A mesenteric cyst is an uncommon ailment that can affect practically any abdominal quadrant in its presentation. They may turn up as an accidental discovery. Although there are a number of hypotheses explaining the genesis of these cysts, the exact etiology is unknown. A 70-year-old female patient came to see us complaining of abdominal pain for a month and had trouble passing stools for 15 days. Contrast-enhanced computed tomography was done for the patient, which revealed a heterogeneously enhancing mass lesion in the abdominal cavity. The patient was then taken for an exploratory laparotomy procedure. To make the procedure thorough and easy, intraoperative partial drainage of the cyst fluid was carried out. We were able to observe the margins of the mesenteric cyst more easily as the partial drainage was carried out. The partial drainage decreased the volume and size of the mesenteric cyst, reducing the pressure effect on the surrounding structures and allowing easy mobilization of the intraabdominal structures during intraoperative examinations. The partial drainage of the cystic fluid also made the dissection process safer. After releasing all adhesions, the cyst was delivered outside and sent for histopathological analysis. The histopathological reports confirmed it to be a mesenteric cyst. The aim of this article is to educate the readers and to make fellow surgeons well aware of this condition. This will not only help fellow clinicians in better diagnosis and treatment but also help in the reduction of the overall burden of the healthcare society by reducing mortality and morbidity. Cysts in the small bowel or colon's mesentery are known as mesenteric cysts ,2. They A 70-year-old female patient came to our emergency department complaining of abdominal pain for a month and having trouble passing stool for 15 days. Clinical evaluation revealed that the patient was in good general condition and that there were no abnormal findings in the systemic examination. However, during the abdominal examination, a mass was palpable in the right iliac fossa region, and it moved perpendicular to the direction in which the mesentery is attached but was constrained in the direction parallel to it. There was a cystic swelling in the lower abdomen after an ultrasonogram (USG) of the abdomen and pelvis. A contrast-enhanced computed tomography scan of the abdomen and pelvis revealed a large, well-defined exophytic, heterogeneously enhancing, solid-cystic mixed-density mass lesion measuring 8.7x8.5x8 cm in the central mesenteric region that abutted the inferior wall of the third part of the duodenum and the proximal jejunum. The mass also abutted the right ureter and inferior vena cava  are advised as the mainline investigations. The ultimate diagnosis must be histologically verified and can be made during laparotomy. Volvulus, leakage of infectious fluid, herniation of the colon into an abdominal defect, and blockage are secondary problems linked to mesenteric cysts . The preThe mesenteric cyst is a rare presentation that might have as few symptoms as short-term abdominal pain or discomfort or as serious symptoms and abnormalities that are consistent with an intestinal blockage. The patient needs to undergo a complete assessment. The partial cyst-draining technique may prove useful intraoperatively to make it easier to expose the operating field. There should always be an effort made to remove the entire mesenteric cyst. The cyst should subsequently be sent for histological and cytological analysis to determine the best course of treatment.


Primary small bowel lymphomas are a rare entity but with significant morbidity and a low five-year overall survival even after surgery. Its diagnosis is often delayed due to the lack of clear specific signs, unfamiliarity amongst clinicians, and the lack of screening tools. This often results in patients presenting with tumour-associated complications such as perforation, obstruction, or gastrointestinal bleeding which warrant urgent surgical intervention. We present the case of a patient presenting with a perforated small bowel lymphoma resulting in a large interloop extraluminal faecaloma causing subacute small bowel obstruction. He proceeded to have an emergency open right hemicolectomy and extended small bowel resection to facilitate gross resection of tumour which in conjunction with adjuvant systemic chemotherapy is considered current best practice to manage such neoplasms. Early referral to specialist centres and raising awareness of this rare entity will allow earlier recognition and therefore a more planned approach to the management of such pathology with fewer post-operative complications. The incidence of primary small bowel lymphoma is on the rise in the western world albeit rare. Previous studies have often treated primary gastrointestinal lymphomas arising from varying sites as a single entity although it is becoming increasingly evident that the optimum treatment regimen and survival rates vary significantly based on site and histology . Given tA 62-year-old male presented to the emergency department with a 3-month history of worsening right lower quadrant pain, constitutional symptoms and a CT scan performed in the community revealing a large soft tissue mass measuring 6 cm × 8.5 cm × 7 cm in the right lower quadrant thought to be arising from the distal ileal loops with associated lymphadenopathy. This was on a background of coeliac disease, gastro-oesophageal reflux, and iron deficiency. The only surgical history of note was a right-sided open inguinal hernia repair as a child.He had recently undergone an endoscopy revealing gastritis and duodenitis and a colonoscopy revealing a 5 mm polyp in the descending colon which was a tubular adenoma with low-grade dysplasia and there was an impression of mass effect at the opening of the terminal ileum; however, no intraluminal sinister lesions were detected. He was awaiting his outpatient appointment with a specialist surgical team regarding his symptoms.9 g/L, thrombocytosis of 600 × 109 g/L, neutrophilia of 14.97 × 109 g/L, lymphocytosis of 4.20 × 109 g/L, and monocytosis of 1.88 × 109 g/L with a normal hemoglobin and the blood film demonstrating toxic changes consistent with an infective process. His electrolytes and liver function tests were grossly normal, with a normal carcinoembryonic antigen (CEA) level. He had an elevated C-reactive peptide of 151 mg/L and was commenced on broad-spectrum IV antibiotics with gut cover and was admitted to the ward for further workup.Given the deterioration of his pain, he presented to the emergency department roughly a few weeks after his colonoscopy. On arrival, he was hemodynamically stable with a low-grade temperature of 37.8°C and tender over his right iliac fossa. Physical examination revealed fullness over his right iliac fossa with a palpable lump over which he was exquisitely tender with focal guarding but without generalised peritonitis. A repeat CT scan was performed as demonstrated in He underwent a CT angiogram of his abdomen and pelvis the following day as depicted on On entering the abdomen via a midline laparotomy, careful adhesiolysis revealed the large 11×11cm collection. Attempts at mobilising this mass resulted in inadvertent spillage of contents which were noted to be enteric in nature. Further abdominal exploration revealed dense fibrotic adhesions to this mass from the terminal ileum to the proximal transverse colon and a few loops of small bowel. An intraopertaive photograph depicts the apperance of this large collection following evacuation of contents in The patient had an uneventful but slow post-op recovery with a gradual upgrade of diet. His drains were removed prior to discharge on day 9 post-op. The histopathology revealed extensive involvement of diffuse large B cell lymphoma not otherwise specified of germinal centre cell type with MYC gene translocation t. Multiple other deposits of lymphoma were also noted within the small bowel mesentery and omentum. His case was discussed at the local multidisciplinary meeting, and he was subsequently referred onto hematology services where he is currently receiving chemotherapy. He was reviewed in the clinic 3 weeks post-operatively with no concerns, his wounds had healed well, and both the patient and their families were very thankful for the care received.Given the relative rarity of small bowel lymphomas which make up about 2% of all GIT neoplasms, there is paucity of data to guide its optimal management . It is bThey often present at an advanced stage due to delays in diagnosis as the symptoms may be vague and on most occasions these tumours are found at surgery indicated for other diagnoses or intestinal obstruction as in this case . The mosEmergency surgery for perforated NHL of the small bowel can be challenging as the underlying pathology is often diagnosed at the time of operation . In thisA recent retrospective study published in 2021 from a national database in the United States found that adjuvant chemotherapy following surgical resection is associated with an improved overall survival in primary small bowel lymphoma as opposed to surgery alone; however, they did exclude patients with stage IV disease and included both elective and emergency presentations making it difficult to apply to our case; however, the patient in our case did proceed to receiving chemotherapy . They alIn conclusion, primary care clinicians need to be aware of the red flag symptoms in the community that warrant urgent referral to specialists to avoid delayed presentations with tumour-associated complications which would allow elective surgical management in a more controlled fashion with fewer post-operative complications, e.g., anastomotic leaks and wound infection, favour bowel preservation and early review by oncologists to facilitate timely healthcare delivery and a better patient experience.


Butterfly spine is a rare benign congenital abnormality. The onset of a minimum of three of the congenital malformations of vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities often characterises the VATER/VACTERL relationship. Recognising this anomaly is crucial for diagnosis, although this rare aberration is thought to be asymptomatic most of the time. Here we are describing a case of a one-year-old female child who has tetralogy of Fallot, congenital anal atresia, vesicovaginal fistula, and butterfly vertebrae which were found as an incidental finding. Furthermore we suggest screening all the children with any one abnormality of VACTERL, usually vertebral anomalies are screened. The butterfly vertebra is a rare and often benign congenital spine abnormality of the vertebrae. It might be associated with kyphoscoliosis . The butIt’s a sagittal deformity in the vertebral body as the two lateral chondrification centers fail to fuse together during development. The name is based on the pattern that the two hemivertebrae appear on x-rays as butterfly wings from the central gap . AccordiWe present a case of a one-year-old female child brought by her parents to the tertiary care hospital with complaints of an imperforate anus since birth and a history of the passage of stools from the vagina. She had a history of nonpassage of stools from two days; later, she passed stools all at once. Her history of abdominal distension is why the child was brought to the hospital for further management.The child had a gross motor developmental delay; clinically, the child had mild lumbar kyphoscoliosis, but no lower back tenderness, and local examinations were suggestive of fused vertebrae. X-ray lumbosacral (LS) spine  was done, showing widening of the L2/L3 and L4 vertebrae and altered intervertebral discs showing concavities along the adjacent end plates suggestive of butterfly vertebrae Figure , 2.The child is also a known case of congenital heart disease  and presented with no cyanosis clinically; on auscultation, there was an ejection systolic murmur best appreciated along the left mid sternal border on X-Ray posteroanterior (PA) view it showed a boot-shaped heart suggestive of TOF Figure . The chiIn between the third and sixth weeks of pregnancy, during somitogenesis, the butterfly vertebra develops in utero ,4. The nUsually, the vertebrae and intervertebral discs above and below elongate toward the midline to make up for the deficiency. The defect is frequently discovered by accident radiologically, but in our instance, we made a clinical diagnosis and radiological confirmation of it, even though it might be connected to lower back pain. This abnormality may be connected to various spinal abnormalities such as spina bifida, diastematomyelia, lumbosacral transitional vertebrae, Alagille and Klippel-Feil syndrome, as well as digestive and renal abnormalities. The etiology is thought to be genetic (chromosome 20 deletions) or congenitally vascularization-deficient. Butterfly vertebra typically has no symptoms. Patients with this deformity may present with unusual chronic lower back pain ,8 or mayA dual-energy X-ray absorptiometry (DEXA) test can be done to differentiate neoplasia, osteoporosis, or infection. There have been occurrences of butterfly vertebrae linked to disc protrusion or nucleus pulposus herniation at the level of the aberrant vertebral body. Additional diagnostic techniques are not required once a diagnosis has been made. This congenital defect, usually mistaken as a spinal fracture, must be known to neurosurgeons. As per our knowledge, this is the second case reported in the pediatric age group .Early diagnosis of the butterfly vertebrae is necessary to initiate early treatment modalities such as physiotherapy and postural corrections to prevent morbidity in adulthood. Even though it is mostly an asymptomatic condition, early screening in the pediatric age group is always a better idea to prevent further complications and is advised.


