Aneurysms of the gastroepiploic arteries are seen only rarely. They are usually diagnosed during autopsy or laparotomy in patients with hemodynamic instability. Although the operation to treat this condition is relatively easy, delay in making the diagnosis affects the course of the disease. A 57-year-old woman was admitted to the emergency department with abdominal pain and unconsciousness. A computed tomography scan showed extravasation of contrast agent at the head-corpus junction of the pancreas, and the patient underwent exploratory laparotomy under general anesthesia. During laparotomy, aneurysmatic rupture of the right gastroepiploic artery was detected. Control over bleeding was achieved by ligating the right gastroepiploic artery at its origin. The aneurysm was also resected and sent for pathological examination. Especially in cases of unidentified shock, splanchnic artery aneurysms should be kept in mind. Moreover, in the light of the data in the literature, the possibility of death should be taken into account seriously and, if feasible, prophylactic aneurysmectomy should be performed. Aneurysms of the gastroepiploic arteries are seen only rarely.A 57-year-old woman was admitted to the emergency department with abdominal pain and unconsciousness. During laparotomy, aneurysmatic rupture of the right gastroepiploic artery was detected. In this study, the diagnosis and treatment of spontaneous right gastroepiploic artery rupture was discussed in the light of the literature .A 57-year-old female patient from whom written informed consent was obtained for publication of this manuscript was admitted to the emergency department with a history of abdominal pain of duration two days and slight confusion for the last six hours.According to the patient’s story, she had a history of atherosclerotic heart disease and hypertension. She was using angiotensin-converting enzyme (ACE) inhibitor, beta blocker and acetylsalicylic acid. She had a history of appendectomy, abdominal hysterectomy, laparoscopic cholecystectomy and incisional hernia operation.On physical examination, the patient was confused but hemodynamically stable. On abdominal examination, there was diffuse tenderness and involuntary guarding. A rectal examination was unremarkable.3/µl, serum hemoglobin concentration 7.2 g/dl, platelet count 342 x 103/µl, hematocrit level 25.8%, creatinine level 4.2 mg/dl and blood urea nitrogen 50 mg/dl. Coagulation tests and other biochemical parameters were normal.In the blood panel analysis, the white blood cell count was 20.6 x 10Following examination, resuscitation was started using crystalloid solutions and blood components, and abdominal ultrasound was performed. There was perihepatic, perisplenic and right paracolic free fluid. A computed tomography (CT) scan of the abdomen using intravenous contrast was then performed to search for possible sources of bleeding. In the CT scan, extravasation of contrast agent at the head-corpus junction of the pancreas was observed.The patient underwent exploratory laparotomy under general anesthesia  and 2. TThe distal part of the stomach was transected using a linear stapler for better exposure. This revealed a ruptured right gastroepiploic artery aneurysm and retroperitoneal hematoma was also seen. Control of bleeding was achieved by ligating the right gastroepiploic artery at its origin. The aneurysm was also resected and sent for pathological examination. The operation was terminated after retrocolic gastrojejunostomy.The CT scan was repeated to confirm that complete resection of the aneurysm and control of concurrent vascular anomalies had been achieved, before discharge . The pat,Idiopathic spontaneous intraperitoneal hemorrhage, which was first reported by Barber in 1909, was later referred to as abdominal apoplexy by Green and Powers.The precise mechanism is not fully known. However, weak tunica media and increased intravenous pressure are thought to be possible causes of rupture. Predisposing factors in the pathogenesis include arteriosclerosis, infection, medial necrosis, trauma, pregnancy and portal hypertension. To confirm the diagnosis, pathological evaluation of the sacrificed specimen is required. In our patient, atherosclerosis was not seen but hypertension was present. Pathological examination of the lesion confirmed that it was a true aneurysm.The symptoms of splanchnic aneurysm are usually nonspecific. There may be vague abdominal pain before the rupture. After the rupture, noticeable abdominal pain accompanied by hypotension is present. Shimada reported that the symptoms of ruptured and un-ruptured aneurysms differed.A ruptured aneurysm is usually diagnosed through laparotomy in a setting of hemodynamic instability. In less urgent cases, abdominal ultrasound examination, CT scans and angiography are helpful tools for making the correct diagnosis. According to the literature, 81% of gastroepiploic artery aneurysms were diagnosed after rupture.CT scans are the most important imaging test in the emergency setting. With CT scans, intravenous contrast is highly recommended and its effectiveness for locating active bleeding has been proven.In cases of hemorrhagic shock, the mainstays of treatment are volume resuscitation (either with fluid or with blood components) and control over life-threatening bleeding. In this context, for better exposure and rapid control of bleeding, the distal stomach was transected using a linear cutter stapler, at the expense of losing intactness of the upper gastrointestinal system. This made it easy to reach the pancreatic head and ligate the bleeding ruptured aneurysm. This procedure was undertaken despite lack of mention of this technique in the literature.Surgical excision, which can be performed through conventional surgery or through laparoscopic surgery, provides complete disease control.Regarding the follow-up of these patients, there is no consensus in the literature, given that most of the data are from case reports. A control radiological examination can be helpful after resection. If embolization is performed in elderly high-risk patients, observation is recommended.Especially in cases with unidentified shock, ruptures of splanchnic artery aneurysms should be kept in mind. Moreover, in the light of the data in the literature, the possibility of mortality should be taken into account seriously and, if feasible, prophylactic aneurysmectomy should be performed.


Hemangioblastoma is considered a benign neoplasm characterized by abnormal vasculature and stromal cells; several pathophysiological mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. Fibromuscular dysplasia is characterized by hyperplasia or thinning of the smooth muscle, elastic fibre destruction, fibrous tissue proliferation, and arterial wall disorganization. We present a cerebellar hemangioblastoma case not associated with Von Hippel Lindau syndrome. Histologically we evidenced big vessels with anomalies of the vascular walls corresponding to fibromuscular dysplasia, and those changes have not been described in these types of tumors. In this light, rare findings could be called vascular malformations or degenerative vascular changes, fibromuscular dysplasia or vascular anomalies. Arterio-venous malformation and hemangioblastoma pathology are rarely presented together. Notwithstanding, we could say that it is a stromal stem cell tumor in a varied stage of differentiation. Hemangioblastoma (HB) is characterized by stromal neoplastic cells and abundant blood vessels. It is an uncommon and highly vascular tumor usually observed in adults that are located in the brain stem, cerebellum, spinal cord and retina, and corresponds histologically to grade 1 neoplasms [A 39-year-old female with a familiar history of unspecified brain tumor was evaluated with a seven-month history of progressive weakness, difficulty in swallowing and staggering gait. On physical examination, gait lateralization, an acute visual deficit of the right eye, left nystagmus, left dysmetria and dysdiadokokinesia were noted. An MRI showed a hypointense on T1 weighted image (T1W) images, and a hyperintense on T2 weighted image (T2W), a mass lesion, occupying the left cerebellar hemisphere associated with a cystic component and obstructive hydrocephalus suggesting a cerebellar HB  are not clear. In the case of hemangioblastoma being a highly vascularized tumor, it is possible to identify blood vessels of greater calibre with irregular and thickened walls at the expense of proliferation of the intimal tunic, myxoid degeneration and secondary loss of the internal elastic lamina. As long as the biopsy corresponds to a peripheral area of the lesion associated with the mural nodule, the lesion could be differentiated from its initial angioglioma description (which corresponds to glioma as a low-grade tumor) with a prominent vascular component that resembles cavernous angioma, arteriovenous malformation or hemangioblastoma. In 1914, Councillmann et al.  coined tAs of today, it is difficult to conclude the coexistence of AVM and HB within the same pathology. Hemangioblastoma shows rare vascular changes, as vessels with disorganized, thickened walls and abnormal proliferation. Consideration for a differential vascular diagnosis suggests hemangioma, fibromuscular dysplasia or arterio-venous malformation. The present case would be the first one reported of hemangioblastoma with histologically dysplastic changes reported. Further research is needed to elucidate the rare intermixture origin of these lesions.


