Cardiac tumors are a rare condition that typically presents with nonspecific symptoms. Among the histologic patterns, myxoid sarcomas are rarely identified and may have a less favorable prognosis. Reporting a case of this type of cardiac tumor can increase awareness about this condition and aid in early diagnosis, potentially leading to better outcomes.We are presenting a case of a 41-year-old female with left atrial myxoid sarcoma, which was presented with a cardiogenic shock picture. She underwent surgical excision of the mass and was discharged in good condition. After discharge, she deteriorated and was found to have lung metastases.Primary cardiac sarcomas, due to their rarity and poor prognosis, are often diagnosed at an advanced stage of the disease and lack sufficient data to establish a standard course of treatment. The cornerstone of therapy is surgical resection. However, novel therapeutic approaches must be developed.Primary cardiac tumors should be suspected in adult patients with progressive dyspnea, and a biopsy should also be done to determine the histopathological pattern of the mass and estimate the overall prognosis and outcomes. HighlightsMyxoid sarcoma is a rare type of cardiac tumor that may be misdiagnosed as a myxoma.Myxoid sarcoma has high metastatic capacity, most likely to the lungs.Echocardiogram is the gold diagnostic tool, computed tomography or positron emission tomography scans for extension and metastases.Surgical resection is the standard of care, and the prognosis depends on metastases.1. Clinically, it can be challenging to differentiate between benign and malignant tumors because symptoms are frequently not related to the type of tumor but rather to its location in the heart2. Primary cardiac sarcomas can develop at any age, with a mean age of 41 years and a higher incidence during the third and fifth decades3.The majority of primary cardiac tumors are benign and infrequent. Myxomas, which have an estimated postmortem incidence of 0.375–11.25 per 100 000 cases, are the most prevalent benign cardiac tumors, while sarcomas, which have an estimated autopsy incidence of 0.275–7.125 per 100 000 cases, are the most prevalent malignant cardiac tumors5. Herein we are presenting a case of a 41-year-old female with left atrial myxoid sarcoma, which was presented with a cardiogenic shock picture. She underwent surgical excision of the mass and was discharged in good condition. After discharge, she deteriorated and was found to have lung metastases. This case has been reported in line with the 2020 SCARE  guidelines6.Surgery continues to be the mainstay of care for these patients’ local control and survival. Nonetheless, considering the use of adjuvant therapies on an individual basis is important, especially when the condition is unresectable. With a median survival of 6–12 months, the prognosis is still poor. Full surgical resection has improved survival when paired with radiation therapy and preoperative chemotherapy2 =91, her heart rate (HR) =130, her respiratory rate (RR) =30, and her blood pressure (BP) =90/50 with good air entry bilaterally, on auscultation. She has bronchial breathing on the right lower zone and bilateral basal crepitation, her jugular venous pressure was not elevated, and she has no lymphadenopathy or breast masses.A 41-year-old female of Middle Eastern ethnicity was presented to the emergency department in September 2018 by her family via ambulance. The patient was unemployed and had a past medical history of left leg fibro-osteoblastic sarcoma since 2015, which was treated with chemotherapy and radiotherapy. She had no other significant medical history and was not on regular medications other than painkillers. She also had an above-the-knee amputation of the left leg. There is no relevant family history reported. The patient is a nonsmoker and uses a wheelchair. She was complaining of a 3 days duration of shortness of breath associated with chest tightness, dry cough, progressive orthopnea, and paroxysmal nocturnal dyspnea. She has no associated chest pain, palpitations, or lower limb edema. On examination, she was conscious, oriented, and alert but in respiratory distress. She was desaturated with SpOAfter primary assessment with a thorough history and physical examination, an ECG was done and showed sinus tachycardia, X-ray showed bilateral apical infiltrate with air bronchogram, lower limb Doppler showed no deep venous thrombosis, her D-dimer was negative, and the labs including complete blood count showed WBC of 19 000 with a shift to the left, creatinine (Cr) =1.7 and troponin =30. She was transferred to the intensive care unit to keep under observation as she was desaturated and her blood pressure was low, and she was given levofloxacin, ceftriaxone, and sulfamethoxazole–trimethoprim because pneumonia was suspected, and septic work-up was done with pending results.2 (FiO2) =100%, and RR =16. Her vitals include BP =90/60, oxygen saturation =88%, and HR =130 despite her being on a maximum dose of norepinephrine of 30 μg. RR was increased to 20, PEEP increased to 14, and given 1000 cm3 running intravenous (i.v.) fluid. Her saturation improved to (99%) and she planned to decrease FiO2 and PEEP according to saturation. But her BP still did not improve; an urgent echocardiogram was done and showed a left atrial mass , and she was intubated. Her mechanical ventilation settings, tidal volume (TV) =380, positive end-expiratory pressure (PEEP) =10, fractional inspired OAfter the echocardiogram findings, the Cardiothoracic team consulted urgently and advised urgent surgery. The decision to proceed with the surgery was made after consulting the anesthesiologist and the cardiologist. The surgery was performed by a highly skilled and experienced cardiothoracic surgeon, who was assisted by a full staff of nurses, anesthesiologists, and other medical professionals. During the surgery, the chest was opened by a mid-sternotomy incision. After heparinization, aortic and bicaval cannulation was performed. Cooled blood antegrade cardioplegia was given after cross-clamping the ascending aorta. The left atrium was opened, and a huge tumor measuring 6×6 cm was resected en bloc Fig. . The mit2 =50%. A chest X-ray post-operation showed bilateral patchy opacities. She was kept on meropenem and sulfamethoxazole–trimethoprim. She was extubated in the morning of the second day post-operation; her vital signs were good without O2 support, and she had a good urine output. After 11 days of the surgery, the patient was in good general condition with a healthy dry sternal wound, so she was discharged with full instructions and to continue her management with the oncology center for possible chemoradiotherapy.On the day of surgery, the patient was in critical condition on noradrenaline, adrenaline, milrinone, and dopamine, and then we started weaning until we reached 10 μg of norepinephrine, and her situation improved and she became hemodynamically stable with a mean arterial pressure of 80. She was on mechanical ventilation: TV =500, RR =22, and FiO3 of normal saline; afterward, she had a BP of 80/60. Because of her low oxygen saturation, she was given oxygen via a facemask, and then her oxygen saturation went up to the 80s. Unfortunately, despite the resuscitative efforts, the patient died.After we discharged her, she did a chest computed tomography, which showed metastatic lung nodules. So chemotherapy was planned. Ten days after her discharge and before her chemotherapy session, her doctor referred the patient to us because of a turbid, moderate discharge from the sternal wound, which was treated successfully. Three months after her second discharge, she developed a general weakness of 2 days duration associated with loss of appetite, diarrhea without blood or mucus, and cough. She had no history of fever or vomiting; therefore, she was brought to the emergency room. On examination, she was pale, not responsive, and had pale extremities; she also had shallow breathing with oxygen saturation of 70% and tachycardia with HR of 137. She was managed as a case of hypovolemic shock initially, two i.v. lines were applied and given 1000 cm7. Autopsies only reveal primary cardiac tumors in 0.001–0.3% of cases, making them extremely rare9. Around 75% of primary heart tumors are benign, and 25% are malignant, and sarcomas make up around 75% of the malignant tumors8. Whereas the reported incidence of metastatic cancers, which ranges in published data from 1.5 to 20%, is 20–40 times higher than primary neoplasms10. The most prevalent histological subtypes include lymphomas, mesothelioma, fibromyxosarcoma, angiosarcomas, and rhabdomyosarcomas10. This variety in histological subtypes and morphology is attributed to their mesenchymal origin8.Sarcomas are diverse, clinically challenging tumors of the soft tissue and bone. Sarcomas are the second most prevalent type of solid tumor in children and adolescents and make up a significant subset of secondary malignancies, although makes up only 1% of all human malignancies. To date, more than 100 histological subtypes have been identified, and molecular profiling is revealing many more. Several sarcoma subtypes fall into the most difficult-to-treat categories due to their generally aggressive biological behavior, relative rarity, and prevalence at almost every anatomical site11. However, when left atrial masses, whether benign or malignant, cause symptoms, dyspnea due to mitral valve obstruction is the most common presentation12. In our case, the patient presented with cardiogenic shock, which is an unusual presentation of a cardiac mass and a life-threatening condition requiring rapid and aggressive management. This presentation diverted our attention away from the possibility of a cardiac mass and delayed the proper diagnosis until further investigations were conducted. The literature suggests that the presenting symptoms of cardiac tumors may mimic those of other heart conditions and depend largely on the location of the tumor and the extent of myocardial invasion rather than histology11.Malignant cardiac tumors are often asymptomatic until metastasis occurs and are typically detected incidentally on imaging studies12. Due to the similarities in presenting symptoms, it can be difficult to distinguish between benign and malignant heart tumors; nevertheless, differences mainly appear in histology and clinical course9. The presence of several masses, non-septal attachment of the mass, extensive attachment of the mass to the left atrial wall, extension into the pulmonary vein, and semisolid consistency are all clues that favor the diagnosis of sarcoma over myxoma12. The first-line imaging modality that provides critical data about mass location, shape, attachment, size, mobility, and hemodynamic impact is typically transthoracic or transesophageal echocardiogram (TTE and TEE)12.The initial clinical and radiologic characteristics of cardiac sarcomas could be confused with other benign cardiac masses, particularly cardiac myxomas, making the diagnosis particularly challenging9. Several experimental procedures are being applied due to the poor results of traditional therapeutic options. The most important of these is cardiac transplantation or autotransplantation, which can only be applied in cases of local invasion without distant metastasis, and their results are not yet fully understood11. The prognosis is quite poor, with a mean survival of 3 months to 1 year, due to the slow diagnostic process, the difficulties of treatment, and the high risk of metastasis12. Most sarcomas develop rapidly and kill their victims by widely invading the myocardium, obstructing blood flow through the heart, or spreading to distant organs11. Distant metastasis was reported in approximately half of the patients, with about 50% having lung metastasis, which made management much more challenging and decreased survival significantly9. Our patient was found to have lung metastasis after the resection of the primary tumor, so chemotherapy was started. However, her condition deteriorated rapidly, and she died after the second chemotherapy session.A complete surgical resection, which is only possible in less than 50% of patients, is the ideal treatment strategy. The local extension may still make resection incomplete or even impossible. However, neoadjuvant chemotherapy may reduce the tumor burden, making large tumors more resectableIn conclusion, primary cardiac sarcoma is a rare but life-threatening condition that can present with nonspecific symptoms and be easily mistaken for other cardiac masses. A thorough diagnostic work-up, including a multiparametric imaging approach, is essential to detect and characterize these tumors accurately. Surgical resection remains the mainstay of treatment, but it may not always be possible due to the extent of local invasion or the presence of metastasis. Therefore, neoadjuvant chemotherapy can be used to downsize the tumor and increase the possibility of resection. While radiation therapy is still controversial, autotransplantation may be a promising alternative approach for selected cases. Conventional heart transplantation is reserved for those patients who have been evaluated thoroughly and have no evidence of metastasis. Early diagnosis and aggressive management are crucial for improving the prognosis of primary cardiac sarcoma.The study is exempt from ethical approval in our institution.Written informed consent was obtained from the patient’s family for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.This research received no external funding.O.R.S.K., L.A.-K., and D.A.: study concept or design; O.R.S.K., B.M.M.O., L.K., and D.T.: writing the manuscript; O.R.S.K., B.M.M.O., and D.A.: review and editing the manuscript; D.A.: data collector; H.A.: supervision.The authors have no conflicts of interest to disclose.Not applied.Omar R.S. Khalil.Data supporting the study results can be provided, followed by a request sent to the corresponding author’s e-mail.Not commissioned, externally peer-reviewed.