Pseudomelanosis (PM) is a rare, benign, condition that is characterized by deposition of melanin and/or melanin-like pigment in mucosal cells and macrophages and is best known as the entity pseudomelanosis coli.Pseudomelanosis primary of the urinary bladder has been reported only in a handful of cases worldwide. This article reports an extremely rare case of pseudomelanosis of the urinary bladder in a 79-year-old male with a history of macroscopic painless hematuria. It is best known as the entity pseudomelanosis coli, and also described in the stomach, duodenum and jejunum.2A 79-year-old male presented with a seven weeks history of macroscopic painless hematuria. Lower urinary tract symptoms (LUTS) were absent, especially stranguria and pollakiuria. His past medical history included cataract and left-sided adrenalectomy. He took medication regularly, including amlodipine, spironolactone and rivaroxaban. There were no risk factors for urothelial carcinoma. Physical examination did not reveal any abnormalities. Urine analysis was negative for nitrite and leukocytes. Microscopic examination of urine sediment showed the presence of erythrocytes, dysmorphic erythrocytes were not found. Urine cytology revealed a severe candida infection with several pigment-laden cells, consistent with urothelial cells with melanin pigment . CystoscAfter consent, the patient underwent surgery through a transurethral approach. A tumor of the bladder was not seen. However, multiple black and brown pigmented lesions were found and biopsied separately A and B.FThe histologic specimen consisted of urothelium with a normal architecture. The upper layers of the urothelium contained cytoplasmic melanin pigment, which was confirmed using a Schmorl stain. No melanocytes were found. The lamina propria contained several pigment-laden macrophages .Fig. 3HiPer-operative a three-way Foley catheter with continuous irrigation system was inserted. The catheter was removed on the first day postoperative. Because of increasing post-void residual urine (>450 cc) the patient was discharged with a two-way indwelling catheter and an alpha-1-blocker was prescribed. After one week the patient was unable to adequately empty his bladder without catheter, clean intermittent self-catheterization (CISC) was educated. The hematuria resolved without further treatment. He will be followed up with cystoscopy 6–8 months later.3Pseudomelanosis is a rare condition of deposition of melanin and/or melanin-like pigment in mucosal cells and macrophages and is best known as the entity pseudomelanosis coli. Pseudomelanosis primary of the bladder or urinary tract is extremely rare, only a handful of cases been reported worldwide. It was first described in 2006 by Wieringa et al. this was also at our institution.,The precise etiology is unknown, though there may be an association with the use of specific categories of medications and chronic diseases, including iron supplements, beta-blockers, thiazide diuretics, diabetes mellitus, hypertension and renal diseases.Pseudomelanosis, in most cases asymptomatic, is often found by accident during cystoscopy or surgery and is on itself no indication for treatment. In our case the patient presented with hematuria, most likely based on candida cystitis as there were no other urinary tract abnormalities found during surgery. However, he had no LUTS and the hematuria resolved after one week without further treatment.Although considered benign, the rarity of the lesions warrants regular cystoscopic evaluation and biopsy to assess the risk of malignant transformation, pseudomelanosis has been described in association with malignant melanoma and urothelial carcinoma.Postoperative the patient showed elevated post voiding residual volumes after removal of the three-way Foley catheter. Re-catheterization was necessary and medical treatment by an alpha-1-blocker monotherapy was started. After one week he failed to void successfully without catheter and CISC was taught. During cystoscopy an enlarged median prostatic lobe was seen. Thereby, it is most likely that the impaired bladder emptying is based on enlargement of the prostate in the context of benign prostatic hyperplasia.4Pseudomelanosis of the bladder is an extremely rare entity with only a few cases reported worldwide. Histopathological examination is essential in diagnosing this condition. Although considered benign, follow up with cystoscopy is recommended by several authors.Informed consent was obtained from the patient for publication of this case report in accordance with the journals patient consent policy.No funding to declare.The study conforms to recognized standards of World Medical Association Declaration of Helsinki.M. Duijn: Conceptualization, Methodology, Writing - original draft preparation, Resources. S.L. Croonen: Investigation, Visualization, Writing - review and editing. J.A. Van der Zee: Writing - review and editing, Validation, Supervision.The data used and/or analyzed ruing the current study are available from the corresponding authors per request.The authors declare no conflict of interest.


Leiomyoma is a benign tumor of smooth muscles and can essentially arise from any part of the body that contains smooth muscles. It is more commonly found in the uterus, while those from the gastrointestinal tract are rare. The spectrum of clinical presentation is rather wide, from a vague abdominal discomfort or acute abdomen mimicking more common diagnoses such as acute appendicitis or acute gastroenteritis to features of intestinal obstruction and gastrointestinal bleeding. Here, we share our experience with a case of a young woman whose symptoms and signs were typical of acute appendicitis, which intraoperatively turned out to be a twisted mesenteric tumor located at 70 cm from duodenojejunal flexure. The tumor was managed by segmental resection and anastomosis. She had an uneventful recovery postoperatively, and histopathological examination of the surgical specimen was consistent with an infarcted mesenteric leiomyoma. The rarity of this entity mimicking appendicitis is highlighted, and should it never get twisted, it would have probably remained unnoticed for the rest of her life. In the current era of laparoscopy, it is always wise to never skip a thorough examination of the whole length of the small bowel in order to locate this type of pathology. Primary mesenteric tumors are rare and are usually of mesenchymal origin. In fact, mesentery is more commonly associated with metastatic deposits. Benign tumors include leiomyoma, lipomas, desmoid tumors, and cystic lymphangiomas, while malignant ones include lymphoma, gastrointestinal stromal tumors (GIST), carcinoids, and leiomyosarcomas . The rarA 25-year-old female with no underlying medical illness presented to us with three-day right iliac fossa pain. She presented on day 3 of illness as the pain was getting more intense; her pain score was 9/10. The pain was colicky in nature and had a migratory character. It began at the left iliac fossa before migrating to the periumbilical region and eventually localized to the right iliac fossa. It was associated with nausea but no vomiting. Menstrual and gynecological history was otherwise unremarkable. Clinical examination revealed normal vital signs. Abdominal examination revealed tenderness at the right iliac fossa with no guarding and no rebound tenderness. Rovsing's sign was negative, and there was no palpable abdominal mass. Gynecological examination with transabdominal scan was normal. With normal urine full examination and microscopy examination (FEME), presence of leucocytosis, and Alvarado score of 7, a diagnosis of acute appendicitis was made. She was then booked for laparoscopic appendicectomy.On laparoscopy, clumping of omentum was noted centrally. Examination of the appendix, fallopian tubes, ovaries, and uterus were all normal. The initially seen omental clump was then dismantled, after which a gangrenous pedunculated swelling arising from the mesenteric border of jejunum was revealed. Its surface was regular and smooth, firm in consistency, but showed adhesions with the omentum. The swelling was 5 x 3 cm in diameter and twisted at its pedicle Figure . No signOn histopathological examination, the swelling showed an extensive area of hemorrhage with scattered blood vessels and neutrophil infiltration. It was well circumscribed and composed of a few viable spindle cells at the periphery, which were uniform, showing oval vesicular nuclei with wavy indistinct cytoplasm. These cells were positive for smooth muscle actin (SMA) while negative for CD117, S100, and CD34. This swelling was connected to the outer serosal surface of the small bowel. The resected small bowel showed normal histomorphology. This was finally concluded as normal small bowel with an attached infarcted smooth muscle tumor, suggestive of mesenteric leiomyoma. Two months after the surgery, the patient was reassessed in the clinic. She was well and had no symptoms. Considering the benign nature of the tumor and adequate resection performed, she was discharged from the surgical clinic.Leiomyoma is a benign tumor that arises from smooth muscles. They are most commonly found in the uterus and may also arise elsewhere in the body, including the gastrointestinal tract and mesentery. It is important to distinguish them from leiomyosarcoma and other types of mesenchymal tumors such as GIST. By immunohistochemistry, both tumors are positive for SMA, but leiomyoma is negative for CD117, CD34, and S100. In smooth muscle tumors, the presence of atypia and high mitotic activity indicates their malignant nature, leiomyosarcoma -4. This As a matter of fact, mesenteric and small bowel tumors tend to have a similar clinical presentation. They may manifest as small bowel obstruction, gastrointestinal bleeding, abdominal mass, a wide spectrum of abdominal pain, or even a small bowel intussusception -7. ObstrIn our case, the migratory nature of the abdominal pain, which eventually resided in the right iliac fossa, presence of right iliac fossa tenderness, normal gynecological assessment, and exclusion of urinary tract infection were all positive points that led us to a diagnosis of acute appendicitis. The clinical diagnosis was convincing enough to proceed with laparoscopic appendicectomy. Of note, we do not routinely perform an ultrasound or CT scan to establish a diagnosis of appendicitis in young adults with a clinically convincing presentation. Retrospectively, it would have been a better move to have a CT of the abdomen performed before the surgery. CT would be appropriate, especially in establishing other causes of right iliac fossa pain, such as appendicular abscess, which can be drained extraperitoneally, and it would aid in the diagnosis of the mesenteric tumor. With this, better preoperative planning would have been undertaken. During the surgery, the rather surprising intraoperative finding was dealt with an oncological resection with a 10 cm margin and primary anastomosis by taking into consideration that a malignant tumor was still a possibility. This procedure is best performed laparoscopically if the expertise is available, and this, in fact, would promote faster recovery. We concluded that her pain was explained by twisting and infarction of her mesenteric tumor. She never had vomiting because there was no obstructing effect by the tumor. It is also worth noting that laparoscopy has a significant advantage in localizing the pathology, especially when the appendix appears normal and, more importantly, when the common alternative diagnosis is not found. A thorough search for the pathology is a must before concluding a negative laparoscopy. On a side note, it would have been challenging to localize the pathology if this case had been performed using the conventional open approach via Lanz incision.In conclusion, clinicians should be aware of the uncommon differential diagnosis of acute appendicitis. As the mimics usually originate from the small bowel and mesentery, the whole length of the small bowel and its mesentery must thoroughly be inspected. Since the differentiation between malignant and benign mesenteric tumors is difficult intraoperatively, resection should be performed following the principles of resection of malignant disease. Diagnostic laparoscopy has been a game-changer as it can readily show the exact pathology, especially when the appendix is normal, or synchronous pathology when the appendix is abnormal. Thorough diagnostic laparoscopy is a key to not missing a rare pathology such as mesenteric tumors.