Clonal evolution in acute leukemias is one of the most important factors that leads to therapeutic failure and disease relapse. Delay in therapeutic intervention is one of the reasons that leads toward clonal evolution. In this report, we present a case of acute lymphoblastic leukemia in which therapeutic delay resulted in clonal evolution that was detected by conventional karyotyping and was responsible for non‐responsiveness of the disease to conventional chemotherapy. A 17‐year‐old boy presented with generalized body aches, rapidly progressive pallor and lethargy. Bone marrow analysis was consistent with the diagnosis of B‐cell ALL. Karyotypic analysis revealed 46, XY male karyotype. The patient left the hospital due to financial reasons and after 40 days came back to the hospital. Repeated bone marrow analysis including cytogenetic studies revealed presence of three different clones of blast cells: one clone showed 46, XY with del(9p) and t , second clone showed 46, XY with del(7q) and del(9p), and the third clone showed 46, XY normal karyotype. The patient did not respond to chemotherapy and died within 1 week of induction chemotherapy (HyperCVAD‐A). Timely diagnosis and institution of chemotherapy in acute leukemias patients is the key to prevent clonal evolution and thus resistance of the disease to therapeutic interventions. ALLLymphoblastic leukemiaALTAlanine transaminaseAPTTActivated partial thromboplastin timeASTAspartate transaminaseBUNBlood urea nitrogenHCTHematocritHyperCVAD‐AHigh‐dose cyclophosphamide‐based chemotherapeutic regime A containing cyclophosphamide, vincristine, adriamycin, and danorubicinNGSNext‐generation sequencingPAX‐5Paired box‐5 immunostainPTProthrombin time1Acute lymphoblastic leukemia (ALL) is the clonal proliferation of immature lymphoid precursors in the bone marrow and/or peripheral blood . ALL hasOver the past half century, there has been significant advances in the field of molecular biology that has led to better understanding of the pathophysiology of leukemias, including acute lymphoblastic leukemia . Where iIn spite of all the current modalities that are utilized for both diagnostic and prognostic purposes, a significant number of patients do not achieve the therapeutic targets . SpontanHere we present an interesting case of ALL that underwent clonal evolution due to delay in institution of therapy, ultimately resulting in resistance of the disease to chemotherapeutic interventions.2A 17‐year‐old boy presented with generalized body aches, rapidly progressive pallor and lethargy, that had progressed over a duration of 4 weeks. Initial investigation demonstrated moderate anemia with moderate thrombocytopenia and presence of more than 65% blast cells in peripheral blood, while liver and renal profiles were unremarkable, as shown in Table 3Pathogenesis of acute leukemia involves acquisition of genetic abnormalities either in the form of chromosomal aberrations or development of mutations, which in turn results in either functional loss or functional gain in the downstream signaling pathways at molecular level , 8. The New clone of cells can arise from the existing clone of leukemic cells, either spontaneously or during the institution of chemotherapy . EvolvedRecent evidence suggests that there are continuous intercellular communications between normal cells as well as cells in a neoplastic growth , 12. It Recent trails that utilized the next‐generation sequencing (NGS) studies for monitoring the clonal markers of acute leukemias identified that clonal evolution played significant role not only in clonal evolution but also in relapse of the disease and chemotherapeutic resistance . It is pIt is clear that the early diagnosis, appropriate institution of diagnostic as well as prognostic modalities, early institution of chemotherapy, and if possible, implementation of targeted therapy are all essential steps to achieve complete remission. With this case, we want to emphasize upon the fact that in Afghanistan, there is a dire need not only for establishment of standard oncology institutions but also for health education of the public about various neoplastic processes and the importance of timely therapeutic intervention. Further multinational studies are required to elaborate about the role of intercellular pathways in clonal evolution and disease relapse. This would potentially allow for identification of novel therapeutic targets.4Our patient developed novel complex chromosomal abnormalities within the first month of diagnosis due to delay in chemotherapy. In our case report, we attempt to elaborate upon the fact that timely diagnosis and institution of therapy in cases of acute leukemia are indispensable to prevent emergence of therapy‐resistant leukemic clones.The authors declare they have no conflicts of interest.AMH, SRN, SHN, NJN, and RS conceived the idea. AMH, SRN, SHN, and ASZ were the major contributor to the writing of the manuscript. AHS, ASZ, AHS, and SS collected the laboratory data via integrated laboratory management system (ILMS). SS, AHS, INM, and MA performed the karyotypic analysis. AMH and SRN diagnosed the case. SHN provided the clinical information of the patient. AHS and SS performed cytogenetic studies. SRN, AMH, ASI, NJN, JAG and HAM were the major contributors for critically revising the manuscript for important intellectual content. JAG, NJN, SRN, and AMH have given expert opinion and final approval of the version to be published. All authors read and approved the final manuscript.Written informed consent was obtained from the patient's legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor‐in‐Chief of this journal.


An uncommon type of urinary calculus, Jackstone was named after its distinct resemblance to the children’s game “Jacks.” It typically involves the bladder and, to a lesser extent, the upper urinary tract.Herein, we report a case of Jackstone vesical calculus in a 75-year-old male undergoing elective open prostate surgery for benign prostate hyperplasia refractory to medical treatment. Preoperative clinical examination revealed intermittent gross hematuria and symptoms suggestive of bladder outlet obstruction, while radiological investigation confirmed the presence of a solitary star-shaped spike-like bladder stone along with an overly enlarged prostate. Following open simple prostatectomy and concomitant intact stone removal, our patient made an uneventful postoperative recovery.This case highlights an infrequent subtype of bladder lithiasis and further expands upon the importance of promptly treating the underlying cause once this rare entity is detected. A comprehensive review of the literature on Jackstone calculi is further presented. The complex pathophysiological mechanisms involved in urinary stone formation never cease to amaze urologists. Despite being previously described, Jackstone calculi remain a rare and underreported form of urolithiasis . The terA 75-year-old Caucasian male attending the outpatient prostate clinic at our hospital underwent an elective prostate surgery due to BPH refractory to combination medical treatment. He reported a history of transient episodes of painless gross hematuria that were self-limited. He also complained of obstructive lower urinary tract symptoms (LUTS) aggravation during the last ten months.Fig.1, the CT scan revealed a solitary star-shaped, spiculated Jackstone inside the bladder with no other pathological findings aside from an overly enlarged prostate (130 ml).He had no previous significant surgical history and denied any history of smoking. No remarkable medical or urological history was present at that time. Due to LUTS investigation months before the scheduled surgery, ultrasonography, computed tomography (CT), and cystoscopy were performed. As shown in We completed the standard preoperative workup, and laboratory examination was within normal limits. In addition, kidney-ureters-bladder (KUB) radiography identified the already confirmed CT finding demonstrating a single radiopaque pelvic shadow with sharp and spiculated margins.. Notably, the stone was black and unusually light, exhibiting a mamillated appearance.After obtaining informed consent and thoroughly analyzing the various treatment options based on the stone and prostate size, the patient elected to proceed with an open transvesical prostatectomy. A single 3.5×3.5×3 cm bladder calculus was concurrently removed with macroscopic features suggestive of the Jackstone calculus (JC) There were no immediate perior postoperative complications, and the patient was finally discharged home on postoperative day 7. Apart from the uneventful recovery, no other abnormality was detected during the 6-month follow-up. More specifically, LUTS showed complete remission, while the quality of life significantly improved.Urolithiasis constitutes a major health problem with an estimated prevalence between 1 and 20% globally . BladderThe pathogenesis is considered multifactorial, with bladder outlet obstruction secondary to benign prostate hyperplasia being a plausible mechanism for stone formation in the vast majority of cases ,10. Outf [Jackstone calculi comprise an infrequent form of vesical lithiasis, morphologically characterized by an atypical shape of elongated protuberances resembling children’s “Jacks” toy  ,11. Thei . Althoug ,13. Furt ,7. Latel .Table 1. Overall, patients experiencing this pathology had a mean age of 73 (range: 55–84), and the majority (78.6%) was found to have a single JC. Concurrent bladder stone removal and prostate surgery were the treatment of choice in approximately half of the patients (42.9%), while open cystolithotomy was solely attempted in a roughly equal percentage (35.7%).A comprehensive literature review, limited to English-only studies, was performed at the end of August 2021 within the Medline via PubMed database using the keyword search term “jackstone calculus.” No specific time boundaries were applied, while animal studies or studies with an irrelevant topic and insufficient data were excluded from further analysis. Initially, 19 articles were identified, 8 of which were excluded based on our search criteria. The remaining 11 studies included in our review were considered eligible for full-text assessment. Additional articles were retrieved by hand searching from reference lists of the eligible studies. A synopsis of studies reporting on Jackstone bladder calculi is presented in Recently, Muneeb et al. reported one of the largest Jackstones to date, measuring 6x6cm, in a 55-year-old male presenting with worsening voiding LUTS. Interestingly, the patient was solely managed with open cystolithotomy and opted for postoperative BPH treatment with tamsulosin . SimilarIn 2018, Heathcote et al. demonstrated a successful simultaneous transurethral resection of the prostate (TURP) and cystolitholapaxy in a 78-year-old male presenting to the emergency department with a 2-week intermittent gross hematuria, bladder fullness, urgency, and suprapubic tenderness . LikewisNot long ago, Carneiro et al. mentioned a 2 cm JC incidental detection in an elder male during a preoperative workup for colorectal cancer . IntriguContrary to popular belief, JC can also be found in the kidney, with such an exceedingly rare case being described in a 63-year-old woman presenting with intermittent visible hematuria of 4-month duration. The stone, measuring 2.4×2.3 cm in size, was successfully fragmented with laser retrograde intrarenal surgery . UnfortuUltrasound imaging is highly endorsed by the European Association of Urology (EAU) as the first-line diagnostic modality for bladder lithiasis . Indeed,The optimal management of bladder lithiasis remains a long-running dispute as diverse therapeutic strategies virtually exist ,10. EquiModern surgical advancements have introduced minimally invasive approaches for vesical calculi removal, thus expanding the therapeutic scope ,21. AlthLastly, the long-standing issue of whether prostate surgery should be an adjunctive procedure to stone surgical removal continues to demand answers . CurrentΒladder lithiasis constitutes a medical problem afflicting 5% of adult males in the Western world. Clinicians should be aware of Jackstone urolithiasis, that once detected, necessitates a patient-tailored approach. Diverse therapeutic options are readily available while eliminating the underlying cause remains the cornerstone of treatment. Our case adds to the growing body of evidence, further providing valuable insights into a rare urological entity. The authors hope that this case report will lead to prompt diagnosis and proper treatment in any such case encountered.