A 6‐year‐old female spayed Great Dane was evaluated for acute onset cluster seizures. Magnetic resonance imaging (MRI) identified a mass in the olfactory bulbs with a large mucoid component caudal to the primary mass. The mass was removed via transfrontal craniotomy and histopathology revealed a tyrosine crystalline‐rich, fibrous meningioma with a high mitotic index. Repeat MRI at 6 months showed no detectable tumor regrowth. The dog is clinically normal with no seizures at the time of publication 10 months after surgery. This meningioma subtype is rare in humans. This unique meningioma occurred in a dog of younger age and uncommon breed for intracranial meningioma. Biological progression of this tumor subtype is unknown; however, growth rate might be slow despite the high mitotic index. CCDcharge‐coupled deviceDAPI4′,6‐diamino‐2‐phenylindoleER2Epitope Retrieval Buffer 2FLAIRfluid‐attenuated inversion recoveryGREgradient echoMRImagnetic resonance imagingPBSphosphate‐buffered salinePMMApolymethylmethacrylateT1WT1‐weightedT2WT2‐weightedTHtyrosine hydroxylaseWHOWorld Health Organization1Intracranial neoplasia occurs in 2.8% to 4% of dogs, and meningiomas comprise ~50% of brain tumors in dogs.22.1A 6‐year‐old female spayed Great Dane was presented to a private specialty hospital for acute onset of 3 generalized tonic‐clonic seizures that occurred within the 24‐hour period before presentation. There was no history of seizures, abnormal medical history, or potential toxin exposure. In retrospect the owners noted some mild lethargy in the weeks to months before the seizures began.On physical examination, the dog was tachycardic (140 beats/min) and mildly tachypneic (40 breaths/min). The rectal temperature was normal. Abnormalities were not detected on general physical examination. After initial recovery from the seizures, the dog had a normal neurological examination other than mild cervical hyperesthesia.2.2Anaplasma phagocytophilum, Anaplasma platys, Erlichia canis, Erlichia ewingii, Borrelia burgdorferi, and Dirofilaria immitis) were normal. Thoracic radiographs showed normal cardiothoracic structures and incidental bilateral osteophytosis of caudal glenoid cavities and humeral heads.Complete blood count (CBC) revealed a mild eosinophilia . Serum biochemistry revealed an elevated alanine aminotransferase , elevated aspartate transaminase , and elevated alkaline phosphatase . Urinalysis, total T4, and ELISA 4Dx snap test (Magnetic resonance imaging (MRI) of the brain (1.5 Tesla Siemens MAGNETOM Symphony) was acquired using T2‐weighted (T2W) sagittal, transverse, and dorsal planes; T1‐weighted (T1W) pre‐ and postcontrast transverse and sagittal planes and T1W postcontrast dorsal plane; and fluid‐attenuated inversion recovery (FLAIR) and gradient echo (GRE) in a transverse plane. MRI revealed a large olfactory bulb/frontal lobe mass with two distinct parts . A polymethylmethacrylate (PMMA) skull cap  was placed over the craniectomy defect before closure. There were no anesthetic complications, and the dog was discharged 48 hours after surgery with instructions for phenobarbital (1.8 mg/kg PO every 12 hours), gabapentin (5.4 mg/kg PO every 8 hours), cefpodoxime (7.2 mg/kg PO every 24 hours), and prednisone (0.35 mg/kg PO every 12 hours).2.42 . There was abundant deposition of extracellular and intracellular eosinophilic crystalloid material multifocally throughout the tumor, more numerous in some areas than others. These crystalloid deposits were primarily arranged in parallel with the fascicles in floret‐like arrangements with numerous petals that surrounded an elongated central core. The crystal aggregates varied widely in size with individual petals measuring 2 to 3 μm in size and large conglomerates measuring up to 30 μm in width. These crystals were strongly reminiscent of tyrosine‐like crystals, seen most commonly in salivary gland neoplasia. Many neoplastic cells contained small hypereosinophilic crystalline material.Histologically, the neoplasm exhibited a Schwannian arrangement where spindle cells were arranged in fascicular architecture and occasionally showed palisading of their nuclei Figure . Individ2.5m robotic staining system. Epitope retrieval was performed through application of Bond Epitope Retrieval Buffer 2 (ER2) for 20 minutes in conjunction with heat. Sections were then incubated with tyrosine hydroxylase  diluted in 0.1% Triton‐X containing phosphate‐buffered saline (PBS). Sections were stained for 4′,6‐diamidino‐2‐phenylindole  and mounted on glass coverslips with Prolong Gold Anti‐fade mounting media. Mounting media was allowed to cure at room temperature in the dark for 24 hours. Sections were stored at 4°C until imaged.Paraffin‐embedded canine brain tissue was sectioned at 5‐μm thickness and mounted onto polyionic slides. Slides were deparaffinized and tissue sections were immunofluorescently labeled on a fully automated Leica Bond RXFull section montage images were acquired on a fully‐automated motorized stage VS200 Olympus microscope equipped with a Hamamatsu ORCA‐Fusion camera and collected using Olympus CellSens software. Whole tissue section montages were generated by compiling ×200 images acquired by using an Olympus UPLX‐Apochomat ×20 (0.8 N.A.) air objective. High magnification inset images were acquired using an Olympus UPLX‐Apochromat ×60 oil immersion objective (1.42 N.A.) z‐stack with post acquisition deconvolution through Weiner method provided on the CellSens platform. All images were acquired simultaneously by a single investigator where charge‐coupled device (CCD) imaging parameters  were held consistent to reduce variability in detected fluorescence intensity.+ puncta, where positive staining is indicative of non‐nucleated red blood cells bordered by epithelial cells, apparent by the elongated DAPI+ nuclei. TH staining within animal tissue sections revealed multifocal extracellular tyrosine‐rich crystalline structures that were apparent in multiple ×60 high magnification imaging planes, confirming crystalline‐like diffraction patterns of intensity, that were not bordered by epithelial cells  administration was continued long‐term. Brain MRI was repeated 6 months after craniotomy Figure . There w3This case report describes a meningioma subtype exhibiting tyrosine rich crystals. Only 3 tumors of this subtype are reported in humans, including 2 intracranial and 1 spinal tyrosine‐rich meningioma.Canine and human meningiomas share many histologic and imaging similarities, and previous studies have attempted to apply WHO classification of meningiomas to dogs.Treatment of intracranial meningiomas can consist of surgical resection, radiation, and less commonly administration of chemotherapies such as hydroxyurea.Authors declare no conflict of interest.Authors declare no off‐label use of antimicrobials.Authors declare no IACUC or other approval was needed.Authors declare human ethics approval was not needed for this study.