It has been almost a decade since the multinodular and vacuolating neuronal tumor (MVNT) was first described. In 2021, WHO classified it as a defined entity, and it is considered one of the glioneuronal and neuronal tumors. Due to its similarities with dysembryoplastic neuroepithelial tumors (DNET), some authors consider it a variant of these, ranking in the category of malformations, but genetic alterations favor a neoplastic origin. We present a 29-year-old male with a generalized onset tonic-clonic seizure after a nightclub party. Imaging studies revealed a right temporal multinodular and vacuolating neuronal tumor confirmed by biopsy. It is considered a nonaggressive, “leave me alone” brain lesion, which does not require biopsy because of well-defined MRI characteristics. Surgery is indicated only in symptomatic cases. We consider that this lesion was revealed by his seizure, most probably provoked  by the consumption of a lot of alcohol, illicit drugs, and sleep loss after a club party. We recommended close monitoring, but our patient preferred the surgery. Our case added more imaging details corroborated with the histopathology features of MVNT. FLAIR images revealed hypointense nodules surrounded by hyperintense peripheral rings and areas of high signal intensity between the nodules, which correspond to the histopathological architecture. To our knowledge, this is the first case of MVNT with diffusion tensor imaging and fiber tractography imaging studies. A 29-year-old male smoker and drug addict (over the last 10 years) presented a generalized onset seizure from the family description. It appeared in the context of 25 h of sleep deprivation along with the consumption of alcohol, energy drinks, cocaine, and cigarettes after a night of partying. He had a seizure-free history and no afflictions or chronic medication, except for a depressive episode one year prior, an episode for which he was treated with antidepressants. His medical background was not relevant. Any neurological deficits were absent. No seizure history was found in his family, and no other medical conditions were found until this event. Lab tests were normal. The 30 min EEG monitoring was normal.The brain CT and conventional and advanced MRI features were appropriate for a multinodular and vacuolating neuronal tumor (MVNT).The CT scan shows a hypodense cortical/subcortical white-matter lesion mainly on the right temporal lobe with partially lobulated margins, such as a cluster of hypodense nodules . On the MRI exam, the patient has an extensive lesion in the right temporal lobe at the anterior pole, inferior, and medial temporal gyrus, extended to the amygdala and hippocampus. The lesion exhibits a non mass appearance with imaging findings consisting of a cluster of nodular lesions, gathered on the inner surface of the cortex at the cortical-subcortical interface. Some of the lesions are arranged tightly together, but some of them are close by and have a small subcortical white matter interface. Furthermore, we have analyzed the imaging findings and tried to correlate them with the data from the literature regarding the imaging and histopathological characteristics of the tumor ,2,3. On The T2wi displays an extensive lesion that has a bunch of grape appearances with lobulated margins and high signal intensity. In this sequence, the nodules seem to be more closely together than on the T1wi, with no normal parenchyma interface between their components . The scaThe tumor does not exhibit restriction of diffusion on DWI and ADC .One of the key sequences is T2 space inversion recovery with fluid attenuation (T2 FLAIR), which has fine slices (in our case 1.3 mm slice) and identifies the clustered and the scattered nodules, but with an interesting characteristic. The larger nodules have a hypointense center with a hyperintense rim with a ring-like appearance, and between the nodules, there are areas with a high signal intensity that connect the nodules. The smaller nodules have nonhomogeneous high signal intensity with a central dot of low signal intensity .Therefore, the question is why it has this appearance . SearchiCombining the signals on T1, T2, and T2 FLAIR, and with the knowledge gathered, we concluded that the signal intensity of the nodules is nonhomogeneous because of the vacuoles that are low T1 high T2 and low on FLAIR. The periphery of the nodules has high signal intensity on FLAIR because of the high protein content and the demyelination of the neurons, and the areas surrounding the nodules have high signal intensity on FLAIR given by the unmyelinated nerve fibers. MRI spectroscopy shows an increase in the choline peak and a decrease in the N-acetyl aspartate peak A. TractoBecause we did not have enough data on the semiology of the seizure, we advised him to take anticonvulsant treatment according to the ILAE recommendation from 2017. Studies show that these lesions occur during neurogenesis [Usually, MVNT is diagnosed due to adult-onset refractory epilepsy, headache related incidents, or incidentally. Especially in childhood, MVNT could be associated with focal cortical dysplasia . A serieBrain CT is usually normal or shows a non-enhancing/non-calcifying cortical/subcortical white-matter hypodense lesion or asymmetry ,8. MRI lOne of the difficulties we encountered was the differentiation between a single situation-related seizure and the onset of structural epilepsy. What may support that the diagnosis in the first scenario was the generalized onset, from the family description, and the context in which it occurred—sleep deprivation and substance use. In this situation, we can say that the tumor was discovered incidentally. On the other hand, in structural epilepsy, seizures are focal with or without lateralization, therefore possibly with temporal lobe semiology. This is difficult to prove in this hypothesis through a semiology based on the relatives’ statements, a short EEG monitoring, and with a variety of possible triggers.Therapeutic management can be achieved with antiepileptic drugs or surgery. Drug treatment has the advantage of being noninvasive, but with the risk of potential side effects. The possible adverse effects, and the fact that its administration is required throughout the entire life, were the main disadvantages for our patient. At the same time, surgical therapy may have the chance of complete remission of epileptic seizures and confirmation of the diagnosis based on histological analysis, which was only a probable diagnosis on imaging criteria, but with the possible disadvantage of presenting postoperative seizures or deficits on the surgical scar. To conclude, we consider that this tumor was discovered due to a generalized onset seizure triggered by multiple factors, and the most correct attitude would have been to carefully monitor. This is a rare case found in medical practice and is also rarely described in the literature. Our case added more imaging details corroborated with the histopathology features of MVNT. FLAIR images revealed hypointense nodules surrounded by hyperintense peripheral rings and areas of high signal intensity between the nodules, which correspond to the histopathological architecture. Additionally, to our knowledge, it is the first case in which spectroscopy and fiber tractography aspects were analyzed, excluding a high-grade tumor, and this confirmed the disruption of the temporal stem fibers.


Celiac artery compression syndrome (CACS) is an uncommon and poorly understood condition. Compression of the celiac artery by the median arcuate ligament causes intractable postprandial abdominal pain, weight loss, vomiting, and nausea. We present a case of a 68-year-old male who suffered recurrent severe episodes of postprandial abdominal pain associated with occasional nausea, vomiting, and elevated blood pressure. The diagnostic workup was significant for celiac artery compression on computed tomography angiography. Diagnosis of CACS was made after the exclusion of the other possible pathologies, and the patient was referred to the surgical team for further management for median arcuate ligament release on an elective basis. Celiac artery compression syndrome (CACS) is a rare condition caused by the compression of the celiac artery by the median arcuate ligament, which is a fibrous band of the diaphragm. It is also known as median arcuate ligament syndrome (MALS). CACS is a rare cause of postprandial abdominal pain with an incidence of two out of 100,000 patients . DiagnosWe present a case of a 68-year-old male with a medical history significant for diabetes and hypertension. He presented with recurrent severe abdominal pain for about two weeks. The pain was tearing in nature, epigastric, and periumbilical. The pain did not radiate or change with certain positions. It was aggravated with food intake and could last for several hours. It was associated with occasional nausea and vomiting. The patient reported that he used to have mild epigastric pain that was aggravated by meals for the past couple of years. There was no diarrhea, constipation, fever, changes in the urine or stool colors, or signs of gastrointestinal (GI) bleeding.On presentation, he was hemodynamically stable, with elevated blood pressure during the pain attacks. Physical examination was significant for epigastric tenderness with normal bowel sounds. His laboratory findings are shown in Table Electrocardiogram (EKG) showed normal sinus rhythm. Abdominal ultrasound was done, and it did not show significant findings. An esophagogastroduodenoscopy (EGD) was done and showed mild nonspecific gastritis. CTA was done with suspicion of chronic mesenteric ischemia, and it showed significant compression of the origin of the celiac trunk by the median arcuate ligament suggestive of MALS, with normal mesenteric circulation. There were no hepatic or biliary abnormalities Figures , 2.Diagnosis of CACS was made based on the clinical presentation and radiological findings. Other possible causes like acute pancreatitis, mesenteric ischemia, peptic ulcers, and symptomatic gallstones were excluded. Conservative management was started then the patient was referred to the surgical team for further management for median arcuate ligament release on an elective basis.CACS is an uncommon condition caused by compression of the celiac trunk by the median arcuate ligament. It causes impaired blood supply to the GI tract . The firCACS can be found incidentally in many asymptomatic patients showing radiographic signs of celiac artery compression . CACS caImaging modalities like CTA or abdominal duplex ultrasound (US) with respiratory maneuvers can be used to make the diagnosis after the exclusion of other potential etiologies ,7. One oDifferent surgical approaches have been discussed in the literature like open, laparoscopic, or robotic ligament release, celiac ganglionectomy, and celiac artery revascularization. Angioplasty has a limited role in CACS management since this intervention does not address the underlying extrinsic compression resulting in symptoms, although angioplasty with stenting may be used in refractory cases . SurgicaCACS is a rare condition with significant consequences. Affected patients may develop intolerable postprandial abdominal pain, causing severe malnutrition and weight loss. The results of surgical management are varied but usually cause significant symptom improvement. Unfortunately, recurrence can occur, and a minority of patients continue to have symptoms despite surgical treatment.