In clinical practice, foreign bodies (FBs) in the digestive tract are more common in children, but intrahepatic FBs are rare, especially those that can cause infection, bleeding, bile leakage, and other complications. However, there is no consensus on its diagnosis and treatment due to the lack of large-scale cohort studies.Case 1 is a 4-years 8-months-old girl, who at the age of 10 months, showed an X-ray finding of a striped FB in her liver, with no symptoms. However, the patient’s parents refused surgery. After nearly 4 years of active surveillance, the patient visited our hospital for surgery. Case 2, a 2-year-old male, reported a sewing needle that completely pierced into the right upper abdomen due to an accidental fall that took place half-a-day before admission. He only had right upper abdominal pain. CT showed a striped FB in the liver.FB in the liver (sewing needle).Both the patients were injected with human tetanus immunoglobulin and underwent surgical removal.Both patients recovered smoothly and had no complications during follow-up.Active surveillance might be considered for cases with no symptoms or complications and no displacement of the FB, but surgery should be the first choice. If the patient’s condition is complicated, it is recommended to use ultrasound or X-ray to help decision-making during the operation. Additionally, tetanus, child safety, and family education are important supportive measures. In addition, pediatric cases with a sewing needle in the liver are very rare and differ from adult cases, such as the route of the needle into the liver. Only 10 pediatric cases with needles in the liver have been reported in PubMed, and there is no consensus regarding diagnosis and treatment. Children are more vulnerable to school violence, domestic abuse, and neglect, and more attention should be paid to the prevention of these accidents. Here, we report our experience and review the literature to provide a reference for the diagnosis and treatment of pediatric patients with a sewing needle in the liver.Patients with foreign bodies (FBs) in the liver are very rare in clinical practice, and most of them are children, elderly individuals, psychiatric patients, and alcoholics.This study was reviewed and approved by the Institutional Review Board of the Children’s Hospital of Chongqing Medical University. Written informed consent was obtained from the legal guardians of the patients.A 4-year- 8-month-old girl-who at the age of 10 months, showed an X-ray finding of a striped high-density shadow in the hepatic region, but she had no symptoms. Interestingly, there was no FB on the radiograph 2 months prior, and her parents denied a history of sewing needle swallowing or trauma. The patient’s parents refused surgical removal because the child was too young. Therefore, medical treatment was limited to injection of human tetanus immunoglobulin. During the active surveillance period, the child regularly underwent radiography or CT, and no FB displacement or symptoms were found. When the patient visited our hospital at the age of 4 years and 2 months, her X-ray  from the right abdominal wall to the right lobe of the liver. The laboratory test results were normal. The patient underwent emergency surgery. We found that the needle pierced the hepatic segment VI, and the tail was left in the abdominal wall. The needle (rust-free) was completely removed, and the liver wound was sutured. There was no bile leakage. He was given human tetanus immunoglobulin immediately after surgery. The patient recovered satisfactorily, and was discharged smoothly 2 days after the operation. No complications occurred during the 11 years of follow-up. There are 3 main routes to the liver for FBs: (1) penetrating into the liver through the abdominal wall (skin), (2) migration from the gastrointestinal tract (GIT), and (3) via the bloodstream. However, to our knowledge, no case of a needle in the liver via the bloodstream has been reported. Studies have shown that the most common perforation sites in patients with FBs in the liver are the stomach, duodenum, and colon; however, gastrointestinal perforation caused by swallowing FBs is very rare, with an incidence of <1%.,6 We conducted a literature search in PubMed about pediatric patients with the sewing needle in the liver, and only 12 cases were reported, including our cases , toothpicks (27.3%), chicken bones 12.5%), and needles (9.1%).2.5%, and Ultrasound is often the first choice for pediatric patients because of its low price, lack of radiation exposure, and ease of access in most hospitals, including primary health care institutions. However, CT is more useful for the diagnosis and decision of the treatment plan because of its higher resolution, better visibility, and understandability, which are helpful for localization. Furthermore, contrast-enhanced CT has important value for the determination of treatment plans because it can provide more information on the relationship of the FB with the surrounding organs and vessels.Children have a poor ability of expression, especially infants, so pediatric patients may express their physical discomfort only by crying. Moreover, needle wounds are often subtle, and most patients have only short-term nonspecific symptoms or even no symptoms Table . TherefoSenol A et al reported that a patient had been asymptomatic for up to 6 years, our case had no symptoms for nearly 4 years, and Crankson SJ reported that 1 pediatric patient who received noninvasive treatment had no symptoms for 3 years , and all of them had good outcomes , which suggests that most patients had high surgical compliance and that the surgery was safe and effective (Table –19 Notably, Stone RK et al reported a pediatric case with a needle in the liver due to suspected abuse, and Saitua F et al reported a case with a needle in the liver that was suspected to be caused by the caregiver’s negligence. Therefore, to prevent the occurrence of intrahepatic needles, the most important measures may be to take full care of children and provide effective education for caregivers and children, in which primary health care institutions or communities may play an important role because of their advantages of coverage and quantity.In addition, once needle injuries have occurred, tetanus should always be considered, especially in children with no, incomplete, or unknown history of immunization and unclean or contaminated wounds.In summary, pediatric cases involving needles in the liver are rare. A history of swallowing and trauma cannot be ignored, and diagnosis and localization depend on imaging, especially CT. In most cases, surgical removal is the first choice of treatment. Moreover, intraoperative localization and confirmation of whether the FB has been removed completely are essential, and whether tetanus treatment should be administered should also be considered. It should not be ignored that caregivers’ care for children is of great value for prevention and timely medical treatment.We acknowledge the parents of the patients for allowing us to learn and share their stories.Conceptualization: Hui-wu Xing.Data curation: Hui-wu Xing.Formal analysis: Hui-wu Xing.Investigation: Chen-yu Yang.Methodology: Hui-wu Xing.Project administration: Bing-qian Tan.Resources: Chen-yu Yang.Software: Hui-wu Xing.Supervision: Ming-man Zhang.Validation: Chen-yu Yang.Visualization: Hui-wu Xing.Writing– original draft: Hui-wu Xing.Writing– review & editing: Ming-man Zhang.


Inflammatory myofibroblastic tumour (IMT) of the common bile duct (CBD) is an extremely rare low-grade malignancy with various biological behaviours and a lack of specific clinical and histopathological features. Preoperative and intraoperative diagnosis are challenging, and a diagnostic delay may increase surgical complexity.We present the case of a 34-year-old male with no relevant medical history who presented with jaundice of 20 days of evolution. Histology and immunohistochemistry confirmed the diagnosis of an IMT with anaplastic lymphoma kinase (ALK)-1 expression. In addition, a review of the relevant literature revealed 13 published reports of biliary IMTs. The clinical history and histopathological features in these 13 cases were compared with those in our case to provide a comprehensive overview of the clinical manifestations and histopathological features of the disease.IMT of the CBD is an extremely rare low-grade malignancy that mainly occurs in middle-aged female patients. The main clinical manifestation is monosymptomatic jaundice. The reported tumours originated in the middle and lower segments of the CBD, with an average size of approximately 3.5 cm × 3.0 cm and tumour cells expressing smooth muscle actin (SMA), vimentin and ALK. Abnormal ALK expression and ALK gene rearrangement represent potential histopathological and differential diagnoses. A clear diagnosis by preoperative biopsy and intraoperative frozen section examination is critical and can significantly reduce surgical trauma. The prognosis is good, and very few patients experience recurrence or distant metastasis. Inflammatory myofibroblastic tumours have been reported in various organs, such as the liver, intestine, spleen, kidneys, bladder, lungs, peritoneum and heart, and are most common in the lungs , 2. HoweWe present the case of a 34-year-old male with no relevant medical history who presented with jaundice of 20 days of evolution. His examination revealed scleral and whole-body skin icterus. There was no abdominal tenderness, rebound tenderness, and the Murphy test was negative, with no palpable mass. There was no evidence of any other organomegaly or lymphadenopathy. His test results on admission were as follows: total bilirubin, 5.17mg/dl; direct bilirubin, 2.49 mg/dl; alkaline phosphatase, 152 U/L; and CA199, 58.5 U/ml. Viral serological tests for hepatitis A, B and C and HIV were negative. Computed tomography (CT) revealed the following: soft tissue nodules in the CBD with clear borders, inhomogeneous morphology, heterogeneous density, equal or slightly low-density changes and nodules bulging into the lumen of the CBD. In the venous phase, there was significant heterogeneous enhancement, which was more obvious than that in the arterial phase and showed gradual strengthening changes  Fig. a, ALK Fb and SMAIMT was once regarded as a benign reactive tumour-like hyperplasia with spindle fibroblasts and myofibroblasts undergoing hyperplasia, accompanied by fibroblast infiltration as the main pathological feature . TumoursAnalysis of tumour pathological characteristics revealed the following: the reported tumours originated in the middle and lower segments of the CBD with an average size of approximately 3.5 cm × 3.0 cm, and the largest tumour, reported by Verma et al., was approximately 10 cm × 6 cm in size . On histDifferential diagnosis of IMT of the CBD includes cholangiocarcinoma, gastrointestinal stromal tumour (GIST), leiomyoma and fibromatosis . (1) ExtAll patients were subsequently diagnosed with infiltrating biliary or peribiliary pseudotumours on laparotomy. No patient was, in fact, thought to have a nonneoplastic lesion before biliary diversion. Extrahepatic biliary resection was performed in 14 patients. However, the final pathological diagnosis was IMT. IMTs of CBD are rare lesions that are often mistaken for aggressive malignancies, and although their appearance is nonspecific, a diagnosis may be made by imaging-directed core biopsy. If biopsy is performed preoperatively or intraoperatively, the trauma of surgery can be reduced.Compared to GIST, leiomyoma and fibromatosis, IMT is considered a less malignant mesenchymal tumour occurring predominantly in children or adolescents or, in the case of IMT of the CBD, in middle-aged female patients. IMT of the CBD is characterized by monosymptomatic jaundice and a tumour in the middle or lower segment of the CBD, with an average size of approximately 3.5 cm × 3.0 cm and diverse histological features. The notable immunohistochemical characteristics of these tumours are SMA expression and partial expression of desmin, vimentin and ALK. Surgical excision is currently considered the treatment of choice for IMT. However, there is some evidence of the effectiveness of corticosteroid therapy. Although IMT of the CBD might be considered in the differential diagnosis of an obstructing extrahepatic biliary lesion, there are no distinctive clinical or imaging features that allow it to be differentiated from cholangiocarcinoma before laparotomy. Awareness of this rare but benign entity, however, might prompt a more limited operative approach when intraoperative resection margins of palpable disease are repeatedly negative for tumour cells. A clear diagnosis by preoperative biopsy and intraoperative frozen section examination is critical and can significantly reduce surgical trauma. The prognosis is good, and very few patients experience recurrence or distant metastasis.


