quadrant. A chest X-ray and thoracoabdominal computerized tomography scan confirmed the diagnosis of bowel obstruction due to a left diaphragmatic hernia. Moreover, a defect was revealed in the posterolateral left diaphragm, and the transverse colon, spleen, and half of the stomach had herniated through it. A segmentectomy was performed on the transverse colon, followed by an end-to-end anastomosis, a diaphragmatic plasty, and the placement of an intrapleural catheter. In conclusion, diagnosing a diaphragmatic hernia before surgery can be difficult due to its rarity and wide range of symptoms. Although prenatal diagnosis using ultrasonography is possible in up to 80% of fetuses, that method may not be available in all regions in Indonesia or in all countries, where doctors thus continue to face a diagnostic challenge when dealing with CDHs. Congenital diaphragmatic hernia (CDH) is defined by an abnormality in the diaphragm that causes the contents of the abdomen to protrude into the thoracic cavity, thereby impairing lung development , 2. CDH CDH includes Bochdalek hernias (BHs) (70%), which are present in the posterior-lateral diaphragm, and Morgagni hernias 25–35%), which occur in the anterior or central diaphragm (2–5%) . Because5%, whichWe herein report an uncommon case in which an adult male was suffering from a left-sided strangulated CDH of Bochdalek's type, presenting with intermittent abdominal pain.An 18-year-old Asian male with a two-day history of sharp and intermittent pain on the left side of his abdomen presented to the emergency department (ED). The pain decreased while lying down and increased while eating. The patient was treated in the ED for three hours. Despite being treated with a strong opioid analgesic (fentanyl), the pain worsened, so he was referred to the surgery department for a consultation. The patient had a history of recurrent pneumonia as a toddler, and he reported experiencing tightness in the chest after eating over the previous 12-month period. This patient had no prior surgeries or trauma, did not drink alcohol, and smoked one pack of cigarettes daily.The patient complained of an inability to pass flatus or stool for 2 days. He was alert, and his vital signs were as follows: blood pressure of 100/70 mmHg; a temperature of 36.5°C; a respiratory rate of 22 breaths per minute; a heart rate of 108 beats per minute; and a Karnofsky score of 80. On auscultation of the right and left hemithorax, the patient's breath sounds were normal. The patient's abdominal examination showed a bowel contour in the epigastric region and left upper quadrant (LUQ) , and, onThe left hemidiaphragm was elevated, and there was minimal pleural effusion evident in the posteroanterior view chest X-ray. The three-view abdominal radiograph series showed that the left hemidiaphragm was filled with gas, distended, and elevated. The thoracoabdominal computerized tomography (CT) scan without contrast showed a left-sided diaphragmatic defect with herniation of the bowel into the thoracic cavity . LaboratAfter this, we performed an exploratory laparotomy via a midline incision, wherein the colon ascendens and colon transversum appeared extremely dilated. An obstruction was observed on the colon ascendens and a small part of the colon transversum. There was an 8 cm defect in the posterolateral left diaphragm, through which the colon transversum, spleen, and half of the stomach had herniated . The colVincent Alexander Bochdalek was the first to describe BH in 1848 , 8. A BHThe etiology of CDH is mostly unknown, but it is currently assumed to be multifactorial. The majority of instances demonstrate a single diaphragmatic abnormality manifesting as pulmonary hypoplasia and persistent pulmonary hypertension in newborns. CDH is related to genitourinary, cardiac, and gastrointestinal abnormalities as well as chromosomal aneuploidy, such as trisomies . A globaThere are two common clinical presentations of adult CDH: (1) incidental detection during X-rays for symptoms unrelated to the diaphragmatic hernia or (2) symptoms that arise from a visceral rupture within the chest cavity, followed by organ strangulation and incarceration. Symptoms vary depending on which organ is affected: vomiting, dysphagia, and intermittent abdominal pain are common digestive symptoms, while chest pain and dyspnea are common respiratory symptoms . PatientA chest X-ray and barium assays were used to make the diagnosis. Organs, fluid, and air can be seen in the chest above the diaphragm . The besPerrone et al. assert in a systematic review that surgery is the preferred treatment and that the preoperative context has a significant impact . The surDue to recent advancements, minimally invasive techniques (thoracoscopy and laparoscopy) have become more feasible and safer, resulting in shorter hospital stays and decreased morbidity rates , 11, 14.Compared to other congenital anomalies worldwide, the CDH mortality rate is high; the rates in Costa Rica and South America are 54.8% and 56.7%, respectively . AccordiIn conclusion, definitive diagnosis of a diaphragmatic hernia before surgery is difficult because it is rare and has a variety of symptoms. X-rays and CT scans are helpful in detecting CDH. Prenatal diagnosis using ultrasonography is possible in up to 80% of fetuses, and that method may not be available in all regions in Indonesia or in all countries, where doctors thus continue to face a diagnostic challenge when dealing with CDHs.