The female genital tract can be involved as a secondary manifestation of disseminated lymphomas or leukemia but can rarely be the primary site of so-called extranodal lymphomas. Primary lymphomas of the female genital tract can affect the uterine corpus, uterine cervix, vulva, vagina or adnexa. Only about 0.008% of all cervical tumors are primary malignant lymphomas. The presenting symptoms are unspecific and also refer to much more common diseases of the female genital tract. Cervical cytology is usually negative. Preoperative diagnosis requires deep cervical biopsy. To date there is no consensus regarding optimal treatment. Radiotherapy, chemotherapy and surgery are used in different association. We report the first case reported in literature managed with an urgent hysteroscopic resection of a primary cervical diffuse large B-cell lymphoma, followed by adjuvant radiotherapy. Relevant literature was reviewed. Our conservative approach needs to be validated in the future, especially for women with pregnancy desire and for those with low performance status. To date, after 24 months from diagnosis, our patient is still disease-free. The female genital tract can be involved as a secondary manifestation of disseminated lymphomas or leukemia, but is rarely the primary site of so-called extranodal lymphomas. Primary lymphomas of the female genital tract can affect the uterine corpus, uterine cervix, vulva, vagina or adnexa. From the early 1970s to the early 21st century, the incidence rates of non-Hodgkin lymphomas (NHL) nearly doubled in United States. Currently, NHLs account for around 4% of all new cancer diagnoses ,6,7,8,9.In early stages, it is quite easy to define it as a primary cervical lymphoma since the disease is confined to the uterine cervix, while it is different in advanced disease due to diffuse lymphadenopathy or even bone marrow involvement. The strictest definition of primary extranodal NHL is “lymphoma presenting only in extra-nodal sites, with no visible lymphadenopathy on imaging” ; thus, oA history of prolonged minor abnormal vaginal bleeding is the most common presenting symptom of primary cervical lymphoma. Heavy bleeding at presentation is rare, with only a few cases reported in literature ,16,17. “To the best of the author’s knowledge, literature about primary lymphomas of the uterine cervix is limited to single case reports and limited case series. There are no greater studies or randomized controlled trials regarding diagnostic algorithms or treatment strategies. Hence, no published guidelines exist.Considering the available literature, chemotherapy seems to be the cornerstone of cervical lymphoma management, alone or in combination with radiotherapy and/or surgical resection. This comes from the established treatment of nodal NHLs . The mosPrognosis of primary cervical lymphomas is usually excellent, with only a few cases of relapse or death reported ,21,22 inThe aim of our study is to present the first case of DLBCL (I E) of cervix uteri managed with an hysteroscopic approach followed by adjuvant radiotherapy. This is a starting point to validate in the future a new conservative and low-invasive treatment that is useful for those women who desire pregnancy or have a low performance status.2 96%). An 83-year-old woman presented to our emergency department for a sudden copious vaginal bleeding without any other symptom. The woman was in good general conditions (ECOG Performance Status 0) with only hypertension and dyslipidemia in her past medical history. Considering her obstetric–gynecologic history, she had two vaginal spontaneous deliveries and a spontaneous menopause without any other reported atypical vaginal bleeding since she was 51 years old. The last Pap test, performed two years before, was reported negative. At time of presentation, her vital signs were all in normal range , and the ovaries were atrophic. During the exam the bleeding worsened, so an urgent hysteroscopy was performed to control the bleeding and obtain at least a biopsy of the mass. The procedure was carried on under general anesthesia without intubation. Hysteroscopy revealed the presence of an endocervical lesion of about 4 cm with a whitish appearance, fibrous consistency and accentuated vascularization.Hysteroscopic resection was performed with monopolar electrodes using glycine 1.5% as irrigant solution. Removed tissue appeared friable and easily bleeding, with areas of cerebroid aspect. Cautious resection of the mass was performed until the internal uterine orifice was visualized. Continuing the hysteroscopy inside the uterus, an apparently regular uterine cavity with an atrophic endometrium was found. At the end of the procedure, hemostasis was achieved through electrocoagulation and iodoform gauze apposition.The patient was dismissed after two days in good general conditions, with no pain, no vaginal bleeding and a reconstituted cervix. Blood tests were unremarkable.Histologic examination of resected tissue showed a pleomorphic population of malignant lymphoid cells with a diffuse growing pattern and high mitotic index . ImmunohThe follow-up consists of hematological and gynecological examination every 3 month during the first 2 years, then every 6 months for the next 3 years, and annually thereafter to monitor for late relapse. In addition, a PET/TC control is performed every 6 months. To date, 20 months after diagnosis, the patient is still disease-free.As often happens for rare diseases, the therapeutic strategy is not uniform due to the lack of cases studied. What we know about lymphoma therapy is taken from the deep-rooted knowledge of nodal NHL treatment. In general, the cornerstone of treatment is chemotherapy; most reported cases are treated accordingly with chemotherapy alone or in association with radiotherapy . SurgeryWe tried a hysteroscopic approach mainly for two reasons: to rapidly achieve hemostasis and to obtain a biopsy of the mass. However, during the procedure, after a partial reduction in the lesion and hemostasis that allowed a better visualization, we identified a cleavage plan that made it technically possible to remove the whole mass ,28.At diagnosis, about 30% of women are under 40 years of age and likely desire pregnancy; another 15% are over 80 years old and should not be eligible for chemotherapy . Taking To date, in young women who want to preserve their fertility the approach has been chemotherapy (CHOP—cyclophosphamide–doxorubicin–vincristine–prednisone chemotherapy regime) and immunotherapy to reduce the need for radiotherapy or surgical resection. The reported evidence regarding non-Hodgkin’s lymphoma in general suggests that in cases of childhood NHL, and in cases where fertility preservation is desired, current chemotherapeutic regimens are safe and can spare fertility, particularly when GnRH agonists are used in conjunction with treatment . Other fIn patients who become pregnant after cervix uteri lymphoma, the mode of delivery should be well-discussed before childbirth, particularly if surgery or RT has been performed. RT does not seem to reduce the possibility of a safe vaginal delivery  even if the safety of vaginal delivery after RT is still not adequately known [For this reason, in young women considering future pregnancy, a hysteroscopic removal of the mass followed by RT could be preferred over conization or trachelectomy as a more conservative approach.Even if avoidance of chemotherapy as a general rule cannot be stated, it will be important to study whether a local approach such as hysteroscopic surgery followed by adjuvant RT could be more effective and safer than a systemic approach with chemotherapy in the early stage of DLBCL of the cervix. A similar approach was described by Lee et al., who performed radiotherapy after radical surgery in two younger women . In 1998In fact, avoiding systemic chemotherapy allows for the bypassing of its possible adverse effects such as anemia, diarrhea, nausea, hair loss, tiredness, mucositis, bladder irritation, neutropenia, infections, fluid retention and edema, appetite loss, stomach ache or indigestion, high blood sugar, numbness in hands or feet, jaw pain, dry skin, weakened nails, weight changes and vomiting . FurtherLonger follow-up will be needed to better evaluate the long-term outcome of our patient. To date, after 24 months from diagnosis, she is still disease-free. Primary cervical lymphomas in early stages usually have excellent prognosis with any treatment combination ; hence, the aim is to find out the most conservative and least invasive treatment approach . In this


Many surgeons are familiar with small bowel perforation-a breach in the continuity of the bowel wall resulting in spillage of contents into the peritoneal cavity. Usually, patients present with severe abdominal pain, and radiological investigations suggest pneumoperitoneum. However, intestinal perforation secondary to electrocautery used for umbilical granuloma excision is rare. We report a case of a 4-month-old boy who presented with primary concerns of constipation, severe abdominal pain, and multiple episodes of vomiting three days following an electrocautery excision of umbilical granuloma. An exploratory laparotomy revealed a perforation of the terminal ileum. Primary repair of the ileal perforation was done, which saved the infant’s life. As this case illustrates, even a minor surgical procedure can lead to a major intraperitoneal injury, and appropriate evaluation based on clinical signs and symptoms is imperative. This case is also a reminder to handle an electrosurgical instrument with proper skill, training, and technical assistance. An umbilical granuloma is an overgrowth of granulation tissue formed during the healing of the umbilical stump after the umbilical cord is separated. It is the most common umbilical abnormality found in infants, occurring in one in 500 newborns . The oveA 4-month-old male child, with good general health, had been operated on at a single-practitioner, private healthcare unit for umbilical granuloma, wherein excision was done three days prior to the presentation via electrocautery, most likely unipolar electrocautery. Figure The child was referred to the emergency unit of a tertiary care center with concerns of fever lasting one day, acute abdominal pain, and multiple episodes of vomiting. There was no history of any illness or trauma prior to the procedure. The child was febrile, with a temperature of 101° F, and irritable, crying excessively, with a pulse of 160 beats per minute and tachypnoea. His abdomen was distended and tender, and he displayed guarding. We noted a few cautery burn marks near the umbilicus. There was no palpable hernia. An erect X-ray of the abdomen was suggestive of pneumoperitoneum. Given his history of prior surgical intervention and clinical and imaging signs, we decided to proceed with an emergency laparotomy by a team consisting of the pediatric surgeon, general surgeon, pediatrician, and pediatric anesthesiologist, with backup support from the pediatric intensive care unit (ICU).An exploratory laparotomy revealed fecal contamination of the peritoneal cavity (as seen in Figure There was no evidence of vitello-intestinal duct remnants. We performed a double layered, primary repair of the ileal perforation. The procedure was uneventful; however, during the postoperative period, the patient had a fever for 48 hours which was managed with broad-spectrum antibiotics and antipyretics.The child developed a burst abdomen on postoperative day eight, for which a secondary closure of the abdomen was done with tension sutures. Mesh or skin graft use was not required. The child needed care in the ICU with a multidisciplinary team, appropriate antibiotics, dietary supplements, and supportive care. He recovered and was discharged home.Bowel injury after excision or electrocautery of umbilical granuloma in a child is rarely seen. A search of the PubMed Index or Google Scholar does not report a single such article ,6. TheseThe electric cautery method is now rarely used, and the application of silver nitrate is the usual treatment . In caseUsing a high- or low-heat cautery point may also be one of the determinants of cautery burns. When the high-heat cautery comes into contact with tissue, charring acts as a protective layer and prevents further destruction of the underlying tissue to a certain extent . With loThermal injury to the bowel in a child undergoing electrocautery excision for umbilical granuloma is rare. This case described a child who underwent a minor procedure and presented with fever and abdominal pain three days postoperatively. As clinical and imaging findings were indicative of perforation, the decision for surgical intervention was evident and life-saving. The classical sign of gas under the diaphragm is diagnostic even in a child. As this case highlights, even a minor surgical procedure can lead to a major intraperitoneal injury, and appropriate evaluation based on clinical signs and symptoms is imperative. Also, handling an electrosurgical instrument requires the proper skill, training, and technical assistance.


