A regional sampling of ACTH was done to confirm the hepatic lesion as the source of EAS, and a definitive ACTH gradient was observed between the hepatic vein and the right internal jugular vein. Further, a preoperative biopsy of the lesion revealed a small round cell tumor with positive immunostaining for ACTH and synaptophysin, suggestive of a neuroendocrine tumor. The patient was managed with partial hepatectomy, resulting in hormonal and clinical remission of Cushing syndrome. In a systematic review of pediatric EAS due to primary hepatic tumors (n = 11), calcifying nested stromal epithelial cell tumors were the most common. EAS-associated hepatic tumors were larger (≥10 cm) except benign primary hepatic neuroendocrine tumors (PHNET). The latter were misdiagnosed as hemangioma in two cases by anatomical imaging but correctly diagnosed by somatostatin receptor scintigraphy. Hepatic tumors causing EAS in children required extensive resection, except benign PHNET. Nevertheless, all benign tumors with an uncomplicated perioperative course demonstrated disease-free survival over a median follow-up period of two years.Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rare in children, and localizing the source of EAS is often challenging. Here, we report EAS in an adolescent boy who presented with Cushingoid features and had endogenous ACTH-dependent hypercortisolism on hormonal evaluation. Abdominal ultrasound and CT revealed a hepatic lesion with characteristics suggestive of hemangioma, whereas the lesion was tracer non-avid on  Endogenous Cushing syndrome (CS) is rare in childhood and adolescence. Endogenous CS is either adrenocorticotropic hormone (ACTH)-independent  or ACTH-dependent. The most common ACTH-dependent CS is excess ACTH production from a pituitary corticotroph adenoma  but rarely may also result from ectopic ACTH syndrome (EAS) due to excess production of ACTH and/or corticotropin-releasing hormone (CRH) from various tumors outside the pituitary gland 10%). Localizing the source of ACTH-dependent CS is often challenging, especially in children. Frequent negative localization of pituitary microcorticotropinomas, the most common cause of CD, by MRI, unavailability of CRH, limited availability of bilateral inferior petrosal sinus sampling (BIPSS) in India, and inaccuracy of high-dose dexamethasone test (HDDST) pose challenges to distinguish CD from EAS. Even when a diagnosis of EAS is inferred from the aforementioned tests, localizing the source of EAS is a further diagnostic challenge. CT and MRI are the most commonly used initial modalities for localizing the source in EAS but may fail in approximately 33-44% of cases. A higher sensitivity (80-90%) has been reported for somatostatin receptor scintigraphy (SRS) . -11. 2-11Here, we report a child with ACTH-dependent CS and several challenges to localize the source of excess ACTH. A negative pituitary MRI suggested either missed microcorticotropinoma or EAS. An unsuppressed HDDST favored EAS whereas CRH-stimulation test or CRH-stimulated BIPSS could not be considered due to the unavailability of CRH in India and, hence, further evaluated with imaging of the chest and abdomen to localize the possible source of EAS . The com68Ga-somatostatin receptor (SSTR) PET/CT to detect the source of EAS [Misdiagnosis of PHNET on ultrasound as hemangioma is not uncommon. In one series, 10 of 11 patients with PHNET were initially diagnosed as hemangioma . EAS-asse of EAS , a subsee of EAS . SSTR PEAs the patient was not adequately responding to medical therapy (ketoconazole), further evaluation to explore the excess ACTH secretion from the liver lesion was considered. A selective venous sampling from common hepatic and right internal jugular veins demonstrated an ACTH gradient of >2 suggesting the lesion as the source of EAS. Peripheral regional venous ACTH sampling has been successfully used previously in a few cases with EAS . This isThe patient had clinical and hormonal remission of CS after excision of the PHNET with disease-free survival of five years. This remission was achieved due to the absence of metastases. Although simple excision may be an option for PHNET, as done in our patient, the majority of the other liver sources of pediatric EAS are large and most often need extensive liver resection. Nonetheless, disease-free survival is good. In the systematic review, except for one EAS patient with a hepatic tumor who died due to sepsis in the perioperative period, all others with benign tumors had disease-free survival until the last follow-up. Nevertheless, close clinical and hormonal monitoring for the recurrence of the disease is essential. With the increasing availability of late-night salivary cortisol, this can a simple and effective tool for the hormonal monitoring of disease recurrence in EAS patients.We report a rare case of pediatric EAS due to PHNET with diagnostic dilemmas and demonstrate the utility of novel diagnostic modalities such as documentation of a higher ACTH gradient in the hepatic vein and preoperative positive ACTH immunostaining of the suspected hepatic lesion to resolve the dilemma. In the systematic review, CNSET was the most common hepatic tumor associated with pediatric EAS. The majority of EAS-associated hepatic tumors (CNSET and hepatoblastoma) are large (≥10 cm) and need extensive liver resection, whereas benign PHNETs are relatively smaller (<5 cm) but are misdiagnosed as hemangioma by anatomical imaging. SRS often helps to diagnose PHNET but may rarely miss the diagnosis, as observed in our patient. In such scenarios, the aforementioned novel diagnostic options may be explored to confirm a hepatic lesion as the source of excess ACTH.


Peliosis hepatis remains a rare focal liver lesion with inconclusive imaging features. The unknown pathogenesis represents a wide possible range of etiologies including the breakdown of the sinusoidal borders, a potential hepatic outflow obstruction or dilatation of the central vein of a hepatic lobule. In histopathology, a blood-filled cystlike appearance with sinusoidal dilatation was reported. On ultrasound, B-mode features are not specific demonstrating a irregular, moreover hypoechogenic focal liver lesions. Postcontrast imaging features on Contrast-Enhanced-Ultrasound may mimic a malignant lesion with irregular contrast inflow and washout during late phase. Our case demonstrates a peliosis hepatis with malignant image features on contrast-enhanced ultrasound, ruled out by PET-CT and core needle biopsy with corresponding histopathological workup. Peliosis hepatis is known as a rare disease showing multiple blood-filled parenchymal cysts in parenchymal organs, which may be associated with a wide range of conditions varying from medication or infectious diseases and autoimmune diseases We demonstrate the case of a 79-year old female patient with conjunctival melanoma diagnosed in 2001. The patient was referred to the Department of Cardiology due to dyspnea under cardiac decompensation and subacute pulmonary embolism. Afterwards, the patient was referred to head and neck surgery to further perform imaging for tumor staging. Magnetic resonance imaging was avoided due to an implanted pacemaker.Positron emission tomography / computed tomography (PET/CT) revealed a 4 × 3 cm mass in the so-called Riedel-lobe, which is described as a normally absent, tongue-shaped process at the anterior margin of the right liver lobe overhanging the right kidney. The focal liver lesion (FLL) was slightly hypodense compared to the surrounding liver tissue on nonenhanced CT (Hounsfield units: 39 HU vs 59 HU in the surrounding liver tissue of the right lobe) and showed no sufficient contrast enhancement  after injection of 80 ml iodine-based contrast agent A and B. To further characterize the FLL, CEUS was performed by an expert in the field with more than 20 years of experience in this field (EFSUMB Level III). The ultrasound (US) protocol using a high-end US system with 1–6 MHz convex array transducer  included color-coded duplex sonography (CCDS) and superb microvascular imaging (SMI) to determine vessel structure. Afterwards, the liver lesion was examined in modified longitudinal and transversal planes with an up-to-date CEUS-specific protocol available at the time of the examination. On B-Mode US, the lesion was inhomogenous and hyperechogenic compared to the surrounding liver tissue A and B. A percutaneous US-guided core biopsy (16 Gauge needle) was performed due to the discordance between CEUS findings and PET/CT findings after an internal discussion of several disciplines. The histopathological report revealed no atypical cells suspicious for malignancy. The hepatic parenchymal specimen showed peliosis-like dilatation of the sinusoids and edematous widened portal fields with a mixed cell inflammatory .Fig. 4HiPeliosis hepatis is known as a rare disease with several underlying conditions, which is a challenge for diagnosis on established imaging modalities. Our case report shows a patient with suspicion of malignancy receiving a PET/CT scan and CEUS with discrepant findings on both modalities.Perfusion pattern of peliosis hepatis in CEUS are quite variable including a variety of B-mode findings with hyper-, iso- and hypoechogenicity In comparison to the results of the study by Dong et al., the lesion we present is in congruence with the described arterial hyperenhancement—but our case showed early progressive washout, whereas the authors concluded washout in the very late portal venous phase (after 1 minute) to be characteristic in peliosis hepatis Several contrast pattern findings for peliosis hepatis are known through the literature, including centripetal contrast enhancement mimicking hemangiomas or atypical enhancement patterns not suggestible for any typical FLL •Peliosis hepatis showed strong enhancement with irregular enhancement pattern•Early washout starting after 40 seconds post injection was present•CCDS depicted no macrovascularization, while microvascular imaging revealed some tiny vesselsCatchword phrases:Our patient demonstrated in the case report signed a written informed consent for publication/research purpose.


