Reports of enterosalpingeal fistulae complicating Crohn's disease are scarce. They involve the last ileal loop and lead to a progressive destruction of the salpinx. Usually, no genital symptoms are found. In all the cases reported in the literature, the fistula was diagnosed intra-operatively and resection of the right salpinx was performed without the patient's pre-operative consent.We describe 2 cases of women presenting with an Enterosalpingeal fistulae complicating Crohn's disease. Radiological findings allowed a pre-operative diagnosis. Thus, the patients were warned of the right salpinx resection and consent was obtained.Enterosalpingeal fistulae complicating Crohn's disease are exceptional. Indeed, to the best of our knowledge, only five cases have been reported till now. In all the reported cases, no genital signs were present. As for our patients who didn't experience such symptoms. Moreover, no radiological evidence of the enterosalpingeal fistula was found in the literature. Consequently, the fistula was always diagnosed intra-operatively. For our patients, radiological findings allowed a pre-operative diagnosis. This permitted to warn them of a possible resection of the fallopian tube. Intra-operative findings were unfortunately conflicting with its preservation.Enterosalpingeal fistula is an exceptional complication of the Crohn's disease. No clinical findings are present. The diagnosis should be evoked when the CT-scan or the MRI show an abnormal apposition between the fallopian tube, the last loop and the cecum. Surgical resection of the involved salpinx with the diseased intestinal segment is unfortunately usually needed in a young patient population. •Report of two cases of an unusual form of Crohn's disease.•Unique preoperative imaging.•Preoperative diagnosis of the fistulae. Thus, patients were warned of salpingectomy. The mosThis case report has been reported in line with the SCARE Criteria .2 showed a severe stenosis of the last ileal loop causing intestinal distension. Sclerolipomatosis and mesenteric adenopathies were present. CT also showed a markedly thickened right fallopian tube having an abnormal contact with the last loop. Conservative management was successfully conducted. Barium meal  revealed a stenosis of the last loot and presence of contrast fluid in the right fallopian tube. Hysterosalpingography  showed an opacification of the ascending colon. Thus, the diagnosis of last loop stenosis and enterosalpingeal fistula complicating Crohn's disease was retained. Surgery was then decided.She was a fifty-year-old woman, with a past medical history of ileocecal Crohn's disease evolving since three years. She was admitted in our department for bowel obstruction. On physical examination, the patient had a mass in the right fossa iliaca. Routine serological tests were normal. Abdominal CT-scan  showed aium meal  revealedgography  showed aA median laparotomy was performed. There was a tumor-like mass of the right fossa iliaca joining the last ileal loop, the cecum and the right fallopian tube with an intense sclerolipomatosis. As dissection was highly haemorrhagic, en-bloc resection of the mass and ileocolic anastomosis were executed. Outcomes were straightforward.33) and C Reactive Protein (142 mg/L). Abdominal CT-scan  showed a 7 cm abscess of the right fallopian tube that was adjoining the last loop. The latter was thickened and enhanced after intravenous contrast fluid injection. Multiple coeliomesenteric adenopathies were present. Abdominal MRI  found an abnormal connection between the fallopian abscess and the last loop that was highlighted in T2 sequences evoking an ileosalpingeal fistula. Colonoscopy with ileocecal biopsies confirmed the diagnosis of Crohn's disease. Therefore, the diagnosis of ileo-salpingeal fistula with right fallopian tube abscess complicating Crohn's disease was retained.She was a twenty-year-old patient, with no past medical history. She was admitted in our department for an appendicular syndrome. Interrogatory discovered a six-month-evolving diarrhea and a recent weight loss. On physical examination, the patient had fever, a sensitive mass and guarding of the right fossa iliaca. Serological tests found heightened white blood count (16,520/mm CT-scan  showed ainal MRI  found an. There also were an intense sclerolipomatosis of the right fossa iliaca and a thickened last ileal loop. After disconnection of the fistula, an ileocecal resection was performed. However, preserving the right salpinx wasn't possible, as it was too distended with a necrotic mucosae and no contractile activity. Outcomes were straightforward.The patient was put on antibiotics. Nevertheless, she developed a severe sepsis syndrome. Emergency laparotomy was then performed. We found an ileosalpingeal fistula powering a right fallopian abscess . There a4Fistula is a common complication of the Crohn's disease. Yet, enterogenital fistulae are rare and mainly rectovaginal . EnterosEnterosalpingeal apposition with progressive perforation of the gut in the fallopian tube leads to the fistula. Likewise, intra-abdominal abscess may drain into the fallopian tube and form the fistula.In all the reported cases, no genital signs - as leucorrhoea, dyspareunia, genital infections - were present. As for our patients who didn't experience such symptoms.Moreover, no radiological evidence of the enterosalpingeal fistula was found in the literature. Consequently, the fistula was always diagnosed