Leiomyomas are a common gynaecological finding affecting 20-30% of women over the age of 35, with prevalence decreasing following menopause. Around 25% of women present clinically with a leiomyoma, which are most commonly found within the uterus. Rarer extra-uterine locations include the broad ligament, cervix, and vagina. We present a rare case of an extra-uterine leiomyoma located in the perineum of a 59-year-old female. Our case highlights the importance for extra-uterine leiomyoma to be considered as a differential diagnosis in patients presenting with a pelvic mass following hysterectomy. Leiomyomas are the most common tumour type found within the female pelvis . Around An asymptomatic 59-year-old female was referred to the colorectal two-week wait clinic from the community. During gynaecological examination at her general practice, a lump was noted in the peri-anal region. She did not report any weight loss, change in bowel habit, or per rectal bleeding. On examination in clinic, the abdomen was soft and non-tender, on per rectal examination an external mass tracking towards the vagina was palpated. She had a previous surgical history of a total abdominal hysterectomy with left-sided ovarian conservation 15 years previously for multiple uterine leiomyomas. After hysterectomy she had been prescribed oestrogen-only hormone replacement therapy. Differential diagnoses included sarcoma and leiomyoma.Initial biopsy of the mass showed appearances in keeping with a benign leiomyoma. Magnetic resonance imaging revealed a right paramedian perineal mass measuring 4.5 cm × 8.2 cm × 4.2 cm. It bordered the rectum posteriorly, the vagina superior-anteriorly, and the right perineal subcutaneous fat inferiorly. The patient was examined under anaesthesia and commenced on letrozole to reduce the size of the mass pre-operatively. Following multi-disciplinary team discussion, the leiomyoma was excised via a perineal approach Figures , 2. FollLeiomyomas are a common gynaecological finding affecting 20-30% of women over the age of 35, with prevalence decreasing following menopause ,3. ArounUterine leiomyomas are benign monoclonal tumours of the myometrium consisting of smooth muscle cells, fibroblasts, and extracellular matrix ,5. They Common presenting symptoms of uterine leiomyomas include heavy menstrual bleeding, dysmenorrhoea, and pelvic discomfort . They maDespite often being found incidentally, uterine leiomyomas can be investigated using ultrasonography. Ultrasound provides a fast method in detailing the size, site, and number of uterine fibroids. Magnetic resonance imaging (MRI) is utilized to evaluate the leiomyoma in relation to the rest of the pelvis, including relations to neurovascular structures and nearby organs . The useFollowing symptomatic treatment, uterine artery embolization, myomectomy, and hysterectomy are used in the treatment of symptomatic leiomyoma. The patient in our case report had previously undergone a hysterectomy for this indication. Recurrence of leiomyoma, as well as benign metastasizing leiomyoma and leiomyosarcoma after hysterectomy, have been reported in the literature ,7.We present a case of perineal leiomyoma after hysterectomy. Furthermore, our case highlights the importance for extra-uterine leiomyoma to be considered as a differential diagnosis in patients presenting with a pelvic mass after hysterectomy.


In the case of a 62‐year‐old man with biopsy confirmed primary diffuse large B‐cell lymphoma of the CNS treated with methotrexate, cytarabine, thiotepa and rituximab (MATRix) chemoimmunotherapy and busulphan/thiotepa autograft, follow‐up contrast enhanced magnetic resonance imaging (CE‐MRI) demonstrated ongoing peripheral enhancement at the site of original lesion (middle left). Here we show that treatment response assessment maps (TRAMs) can be used to differentiate active disease from benign enhancement.‘Red’ regions have retained and ‘blue’ regions cleared contrast; contrast clearance is associated with active disease. TRAMs efficacy in discriminating viable lymphoma from post‐biopsy change at baseline is shown in another patient by the right sided images. In the case of this 67‐year‐old man, with biopsy proven primary CNS lymphoma, it is not possible to distinguish post‐biopsy change from possible remaining macroscopic lymphoma on the top right CE‐MRI. The bottom right TRAMs however discriminates the MRI findings into blue (viable lymphoma) and red (post‐biopsy benign) changes.Gadolinium enhancement is a feature of active lymphoma, but contrast enhancement is not specific as it is also seen following disruption of the blood brain barrier due to biopsy, inflammation and haemorrhage. With TRAMs, further magnetic resonance images are acquired approximately an hour post‐contrast administration and subtracted from the initial images providing a colour map. In the present case, the lesion (top left) had residual peripheral contrast enhancement following treatment (middle left). The red signal on TRAMs (bottom left) though indicates benign enhancement, enabling a decision not to proceed to radiotherapy. To the best of our knowledge the patient is still in complete remission nearly two years post‐imaging.Early work has been published in some tumour types, however, to our knowledge this is the first report describing TRAMs as a new concept in CNS lymphoma. TRAMs has potential as a problem solving tool in CNS lymphoma as it allows MRI contrast enhancing regions to be stratified into areas of viable lymphoma and benign enhancement, increasing accuracy of the staging and response assessment used to guide patient management decisions.


Teaching point: An ureteroinguinal hernia is a very rare diagnosis with typical radiological findings that must be reported, particularly in surgical candidates. Figure 1, arrow on axial image) with an incomplete duplex collecting system, with a bifid pelvis and a single ureter. The ureterovesical junction was normally positioned. Hydroureteronephrosis was present, and the ureter descended into the right inguinoscrotal region , with stenosis at the hernia defect . Renal function tests showed high creatinine  and low glomerular filtration rate . The patient underwent surgery for ulcerorrhaphy, during which a right indirect inguinal hernia with peritoneal fat was found. The ureter was in a retroperitoneal location, accompanying the hernia. The final diagnosis was a paraperitoneal ureteroinguinal hernia. This was not surgically approached over hemodynamic instability. The patient improved, renal function tests normalized, and the hydroureteronephrosis decreased at follow-up imaging.An 86-year-old man with hypertension, type II diabetes, and no surgical history was admitted for acute upper gastrointestinal bleeding due to a bulbar ulcer. For clinical worsening after the upper endoscopy, an abdominopelvic computed tomography  with intravenous contrast  on arterial, venous, and urographic phases  was performed. Images showed signs of gastrointestinal perforation. Incidentally, a right single kidney was detected , and magnetic resonance imaging (MRI) can perform the diagnosis. The classic “curlicue” or “loop-the-loop” sign can be seen on reconstructed CT and MRI images, representing a redundant loop of the ureter and its abrupt change in calibre as it passes through the hernia defect. Urographic phase images are crucial in doubtful cases. There are two types of ureteroinguinal hernia. The paraperitoneal type (80% of cases) is acquired and is a sliding hernia, as the herniation sac drags the ureter due to adhesions to the parietal peritoneum, frequently along with viscera. Hydroureteronephrosis develops as there is a battle for space at the hernia defect. These are generally indirect hernias, large, reducible, and asymptomatic. The extraperitoneal type (20% of cases) is congenital and lacks a peritoneal sac. The ureter herniates along with retroperitoneal fat because insufficient ureteral differentiation from the Wolffian duct leads to traction of the ureter through the persistence of genitoinguinal ligaments. These hernias are usually minor defects, nonreducible, and symptomatic. They are commonly associated with renal or ureteral malformations. Ureteroinguinal hernias are most frequently found at the time of surgery. The same surgical techniques used in inguinal hernia repair can be employed in ureteroinguinal hernias, but placement of a ureteric stent is recommended to localize better and protect the ureter .


Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor mainly seen in the pediatric population. Here, we describe a case of an aggressive sinonasal RMS with distant metastasis in an adult patient. A 51-year-old male presented to the otolaryngology clinic with a unilateral painless neck mass and nasal obstruction. A flexible transnasal endoscope showed a huge fungating mass obstructing more than 80% of the right nasal cavity. A contrasted computed tomography (CT) scan of the paranasal sinuses showed an enhancing soft-tissue density mass involving the right nasal cavity. A biopsy revealed RMS, an embryonal variant. The patient responded well to chemoradiotherapy but later developed spinal metastasis and cord compression. He was admitted for palliative care but died due to cardiopulmonary arrest 10 months after diagnosis. A high index of clinical suspicion for malignancy is required in adult patients with unilateral nasal symptoms. Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor usually seen in the pediatric population. It accounts for 5% of all pediatric malignancies and around 50% of soft-tissue sarcomas diagnosed in children . On the Sinonasal RMS has an overall worse prognosis with a high mortality rate, mainly due to its usual presentation in advanced stages and its tendency for intracranial spread ,5. The pA 51-year-old male presented to the otolaryngology clinic with a painless right-sided nonprogressive neck mass and a right-sided nasal obstruction for a one-month duration. He did not have other sinonasal symptoms , compressive symptoms , and neither fever nor constitutional symptoms. The patient’s medical history was only significant for diabetes mellitus and smoking. Physical examination revealed a palpable, hard, non-tender level II cervical lymph node on the right side measuring around 3.0 × 1.0 cm. Flexible transnasal endoscope showed a huge fungating mass obstructing more than 80% of the right nasal cavity, adherent to the lateral nasal wall and extending superiorly  scan of the paranasal sinuses and neck showed a faintly enhancing soft-tissue mass in the right nasal cavity with extension to the left nasal cavity, right frontal sinus, bilateral ethmoidal sinuses, left maxillary wall and left sphenoid Figure . MultileAfter conducting a tumor board meeting, the patient was referred to the oncology department and received two cycles of multiagent chemotherapy plus radiotherapy. Following the completion of treatment, a repeated CT scan showed a marked interval improvement and shrinkage of the tumor and previously enlarged lymph nodes Figure . One monSinonasal RMS has an incidence of 0.034 per 100,000 cases, predominantly reported in the pediatric population . In adulThe head and neck region represents the most common site for the primary tumor accounting for one-third of the cases, followed by the genitourinary tract and extremities ,4. The pImaging studies for the primary tumor include CT scan and MRI. CT scan helps determine the size of the tumor, bone erosion, and metastatic evaluation. On the other hand, MRI evaluates orbital and intracranial involvement in case of suspicion. However, the diagnosis is established based on histopathological characteristics .Studies on the management of sinonasal RMS in adults are limited, mainly due to the rare nature of the disease and its limited prevalence in adults. The current standard of care is a multimodality treatment that includes chemotherapy and radiotherapy ,7. SurgiMultiple factors influence the prognosis and course of the disease, including age, anatomical site, tumor size, histological subtype, and the presence of regional or distant metastasis ,15. SinoRMS is a rare tumor in adults with poor outcomes compared to the pediatric population. Due to its usual presentation in advanced stages, sinonasal RMS has a high mortality rate. Multimodality treatment, including chemotherapy and radiotherapy, is the preferred plan for the treatment with multidisciplinary team care to optimize patient health and outcomes. A high index of clinical suspicion for malignancy is required in adult patients with unilateral nasal symptoms.