Dear Editor,.Wunderlich syndrome (WS) is an uncommon but potentially life-threatening entity andconsists of spontaneous renal or perirenal hemorrhageth postoperative day he was discharged. The pathological examination ofthe surgical specimen confirmed acquired cystic kidney disease (ACKD) and identifiedbleeding in some of the cysts, through the lacerated renal capsule and into theperirenal adipose tissue. There was no evidence of neoplastic disease.We report a case of a 56-year-old man with a history of end-stage renal disease due toAPOL1-mediated kidney disease on maintenance hemodialysis who was referred to theemergency department with left flank pain, hypotension, nausea, and vomiting of suddenonset during his routine hemodialysis session. He denied other complaints and was not onantiplatelet or anticoagulant drugs. Computed tomography (CT) angiography was performed,revealing 7-centimeter-long kidneys with several cystic lesions, as well as a subacuteleft kidney hematoma that extended into the perirenal space and pelvic cavity, with noevidence of active bleeding . The pat. Although cyst hemorrhage is a frequent complication in autosomal dominantpolycystic kidney disease (ADPKD), WS is uncommon both in ADPKD and ACKD. In patients on hemodialysis, it appears to result from a combination of factors,namely platelet dysfunction, oral anticoagulant medications, and heparinization of theextracorporeal circuit – nonetheless, WS may be associated solely with cyst or tumor development,without any other predisposing factors. The diagnosis must be confirmed radiologically, ideally by contrast-enhanced CT. Management may be conservative  or interventional . The first option entails the need for close initial surveillance (in order tointervene in case of deterioration) and subsequent radiological follow-up . While stable patients with benign conditions may be treated conservatively,angiographic embolization might be attempted when active bleeding is detected, andnephrectomy is preferred in the presence of instability or high likelihood ofmalignancy. Ultimately, the most appropriate treatment should be chosen on an individualbasis, depending on clinical stability, evidence of active hemorrhage, suspected cause,and risk/benefit ratio of the available alternatives.WS may present with the classic Lenk’s triad, characterized by acute flank pain, palpablemass, and hypotension; nevertheless, atypical presentations are more frequent. Potentialunderlying etiologies can be varied, and include mostly neoplasms, but also vasculardisease, cyst rupture, coagulopathy, or infectionR. Birne reports the following: employed by Diaverum; research funding from Gilead,Bayer, and Boehringer; member in advisory board of Bayer and Boehringer; and memberof the speakers bureau of AstraZeneca, Bayer, Boehringer, MSD and Bial. M. Roxoreports the following: employed by Fresenius Medical Care.


Lymphoepithelioma-like intrahepatic cholangiocarcinoma (LEL-ICC) is a rare variant of intrahepatic cholangiocarcinoma. Epstein–Barr virus (EBV) infection was considered to play a pivotal role in the tumorigenesis of LEL-ICC. It is difficult to diagnosis of LEL-ICC due to the lack of specific features regarding the laboratory test results and imaging findings. At present, the diagnosis of LEL-ICC mainly depends on the histopathologic and immunohistochemical examinations. In addition, the prognosis of LEL-ICC was better than classical cholangiocarcinomas. To our knowledge, only few cases of LEL-ICC have been reported in the literature.We presented a case of a 32-year-old Chinese female with LEL-ICC. She had a 6-month history of upper abdominal pain. The magnetic resonance imaging (MRI) showed a 1.1× 1.3 cm lesion in the left lobe of liver, appearing low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The patient underwent laparoscopic left lateral sectionectomy. The postoperative histopathologic and immunohistochemical examinations results allowed for the definitive diagnosis of LEL-ICC. The patient was free from tumor recurrence after a 28 months follow-up.In this study, we reported a rare case of LEL-ICC associated with both HBV and EBV infection. EBV infection might play a pivotal role in the carcinogenesis of LEL-ICC, and surgical resection is still the most effective treatment at present. Further research on the etiology and treatment strategies of LEL-ICC is required. Epstein–Barr virus (EBV) belongs to the herpesvirus family with oncogenic properties . EBV inf9/L (3.5-9.5), platelets 249×109/L (100–300), total bilirubin 12.5 umol/L (5.0-28), and AST 35 IU/L (<50). Serological testing for tumor marker of carcinoembryonic antigen (CEA) was 5.54 ng/ml  and hepatitis B surface antigen (HBsAg) was positive. The hepatitis B virus DNA (HBV-DNA) was less than 1×102 IU/ml (HBV-DNA ≥ 1×102 IU/ml was defined as HBV infection active), suggesting that HBV infection was inactive. The cancer antigen19-9 , CA125  and α-fetoprotein  was 25.6 U/ml, 13.3U/ml and 3.37, respectively. Abdominal computed tomography (CT) showed the lesion in the left lobe of liver was detected, and no tumor was detected in any other organs , white blood cells 7.12×10Post-operative recovery of the patient was well. The patient was discharged on postoperative day 5 with good general condition. The laboratory parameters were normal and we recommended regular follow-up in the outpatient clinic.via blood examination, ultrasonography (US), CT, and MRI. The systematic update of patients’survival information was performed once a year. The last outpatient follow-up was in August 2022, and the tumor markers were normal. The patient was free from tumor recurrence after a 28 months follow-up   . Previouization) , 3.A recent review by Ding et al.  identifiClinical presentations of LEL-ICC are nonspecific. Most patients were almost asymptomatic, and the hepatic lesion was identified during a routine health examination. Some patients have the upper abdominal pain, abdominal fullness or fever . The defThe prognosis of LEL-ICC was better than classical cholangiocarcinomas . NotableIn this study, we reported a rare case of LEL-ICC associated with both HBV and EBV infection. The patient underwent laparoscopic left lateral sectionectomy based on the preoperative diagnosis of HCC, and LEL-ICC was confirmed by histopathologic and immunohistochemical examinations. EBV infection might play a pivotal role in the carcinogenesis of LEL-ICC, and surgical resection is still the most effective treatment at present. In addition, the favorable prognosis could be generally achieved after radical resection. Further research on the etiology and treatment strategies of LEL-ICC is required.The original contributions presented in the study are included in the article/Written informed consent was obtained from the individual (s) for the publication of any potentially identifiable images or data included in this article.FL and QX contributed to the data acquisition and drafted the manuscript. PR contributed to data acquisition. Y-XL and F-YL contributed to the study design and revised the manuscript. All authors contributed to the article and approved the submitted version.


Background: Bladder rupture is more frequently encountered in blunt pelvic trauma, but can also be spontaneous or iatrogenic. Laparoscopic repair has been widely used during the last few years as a treatment for intraperitoneal bladder perforation. The bladder is the genitourinary organ most often affected by iatrogenic injury. The purpose of this article is to report what is, to our knowledge, the first documented case of bladder rupture as a complication of laparoscopic cholecystectomy. Case description: A 51-year-old female presented to the emergency department complaining about generalized abdominal pain on the sixth postoperative day after laparoscopic cholecystectomy. Laboratory results highlighted a significant impact on renal function while the abdominal CT scan demonstrated free intraperitoneal fluid collection and surgical clips in the anatomic region of the liver and in an ectopic position near the ileocecal valve. An explorative laparoscopy revealed a 2 cm defect in the superior bladder wall, which was repaired in one layer in a continuous-locking fashion. The patient was discharged home on the fifth postoperative day having an uneventful recovery. Conclusion: Bladder rupture frequently presents with non-specific clinical signs; as a result, it is easily misdiagnosed, especially when it occurs with a non-typical mechanism of injury. Pseudorenal failure is a relatively obscure entity that may help the clinician suspect a bladder perforation. Laparoscopic repair with a single-layer continuous suture technique is a safe and feasible treatment in hemodynamically stable patients. Prospective research is required to specify the optimal timing of catheter removal after bladder repair. Bladder rupture, although a rare entity, is more frequently encountered in blunt pelvic trauma, but can also be spontaneous or iatrogenic . BladderThe typical clinical presentation of a patient with bladder injury is suprapubic tenderness, gross hematuria, and/or blood per urethra . MoreoveAlthough iatrogenic bladder rupture is less common than traumatic, the bladder is the genitourinary organ most often affected by iatrogenic injury . IatrogeThe most common complications following laparoscopic cholecystectomy are bile duct, bowel or vascular injuries, and postoperative bile leak or hemorrhage. Bile duct injury (excluding the cystic duct) has an average rate of occurrence of 0.6%. However, the most dangerous complications are bowel and vascular injuries, which occur in 0.14% and 0.25% of cases, respectively. Postoperative bile leakage is identified in 0.3% of patients, with the most frequent source being the cystic duct .A 51-year-old female presented to the emergency department complaining about generalized abdominal pain on the sixth postoperative day after laparoscopic cholecystectomy. The patient had a clear further medical history, was not taking any medication, and did not have any allergies. Moreover, she reported smoking 1 pack/per day and no alcohol consumption. Vital signs were 101 beats/min, blood pressure 110/60 mmHg, respiration 18 breaths/min, and body temperature 37 °C. Physical evaluation of the patient revealed severe diffuse abdominal pain, muscular defense, and rebound tenderness periumbilicus and in the hypogastrium area. Laboratory results at the time of admission did not reveal markers of inflammation, but highlighted a significant impact on renal function . An abdoParticularly, with the patient under general anesthesia, in a supine position, the pneumoperitoneum was obtained using Hasson’s technique via a sub-umbilical curvilinear incision and insertion of an 11 mm Hasson trocar. Another two 5 mm trocars were inserted under direct vision in the previous sites of the laparoscopic cholecystectomy (right midclavicular line and right anterior axillary line). Inspection of the peritoneal cavity revealed a collection of serous fluid in the lesser pelvis while the surgical clips of the laparoscopic cholecystectomy were on site with no sign of bile leak. A more detailed inspection of the lesser pelvis revealed a 2 cm defect in the superior bladder wall . The blaUrinary bladder perforations are more often encountered in polytrauma patients with blunt pelvic injury. However, they can also be iatrogenic or spontaneous . The occThe clinical signs that are often present in bladder rupture are, in the majority of cases, non-specific. Nonetheless, gross hematuria, suprapubic pain or tenderness, and difficulty or inability to void are a triad of symptoms that are frequently noticed . CT cystEven though EBR is typically managed conservatively with bladder catheterization for drainage and supportive care, IBR principally requires laparotomy and bladder perforation suturing . When heThe placement of a suprapubic catheter is not supported by evidence either alone or combined with a urethral catheter, but it may be helpful in selected cases when prolonged catheterization is anticipated or when there is tension in the bladder closure ,3. TransIn summary, although rare, bladder rupture is a complication that may be encountered after laparoscopic cholecystectomy. As the associated clinical signs are frequently non-specific, it is a condition easily that is misdiagnosed. Pseudorenal failure is a relatively obscure entity that may help the clinician suspect bladder perforation. Furthermore, laparoscopic repair of a bladder rupture in hemodynamically stable patients is a safe and sufficient procedure with additional benefits compared to laparotomy. As a final point, more prospective research is required to specify the optimal timing of catheter removal after bladder repair.


