We present the case of a 64-year-old woman with type 2 diabetes who was diagnosed with early-stage intrahepatic cholangiocarcinoma and underwent partial hepatectomy followed by adjuvant chemotherapy. The patient simultaneously developed skin lesions compatible with acanthosis nigricans (AN). Thirty-seven months after completing chemotherapy, the patient had a recurrence of extensive skin and mucosal lesions compatible with AN. A thoracic-abdominal-pelvic (TAP) CT showed a relapse with hepatic hilar adenopathy. Currently, she is under evaluation to undergo radical treatment. Malignancy is a rare cause of AN and skin lesions can arise before, during, or after the diagnosis. As a paraneoplastic syndrome, it is usually related to gastric adenocarcinoma, with cholangiocarcinoma being a rare entity in this setting. Although an uncommon manifestation, the malignant etiology should be considered among other prevalent causes, such as metabolic disorders, and establishing an association can lead to an early diagnosis and initiation of curative treatment. Acanthosis nigricans (AN) is a common skin condition characterized by velvety and hyperpigmented plaques on the skin at intertriginous sites such as the neck and axillae. Other cutaneous or mucosal sites are less frequent . This diGastric adenocarcinoma is the most frequent tumor associated with ANM, but other tumor sites have also been described -3. HowevCholangiocarcinoma is a rare tumor with a particularly poor prognosis. Only 30% of the patients are diagnosed with resectable disease and, of these, 60% experience a post-surgical relapse. Thus, an early diagnosis is pivotal for initiating treatment with curative intent [A 64-year-old-woman, a factory worker with a past medical history of metabolic syndrome  and cerebrovascular disease, presented with complaints of darkened skin in the armpits and neck along with considerable weight loss (12% of body weight) in the past year. The laboratory workup revealed cholestasis. A thoracic-abdominal-pelvic (TAP) CT scan was performed, which revealed a large hepatic nodule (88 x 74 x 65 mm), without any further suspicious lesions. The abdominal and pelvic MRI confirmed the presence of this lesion , with a normal carcinoembryonic antigen (CEA). The case was discussed at the Multidisciplinary Hepatobiliary and Pancreatic Tumours Board and surgery with curative intent was proposed. A partial hepatectomy was performed (December 2017) and the pathological report showed poorly differentiated intrahepatic cholangiocarcinoma (pT2aN0R0). After surgery, the serum CA 19.9 normalized and the skin lesions improved. During the cholangiocarcinoma diagnosis and treatment, the dark skin lesions were assumed to be AN secondary to poorly controlled type 2 diabetes.de novo bilateral pulmonary nodules (the largest one measuring 13 x 8 mm) and a large hepatic hilar adenopathy.After adjuvant chemotherapy, the patient underwent regular follow-ups. In December 2022, she reported a rapid onset of dark skin lesions  in the past month. Physical examination revealed extensive lesions of AN scattered throughout the body  was also done and showed a mild uptake on pulmonary nodules, in line with their inflammatory etiology Figure . On the Currently, the patient is under evaluation by the Multidisciplinary Hepatobiliary and Pancreatic Tumours Board regarding a loco-regional curative treatment for the recurrence.Cholangiocarcinoma is an aggressive tumor and its incidence is on the rise in Western countries. Typically, it is asymptomatic in the early stages and is often diagnosed as an advanced disease, thereby compromising the opportunity for a curative treatment . AN is aIn our clinical case, the patient had AN manifestation as a paraneoplastic syndrome secondary to cholangiocarcinoma. AN's association with this tumor has been very rarely described and the patient's type 2 diabetes diagnosis was a confounding factor to the etiology of AN. However, an unexplained weight loss and the appearance of extensive lesions in our patient make the paraneoplastic syndrome hypothesis more likely . The recOur case sheds light on the role of AN as a paraneoplastic syndrome. It also underlines the impact that an expeditious acknowledgment of a paraneoplastic syndrome could have on the prognosis of a dismal tumor. Despite being rare, a malignant etiology should always be on the list of potential causes of AN. A diagnosis of ANM can lead to an early diagnosis of cancer, enabling the initiation of immediate treatment of an aggressive disease. Fortunately, in our patient's case, her cholangiocarcinoma recurrence will probably benefit from a curative approach.AN is a rare paraneoplastic syndrome and its association with cholangiocarcinoma has been scarcely reported. In our case, the patient's type 2 diabetes medical history was an initial confounding factor with regard to the etiology of AN. Despite being rare, a malignant etiology should always be part of the list of potential causes of AN. Prompt recognition of AN can lead to an earlier diagnosis and a higher chance of providing a curative treatment. This is crucial in the setting of cancers with a dismal prognosis such as cholangiocarcinoma.


A 13-year-old neutered male Korean short-hair cat presented with anorexia, lethargy, and a severely distended abdomen, suggestive of ascites. Abdominocentesis yielded serosanguineous fluid. A subsequent diagnostic workup, including blood tests, ascitic fluid analysis, imaging studies , and histopathological examination, was performed to identify the underlying cause. Imaging studies revealed characteristics of encapsulating peritoneal sclerosis (EPS) such as peritoneal thickening, fat stranding, and calcification. During laparotomy, fibrous membranes encapsulating the abdominal organs and ascites were observed, and multiple calcified regions were detected on the abdominal wall. Histopathological analysis confirmed the diagnosis of poorly differentiated invasive malignant neoplasms, which were further classified as carcinomatosis based on positive cytokeratin and negative vimentin immunohistochemistry results. To our knowledge, this is the first report of sclerosing peritoneal carcinomatosis with osseous metaplasia in a cat. Given the challenging nature of EPS diagnosis and its potential therapeutic implications, we aimed to explore the diagnostic process and highlight the importance of considering SPC with osseous metaplasia as a differential diagnosis, when EPS is suspected based on imaging findings. This case report provides valuable clinical insights, underscoring the significance of histopathological analysis for establishing an accurate diagnosis and guiding the appropriate management of complicated cases.2Chlamydia psittaci) were administered during its young-adult stage. While deworming was performed annually, preventive measures against ectoparasites were not applied. There was no history of trauma. The cat had only undergone surgery twice, including neutering when it was under 1 year old and wart removal 2 years ago. The wart, located on the instep of the right forelimb, measured less than 0.5 cm in diameter, was encapsulated, and not grown for several years, suggesting it was considered a benign tumor.A 13-year-old neutered male Korean short-hair cat presented with anorexia and lethargy that had been ongoing for at least 2 weeks. The cat was kept indoors and had never been taken for walks outdoors. Basic feline vaccinations . The NT-proBNP level was below 50 pmol/L (reference range: 0–270 pmol/L), ruling out a cardiogenic etiology for the ascites. Additionally, the feline leukemia virus (FeLV) antigen, feline immunodeficiency virus (FIV) antibody, and heartworm antigen test results using SNAP Feline Triple Test kit  were negative. In the ascitic fluid analysis, the total nucleated cell count (TNCC) was 2040 cells/μL, and the total protein (TP) was 3.4 g/dL, confirming the ascites as a modified transudate. In cytology, the ascitic fluid contained small numbers of macrophages, neutrophils, lymphocytes, and red blood cells (RBCs), without any evidence of neoplastic cells. Furthermore, PCR testing for feline coronavirus and microbial culture of the ascitic fluid yielded negative results, indicating a low possibility of feline infectious peritonitis (FIP) or septic peritonitis as etiologies.Radiographs following abdominocentesis revealed an abnormally heterogeneously high fat opacity in the upper and right mid-lower abdominal areas . FurtherThe cat received fluid therapy, antibiotic treatment, and H2 blocker in accordance with colitis management. Additionally, to address the issue of anorexia, a nasoesophageal tube was inserted for enteral feeding.Computed tomography  was performed under general anesthesia in sternal recumbency on next day to achieve an accurate diagnosis and for surgical planning . For CT After CT examination, elevated temperature (body temperature 40°C) was noted on regular vital check, and normalized after physical cooling. Fluid therapy, antibiotics, antiemetics, proton pump inhibitor, appetite stimulator, and pain medication (butorphanol 0.2 mg/kg q6h) was administered during hospitalization.Two days after CT examination, to determine the cause of the suspected EPS, multiple biopsies were performed via laparotomy. Cefazolin 30 mg/kg IV and hydromorphone 0.1 mg/kg IV was used as pre-medication for laparotomy and propofol 6 mg/kg IV was used as induction agent. General anesthesia was performed for a total 90 min from induction to endotracheal tube extubation, major vital signs were measured at 5 min intervals. Isoflurane was used for anesthesia maintenance and depth was adjusted upon procedure’s type and patient’s vital sign. Upon incision of the abdominal wall, a fibrous membrane was observed connecting the wall to the encapsulated abdominal organs and ascitic fluid . Additio2 fields) . Hematoxylin and eosin (H&E) staining revealed poorly differentiated, invasive malignant neoplasms (mitotic count 5–8 in 10 high powered 0.237 mm fields) –C. The nImmunohistochemistry (IHC) was conducted at IDEXX Laboratories to categorize neoplastic cell types. Cytokeratin (AE1/AE3) and vimentin were used as stains to further classify the tumor type as carcinoma, sarcoma, or mesothelioma. A diagnosis of mesothelioma typically involves positivity for both cytokeratin and vimentin markers. Conversely, if neoplastic cells are positive for only cytokeratin, it suggests carcinoma, while positivity for only vimentin indicates sarcoma. The results revealed that over 90% of invasive, poorly differentiated neoplastic cells exhibited strong immunoreactivity to cytokeratin (AE1/AE3) in the cytoplasm . HoweverAs no suspicious primary tumor was observed during ultrasound, CT scans, or laparotomy, the cat was diagnosed with peritoneal carcinomatosis. Furthermore, taking into consideration the presence of fibrous tissue encapsulating abdominal organs and bone formation, a diagnosis of SPC with osseous metaplasia was established. Unfortunately, due to the rapid deterioration of the patient following laparotomy, euthanasia was ultimately performed, and no treatment trial was initiated. Euthanasia was carried out by administration of T-61™  at a dose of 0.5 mL/kg after anesthetization