intra-operatively. Nevertheless, radiological findings allowed a pre-operative diagnosis for our two patients.Actually, CT-scan revealed an abnormal contact between the right fallopian loop and the intestine in both cases. Besides, it diagnosed a salpingeal abscess for our second patient. Furthermore, barium meal and hysterosalpingography exposed the fistula as contrast fluid reached the right fallopian tube and the cecum in both examinations for our first patient. In addition, MRI discovered the fistula showing an abnormal connection between the right salpinx and the last loop with T2 hypersignal for our second patient.It is to know that enterosalpingeal fistula may lead to salpingeal abscess and progressive destruction of the fallopian tube, like for our second patient. Thus, surgical treatment of the Crohn's disease should be indicated. Resection of the diseased intestinal segment with en-bloc dissection of the involved salpinx was the performed procedure in all the reported cases.As dissection was hard and haemorrhagic due to intense sclerolipomatosis, we performed an en-bloc resection of the fallopian tube with the diseased intestine for our first patient, especially that she was fifty-year-old. But, salpingeal preservation was initially meant for our second patient, knowing that she was only twenty-year-old with no children. Pre-operative diagnosis permitted though to warn her of a possible resection of the fallopian tube. Intra-operative findings were unfortunately conflicting with its preservation.5Enterosalpingeal fistula is an exceptional complication of the Crohn's disease. No clinical findings are present. The diagnosis should be evoked when the CT-scan or the MRI show an abnormal apposition between the fallopian tube, the last loop and the cecum. Barium meal, hysterosalpingography and MRI may visualize the fistula. Surgical resection of the involved salpinx with the diseased intestinal segment is unfortunately usually needed in a young patient population.The authors declare that they have no conflict of interest.No source has funded this manuscript.All the authors have read and complied with the policy of the journal on ethical consent.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.Haddad Anis: Writing – original draft. Sebai Amine: Writing – original draft. Maghrebi Houcine: Study concept and design. Chaker Youssef: Methodology. Mohamed Jouini: Supervision. Montasser Kacem: Supervision.Sebai Amine.Not appliable


In forensic pathology, apparently straightforward cases can often hide rarities that, if not correctly interpreted, can alter the results of the entire investigation, leading to misinterpretations. This occurs when the investigation is conducted to assess medical malpractice. An unexpected death, with no known apparent cause, is often linked to an underlying disease process of unclear etiological origin whose nature can, unfortunately, be properly investigated only post-mortem. This presentation shows a case study, in which it was possible to reconduct the death of a patient to a natural pathology and not to medical treatment. Here, the authors illustrate a case with a hamartoma developed in chronic inflammatory conditions (bronchiectasis) that was difficult to differentiate from lung cancer due to the inability to perform specific instrumental examinations. The hamartoma, usually benign and identifiable by standard instrumental investigations, in this case, led to the patient’s death precisely during the execution of a bronchoscopy. However, in the absence of a certain cause of death, public opinion unanimously attributes a patient’s disease to medical error. Indeed, a routine practice such as bronchoscopy should not cause death and consequently, the doctor must have made a mistake. Fortunately, the autopsy not only demonstrated the origin of the bleeding but also unveiled the reason for this, as rare congenital lung disease. Fate, one might say. In forensic pathology, apparently straightforward cases can often hide rarities that, if not correctly interpreted, can alter the results of the entire investigation, leading to misinterpretations. This occurs, in particular, when the investigation is conducted to assess medical malpractice. In these circumstances, apart from the normal individual variables, the fatal outcome of a medical procedure  may be correlated, rather than with medical negligence, with rare diseases that are difficult to diagnose, little studied and hard to recognize, and whose evolution may be difficult to predict ,2. An unSuffice it to think, in the present, how the figure of the doctor in the era of COVID-19, from an initial exaltation to hero, has become the subject of complaints, following the occurrence of deaths. Deepening, therefore, through the autopsy of medical-legal investigation of “doubtful” cases. Moreover, in the context of a pandemic, it promotes the scientific understanding of the events, but can also help in restoring confidence to health care. Here, the authors illustrate a case with a hamartoma developed in chronic inflammatory conditions (bronchiectasis) that was difficult to differentiate from lung cancer due to the inability to perform specific instrumental examinations.The authors report the case of a 49-year-old man with anatomical-dysfunctional abnormalities in the lungs , who underwent fibrobronchoscopy at the Hospital in the South of Italy to define the nature of a mass. In this case, the mass was already identified by