Intraoperative direct cortical stimulation (DCS) is the gold standard technique to maximize the extent of resection of tumors located in eloquent areas. To date, there are three cases reported of awake mapping for language centers in deaf patients who could communicate only with sign language. We present the case of DCS in a deaf patient who could communicate vocally, native to American Sign Language and English, that underwent intraoperative awake mapping. DCS showed similar disruption of expressive phonology to both pictorial and gestural stimuli, confirming that sign language follows the same pattern as oral language. In patients with intrinsic brain tumors involving eloquent areas, intraoperative direct cortical stimulation (DCS) is considered the gold standard technique to maximize the extent of resection while preserving function when feasible. One such application of intraoperative DCS is to maximize resection in lesions associated with critical language regions. However, few cases have been reported using awake mapping techniques for language centers in deaf patients . To our We present the case of awake DCS in a post-lingual deaf patient who was able to communicate vocally, native to American Sign Language and English, who underwent intraoperative awake mapping for both languages. This is the first case report where both modalities, sign language and spoken language, were tested in the same patient during awake mapping. Furthermore, we describe a pre-operative functional MRI (fMRI) and baseline naming approach for language mapping and discuss the neuroscientific implications of our awake mapping findings for hearing-impaired patients.We report the case of a 66-year-old right-handed gentleman with no hearing on the right side and profound sensorineural hearing loss on the left side since childhood; he had some serviceable hearing function on the left side using a hearing aid, was a fluent communicator with American Sign Language (ASL) and had functional comprehensible speech in English. He was also proficient in lip-reading. The patient provided informed consent to participate in this case report.Following initial presentation with a focal impaired awareness seizure, magnetic resonance imaging (MRI) revealed an intra-axial lesion involving the posterior aspect of the left temporal lobe extending to the angular gyrus with local gyrus expansion, blurring of the grey-white junction and mass effect causing effacement of the surrounding sulci. The lesion did not demonstrate gadolinium enhancement and was hypointense on T1-weighted sequences and hyperintense on T2-weighted imaging and FLAIR. The lesion had characteristics consistent with our working diagnosis of lower-grade glioma. However, given the patient’s age, there was a concern for an increased risk of this lesion being higher grade in nature.Preoperative studies were completed, including task-based functional MRI (fMRI) and diffusion tensor imaging (DTI) for tractography reconstruction, as well as a baseline assessment of naming for stimuli to be used during DCS. In addition, two visual tasks were used to assess language in fMRI, sentence completion and antonym generation; both required covert responses. As shown in Fig. The tractography reconstruction was performed with the Modus Plan™ software , using a fractional anisotropy set to an arbitrary threshold of 0.15 .The DTI revealed a white matter tract consistent with the inferior fronto-occipital fasciculus (IFOF) located inferior and medial to the lesion, as well as arcuate fasciculus (AF) fibers anterior and medial to the lesion Fig. . Given tUnder conscious sedation, the patient was positioned supine, his head fixed in a Sugita frame, and his head turned 30° to the right side. Local anesthetic was administered at the pin sites, as well as a local operative field block circumferentially around our planned operative site based on neuronavigation. Neuromonitoring to facilitate intraoperative cortical/subcortical language mapping was established. An awake anesthetic with conscious sedation was administered using dexmedetomidine. Sterile draping of the surgical field was performed using a mayo stand to lift the drapes away from the patient’s face. The right arm and hand were freely movable, and an iPad with visual stimuli was visible to the patient. Once the craniotomy had been performed, the patient was awakened for language mapping. We performed cortical mapping using an Ojemann bipolar handheld probe with 5-mm spacing between the probe tips, delivering biphasic current  to the brain.Language mapping was performed using a testing platform, Neuromapper, developed by Dr. Sabsevitz. Stimulation was followed 500 ms later by the presentation of a new visual stimulus with the prompt for the patient to name the presented object verbally. Naming with sign language, which would require bimanual dexterity, was not tested, given the constraints of the surgical procedure setting. Instead, vocal naming was tested for both languages, with both pictures and ASL gestures as stimuli, through collaboration intraoperatively with neuropsychology and an ASL translator.The result of the cortical mapping was negative across both modalities. Therefore, the resection was started, and subcortical mapping was serially performed at various stages of tumor removal. While advancing the resection, we noticed that the subcortical mapping at the medial margin of the tumor elicited phonemic paraphasias, including phonemic substitutions, neologisms, and perseverations of initial phonemes for both types of cues (pictures and ASL gestures). The gross anatomical location and neuro-navigation (which included pre-operative DTI maps) confirmed that these paraphasias occurred during stimulation at the level of the AF and IFOF Figs.  and 4. AThe postoperative MRI demonstrated gross total resection Fig. . The hisHearing loss can be either congenital or, more commonly, acquired after birth and is described in relation to spoken language acquisition as pre-lingual or post-lingual . Many inA recent study found that early language acquisition, whether oral or signed, enabled the normal development of the different white matter tracts involved in language . In contThe feasibility of performing awake DCS with sign language for glioma resection in deaf-mute signers has been previously reported , 11, 13 Our case is the first to report a deaf patient native to sign language and English, able to communicate vocally, where verbal responses to both pictures and sign gestures were tested using intraoperative DCS. Furthermore, our pre-operative structural and functional imaging correlated with intra-operative subcortical stimulation, emphasizing the importance of these pre-operative investigations. We elicited classic paraphasias, including neologisms, by stimulating the medial portion of the resection cavity. Furthermore, our intra-operative navigation corresponded anatomically to the AF and the IFOF. We corroborated that the sign language followed the same pattern as the spoken language, as we identified the same type of error in the same areas for both types of expression. This suggests a similar phonological organization in deaf patients fluent in sign language , 11, 13.This case shows the feasibility of awake surgery with cortical and subcortical mapping, testing receptive and expressive speech in hearing-impaired patients who communicate with sign and oral language. Furthermore, neuroimaging and DCS for language mapping in awake craniotomies of deaf signers with brain tumors suggest that sign language follows the same localization as spoken language. Our report contributes to this body of knowledge and, importantly, demonstrates the similar disruption of expressive phonology to both pictorial and gestural stimuli. Therefore, we recommend treating brain lesions in the dominant hemisphere language regions of deaf patients as if they were vocal language speakers and emphasizing the correlation between pre-operative language and imaging studies with operative strategies.