Hypersensitivity reactions to the COVID-19 mRNA vaccines were identified in the initial 2020 trials. Appearance of a soft tissue mass is a rare manifestation of this hypersensitivity reaction. In this patient, bilateral injections resulted in the appearance of shoulder masses. Magnetic resonance imaging showed localized pseudo-tumorous edema in both shoulders, one subcutaneous and the other intramuscular. This is only the second case of a mass-like reaction to the COVID-19 vaccine mimicking a possible soft tissue neoplasm. Improper vaccination administration technique may have contributed to this complication. The case is presented to increase awareness of this potential pseudotumor.  Phase 3 trials of the mRNA-1273 (Moderna) COVID-19 vaccines found delayed injection-site inflammatory reactions known as type IV hypersensitive reactions in 0.8% of participants after the first dose and 0.02% of participants after the second dose . GranuloType IV hypersensitivity-related swelling is not the only vaccine related adverse reaction that may come to the attention of the orthopedic surgeon. Shoulder injury related to vaccine administration (SIRVA) is an uncommon vaccine injection complication that causes prolonged shoulder pain ,8. It isWe present a very rare case of the COVID-19 vaccine prompting the growth of an injection site pseudo-tumor. The case is presented to inform proper vaccine injection technique and to bring awareness to this rare clinical occurrence that may mimic soft-tissue tumors.A 53-year-old female presented with an 8-month history of firm, posterior-lateral, bilateral upper-arm soft-tissue masses. The location and temporal onset of the masses were directly related to her first COVID Moderna vaccine injection in her right shoulder and the second Moderna shot 1 month later in the left shoulder by her pharmacist. A discussion with the patient suggested that both vaccines were administered at approximately three finger breadths below the acromion. The patient experienced typical transient shoulder soreness in her right shoulder immediately after the first injection, which was unrelated to the soft tissue masses and associated symptoms. During her second injection, she did not have symptoms in her right shoulder, but she preferred to have the second injection in the left shoulder because she is right-hand dominant. Two months later, she developed bilateral injection-site pain, urticaria, decreased active range of motion, induration, and tenderness. The left shoulder had a less florid reaction than the right. The patient reported her pain as 5 out of 10 on the visual analog scale. The pain was described as a persistent discomfort which limited activities of daily living including driving, cooking, and work. These areas of localized swelling failed to resolve, despite treatment with heating pads and ice packs. No other trauma occurred, there was no history of underlying medical, oncologic, orthopedic, or rheumatologic conditions, and there were no prior known soft-tissue masses. She had no history of drug allergy or allergic comorbidities. There were no constitutional symptoms or adenopathy.Initially unconcerned of her symptoms, the patient took intermittent Tylenol for the pain and initiated a home exercise program. She had an appointment with her primary care physician (PCP) for an annual check-up 2 months after the symptoms had developed (4 months after completing the injections). Her PCP referred her to orthopedics due to concerns of bilateral shoulder pain and swelling of unknown etiology. She waited another 3 months before consulting orthopedics. By this time, her pain ceased but the bilateral swelling persisted. Her initial treating orthopedic surgeon ordered a right shoulder magnetic resonance imaging (MRI) to rule out malignancy on the more swollen side. The imaging showed abnormal regions within the subcutaneous tissue at the sites of the mass . SubcutaThree months after visiting the initial orthopedist, the patient attended a different orthopedic clinic to address the masses. At this visit, 10 months had passed since receiving her injections and the clinical examination showed normal range of motion with no tenderness to palpation. There were no rashes on inspection; however, edema and nodules to palpation were found in the lateral deltoid heads. Due to the persistent, previously unimaged left shoulder swelling, an MRI was performed, which showed similar findings of edema but in an intramuscular rather than subcutaneous location and the presence of a slightly enlarged axillary node . UltimatShe did not experience similar reactions to prior vaccines. The adverse reactions to the initial vaccine injections dissuaded the patient from receiving a recommended booster dose for COVID-19.Type IV hypersensitivity reactions following COVID-19 vaccination are known as “COVID arm” . DelayedSpecific vaccine components that elicit a granulomatous reaction were identified. In the pertussis vaccine, aluminum salt adjuvants were identified as the responsible ingredient that led to a reaction in 77% of children . While tWith respect to treatment, a type IV hypersensitivity granulomatous reaction, as presumed to have occurred in this case, typically resolves without medical intervention, possibly led by an in vivo reactive oxygen species-mediated cascade .Injection site granulomas were attributed to improper injection technique . The intOther than granulomatous hypersensitivity reaction, a neoplasm and SIRVA were also considered in the differential diagnosis for this patient. In any presentation of an unexplained soft tissue mass or localized swelling, a neoplasm must be considered in the differential. Arguing against that in this case, however, were the bilaterality, temporal association with vaccination, and relatively diffuse nature of the process on each side. Ultimately, imaging excluded this possibility, and so, no biopsy was performed. Neoplastic etiology was unlikely, and we believed that this inflammatory reaction led to the appearance of pseudo-tumors that could be mistaken for a neoplasm.SIRVA is attributed to incorrect injection technique with injection into the shoulder joint, rotator cuff, subacromial bursa, or regional peripheral nerves ,8. The dTo prevent these rare complications, proper injection techniques should be used. The deltoid is the most common IM injection site due to easy exposure of the overlying skin . DeltoidAdverse reactions to vaccines are usually mild and transient. Unfortunately, several cases of prolonged symptoms were reported, possibly linked to improper injection technique. A neoplastic condition should be considered in the differential diagnosis when swelling occurs post-vaccination, but imaging distinguishes this inflammatory response from a potential tumor. The information presented in this case may be used to inform providers on proper injection techniques to decrease the incidence of vaccine complications. In addition, orthopedic oncologists should be aware of pseudo-tumors following vaccination before ordering biopsies. While SIRVA or type IV hypersensitive reactions are possible, their uncommon occurrence should not deter patients from obtaining the recommended vaccinations for COVID-19. An initial reaction is not a contraindication to subsequent boosters.


Conjunctival melanocytic tumours comprise benign and malignant neoplasms. Nevi are congenital benign conjunctival melanocytic tumours. Primary acquired melanosis (PAM) can either be regarded as benign (PAM without atypia) or represent a precancerous lesion (PAM with atypia). In contrast, melanoma is, by definition, a malignant melanocytic tumour. This is a case of a 22-year-old female with a mass in her left eye (A). On slit lamp examination, the nevi were detected as shades of brown of size 5mm x 3mm located at the interpalpebral conjunctiva near the limbus involving the cornea at three o’clock, separated by normal conjunctiva. Increased blood supply and adherence to the underlying sclera were noted (B). Nevi can change colour and size during adolescence, but if a change is seen in an adult, a suspicion of malignancy should be ruled out. The malignant transformation risk is 1%. Inspection of all ocular structures was done as multicentric lesions could be present. It was excised and submitted for a histopathological investigation that confirmed the diagnosis (C). There is a social stigma about conjunctival nevus as it is cosmetically appealing. Surgical excision with an amniotic membrane graft or mitomycin C or conjunctival autograft is the mode of treatment, though the corneal opacity cannot be reversed.


Objective: Bladder lesions like urothelial carcinoma are rare in the first two decades of life. A biopsy of the bladder or urinary cytological examination is seldom required. Gross painless hematuria is the most relevant clinical syndrome. Methods: A retrospective analysis of surgical pathology records collected between 1984 and 2014 at our institution was performed in a search for cases of urothelial neoplasms originating within the urinary bladder in pediatric patients. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization (WHO) classification system. We selected keywords such as bladder neoplasia, bladder lesion, urothelial neoplasia, rhabdomyosarcoma, and children. In addition, we describe clinical presentation and diagnostic procedures as well as treatment and follow-up of two patients. A review of the literature was performed to analyze recommendations concerning diagnostic staging, treatment, and follow-up examinations as well as surveillance of urothelial tumors in the pediatric population. Results: Screening the pathology database of the Institute of Medical Genetics and Pathology of the University Hospital Basel between 1988 and 2014 yielded 287 samples involving the urinary bladder, 110 autopsies, 135 biopsies, and 42 cytology specimens. Of these, most samples originated from malformations and inflammation. Only five were tumors: two were urothelial tumors and three were rhabdomyosarcomas. The majority of specimens comprised resections of the diverticula or distal ureter. Our case reports include two patients with a urothelial tumor. Among the urothelial tumors, one was a papillary urothelial neoplasm of low malignant potential (PUNLMP). Painless hematuria was the directing clinical symptom. The tumor was investigated by FISH, and a 9p21 deletion was found. The second tumor-like lesion was a fibroepithelial polyp arising from the bladder neck. Conclusions: Bladder tumors in children are rare and mostly consist of urothelial and mesenchymal neoplasms. Rhabdomyosarcoma is the most common malignant bladder tumor in childhood. Similar to adult urothelial neoplasms, the loss of 9p21 is also implicated in urothelial neoplasms in childhood. Despite an increasing number of case reports and small series published within the last 2 decades, general treatment protocols including recommendations for staging, tumor markers, and follow-up examinations are still not yet available for this tumor entity in the pediatric population. Bladder tumors, especially urothelial carcinoma, represent a rare entity in the first two decades of life. Under the age of 20, they are distinctly unusual with an incidence of 0.1 to 0.4%; most of them are described in case reports and small series ,14,15,16A retrospective analysis of surgical pathology records collected between 1984 and 2014 at the Institute of Medical Genetic and Pathology of the University Hospital Basel was performed. We applied keywords such as bladder neoplasia, bladder lesion, urothelial neoplasia, rhabdomyosarcoma, and children. The age group “children” was defined from newborn to 16 years of age. We identified five pediatric patients with a tumor. Two out of five patients underwent transurethral resection of their tumor. Clinical presentation, applied diagnostic procedures, treatment, and follow-up of these two patients are described in detail.A review of the recent literature was performed to analyze recommendations for diagnostic staging, treatment, and follow-up examinations as well as surveillance of patients suffering from these lesions in the pediatric population.We performed a retrospective analysis of the pathology database at the Institute of Medical Genetic and Pathology of the University Hospital Basel, considering samples