with a propofol IV bolus of 6 mg/kg. A post-mortem examination could not be conducted, as it was declined by the owner.3This case report highlights the diagnostic challenges encountered in a feline patient presenting with ascites and suspected EPS based on imaging characteristics. Its clinical significance lies in the unexpected histopathological confirmation of SPC with osseous metaplasia, emphasizing the importance of considering SPC as a differential diagnosis when EPS is suspected on imaging.In humans, distinctive EPS findings on CT include peritoneal thickening, loculated fluid collection, peritoneal calcification, bowel tethering or matting, peritoneal enhancement, and occasional bowel wall thickening . Some stLimited information is available on the imaging features of EPS in veterinary medicine. A study of animals with presumptive EPS included seven dogs and two cats, all of which presented with ascites . RadiogrIn our case, the cat exhibited imaging features suggestive of EPS, including a one-sided stacked intestine on radiography, and a multilobulated intestinal mesentery and omentum with increased fat echogenicity, peritoneal thickening with calcification, and corrugated intestines on ultrasonography. Additionally, the CT findings revealed mesenteric and omental fat stranding, one-sided stacked bowels, gas-filled bowels, peritoneal thickening, and calcification. Based on these combined imaging findings, the patient was presumptively diagnosed with EPS. During laparotomy, we observed a fibrous membrane encapsulating the abdominal organs and ascites, similar to the previously reported gross features of another cat with EPS .Histopathological examination and IHC of the biopsy samples were crucial for providing a definitive diagnosis, revealing poorly differentiated, invasive malignant neoplasms that were positive for cytokeratin (AE1/AE3) and negative for vimentin, and therefore consistent with carcinoma. In this study, no suspected primary tumors were identified during CT or ultrasound examinations, nor were they detected on visual inspection during laparotomy. Therefore, this case was diagnosed with peritoneal carcinomatosis, desmoplasia, and osseous metaplasia. To the best of our knowledge, there have been no reported cases of peritoneal carcinomatosis with osseous metaplasia, calcification, or mineralization in the veterinary literature to date. In two retrospective studies of the imaging features of feline peritoneal carcinomatosis, none of the 24 cats examined showed mineralization on radiographs, ultrasound, or CT scans , 11. ThiThe etiology of desmoplasia and osseous metaplasia in peritoneal carcinomatosis is presumed to be similar to the mechanisms by which desmoplasia and osseous metaplasia occur in carcinoma. The pathogenesis of desmoplasia in carcinoma involves complex interactions between neoplastic cells and the tumor microenvironment, which includes a significant role played by cancer-associated fibroblasts (CAFs) , 13. CAFBone formation in tumor can be categorized in three types; osteogenesis in osteosarcoma, osteoblastic differentiation in sarcomatoid carcinoma, and osseous metaplasia . CarcinoIn conclusion, this case report offers valuable clinical insights into the diagnostic process for a feline patient with suspected EPS, where histopathological analysis revealed an unexpected diagnosis of SPC. Veterinarians should consider SPC as part of the differential diagnosis when encountering cases of suspected EPS, especially when radiological and clinical features are atypical or inconclusive. The sharing of similar clinical experience will improve diagnostic accuracy and potentially enhance therapeutic approaches for feline peritoneal diseases.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Written informed consent was obtained from the owner for the publication of this case report.S-JN: Writing – original draft, Conceptualization, Investigation. S-HS: Formal analysis, Writing – original draft. Se-HL: Investigation, Writing – review & editing. S-YJ: Investigation, Writing – review & editing. JA: Project administration, Writing – review & editing. Su-HL: Formal analysis, Writing – original draft, Writing – review & editing. M-OR: Conceptualization, Writing – original draft, Writing – review & editing.


Dentigerous cysts are the second most common odontogenic lesion, after radicular cysts. Dentigerous cysts mainly affect individuals in their second to fourth decades of life, with a slight male predominance. Because diagnosis is often late, surgical procedures like enucleation and removal of the impacted tooth misplaced are often necessary.However, if a dentigerous cyst is detected early in a child with delayed tooth eruption, the treatment goal is to preserve and properly position the permanent tooth within the arch. In such cases, conservative approaches like cyst decompression may be considered appropriate.We present a case of a dentigerous cyst in a 10-year-old child with delayed eruption of teeth 22 and 23. The condition was managed using decompression alone and orthosurgical traction, which facilitated the proper placement of the impacted teeth within the arch. This article emphasizes the significance of a multidisciplinary approach involving surgical and orthodontic management for dentigerous cysts in children, along with the importance of patient compliance with the treatment plan. Dentigerous cysts (DC) are a type of inflammatory odontogenic cyst associated with an included or impacted tooth according to the 2017 WHO classification [DC are benign lesions that encompass the crown of an unerupted tooth and can grow a very large size. Various therapeutics exist and are used with a therapeutic approach based on the patient’s age, localization of the cyst, and the tooth implicated. Conservative techniques, such as marsupialization or cystic decompression, are particularly indicated in children with immature permanent teeth contained within the DC . MarsupiThis case report follows the CARE (2017) guidelines for case reports . We presA 10-year-old male patient was referred to the Oral Medicine Department of the Reims University Hospital for an orthodontic consultation due to the unilateral non-eruption of the left lateral incisor. The patient had no particular medical or surgical history.Extra-oral clinical examination showed no abnormalities, including no swelling or lymphadenopathy.Intra-oral clinical examination Figure  revealedAt first, panoramic radiography was performed, uncovering a radiolucent image, homogeneous, unilocular, well-delimited, and located within the left maxillary permanent incisor and canine, both enclosed in an ectopic position Figure . The perThe deciduous left lateral incisor, in direct communication with the cystic lesion, of this 10-year-old child was then extracted and a sample of the cystic membrane was obtained for histological analysis. A silicone tube was placed in the cystic cavity through the socket of the extracted tooth in order to decompress the cyst Figure . AmoxiciAnatomopathological analysis revealed a non-keratinized squamous epithelium of variable thickness containing inflammatory remodeling with polynuclear cells and lymphocytes, without any suspicious character. This appearance is compatible with the diagnosis of an inflamed DC, given the context of impacted teeth Figure .In order to avoid mesialization of the left maxillary posterior region, a Nance arch was placed with regular orthodontic appointments. After four months of cystic decompression, the drain was removed; the left deciduous canine and the left deciduous first molar were extracted. Clinical and radiographic follow-up, including dental panoramic radiography, were performed and showed progressive regression of the cyst and progression in the coronary direction of the permanent left lateral incisor and maxillary canine with root apexification Figure .The permanent maxillary lateral incisor continued to erupt naturally over the next nine months without orthodontic intervention. However, orthodontic-surgical traction was required for the permanent left maxillary canine after one year of cyst decompression. The left maxillary canine became fully functional four years after the initiation of cyst decompression Figure .Finally, with a conservative approach combining a minimal surgical technique by decompression and orthodontic treatment, the DC appeared to have been completely eliminated by the placement of the causal impacted tooth within the dental arch Figure .DC arise from the dental follicle of an unerupted or developing teeth, attached at the cementoenamel junction. They represent the second most common odontogenic lesion after radicular cysts . HoweverThe pathogenesis of DC is related to pressure from an erupting tooth on the follicle, potentially obstructing circulation and leading to the accumulation of exudate between the reduced enamel epithelium and the tooth crown . ManaginAccording to Berretta et al. , both cyA recent study by Nahajowski et al.  investigIn our patient's case, the left maxillary lateral incisor was at Nolla stage 7, while the permanent left canine was at stage 6. His young age at the time of the fortuitous discovery of the cyst, the absence of complete root edification of the impacted teeth, and his compliance with treatment were key factors that had a major impact on the prognosis of the teeth, which are now functional. The cyst decompression procedure lasted approximately four months. These results seem to be in agreement with those reported by Allon et al. , where tIn this case, follow-up every two months was carried out by both an oral surgeon and an orthodontist, enabling us to effectively manage permanent tooth placement in the arch. Close collaboration is essential to ensure that orthosurgical traction of the canine is performed at the right time, depending on its intraosseous three-dimensional position and degree of root development. It is also important to emphasize that no additional cyst enucleation surgery was required, and complete cyst recurrence was confirmed through three-dimensional imaging. A similar finding is supported by Lizio et al. , who alsA few similar cases published in the scientific literature have shown a similar approach to the management of DC in children. Takagi et al.  reportedHowever, long-term follow-up remains essential until the impacted tooth is properly placed in the arch. The recurrence of DC is uncommon, particularly after complete cyst removal or tooth eruption.Given its prevalence, knowledge of the different treatments for DC, depending on the patient's age and dental training, is fundamental. In children, a conservative approach by marsupialization or decompression will always be favored in the first instance so as to encourage the maintenance of the affected permanent teeth within the dental arch. Close collaboration between orthodontists and oral surgeons is essential.Nevertheless, a histological analysis remains essential to avoid misdiagnosing cysts or more aggressive tumors such as keratocyts, ameloblastomas, or other aggressive tumors. Finally, to ensure the effectiveness of the treatment, strict compliance and hygiene must be maintained throughout the treatment course as a guarantee of success.