chest X-ray examination and confirmed by a thorough computerized tomography (CT) examination. During the examination, especially after introducing the tool and having aspirated a large amount of thick mucus from the tracheobronchial level in an attempt to make a second bronchial aspiration, massive bleeding occurred which dictated the infusion of cold distilled water to induce vessel constriction. The endoscopic maneuver was interrupted . ShortlyThe autopsy was required by the magistrate to evaluate whether the doctor who had performed the bronchoscopy was liable for malpractice. It was assumed that a wrong introduction of the endoscopic instrument in the airways might have damaged a part of a large vessel and that the copious hemorrhage was consequent to an inadvisable biopsy. The preliminary examination of the corpse excluded any sign of external traumatic injury. In addition, the examination showed that abundant blood contaminated the clothes worn by the victim. Moreover, when handling the corpse, the leakage of bright red blood from both nostrils was shown.A further examination of the cadaver revealed myocardial hypertrophy and a marked anatomical alteration of the right lung , that showed a much smaller size and volume than the left lung . The entire parenchyma had a greater consistency A,B. The An histological examination demonstrated that the mass consisted of a congenital vascular anomaly of hamartoma type . MoreoveDespite these macro and microscopic findings, doubts remained about the work of the health professionals who were responsible for the patient: Was the presence of a richly vascularized formation enough to contraindicate a fibrobronchoscopic examination with biopsy? Why was it performed? The answer to this question was provided by a careful examination of the health records. In reality, neither the chest X-ray examination nor the CT scan could distinguish the vascular nature of the lesion. A CT scan with a contrast medium would have been necessary, but although initially recommended, it could not be performed since the patient had severe chronic kidney disease (stage IV) and could not tolerate the administration of the appropriate contrast medium. At this point, the only possibility to define the nature of the lung mass was, according to the guidelines, to perform fibrobronchoscopy. Therefore, this was performed in this case. At the present time, although this procedure was conducted according to lege artis, the tangle of vessels broke and triggered the lethal hemorrhage. This prevented biopsy sampling.The histological examination showed the absence of iatrogenic injury to load both of the hamartoma of the surrounding tissues. Bronchoscopy had not affected the tumor exactly as described in the medical report. The rare findings, in this case, confirmed that the copious bleeding was a consequence of the laceration caused by the pressure induced by insertion of the bronchoscope in the bronchial tree, owing to its intrinsic morphologic-structural characteristics .In particular, the intrinsic morphologic-structural characteristics of the bronchial malformation solicited by a hypertensive state baseline , the vasal fragility resulting from kidney failure and hypertension caused by the agitation of the patient (described in documents), and the resulting pressure jump aspiration of the mucus, determined the lethal hemorrhage.Direct communications between the branches of the pulmonary artery and pulmonary veins, without an intervening pulmonary bed, are probably the most common anomalies of the pulmonary vascular tree. In addition, they have been variously called a pulmonary arteriovenous fistula, pulmonary arteriovenous malformation (PAVM), pulmonary arteriovenous aneurysm (PAVA), pulmonary angioma, arteriovenous angiomatosis, cavernous haemangiomas, and pulmonary hamartomas ,11,12,13In particular, hamartomas are tumor-like malformations due to the abnormal mixing or abnormal development of normal tissue components of the organ in which they occur. This abnormality is thought to be the result of variations in the quality, arrangement or degree of differentiation of the tissues. Hamartoma has a high frequency accounting for 77% of all the benign lung tumors. Often discovered incidentally, they are typically well-circumscribed nodules or masses  with smooth or lobulated margins. Approximately 60% have fat  and apprThe presence of mutations in the PTEN gene increases the risk of developing benign and malignant tumors in many other districts . It woulThere are several cases of hamartomas in the literature, not only in the lungs. A few of them were identified by CT or FDG-PET , while oIn the work of the doctors involved in the case, there was nothing wrong. Therefore, the decision to deepen the diagnostic investigation was correct, as it was not possible to determine in advance that it was a rare malformation.However, in the absence of a certain cause of death, public opinion unanimously attributes a patient’s decease to medical error ,26,27. IThere is an increasing tendency to blame the doctor for medical failures. If this is not restrained, it will lead to unwarranted “defensive” medicine, ultimately harming patients, above all ,29,30,31The improved living conditions and the models shared by media and advertising are the cause of an exasperated “witch hunt”, unfortunately, related only to economic causes based on the inauspicious principle that “someone must pay” and not on real health needs.