The incidence of leiomyosarcomas (LMS) has declined drastically. In fact, the introduction of immunohistochemistry (IHC) helped to differentiate LMS from other gastrointestinal stromal tumors (GIST) by receptor tyrosine kinase (KIT)-mutation detection making gastric LMS a sporadic tumor recently. We report a 60-year-old female who presented with a three-week history of abdominal pain. An abdominal computed tomography scan showed a large exophytic mass (22 ×19 ×15 cm) arising from the greater curvature of the stomach with multiple metastases. A biopsy was taken, and the initial histopathological examination was suggestive of GIST. However, further histopathological examination confirmed a high-grade gastric LMS. The patient refused any surgical intervention. Therefore, the patient had only received chemotherapy. On 9-month follow-up, the patient is still alive without disease progression. In conclusion, gastric LMS is a rare tumor. Due to the possibility of being misdiagnosed with other GIST, extensive pathological evaluation through specialized experts and IHC analysis is recommended. Sarcomas are cancerous mesenchymal malignancies that make up less than 1% of all adult solid hematological malignancies ,3.Because this tumor's growth is often insidious, and it is usually discovered at an advanced stage. As a result, knowledge of the biology and scientific behavior of LMS in the stomach is restricted, and the presentation of new cases is vastly necessary ,5. We prPatient information: a 60-year-old woman with morbid obesity presented to our outpatient clinic with a chief complaint of mild abdominal pain and left lower extremity edema that started three weeks ago. Her abdominal pain was localized in the epigastric area and radiated to the left lower quadrant, puncturing type, worsened with lying down, and not relived with analgesic therapy. The patient had a history of chronic anemia that started six months ago without response to medication or blood product transfusion. The patient had diabetes mellitus type 2 on oral hypoglycemia drug agents. No history of melena, jaundice, fever, vomiting, nausea, or urinary tract symptoms was mentioned. Her family had no history of malignancy or other chronic medical illness.Clinical findings: in physical examination, the patients' vital signs were blood pressure: 100/70 (mmHg), respiratory rate: 14 (respirations per minute), pales rate: 61 (beats per minute), oral temperature: 37.5°C, and body mass index (BMI): 35 kg/m2. She was pale, and an abdominal exam revealed a mildly distended abdomen with palpated abdominal mass in the upper and lower left quadrant with mild tenderness. There was mild pitting edema in both lower extremities.Diagnostic assessment: the white blood cells: 11 ×103/ml, hemoglobin: 7 g/L, platelets count: 200 × 103/L, reticulocyte's count: 9.5%, erythrocyte sedimentation rate (ESR): 24 mm/hr, and serum ferritin: 10 ng/L. The stool exam was positive for occult blood. The other blood investigation tests, including liver function tests, coagulation tests, and renal function tests, were within normal limits. Abdominal and chest computed tomography (CT) scan showed a 22 ×19 ×15 cm ill-defined, heterogeneous, lobulated mass with main bulk in the left hypochondrium, arising from the gastric wall (greater curvature). The mass cross the midline causing displacement of the aorta and the celiac artery, and had a pressure effect on the pancreas, stomach, spleen, and bowel loops. There were multiple metastases of the lung, liver, and multiple pulmonary and mesenteric lymphadenopathies (opathies .The ultrasonography (US) guided true-cut biopsy from the abdominal mass was performed. The initial histopathology result was highly suspected of GIST. The histopathology was reviewed by an expert pathologist, which showed a fascicular growth pattern of smooth muscle tumor and nuclear pleomorphism suggestive of a high-grade gastric LMS . ImmunohTherapeutic interventions: the patient has rejected any surgical interventions. For that, the multidisciplinary team recommended the treatment with chemotherapy agents with paliation intent. The patient received five cycles of doxorubicin (90mg/cycle) in monotherapy but showed disease progression after five months of chemotherapy with increasing size of the mass and the number of hepatic lesions. Subsequently, the chemotherapy was changed into six cycles of gemcitabine-docetaxel combination, starting with gemcitabine (1400 mg) on day one, followed by a combination of gemcitabine (1200 mg) with docetaxel (100 mg) on day 8.Follow-up and outcome: within nine months of follow-up, the patient was still alive, had no disease progression, and with an acceptable condition.Patient perspective: during treatment, the patient was satisfied with the level of care provided to her.Informed consent: written informed consent was obtained from the patient for participation in our study.et al. first described GIST with C-kit mutations, gastric LMS has been rarely mentioned and accounts for 1% of all malignant gastric tumors [Leiomyosarcoma (LMS) accounted for nearly 5%-10% of all soft-tissue sarcomas and are most commonly found in the uterus, digestive system, and retroperitoneal region. Since Hirota c tumors ,6. The cc tumors .Radiologic images such as the ultrasound scan (US), CT scan, and magnetic resonance imaging (MRI) can help in LMS diagnosis. However, they cannot provide a definitive diagnosis. The US has a lower-cost and used to evaluate the tumor size, location, and cystic versus solid lesions. Nevertheless, it is ineffective for follow-up tumor sizes after treatment . The priet al. [Microscopically, LMS should be distinguished from other spindle cell tumors, especially GIST and nerve sheath tumors . Distinction from benign LMS is mitosis >10/50HPF), atypia, and coagulative necrosis, which is found in LMS and absent in benign leiomyoma. The primary differential diagnosis histologically is GIST, which should be notably excluded in all gastric cases. In our case, the initial histopathology result was highly suspected gastrointestinal stromal tumors. The histopathology was reviewed by an expert pathologist, which showed a high-grade gastric LMS. A similar report was mentioned by Garg et al. . GIST shet al. ,10,11. I/50HPF, aet al. reported that gemcitabine and doxorubicin could regress the LMS in the patient-derived orthotopic xenograft model [et al. retrospectively studied 147 adult patients with metastatic LMS. The authors concluded that maintenance of the doxorubicin alone was associated with improved progression-free survival, and combined chemotherapy regimens did not improve the outcome [Gastric LMS is a cruel disease that does not respond to chemotherapy or radiation . Only suft model . Additio outcome .et al. reported that both neoadjuvant and adjuvant radiotherapy was associated with improved 5-year-survival compared with surgery alone [et al. in the multivariate analysis, showed that the overall survival was not affected by receiving radiotherapy [In literature, the benefits of radiotherapy in addition to surgery are controversially discussed. While Nussbaum ry alone . Hager eotherapy .et al. reported a 47-year-old man with a gastric LMS and metastasis to the liver treated with doxorubicin and a docetaxel-gemcitabine combination and stable disease achieved with pazopanib [Leiomyosarcoma (LMS) has a propensity for hematogenous spread. Distant metastases are present in approximately 40% of diagnosed patients, and most patients finally develop metastasis ,18. AddiGastric LMS is a rare cancer. Histopathological examination of the specimen with IHC analysis is the optimal choice for making an accurate diagnosis. Due to the possibility of being misdiagnosed with other gastrointestinal stromal tumors, extensive pathological evaluation through specialized experts and IHC analysis is recommended.


Traumatic rectal injuries (TRIs) are challenging for surgeons because of their high morbidity and mortality. Considering the well-known predisposing factors, enema-associated rectal perforation seems to be the most overlooked entity that leads to devastating rectal injuries. A 61-year-old man with a three-day history of painful swelling around his perirectal area after enema application was referred to the outpatient clinic. CT demonstrated the presence of a left posterolateral rectal abscess compatible with an extraperitoneal rectal injury. Sigmoidoscopy revealed the perforation started 2 cm above the dentate line with a diameter of 10 cm and a depth of 3 cm. Endoluminal vacuum therapy (EVT) and laparoscopic sigmoid loop colostomy was performed. The patient was discharged after removing the system on postoperative Day 10. On his follow-up, the perforation side was totally closed and pelvic abscess was completely resolved two weeks after his discharge. EVT appears to be a simple, safe, well-tolerated and cost-effective therapeutic procedure in the management of delayed extraperitoneal rectal perforations (ERPs) with large defects. To our knowledge, this is the first case that reveals the potency of EVT in the management of a delayed rectal perforation associated with an uncommon entity.  Traumatic rectal injuries (TRI) remain a challenging entity for the surgeons because of their unpredictable extent of the damage through the rectal tract and around the perirectal area. Many risk factors leading to TRI have been reported in the current literature. Among these, enema application is an uncommon entity ,2. The eEndoluminal vacuum therapy (EVT) is a modification of conventional vacuum-assisted wound closure (VAC) system that has been adapted for internal use, which yields promising results in the management of anastomotic leakage after rectal surgery . In spit3) and increased C-reactive protein (72 mg/dL). Abdominal CT demonstrated the presence of a left posterolateral rectal abscess extending to the mesorectum compatible with an extraperitoneal rectal injury. On sigmoidoscopy, the perforation started from 2 cm above the dentate line extending up to 10 cm along the rectum with a diameter of 10 cm and a depth of 3 cm . On physical examination, painful fluctuating abscess located at the left lateral wall of the anus was observed. On digital examination, more than a half of the posterior wall of the rectum had perforated. His abdominal examination was normal. Laboratory parameters revealed mild leukocytosis 14,200/mm00/mm3 an Enemas are injections of fluid used to clean distal bowel before colonoscopy as well as to treat constipation or inflammatory bowel diseases. In spite of the fact that enema application seems to be a relatively innocent and easy procedure, clinicians should be aware that it is a penetrating trauma to the rectum. Considering that most of the patients perform this application on their own, careless practices may lead to life-threatening complications such as rectal perforation, as in the present case. The rarity of enema-related complications in the current literature should not mislead the clinician since this subject might be underestimated because of underreporting . Hence, The lower one-third of the rectum is located extra-peritoneally, so there is a low possibility of fecal contamination into the abdominal cavity which is a condition that does not require prompt surgery. However, comparing with intraperitoneal injuries, the optimal treatment method is more controversial.  ConservaEVT is a modification of conventional VAC system that has been adapted for internal use. This system provides continuous or intermittent drainage via an open-pored polyurethane sponge that decreases bacterial contamination, secretion, and local edema, thereby enhancing recovery by promoting perfusion and granulation of the injured tissue ,6. VAC tSponge dislocation, persistent anal pain, and tissue necrosis due to the system being set at high negative pressure, discomfort due to the repeated colonoscopy and the placed system are the most frequent adverse events that can be observed during EVT ,5. MoreoWe presented a case with delayed rectal perforation who was successfully treated by EVT. Despite the large defect, EVT ensured a significant short recovery period with proper control of the source of abscess. We are aware that this is an anecdotal case presentation, so it is difficult to make a definitive comment on the exact potential of EVT. However, our clinical outcome encourages us to suggest that EVT may be considered as a simple, safe, well-tolerated, and cost-effective therapeutic alternative procedure when endoscopic or surgical interventions fail. This suggestion should be investigated with further cases regarding this situation.