The case at hand involves a 25-year-old woman suffering from a gradual enlargement of a gluteal mass over a five-year period. This rare medical condition is classified as superficial CD34-positive fibroblastic tumor (SCPFT), an intermediate form of mesenchymal malignancy, originally identified in medical literature in 2014. Initial physical examination revealed a 10 x 10 cm lump in the right upper gluteal region. Magnetic resonance imaging demonstrated a well-circumscribed lesion within the right gluteus maximus muscle. The patient had the lump removed surgically. Examination under a microscope revealed tumor cells with extensive pleomorphism, high cytokeratin (CK) and CD34 positivity, and a low Ki67 index, all of which are consistent with SCPFT. Postoperatively, the patient showed marked improvement, and ongoing monitoring was initiated due to the potential for local recurrence. This case reinforces the importance of considering SCPFT in the differential diagnosis of soft tissue masses, emphasizing the role of immunohistochemical staining in reaching a correct diagnosis. Given the rarity of SCPFT, more studies are necessary to refine our understanding and treatment approaches. In 2014, Carter and his team first described superficial CD34-positive fibroblastic tumor (SCPFT), categorizing it as an intermediate mesenchymal cancer . Given iA 25-year-old married female patient with no prior health conditions was presented to our medical center via the clinic, reporting a right upper gluteal mass with five-year duration. The patient first noticed a chickpea-sized, painless lump in her right outer gluteus, which had slowly increased over the years, growing more rapidly recently to reach the size of a hand fist at presentation.Over the last six months leading up to the admission, the patient reported experiencing generalized weakness, anorexia, and a weight loss of 6 kg. No changes were noted on the skin overlying the mass, no discoloration, and no discharge. There were no accompanying symptoms of leg swelling, leg numbness, visible dilated veins, or the presence of other leg masses.The patient denied having any abdominal pain, changes in bowel habits, nausea, vomiting, abdominal distension, chest pain, shortness of breath, palpitation, dizziness, low back pain, joint pain, or headache. There was no history of recent trauma, travel, or new drug use.2 sat) of 98%, and weight of 64 kg. During physical examination, a noteworthy finding was a lump situated on the upper lateral aspect of the right gluteal region. This lump was round, approximately 10 x 10 cm, and characterized by a smooth surface, well-defined boundaries, and limited mobility. It did not exhibit any signs of tenderness or pulsatility. In addition, the patient's inguinal lymph nodes were not palpable, indicating no signs of lymphadenopathy. The rest of the examination incorporated a thorough neurological assessment, with normal patellar and ankle reflexes. Furthermore, the examination of peripheral pulses revealed symmetry on both sides, suggesting a normal vascular function.On presentation, the patient was conscious, alert, and oriented. Vital signs were within the normal range: temperature of 36.7 °C, heart rate (HR) of 82 beats per minute (BPM), blood pressure (BP) of 105/60 mmHg, oxygen saturation (OA pelvic MRI Figure  revealedThe pathological examination Figure  showed aThe patient was followed up postoperatively, showing significant improvement and resolution of the mass. Long-term follow-up and monitoring have been planned for the patient due to the potential for tumor recurrence.SCPFT is a relatively new entrant in the medical literature . This tuHistologically, SCPFT exhibits no unique characteristics but possesses several identifiable features. It confines primarily to the deep dermis and superficial fibro adipose tissue, with the cells often appearing as a plump spindle to epithelioid . The enlTreatments typically involve surgical excision, showing a generally favorable outcome with only one reported instance of lymph node metastasis post-operation . RecurreThis case report sheds light on diagnosing and managing an SCPFT, a relatively rare entity. Despite its typical presentation as a slow-growing, painless lump, its accurate identification requires a detailed histopathological and immunohistochemical examination, with key features, such as strong CD34 positivity assisting in its differentiation from other mesenchymal tumors. Surgical excision remains the primary treatment, and vigilant postoperative follow-up is essential due to the potential for local recurrence. Our report emphasizes the need for further research on SCPFT to refine diagnostic and management strategies.


In this case report, we utilized a three-dimensional printing model to replicate the complex anatomy of a criss-cross heart with double outlet right ventricle—an extremely rare congenital cardiac abnormality. This approach facilitated our understanding of the patient's unique condition and enabled us to plan the surgical procedure with greater precision.Our department received a 13-year-old female patient who presented with a pronounced heart murmur and a decrease in exercise capacity. Subsequent two-dimensional imaging revealed the presence of a criss-cross heart with double outlet right ventricle—an intricate and uncommon cardiac malformation that poses challenges for accurate visualization through conventional two-dimensional modalities. To address this challenge, we constructed and printed a three-dimensional model using computed tomography data, which enabled us to visualize and understand the complex intracardiac structures and plan surgical interventions with greater precision. Using this approach, we successfully performed a right ventricular double outlet repair, and the patient made a full recovery following the procedure.The criss-cross heart with double outlet right ventricle constitutes a complex and uncommon cardiac anomaly that poses considerable challenges in terms of diagnosis and surgical intervention. Employing three-dimensional modeling and printing represents a promising approach, given its potential to enhance the precision and comprehensiveness of the anatomical evaluation of the heart. As a result, this method holds significant promise in facilitating accurate diagnosis, meticulous surgical planning, and ultimately improving clinical outcomes for patients affected by this condition. Criss-cross heart (CCH) is an infrequent cardiac anomaly characterized by the crossing of inflow streams of both ventricles, caused by twisting of the heart along its long axis. This condition may arise due to malformations in any cardiac segment, causing disruptions in the connections between atria, ventricles, and great vessels . The useA 13-year-old female patient, lacking a family history of cardiovascular disease, was admitted to our department with a notable heart murmur and deteriorating exercise capacity. At five months of age, the patient had been diagnosed with a multifaceted congenital heart malformation at a local hospital; however, inadequate medical technology and financial constraints prevented further diagnostic evaluation at a more sophisticated medical center. The patient thrived during her early developmental years, and the issue went unnoticed by the family until recently when her tolerance to exercise significantly declined. Upon physical examination, the child exhibited a height of 1.52 m and weight of 36 kg, with a resting heart rate of 118 beats/min and an oxygen saturation level of 97%. Notably, apical pulsation was detected in the right anterior chest, and systolic murmurs were auscultated. Following the completion of conventional imaging procedures, including transthoracic echocardiography (TTE) , computeCCH anomaly is extremely rare, accounting for less than 0.1% of all congenital heart defects. The diagnosis of CCH is based on the intersection of the axes of the ventricular entries. This condition is characterized by the mass of the ventricular wall rotating along its major axis. This promotes a change in hemodynamics characterized by crossing flows through the atrioventricular valves, resulting in the false impression that each atrium is being directed to the contralateral ventricle . The venSeveral studies have highlighted the potential utility of 3D technology in medical education, surgical training, and pre-operative planning for CHD and then improve surgical outcomes and reduce costs , 6. Sun Strengths of this report include the utilization of 3D printing technology to construct a detailed model of a CCH with DORV, which enabled the medical team to better visualize and understand the unique intracardiac structures. This approach allowed for more precise preoperative planning and accurate surgical intervention, resulting in a successful outcome for the patient. The case report also highlights the potential benefits of employing 3D modeling and printing in cases of rare and intricate cardiac anomalies. This is one of the rare reports that illustrated the anatomy of the CCH using 3D technology and assisted in preoperative planning. This case report has some limitations that must be considered. Firstly, its focus on a single patient, without a comparison group, may limit the generalizability of the findings. Additionally, the costs and time required to create a 3D print model may hinder widespread utilization of this approach. Furthermore, the use of virtual reality could aid in further illustrating the complex anatomy of CCH for diagnosis and surgical planning. Lastly, it should be noted that the 3D model employed was a hollow heart lacking valves and coronary arteries, which may impact its clinical relevance and applicability.The present study describes a case of CCH with DORV, an extremely rare congenital cardiac abnormality that poses substantial challenges for diagnosis and surgical intervention. We report successful diagnosis and surgery through the utilization of 3D imaging and printing, yielding excellent clinical outcomes. Our findings provide compelling evidence that 3D printing technology can be employed to create highly detailed models of complex intracardiac structures, offering greater clarity and precision in anatomical evaluation and facilitating more accurate preoperative planning and surgical interventions. Nevertheless, it should be noted that the limited availability and high cost of this technology may impede its widespread adoption. Future research endeavors could further elucidate the potential benefits of 3D modeling and printing in cases of intricate and uncommon cardiac anomalies, ideally incorporating larger sample sizes and comparison groups.