collected between 1988 and 2014 (corresponding period comparable to other studies). We identified 287 samples involving the urinary bladder, 110 autopsies, 135 biopsies, and 42 cytology specimens. Most of these samples originated from  malformations and inflammation. The majority of specimens comprised resections of the diverticula or distal ureter. Only five were tumors: two were urothelial tumors and three were rhabdomyosarcomas.Among the urothelial tumors, one was a papillary urothelial neoplasm of low malignant potential (PUNLMP). The tumor was investigated by FISH, and a 9p21 deletion was found (patient No. 1). The second one was a fibroepithelial polyp arising from the bladder neck (patient No. 2).A 13-year-old boy presented with one episode of painless macrohematuria. The patient’s history before presentation was completely uneventful. The family’s history was negative for bladder neoplasia. There was no traveling to foreign countries and no exposition to well-known risk factors like cigarette smoking or occupational exposures such as aniline dyes, phenacetin, and low fluid consumption. Clinical examination demonstrated no significant abnormality, without any evidence of trauma or manipulation at the external genitalia. Blood tests including coagulation status as well as urinalysis including bacteriology revealed no pathological result.Abdominal ultrasound showed a papillary mass measuring 1.5 cm in diameter, located on the lateral bladder wall close to the left ureteral ostium . An abdoWe performed transurethral cystoscopy which confirmed a papillary tumor close to the left ureteral ostium . The tumHistopathology showed a papillary urothelial tumor with focal urothelial atypia. Immunohistochemistry revealed an increased proliferation up to the upper urothelial layers . In addiThe postoperative course was uneventful. Follow-up examinations included urine cytology, abdominal ultrasound, and cystoscopy including application of 5-ALA (fluorescence marker) after 3 months. Both examinations showed negative results. Urine cytology and abdominal ultrasound were repeated after 6 months and after 12 months. Afterward, annual follow-up included urine cytology and abdominal ultrasound for more than 7 years. No further routine cystoscopy was performed. Long-term follow-up for 120 months did not reveal any recurrence.A 4-year-old boy presented to our emergency room with two episodes of acute urinary retention within the last 48 h. The parents reported a history of several months of recurrent voiding disorders. Two years before the current admission, the boy presented for the first time with painful micturition and acute abdominal pain. At that time, clinical examination demonstrated mild abdominal tenderness, no other significant abnormality, no phimosis, and no evidence of trauma or manipulation at the external genitalia. Blood tests including coagulation status as well as urinalysis including bacteriology revealed no pathologic result. Abdominal and renal ultrasound revealed no pathologic finding. Afterward, the parents observed at least 10 additional episodes with similar symptoms within the last two years. Due to the recurrent painful episodes, the patient was scheduled to visit a pediatric urologist to discuss the indication for circumcision. At the current admission, clinical examination demonstrated a palpable, painful mass in the lower abdomen, but no other significant pathology and notably no phimosis. Again, blood tests including coagulation status as well as urinalysis including bacteriology revealed no pathologic result. Abdominal ultrasound showed a solid mass measuring 1.5cm in diameter, located close to the bladder neck . We perfHistopathology showed a fibroepithelial polyp  covered The postoperative course was uneventful. After 3 days, the transurethral catheter was removed. The patient micturated spontaneously without any pain, and an ultrasound showed no significant residual urine. Follow-up examinations demonstrated normal uroflowmetry and abdominal ultrasound after 3 months. Uroflowmetry and abdominal ultrasound were repeated after 6 months and after 12 months, again with negative results. Long-term follow-up to 48 months did not reveal any recurrence.Bladder tumors are still rare in the first decades of life ,14,15,16The most important onset symptom was predominantly gross painless hematuria, followed by dysuria, isolated or recurrent. Painless hematuria as a directing symptom should never be underestimated. This observation is in accordance with the experience of nearly all other authors. Abdominal ultrasound was almost the first investigation and confirmed the diagnosis of a urothelial tumor in the great majority of patients. Transurethral resection is considered as the standard procedure to remove the tumor, performed by the majority of pediatric urologists during the same procedure. However, some authors perform transurethral biopsy followed by transurethral tumor resection in a second step ,16. Due to the different etiology of our two patients, we will discuss these two entities in detail below. In addition, we amend a review of the recent literature to analyze recommendations for diagnostic staging, treatment, and follow-up examinations as well as surveillance of patients suffering from urothelial tumors (excluding RMS) in the pediatric population.Noninvasive papillary neoplasms (stage pTa) represent approx. 45% of all bladder tumors . The proFGFR3-associated pathway, and the p53-associated pathway [FGFR3 mutations are usually present in low-grade papillary carcinomas, whereas high-grade carcinomas are characterized by p53 mutations [According to current understanding, bladder cancer is thought to arise from at least two different pathways: the  pathway . FGFR3 mutations . FGFR3 mutations ,22.p53 gene product overexpression is common in bladder cancer in young patients. Alteration of the p53 gene is thought to be strongly associated with PUNLMP, although the pathogenetic mechanism is still unclear. Kalantari et al. showed that nuclear p53 protein in invasive high-grade transitional cell carcinoma (TCC) was slightly more frequent than that in noninvasive low-grade papillary TCC [p53 in papillary low-grade TCC and invasive high-grade TCC and the lack of p53 expression in PUNLMP indicate that mutations of the p53 gene are not usually associated with the development of urothelial neoplasms and may play a crucial role only in progression of PUNLMP to a higher-grade TCC [Some molecular markers have been evaluated for the differential diagnosis of low-grade urothelial tumors, but there is no consensus regarding their usefulness or clinical implications. Immunohistochemical evidence of lary TCC . Only terade TCC .Other genes and/or factors whose involvement is hypothesized are fibroblast growth factor-3 receptor (FGFR3) mutations as well as loss of heterozygosity (LOH) on chromosomes 3, 7, 9p, 9q, and 17p . HoweverUrine cytology is a minimally invasive method of following adult patients. However, it must be kept in mind that the sensitivity decreases in well-differentiated tumors. Additionally, there is little experience reported with cytology in children ,26. FurtDefinitive diagnosis of PUNLMP is performed by cystoscopy, which also allows evaluation of tumor extension, excision, or biopsy for histopathologic evaluation. As PUNLMP is generally solitary, non-muscle-invasive, and a low-malignancy tumor, its treatment is oriented to local excision only, and the prognosis is excellent in pediatric patients. Transurethral resection is considered as the “gold standard” procedure, performed by the majority of pediatric urologists during the same procedure. Recurrence or progression is uncommon in the young population . In contA designation of PUNLMP avoids labeling a patient with “cancer” but does not equate these lesions with benign papillomas. The review by Fine et al. reclassifies the bladder lesions in a series of 23 patients with ages ranging from 4 to 20 years [Due to the ongoing controversial histopathological nomenclature and the fact that PUNLMP represents still a rare entity in children, there is still no validated protocol or recommendation concerning preoperative staging as well as standardized follow-up examinations. Gross painless hematuria is the most relevant clinical syndrome. There is no doubt that abdominal ultrasound is the first investigation, performed by an experienced pediatric radiologist with a high specificity. However, there is no common consensus about any additional routine imaging before transurethral resection. In accordance with our oncology team, we performed preoperative computer tomography (CT) and MRI of the abdomen which showed no evidence of localized tumor spread and/or metastatic disease. Indication for thoracal CT, PET scan, and skeletal scintigraphy was discussed, but after intensive interdisciplinary discussion, we decided to refrain from performing these examinations. Costs and in particular risks of exposure to radiation have to be balanced against disease likelihood in pediatric patients.Furthermore, there is still no standardized protocol for follow-up examinations available. The intensity of follow-up should be proportional to the risk of disease recurrence or progression. Patients with PUNLMP are often still followed for recurrences, in a manner comparable to those with a diagnosis of urothelial carcinoma. The best method of following these young patients after transurethral resection has been debated. Some authors advocate careful and extensive follow-up with interval cystoscopy as the primary modality. In contrast, others argue that ultrasound is extremely effective in identifying bladder tumors, and they recommend using ultrasound for initial diagnosis and disease surveillance. Adapting protocols from adult oncology, urine cytology is often a routine part of the follow-up program. Our first follow-up examinations after 3 months included urine cytology, abdominal ultrasound, and cystoscopy including application of 5-ALA, as recommended in the literature . All exaIn our series, PUNLMP was less common than RMS, which is in contrast to the results of Alanee et al. . TherefoA fibroepithelial polyp (FEP) of the urinary bladder is a very rare benign congenital lesion in the pediatric population. An FEP is considered to be a non-neoplastic condition ,6. The mSince an FEP is considered as a benign lesion and no reports of recurrence and/or malignant transformation are known, recommendations for follow-up are different in comparison to PUNLMP. Follow-up examinations in our case included uroflowmetry and abdominal ultrasound after 3 months with negative results. Uroflowmetry and abdominal ultrasound were repeated after 6 months and after 12 months, again with negative results. After long-term follow-up for 48 months without any evidence of recurrence, scheduled follow-up was ceased, in accordance with recommendations of the literature [We report on a retrospective analysis from a single-center database, finally corroborating our hypothesis with only two case reports. However, with our data, we could confirm the experience mentioned in the recent literature reported from high-volume centers including a larger number of pediatric patients.Both the review of the recent literature and the retrospective analysis of our surgical pathology record database confirm that bladder tumors in children are still rare and mostly consist of urothelial and mesenchymal neoplasms. In our series, RMS was the most common malignant bladder tumor in childhood. Urothelial lesions like PUNLMP as well as FEP are still rare in the pediatric population. In pediatric urothelial neoplasms, similar to those in adults, loss of 9p21 is implicated. The most important onset symptom is predominantly painless hematuria followed by dysuria, isolated or recurrent. Abdominal ultrasound is the first-line method to confirm the diagnosis of a urothelial tumor, and transurethral resection is the standard procedure to remove the tumor. General treatment protocols including recommendations for staging, tumor markers, and follow-up examinations are still not yet available for this tumor entity in the pediatric population. Implementation of a central international database would enable us to develop a standard operating procedure for diagnosis, treatment, and follow-up of these patients.