Large and rapidly growing abdominal tumors may result in fatal outcomes in newborns. In some cases, a rapidly worsening clinical condition requires surgical decision‐making despite the absence of a precise histological diagnosis. In these situations, there is neither a guide nor consensus.We highlight our experience with five patients with large abdominal tumors and assess the available literature for the best possible management of a rare condition.In these cases, laparostomy should be considered as a life‐saving procedure. If the liver is involved and coagulopathy is present, prognosis is often compromised. For each patient, we collected data concerning sex, antenatal history, birth weight, age at surgery, type of tumor, type of surgery, type of imaging and clinical results at the last control.Table 3Case 1A newborn boy delivered at 39 weeks of pregnancy (WoP) + 5 days weighing 3540 g with a normal antenatal history presented with significant abdominal distension causing respiratory distress at birth. The first postnatal abdominal ultrasound (US) did not show the origin of the tumor; however, magnetic resonance imaging (MRI) showed a solid intraperitoneal mass measuring 93 × 100 × 120 mm. Due to the increasing need for respiratory support, laparotomy was performed at 8 days of life. The intraoperative findings revealed an intramesenteric tumor, which was completely resected. Pathological analysis revealed an immature grade III teratoma. Before surgery and postoperatively, alpha‐fetoprotein levels were within normal limits. At follow‐up 3 years after surgery, there was no evidence of complications or recurrence. The follow‐up was based on clinical examination, monitoring of blood markers and hepatic ultrasonography twice a year.Case 2A newborn girl delivered at 37 WoP + 1 days and weighing 3600 g was revealed to have a large abdominal tumor on US the day before delivery. All previous US findings were normal. On MRI, a heterogeneous (solid and cystic) mass measuring 111 × 67 × 124 mm originating from the mesenteric root was observed Figure . UrinaryCase 3A newborn girl delivered at 34 WoP and weighing 3850 g with a normal antenatal history presented with a large abdominal mass at birth. Alpha‐fetoprotein and urinary catecholamine levels were within normal limits. The MRI measured a solid mass of 11.5 × 75.5 × 99 mm, revealing areas of central necrosis, which was postulated as responsible for acute progressive respiratory distress Figure . The delCase 4A newborn girl delivered at 40 WoP and weighing 3030 g with a normal antenatal history was admitted to the neonatal intensive care unit for cardiogenic shock, acute respiratory distress, and hypertension (123/78) 3 h after birth. Cardiomegaly was noted on the chest radiograph, and abdominal US revealed a left adrenal mass measuring 41 × 40 × 42 mm. Nicardipine was initiated immediately at 1 μg/kg/min, allowing for blood pressure correction (89/66 mmHg) and progressive resolution of the hemodynamic status. Nicardipine was successfully shifted to oral nifedipine on the sixth day of life.Vanillylmandelic acid (VMA) and homovanilic acid (HVA) were measured in the urine on the first day of life. After 15 days, the results revealed that the VMA and HVA levels were 50.19 mmol/L  and 10.17 mmol/L .Case 5A newborn boy delivered at 37 WoP and weighing 3160 g with a normal antenatal history was admitted to the neonatal intensive care unit at 10 days of life for vomiting, polypnea, and a rapidly increasing abdominal girth. MRI showed a large abdominal mass that probably originated from the liver and occupied the entire abdomen. In 2 h, the abdominal diameter increased from 38 to 40 cm. The alpha‐fetoprotein level was remarkably increased (13 354 mcg/L), the beta‐HCG level was 0.5 mcg/L, and levels of urinary catecholamines were within normal limits . The aspartate transaminase/alanine transaminase ratio was 2097:309. Kasabach–Merritt syndrome was suspected as hypofibrinogenemia (0.9 g/L), consumptive coagulopathy , and disseminated intravascular coagulation were noted. Cytoreductive chemotherapy was initiated at the time of admission using corticosteroids (methylprednisolone 1 mg/kg) and completed with etoposide and carboplatin.On MIBG scintigraphy, no metastatic tumor was observed in the suprarenal region. The patient was referred to the national staff of pediatric oncology, and the decision for surgical removal was made. The patient underwent open surgery at 28 days of life. Pathological examination of the tumor revealed an adrenal neuroblastoma. Four years later, the patient remained disease‐free.44.1In newborns, large benign and malignant tumors found in the abdomen can originate from either peritoneal or retroperitoneal structures . These tumors are extremely rare; however, consequent acute decompensation with emergent symptomatology in newborns is even rarer.A rapidly growing abdominal mass may threaten the lives of newborns. For example, in 2015, Majjiga et alPrenatal diagnosis of solid abdominal masses has also been reported. For example, in a case series, De Backer et alSuch examples, as in our own case series, emphasize the difficulties in making the most ideal decision. Furthermore, although there are protocols for each type of tumor,4.2In our study, among the five newborns who underwent surgery, increasing need for ventilatory support was the primary indication for surgery in four newborns. Even though one of the masses (hepatic hemangioma in case three) could have been treated with systemic corticosteroids or propranolol,Abdominal compartment syndrome is defined as “sustained elevation in intra‐abdominal pressure >10 mmHg associated with new or worsening organ dysfunction that can be attributed to elevated intra‐abdominal pressure.” Intra‐abdominal pressure is defined as “the steady‐state pressure concealed within the abdominal cavity at the end of expiration in the supine position, after ensuring that abdominal muscle contractions are absent and with the transducer zeroed at the level of the mild axillary line”.Another life‐threatening condition is Kasabach–Merritt phenomena.In 2003, Bärtsch et alSurgical removal of the tumor causing coagulopathy is associated with normalization of hematological abnormalities. However, due to evolving coagulopathy and size and infiltrative nature of the tumor, surgery is considered a high‐risk procedure that may cause bleeding.Hypertension is also an indication for early surgical treatment in newborns. Many reports have described abdominal neonatal masses that were surgically resected early due to non‐responsive hypertension. The masses were mostly renally or adrenally located such as in congenital mesoblastic nephroma or intrarenal neuroblastoma. As shown in the fourth case in our series, the baby underwent surgery for treatment‐resistant hypertension. His condition improved significantly shortly after surgery. In 2017, Robertson Bell reported a case of a baby diagnosed with congenital mesoblastic nephroma who, despite a high dose of hydralazine, had an average blood pressure that remained elevated. Surgical resection was performed at 2 days of life, and the renin level decreased 2 days postoperatively.Overall, the benefit of surgery in the absence of a definite diagnosis must be considered. Surgeons and pediatricians should share common variables and scoring systems to monitor the patients' clinical courses.In 1996, Hsu et al published an article about a long case series of neuroblastomas,4.3If a surgical option is chosen, the optimal form of intervention should be determined next. Based on our experience, choosing between laparostomy and laparotomy and excision was difficult. In the reviewed literature, laparoscopy seems to have no role in this rescue indication, and authors believe that there is insufficient space in the abdominal cavity, such as in cases of overdistention.It is unclear whether decompression could lead to an improved prognosis because of the heterogeneity of the reports and the absence of comparative trials. In 2008, Harper et alThe discussion regarding laparostomy or excision versus biopsy and chemotherapy‐delayed surgery remains controversial. Furthermore, the individual presentation of patients requires tailored responses.To summarize, we suggest following the management algorithm in flow chart n.1 based on our experiences in the management of these situations.The strength of the study lies in trying to provide a clinical algorithm in front of a rare situation in which difficult decisions with potentially fatal outcomes for the patient need to be made quickly. In addition, an attempt was made to provide objective parameters to be calculated that can help in making a choice about whether or not to take surgical care.This study has some limitations. Due to the rarity of the disease, heterogeneity of the reports in the literature, and a short case series, we could not draw conclusions based on the literature alone. Most importantly, we could not infer the statistics. Even with a standardized protocol for surgical management, multiple questions remain unanswered on whether the antenatal diagnosis can affect prognosis.5In these cases, management should be based on the clinical status of the patient and not on the correct diagnosis; if the patient's condition worsens, decompression surgery should be considered even if the origin of the mass is unknown.An increasing abdominal diameter, increasing need for ventilatory support, worsening urine output, and NIRS are the most important warning signs. If the liver is affected by the tumor and coagulopathy is present, prognosis is often compromised.Selecting between the laparostomy and full excision requires a tailored approach and multidisciplinary evaluation.In conclusion, we should infer that:Francesco Laconi: Conceptualization (lead); data curation ; formal analysis ; investigation ; methodology (lead); project administration ; resources ; software ; supervision ; validation ; visualization ; writing – original draft (lead); writing – review and editing (lead). Charline Bischoff: Conceptualization ; data curation (lead); formal analysis ; investigation ; methodology ; project administration ; resources ; software ; supervision ; validation (lead); visualization (lead); writing – original draft ; writing – review and editing . Daphne Michelet: Conceptualization (supporting); data curation ; formal analysis ; investigation ; methodology (supporting); project administration ; resources ; software ; supervision (supporting); validation ; visualization ; writing – original draft (supporting); writing – review and editing (supporting). Gauthier Loron: Conceptualization (supporting); data curation ; formal analysis ; investigation ; methodology ; project administration ; resources (lead); software ; supervision (supporting); validation (supporting); visualization ; writing – original draft (supporting); writing – review and editing . Nathalie Bednarek: Conceptualization (supporting); data curation ; formal analysis ; investigation ; methodology ; project administration (supporting); resources ; software ; supervision (supporting); validation ; visualization ; writing – original draft (supporting); writing – review and editing (supporting). Marie‐Laurence Poli‐Merol: Conceptualization ; data curation (supporting); formal analysis (supporting); investigation (supporting); methodology (supporting); project administration (lead); resources (supporting); software (supporting); supervision (lead); validation (lead); visualization (supporting); writing – original draft ; writing – review and editing (lead). Maguelonne Pons: Conceptualization ; data curation ; formal analysis ; investigation ; methodology ; project administration ; resources ; software (lead); supervision ; validation ; visualization ; writing – original draft (supporting); writing – review and editing . Nadia Boudaoud: Conceptualization ; data curation ; formal analysis ; investigation ; methodology ; project administration (lead); resources (lead); software (lead); supervision (lead); validation (lead); visualization (lead); writing – original draft ; writing – review and editing .The authors declare that they have no conflict of interest.All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all participants included in the study.