The resection of tumors within the primary motor cortex is a constant challenge. Although tractography may help in preoperative planning, it has limited application. While it can give valuable information on subcortical fibers, it is less accurate in the cortical layer of the brain. A 38-year-old patient presented with paresis of the right hand and focal epileptic seizures due to a tumor in the left precentral gyrus. Transcranial magnetic stimulation was not applicable due to seizures, so microsurgical resection was performed with preoperative tractography and intraoperative direct electrical stimulation. A histopathological assessment revealed a diagnosis of glioblastoma. Postoperative magnetic resonance imaging (MRI) showed complete resection. The paresis dissolved completely during follow-up. Surgery within the precentral gyrus is of high risk and requires multimodal functional planning. If interpreted with vigilance and consciousness of the underlying physical premises, tractography can provide helpful information within its limitations, which is especially subcortically. However, it may also help in the identification of functional cortex columns of the brain in the presence of a tumor. Operations within cerebral areas of primary function, where tissue damage will most likely lead to clinically relevant neurological deficits, present a constant challenge in the field of neurosurgery . EspeciaThe key advantage of tractography is the anatomical identification of subcortical nerve fibers. This has advanced neurosurgical planning and assists in risk stratification before surgery . TherefoThis case report demonstrates how these findings and considerations may be useful for improving patient safety in high-risk surgery.A 38-year-old male patient presented to our department after first onset of focal seizures in the right hand. Magnetic resonance imaging (MRI) revealed a cystic tumor of 32 mm with bordering contrast enhancement in the left precentral gyrus. After the start of anticonvulsant treatment with levetiracetam by our department, no further seizures occurred. A mild weakness of the right hand slowly improved after the start of antiedematous treatment with dexamethasone. It may be of further interest that the son of the patient had succumbed to an anaplastic ependymoma at the age of four, which is an unusual coincidence and raises the question of an underlying cause, considering that ependymoma is sometimes classified as a kind of glioma [Diffusion tensor imaging tractography was performed as described before  . We acquThe tumor was found to dissect the origin of the corticospinal tract (CST), and the tractogram suggested that the tumor lay beneath the primary motor cortex, with axons displaced ventrally . PreoperTherefore, we planned a microsurgical resection of the tumor using intraoperative electrophysiological monitoring and 5-aminolevulinic acid fluorescence. The patient gave his informed consent after weighing the risks and benefits with the assigned surgeon. Due to the partially inconclusive results of preoperative tractography, direct electrical stimulation (DES) at 15 mA was used during surgery, complemented by ultrasound guidance. This disclosed the functional location of the tumor 5 mm deep within the left precentral gyrus without provoking an epileptic fit. With regard to the delicate location of the tumor, the cyst was aspirated with a Cushing cannula and DES was then performed at 2 mA within the cyst. This revealed that primary motor neurons were only present dorsal to the cyst, so that a microsurgical resection of the tumor could be performed. Due to the excitability of the right hand by DES, some remnants of fluorescent tissue had to be left in situ.Postoperatively, there was no aggravation of the preexisting paresis, which completely resolved after two weeks. Postoperative MRI showed a complete resection of the cyst with its contrast enhancing capsula . The posAnticonvulsant medication was continued after surgery for prophylactic reasons.Histopathological assessment and further molecular analysis of the specimen resulted in the diagnosis of glioblastoma WHO grade 4, isocitrate dehydrogenase wildtype, O(6)-methylguanine-methyltransferase promoter methylation 26.6%. It also revealed gains in chromosomes 7 and 19 as well as the loss of chromosomes 10, 12, 13q, and 22q. Radiotherapy with concomitant temozolomide and lomustine was initiated due to young age and promoter methylation .The anatomical approach of tractography has been proven to accompany techniques such as transcranial magnetic stimulation or functional MRI in a valuable and reliable fashion ,12. HoweFocal epileptic seizures are a common symptom of glioblastoma and require immediate anticonvulsant treatment. Anticonvulsant treatment has a good success rate, which is mirrored by the presented case .Microsurgical tumor resection was assumed to be of high risk in the present case and was therefore meticulously planned as well as thoroughly discussed with the patient. transcranial magnetic stimulation may have yielded valuable information on the exact location of primary motor neurons . HoweverNevertheless, intraoperative DES revealed primary motor neurons to be placed only dorsally to the lesion. Most importantly, DES was in line with the postoperative course of neurological improvement.This may raise the question of whether DES alone would have been sufficient. We would like to discourage this conclusion, since it is our belief that the correct application of different diagnostic modalities can deliver valuable additional information by complementation. In this case, the success of DES was at least partially due to preoperative tractography, which correctly included primary motor neurons, although it remained partially inconclusive regarding their precise location.Although fluorescent tissue rims had been left during resection, postoperative MRI disclosed complete resection of the contrast-enhancing tumor parts. Postoperative tractography showed larger portions of the CST dorsally to the situs than preoperatively . One reaNotably, the ventral fibers do not appear severed in postoperative tractography. This may have been important for neurological recovery, as white matter integrity of the premotor regions has been found to be associated with motor output after stroke .As familial glioblastoma is extremely rare in adults, the prior anaplastic ependymoma in the patient’s son was most likely a statistical coincidence . This isCST tractography can assist in neurosurgical planning for the resection of lesions in the precentral gyrus. In selected cases, the resection of glioblastoma in this region is possible without neurological deficit. However, functional planning is crucial and cannot rely on one technique alone. As each modality has its own limitations, this case highlights the necessity to interpret tractography results with vigilance and consciousness of the underlying physical premises. Patients have to be informed about the high risk of postoperative paresis.


Lipoblastoma is a rare benign soft tissue neoplasm rising from embryonic white adipose tissue known as lipoblast that keeps proliferating during the postnatal period. Although lipoblastomas are benign, they often grow rapidly. Most lipoblastomas are asymptomatic at presentation; they can present as a growing painless palpable mass and progressive symptoms of various organ compression depending on localization. A giant mesenteric lipoblastoma is a rare case with only a few cases reported. An infant with large intraabdominal masses may present preoperative diagnostic difficulties. Differential diagnoses are broad and may include sarcomas, germ-cell tumors, lipomas, lymphomas, hepatoblastomas, Wilm’s tumors, and neuroblastomas. Thorough clinical, radiological, and pathological investigations are ultimately required to obtain a definitive diagnosis. Regardless of location, the treatment of choice for lipoblastoma is complete surgical resection. All patients should be followed up for a minimum of five yearsWe report a rare case of a giant compressive mesenteric lipoblastoma that was initially suspected as abdominal malignancy in a nine-month-old infant. As physicians, we must always consider the underlying cause as well as the malignant or benign nature of a growing mass to treat the patient appropriately. Lipoblastoma is a rare benign soft tissue neoplasm originating from embryonic white adipose tissue known as lipoblast that continues to proliferate during the postnatal period -5. ThereLipoblastoma primarily occurs in infancy and early childhood, with approximately 90% of cases occurring before the age of three and having a slight male predominance. The extremities and trunk are the most prevalent sites ,7,8. OthWe report a case of a giant compressive benign mesenteric lipoblastoma in a nine-month-old infant presenting with a painless growing abdominal mass with no notable symptoms.A nine-month-old infant was referred to our pediatric surgery clinic for evaluation of a distended abdomen. He presented with an enlarging mass on the left side of his abdomen with an abnormal ultrasonography (USG) examination provided by the referring pediatrician. He was born pervaginally with no complications perinatally. There were no obvious congenital anomalies at birth, nor was there a family history of sickness. At the age of eight months, the infant was first brought to a healthcare facility by his parents with the chief complaint of an enlarging mass on the left side of his abdomen. His parents had noticed the growing mass two months ago. The mass was said to be growing rapidly. At the first presentation, the mass measured about the size of a tennis ball (~6 cm ✕ 6 cm) at the left hypochondriac region. There were no notable symptoms other than a delay in his gross-motor developmental milestones (he was not able to sit by himself yet). The USG examination revealed there was a left suprarenal inhomogeneous mass with suspicion of neuroblastoma. The patient was subsequently referred to the pediatric surgery department at our hospital for further investigation of abdominal malignancy.The patient came to our hospital one month after the first encounter with the healthcare provider. The patient’s abdomen was distended Figure  A with aBased on the physical and radiologic examination, the doctor ordered an USG-guided fine needle aspiration biopsy (FNAB) to be done at the targeted mass. Cytopathology showed mature adipocytes and lipoblasts, both uni-vacuolated and multi-vacuolated lipoblasts Figure . CytopatThe pediatric surgeon decided to do a laparotomy and radical resection on the suspected benign mass Figure . The proAdipose and myxoid tumors in children are uncommon, and the majority are benign. In the first two decades of life, less than 10% of soft tissue lesions are tumors originating from adipose tissue . LipoblaAlthough lipoblastomas are benign, they often grow rapidly. Most lipoblastomas are asymptomatic at presentation and can present as a growing painless palpable mass with progressive symptoms of various organ compression depending on localization ,6,14. PaA giant mesenteric lipoblastoma is a rare case with only a few cases reported -17. TherIn our case, the pediatrician first diagnosed the patient with neuroblastoma from the clinical and the USG findings. Neuroblastoma is the most common intra-abdominal and extracranial solid tumor found in infants . With ouHistologically, lipoblastoma characteristically demonstrates lobulated architecture formed of sheets of adipocytes with variable maturation  separated by fibrovascular septa. Lipoblastoma may exhibit a zonal pattern of maturation with more immature myxoid cells at the periphery adjacent to fibrous septa, and mature adipocytes at the center of the lobule. The myxoid regions can occasionally show pooling of matrix similar to myxoid liposarcoma. However, mature areas may resemble lipoma ,3. DiffeIf clinical and histologic examinations are not sufficient to differentiate between these two tumors, cytogenetic testing may be indicated ,10,14,22Regardless of location, the treatment of choice for lipoblastoma is complete surgical resection ,5,9,15. In conclusion, we report a rare case of a giant compressive mesenteric lipoblastoma that was initially suspected as abdominal malignancy in a nine-month-old infant. In patients with a large distended abdomen that grows progressively, abdominal ultrasonography and CT scan aid in the initial diagnosis to adjust the preferred management. Meanwhile, histopathology and immunohistochemistry examinations emphasize the definitive diagnosis of these cases for the prognosis and treatment. As physicians, we must always consider the underlying cause as well as the malignant or benign nature of a growing mass in order to treat the patient appropriately.