Bowel obstruction is a common cause for the acute abdomen with different aetiologies that shapes subsequent management plans. Small bowel obstruction often develop due to intra-abdominal adhesions in patients with prior abdominal surgery and for large bowel obstructions, more commonly due to tumours and lesions. Disruptions to normal intra-abdominal anatomy as seen in pancreatic–kidney transplantation or kidney transplant alone can result in increased risk of bowel obstruction—especially if the donor graft is implanted within the intraperitoneal plane. We present two patients from separate institutions with history of simultaneous pancreas–kidney (SPK) transplantation (Patient 1) and intraperitoneal renal (Patient 2) transplant whom both presented with bowel obstruction requiring surgical intervention. Given the specificity and operative intricacies of our cases, we aim to present our findings and surgical management of these rare presentations in hopes of increasing awareness to this uncommon but significant cause of bowel obstruction in a transplant patient. Bowel obstruction is a relatively common cause of abdominal pain and presentation to the emergency department. It is associated with a high risk of perforation and is a major cause of morbidity and mortality , 2. TreaA 47-year-old female presented to the emergency department with severe abdominal pain, nausea, and vomiting that developed acutely overnight. She had recently been discharged from hospital 4 days earlier, following an admission for urosepsis secondary to ureteric obstruction of her renal transplant graft. In that admission, she was treated with intravenous meropenem, percutaneous nephrostomy, and stenting of her transplanted ureter.Her background medical history included type 1 diabetes mellitus with significant micro and macrovascular complications, involving profound gastroparesis and diabetic nephropathy which she received an SPK transplant 2 years prior for end-stage renal failure. Her past surgical history is notable for extensive peripheral vascular disease resulting in a right below knee amputation as well as diabetic retinopathy with bilateral cataract surgeries. Her regular medications included triple immunosuppression with prednisolone, tacrolimus, and mycophenolate.The patient reported feeling generally well since her recent hospital discharge, denied any pain, fevers or urinary symptoms, and tolerated substantial oral intake. However, she had not opened her bowels for 4 days. On arrival to hospital, she was hypotensive, tachypnoeic, and hypothermic with a Glasgow Coma Scale score of 11. Initial examination demonstrated cool and clammy peripheries with no palpable radial pulse. Her abdomen was grossly distended and diffusely tender on palpation. Immediate resuscitation was commenced with intravenous fluids, antibiotics, and a noradrenaline infusion was started.9/l, haemoglobin (Hb) of 100 g/l, c-reactive peptide (CRP) of 15 mg/l and arterial blood gas showing a serum lactate of 0.6 mmol/l and an acidemia with pH of 7.26. Bedside urinalysis demonstrated leucocytosis and microscopic haematuria. Computed tomography (CT) scan of her abdomen and pelvis revealed diffuse dilatation of the large bowels with a clear transition point at the rectosigmoid junction  of 5.2 × 10junction . There wA decision was made for urgent surgical intervention due to her rapidly deteriorating haemodynamic status despite appropriate fluid resuscitation.The patient underwent a midline laparotomy which revealed extensive adhesions requiring significant dissection in order to adequately visualize and assess the viscera. Intraoperative examination revealed large volumes of turbid serous fluid in the abdominal cavity. Identification of the transplanted pancreas showed its enteric anastomosis with the proximal small bowel, a venous anastomosis to the right external iliac vein, and an arterial anastomosis to the right common iliac artery. The transplanted kidney was situated in the left iliac fossa and was anastomosed to the left external iliac vein and the left common iliac artery. The transplanted ureter was anastomosed to the dome of the bladder and given its anatomical placement, was situated intraperitoneally .in situ. On discussion with her transplant surgeon, a de-functioning loop colostomy was created.The colon was visibly dilated and macroscopically appeared viable with no external evidence of ischemia; however, on-table flexible sigmoidoscopy revealed an acute angulation at the rectosigmoid junction with evidence of mucosal ischemia distally . ImmediaPostoperatively, the patient was transferred to the intensive care unit for a further 4 days before being stepped down to the ward. She recovered well and remained in hospital for another week with ongoing stoma education before being cleared for discharge home. The patient returned 6 weeks later for a planned stent exchange, which proceeded without any issues. Her colostomy was functioning normally, and she had no further symptoms since her discharge. Her ongoing follow-up was transferred back to her usual transplant surgeon and renal physician, and a joint decision was made to keep her colostomy.A 71-year-old male was referred in by GP for sudden onset of colicky lower abdominal pain associated with bilious vomiting and fevers. This was on background of a live right renal transplant 14 years ago for reflux nephropathy. The transplanted ureter was anastomosed to a pre-existing ileal conduit that was previously fashioned due to a congenital hypoplastic kidney in the right iliac fossa. His other medical history was notable for significant ischaemic heart disease, peripheral vascular disease, and recurrent skin cancers in context of his long-standing immunosuppression. On initial examination, his abdomen was noticeably distended and tender centrally with localized peritonism. His ileal conduit was pink and appeared healthy, however had markedly reduced urine output.9/l, serum lactate of 6.6 mmol/l, and severe decline in renal function as demonstrated by acute rise of serum creatinine to 285 umol/l (baseline 103 umol/l). A subsequent CT scan showed substantial hydronephrosis and hydroureter of the transplanted kidney with distended small bowel loops established in close proximity to the ureteric-ileal conduit anastomosis with an identifiable transition point (His full blood count and biochemistry showed a significant leucocytosis with a WCC of 36.2 × 10on point . InitialThe patient underwent an emergency laparotomy which revealed a transition point caused by significant adhesions of the mid-small bowel that was entrapped between the ileal conduit and its anastomosis to the stretched transplanted ureter. Intraoperative examination showed a grossly dilated ureter with marked tension at the site of small bowel adherence but fortunately no evidence of ischemia. Given the ureteric connection with the ileal conduit, the ureter was anatomically positioned intraperitoneally at the time of operation. Dilated small bowels were encountered proximally with extensive intra-abdominal adhesions but no compromised bowel that required resection.Escherichia coli urosepsis, which contributed to his increased vasopressor support, and was treated with intravenous meropenem under the advice of infectious disease specialists. He was soon after stepped down from ICU and made a uneventful recovery in hospital with a resolution of infective and obstructive symptoms, and discharged on Day 7 postoperation with a creatinine of 153 umol/l, eGFR 39 ml/min, and WCC of 15.2. He was followed-up with multiple outpatient appointments with no other persisting complications and on repeat bloods 1-year postsurgery had a normalized creatinine of 99 umol/l.The patient was subsequently admitted to the ICU postoperatively with ongoing vasopressor requirements augmented by intravenous hydrocortisone for stress dosing. His course was complicated by multiresistant SPK or individual kidney transplantation are widely recognized management options for patients meeting specific clinical criteria usually involving end-stage renal failure and type 1 diabetes mellitus . TransplIn most SPK transplantations, there is preferential use of the intraperitoneal approach for graft implantation, the pancreas is usually transplanted to the right iliac fossa, and the kidney to the left iliac fossa , 12. TheObstruction of the large or small bowel can be a surgical emergency and is a significant cause of morbidity and mortality accounting up to 15% of all hospital admissions for the acute abdomen . The aetet al. [Bowel obstruction involving transplanted renal grafts is very rare and scarcely documented within the literature. Tovmassian et al.  reportedet al. , 18 furtWe report two unique cases of LBO and SBO in patients that have undergone organ transplantations. Due to the intraperitoneal location of the graft ureter post transplantation, a bowel obstruction ensued requiring surgical intervention to resolve. Both patients were followed up postoperatively and have recovered exceptionally well.


Rare developmental anomalies known as urachal remnants are brought on by flaws in the foetal developmental process. However, depending on the location and degree of incomplete obliteration, the urachus can undergo a variety of urachal anomalies. An umbilical fistulogram and a voiding cystourethrogram both supported the existence of the adult urachal cyst in this case. To treat the sepsis, we provided the patient with antibiotics first, then a surgical procedure. The entire vesico-umbilical tract with the urachal cyst was removed using the open approach. The excised specimen's histology revealed a foreign body giant cell reaction without any indication of malignancy. The presentation and diagnosis of vesico-umbilical urinary fistula (VUUF) in adults can occasionally be difficult. They happen very rarely. So we began putting forward this case for the same reason. Urachal anomalies are uncommon, but cases have been frequently reported in children and extremely infrequently in adults . During A 48-year-old male patient came in with the main complaint that urine production from the umbilicus had been like a jet for the past three months, with no difficulty emptying the bladder through the urethra. Three days ago, symptoms began, including a fever, colicky-like pain in the hypogastrium, and a thick, yellowish-white discharge with pellets through the umbilicus. These symptoms subsided with symptomatic treatment; there was no prior history of vomiting, nausea, or changes in bowel habits. No abnormalities were found after a systemic review. He was hemodynamically stable upon examination. Upon abdominal examination, it was discovered that the patient had urine dribbling from the umbilicus Figure , mild suA one-week course of antibiotics was administered to the patient, and open surgery was scheduled upon completion of this time. Before surgery, the bladder was saline-distended and clamped using Foley's indwelling catheter. Under spinal anaesthesia, a sub-umbilical midline incision was made through the skin and subcutaneous fascia, keeping the peritoneum unsevered. By blunt dissection of the apex of the bladder, the attachment of the urachus was identified and separated extraperitoneally. Through a racket incision around the umbilicus, the entire tract was separated and dissected. The urachus was divided at the apex of the bladder, and the cut end of the urachus was clamped. The opening of the bladder was closed extramucosally with a chromic 1-0 continuous suture in two layers. The entire urachus tract, including the umbilicus, was excised along with the urachal cyst Figure . HaemostUmbilical remnants develop when the foetal bladder and allantois are partially or completely obliterated. Complete communication between the bladder and the umbilicus is indicated by a patent urachus. The remnants found in neonates younger than six months usually resolve spontaneously without the need for surgery . UrachalIn our case, urine flowed out as a jet from the umbilicus along with the normal micturition from the urethra. This is not secondary to a distal obstruction. This was a very rare phenomenon. There was no suspicion of malignancy with a negative history of hematuria. During primary diagnosis, ultrasound, CT, and MRI were performed to determine the size of the cyst and its relationship with the peripheral tissues. In the presence of umbilical discharge, an umbilical fistulogram was performed to confirm the existence of the fistula, and a voiding cystourethrogram was performed for the reflux. With the above symptoms, signs, and other investigation reports, the case was primarily diagnosed as a patent urachus cyst with a vesico-umbilical urinary fistula. The treatment of choice for the above diagnosis is a complete primary excision in a single-stage approach. The open surgical approach is advantageous over the laparoscopic approach, in which there is a chance of incomplete excision and exploration, local hematoma, entry into the peritoneal cavity, and complications of peritonitis. The surgical approach is necessary for the confirmation of the diagnosis and the need for excision . HistopaA patent urachus is an extremely rare urachal remnant. It usually presents in the neonatal period with urinary discharge from the umbilicus. In our case, the extraperitoneal approach was preferred instead of laparotomy to avoid the risk of recurrences in the laparoscopic approach and attendant laparoscopy complications. Umbilical cord cysts and large umbilical cords should be evaluated carefully to distinguish between coexisting conditions. We present a case in which an adult with a patent urachus fistula was successfully treated.