Eosinophilic cholecystitis (EC) is an uncommon cause of acute cholecystitis; the clinical presentation is indistinguishable from other types of cholecystitis, and the diagnosis is made by histopathology study. We present the case of a 73-year-old male patient with right hypochondrial abdominal pain suggestive of symptomatic cholelithiasis. There were no significant findings at the blood workup or physical examination; he underwent a cholecystectomy and was later diagnosed with eosinophilic cholecystitis by histopathology. EC may be associated with some other systemic conditions, such as eosinophilic granulomatosis, eosinophilic ascites, or parasitosis, which will require specific management. Acute cholecystitis has a wide range of etiologies; among them, lithiasis accounts for 90% to 95% of cases ,2. OtherThe literature has reported a prevalence of EC ranging from 0.25% to 6.4% [Clinically, EC is indistinguishable from other types of cholecystitis ,5, the dA 73-year-old male patient from Guerrero, Mexico, comes as an outpatient for medical evaluation at a private hospital in Mexico City. He has been presenting intermittent episodes of abdominal pain for seven days, predominantly in the epigastrium and right hypochondrium, colic type, intensity 10/10 on the pain scale, which is exacerbated with fatty meals and associated with nausea without vomiting; no previous management has been established. He has been diabetic for 30 years and is being managed with glimepiride and metformin. He has also smoked 20 cigarettes per day for 38 years. He also refers to being allergic to penicillin. Physical examination revealed good general condition. Abdomen without pain on palpation and no negative murphy sign. Rest of his examination including cardiovascular, respiratory and neurological showed no abnormalities.Liver and gallbladder ultrasound was requested as part of the diagnostic work-up where the findings were: gallbladder of 56.7 x 32.5 x 30.3 mm, thin wall of 2.2 mm, the content was an oval echogenic image which projects a posterior acoustic shadow with a diameter of 14 mm suggestive of one gallstone, choledochus of 3.1 mm , and findings in imaging studies have a sensitivity of 91.2% and specificity of 96.9% for cholecystitis . More reFor EC, the definitive diagnosis is made through the histopathology report, where >90% of the gallbladder wall is infiltrated by eosinophils -7. It caEC is usually a benign condition; in those patients who only present inflammation of the gallbladder, it resolves with the surgical intervention "cholecystectomy" -7. HowevEosinophilic cholecystitis is a rare diagnosis with a benign course that will require a cholecystectomy as part of the management. However, since the diagnosis can only be made postoperatively through the histopathological report, it is the task of physicians to intentionally look for other associated pathologies because EC has been associated with some other syndromes and diseases that will require specific management to avoid possible complications.


Early traumatic diaphragmatic rupture diagnosis using radiological investigations and early surgical management is critical to avoid complications.Traumatic diaphragmatic rupture (TDR) is a rare presentation reported following blunt trauma due to road traffic accidents. Our case showed the importance of early diagnosis of TDR using radiological investigations. Also, early surgical management is critical to avoid complications. Traumatic diaphragmatic rupture (TDR) is an uncommon occurrence and is reported in 0.3%–1.6% of patients with a history of trauma.Herein, we present the case of a 28‐year‐old man who presented to ER with traumatic diaphragmatic rupture with herniation of abdominal contents into left hemithorax, multiple rib fracture, right scapular fracture, and left lung collapse following RTA. CXR revealed an elevated left hemidiaphragm with multiple rib fractures Figure . FocusedThe mainstay of the management of diaphragmatic rupture is the surgical approach. Silva et al. reported that a higher proportion of patients were treated by an abdominal approach, more so in the acute phase.Our case showed that a laparoscopic approach is preferred over an abdominal as a diagnostic and therapeutic tool in acute traumatic cases with diaphragmatic rupture. Its advantages include excellent view and access to the surgical field, faster recovery, and lower postoperative pain with less recurrence rate. In addition, regular follow‐up of patients is crucial to detect recurrences.Banwari Lal Bairwa: Conceptualization; data curation; investigation; project administration; resources; supervision; validation; visualization; writing – original draft; writing – review and editing. Ayush Anand: Conceptualization; data curation; investigation; resources; supervision; validation; visualization; writing – original draft; writing – review and editing.The authors did not receive any funding for this manuscript.The author(s) have no conflict of interests to declare.Our institution does not require ethical approval for reporting individual cases or case series.Written informed consent was obtained from the patient(s) for their anonymized information to be published in this article.


Myxomas are rare in the vocal cords. A 69-year-old man was admitted with one-year history of progressive dysphonia. Laryngoscopy revealed a polypoid mass on the right vocal cord. The diagnosis was cellular myxoma. A review of the literature including the present case revealed eleven reported cases of myxoma. Ten cases were classic myxoma. To the best of our knowledge, cellular myxoma has not been previously reported in the vocal cord. Hypercellularity does not affect the behavior of cellular myxoma. However, its recognition is important to prevent confusion with the group of low-grade myxoid sarcomas. Cellular myxoma should be considered in the differential diagnosis of any vocal cord mass. Classic myxoma is a benign mesenchymal paucicellular tumor characterized by bland spindle and stellate shaped cells embedded in hypovascular, abundant loose myxoid stroma. The cellular variant of this tumor shows hypercellularity, more numerous collagen fibers, and increased vascularity ,2.Myxomas of the larynx are very uncommon. The reported sites of involvement are the vocal cords, the aryepiglottic fold, and the epiglottis. They are more common in the vocal cords. As far as we are aware, only ten cases of vocal cord myxomas have been previously reported. We describe herein a case of the cellular variant of myxoma in the right vocal cord and review the literature. To the best of our knowledge, a cellular myxoma (CM) in a vocal cord has not been previously reported.A 69-year-old man was admitted to the hospital with one-year history of progressive dysphonia. There was no associated pain, stridor, hemoptysis or weight loss. He was currently consuming 40 g of alcohol a day and had quit smoking 20 years ago. The patient was diagnosed with a polyp of the right vocal cord 20 years ago, but he refused the surgical treatment. Medical history was also significant for atrial fibrillation with multiple embolisms in the vertebrobasilar artery, brachiocephalic trunk, and mesenteric artery and chronic hepatic disease with thrombosis of the right portal vein. Syndromic associations were not present. Flexible laryngoscopy revealed a large polypoid lesion on the right cord with preserved mobility.The patient underwent phonosurgery under general anesthesia.The specimen consisted of a glistening white, gelatinous, polypoid mass measuring 0.9 x 0.6 x 0.3 cm. Histopatho-logical examination revealed an excrescent tissue fragment consisting of squamous mucosa that was partially atrophic and a mesenchymal neoplasm . The tumThe patient was discharged in hours. One month later his voice was much improved. No signs of recurrence were observed.Pure myxomas are very infrequent in the vocal cords. A review of the literature revealed only ten previously reported cases  3–12). . 3–12). Cells of a myxoma originate from modified fibroblastic cells that lack the ability to polymerize collagen. As an alternative, they produce an excessive amount of glycosaminoglycans giving them a gelatinous appearance on gross examination. The process suggests an underlying localized error in tissue metabolism .CM is characterized by hypercellular areas that occupy from 10 to 90% of the tumor. These foci have increased number of cells, more prominent vascularity, increased collagen content and less extracellular myxoid matrix than classic myxoma. The hypercellular regions are not associated with cytologic atypia, multinucleated giant cells, mitotic activity, or necrosis. Vessels are capillary-sized but occasional thick-walled vessels with smooth muscle in their walls can be present. CMs usually show sparse paucicellular areas of classic myxoma with scant capillary-sized vessels .All the cases of CM reported out of the larynx have behaved in a benign fashion with only a small risk of local non-destructive recurrence if not excised completely ,2. Thus,The main differential diagnosis includes myxoid neurofibroma, low-grade myxofibrosarcoma, low-grade fibromyxoid sarcoma, and myxoid liposarcoma. Myxoid neurofibroma shows spindled elongated cells with tapering, wavy or bent nuclei and pale indistinct cytoplasms embedded in abundant myxoid background. Intralesional neural fibers are demonstrated with neurofilament protein. Besides, a considerable number of cells are positive for S100 protein ,15. CM, In conclusion, CM of the vocal cord is a benign mesen-chymal tumor that shows foci of increased cellularity and vascularity, with presence of thick-walled vessels, and increased collagen content. The recognition of this tumor is important to avoid a misdiagnosis of any type of low-grade myxoid sarcoma. Although very rare, CM should be considered in the differential diagnosis of any vocal cord mass to allow for adequate treatment. Surgery is considered curative.