Hemangioma is a common tumor accounting for 8 to 10 % of benign neoplasm. However, hemangioma arising from blood vessels is rare and even rarer if the vessel involved is anterior jugular vein (AJV). AJV hemangioma can be confused with jugular phlebectasia, laryngocele, thyroglossal cyst, simple cyst, or other vascular malformation of same origin. They should be considered in differential of midline/paramedian neck swelling. Surgical resection is the treatment of choice whenever possible, and even allows for histopathological evaluation and a confirmatory diagnosis. Being an extremely rare entity, there is paucity in literature about it and more publications are required to extend understanding and eliminate existing doubts about the pathology. We present an extremely rare case of AJV hemangioma that presented as a painless midline swelling and was initially confused as simple neck cyst on radiological assessment. We believe that this is the second only case of AJV hemangioma reported in English literature. Vascular tumors and anomalies can present as solitary neck swelling in adults. Their designation into hemangiomas or vascular malformations has never been easy, since more classification systems have been proposed over the years, as these anomalies have been thoroughly studied.A 36-year-old female presented with a complaint of painless midline neck swelling. She first noted a small nodular swelling 6 months before, which had gradually increased in size. She was otherwise healthy and did not report a history of alcohol consumption or of smoking. No genetic or syndromic abnormalities were reported from her family. There was no history of preceding neck trauma, surgery, or upper respiratory tract infection.On physical examination, there was a nonpulsatile, soft, and nontender left paramedian neck swelling that moved slightly with deglutition. Overlying skin illustrated no erythema, warmth, or tenderness. No cervical lymphadenopathy was noted. Her laboratory reports including complete blood count, basic metabolic panel, chest X-ray, and electrocardiography were unremarkable. On ultrasonography, a well-defined multiseptate, cystic lesion measuring 5.5 × 2.4 cm seen within the subcutaneous tissue in the left cervical region, anterior to the thyroid gland. A simple neck cyst was suspected on radiological assessment. Being uncertain about the diagnosis, the lesion was explored under general anesthesia.A surgical exploration was undertaken under general anesthesia with oral intubation using flexometallic endotracheal tube and following anesthesia medications: premedication , induction (injection propofol 100 mg and injection succinylcholine 100 mg), relaxant (injection cisatracurium 10 mg), and analgesia (injection fentanyl 100 µg and injection paracetamol 1 gm). Patient was kept in supine position with neck slightly extended and a horizontal skin crease incision was taken. Subplatysmal skin flaps were raised both superiorly and inferiorly. A mass arising from the left AJV was isolated after careful dissection . The maThe histopathological examination of resected tissue showed collection of abnormal veins of varying sizes and proportions. Some vessels are large and thin walled whereas others showed compressed smooth vessel in the wall. Foci of wall thickening with infiltration by siderophages, histiocytes, and lymphocytes were noted. Organized thrombi were also noted . The enA hemangioma is a usually benign vascular tumor derived from blood vessel cell types. There are various types of hemangiomas. The more common types include capillary, cavernous, combined, and lobular capillary types. There are very few reports of primary venous hemangiomas in the literature. Some authors consider these lesions to be present since birth, while others consider it to be an acquired process caused by trauma, sepsis, pressure changes, and hormonal alterations.In contrary to various childhood vascular tumors, venous hemangiomas, like the one in present report, do not show signs of involution.As a primary tumor, hemangioma that originates from the vessel wall and particularly in the wall of EJV or AJV is extremely rare, while that arising from the wall of AJV is slightly more uncommon. The first report of preoperative diagnosed case of hemangioma arising from EJV was reported by Meyers in 1967.We report a particularly rare case of hemangioma arising from AJV. We believe that this is the second case of AJV hemangioma reported in English literature; the only prior case of AJV hemangioma was reported by Irfan et al in 2010.Treatment options include watchful waiting, sclerotherapy, embolization, and surgical excision.Differentials of AJV hemangioma/EJV hemangioma include jugular phlebectasia, laryngocele, thyroglossal cyst, branchial cyst, simple cyst, or other vascular malformation of same origin, including aneurysms.AJV hemangioma is an extremely rare lesion and should be considered in differential diagnosis of midline/paramedian neck swelling. They can be confused with jugular phlebectasia, laryngocele, thyroglossal cyst, simple cyst, or other vascular malformation of same origin. Clinical examination and radiological assessment can aid in diagnosis and surgical planning; however, they can sometimes cause diagnostic dilemma with close differentials. Surgical excision is the preferred treatment whenever possible, and even allows for histopathological evaluation and a confirmatory diagnosis. Because of its rarity, a comprehensive data is lacking and more publications are required to have a better understanding about the pathophysiology, presentation, and management of this tumor.