Infantile fibrosarcoma (IF) is a rare malignant fibroblastic tumor that affects infants and young children, occurring most commonly in the extremities. Here, we present a 14-year-old patient with an abdominal mass incidentally detected after a blunt injury to the abdomen. The initial trauma protocol CT revealed a high attenuation mesenteric lesion in the left central abdomen suggestive of mesenteric hematoma. However, the possibility of a solid neoplastic mass lesion could not be excluded. Further evaluation with dynamic contrast-enhanced serial MRI showed a progressive enhancing mass and excluded a hyperacute hematoma with active bleeding. The mass was resected, and histopathological examination and molecular analysis of tumor cells were consistent with a high-grade fibrosarcoma with KMT2D : BCOR fusion. MRI is a highly sensitive and specific imaging modality and widely considered the modality of choice for evaluating soft tissue tumors . DespiteA 14-year-old patient with no significant past medical history presented to the emergency room after an all-terrain vehicle (ATV) accident. The patient had a handlebar injury to the abdomen. The physical examination revealed tenderness in the abdomen.Lab investigation showed anemia (Hb = 11.3). However, all other hematological and biochemical analyses were normal and stable. An abdominal CT scan with IV contrast  was perfThe following day, the abdominal MRI redemonstrated an intraperitoneal mass in the left upper quadrant  without Laparoscopic surgery was performed, which showed that the primary mass was between the greater omentum leaves that extend between the transverse colon and the stomach. Without evidence of mesocolon, stomach, or colon invasion, a successful resection of the mass was executed. An omental satellite lesion measuring 1.5 cm near the larger mass was excised, including two additional subcentimeter lesions confirming observations seen on MRI. Total blood loss was 10 ml, and the patient was discharged the day after surgery, indicating a rapid and uneventful postoperative recovery.A gross examination of the resected tumor (9 × 5.5 × 4 cm) revealed a nodular soft tissue mass with surrounding membranous tissue, focally haemorrhagic cut surfaces with areas of necrosis and possible calcifications. Microscopy revealed a lobulated cellular tumor with interlacing bundles of monotonous spindle to ovoid cells, loose myxoid areas, extensive necrosis, and focal dystrophic calcifications , involviImmunohistochemistry analysis showed that the tumor cells were positive for beta-catenin (nuclear staining), inhibin, bcl2, and PanTRK, while PAX8 demonstrated a patchy positivity of some spindle cell populations. Most other type and site-specific markers were negative. Since the NTRK-rearranged tumors usually show positivity with PanTRK antibodies , the molth edition classification system was stage III A . Three weeks after the FDG-PET/CT, the patient received 3-days of ifosfamide and doxorubicin induction therapy, with concurrent mesna and dexrazoxane administration.FDG-PET/CT performed two months after surgery revealed 5 foci of tracer uptake in the left upper quadrant and pelvis consistent with hypermetabolic mesenteric tumor implants consistent with previously undetectable deposits and/or disease progression. There was no detectable metastatic disease. Clinical staging as per the AJCC 8A case report of a 14-year-old child with abdominal high-grade fibrosarcoma was presented in this study. Infantile fibrosarcoma is a rare malignancy that metastasizes infrequently among infants and young children and constitutes less than 1 percent of all childhood cancers. In contrast, the adult form is highly invasive and can affect older children, usually between 10 to 15 years old. Adult fibrosarcoma accounts for nearly 7% of all sarcoma diagnoses . This tyIn our case, the initial CT findings suggested that the detected mass was more likely to be a traumatic hematoma as the patient was involved in an ATV crash and experienced blunt abdominal trauma. Acute mesenteric hematomas can have well-defined margins and attenuation ranging from 40 to 70 HU , like ouTo further confound interpretation, differentiating hematomas from soft tissue tumors on noncontrast sequences can be challenging given the variable appearance and signal intensities in hematomas depending on the stage of metabolism –16. In tNTRK fusions, such as infantile fibrosarcoma [In histologically examining the tumors with fibrosarcoma-like patterns, malignant fibroblasts are the predominant cell type with variable collagen production levels. Given the morphologic heterogeneity of these tumor types, diagnosis is often based on immunohistochemical and molecular diagnostic markers . In our osarcoma . Howeverosarcoma .The role of FDG-PET/CT in diagnosis of soft tissue sarcomas is not well established as there remain challenges in differentiating malignant from benign lesions . HoweverIn conclusion, we report a rare case of a primary high-grade mesenteric sarcoma incidentally identified on initial CT evaluation following traumatic injury to the abdomen. Due to the potential overlapping appearance between neoplasms and hematomas, this case illustrates the importance of dynamic contrast-enhanced serial MRI as a problem-solving tool when the less intuitive diagnosis of neoplasia is considered in the post-traumatic setting.


Small bowel obstruction (SBO) is common in daily practice in emergency department. It has many causes such as adhesions or hernias. Other causes are obstruction by a bulky mass such as tumors of the intestine.We present a rare case of small bowel obstruction in a 48 years old male patient with Crohn's disease. Laparotomy showed a mass that caused the obstruction. Pathology report demonstrated that it is leiomyoma. This finding may cause a delay in making the diagnosis of SBO.Small bowel obstruction is considered an emergency case in the emergency. It needs special attention and advanced medical practice to reach the diagnosis. Rare cases may lead to delay the diagnosis because they present unusually.Physicians should consider leiomyoma as a cause of SBO with patients without clear cause of SBO. •Small bowel obstruction (SBO) is a condition with many symptoms, including constipation, vomiting, and abdominal distension.•Leiomyoma’s (fibroids) are benign smooth muscle tumors of the uterine myometrium.•Small bowel obstruction with leiomyoma is considered a rare entity and drain special attention.•Accurate monitoring and imaging can serve to reach the definitive diagnosis and avoid bowel ischemia, perforation, peritonitis, and sepsis. Postoperative adhesions (60 %) are the most common cause of small bowel obstruction, followed by malignant deposits or extrinsic compression of the small bowel by other primary malignancies (20 %) and intestinal hernias 10 %) [0 % [1].Intraluminal causes of small bowel obstruction in adults are rare.Emergent surgery is necessary for patients with clinical or radiological signs suggestive of bowel ischemia. Even with the advent of laparoscopic surgery, intra-abdominal adhesions remain a significant cause of SBO, accounting for 65 % of cases, among other etiologies such as hernias, neoplasms, and Crohn's disease. Adhesive SBO (ASBO) contributes substantial financial burden, need for hospitalization, time away from routine life activities, and psychological impact .Here, we report a rare case of small intestinal obstruction caused by submucosal leiomyoma in a patient with previously diagnosed with Crohn's disease.We declare that our work has been reported in line with the SCARE criteria .2A 48-year-old male patient presented with three days of progressive generalized abdominal pain, bloating, and weight loss. He was diagnosed in another hospital with Crohn's disease 25 years previously, which was controlled poorly. Vital signs were as follow: blood pressure was 113/60 mm Hg, pulse was 98/min, temperature was 38.1 C0, and respiratory rate was 22/min.On physical examination, there were abdominal tenderness and distension without rebound or guarding. There were no hernias or abdominal scars. Other systems were normal. Laboratory findings on admission are shown in We started with intravenous antibiotics and resuscitation with normal saline. We inserted a nasogastric tube for bowel relief. We monitored vital signs hourly.On the next day of admission, we decided to perform an exploratory laparotomy under general anesthesia because of the increase in abdominal pain.Laparotomy was performed, through a mid-line incision. There was minimal intraperitoneal serous fluid and 25 cm. Proximal to the mid portion of the jejunum; we found a severely dilated loop with a palpable mass after the dilation. We resected this segment containing the mass and it was sent to the pathologist. Stapled anastomosis was carried out. We close the incision with 2-0 simple nylon sutures.Histopathology showed 2.5 × 3 cm submucosal leiomyoma . The couOn day 5 after surgery, we discharged him with no complaints. He presented to the out-patient clinic for follow-up after 13 days of discharge. He had no symptoms, and he could eat more food with good defecation. His wound showed healing well, so we removed the sutures. We sent him to complete his treatment of Crohn's disease with his gastroenterologist.In addition, he was advised to stop smoking completely, reduce the use of NSAIDs, and increase the intake of fibers in his meals .Fig. 3Hi3While SBOs are a known complication of Crohn's disease, these blockages are usually the result of inflammatory strictures or fistulas that narrow the intestinal lumen. However, several case reports have described obstructions resulting from intraluminal tumors in patients with Crohn's disease, with adenocarcinomas being the most common .Leiomyomas are benign tumors that originate from smooth muscle cells derived from mesenchymal tissue. Although they are most commonly found in the uterus, leiomyomas can occur wherever smooth muscle is present, including along the entire length of the intestine. Within the small bowel, leiomyomas are predominantly found in the jejunum .While no direct links have been made in the literature between Crohn's disease and leiomyoma formation, Crohn's has been identified as a risk factor for the formation of adenocarcinomas, which have a similar potential to obstruct the intestinal lumen .Our case was different. We had a rare cause of small bowel obstruction. It was a leiomyoma that led to SBO in a male patient with Crohn's disease. Conservative treatment did not success. We performed laparotomy to relieve the obstruction and reach the definitive diagnosis.4This case is considered very rare and it needs special consideration of physicians. SBO have many common causes. Nevertheless, there are rare causes that make the diagnosis too difficult to make. Leiomyoma is one of these rare causes.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.As this publication is a case report that contains no identifiable content to the patient, this publication was exempt from ethical approval by the Human Research Protection Program (HRPP) and its Institutional Review Board (IRB) at the Aleppo University Hospital, Aleppo, Syria.We declare that we didn't require any funding.Husam Matalaqah, Razan Yassen, Lina Matalkeh, Mohammad Matalkeh, and Basil Al Taani: writing the case report.Maher Al-Hajjaj: Data collection and Writing-Original draft preparation.Maher Al-HajjajNot applicable.The authors declare that they have no conflict of interest.