Laryngeal chondrosarcoma is rare and accounts for 0.2% of all larynx malignancies. Although chondrosarcoma is the most common sarcoma seen in the larynx, laryngeal involvement by cartilage tumors is rare. In this article, we aimed to present the differential diagnosis of chondrosarcoma located in the thyroid cartilage, which is a rare site, in a 75-year-old male patient. The patient underwent total laryngectomy by the otolaryngology department. The macroscopy of the laryngectomy material sent to the pathology laboratory revealed a 3x2 cm tumor with a polypoid extension to the lumen from the bottom of the right vocal cord. Although clinical and radiological findings are important in the diagnosis, the definite diagnosis is based on the pathological examination. It is especially important to differentiate the lesion from chondromas. Laryngeal chondrosarcoma is rare and accounts for 0.2% of all larynx malignancies . AlthougThe most common symptom is hoarseness and may come with complaints of difficulty breathing, dysphagia, and hemoptysis ,6. TumorIn this article, we aimed to present the differential diagnosis of chondrosarcoma located in the thyroid cartilage, which is a rare site, in a 75-year-old male patient.A 75-year-old male patient was admitted to the chest diseases department at an external center with the complaint of shortness of breath lasting for three weeks. He was referred to the ear, nose and throat diseases clinic by chest diseases clinic, considering that the patient’s complaint might have been caused by a thyroid disease. Neck laryngography performed on laryngoscopy at the subglottic level reported a hypoechoic nodule of 4.5 x 5 cm size with coarse calcifications that covered a large part of the right lobe of the thyroid gland and had an intrathoracic extension. On thyroid scintigraphy, there were nodules that filled the entire right lobe with a hypoactive appearance in the lower part of the left thyroid lobe, and the appearance was interpreted as a multinodular hyperplasic thyroid gland. On neck MR, there was a nodule of 49x50 mm size with contrast enhancement that created partial obliteration to the trachea by filling the entire thyroid gland, and histopathological correlation was recommended to exclude malignancy.As a result of these examinations, thyroidectomy and excision of the mass were performed by the otorhinolaryngology diseases clinic, and a temporary tracheostomy was opened. On the 15th day, the tracheostomy was closed. The surgical material sent to the pathology department at the external center was reported as a grade 2 chondrosarcoma invading the thyroid tissue.PET-CT was taken one month later. F-18 FDG involvement was more pronounced in the non-homogeneous periphery of the lesion, which extended to the thyroid tissue in the neck, and narrowed the air column while destroying the larynx on the right side, while its borders could not be clearly evaluated (SUV max 5.2). Metabolic activity was observed in the lesion described in right thyroid cartilage.As a result of these examinations, the patient applied to the Otorhinolaryngology Diseases clinic of Abant Izzet Baysal University Izzet Baysal Training and Research Hospital. The CT showed a subglottic mass compatible with chondrosarcoma arising from the right thyroid cartilage. On axial and coronal MRI images, a malignant mass invading the thyroid, arytenoid and cricoid cartilages with heterogeneous contrast enhancement is observed.  On thoraThe case with pathological stage 4a received radiotherapy. The patient was followed up for 2 years. He is presently without any sign of recurrence.Although the etiology of laryngeal chondrosarcoma is not clearly understood, radiation exposure, teflon injection, and disorganized ossification are important risk factors ,8. ThereAlthough symptoms vary according to the localization of the tumor, dyspnea and airway obstruction and a painless mass may be observed in subglottic lesions .It is difficult to estimate the true incidence of laryngeal chondrosarcoma, as there have been cases of low-grade chondrosarcoma diagnosed in the past as chondroma .The average age at the time of diagnosis is 60-64 years and it is more common in men . It is mA 61-year-old patient reported by Moerman et al. had a low-grade chondrosarcoma located in the anterior part of the thyroid cartilage . Sauter All of the 6 cases by Buda et al. consisted of male patients . In addiOne of two cases of Nao et al. was diagnosed with low-grade chondrosarcoma located in the thyroid cartilage .Oliveira et al. presented 6 cases by reviewing the 10-year larynx chondrosarcoma cases . Five ofThe diagnosis of low-grade chondrosarcoma can be delayed as it is clinically a slow lesion .On endoscopic examination, it can be observed as a submucosal mass under smooth-looking mucosa, especially in the posterior region, as well as with movement restriction in the vocal cord .Typical finding in CT is hypodense, well-cartied mass with limited calcification . MRI is Expansile lesions with popcorn-style calcifications on MRI or CT are important in the diagnosis ,9,10. ThChondrosarcoma is macroscopically flat, in the form of a lobular mass, and has a translucent appearance ,10. TumoIn the diagnosis of chondrosarcoma, histological criteria such as cell and nucleus size irregularities, excessive number of cells, hyperchromia, and chromatin aggregation in single or multiple chondrocytes, which were defined by Lichtenstein and Jaff in 1943, are used ,9. AlthoIn the case reported by Ghatak et al., a 42-year-old male patient was diagnosed with the biopsy of a 2 cm diameter mass with an arytenoid location, and a low-grade chondrosarcoma was diagnosed after the operation (Laryngeal chondrosarcoma is graded histologically as grade 1, grade 2, and grade 3 . Most laGrade 1 chondrosarcomas are tumors with a good prognosis, and the differential diagnosis with chondroma is clinically, radiologically and pathologically difficult . In caseIn a review by Chin et al., 163 articles were reviewed among articles published over 66 years (Histologically, nuclear atypia, increased cellularity, and invasion to neighboring structures are important in the diagnosis . ExcessiChondrosarcoma should be differentiated from laryngeal nodular chondromalacia, fibrosarcoma and osteosarcoma, as well as chondroma ,4. The pAlthough the treatment protocols vary, the main treatment protocol is surgery . In low-Laryngeal chondrosarcoma has a better prognosis than non-larynx localized chondrosarcoma and can be detected at an earlier stage .Laryngeal chondrosarcoma has a good prognosis and rarely metastasizes . MetastaSince laryngeal chondrosarcoma may relapse in the late period, it requires long-term follow-up .Laryngeal chondrosarcoma is a rare, low-grade tumor with a good prognosis. This case was a chondrosarcoma located in the thyroid cartilage, which is a rare site. Although clinical and radiological findings are important in the diagnosis, the definite diagnosis is based on the pathological examination. It is especially important to differentiate the lesion from chondromas. Adequate macroscopic sampling to evaluate the tumor site and invasion areas clearly after macroscopic evaluation is important in the correct diagnosis.


Venous thrombosis associated with the use of oral contraceptives (OCs) occurs mostly in the deep veins of the lower extremity. A lesion of the upper extremity is rare, and the majority of thrombotic events that occur in the superficial vein of the upper extremity are caused by intravenous catheters. We present a rare case of superficial venous thrombus on the upper extremity in a woman with a history of long-term OC use.A 35-year-old woman, with an 8-year history of OC use, presented with a 2-year history of painfully palpable masses on her left forearm. The lesion mimicking soft tissue mass was confirmed to be superficial venous thrombi through ultrasound and magnetic resonance imaging. Conservative treatment including non-steroidal anti-inflammatory drugs, vasoprotective agents, and aspirin was prescribed. Through consultation with the Department of Obstetrics and Gynecology, it was confirmed that the current OCs could be discontinued, and the pain was almost relieved after conservative treatment.If thrombotic events occur in the superficial vein of the upper extremity without intravenous catheters, detailed medical history taking and the possibility of OCs should be considered. A superficial soft tissue mass is a common indication for performing an imaging examination of the limb. Diseases of various pathologies, ranging from benign to malignant, appear as palpable masses or nodules in the skin. Soft tissue masses have different locations, and therefore, the location of the lesions sometimes aids in diagnosis. For example, epithelioid sarcoma can appear as single or multiple nodular lesions on the forearm, hand, and finger .On the other hand, superficial venous thrombus (SVT) may also appear in the form of a palpable nodule and may be palpated like a cord depending on the length of the lesion. SVT occurs mainly in the lower extremities and is associated with immobilization, blood hypercoagulability, malignancy, and female hormonal therapies . In contIn this study, the authors report a rare case of SVT of the upper extremity that was misdiagnosed as soft tissue masses that occurred at an unusual site of the venous thrombus associated with long-term OC administration.A 35-year-old  female patient presented with a painful mass in her left forearm that first occurred 2 years ago. She stood 161 cm tall and weighed 54 kg. At that time, she first discovered a mass on her left forearm with a heating sensation, swelling, and pain. After she took a non-steroidal anti-inflammatory drug (NSAID), the size of the mass decreased and the pain was relieved. However, the pain recurred repeatedly, and an additional mass occurred in the proximal part of the existing mass 6 months before she visited our clinic. In addition, from that time on, she was prescribed an NSAID without any imaging study, and the pain continued without subsiding. She was referred to our clinic after visiting a primary medical institution, with suspected soft tissue masses on the left forearm.Two linear masses were palpated in the longitudinal direction on the dorsal side of the left forearm of the patient . The masIn an interview conducted after the imaging tests, the patient, who did not mention any special past medical history, reported that she had been repeatedly taking and discontinuing OCs (ethinyl estradiol 0.03 mg + drospirenone 3 mg) for 8 years due to endometriosis and that she had been taking OCs steadily for the last 2 years. Tumor marker, autoimmune antibody, and hematologic laboratory tests were performed to identify the risk factors of venous thrombus other than OCs. Protein C Ag level was 80% (70%–140%), and Protein S Ag level was 95% (70%–140%). The patient tested negative for anticardiolipin antibody, antiphospholipid antibody, and lupus anticoagulant antibody. AFP, CEA, CA 125, CA 15-3, and CA 19-9 levels were normal. The patient showed no history of immobilization of the left upper limb, smoking, air travel, local trauma, insect bite, or repeated local compression related to her professional or personal activities, and in recent years, there has been no instance of catheterization placed around the forearm lesion, such as intravascular injection or fluid therapy. In addition, no venous thrombi or varicose veins were observed in other parts of the body.The patient was diagnosed with SVT in the left forearm caused by long-term use of OCs confirmed by the imaging tests, with the exclusion of other etiologies through medical history taking and blood tests. The patient did not complain of any respiratory symptoms, and fibrinogen degradation products (FDP) and D-dimer were in the normal range. No thrombus in the deep veins of the upper extremity was seen on the MRI, and computed tomography (CT) was not performed to confirm pulmonary embolism.As part of conservative treatment, NSAIDs  were administered, and local heat treatment was additionally performed as the inflammation was not in the acute phase. In addition, a vasoprotective agent  was added after consultation with the Department of Vascular Surgery, and aspirin 100 mg 1 T q.d. was administered as part of prophylactic treatment for recurrent venous thromboembolism and major vascular events. Through consultation with the Department of Obstetrics and Gynecology, it was confirmed that the current OCs could be discontinued, and the pain mostly relieved after conservative treatment.Three months later, the patient visited our clinic for a follow-up, and the ultrasound examination performed at that time showed that the blood clot remained in the vein. However, the pain relieved completely, following which the administration of anti-inflammatory drugs and vasoprotective drugs was discontinued. Aspirin was also discontinued after 3 months of administration. However, the patient experienced recurrent pelvic pain and vaginal bleeding because of endometriosis 6 months after discharge. After visiting a gynecologist, she was treated with a dienogest, an oral progestin that has a low risk of thrombosis. The pelvic pain relieved after the ingestion of dienogest.OCs usually consist of estradiol (E2) or ethinylestradiol (EE), which increase procoagulatory factors to form a thrombus. In addition, depending on the type and administration method, progestin preparations incorporated into estrogen may promote or inhibit thrombus formation, and therefore, care should be taken in selection . For exaSVT is a disease that has not yet been actively studied because its symptoms are mild and have a self-limiting course. Both superficial and deep vein thromboses are common in the lower extremities, and malignancies, autoimmune diseases, hypercoagulant conditions, and blood vessel damage increase the risk . On the The standard guidelines for the treatment of isolated SVT without deep vein thrombosis or pulmonary embolism are not clear . First, On the other hand, there is no approved treatment guideline for SVT in the upper and lower extremities . In the As described above, the SVT of the upper extremities can be commonly seen in relation to the intravenous route of administration of drugs and fluids. However, in order to accurately diagnose the disease as in this case, it is necessary to consider not only clinically common situations but also rare situations, and accurate and detailed medical history taking is required.We report a rare case of a patient with an SVT in the upper extremity, which was misdiagnosed as a soft tissue mass that occurred in an unusual site and was associated with OC administration without a catheterization history around the lesion.