Hepatoblastoma is the most common malignant liver tumor in early childhood. The metastatic extension of hepatoblastoma into the left atrium via the pulmonary vein and left ventricle is rare. Reported cases almost always involve right-sided approaches and occur in pediatric patients. However, we are reporting a case of a 22-year-old female with recurrent hepatoblastoma at multiple sites, including the left atrium, left ventricle, brain, and lung. Hepatoblastoma is a rare tumor overall, but it is the most common primary malignant hepatic neoplasm in children less than five years of age, accounting for two-thirds of primary liver tumors in this population . Over thCardiac metastases of hepatoblastoma are extremely rare (0.67% to 3% of cases) . PreviouHowever, no case report has described the recurrence of childhood hepatoblastoma with left atrial and ventricular involvement in adulthood. This report presents a patient with diffuse metastatic foci and left atrial and ventricular metastases. We discuss the status of the cardiac mass in this patient, who was deemed surgically inoperable after ongoing chemotherapy and completed radiotherapy courses.2. The patient had bilateral peripheral edema (right leg ++/left leg ++) and a feeling of bloating in the abdomen. However, ascites was not detected in the abdomen on physical examination. Jugular venous distension was also not observed. There were no pathological features in other system examinations. The patient's vital signs were as follows: oxygen saturation (98%), temperature (37°C), blood pressure (110/60 mmHg), and pulse (75 beats per minute) measurements were within normal limits. Electrocardiography revealed no arrhythmia or ischemic changes  apicolateral, with trabeculations measuring 11 x 15 mm at the widest point. The LV mass did not negatively affect left ventricular hemodynamics or performance. We detected a mass image measuring 9 x 6 mm in the posterior of the left atrium (LA) and a 10 mm pericardial effusion that did not create compression at the broadest point adjacent to the LV lateral wall  obtained in January 2022, which was the time of initial diagnosis, we observed the progression of the LV and LA masses. In the cardiac CT of January 2022, we observed a metastatic mass with polypoid extensions into the cavity on the left ventricular apical lateral wall measuring 39 x 38 mm and a tumoral thrombus extending to the LA by filling the left inferior pulmonary vein. The contrast-enhanced cardiac CT planned during hospitalization showed that the mass had transmural spread in the apical region and exhibited an extension of up to 50 mm toward the LV cavity from the apex at its widest point , she attained remission. In 2016, left upper lobe posterior segment tumor resection was performed due to the observation of lung metastasis, followed by nine cycles of chemotherapy. In 2021, after skin and brain metastases were detected, doxorubicin-carboplatin chemotherapy was administered for three months. Despite receiving regular chemotherapy and radiotherapy, a metastatic lesion was observed in the interhemispheric fissure, measuring 40 x 71 mm with lobulated contours, intense contrast enhancement, and hemorrhagic component on cranial MRI in February 2023, which showed progression compared to the earlier MRI in July 2022  mass lesion measuring 24 x 28 mm was observed, obstructing the left lower lobar bronchus (Figure In January 2022, the heart team evaluated the patient due to cardiac metastases deemed inoperable due to the high mortality risk of possible cardiac tumor resection. The heart team suggested follow-up with chemotherapy and radiotherapy. Despite receiving 10 courses of radiotherapy and regular treatment with paclitaxel and carboplatin agents, the metastatic foci were widespread, and regression could not be achieved in these foci. We treated the patient with diuretics during hospitalization. The patient's peripheral edema and pulmonary congestion improved during the hospitalization, and the orthopnea and dyspnea subsided. Upon re-evaluation for cardiac mass surgery, the heart team recommended palliative care due to the widespread metastatic foci and spread of the cardiac mass. We decided on the follow-up of the cardiac mass with the continuation of chemotherapy and the patient was discharged. We planned diuretic treatment to prevent cardiac overload findings of our patient. We started low-dose anticoagulant therapy because cardiac masses may cause cardioembolic events. We could not plan aggressive medical treatments to prevent cardioembolic events because of the hemorrhagic component observed in the cranial mass in the control cranial MRI. Our patient, who was immobile due to cachexia and terminal malignancy, was followed up with low molecular weight heparin and was discharged.We present this case report to emphasize the widespread metastases and recurrence with cardiac involvement of an aggressive hepatoblastoma case. We aimed to draw attention to the rare occurrence of childhood hepatoblastoma with widespread metastases and cardiac involvement in adulthood.Reports on the metastatic spread of hepatoblastoma cases have been previously described in the literature. Especially in patients with cardiac involvement, metastases to the right atrium and right ventricle have frequently been localized and reported ,6. Even Previous reports on hepatoblastoma have described cases where cardiac mass regression and cure were achieved with chemotherapy . Some paChildhood hepatoblastoma may recur in adulthood with distant metastases. The brain, lung parenchyma, and cardiac cavities may be possible focal points for these metastases. Hepatoblastoma cases should not neglect the possibility of left ventricular involvement. Early, regular, and aggressive screenings are essential in hepatoblastoma cases. Despite regular chemotherapy and radiotherapy applications, regression may not be achieved in cardiac masses. Early detection of cardiac metastases can increase the success of treatment protocols.


Pheochromocytoma is a rare catecholamine-secreting tumor derived from chromaffin cells. The diagnosis is usually suggested by its classic history, presence of a strong family history, or discovery of an incidental mass on imaging in an asymptomatic patient. Hemorrhage into an occult pheochromocytoma is a rare complication with ∼1 to 2 per 100,000 individuals diagnosed annually. We report a case of a 29-year-old woman, who presented with abdominal pain (with no other significant history) due to a right hemorrhagic pheochromocytoma. Computed tomographic imaging and magnetic resonance imaging revealed the source of retroperitoneal hemorrhage as the right adrenal mass. They lacked the typical features of a pheochromocytoma which was eventually proven by the biochemical tests. The patient underwent preoperative stabilization with α and β adrenergic receptor blockers for 7 days following which laparoscopic adrenalectomy was performed successfully with an uneventful postoperative period. This is the eighth reported case in literature managed laparoscopically. Histopathology confirmed it as pheochromocytoma. The treacherous and deceptive nature of pheochromocytomas and its hemorrhage make it crucial to detect and treat it promptly; otherwise, it will almost certainly be fatal from cardiovascular complications or metastasis. Pheochromocytoma is a rare catecholamine-secreting tumor of the adrenal glands presenting with a myriad of clinical and imaging manifestations. It can lead to over whelming cardiovascular crises if left undiagnosed or if appropriate treatment is delayed.Video 1Hemorrhagic pheochromocytoma.A 29-year-old female kabaddi athlete visited the outpatient department due to progressive upper abdominal pain for the past 4 months with no other complaints, no history of steroid use in the past, and no comorbidities of note. Clinical examination was unremarkable except for tachycardia. She was further evaluated with an ultrasonography of the abdomen and pelvis which was suggestive of a large right adrenal mass composed of hypoechoic fat with cystic areas and mild surrounding fluid extending into the right perinephric space and paracolic gutter. These findings being suggestive of a ruptured adrenal myelolipoma with surrounding hemorrhage, a magnetic resonance imaging (MRI) of the abdomen was done for further evaluation and characterization. It showed a well-defined heterogenous lesion in the lateral limb of right adrenal gland ∼6.7 × 5.9 × 6.4 cm in size, hypointense on T1-weighted (T1W) and T2-weighted (T2W) sequences. No loss of signal is seen in the T1W out of phase images, representing no fat within the lesion with moderate free fluid in the right subhepatic, paracolic, and perinephric space suggestive of a hemorrhagic right adrenal adenoma . A coagAccordingly, blood and 24-hour collection of urine for metanephrine were ordered. It returned with a marked elevation of plasma-free metanephrine , 24 hours urine metanephrine  and metanephrine to creatinine ratio  confirming the diagnosis of pheochromocytoma.The patient was optimized with preoperative prazosin (α-1 receptor blocker) and metoprolol (selective β-1 receptor blocker) and fluid loading for 7 days. Thereafter, laparoscopic right adrenalectomy was performed successfully. Intraoperatively, the right adrenal gland was identified with dense adhesions around the hilum due to hemorrhage and subsequent fibrosis and with a contained retroperitoneal hemorrhage . As theThe patient's blood pressure was within normal range in the postoperative period eliminating the need for any antihypertensives. She was discharged on postoperative day 3.Histopathological evaluation confirmed it as a PASS score  of 2 and MIB1 proliferative index of 8% at the margin suggestive of a benign tumor . ImmunoGenetic testing ruled out suspected germline mutations. Patient has been advised about continued annual endocrinology follow-up with biochemical tests.Pheochromocytomas are rare, catecholamine secreting tumors with ∼1 to 2 per 100,000 individuals diagnosed annually.The key to diagnosing pheochromocytoma is to think of it first. Often referred to as “The Great Masquerader”Several biochemical assays are available to facilitate diagnosis; however, plasma-free metanephrines have had the highest sensitivity and specificity in a recent multicenter cohort trialOnce biochemical evidence of pheochromocytoma is obtained, imaging for localization should be undertaken to guide surgical resection .Spontaneous rupture of pheochromocytoma is a rare event, with only ∼83 cases reported in the literature. Its presentation being highly varied, ranging from nothing more than nausea and abdominal pain to hemodynamic shock and abdominal catastrophe, resulting in delayed diagnosis. The exact mechanism of pheochromocytoma rupture remains unknown. A massive release of catecholamines is probably associated with vasoconstriction in the tumor and subsequent necrosis and hemorrhage. Consequently, elevated intracapsular pressure may result in a tear in the capsule, causing further hemorrhage into the retroperitoneum.Clinical diagnosis of a ruptured pheochromocytoma is difficult and only 30.2% of patients have been diagnosed preoperativelyIn our experience with laparoscopic adrenalectomy, this is the first encounter with a ruptured hemorrhagic pheochromocytoma and the eighth documented case in literature managed laparoscopically.The treatment and outcome of the 83 cases are summarized inUrgent surgery is not indicated even in the presence of hemorrhagic pheochromocytoma because it has been shown that delayed surgery is associated with fewer intraoperative and postoperative complications and also lower mortality rate.An alternative that has been receiving attention is TAE of the adrenal vessels which provides good control of ongoing hemorrhage in a patient who is unstable and unprepared for an exploration.123I-MIBG scan to look for metastasis.A large pheochromocytoma (>5 cm) warrants anSpontaneous intraperitoneal hemorrhage remains a rare complication of pheochromocytoma, though the physiologic consequences present considerable medical and surgical challenges. Unfortunately, emerging clinical scenarios do not allow for optimal preoperative medical preparation with α-adrenergic blockade, and are associated with a high mortality rate. In the present case, as the patient was hemodynamically stable, she was optimized with antihypertensives preoperatively and managed through elective laparoscopic approach. Most importantly, a high index of suspicion must be maintained in similar cases so that the highly lethal hemodynamic sequelae may be anticipated and managed with the appropriate pharmacologic agents to ensure optimal outcomes. In unstable patients, a trial of TAE can be done.