The present study focuses on ileosigmoid knotting (ISK), an infrequent but potentially lethal surgical emergency. ISK is more frequently observed in males and is prevalent in regions with high rates of sigmoid volvulus. The current medical scenario revolves around the intricate intertwining of the ileum and sigmoid colon, leading to a complicated volvulus. This condition often results in acute intestinal obstruction, which can subsequently cause bowel necrosis. The clinical presentation of the condition is characterized by symptoms such as abdominal distention, pain, vomiting, and obstipation, which can be misleading as they are similar to other common abdominal conditions. A delay in definitive diagnosis and intervention is likely, which can lead to severe consequences such as peritonitis, bowel necrosis, sepsis, and even septic shock. Our case report discusses an instance of ISK presenting as an acute abdomen requiring an emergency laparotomy and detorsion of the volvulus-knotted segment, followed by an elective sigmoidectomy. The significance of a heightened level of suspicion, prompt decision-making, and timely surgical intervention in achieving better patient outcomes cannot be overstated. The objective is to enhance the medical community's understanding of ISK, focusing on early diagnosis and effective treatment of this rare but life-threatening disease. Ileosigmoid knotting (ISK), also known as compound or double volvulus, is a rare yet potentially lethal surgical emergency that results in a closed-loop intestinal obstruction, often involving the ileum and the sigmoid colon -2. This A previously healthy nine-year-old male presented with acute abdominal pain for 24 hours, associated with abdominal distension and repetitive vomiting of approximately 30cc of bile. He had a history of chronic constipation; no blood or fecal material was detected in the vomitus. The patient was visibly distressed and exhibited signs of dehydration. On examination, the patient was alert, responsive, oriented, and appeared agitated. Vital signs revealed a pulse rate of 140 beats per minute, blood pressure of 90/60 mmHg, temperature of 38.5°C, and a respiratory rate of 25 breaths per minute. Oxygen saturation was measured at 99% on room air.Chest examination was clear, and abdominal examination revealed distention, rigidity, and guarding, with hypertympanic bowel sounds on auscultation, suggesting peritonitis. Per rectum examination showed good sphincter tone with normal stool on the tip of the finger. No blood was detected. Laboratory investigations showed a white blood cell (WBC) count of 18 x 10^9/L and a hemoglobin level of 13 g/dL. Kidney function tests were within normal range. Abdominal computed tomography (CT) scan from another hospital, performed without contrast, revealed "whirl signs" at the level of the superior mesenteric artery (Figure The patient was resuscitated with intravenous fluids upon transfer to our pediatric tertiary hospital. Broad-spectrum antibiotics were also administered. He underwent an exploratory laparotomy within an hour of admission. Intraoperatively, viable but ischemic ileal loops were noted twisted approximately 360 degrees anticlockwise around a normal, redundant sigmoid colon loaded with fecal material Figure . ApproxiAn elective total sigmoidectomy with colorectal anastomosis was scheduled one month later. Despite significant intraoperative adhesions, a total sigmoidectomy was successfully performed, during which a 35 cm segment of the patient's sigmoid colon was removed, and a colorectal anastomosis was created. The patient was discharged on the fifth day after surgery, having experienced a smooth recovery. Follow-up appointments were regularly scheduled to keep a close watch on the patient's condition, during which no recurrence of the symptoms has been observed.ISK, often known as a "double volvulus," is a rare but serious medical condition where the ileum and sigmoid colon intertwine and block the intestinal loop . It can ISK's clinical presentation typically encompasses abdominal pain, bloating, nausea, vomiting, and in severe instances, shock . These sPrompt medical action is essential due to ISK's rapid progression to a gangrenous bowel. Initial measures include resuscitating the patient and stabilizing hemodynamics before surgical intervention . The potISK is a rare, potentially lethal surgical emergency demanding prompt diagnosis and decisive intervention. Our report stresses the need for a high clinical suspicion. A deeper understanding of ISK risk factors and early diagnostic strategies can improve patient outcomes in this complex and rapidly progressing condition.


Epiploic appendagitis (EA) is an uncommon condition caused by infarction of epiploic appendages “small fat outpouchings present on the outside of the colon wall” because of torsion or thrombosis of the main draining vein. It is sometimes misdiagnosed as diverticulitis or appendicitis. Lab tests usually are normal, and the diagnosis is mainly by computerized tomography (CT) scan. Treatment is conservative as it is a self-limited condition, and the symptoms will resolve spontaneously within 2 weeks. However, surgical appendage removal could be necessary if symptoms increase or continue. Here, we report our experience with a 21-year-old male patient, who presented with a 1-day duration of localized right lower quadrant (RLQ) abdominal pain within 18*10 cm incisional hernia, imaging revealed signs of epiploic appendages infarction within the huge incisional hernia. This case describes an atypical scenario for EA, which was successfully managed with surgery. The final pathology report confirms the diagnosis. Epiploic appendages are normal peritoneal fat outpouchings on the anti-mesenteric surface of the colon , they weA 21-year-old male patient, obese with BMI of 35, known case of incisional hernia that occurred after an open appendectomy one year ago, was admitted to the emergency department complaining of acute abdominal pain for one-day duration. The pain was localized to the RLQ at the site of the incisional hernia, sudden in onset, sharp in character, not radiated with no known aggravating or relieving factors, and not associated with nausea, vomiting, abdominal distention, or change in bowel habit. On admission, he had normal vital signs. Physical examination showed a huge reducible incisional hernia in the RLQ area, at the McBurney scar with no signs of inflammation. There was significant tenderness at the McBurney point. Laboratory tests showed an increase in C-reactive protein (CRP) 180.00 g/l (0–5 g/l) and leukocyte count 17 000*109/L (4.3–10.8 × 109/L). Other Laboratory tests are all within normal. Abdomen CT scan with IV contrast  demonstrThe distribution of epiploic appendages through the colon have a variable frequencies, the most common location is on the rectosigmoid junction, which accounts for 57% of epiploic appendages, whereas the least common location is the descending colon represents 2% . EA is aEA in an incisional hernia is rare and difficult to diagnose. In our case, the patient presented to emergency complaining of acute abdominal pain localized to the incisional hernia without signs of incarcerated or strangulation of the incisional hernia. CT scan results showed the diagnosis of EA, which was effectively treated with surgical resection following unsuccessful conservative treatment. The study’s goal is to provide information on this rare entity and warn surgeons to be aware of the image findings to avoid misdiagnosis.


Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management.This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery.Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival. Soft tissue sarcomas (STS) are rare malignant tumours arising from mesenchymal tissues which make up around 2% of all adult malignancies . UndiffeGiven the disease’s prevalence of 0.08 to 1 per 100,000 people, little is known regarding the ideal management of such cases, having no established staging criteria or oncological treatments. The generally accepted treatment involves radical surgical resection followed by chemotherapy or radiotherapy, depending on the tumour size or the presence of metastatic disease. Despite such aggressive therapy, recurrence rates range after 5 years range between 10 and 42%, whilst 31 to 40% develop distant metastasis [It remains a diagnostic challenge, being characterized by the absence of genetic characteristics and specific lines of differentiation on histological examination . Patholo9/L, a haemoglobin level of 12.1 g/dL, a platelet count of 201 × 109/L and a C-reactive protein (CRP) of 84.2 mg/L.This report presents the case of a previously healthy 40-year-old Caucasian male with no history of malignancies or significant radiation exposure. He presented to the Emergency Department with an 8-day history of central epigastric pain which radiated to the back and was associated with generalized malaise and low-grade fever. Clinical examination demonstrated palpable splenomegaly of around 4 fingerbreadths below the left costal margin with an otherwise soft abdomen. He had a raised white cell count (WCC) of 24.3 × 10Ultrasonographic (US) studies showed massive splenomegaly of 20.1 cm in diameter with a heterogeneous mass in splenic hilum measuring 12 cm by 9 cm. A homogeneous fluid collection was also noted adjacent to cranial aspect of spleen measuring 16.7 cm by 15 cm. The liver was normal on ultrasound studies. Computed tomographic (CT) imaging demonstrated a 16 cm × 14 cm × 21 cm partially solid partially cystic lesion in spleen with mass effect on surrounding structures, as seen in Fig. An broad infectious screen, including human immuno-deficiency virus, hepatitis A, B and C, toxoplasma, rubella, cytomegalovirus, herpes simplex, Epstein–Barr virus, rickettsia, leishmaniosis, leptospirosis, mycoplasma and parvovirus, was negative.Core needle biopsies of the spleen were attempted twice, both of which comprised entirely necrotic material with no viable tumour tissue present. Immunohistochemistry only demonstrated non-specific expression of CD10, along with a high Ki67 index. Whilst the possibility of a necrotic lymphoma was entertained, the biopsies were deemed non-diagnostic.A positron emission tomography (PET) scan was subsequently performed, demonstrated in Fig. Given that no definitive diagnosis was achieved at this stage, a diagnostic and therapeutic splenectomy was planned. Access to the abdominal cavity was achieved by an L-shaped laparotomy incision. A massive spleen adherent to the pancreatic tail at the hilum was identified and resected. In addition, three small liver lesions were detected in segment V, VII and VIII, respectively, as seen in Fig. Frozen section analysis on one of the metastatic liver deposits showed a well-circumscribed tumoural nodule comprising pleomorphic neoplastic cells with frequent bizarre nuclear forms. The findings were interpreted as a high-grade poorly differentiated malignancy, with lymphoma being the most likely diagnosis.Gross pathological analysis showed a large splenic mass weighing 4 kg and measuring 28 cm, which was affixed to the pancreatic tail. On sectioning, the spleen was almost entirely replaced by a centrally cystically necrotic mass. The mass was largely non-viable however, towards the periphery, had a pale white, fleshy appearance. The tumour was centred on the spleen and did not appear to arise from the pancreas, with the resected pancreatic tail being unremarkable and free of malignancy. Analysis of the resected liver lesions demonstrated well-circumscribed fleshy white subcapsular tumour deposits, which were similar in morphology to the splenic mass.Histological analysis of the splenic tumour showed diffuse effacement of the splenic parenchyma by a high-grade neoplasm. The tumour variably comprised neoplastic spindled and epithelioid cells. The spindle cell component was organized in short fascicles while the epithelioid component infiltrated the splenic parenchyma in cohesive sheets. The neoplastic cells were bizarre, pleomorphic and showed very brisk mitotic activity. Widespread areas of tumour necrosis were seen. The tumour invaded beyond the splenic capsule into the surrounding soft tissues. Slides demonstrating these findings are found in Fig. A broad immunohistochemical panel was applied. Cytokeratin stains  were negative, as were markers of melanocytic , haemato-lymphoid/histiocytic , dendritic cell  and germ cell  differentiation. Immunohistochemical attempts at identifying a specific sarcomatoid lineage of differentiation were also negative, with vascular , neural  and adipocytic  markers being negative. The tumour only showed non-specific expression of CD10, cyclin D1 and a high Ki67 proliferation fraction (around 80%).The overall histological and immunohistochemical findings were thus supportive of a diagnosis of undifferentiated pleomorphic sarcoma (UPS) of the spleen, with metastatic deposits in the liver. The excision of both the splenic tumour and the hepatic metastatic deposits was deemed complete.2 weekly for 3 cycles when fully recovered from surgery. However, he presented to the Emergency Department yet again three weeks post operatively with persistent lower back and abdominal pain associated with fever. A CT scan of his abdomen was performed, showing extensive recurrent disease burden in the peritoneum and liver as seen in Fig. The patient recovered well post operatively and was discharged home 11 days later, with a plan to start single-agent Doxorubicin at a starting dose of 30 mg/mA literature search was performed using Medline, PubMed and Elsevier. 41 published cases were identified and are depicted in Table Splenic UPS in an extremely rare entity with only 41 other cases reported within English literature at the time of writing this report. UPS pathologically defines a sarcoma subtype lacking any specific lineage differentiation and remains a diagnosis of exclusion after other pleomorphic sarcoma subtypes have been excluded. Whilst still in use, the term ‘malignant fibrous histiocytoma’ has fallen out of favour in recent WHO classifications as the tumour does not show clear evidence of fibrohistiocytic differentiation. UPS may be divided into five histological subtypes which are pleomorphic, inflammatory, myxoid, giant cell and angiomatoid , 29. HowThis first case of splenic UPS was described by Govoni et al. , 29. TheThe age at presentation ranges from 17 to 82 , 41. TheThe histopathological features are similar to conventional UPS in soft tissues. The histological appearance is by no means distinctive but is often a combination of spindled, epithelioid and bizarre pleomorphic multinucleated cells exhibiting brisk mitotic activity, with or without tumour necrosis. Osteoclast-like giant cells are typically noted in the ‘giant cell’ variant, while a brisk inflammatory infiltrate is typically noted in the ‘inflammatory’ variant. A myxoid stroma background may be seen and is typically extensive in the ‘myxoid’ variant, making differentiation with high-grade myxofibrosarcoma challenging. A pseudovascular configuration may be noted in the 'angiomatoid’ variant, with differentiation from angiosarcoma being established through the absence of vascular marker expression on immunohistochemical grounds. The latter serves to distinguish UPS from somewhat commoner splenic sarcomas including splenic angiosarcoma, Kaposi sarcoma and littoral cell angiosarcoma.The immunohistochemical profile of UPS is non-specific and is principally geared at excluding other diagnoses. The tumour is persistently negative for cytokeratins and markers of melanocytic differentiation. Non-specific expression of lysozyme, CD68, fascin, CD10 and cyclin D1 may be observed. However, expression of histiocytic markers is only focal which helps to differentiate this tumour from histiocytic sarcoma.Due to the paucity of data and rare nature of the disease, there are no established staging criteria for splenic UPS. This has implications on stratifying patients and guiding management , 9. The Response to adjuvant treatment was generally poor, with only three papers reporting a positive response. The initial complete response quoted in Colovic et al. was based on the complete disappearance of all liver lesions on ultrasound. This form of imaging is not recommended by the latest RECIST criteria which recommend cross-sectional imaging for assessing treatment response. In addition, the good initial response quoted by Audouin et al. was not supported by any objective measures such as imaging or tumour markers. Hence, the adjuvant treatment in both of these papers was not deemed to have any positive response using the RECIST criteria , prior tTable Table Table As the preoperative imaging in our case did not demonstrate evidence of metastatic disease, surgery was performed with curative intent. As previously described, these three sole liver lesions were easily resectable and so the decision was made to proceed with surgery. In addition, the removal of such a large 4 kg tumour relieved its burden, including discomfort and early satiety. Other than a few select cases from the literature review, surgical resection in established metastatic disease did not appear to offer much survival benefit, including in the presented case. Hence, the presence of metastasis should generally be considered as a contra-indication to surgical resection.The four cases with a reported survival of more than two years differed greatly in patient characteristics and management. Kimura et al. report a case of a 29-year-old male who underwent elective splenectomy four months after diagnosis. The spleen size was 17 cm × 13 cm × 8 cm and weighed 735 g. No metastases were detected before or during surgery and he did not receive any chemotherapy or radiotherapy. The patient subsequently developed liver metastases 3 years after surgery and a liver metastasectomy was performed, followed by an unspecified regime of adjuvant chemoradiotherapy. Out of all the included cases, this was the longest documented survivor at 5 years from presentation.Mantas et al. report a 66-year-old lady who underwent immediate splenectomy following diagnosis. The splenic size was similar to the prior case at 18 cm × 13 cm × 10 cm. However, the splenic weight was almost double at 1300 g. Similarly, no metastases were detected before or during surgery and she did not receive any chemotherapy or radiotherapy. She was reported as alive, asymptomatic and free of local recurrence and metastatic disease three and a half years after her operation.Darwish et al. report a 49-year-old gentleman who had initially refused treatment. Imaging had shown multiple splenic lesions with the largest being 11 cm × 9 cm × 10 cm. However, the patient presented eight months later with splenic rupture which was initially treated conservatively. The patient then underwent elective splenectomy 11 months after initial presentation, after which he received three cycles of doxorubicin and ifosfamide. New liver lesions were later detected on follow-up imaging for which the patient underwent microwave ablation (MWA) and a left hepatectomy. Despite post-operative recurrence, the authors report that the patient was still alive 2 years following presentation.Lastly, Yamamoto et al. report a 69-year-old male who presented with a splenic mass measuring 4.5 cm × 5.4 cm × 5 cm with liver lesions. The patient had a good response to two cycles of neoadjuvant carboplatin, etoposide and paclitaxel, after which he underwent elective splenectomy. Following recovery, he received six cycles of mitomycin and etoposide via hepatic arterial infusion (HAI). He was reported alive three and a half years following presentation.Therefore, survival was not found to be associated with any specific patient characteristic or management strategy.UPS of the spleen is a malignancy characterized by pleomorphic spindle cells with bizarre looking nuclei, arranged in a storiform pattern. It is generally treated by splenectomy and in some instances chemotherapy. The disease carries an overall dismal prognosis, particularly when combined with distant metastasis. Currently available literature fails to delineate a particularly advantageous or superior management strategy which increases survival.