Neuroendocrine tumors most frequently originate from the gastrointestinal tract (GIT). Their presentation in tissues other than the GIT and pancreas is usually due to metastatic involvement from lesions at these sites. There have been a few cases of neuroendocrine tumors identified in tissues such as the mesentery and peritoneum, without identification of a primary lesion supporting their origin as metastasis.We present the case of a patient with abdominal pain, in whom a primary mesenteric neuroendocrine tumor was identified. The patient completed one year of follow-up without identification of an additional lesion. Case Reported in line with the SCARE criteria.This is a rare condition with few reports in the literature, without significant changes in its classification or management.The search for a primary lesion and follow-up are essential to characterize the presence of primary mesenteric neuroendocrine tumors. •Neuroendocrine tumors commonly originate from the gastrointestinal tract.•There are few reports in the literature on primary neuroendocrine tumors of the mesentery.•The follow-up of neuroendocrine tumors is done using chromogranin A, 5-HIAA, scintigraphy, and octreotide scan.•The management of neuroendocrine tumors typically involves surgery, including in this presentation. They play a crucial role in various functions of the GIT, such as motility and secretion. Neuroendocrine tumors (NETs) predominantly arise from these cells and are mostly benign ,2. NeuroNETs are classified according to their location. For the gastrointestinal tract, they can be divided into Gastropancreatic NETs and Gastrointestinal NETs. In 2015, the National Comprehensive Cancer Network (NCCN) included tumor differentiation, mitotic rate, and Ki-67 in the classification of NETs. These factors determine the tumor grade, which guides treatment decisions .Considering the origin of NETs, the occurrence of extra-GIT  lesions is infrequent, assuming the previously described information. In such cases, the presence of a primary tumor should be ruled out, as we seen in the mesenteric carcinoid tumors . However, some reports in the literature describe the presence of extra-GIT lesions without identification of an alternative origin ,6. It isThe presence of chromogranin A and 5-hydroxyindoleacetic acid (5-HIAA) indicates the presence of NETs and is supplemented with imaging studies such as scintigraphy and octreotide scan to rule out metastasis and identify the primary tumor . Here, w2A 56-year-old man with a history of appendectomy presented to the emergency department with acute abdominal pain in the right hypochondrium and epigastrium, associated with oral intolerance. Upon admission, there were no signs of peritoneal irritation, clinical signs of systemic inflammatory response, or positive inflammatory markers. Due to persistent symptoms, an abdominal ultrasound was performed, showing suggestive images of retroperitoneal lymph node involvement. Complementary contrast-enhanced abdominal CT showed a round, homogeneous, solid mass in the mesogastrium, causing compression on the inferior vena cava (IVC) and displacement of the second portion of the duodenum , Fig. 2.The patient underwent exploratory laparoscopy, identifying a mass of approximately 5 × 6 cm located at the root of the mesentery, in contact with the inferior vena cava (IVC), but with a cleavage plane between this structure. Vascular compromise of the small intestine was identified from 210 cm of the suspensory angle of the duodenum to 60 cm of the ileocecal valve, with impaired perfusion in this portion of the ileum. It was decided to convert to laparotomy, performing dissection using ultrasonic energy around the described mass. Due to direct involvement of the vasculature of the distal ileum, 60 cm of compromised small intestine were resected, and a lateral-lateral mechanical anastomosis was performed using the Barcelona technique. At the end of the procedure, the abdominal cavity was explored, and no evidence of lesions in the length of the small and large intestines, stomach, liver, pancreas, and intraperitoneal rectum was found.The patient's hospital stay was prolonged due to postoperative ileus, initially managed with intravenous fluids and hydroelectrolyte replacement, however, a reintervention was required on the tenth day of management, intraoperatively identifying early adhesions. Adhesiolysis was performed without additional documentation of other alterations. Subsequently, the patient had a satisfactory postoperative recovery and was discharged on the fifth day after the reintervention.In pathology, a specimen was received including 60 cm of congested small intestine adhered to the mesentery, with a large congestive mass measuring 7 × 5 × 2 cm, with fibrous and hemorrhagic areas. No involvement of the intestine by the mass was defined , Fig. 4.During the first six months of follow-up, the patient was evaluated by endocrinology and oncology, and the study was complemented with upper gastrointestinal endoscopy and colonoscopy. A small hyperplastic polyp was resected during the colonoscopy, which tested negative for malignancy. Negative results were also obtained for chromogranin A (90 ng/ml) and 5-HIAA (9 mg/24 h). Additionally, a technetium scintigraphy showed somatostatin receptor expression in the ascending and transverse colon, leading to direct confirmation with repeated endoscopic studies, which did not identify any additional lesions.Based on the results of the scintigraphy, a PET-CT scan with gallium was performed, which showed no evidence of somatostatin receptor overexpression. Chromogranin and 5-HIAA controls at ten months of follow-up remained negative, and the patient is currently asymptomatic.3Primary mesenteric tumors are a rare entity, with few reports available in the literature . The majRadiological differential diagnoses for hypervascular extramural subepithelial lesions include Castleman's disease, metastatic disease, lymphadenopathies, and neuroendocrine tumors . On compNeuroendocrine tumors, previously referred to as carcinoids, are more frequently generated in the gastrointestinal tract; Histopathological evaluation typically reveals structural patterns such as nests, cords, and ribbons, with strong expression of neuroendocrine markers like synaptophysin and chromogranin A, which are shared with paragangliomas, excluding this diagnosis based on cytokeratin positivity ,6.These tumors often present with symptoms of intestinal obstruction, abdominal pain, or a mass sensation, and in other cases, they may be associated with carcinoid syndrome, which is absent in non-secreting tumors . CarcinoThe World Health Organization introduced a classification system for NETs based on their proliferative activity determined by mitotic rate and the Ki-67 index . Low-graWhen identifying a localized NET in the mesentery, it is always important to rule out metastatic involvement, as it is documented in 40–80 % of gastrointestinal NETs ,2. ComplSurgical resection is usually the cornerstone of management for carcinoid tumors, including the resection of surrounding lymph nodes ,5, as peSimilar cases were found in the literature. Lee et al.  reported4There are few reported cases of peritoneal NETs in the available literature. In the present reported case, after one year of follow-up, there is no evidence of a primary lesion to determine its metastatic origin. This case adds to the previously described cases, which amount to no more than ten cases in the available literature. Metastasis of the lesion cannot be entirely ruled out.According to the reviewed literature, the identification of extra-gastrointestinal and pancreatic NETs warrants an exhaustive search for a primary tumor in these locations, as was done in the present case. However, no additional lesions have been identified at the moment, leaving the possibility of identifying these extra-GIT as a rare occurrence, as in the present case.AGM: Data collection, photography, literature review, writing.EJA, NAR, CRG: Thematic consultation, writing.JGR, PGS: Literature review, writing.Informed consent was obtained from the patient, which was accepted by the ethics committee of the Hospital Universitario Mayor Mederi, Bogotá, Colombia.Ethical approval for this study (DVO005 2315 CV21696) was provided by the ethical committee of the Hospital Universitario Mayor Mederi, and Rosario University, Bogotá, Colombia (06/05/23).None of the authors have any conflicts of interest.