Fibrosarcomas occur as a mesenchymal tumour of malignant fibroblasts in a collagen background and are usually found in the female genital organs and rarely involve the skin. A 5‐year‐old female Holstein cow with a raised mass at the base of right ear was referred. On gross examination, the mass was approximately 13.00 × 10.00 × 7.00 cm in size. Finally, complete surgical removal was selected. The mass was encapsulated and the dermis was expanded by spindle‐shaped to polygonal neoplastic cells. These cells were arranged in interwoven pattern. Mitotic figures were infrequent. Masson's trichrome demonstrated the positive and blue staining of collagen. Immunohistochemically, the sections were uniformly positive for Vimentin and negative for Desmin, SMA and GFAP. A well‐differentiated fibrosarcoma was diagnosed based on histopathological features. Surgical excision is the treatment of choice for this neoplasm. In the present case, surgery was also performed successfully and no new growth of the mass was observed 4 months following the surgical procedures. To our knowledge, this is the first report of well‐differentiated fibrosarcoma in a Holstein cow. This report describes the aural fibrosarcoma in a 5‐year‐old female Holstein cow. In the text of the study, macroscopical, microscopical and immunohistochemical findings are described. The sections were rinsed with PBS and reacted with streptavidin‐peroxidase conjugate for 30 min. After the slides were washed, treated with the substrate chromogen , counterstained with Mayer's haematoxylin, dehydrated, and mounted. Antibacterial therapy was applied with a pen & strep  and flunixin meglumine 5.00% . In histopathological investigations, haemorrhage, necrosis and peripheral aggregates of lymphocytes were observed  may be difficult. So, concurrent evaluation of both histopathological and IHC features is required for the definitive diagnosis of this tumour.Sara Shokrpoor: validation; writing‐original draft. Morteza Gorjidooz: investigation. Peyman Azizi: investigation.The authors declare that there is no conflict of interest regarding the publication of this article.This research received no specific grant from any funding agency.The authors confirm that the ethical policies of the journal, as noted on the journal's author guidelines page, have been adhered to and the appropriate ethical review committee approval has been received. This study was performed based on guideline for the care and use of laboratory animals in Iran. https://doi.org/10.1038/s41684‐021‐00871‐3https://publons.com/publon/10.1002/vms3.1126.The peer review history for this article is available at


Myxoid liposarcoma of the inguinal region is rare, can mimics an inguinal hernia. Any swelling in the inguinal region, that is partially reducible, fluctuant, soft, and without any signs of bowel obstruction should be evaluated further. Inguinal swellings in women are uncommon in comparison to men. These masses can arise from various locations in the inguinal region. Here, we present a rare case of myxoid liposarcoma of the inguinal region in a female presenting as a fluctuant, partially reducible swelling mimicking inguinal hernia. On examination, it was around 7 × 5 cm in size with a soft cystic component in the left groin extending to the labia majora and was partially reducible and fluctuant. Ultrasonography was done which was suspicious for a lipoma. Magnetic resonance imaging of the lesion diagnosed inguinal hernia. A fine‐needle aspiration cytology was performed that showed scanty cellularity composed of occasional spindle cells in greasy background, possibly a benign lipomatous growth. On exploration, a 12 × 5 cm cystic reddish brown pseudo‐encapsulated lobulated mass was found. Histopathology confirmed the diagnosis of well‐differentiated myxoid liposarcoma. Inguinal sarcomas are rare, hence when any fluctuant inguinal swelling without any history or signs suggestive of bowel obstruction presents for evaluation, sarcomas should be kept in differential with a high degree of suspicion and should be thoroughly worked up.The inguinal region is a common site for swellings in men, the most commonly caused by a hernia. However, in women, they are rare, but still, inguinal hernia is the leading cause.There are a few case reports of liposarcoma of the inguinal region, either arising from the spermatic cord or as a direct extension of intra‐abdominal sarcomas,2A 35‐year‐old Asian lady presented to the surgery department with complaints of swelling in her left groin noticed 2 years ago. On examination, vitals were stable. A 7 × 5 cm partially reducible, fluctuant swelling was present in the left inguinal region, lateral to the pubic tubercle, extending up to the labia majora. There was no cough impulse or signs of bowel obstruction. Given the above findings, the patient was evaluated with ultrasonography, which revealed an oval isoechoic to hypoechoic soft tissue mass of 11.6 × 2.6 cm in the left inguinal region in the superficial fat plane with a smooth regular margin, no vascularity, suggestive of a lipoma. Magnetic resonance imaging showed a well‐encapsulated cystic lesion in the left inguinal region extending towards the labia majora, hyperintense on T1, profoundly hyperintense on T2 images, thin septations within, likely a hernia (Figure Fine‐needle cytology was done showing scanty cellularity composed of occasional spindle cells in greasy background, possibly of benign lipomatous growth.The patient underwent excision through an inguinal incision, and a 12 × 5 cm cystic reddish brown pseudo‐encapsulated lobulated mass was found in the left inguinal region Figures .The final histopathology report showed soft tissue sarcoma with the presence of plump spindle cells in a myxoid matrix with the presence of numerous branching blood vessels and capillary channels. There are numerous lipoblast‐like cells with the presence of a scalloped nucleus and clear multiloculated cytoplasm. Numerous mature adipocytes were also identified. Mitosis was less than 10 per high power field. The tumor was positive for S100 and MDM2, and negative for MCV4, EMA, BCL2, CD99, and CD56, suggestive of well‐differentiated myxoid liposarcoma (Figure 3The inguinal region is a common site for swelling, the most common being an inguinal hernia. Lipoma of the spermatic cord forms up to 80% of inguinal masses, the rest by some form of sarcoma. A study of 179 patients with abdominal liposarcoma showed six patients have an inguinal extension of retroperitoneal liposarcoma, and 7 patients with liposarcoma isolated to the inguinal region.There are many case reports of spermatic cord liposarcoma presenting as an inguinal hernia, causing a diagnostic dilemma. There are very few case reports regarding liposarcoma arising in the inguinal region in a female. In a study consisting of 33 patients with inguinal sarcoma, 19 patients had an initial diagnosis of inguinal hernia, and only 3 were females. The rest of the females had some other diagnosis initially. The histology of 17 patients was one or the other form of liposarcoma, most commonly dedifferentiated liposarcoma.Myxoid/round cell liposarcoma constitutes 40% of all liposarcoma. These are most commonly seen in the deep soft tissue of extremities, most commonly the thigh.4Myxoid liposarcoma of the inguinal region is rare, can mimics an inguinal hernia. Since the diagnosis of inguinal hernia is clinical, any swelling in the inguinal region, that is partially reducible, fluctuant, soft, with solid‐cystic consistency, and without any signs of bowel obstruction, should be investigated further to rule out malignancy.Shantanu Agarwal: Data curation; resources; writing – original draft; writing – review and editing. Lovenish Bains: Conceptualization; data curation; resources; supervision; writing – original draft; writing – review and editing. Sripooja Makthala: Data curation; resources; writing – original draft; writing – review and editing. Pawan Lal: Supervision; writing – review and editing. Meeta Singh: Data curation; writing – review and editing.None.The authors declare that they have no conflicting interests.Written consent for the publication of this case report was obtained from the patient. Approval for case report by the institutional ethics committee is not required.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Myofibromas are rare benign tumors with myofibroblastic origin. They occur especially in cutis and subcutaneous tissue of the head and the neck, less frequently on the extremities. Myofibromas grow very slowly and are often painless, which is why patients often present relatively late. In the literature, there were many reports about intraosseous myofibromas of the craniofascial bones but reports of the trunk and extremities in adults are very rare. The authors present a very rare case of an intraosseous myofibroma of the ribs resulting in pathological fracture, including a research of literature from other cases of intraosseous myofibromas of the trunk or extremities. They occur especially in cutis and subcutaneous tissue of the head and the neck, they are observed less frequently on the extremities. and do not differ histopathologically from solitary lesions. Infantile myofibromatosis is caused by a PDGFRB (platelet-derived growth factor receptor beta) gene mutation with manifestations ranging from solitary or multiple tumors of the skin to generalized type with tumors of the skin and visceral involvement. A precise family history and physical examination often lead to the diagnosis, with histopathology being the gold standard to confirm the diagnosis. An autosomal dominant and recessive pattern of inheritance has been described, but most cases occur sporadically. Visceral lesions mean a very unfavorable course with high mortality. If the internal organs are not involved, the prognosis is very favorable, with often spontaneous regression.In children, multicentric myofibromas are often associated with infantile myofibromatosis Patients usually present with painless soft tissue swelling or, in the case of intraosseous lesions, occasionally with pain or pathological fractures.6 Imaging of intraosseous myofibromas often shows a sharply demarcated, nonspecific lytic mass with a sclerotic border, with or without pathologic fracture.2 Radiographically, myofibromas particularly resemble non-ossifying fibromas, fibrous dysplasia, and histiocytosis. But enchondromas, hemangiomas, aneurysmal bone cysts, and an infectious process can also look similar.2Since myofibromas typically grow very slowly and are often painless, patients often present relatively late.We report on a 65-year-old female patient who was referred to our center with an intraosseous mass of the eighth rib and history of a fall on the left chest wall 2 months ago. A diagnostic outpatient chest radiograph was performed for pain in the left hemithorax, revealing a pathological fracture with an expansive osteolysis and unclear intraosseous of the eighth rib on the left lateral side. Following additional computed tomography (CT) and magnetic resonance imaging (MRI) scans, the patient was referred to our sarcoma center.At the time of presentation, almost 