Hepatocellular Adenomas (HA) are rare benign tumors of the liver which occur predominantly in young women. Although benign, HA may have complications such as hemorrhage and malignant transformation. So, sometimes conservative management is not enough.We report a case of a 26-year-old woman on oral contraceptives who presented with acute abdominal pain and signs of hemodynamic shock. She underwent transarterial embolization due to the presence of multiple HA with rupture and active hemorrhage. This minimally invasive treatment failed to control the disease. The patient presented a progressive increase in the size of the masses with signs of recent hemorrhage, and the HA became unresectable, so she underwent liver transplantation.Liver transplantation is rarely indicated for the treatment of HA; however, in unresectable masses, it should be considered to prevent potential rupture with hemorrhage or malignant transformation. Hepatocellular Adenoma (HA) are benign liver tumor with an incidence of 1-4 per 1000,000 adults per year ,3.Although they are benign lesions, HA can undergo two life-threatening complications. Rupture and subsequent hemorrhage is a complication found in 15%-40% of patients with HA, and malignant transformation into hepatocellular carcinomas is found in 5% of those tumors. The risk of both complications is higher with increasing tumor size -7.Treatment strategies for HA range from conservative measures with oral contraceptive discontinuation and radiologic surveillance to liver resection or transplantation -11.Liver transplantation for HA has been reported but is a rare indication. So far, a few case reports were described, a large multicenter study from Europe reported 49 cases of liver transplantation for liver adenomatosis between 1986 and 2013, and a systematic review in the United States reported 142 cases of transplantation for HA between 1987 and 2020 [We report a case of a young woman with ruptured hepatocellular adenoma with hemorrhage, who underwent liver transplantation after failure of disease control with transarterial embolization (TAE).A 26-year-old woman was admitted to the emergency department complaining of abdominal pain for two hours in February 2021. Her medical history included obesity, diabetes mellitus, arterial hypertension, autoimmune thyroiditis, depressive disorder, and oral contraceptive use.On physical examination, the patient was afebrile, with an initial blood pressure of 130/78 mmHg, heart rate of 68 beats per minute, and significant tenderness to palpation over the right upper quadrant. The rest of the examination findings were unremarkable. The initial laboratory results showed a hemoglobin of 14.5 g/dl, alanine aminotransferase (ALT) of 75 U/L, and aspartate aminotransferase (AST) of 66 U/L. A contrast-enhanced computed tomography (CT) of the abdomen demonstrated a right subcapsular hematoma, measuring 22x16x10 cm, with active hemorrhage and various HA (the largest being on the right lobe with 11 cm and one in the left lobe with 6.5 cm) Figures , 1B. A repeated blood test showed a drop in hemoglobin level to 9.5 g/dl, so she was transferred to another hospital for interventional radiology. She underwent selective TAE on suspected arteries providing a vascular supply to three hypervascular masses in the right lobe of the liver. A few hours later, she became disoriented, her blood pressure was 134/81mmHg, her heart rate raised to 130 beats per minute, and she had a hyperlactatemia of 16 mmol/L and hemoglobin of 7.7g/dl. A repeat CT scan showed an increased size of adenomas, signs of active hemorrhage of one hepatic mass in the right lobe, and intraperitoneal effusion Figures , 2B. A diagnosis of hemorrhagic shock was made, so she received a blood transfusion and underwent a second selective TAE of the vessels that supplied the hypervascular mass in the right lobe.After the second TAE, she remained in the hospital. During hospitalization, the oral contraceptive pills were discontinued. The patient underwent surveillance of the adenomas by serial CT scans that showed a progressive increase in the dimensions of the masses, without active hemorrhage but with signs of recent hemorrhage. Twenty-four days after the second embolization, she presented acute abdominal pain, hypotension of 88/50 mmHg, tachycardia of 150 beats per minute, and hemoglobin drop from 8.9 g/dl to 6.4 g/dl. A CT scan showed an increase in the size of HA and subcapsular hematoma, with signs of recent hemorrhage Figures , 3B. She received a blood transfusion and became hemodynamically stable. Considering the lack of clinical stability, the unresectable liver lesions due to insufficient remaining liver volume, and the high risk of rupture and hemorrhage of the lesions, the patient was proposed and approved for liver transplantation. The patient was transferred to a referral center for liver transplantation. Laboratory evaluation showed abnormal liver tests, including an increased ALT of 106 U/L, AST 53 U/L, alkaline phosphatase 579 U/L, gamma-glutamyltransferase 515 U/L, prothrombin time of 19 seconds, and a decreased albumin of 2.7 g/dl. The α-fetoprotein was normal.In April 2021, she underwent deceased donor liver transplantation with the piggyback technique. The explant liver weighed 4050 g, and there was no evidence of tumor invasion into the capsule or blood vessels. The major part of the tumor mass was hemorrhagic and necrotic. Based on the molecular classification, the final pathologic diagnosis was exon 7-8 β catenin activated HA and unclassified HA.A follow-up two months after the surgery revealed normal liver tests, and the CT scan showed no sign of recurrence of tumor or metastasis. She is well one year after liver transplantation, immunosuppressed with tacrolimus and mycophenolate mofetil.HA are rare benign liver tumors. Its incidence has risen in the last decades due to the increased use of oral contraceptives, the prevalence of obesity and metabolic syndrome, and the diffuse use of imaging radiology with its incidental discovery ,14,15. TBased on the molecular behavior, HA has been classified into five main groups: hepatocyte nuclear factor 1α-mutated HA, inflammatory HA, exon 3 β-catenin-mutated HA, exon 7-8 β-catenin mutated HA, and unclassified HA. In 2017 Jean-Charles Nault et al. proposed a new classification with three more subtypes. It is known that β-catenin-mutated HA are at increased risk for hemorrhage and inflammatory HA are at increased risk for hemorrhage and malignant transformation, so this classification can be useful to decide the best treatment option for each patient. However, although magnetic resonance imaging is the modality of choice for the diagnosis of HA, it does not allow to reach an accurate differentiation between subtypes ,7,15,16.HA is typically asymptomatic and incidentally found on physical examination, when abdominal imaging is performed for other reasons, or during exploratory laparotomy for another indication. The remaining patients are symptomatic and could have abdominal pain due to hemorrhage within the tumor or acute intraperitoneal hemorrhage due to rupture of the tumor. Rarely HA can undergo malignant transformation -10. Our The management of HA can vary according to patient and tumor characteristics. All women should discontinue oral contraceptives, and the obese should lose weight. In most patients, considering the risk of bleeding or malignant transformation, conservative management with radiologic surveillance is not enough. So, surgical resection is recommended for women with tumor diameters greater than 5 cm, for all men regardless of the tumor size and if there is a proven β-catetin activated HA. The optimal treatment for ruptured HA is not established. However, considering the high risk of morbidity and mortality with emergent surgery, TAE should be considered to stabilize the patient, and the liver resection should be delayed to an elective procedure. Also, some authors have suggested that TAE can reduce or even lead to the disappearance of some tumors -8,10,15.If resection is not technically feasible or brings high operative morbidity or mortality, liver transplantation should be considered. In rare cases, it should also be considered for patients with ruptured HA when conservative measures fail, and surgical resection is not feasible ,10,13,14It is difficult to make evidence-based criteria for liver transplantation in HA. All cases should be discussed by a multidisciplinary group since this is a benign disease. There is high morbidity and mortality associated with transplantation, and liver grafts are scarce ,14.HA are rare liver tumors that most commonly occur in young women with oral contraceptive use. Although benign, HA can undergo two life-threatening complications: rupture and subsequent hemorrhage and malignant transformation. These risks increase with increasing tumor size. The treatment should be individualized and can range from conservative measures to surgical resection or liver transplantation. Although liver transplantation is a rare indication, it should be considered if the resection is associated with high morbidity and mortality or is technically not feasible.