The septochoanal polyp is one of the choanal polyps derived from the nasal septum. They rarely occur, with only a few cases reported in the English literature. The etiology is still uncertain though it is thought to be associated with inflammation. Pathological findings generally show chronic inflammatory polyps and should be differentiated from other tumors. We report a case of a 32-year-old man diagnosed with a septochoanal polyp on the concave side of the deviated nasal septum. Previous reports have not mentioned the relationship between septochoanal polyp and the direction of septal deviation. This case is a practical example when considering the potential causes of the septochoanal polyp. Choanal polyps are one form of nasal polyps that extend toward the choana . They arWe herein report a 32-year-old man diagnosed with a septochoanal polyp on the concave side of the deviated nasal septum. This case is a practical example of pondering the pathogenesis of septochoanal polyp and their characteristics compared to the other tumors.A 32-year-old man presented to our hospital after a polyp in the right nasal cavity was found at the otolaryngology clinic near his home. He had not complained of rhinorrhea or nasal pain but right nasal obstruction for three months. He had no history of allergic disease. We performed an endoscopic examination and found a white-yellow mass arising from the posterior part of the nasal septum and extending into the nasopharynx Figure .Computed tomography revealed a soft tissue mass clinging to the right nasal septum without any signs of bone destruction and sinus invasion Figures -2C. AddiWe excised the mass endoscopically along with its base, including the surrounding healthy mucosa of the right septum under local anesthesia Figure . We deciAny signs of dysplasia or malignancy were not found. At the three-week visit, the lesion was replaced with healthy mucosa Figure .We experienced the case of a patient with a septochoanal polyp on the concave side of the deviated nasal septum. The pathogenesis is still unknown, although the pathological findings suggest it is an inflammatory change. It is worth considering that the increased air stream on the concave side might have triggered the inflammation, forming a septochoanal polyp.There have been only 13 cases of septochoanal polyp reported in the English literature. Four cases are men, five cases are women, and other cases are unknown . No caseAlthough the pathogenesis is still unknown, allergy and mechanical pressure are thought to be the potential cause. Cook et al. reported that antrochoanal polyp was associated with allergic disease, including allergic rhinitis, bronchial asthma, and syndrome of aspirin sensitivity . But theOn the other hand, mechanical pressure might be related to the occurrence of septochoanal polyps. Most nasal polyps arise from the lateral walls of the nasal cavity, the anterior ethmoidal sinus mucosa, the contact areas of the uncinate process, and the middle turbinate . These aOur patient showed a septochoanal polyp occurring on the concave side of the deviated nasal septum. Unfortunately, we didn’t measure the airflow volume in our patient. But increased airflow in the right nasal cavity might have caused mucosa inflammation at the posterior nasal septum, leading to polyp formation. The relationships between the side of the septochoanal polyp and the direction of septal deviation have rarely been mentioned. Only one case in which a septochoanal polyp arose on the concave side has been documented in the English literature, while one is on the convex side ,5. The nPathological findings of choanal polyps are almost identical to those of an inflammatory polyp. They generally show inflammatory cell infiltration and edema in stroma while some reports point out that fewer eosinophils and submucous glands are confirmed in choanal polyps . HistopaThe standard treatment for choanal polyp is the surgical removal of the polyp. All 13 patients of septochoanal polyp underwent surgical resection. Additionally, there were no cases of recurrence in septochoanal polyp (follow-up period: 3-12 months), while the recurrence rate of the choanal polyp is reported to be 6 to 7.5% . The proWe encountered a 32-year-old man with a septochoanal polyp on the concave side of the deviated nasal septum. There are two possible causes for the development of such polyps, allergy and mechanical pressure. In the present case, increased airflow on the concave side might have caused inflammation of the mucosa at the posterior nasal septum, leading to polyp formation. We treated our patient by excising the polyp completely and our patient didn't show any signs of recurrence at three weeks follow-up.


Adrenal cavernous hemangiomas are rare benign vascular tumors that pose significant diagnostic challenges. Despite their benign nature, features overlapping with malignancies often complicate management decisions.A 64-year-old male presented with a 4.4 cm necrotic left adrenal mass discovered incidentally on imaging. His medical history included papillary thyroid carcinoma, with subsequent thyroidectomy and radioactive iodine ablation. Evaluations for hiccups revealed multiple lung nodules, hypertrophic cardiomyopathy, and anemia. Given the patient’s previous cancer history, elevated aldosterone/renin ratio, and mass size, our multidisciplinary tumor board decided to proceed with a left adrenalectomy. Post-surgical pathology confirmed a diagnosis of adrenal cavernous hemangioma.The occurrence of ambiguous adrenal mass with other pathologies, such as our patient’s papillary thyroid carcinoma, complicates the diagnostic and therapeutic landscape. As demonstrated in our case, opting for surgery remains a viable solution for adrenal cavernous hemangiomas, especially for masses greater than 4 cm. Interdisciplinary collaboration, exemplified by our tumor board’s decision-making process, is crucial for optimal management. This case underscores the need for a multifaceted approach when confronting adrenal masses with such diagnostic ambiguity. Adrenal cavernous hemangiomas are rare benign vascular tumors that present significant diagnostic challenges in urologic practice. Since first being recognized in the mid-1950s, adrenal cavernous hemangiomas have only been documented in approximately 70 case reports . DespiteWe report the case of a 64-year-old male who presented with a 4.4 cm necrotic left adrenal mass found incidentally on imaging. The patient has a history of papillary thyroid carcinoma and underwent partial thyroidectomy in May 2010, followed by complete thyroidectomy in July 2010 and radioactive iodine ablation in September 2010. Our patient has been on suppressive and substitutive treatment with thyroid hormone since his surgery in 2010. However, increased treatment doses were required in 2020 due to challenges in maintaining optimal thyroid hormone levels.In February 2022, the patient underwent a diaphragm fluoroscopy (fluoroscopic Sniff test) during a workup for intractable hiccups by his endocrinologist, which revealed multiple lung nodules. A subsequent computed tomography (CT) scan of the thorax in August 2022 revealed suspicious solid lung nodules, with the largest being 1.5 cm, along with a 4.4 cm left adrenal mass with peripheral enhancement and central heterogeneous hypoenhancement suggestive of necrosis. The differential diagnosis provided by the attending radiologists at the time of imaging included adrenal myelolipoma, metastatic disease from the patient’s previous thyroid cancer, or primary adrenocortical carcinoma. However, there was no discernable macroscopic fat within the mass, making the diagnosis of myelolipoma less likely. The adrenal mass was better visualized on CT Abdomen from the month prior . A routiOur patient’s blood pressure was well-controlled by anti-hypertensive medications  and did not display any stigmata of Cushing’s Syndrome or pheochromocytoma, later confirmed by biochemical testing. Notably, the patient was also anemic during his clinical course, found to be 7.5 gm/dl in November of 2022. Our patient’s anemia slightly improved to 8.5 gm/dl one week before his operation. Before surgery, his laboratory studies were notable for a high aldosterone/renin ratio of >117.4 ng/dl per ng/ml/hr (reference range 0.0–30.0), with aldosterone at 19.6 ng/dl (reference range 0.0–30.0) and low renin at <0.167 ng/ml/hr (reference range 0.167–5.380) in April 2023. Plasma metanephrines collected simultaneously were within normal limits , lessening the suspicion for pheochromocytoma.The differential diagnosis remained broad, with options between metastatic thyroid carcinoma, adrenal cortical carcinoma, and functional adenoma. Our institution’s multidisciplinary tumor board decided to proceed with a left robotic adrenalectomy and left retroperitoneal lymph node dissection in June 2023. Intraoperatively, no visceral injury was noted, and there was no evidence of metastatic disease. Moreover, there was no suspicious renal hilar or para-aortic lymphadenopathy. A branching renal vein and a single renal artery were noted. The left gonadal artery was spared, and the left adrenal gland was removed with the mass intact without capsule rupture and injury to the adjacent kidney, splenic hilum, or pancreas. Postoperatively, the patient was discharged after one day.The surgical specimen submitted as a left adrenal mass  was examThe histopathological differential diagnoses include other morphologic variants of hemangioma, a lymphangioma , angiosarcoma .Adrenal masses are relatively common, with a 5%–10% prevalence depending on the age and population studied . HoweverThe association between adrenal masses and other pathologies, as seen in our patient’s papillary thyroid carcinoma history and hypertrophic cardiomyopathy, may add complexities to diagnostic and therapeutic decisions. Although rare, four published cases have described the metastasis of papillary thyroid cancer to the adrenal glands . Thus, tPreoperatively, our patient’s hemoglobin was 8.5 gm/dl, indicating anemia during his care period. There have been two documented cases aside from our patient who presented with anemia alongside abdominal symptoms, potentially due to the sequestration of the blood in conglomerate vessels to the hemangioma . A thoroTumor boards are associated with changes in patient management and improved clinical outcomes, potentially attributable to clinicians’ opportunity to exchange knowledge to explore alternative treatment modalities while adhering to clinical guidelines . CoordinTumor boards can be beneficial in discussing workup for adrenal hemangiomas due to the breadth of the differential diagnosis. Biopsy presents unique challenges. Given the nature of these vascular tumors, biopsy carries the risk of causing adrenal hemorrhage. Additionally, some of these tumors are metabolically active in the case of pheochromocytomas and may be associated with a catecholamine surge with biopsy and must be pretreated with alpha-blockers. Moreover, while hemangiomas are benign tumors, the differential diagnosis includes several malignant tumors, which leaves observation as a potentially harmful option to the patient. Our case was complicated by radiographic findings, which included peripheral enhancement and central heterogeneous hypoenhancement suggestive of necrosis, suggesting that there was a possibility that this tumor was malignant. Despite these complexities, in cases where adrenal masses are >4 cm in size, such as the one presented in this case, adrenalectomy often emerges as the preferred solution . One optAdrenal cavernous hemangiomas are rare benign tumors that pose significant clinical and radiologic diagnostic challenges due to overlapping findings with malignancies. Our case report underscores the nuances in managing such adrenal masses, particularly when they coexist with other confounding conditions. As demonstrated through our tumor board’s decision for adrenalectomy, the role of interdisciplinary collaboration emphasizes the importance of a multifaceted approach in managing these complex cases.