3 months after the fall, the patient no longer reported any symptoms. Pain or shortness of breath was denied, as well as B symptoms or a known tumor disease. The clinical examination showed normal soft tissues on the left hemithorax without triggerable pressure or chest compression pain, a tumor in the area of the ribs/chest wall was not palpable.External X-ray diagnostics showed a pathological fracture of the eighth left lateral rib with an unclear osteolysis . The CT After presenting the findings to our interdisciplinary tumor board, a biopsy was indicated. For this purpose, a CT-guided biopsy of the mass was performed. Histologically, there was a spindle cell lesion with mild nuclear atypia and pleomorphism with included trabecular bone. The immunohistochemical examination revealed a strong diffuse-positive immune reaction for vimentin, a nuclear-positive immune reaction for special AT-rich sequence-binding protein 2 (SATB2)  and a positive immune reaction for smooth muscle actin (SMA) in the absence of the other markers . Due to the only sparse sample material that was submitted, a further molecular pathological examination was not possible, so that a further classification of the lesion was not possible and an open re-biopsy was recommended. The samples obtained again showed an indicated biphasic neoplasia consisting of fascicular or cell-rich areas next to cell-poor, increasingly hyalinized, or chondroid-appearing areas without higher-grade cellular atypia, which was differentiated myofibroma-like from the histological aspect . MitosesDue to the lack of symptoms, the patient decided against surgical treatment. The CT follow-up after 3 months showed an unchanged lesion of constant size, which is why the patient continues to favor conservative therapy. identified fewer than 40 cases described in the literature since 1966. Since then, there have been mainly single case reports on patients with myofibromas of the mandible, temporal bone, orbit, and a literature review of six patients with myofibroma of the maxilla.710 In the international literature intraosseous myofibromas on the trunk or the extremities are very rare; there are often only single case reports on lesions on the clavicle (2 patients),11 spine (2 patients), ilium (1 patient), femur/hip (3 patients),13 tibia (3 patients),14 humerus (1 patient), and ulna (2 patients).16 Oudijk et al. analyzed 114 patients with myofbromas in 2012, only 8 lesions were intraosseous, 3 were located in the cranio-fascial area , the others involving the clavicle, scapula, and tibia. All of these patients were younger than 25 years, 6 patients were even younger than 15 years. Yi et al. found 24 patients with myofibromas aged 8–64 years. The lesions were most frequently localized in the extremities (15 patients), followed by the head and trunk (6 patients each) and neck (2 patients), without further differentiation between intraosseous and subcutaneous locations. There is one previous report of a solitary myofibroma of the chest wall involving the rib.The authors present a case of an intraosseous myofibroma of the ribs resulting in pathological fracture. Intraosseous myofibromas are very rare, there is only a small number of case reports, which mostly describe a craniofascial localization. In 2009, Nirvikalpa and Narayanan2The list of possible differential diagnoses is long and mainly relates to benign and malignant spindle cell tumors. Histologically, myofibromas can easily be confused with neurofibromas, desmoid tumors, fibromatoses such as fibrous histiocytoma, solitary fibrous tumors/hemangiopericytomas, and various types of sarcomas.3 The Ki67-index is often low, reflecting the low proliferation and slow growth progression of the lesions, which is consistent with our immunohistochemical results. With regard to the activating PDGFRB mutation, as it was found in our patient, the literature primarily contains associations with infantile myofibromatosis in children. Dachy et al. found gain-of-function mutations in samples from 25 children in their study with 69 patients, these were associated with severe multicentric diseases in 68%. Hung and Fletcher were able to demonstrate PDGFRB mutations not only in the context of myofibromatosis but also in conventional myofibromas in adult patients. Koo et al. found mutations in 8 out of 10 cases. Even though PDGFRB mutations may be detected more frequently in cases of multicentric lesions, the detection of this mutation may facilitate a difficult pathologic diagnosis and enable targeted therapies.20The literature research shows that intraosseous myofibromas are histologically often positive for SMA and muscle-specific actin (MSA) and other markers  are often negative or only positive in a few cases (Desmin).3 Spontaneous remissions can occur in soft tissue and bone. The most common surgical procedure was complete excision, in two cases with replacement by an iliac bone grafting. In some cases, an intralesional curettage was performed without further defect reconstruction, occasionally a bone graft is described.10 A defect filling with cement or other adjuvants has not been reported. There were also a few patients without further therapy after biopsy.With a benign lesion like this, there is basically no need for surgical/interventional therapy. However, it may be indicated if symptomatic complaints develop. In the literature, local surgical resection is usually described as the treatment of choice for complaints, which usually results in cure. observed a recurrence of an intraosseous myofibroma on the finger after one year, Yi et al. reported 7 patients with recurrence in their case series of 24 patients and identified the absence of a pseudocapsule and younger age as possible risk factors for recurrence. Hung and Fletcher reported a patient with soft tissue myofibroma which recurred twice. Fu et al. described two recurrences of subcutaneous myofibromas. The surgical procedure has not been identified as a risk factor for recurrence in the current literature, possibly due to the small number of cases and the even lower number of reported recurrences. In our case, the patient refused therapy because her symptoms, which occurred temporarily due to the fall, had completely resolved. The CT follow-up after 1 year showed no size progression of the lesion, so that an operative therapy was not indicated until now.Recurrences have only been described in a few cases. In their case report, Ma et al.Intraosseous myofibromas are rare, benign neoplasms that can be easily confused with other benign and malignant spindle cell tumors clinically, radiologically, and histopathologically. Additional immunohistochemical and molecular pathological examinations such as SMA and a gain-of-function mutation in PDGFRB are reliable methods that enable a reliable diagnosis, so that unnecessarily aggressive therapy in benign tumors can be avoided. The prognosis for solitary lesions is excellent; for multicentric lesions, particularly in children with infantile myofibromatosis, the prognosis depends on the extent of the disease and the organs affected. Surgical therapy is indicated if patients complain of painful symptoms or pathological fracture. A complete excision of the lesion should then be performed, which usually results in lasting cure.


Paraduodenal hernia (PDH) is the most frequent type of congenital hernia, especially on the left side. Although acute intestinal obstruction due to internal hernias is rare, it can be life-threatening if not detected and treated promptly. Here, we present the case of a 36-year-old man who presented to the emergency department with an acute intestinal obstruction that had been developing for three days. The patient had a history of recurrent abdominal pain but had never undergone any abdominal surgery. Surgical exploration revealed a left internal PDH with a collection of incarcerated jejunal loops retrocolically encased within the hernial sac. Resection of the gangrenous jejunal loops was performed, followed by hernia reduction and neck closure. This case highlights the importance of timely and accurate diagnosis of left PDHs, particularly in patients with a history of recurrent abdominal pain and no prior abdominal surgeries. Early detection and treatment can prevent life-threatening complications such as intestinal perforation and peritonitis. Paraduodenal hernias (PDHs) are rare but the most common type of congenital internal peritoneal recess hernia . Its incRadiologists and clinicians are challenged by internal hernias due to their rarity and the complexity of their diagnosis and treatment . The cliPreoperatively, timely diagnosis is often difficult, leading to grave consequences and a reported mortality rate of more than 20% . The ultA 36-year-old married man presented to the emergency room with a three-day history of colicky abdominal discomfort, vomiting, and constipation. The pain had started three days prior and gradually worsened over the past week, accompanied by non-projectile bilious vomiting and nausea. He had also experienced a decrease in urine output and was unable to pass stool or flatus. He had no comorbidities, and his drug history consisted of painkillers and laxatives taken for constipation. Moreover, he had no significant family history.9/L. Abdominal ultrasound showed gaseous distention of his abdomen and gallbladder sludge. An erect X-ray of the abdomen showed multiple air-fluid levels , paranormal , and abnormal . These defects may be congenital due to intestinal malrotation or the absence of retroperitoneal attachments or acquired due to past abdominal surgery, trauma, or any ischemic process .Schizas et al. stated that PDH typically develops between the ages of 40 and 60, while Muneer et al. reported that PDH typically develops at a mean age of 44.1 years .The diagnosis of PDHs is the most challenging aspect of management. This is because of the non-specific presentation of PDH. Presenting symptoms can range from diffuse abdominal pain that is frequently eased by positional modifications to acute intestinal obstruction, which is the most common presentation . A contrThe importance of CT in the early preoperative identification of PDH is justified by the significant death rate associated with these complications . The typThe ultimate modality of treatment is operative repair, either laparoscopic or open repair, depending on the operative facilities and level of competency available ,3,6. TheThis case report highlights the importance of early and accurate diagnosis of left PDHs, which can be life-threatening if not detected and treated promptly. The findings of this study demonstrate the necessity of a thorough physical examination and prompt imaging in patients with a history of recurrent abdominal pain and no prior abdominal surgeries. The successful treatment of this patient through laparotomy and resection of the gangrenous portion of the gut, followed by hernia reduction and neck closure, emphasizes the critical role of surgical intervention in such cases. This case report contributes to the existing literature on PDHs and provides valuable insights into the diagnosis and management of this rare condition.