Mandibular pseudotumors, also known as blood cysts, are rare complications which occur more frequently in patients with an associated bleeding disorder such as hemophilia.We present a case of a 2-year and 6-month-old patient with a hemophilic pseudotumor associated with Von Willebrand's disease, who consulted the emergency room due to spontaneous increase in volume of the left maxillary region, with no previous relevant medical history.Different imaging studies were carried out to characterize the lesion, providing the necessary information for the correct approach. Due to the low prevalence of this complication, we believe it is of vital importance to understand the adequate management in this patient population. Hemophilic pseudotumor is caused by a hemorrhagic diathesis that manifests itself in bone and soft tissues, and is considered to be an infrequent complication that can become serious . The pseudotumor is described as being similar to a progressive cystic tumor, which involves the extra-articular musculoskeletal system, causing pressure on the adjacent tissues, which can lead to repercussions in the skin and muscle vasculature ,3. It is estimated that this complication occurs in 1% to 2% of patients with a history of severe hemophilia, and a lower percentage in those with Von Willebrand disease, thus making this a very rare case , and no other significant family history or pregnancy complicationswere noted. The computed tomography (CT)  of the nA magnetic resonance arteriography ruled out a tumor of vascular origin, and a hypointense lesion located in the maxillary antrum was also observed.Laboratory tests showed prothrombin time of 12.1 seconds, partial thromboplastin time of 34.0 seconds, as well as a normal platelet count. A biopsy was done of clot fragments removed from the left maxillary sinus cavity and also of the tumor  due to t24 hours after surgery, an increase in volume was noted due to hematoma in the surgical area that improved 72 hours after the application of plasmapheresis. Due to the improvement seen after the plasmapheresis, the possibility of a deficiency of a coagulation factor was taken into account, therefore hematology was consulted and the patient was found to have low factor VIII and decreased Von Willebrand's factor, confirming the suspicion of Von Willebrand's disease. Therefore, plasma transfusions were given in order to correct the deficiency of this coagulation factor.Pseudotumors arise as a complication of bleeding disorders, and consist of a collection of encapsulated blood generated after recurrent bleeding in bones or soft tissues , and canThis interaction between platelets and the vessel wall alters primary hemostasis, leading to heavy and continuous bleeding after any type of injury ,6. PseudThe increased pressure generated by the hematoma leads to a weathering of the contiguous tissues, having repercussions on the neighboring skeletal muscle and vascular structures. This occurs mostly in adult long bones, and in the iliac, vastus lateralis and soleus muscles . Less coThe reported cases of hemophilic pseudotumor of the upper and lower jaw have occurred in pediatric patients  , with thThe classic clinical presentation is painless swelling unless it involves nerve structures, pathologic fractures, or aggregated infections . When thThey usually present as very well defined unilocular or multilocular radiolucent lesions, with thin septa or trabeculations. There may be intralesional foci of dystrophic calcifications, cortical thickening or thinning, and marginal sclerosis .The CT sHistological examination of hemophilic pseudotumors  and 4 caAs mentioned previously, trauma is an etiological factor to take into account, however, cases have been reported in which there is an absence of any history of trauma, leading to a possible suspicion of dental eruption to be a cause of the bleeding .For the treatment of hemophilic pseudotumor, it is important to carry out proper prevention in those patients with bleeding disorders, which must be accompanied by imaging tests, together with the correct management of the underlying pathology. In our case of Von Willebrand's disease, substituting the missing factors would be the correct intervention of choice ,9.Most researchers have advised for a cautious approach to the treatment of pseudotumors of the mandible. In cases when a conservative treatment has failed, cryotherapy, aminocaproic acid injection, or ethanol embolization, as well as surgical intervention, are required . SurgeryThe approach to this complication can be hemimandibular resection, enucleation, curettage with or without tooth extractions, aspiration, and replacement therapy ,8. RegarConsidering the infrequency of this complication, it is important to evaluate the pseudotumor as a possible differential diagnosis in lesions with mass effect in the context of patients with blood dyscrasias. It is important to emphasize the use of imaging studies for diagnosis, with characterization of the lesion and the choice of an appropriate treatment, without neglecting the importance of laboratory studies to recognize if there is a history of hemorrhagic diseases.


Malignant nodular hidradenoma is a rare eccrine sweat gland neoplasm characterized by recurrence, metastasis, and a locally aggressive course. In our case report, a 74-year-old man presented with a seemingly benign swelling which was persistent for the last 30 years, which was excised at an outside institute. Since the patient presented to the hospital during the peak of the pandemic, considering the age of the patient, the pandemic situation, the logistics of radiotherapy during such a situation, preserving the knee joint function in view of close proximity of the tumour to the joint, it was decided to keep the patient on follow up and continue expectant management. After a follow-up period of 24 months, no locoregional recurrence or metastasis has been observed. The patient is on annual follow-up with clinical examination and PET-CECT imaging. Malignant nodular hidradenoma is a rare eccrine sweat gland neoplasm notable for recurrence, metastasis, and a locally aggressive course, and has an incidence of 0.001% . Here weHistory and clinical assessmentOur patient initially presented with a complaint of swelling over the lateral aspect of the left knee. The swelling was persistent over the area for 30 years previously and was not associated with any symptoms till recently. The swelling started becoming painful over the last two months. The pain was dull aching in type with no temporal variation, had no aggravating factors, and was relieved on taking analgesics. It was not associated with trauma, ulcers, or pre-existing scars. The patient did not report any discharge from the swelling, nor any redness. No secondary changes like ulceration, fungating, and softening were observed. There was no history of similar lumps in the body, and it was not associated with fever. He did not report any bleeding or suppuration from the lesion.Initially, the swelling was 50x40 mm in size, with a nodular surface. Patient-reported mild tenderness on palpation. The swelling was firm in consistency. No fixity to local structures was observed. No distal neurovascular deficits were observed. No ulceration, erythema, or tenderness was present over the swelling. No discharge, bleeding, nor locoregional lymph node enlargement was present. No systemic symptoms were present. Differential diagnoses were fibroma, synovial or meniscal cyst, lipoma, and sarcoma.Surgical excision of the lesion was planned at an outside institute and the patient was referred to our institute for subsequent management.Post-operative histopathological examination of excision biopsy done at another instituteA nodular swelling measuring 30x35x25 mm is observed, its cut surface is grey white and lobulated with cystic spaces. Sections show skin and dermis and lobulated tumour composed of anastomosing cords of cuboidal cells, loose mesenchymal stroma, and cystic spaces lined by cuboidal cells and filled with proteinaceous material. The focal area shows sheets of large cells with large vesicular nuclei and prominent nucleoli. The superior, inferior, medial, lateral, and deep resected margins are free of tumour, with the closest margin being the deep resected margin, which measured 19mm. Overall features were suggestive of a malignant nodular hidradenoma.On examination, a 66 mm linear scar was present over the posterolateral aspect of the left knee with no residual swelling. The skin on and around the scar is thickened. There was no tenderness on palpation. A picture of the postoperative site is attached in Figure MRI of the left kneeA T1 and T2 hypointense irregular scar involving skin and the subcutaneous plane of the posterolateral aspect of the knee is seen. There is no extension into the deep fascia. On post-contrast scans, mild enhancement is seen. Mild effusion is also seen. Periarticular soft tissue planes appear normal. No suspicious features were noted. As a result, re-excision was deferred.Histopathological slide review at the instituteThe histopathological slides of the operated lesion were referred for further analysis to our institute. This section shows skin with an epidermis that is unremarkable. The deep dermis shows a tumour with cells arranged in solid sheets, nests, and anastomosing cords with intervening cystic spaces filled with eosinophilic material. Cells show moderate pleomorphism with a vesicular nucleus and prominent nucleoli with mitotic activity. Tumour cells seem to invade as tiny nests into the subcutaneous adipose tissue. Features are those of a malignant adnexal tumour, consistent with malignant nodular hidradenoma. The tumour was positive for cytokeratin 5 and 6, EGFR, and EMA. The MIB-1 labelling index was 28%. Histopathological pictures are available for review in Figure Follow-up protocol and further managementTreatment modalities for malignant nodular hidradenomas are under-researched and few in literature. Considering the age of the patient, the close proximity of the post-operative tumour cavity to the knee joint, and the pandemic situation, expectant management seemed a prudent course of action to follow. Our patient was put on close monitoring and is on regular follow-up as a part of expectant management.As a part of expectant management, the patient underwent a PET-CT scan in June 2022 and there was no FDG uptake in the primary site or any distant sites. The patient is asymptomatic, has not reported any new swellings, and is on regular follow-up under the Department of Radiation Oncology. The patient is planned for an annual follow-up with PET-CECT imaging and clinical review.Malignant nodular hidradenomas are a group of aggressive and poorly differentiated eccrine sweat gland tumours that invade surrounding tissue and lymphovascular structures. It has equal male-female distribution and a high local recurrence rate, usually, within the first two years from diagnosis . While uClinically, hidradenomas present in an asymptomatic indolent manner as swelling with slow progression, with drastic unheralded malignant transformation with lymphatic spread  and metaThe histopathology and immunohistochemistry of malignant nodular hidradenoma appear similar to ductal adenocarcinoma of breast and salivary gland tumours .Histology of the primary neoplasm usually shows nodular dermal proliferation composed of poorly differentiated clusters of epithelioid cells with the pseudopapillary arrangement. For diagnosing a malignant hidradenoma, criteria typically include deep extension with an infiltrative pattern, nuclear pleomorphism, necrosis, increased mitoses (>4 per HPF), and lympho-vasculoneural invasion . HoweverThere are few existing immunohistochemical studies due to the rarity of the disease and due to misdiagnoses. Some markers that were identified were p53, Ki-67 protein (as a proliferation marker), MIB-1 index , PHH3 marker (specific for mitosis), epithelial membrane antigen, CK 5/6, BCL - 1/2 and EGFR . In the study done by Ko et al., all tumour specimens included in the study showed positivity for keratin AE1/3 and cytokeratin 5/6 .Radiologically, magnetic resonance imaging shows soft tissue mass in subcutaneous layers with well-delineated margins and heterogeneous appearance - lobulated, cystic, or solid. T1 weighted images can reveal low to intermediate signal intensity lesions with markedly isointense areas. On T2 weighted images, the lesion shows intermediate to high signal compared to muscle with heterogeneity. The variation is most likely due to the variations in adnexal secretions, blood, and fatty tissues in the cystic fluid. PET CT usually shows hypermetabolic activity. The utility of PET-CT has been demonstrated in following up on post-treatment patients by monitoring the metabolic response. PET CT has been previously used for patients with abnormal skin neoplasms, but with a caveat that dermal metastases of internal malignancies might be a false positive, albeit low in incidence ,11.It is primarily treated by surgical excision with a tumour-free margin of 2 cm . It was Systemic chemotherapy regimens based on fluorouracil, cisplatin, and doxorubicin have not shown any significant therapeutic benefit. In a few cases, malignant eccrine tumours with receptors positive for estradiol showed response to tamoxifen therapy with complete resolution of lymph node spread and full pain relief from bone metastasis. The pain relief from bone metastasis was present for a period of nearly three years ,16.Radiotherapy as an adjuvant modality of treatment has been established in the 1990s for head and neck malignant hidradenoma with positive margins. Adjuvant modalities of treatment acquire more importance as the tumour has a 10-50% chance of recurrence despite aggressive surgical treatment . HistopaAdditionally, 71.6 Gy high dose irradiation with 57.6Gy to the tumour bed and 14Gy boost to the primary lesion using high energy photon with or without electron boost provided a disease-free period of 35 months after treatment completion. Hyperfractionation is recommended to prevent adverse reactions, especially in the head and neck area .Another modality of treatment is electrochemotherapy involving local or intravenous antineoplastic agent instillation, usually bleomycin or cisplatin with electroporation of the tumour, thus exposing the tumour cells to cytotoxic drugs. It is a well-tolerated treatment modality suitable for cutaneous and subcutaneous tumours with minimal side effects, with the maintenance of cosmesis and function of surrounding tissues, but warrants a larger sample size and widespread usage before deciding upon it as a standard of care .As a rule, the principles of treatment of melanoma and other skin cancers have been applied to the treatment of adnexal skin tumours. Thus, it warrants precise and careful surgical excision, regular follow-up, and investigations into the utility of sentinel lymph node biopsy.Due to the pandemic and the age of the patient, we had opted for a wait-and-watch approach rather than the usual modalities of treatment. The patient is under expectant management with our department.This case report is to highlight the need to be aware of and recognize a seemingly harmless swelling before it manifests in its natural course of aggression. It also suggests the need to evaluate such rare cases on a per-patient and per-situation basis and decide on treatment accordinglyRobust follow-up policies are required for enabling wait and wat,ch protocols, and in malignant nodular hidradenoma, it could be scheduled as six-month follow-ups imaging reviews for the first three years and annually thereafter, with clinical examination and assessment every three months for the first two years, every six months for the next three years and annually thereafter.