Nontraumatic surgical emergencies constitute a major bulk of general surgical practice. Most of the cases seen fall under routine, but now and then, a surgeon or emergency physician is faced with an unusual diagnosis or a rarer presentation of a usual diagnosis. Sharing among peers their experiences with these outliers of practice helps spread knowledge and increases the experience pool. We share our experience of a 66-year-old female who presented to our emergency with upper abdominal pain of one-day duration. Diverticula are a fairly common entity encountered in a general surgical practice with the majority of the encounters being limited to the colon. The duodenum is widely recognized as a distant second with incidence in the general population estimated to be around 22% . As the A 66-year-old female who has a history of acute-onset upper abdomen pain for one day was brought to the emergency department. Associated with nausea and vomiting, the pain was radiating to the back. The vomitus was not blood-stained, and the pain had no aggravating or relieving factors. There was no history of diarrhea, fever, chest pain, or shortness of breath, but she had a past history of gastric bypass done three years ago. The patient was known to have type II diabetes mellitus, hypertension, thalassemia minor, and dyslipidemia. There was no family history of inflammatory bowel disease or malignancy. On examination, the patient was in acute discomfort due to severe pain with a pain scale of 10/10. The patient was afebrile. Her blood pressure was 121/63 mmHg, and her heart rate was 63/minute, regular. Cardiovascular and respiratory system examination was normal. On abdominal examination, the patient had epigastric and periumbilical tenderness with guarding. Bowel sounds were heard normally. Initial investigations revealed random blood glucose of 7.8 mmol/L. White blood cells were 11.6 K/uL , neutrophils 9.52 K/uL , hemoglobin 11 gm/dL , amylase 186 IU/L , lipase 325 U/L , and C-reactive protein <0.40 mg/L . Liver function tests, troponin I, and renal function tests all were reported as normal. In view of elevated pancreatic enzymes and acute abdomen, computed tomography (CT) of the abdomen with intravenous and oral contrast was done. CT revealed multiple gallstones and a 4.4×2.4 cm radiopaque structure lying in the retroperitoneum (Figure The patient was taken to the operating theater. Initially, a laparoscopy was performed, and free, bile-stained fluid was identified around the liver and Morrison’s pouch. Extensive retroperitoneal edema and peritoneal inflammation were encountered, and a perforated duodenal diverticulum was identified arising from the second and third part, of which a large 4-cm stone was located in the retropancreatic region with extensive sloughing of surrounding tissues Figure . InitialDuodenal diverticula together with small bowel diverticula, as described by Eggert et al.  in 1982,As far as the clinical impact of these diverticula is concerned, Shuck and Stallion  stated tApart from nonspecific symptoms, Schnueriger et al.  implicatStones in the duodenal diverticula are an even rarer occurrence. By itself, the perforation of duodenal diverticula has been reported less than 200 times in case reports according to Kimyaghalam et al.  in 2018.The array of intervention choices, once a perforation is identified, may range from simple closure to the need of performing pancreaticoduodenectomy. Recently, there have been reports in the literature where a nonsurgical approach comprising fasting, nutritional support, and drainage has been successful . HoweverThe routine use of advanced radiology in emergency departments has decreased the need for surgical interventions with uncertain diagnoses. However, during the course of one’s career, there might be instances where despite scans showing a positive finding, a degree of diagnostic uncertainty is present, leading to unexpected surgical findings and diagnosis. This case is shared as a similar unique instance where the final diagnosis was not established until the operative findings were noted. Rare instances like this will help if kept in mind so that if the surgeon does encounter unexpected “surprises” during an operation, he can utilize various surgical options and can operate with a good surgical plan to deal with any complex scenario.


To investigate the clinical features, diagnosis and treatment methods and prognosis of retroperitoneal Kaposiform hemangioendothelioma (R-KHE) in children.The clinical data of an infant with R-KHE was retrospectively analyzed. Literature on R-KHE in pediatrics were retrieved in databases including Wanfang, CNKI and PubMed as of April 2022.A 1 month and 6 days female infant with R-KHE was reported. After the diagnosis was confirmed by biopsy and pathological examination, the patient was treated by interventional embolization, and a combined therapy with glucocorticoid, vincristine, sirolimus and propranolol. The patient has been followed up for 1 year and 2 months, and is still alive with tumor. Through literature search, a total of 15 children, together with the case in our report, were included. The main manifestations were diversity among those patients. 14 cases have combined Kasabach-Merritt phenomenon (KMP). 6 cases accepted surgery plus drug therapy. 4 cases accepted only surgery, and 4 cases only accepted drug therapy. While drug therapy plus radiotherapy were employed to 1 case. Improvement was observed in 11 cases, with significantly reduced tumor and survival with tumor. Tumor disappeared completely in 2 cases. While 2 cases suffered death.R-KHE has diverse clinical presentations and non-specificity in symptoms and imaging examinations, and most cases accompanied with KMP. Methods for R-KHE treatment include surgical resection, interventional embolization and drug therapy. Close attention needs to be paid to the adverse reactions of the drug during the course of treatment. KMP i2.2.1.9/L, RBC 1.52 × 1012/L, Hb 53 g/L, PLT 5X109/L, and coagulation factors ATTP > 150 s, PT > 150 s, fibrinogen 0.5 g/L, FDP 62.6 mg/L, and D-dimer 12.5 ng/L. CT scan of abdomen on March 11, 2021 showed in The patient was a 1 month and 6 days female infant who was admitted to the hospital on March 6, 2021 with “abdominal distension for 20 days, skin petechiae and ecchymoses for 14 days and aggravation for 2 days”. The patient had progressive abdominal distension. Physical examination on admission revealed that the patient had pale complexion and lips, slightly swollen face, trunk and perineum, and scattered petechiae and ecchymoses all over the body, especially on the right waist. As to the auxiliary examinations, the findings of abdominal color Doppler ultrasound on March 7, 2021 included: (1) slightly enlarged liver, but no obvious abnormality in its internal structure; (2) immobile biliary sludge in the gallbladder; and (3) a medium-large amount of peritoneal effusion with cloudy components. Blood test at admission showed: WBC 8.48X102.2.The scope of published literature research was databases including Wanfang, CNKI, and PubMed by March 2022. Key words included Kaposiform hemangioendothelioma (KHE), retroperitoneal Kaposiform hemangioendothelioma (R-KHE), and KMP. After excluding adult cases and those children's cases without full-text articles, a total of 14 cases of R-KHE were retrieved from Chinese and English literatures –16.3.3.1.As informed by the follow-up phone calls for 1 year and 2 months, the patient was in good growth and development. The retroperitoneal tumor was significantly smaller as shown by subsequent CT scan, and follow-up tests indicated normal platelet and coagulation functions.3.2.Together with the case in this report, there are 15 cases of R-KHE in children, as shown in 4.Kaposiform hemangioendothelioma (KHE) is an aggressive tumor that resembles Kaposi's sarcoma (KS) in histology, and most KHE cases manifested in infancy and childhood . First rThe tumor in our case was located in the retroperitoneum, with severe thrombocytopenia, severe anemia, and coagulation dysfunction when she was admitted to the hospital. Initially we considered it a bleeding disorder caused by some internal disease: immune thrombocytopenia (ITP) or thrombotic thrombocytopenia (TTP)? The patient received glucocorticoid therapy, high-dose gamma globulin pulse therapy, and plasma exchange therapy, but the symptoms showed no improvement. A multidisciplinary consultation was organized, which recommended exploratory laparotomy after infusion of platelets and improvement of coagulation. The subsequent pathological biopsy suggested KHE. Based on this definitive diagnosis, oral administration of sirolimus and dexamethasone was prescribed for the patient.The incidence of R-KHE is low, and most of the reports are single case study. Both the clinical presentations and auxiliary examinations are non-specific. The diagnosis is mostly based on pathological biopsy, and there are no standard treatment protocols. Most R-KHE in previous reports occurred in the pancreas, and the common clinical presentations include abdominal pain and jaundice. The lesion of our case was in the kidney, with abdominal distension and skin petechiae as the main manifestations. The common auxiliary examinations for R-KHE include abdominal contrast-enhanced CT scan, contrast-enhanced MRI , and bioThere are several options for the treatment of KHE, including drug therapy, surgery and arterial embolization. In clinical practice, a combined therapy is often preferred due to the limited efficacy of a single regimen . Most caDespite the multiple drug options for KMP treatment, the reported efficacy varies greatly. Main drugs used in clinical practice include VCR, steroids, sirolimus and propranolol . The ExpTogether with the cases in the literature and our study, we conclude the following revelations: (1) R-KHE occurs in very young patients, mostly in infants of 1 to 4 months old, and R-KHE is often accompanied with KMP; (2) R-KHE shall be distinguished from solid retroperitoneal tumors and hematological tumors, such as idiopathic thrombocytopenic purpura; (3) the clinical presentations of R-KHE are diverse, mainly including abdominal distension, abdominal mass, jaundice, skin petechiae and ecchymoses. The symptoms are nonspecific; (4) Imaging manifestations of R-KHE: CT scan without contrast showed mass or diffuse low-density shadows in the retroperitoneum, and the lesions were obviously enhanced when with contrast, and some were accompanied by peritoneal effusion. Contrast-enhanced MRI may also be used to assist diagnosis; (5) for patients with above symptoms and imaging manifestations, if with accompanied KMP and the condition is critical, surgical biopsy can be performed after improvement of coagulation and infusion of platelets. If biopsy is not feasible due to the condition, clinical judgement could be made based on previous diagnosis and treatment experience, and diagnostic treatment with drugs could be performed; (6) if the effect of single drug therapy is unsatisfactory, a combination therapy could be considered.5.The clinical presentations of R-KHE are diverse and non-specific, most of which are associated with KMP, with rapid onset, rapid progression, and high mortality. Therefore, we should be wary of this disease in children with severe thrombocytopenia, anemia, severe coagulation dysfunction combined with intra-abdominal hemorrhage. Intervention-guided needle biopsy can be performed, It can also be treated with surgery if the condition permits. But, if the patient is in a critical condition and cannot tolerate surgery, a comprehensive judgment shall be made with combined consideration of the medical history, and diagnostic treatment with drugs could be performed. At present, sirolimus shows good outcomes in KHE treatment, but the plasmatic concentration should be monitored during medication, and attention should be paid to the side effects. If the treatment effect is not good, a combination with other drugs should be considered.