Primary intraosseous cavernous hemangioma is a benign tumor with slow growth and is rarely seen in clinics. The clinical manifestations of most patients are progressive enlargement of the head mass.We report a 30-year-old female patient with cavernous hemangioma at the frontoparietal junction. Upon admission, the right frontal lobe mass was progressively enlarged for 3 years and underwent lesion resection and stage I skull reconstruction. The postoperative outcome was good, with no recurrence at 1-year follow-up.Primary intraosseous cavernous hemangioma is a relatively rare clinical tumor, the pathogenesis of which is still unclear, and most of them have no specific clinical manifestations. Characteristic imaging findings are highly suspicious of this disease, but the definitive diagnosis still depends on histopathological examination. Currently, total surgical resection of the tumor is a relatively effective and preferred treatment. Primary intraosseous cavernous hemangiomas (PICHs) are benign, slow-growing tumors that are clinically rare, accounting for approximately 0.2% of all bone tumors, and can occur in different parts, often in the frontal bone . PICHs aThe patient was a 30-year-old female who had a progressive enlargement of the right frontal mass for 3 years. Physical examination at admission showed a right frontal mass, which was hard, had no tenderness, and had poor range of motion. The patient was conscious. The bilateral pupils were equally large and rounded, and the reflex to light was sensitive. The neck was soft. The limbs could move as instructed, and the muscle strength and tension of the limbs were normal. The limbs appeared to be normal. Physiological reflexes were normal, and no pathological signs were elicited. CT scan manifestation showed no abnormal density shadow in all layers of brain parenchyma, round swelling bone changes in right frontoparietal junction, thinning of bone cortex, radiolucent internal bone, uneven density, and adjacent brain tissue compression. Bone changes in the right frontoparietal junction, benign bone tumor, or meningioma cranial changes were also observed . CranialPICH is a rare benign tumor, accounting for approximately 0.2% of all bone tumors. The incidence occurs in 40- to 50-year-olds. It can occur in both men and women, with a male-to-female ratio of 3:1 to 2:1 (KRIT1/CCM1 gene frameshift mutation. Others (Its pathogenesis is still unclear at this point, but some scholars  suggeste. Others  suggestePICHs mostly have no obvious clinical symptoms, and most of them are painless progressive enlarging masses. Sixteen patients with PICH reported by Chengjun Wang et al.  mainly sCT is the most commonly used imaging examination. PICH is characterized by mostly expansive growth, showing an expansion of the plate barrier, mostly osteolytic destruction, thin borders, integrity of the internal and external plates of the skull. There are radial bone septa and radial bone needles perpendicular to the inner plate of the skull. It shows typical osteolytic “honeycomb,” “soap bubble,” or “sunburst” changes , 8–10. MFor patients with PICH, surgical complete resection of the entire tumor is often an effective and preferred treatment option . One stuIn summary, PICH is a rare clinical tumor, the pathogenesis of which is still unclear, and there may be specific clinical manifestations depending on the location of tumor growth, but most of the patients come to the hospital with progressive enlarging masses. Although characteristic imaging findings are highly suspicious of the disease, definitive diagnosis still depends on histopathological examination. At present, surgical total excision of the tumor is the more effective treatment, but for those tumors with a special location or that were unresectable, radiotherapy can be considered.


Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease.We present a rare case of a 76-year-old female patient with no previous pulmonary history who presented with progressive dyspnea, fatigue, and involuntary weight loss. On chest X-ray and computed chest tomography scan, she was found to have a 16.7 cm × 12.8 cm × 10.1 cm bulky mass occupying the left hemithorax with associated compressive atelectasis of the lung. She underwent a computed tomography guided biopsy that revealed the mass to be a solitary fibrous tumor. The patient underwent left muscle sparing lateral thoracotomy with complete resection of the tumor. Post procedure, the left lung fully expanded. 18 months post-resection, she developed a 3.3 cm × 1.7 cm tumor along the left internal thoracic artery lymph node chain which was histologically identical to the resected tumor. The patient is currently being treated with bevacizumab and temozolomide.Solitary fibrous tumors are very rare pleural tumors. Surgical resection is the treatment of choice followed by close post-operative surveillance. Solitary fibrous tumors (SFTs) are rare, mesenchymal neoplasms originating from fibroblastic or myofibroblastic tissue . AlthougA 76-year-old Caucasian female never smoker with no previous pulmonary history was referred to our institution for evaluation of a biopsy proven 17 cm fibrous tumor involving the left hemithorax. The patient related of a two-month history of dyspnea, lack of energy, poor appetite, and 10-pound involuntary weight loss. She denied any fevers, chills, sweats, or hemoptysis. As part of her evaluation, she had a chest X-ray and a computed tomography (CT) scan of the chest that revealed a bulky 16.7 × 12.8 cm × 10.1 cm heterogeneous mass occupying the left hemithorax with a mass effect involving the left side of the anterior mediastinum  rather than the size of the tumor; benign tumors have an 8% recurrence rate compared to 63% for malignant tumors, even following complete resection . MalignaComplete en bloc surgical resection is the primary treatment for SFTP . For smaSFTPs are rare thoracic tumors that can grow silently. Complete surgical resection is recommended for patients with SFTP. Careful post-operative monitoring is advised after surgery due to the possibility of recurrence.


Epithelial conjunctival malignancies are one of the most prevalent ocular surface tumors. Primary basal cell carcinoma (BCC) of the conjunctiva is extremely rare. We report the case of a 67-year-old Indian gentleman who presented with a fleshy conjunctival lesion for one year on his right eye. Examination revealed a lightly pigmented conjunctival mass adjacent to the limbus. The surface was irregular and non-ulcerated with few feeder vessels. The working diagnosis was ocular surface squamous neoplasia (OSSN). A wide excisional biopsy using the no-touch technique and double-freeze-thaw cryotherapy to the conjunctival margins was performed. The bare scleral area was covered with an amniotic membrane. Histopathological examination revealed a basaloid cell neoplasm favoring BCC, and a tumor-free margin was achieved. Three cycles of topical mitomycin-C 0.02% were prescribed as adjunct chemotherapy postoperatively. There was no evidence of recurrence three months after treatment. The primary BCC of the conjunctiva is unusual and can resemble OSSN. Therefore, it should be considered in the differential diagnosis of patients presenting with atypical features of OSSN. Conjunctival tumors include a spectrum of benign and malignant neoplasms arising from the conjunctival epithelium, conjunctival stroma, and structures within the stroma, including the blood vessels, nerves, fat, and lymphoid tissue. The most frequent invasive epithelial tumors of the ocular surface are ocular surface squamous neoplasia (OSSN) . On the A 67-year-old Indian gentleman presented with a fleshy conjunctival lesion for one year in his right eye Figure .He was a construction worker with long hours of sun exposure. He had no comorbidity, history of trauma, or history of malignancy.Examination revealed a raised, lightly pigmented conjunctival nodular lesion measuring 3 mm (height) × 3.5 mm (width) × 3 mm (length) located at the 10 o’clock position adjacent to the limbus on the right eye. The surface was irregular, fleshy, and non-ulcerated with prominent feeder vessels. Leukoplakia and papillomatous signs, which are frequently seen in OSSN, were not present. The posterior segment of both eyes was normal. There was no evidence of systemic involvement or involvement in the adjacent structures, including the cornea, caruncle, plica semilunaris, or eyelid. The working diagnosis was OSSN.A wide excisional biopsy using the no-touch technique and double-freeze-thaw cryotherapy to the conjunctival margins was performed. The bare scleral area was covered with an amniotic membrane. Histopathological examination revealed a basaloid cell neoplasm favoring BCC with positivity for p53, p63, CD10, and BCL2 and negativity for BerEP4 and EMA immunohistochemically. An adequate surgical free margin was achieved, and the patient was prescribed three cycles of topical mitomycin-C (MMC) 0.02% postoperatively. Follow-up examination over three months after completion of MMC showed no evidence of tumor recurrence.BCC develops almost exclusively on hair-bearing skin, especially on the face due to chronic exposure to sunlight. It accounts for 85-95% of all malignant tumors of the eyelid . BCC hasOf the five primary conjunctival BCC reported in the literature, male cases (n = 4) were more common than female cases n = 1) -7. Patie -7. PatiCausative factors for the development of BCC in the conjunctiva are unclear. Several mechanisms have been postulated. Metaplasia of the conjunctival epithelium secondary to ultraviolet damage or development of BCC from a dermoid choristoma has been suggested in causing BCC in the conjunctiva . FurtherIt is challenging to diagnose primary conjunctival BCC clinically due to its rarity. Occasionally, it can mimic other conjunctival tumors and leads to misdiagnosis. Aftab and Percival reported a primary BCC of the conjunctiva resembling a papilloma clinically . In one The management of OSSN involves surgical removal using the no-touch technique and non-surgical treatments including topical chemotherapy , topical/injection immunotherapy , topical antiviral medication (cidofovir), or photodynamic therapy . ComparaAlthough BCC is a slow-growing tumor, rarely, it can be aggressive locally and can possibly spread intraocularly. In one of the previous reports , tumor cPrimary BCC of the conjunctiva is rarely encountered and can resemble OSSN. Therefore, BCC should be considered in the differential diagnosis of patients presenting with atypical symptoms of OSSN. Timely histopathological examination of suspicious lesions is crucial. Complete excision with tumor-free surgical margins may be sufficient, but may also be combined with adjunctive therapeutic modalities, which may enhance treatment in cases of incomplete surgical removal.