A 55-year-old man with a history of Crohn’s disease with multiple abdominal resections, poor wound healing, and rectal cancer with pulmonary metastasis presented with abdominal distension, pain, and absence of bowel movement. A computed tomography scan showed a distended colon, with the known obstructing malignant lesion in the rectum. A surgical colostomy was judged not to be possible owing to his history of multiple and extensive abdominal surgical procedures. A colonic stent was not possible owing to the distal position and length of the lesion.After discussion with the patient and inspired by the case report of Canakis and BaronVideo 1 A colostomy is created using a percutaneous lumen-apposing metal stent.Over the following days, the stent did not deploy sufficiently and attempted CRE balloon dilation up to 15 mm was unsuccessful. A decision was made to incise the abdominal fascia around the stent, after which it deployed fully and functioned as a colostomy for at least the next 3 weeks, at which time the patient was discharged home with palliative care.Endoscopy_UCTN_Code_TTT_1AQ_2AF


Hepatoblastoma is the most common primary malignancy of hepatic origin in children, with an estimated incidence of 0.5–1.5 per million children. Hepatoblastoma classically has an intraparenchymal location, and pedunculated hepatoblastoma is a relatively rare entity. Accurate diagnosis can be challenging due to its extrahepatic location and possibly its thin peduncle, which is not easily identified in imaging.Here, we report a case of asymptomatic giant palpable hepatoblastoma in the LUQ of a four-month-old male infant, initially suspected of neuroblastoma based on abdominal ultrasound findings. The final diagnosis of giant pedunculated hepatoblastoma was made based on the abdominal CT scan and the diagnosis was confirmed by percutaneous biopsy. Due to the size of the tumor, complete removal of the tumor was not initially possible. Therefore, the patient was treated with several courses of chemotherapy. The tumor was shrunk and then completely removed. The patient was treated, and no complications were found in the 6-month follow-up.Pedunculated hepatoblastoma is rare but should be considered as a possibility in the case of a perihepatic mass in a pediatric patient that can be confused with other upper abdominal masses such as an adrenal mass. Therefore, in such cases, we must look for the vascular pedicle in the imaging and keep the AFP check in mind. Hepatoblastoma is the most common primary malignancy of liver origin in children, with an estimated incidence of 0.5–1.5 per million children from birth to 14 years of age . The conPediatric hepatoblastoma is diagnosed using laboratory markers and imaging studies, but a definitive diagnosis is based on pathological examination . A commoPedunculated hepatoblastoma is a relatively rare subtype of hepatoblastoma that is characterized by a tumor mass attached to the liver by a pedicle or stalk and to the best of our knowledge, few cases have been reported in the literature , 8.The diagnosis of pedunculated hepatoblastoma can be challenging as there is difficulty in determining the origin of the mass as the thin peduncle/stalk may be unrecognizable on imaging.Here, we report a four-month-old male infant with a large, asymptomatic, palpable abdominal mass, initially thought to be neuroblastoma, but later diagnosed as a pedunculated hepatoblastoma.A four-month-old male infant was admitted for a palpable mass in the left upper quadrant (LUQ). The mass was first detected by his mother a week ago. The patient did not have any specific signs and symptoms and did not have failure to thrive or developmental delay. The mother’s pregnancy and delivery were uneventful. His birth weight was 2800 g. The CBC, and liver functions test, including liver enzymes, prothrombin time and partial thromboplastin time, bilirubin, blood urea, and creatinine were normal.Abdominal ultrasound showed a large, slightly heterogeneous solid mass with clear borders containing scattered calcifications of approximate dimensions 70 × 55 × 40 mm in the LUQ, which was in front of the left kidney and inferior to the spleen and pancreas without a definite claw sign with any adjacent solid organs. Considering the infant’s age, the above findings led to the clinical suspicion of neuroblastoma. To rule in or rule out the diagnosis of neuroblastoma, bone marrow aspiration and 24-hour urine collection (VMA and HVA) were performed, which were negative.In the next step, a CT scan of the abdomen and pelvis with intravenous contrast was performed which confirmed the ultrasound findings. Fig. On CT scan, a few vascular channels were identified from segment III of the left liver lobe passing into the upper part of the above mass without peripheral tissue.According to the negative results for neuroblastoma and considering the entry of nutrient vessels into the mass from the left hepatic vein and the coarse calcification areas and the pattern of enhancement, pedunculated hepatoblastoma was suggested and AFP was measured which was more than 100,000 ng/ml . β-HCG was within the normal range. the patient subsequently underwent a core needle biopsy. The pathology report revealed fetal hepatocytes with enlarged nuclei, and abundant cytoplasm arranged in a light-dark pattern due to admixture of glycogenous and eosinophilic hepatocytes with rare mitosis, consistent with the diagnosis of epithelioid hepatoblastoma Fig. . AccordiDue to the size of the tumor, complete removal of the tumor was not feasible at first. Therefore, the patient was treated with several courses of chemotherapy Fig. . The prePrimary liver tumors account for 1–2% of all childhood tumors , 10. HepThis report presents a case of a large pedunculated hepatoblastoma in an asymptomatic four-month-old infant who was initially treated with chemotherapy to make the tumor resectable. We found only two reports of pedunculated hepatoblastoma in the literature, one from the right lobe and the other from the hepatogastric ligament , 8.These cases were 3 and 7-year-old children who presented with peduncle hepatoblastoma measuring 3 × 4 cm and 8.1 × 7.4 × 4.4 cm respectively [The case presented in this article is a four-month-old baby with a 70 × 55 × 40 mm mass, which is extremely large for an infant of this age.Unlike pedunculated hepatoblastoma, several case reports of pedunculated hepatocellular carcinoma have been published, which may help us better understand pedunculated hepatoblastoma. Well-developed hepatoblastoma may mimic hepatocellular carcinoma.The stalk transports nutrients from the liver and connects a tumor to the liver.Without the protection of the liver parenchyma, a pedunculated liver tumor is vulnerable to bleeding, which rapidly leads to tumor spread , 14.No bleeding or spread or relapse was found in our case.Pedunculated hepatoblastoma is rare but should be considered as a possibility in the case of a perihepatic mass in a pediatric patient that can be confused with other upper abdominal masses such as an adrenal mass. Therefore, in such cases, we must look for the vascular pedicle in the imaging and keep the AFP check in mind.