Giant cell angioblastoma is a relatively rare vasogenic tumour. To date, studies on its clinical manifestations, imaging characteristics, pathological features, and prognosis are extremely limited and unknown, with only a few cases recorded. In this study, four cases of giant cell angioblastoma confirmed by pathological examination were reported to improve our understanding and deep exploration of the tumour spectrum. All cases in our study were male, including two adults and two boys. The lesions were located in the lower segment of the femur, medial condyle of the femur, knee joint, and popliteal fossa. Regarding the imaging characteristics, two patients with lesions in bone showed bone destruction, while the other two had lesions that invaded soft tissues, showing irregular, abnormal signal shadows and obvious enhancement. Histopathological analysis revealed that the nodular tumour tissue was mainly composed of oval and spindle cells, with varying numbers of osteoclast-like multinucleated giant cells, and the interstitial tissues were often filled with blood vessels of different sizes. The immunophenotype demonstrates that endothelial cells of small vessels in nodules expressed CD31, SMA, and ERG, while osteoclast-like multinucleated giant cells and histiocytes expressed CD68 and CD163, and the surrounding cells expressed SMA. All four patients were treated with surgical resection. One of them relapsed 1 month after surgery and received a second surgical resection. No distant metastasis or death occurred during the follow-up period. This study indicates that giant cell angioblastoma is a local invasive vascular tumour that can develop both in children and adults with skin, mucous membrane, soft tissue, and bone involvement. Imaging characteristics show bone destruction and irregular, abnormal signal shadows; in addition, obvious pathological morphological features can be observed. Currently, the treatment is mainly surgical resection, and interferons may be used as adjuvant chemotherapy. Giant cell angioblastoma (GCAB) is a rare vascular tumour that was first reported in 1991 by Gonzalez-Crussi et al. ; howeverData on four cases of giant cell angioblastoma diagnosed in Foshan Hospital of Traditional Chinese Medicine from 2011 to 2021 were collected. The follow-up time ranged from 0.5 to 10 years. The clinical data, imaging data, and pathological results of all patients were provided by the inpatient data system.After surgical resection, all samples were fixed with a 10% neutral formaldehyde solution and dehydrated with ethanol immediately. Then, the paraffin-embedded pathology samples were sectioned to 4 μm, stained with haematoxylin and eosin, and observed with low- and high-magnification microscopes. Meanwhile, continuous sections were marked by immunohistochemistry, and the envisioned two-step method was adopted. The selected antibodies included CD31, F8, CD34, ERG, FLi-1, SMA, calponin, desmin, CD68, and CD163.A 15-year-old boy with left knee pain and limited mobility for 10 months was admitted to the hospital. The physical examination revealed that the patient's left knee was slightly swollen, the skin temperature and skin color were normal, the inner space of the knee joint was tender, and the joint movement was restricted. Preoperative x-ray showed bone destruction of the left medial femoral condyle . CT was A 3-year-old boy with left leg claudication for 2 years was admitted to the hospital. The physical examination showed mild swelling of the lower left thigh and knee, normal skin color, and high temperature with no obvious tenderness. X-ray showed uneven density in the lower segment of the left femur with patchy bone destruction and marked osteosclerosis around the lesion. MRI revealed that the periosteal reaction was detected in the middle and lower parts of the left femur, and the intramedullary cavity density was uneven, which had spread to the epiphyseal area of the distal femur. In the beginning, he was diagnosed with chronic osteomyelitis, but to our surprise, the symptoms did not improve significantly after 2 weeks of continuous antibiotic treatment. Therefore, lesion-removal surgery was performed. Some bones that had been hardened and whitened, and mucus-like tissue were scraped out. Postoperative pathology was diagnosed as giant cell angioblastoma . The patA 23-year-old men with recurrent pain on the outside of his left knee joint was admitted to the hospital. The physical examination demonstrated that his left knee was slightly swollen, the lateral part of the joint was obviously tender, and the joint movement was limited. MRI showed a patchy abnormal signal shadow in the vastus lateralis, equal or slightly low signal shadow on T1WI, and high or low mixed signal shadow on T2WI-FS, with clear edges and mild enhancement on an enhanced scan. The proximal femur bone was not advanced or eroded, and the cortical bone was intact . He was An 18-year-old men with left popliteal fossa pain and limited knee movement for 7 years was admitted to the hospital. The physical examination showed obvious muscle atrophy of the left lower limb and swelling of the knee joint with obvious tenderness in the popliteal fossa. The movable joint was painful, and the movement was restricted. MRI showed an abnormal signal shadow between the posterior upper margin of the left medial femoral epicondyle and the vastus medialis. T2WI showed a high signal with a slightly unclear boundary, and an enhanced scan showed obvious enhancement and patchy T2WI-FS high signal oedema shadows with an unclear boundary from the lesion . DopplerGCAB was first reported by Gonzalez-Crussi in 1991, and he proposed the diagnosis of giant cell angioblastoma. To date, 12 cases have been reported, as per our search . This stIn terms of imaging features, x-ray and CT scans of those cases with bone involvement showed increased bone density, thickened cortical bone, different degrees of bone destruction, and insignificant swelling of surrounding tissues, which could easily be misdiagnosed as osteomyelitis and tuberculosis. Fortunately, we can distinguish through infection indicators and the mycobacterium tuberculosis test , 9. MRI At present, there is no optimal treatment for giant cell angioblastoma. The principal treatment is surgery, aiming to remove the tumour as completely as possible and to ensure negative margins. There are no reports or experiences of chemotherapy and radiotherapy after or before the operation. In this study, four patients were treated with surgery, of which three did not relapse during follow-up. One case of a 23-year-old men (Case 3) showed no significant relief of pain after surgery, and an MRI re-examination suggested residual tumour 1 month after the surgery . He undeIn summary, giant cell angioblastoma is a locally invasive vascular tumour that can develop both in children and adults, although it is more common in males. It can involve skin, mucosal tissues, soft tissues, and bone. The imaging characteristics show bone destruction or irregular, abnormal signal shadows with obvious morphological features. Surgery is now considered the primary treatment, while interferons may be used as an adjuvant treatment for giant cell angioblastoma.