During fatal development, the testes grow in the abdomen and descend into the scrotal sac. It can be stopped at any point along its path (cryptorchidism) or migrate to an atypical side (ectopic testis).A 47-year-old man from Somalia's lower Shabelle region was presented to the urology OPD, He had a history of non-tender abdominal mass for the previous four months, An examination revealed a mass in his abdomen that was firm, non-tender, and immobile, an abdominal ultrasound and a contrast enhanced CT abdomen showed a 15-cm heterogeneous bean-shaped mass above the bladder.After patient counseling and informed written consent, a laparotomy was done to remove the tumor. A seminoma of the undescended testis was identified during a histological examination.An intra-abdominal mass with empty scrotum should raise concerns about an intra-abdominal testicular tumor. To prevent/early discover these types of tumors, cryptorchidism should be treated at a young age, particularly before to the first year. The testes grow in the abdomen throughout fatal development and then descend into the scrotal sac in the third trimester, usually between weeks 28 and 32. It may be stopped at any time along its path (cryptorchidism) or migrate to an abnormal side throughout the descent (ectopic testis) The absence of one or both testes from the scrotum is known as cryptorchidism. It is the most frequent male genital birth defect, with an incidence of 3% in full-term male new-borns compared to 30% in preterm male neonates making it one of the most frequent congenital abnormalities The cryptorchid testis predisposes to testicular cancer, ischemia, and infertility later in life. Testicular seminoma is the most prevalent malignant transformation of an undescended testis A 47-year-old man from Somalia's lower Shabelle region presented to the urology Outpatient Department with a history of a non-tender abdominal mass for the previous 4 months, gradually increasing in size for 3 months and rapidly growing for the last 10 days. He also described a history of sexual dysfunction, libido loss, and infertility. There was no history of pain radiation, no aggravating or relieving factors, no change in bowel habit, melena, weight loss, or lower urinary tract symptoms.He doesn't drink, smoke, or use drugs in any way. His birth history, on the other hand, could not be examined further.A physical examination revealed a patient who appeared to be in good health, with a blood pressure of 130/80 mm Hg, a heart rate of 75 beats per minute (bpm), and a temperature of 37.4°C. An abdominal examination revealed a suprapubic mass that was firm, non-tender, and immobile. His genitalia were examined and revealed the absence of the right testicle in the scrotum and in the inguinal canal, as well as a small testis in the left scrotum.Paraclinical tests revealed no hematuria or proteinuria in the urine, a normal full blood count, no blast cells on the blood smear, and a negative serology for the human immunodeficiency virus (HIV) and hepatitis viral markers.An abdominopelvic ultrasound showed a 15-cm hypoechoic heterogeneous bean-shaped mass above the bladder with internal vascularity and calcification . His livFollowing patient counseling and written consent, an open exploration was performed using a modified Gibson's incision. The right testicular mass was mobilized all around, vessels were ligated, and the specimen was removed.In the histopathological examination, a seminoma of the undescended testis was discovered. On the postoperative fifth day, the patient was discharged.Further management options were discussed with the patient, who was referred to an oncologist and andrologist for further evaluation.The testes grow in the abdomen throughout fatal development and then descend into the scrotal sac in the third trimester, usually between weeks 28 and 32. It may be stopped at any time along its path (cryptorchidism) or migrate to an abnormal side throughout the descent (ectopic testis).Because of the rapid increase in tumor size and free mobility, as well as androgen function deficiencies. Such an organ is at high risk of torsion, trauma, infertility, and malignancy Malignant intraabdominal testes might present as anything from an asymptomatic, incidental mass to an acute abdomen caused by torsion or hemorrhage. The radiologic diagnosis is often challenging since the patient's history of cryptorchidism is not provided, and individuals who had cryptorchidism surgery as a kid are unaware that orchiectomy may not have been done Cryptorchidism should be treated at a young age, preferably prior to the first year, to prevent/early detect these types of tumors In the case of an empty scrotum, an intra-abdominal mass should arouse concerns about an intra-abdominal testicular tumor. Adults with intra-abdominal testicular tumors are more likely to present at an advanced stage than those with intrascrotal testicular tumors. Cryptorchid testis should be investigated with CT or MRI to locate the testis and have orchiectomy performed if they are present in childhood. Such cases of intra-abdominal testicular cancer can only be prevented or detected early in this way.Ethical approval was waived by the ethical committee of Mogadishu Somali Turkey, Recep Tayyip Erdogan Training and Research Hospital.Written informed consent was obtained from the patient for the publication of this case report and accompanying images.The data that support the findings of this study are available in Mogadishu Somali Turkey, Recep Tayyip Erdogan Training and Research Hospital information system. Data are however allowed to the authors upon reasonable request and with permission of the education and research committee.


SDHx mutations. In children however, very little data is available. In this report, we present the case of a 12-year-old female with a SDHB-related metastatic paraganglioma treated with surgery followed by temozolomide therapy. The patient presented with symptoms of palpitations, sweating, flushing and hypertension and was diagnosed with a paraganglioma. The primary mass was surgically resected six weeks later after appropriate alpha- and beta-blockade. During the surgery extensive nodal disease was identified that had been masked by the larger paraganglioma. Histological review confirmed a diagnosis of a metastatic SDHB-deficient paraganglioma with nodal involvement. Post-operatively, these nodal lesions demonstrated tracer uptake on 18F-FDG PET-CT. Due to poor tumour tracer uptake on 68Ga-DOTATATE and 123I-MIBG functional imaging studies radionuclide therapy was not undertaken as a potential therapeutic option for this patient. Due to the low tumour burden and lack of clinical symptoms, the multi-disciplinary team opted for close surveillance for the first year, during which time the patient continued to thrive and progress through puberty. 13 months after surgery, evidence of radiological and biochemical progression prompted the decision to start systemic monotherapy using temozolomide. The patient has now completed ten cycles of therapy with limited adverse effects and has benefited from a partial radiological and biochemical response.There is increasing evidence to support the use of temozolomide therapy for the treatment of metastatic phaeochromocytoma/paraganglioma (PPGL) in adults, particularly in patients with  A 12-year-old girl presented to a clinic appointment with a two-year history of sweating, intermittent flushing and occasional headaches. After monitoring her heart rate using a wearable device, she and her parents noted tachycardia even at rest, with a heart rate ranging from 120 to 145 beats per minute.th centile) while her target height was near the 70th centile. In the absence of any pubertal development, she was also diagnosed with delayed puberty.Upon presentation, she was tachycardic, hypertensive (207/184 mmHg) and a left parasternal heave was palpable. She was noted by the assessing clinician to be of short stature, with a current height of 136.4 cm (0.4A magnetic resonance imaging (MRI) scan of the abdomen was performed, which showed a 6 cm x 3 cm right-sided suprarenal mass abutting the liver, inferior vena cava and hilum, but without features of direct invasion on imaging (68Ga-DOTATATE positron emission tomography (PET-CT) was performed. The mass demonstrated heterogenous 68Ga-DOTATATE uptake  spectroscopy was performed . Due to Six weeks after the patient’s presentation, an open resection of the mass was performed. As the mass was adherent to the gallbladder and liver, a cholecystectomy and partial caudate lobe resection were necessary. Additionally, the mass was found to be invading the inferior vena cava, requiring resection and reconstruction of the retro-hepatic vena cava with a deceased donor aortic graft. Intra-operatively, extensive nodal disease (not identified on pre-operative imaging) was noted including para-aortic and hilar nodes; some of the latter involved the first order bile ducts at the hilar plate which were not amenable to resection.SDHB-deficient paraganglioma. Extensive lymph node involvement was also shown, confirming the diagnosis of a malignant SDH-deficient paraganglioma. Post-operatively a restaging 18F-fluorodeoxyglucose (FDG) PET-CT was performed  and predictive testing has identified several SDHB carriers on the maternal side of the family  single positron emission computerised tomography (SPECT-CT) scan was carried out  are neuroendocrine tumours arising from neural crest cells. These can be functional (catecholamine-secreting) or non-functional. The term phaeochromocytoma refers exclusively to tumours arising within the adrenal medulla, whilst tumours arising in sympathetic and parasympathetic paraganglia elsewhere in the body are referred to as paragangliomas. PPGL are rare tumours in children, with an estimated overall incidence of 0.6 per 100,000 person-years . Of thesSDHx) also predispose to the development of PPGL. Such pathogenic variants are autosomal dominant, and require biallelic inactivation for tumorigenesis to occur (SDHx-deficient cells lead to increased stabilisation of hypoxia-inducible factor 1α (HIF-1α), as well as DNA hypermethylation. This can lead to neoplasia by driving angiogenesis, anaerobic metabolism and promoting hypermethylation (Up to 80% of paediatric PPGL cases are thought to have a hereditary basis . These ito occur . Higher hylation .SDHA/B/C/D). Of these, pathogenic variants in the SDHB gene confer the highest malignant potential, such that the majority (80%) of metastatic PPGL in children occur in patients with SDHB variants , gastrointestinal stromal tumours (GISTs) and pituitary adenomas  . Of thesSDHB mutation . 131I-MIBG is an effective palliative treatment for PPGL , but this is associated with significant side effects such as myelosuppression, peripheral neuropathy and gastrointestinal toxicity . Systemifor PPGL , but canfor PPGL . If the nt stays . In SSTRnt stays .SDHx-deficient PPGL includes a child aged 13 within its cohort ’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.CU drafted and revised the initial manuscript, Ruth Casey obtained funding from GIST Support UK.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Appendiceal diverticulitis, a frequently underdiagnosed entity, differs from typical appendicitis by the presence of an inflamed appendiceal diverticulum. Appendiceal diverticulitis is a surgical emergency which has an increased risk of perforation compared to typical appendicitis. We will discuss a surgically and pathologically confirmed case of complicated appendiceal diverticulitis and its management implications. Appendiceal diverticulitis, a frequently underdiagnosed entity, differs from typical appendicitis by the presence of an inflamed appendiceal diverticulum. Appendiceal diverticulitis is a surgical emergency which has an increased risk of perforation compared to typical appendicitis. We discuss a surgically and pathologically confirmed case of complicated appendiceal diverticulitis and its management implications. Acute appendicitis is one of the most common surgical causes of acute abdominal pain. Appendicular diverticulitis is a rare but important counterpart to appendicitis.Acquired bowel diverticula are blind‐ended outpouchings arising through muscular mural defects connecting with the bowel lumen.Informed consent was obtained from the patient for publication of this case report.A 75‐year‐old male presented to emergency with 2 days of peri‐umbilical and right iliac fossa pain, with tenderness at McBurney’s point and a raised white cell count (15.2x10^9/L).A computed tomography (CT) revealed a thin‐walled 15‐mm diverticulum arising from the appendix tip Figs , with adCT imaging 2 years prior Fig.  revealedThe patient proceeded to appendicectomy, demonstrating appendiceal rupture with gangrenous change.Pathology confirmed that the appendix contained a perforated diverticulum at its tip Fig. . MicroscAppendiceal diverticulitis is rare, with reported surgical incidence up to 3.7%.In practice, preoperative radiological diagnosis is also frequently missed.Radiologically, CT is the imaging modality of choice, by which the patterns of appendicular diverticular disease can be recognised.The rate of perforation in appendiceal diverticulitis ranges from 30 to 70%, more than four times higher than appendicitis, which is likely due to the thin‐walled diverticulum providing a weak point for rupture, further necessitating distinction between the two.In regard to management, there is growing evidence and support for early appendicectomy for appendiceal diverticulitis and also for diverticulosis.Imaging with modern thin slice CT scanners, awareness of the subtle findings of this entity and comparative studies, (as in our case), will help in detecting appendiceal diverticula and aid in differentiating diverticulitis from isolated or accompanying appendicitis.Lee et al. postulate that accompanying appendicitis is often secondary and that these two subtypes (i.e. diverticulitis with or without appendicitis) represent different stages in the progression of same disease process.In summary, significant complications and morbidity associated with appendicular diverticular disease could be potentially avoided by early diagnosis and management as suggested by our case.