Pituitary macroadenoma and angiofibroma are two distinct and diverse types of tumors that can develop in different anatomical locations and clinical characteristics and are not typically related to each other in terms of their hormonal or developmental aspects. This case describes an adult male with pituitary macroadenoma with nasal angiofibroma. A 35-year-old male was diagnosed with pituitary macroadenoma and incidentally found to have juvenile nasopharyngeal angiofibroma (NPA). The patient underwent a diagnostic workup, including imaging studies and hormonal assays, which confirmed the concomitant presence of both tumors. The patient underwent successful endoscopic surgical excision of the NPA and transnasal transsphenoidal endoscopic pituitary macroadenoma excision as a two-stage operation. The patient was followed up postoperatively and had no evidence of tumor recurrence or hormonal imbalances. The importance of complete and comprehensive diagnostic workup and multidisciplinary management in achieving successful and optimum treatment outcomes for coexisting NPA and pituitary macroadenoma in an adult patient is highlighted in the present report. Nonfunctioning type pituitary adenomas are the second most frequent form of tumor of pituitary origin . These nNasopharyngeal angiofibroma is an extremely vascular lesion and may have one or more than that arterial vascular pedicles . It is uA 35-year-old male presented to the ENT OPD with a complaint of recurrent right nasal bleeding for the past two years. The patient reported experiencing painless episodes of epistaxis lasting for 15 to 20 minutes each time, resulting in blood loss of 30 to 40 ml. He also complains of mucopurulent nasal discharge for three months occasionally. Epistaxis was profuse and was managed by anterior nasal packing and blood transfusion. The patient had been experiencing headaches, vision problems, and reduced visual field with diminution of vision at the periphery for several months. The physical examination of the patient revealed prominent supraorbital ridges, a broad nose, mandibular prognathism, thickened lips and thickened skin folds on the scalp, which are consistent with acromegalic facial features. In the backdrop of these older photographs, facial features were conspicuously different, when compared with recent photographs. Figure Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans Figure  of the bFunctional endoscopic sinus surgery (FESS) was planned for the removal of nasal mass as first-stage surgery. On nasal endoscopy, mucopurulent nasal discharge was visualized arising from the middle meatus. Intraoperatively, a fleshy, vascular mass measuring approximately 2.5x 2 cm seen arising from the right middle turbinate was excised, and a diagnosis of angiofibroma was made. Histopathological examination of the mass revealed angiofibroma. Gross examination revealed a mass of approximately 3.5x4 cm , prolactin (15ng/ml), cortisol (2 microgram/dl); thyroid profile was found to be normal. The second stage of the operation involved transnasal transsphenoidal endoscopic excision of pituitary macroadenoma surgery. This surgery was performed six weeks after the first surgery to allow for proper healing. The tumor size along with its proximity to vital structures made it critical and challenging, nonetheless, the team was able to remove the tumor completely with no major complications. Patient was rigorously monitored in the immediate post-operative phase to assess vital signs, neurological condition, and the presence of any potential complications, including cerebrospinal fluid (CSF) leakage or bleeding. Hormonal analysis yielded normal results, and an MRI was conducted to confirm the absence of any residual mass.Postoperatively, the patient had a gradual recovery. He was followed up regularly to monitor the postoperative course, and MRI scans showed no recurrence of the tumors.The successful two-staged operation for the incidental angiofibroma with pituitary macro adenoma highlights the complex situation of twin lesions existing together. The use of advanced imaging techniques, meticulous surgical planning, and careful execution by the surgical team was crucial in achieving a positive outcome for this patientThis case describes an adult male with pituitary macroadenoma and nasal angiofibroma. Pituitary adenomas, with most of them being benign and slow-growing, are the tumors of anterior pituitary. These are categorized based on size and on the basis of size or cell of origin and can be designated as microadenoma, macroadenoma and giant tumors. Microadenoma describes a tumor less than 10 mm, whereas macroadenomas are considered as tumors greater than 10mm . In the These pituitary adenomas are of two types, functioning and non-functioning. The functioning one causes enlarged secretions of single or multiple hormones of the anterior pituitary depending on their cell type are functional whereas alternative to this, the non-functioning type does not secrete hormones, however there is the possibility that these can compress the adjacent areas of the anterior pituitary leading to hormonal deficiencies. Hence, patients with pituitary adenoma are required to be analysed by a multidisciplinary team that must comprise specialists in endocrinology, ophthalmology and neurosurgery .Adenomas of pituitary are the most frequent source of a mass in the sella with approximately 9% of cases reported with other etiologies for mass lesions in the sellar region. The differential diagnosis for lesions in the sellar and suprasellar area is diverse and consists of neoplastic pathologies such pituitary adenoma, Rathke's cleft cyst, metastases or craniopharyngioma. The other manifestations of these are meningioma, vascular lesions, and inflammatory illnesses like sarcoidosis and autoimmune hypophysitis. Therefore, it is essential to consider this wide range of possibilities while evaluating such cases. Precise preoperative diagnosis is crucial clinically as the preferred treatment for various nonpituitary sellar masses is different from that of a pituitary tumor .Patients with pituitary macroadenomas should undergo a thorough evaluation for hypopituitarism, which involves measuring serum levels of pituitary hormones. The diagnosis of acromegaly is inveterated on a biochemical basis, which includes increased serum insulin-like growth factor 1 as well as lack of suppression of growth hormone after administration of glucose. Pituitary magnetic resonance imaging is recommended in cases with acromegaly to recognise an underlying pituitary adenoma . Facial The first-line therapy for patients with acromegaly is generally transsphenoidal pituitary surgery. However, patients with macroadenomas are frequently not remitted postoperatively. Medical therapies that comprise somatostatin receptor ligands, pegvisomant and cabergoline can be recommended to patients with relentless disease after surgery. Radiation therapy is mainly a third-line choice and is progressively administered by various stereotactic techniques.Assessing the para-sellar extension of a macroadenoma is essential in preoperative MRI studies. Para-sellar involvement in pituitary macroadenomas can be difficult to diagnose due to limited clinical features. Direct visualization of dural penetration on MRI can also be challenging. To determine whether an adenoma has infiltrated the cavernous sinus rather than causing lateral bulging, the Knosp criterion and other MRI evaluation standards have been created Figure . These cAccording to Mario-Enrquez and Fletcher's 2012 analysis of 37 instances, juvenile angiofibroma (JA) is a benign fibrovascular neoplasm that makes up 0.5% of all head and neck tumors. The clinical presentation typically involves unilateral epistaxis, nasal obstruction, and an intranasal mass. However, it can also present as a mass in the back, chest wall, abdominal cavity, or pelvic cavity. Epistaxis, or nosebleed, can range from mild to severe and may require various treatments such as nasal packing, vasopressors, antifibrinolytics, and even transfusions . JuvenilPituitary macroadenomas and angiofibroma are two distinct types of tumors that can occur in the head and neck region, and their co-occurrence is not well established. But, a genetic condition i.e., multiple endocrine neoplasia type 1 (MEN1) is associated with an increased risk of angiofibroma and pituitary tumors . Tumors We presented a case report on co-existence of angiofibroma and pituitary macroadenoma. While both are rare, their occurrence together is the rarest of rare situations. This can prod researchers and healthcare professionals to gain insight into the potential relationship between these tumors and better understanding of the underlying mechanisms that may contribute to their development. It highlights the importance of early diagnosis and appropriate management, along with long-term follow-up among patients with these conditions. It also contributes to comprehending the pathogenesis of tumors of this type of origin which serves as a basis for further research into the relationship between these two conditions, potentially leading to the development of new diagnostic or treatment approaches.


Enterobius vermicularis is a threadlike parasite also known as “pinworms”. It is the most common helminth infection, affecting the gastrointestinal tracts of children worldwide, although it seldom causes any fatalities. Enterobius vermicularis infections are usually asymptomatic and may only cause anal pruritis, with occasional reported cases of ectopic migration into the appendix or the female genital tract by adult pinworms. Here, we report a case of a 15-year-old girl who presented to the emergency department with high-grade fever, vomiting, and vague abdominal pain for three days. She was diagnosed with acute abdominal pain and underwent emergency ileocecectomy, but died the following day. Pathological examination of ileocecal junction showed intraluminal and intramural Enterobius vermicularis, which were attributed as the cause of her death in the absence of any other pathologies. Death due to Enterobius vermicularis is rare; this case calls for clinicians to be vigilant in exploring Enterobius vermicularis infections in patients with undiagnosed acute abdominal pain, since it could be a potential cause of death. Enterobius vermicularis, also known as “pinworms” is considered the most common helminth infection , affecting the gastrointestinal tract of children worldwide [Enterobius vermicularis in the urinary tract, kidney, biliary tree, fallopian tube, and eye  reported a case of ectopic intramural Enterobius vermicularis eggs [Enterobius vermicularis infestation, which calls for the need for continues vigilance and surveillance. A cross sectional study conducted on 200 patients who had an appendectomy showed that 30 of 200 appendices (15.0 %) had Enterobius vermicularis in histopathological examination; this was found to be more common in females compared to males [The severe and worsening abdominal pain experienced by the patient, in the absence of any other relevant medical history, is consistent with other patients in which eosinophilic colitis has been associated with larvae of icularis . Althougicularis . Pinwormicularis . While iammation . Howevers et al. 22 reportto males .Enterobius vermicularis infection.Our hypotheses for the probable cause of death is that peripheral blood eosinophilia caused by the parasitic infection promoted a hypercoagulable state that may have resulted in thrombosis leading to ischemia, bowel infarction, and death, which is in line with previous reports with other parasitic infestations . AlthougOur case provided limited information since the patient died within hours of admission, which impeded the ability to investigate and check the presence of eosinophilia. In addition, no differential diagnosis was provided, since the patient was rushed to the operating room for an emergency exploratory laparotomy. Moreover, post-mortem examination was not performed because there is no clinical post-mortem examination service available at the hospital, and the hospital does not have a policy on referring autopsies to any other hospital that provides them.Enterobius vermicularis in the absence of classical symptoms, intestinal parasitosis should be considered in the differential diagnosis of clinical syndromes like gastrointestinal hemorrhages [Enterobius vermicularis in the absence of clinical symptoms? A type-2 oriented immune response has been suggested to be elicited against Enterobius vermicularis with potential for balancing activation of the immune system [It has been suggested that, owing to the difficulty of diagnosing orrhages . Could se system . In spite system . Moreovee system . MoreoveEnterobius vermicularis can be primarily controlled by taking daily showers, continued handwashing, and fingernails cutting. A single dose of Mebendazole or Albendazole is effective in reducing the risk of reinfection and complications [ications .Enterobius vermicularis eggs in a 15-year-old female patient, who unfortunately died. All lifesaving measures to try to save her life were performed; however, it was too late due to gangrene of the bowel. Since the bowel was already gangrenous, all the air in the bowel distended the abdomen. Gangrene from vascular thrombosis and vasculitis led to shock and death. Cases have been reported showing the diversity of the pathophysiology and mode of infestation of Enterobius vermicularis, which may cause serious complications, with significant morbidity and mortality, and patients may present with atypical signs and symptoms. Therefore, clinicians should not rule out pinworm infestation in patients with abdominal pain or suspected appendicitis. Our ability to differentially diagnose Enterobius vermicularis in the absence of its typical clinical presentations will prevent such fatalities. Unusual parasitic causes such as Enterobius vermicularis should be considered in patients with undiagnosed acute abdominal pain by clinicians and pathologists. It is also worth looking into implementing the use of molecular diagnostic tools, such as RNA detection, in these cases.Here we report intraluminal and intramural