Omental infarction (OI) is a rare disease occurring in children. Important risk factors include overweight and obesity. The clinical presentation is often non-specific, and the main symptom is acute abdominal pain. In addition, infarcted omentum may present with fever, anorexia, nausea, vomiting, diarrhea and dysuria. Due to the localisation of the pain, OI should be differentiated from acute appendicitis. The diagnosis of OI is sometimes made intraoperatively, during appendectomy for suspected acute appendicitis. Hence, it is important to state a correct preoperative diagnosis, which is commonly based on abdominal ultrasound and computed tomography. The treatment of OI is still inconclusive. Both conservative and surgical treatments are used. Both methods have their advantages and disadvantages. The decision of which treatment to follow should be multifactorial and include the patient’s clinical condition at the time of admission, the progression or regression of symptoms during hospitalization and laboratory and imaging findings. We present a clinical case of a 9-year-old overweight girl with OI, whose diagnosis was based on imaging diagnostics and enabled conservative treatment with no complications. The case we have described confirms that the conservative treatment is an effective and safe therapy. Omental infarction (OI) is a rare cause of acute abdominal pain in children, occurring in approximately 0.1% to 0.5% of children undergoing surgery for suspected appendicitis ,3. It re2, which indicated that she was overweight.A 9-year-old, previously healthy Caucasian girl was referred to the Department of Pediatric and Oncological Surgery, Urology and Hand Surgery of the Pomeranian Medical University in Szczecin from a district hospital. The patient’s body weight was 41 kg, her height 140 cm and BMI (body mass index) 20.92 kg/mOn admission, the patient presented with severe epigastric pain lasting for 3 days. In addition, the parents reported one episode of vomiting and stool retention lasting 2 days. The girl had no fever. On physical examination, the abdomen was distended. On palpation, significant pain, with increased tension of abdominal integuments in the right and central epigastrium, was observed. The lower quadrants of the abdomen were soft with mild tenderness, with palpable stool masses in the lower left iliac fossa. The percussion examination was impaired by the excess amount of subcutaneous tissue with tympanitic sounds in the epigastrium and slightly dull sounds over the left iliac region. Peristalsis was heard in the umbilical region. The pain intensified with changes in position. Diagnostics for acute abdominal diseases were implemented. The differential diagnosis included acute pancreatitis, gastrointestinal obstruction, gastrointestinal perforation  and acute appendicitis. Laboratory tests showed elevated inflammatory parameters: CRP (C-reactive protein) at 69.42 mg/L, insignificant leukocytosis with a WBC (white blood cell count) of 10.65 thousand/μL, with a neutrophil predominance of 71%, and fibrinogen at 524.7 mg/dL; the serum amylase and lipase levels were within normal limits. As for imaging examinations, a plain abdominal X-ray was initially performed, which showed no signs of obstruction or perforation of the gastrointestinal tract but did show fecal masses in the ascending colon and rectum .An abdominal ultrasound was then performed  and showAs the ultrasound examination was inconclusive, a team of doctors consisting of a pediatric surgeon, a pediatric gastrologist and a radiologist decided to expand the diagnostics with a non-contrast CT of the abdomen and pelvis. The examination showed a focal area of fat stranding, measuring 58 × 20 mm (transverse dimension), in the right epigastrium, anterior to the site of the gastric connection to the duodenum, along with small-banded thickenings along both flanks and in the lower abdomen. In addition, there was no free gas in the abdominal or pelvic cavities and the appendix presented with no signs of inflammation .The clinical picture, along with laboratory and imaging investigations, led to the diagnosis of omental infarction. Conservative treatment was introduced. Enema was performed with a good effect. Antibiotic therapy (cefuroxime), analgesic treatment and fluid therapy were implemented. During hospitalization, the subsidence of clinical symptoms and a decline of the inflammatory parameters were observed. In the control abdominal ultrasound, performed on the seventh day of hospitalization, a partial regression of lesions was noted .The patient was discharged after 7 days of hospitalization. Oral antibiotic therapy was continued for the next 5 days. Two weeks after the hospitalization, a follow-up visit to the pediatric surgery outpatient clinic took place. The patient did not report any complaints. A follow-up ultrasound examination two weeks after hospitalization showed regression of the abdominal lesions—adipose tissue in the middle-right epigastrium had still partially increased echogenicity, but with neither increased vascularity on Doppler nor fluid collections .The long-term follow-up of the patient showed no complications.Omental infarction (OI) occurs in approximately 0.1% to 0.5% of children undergoing surgery for suspected appendicitis ,9, whichThe symptoms of OI are often non-specific. A commonly reported symptom is acute abdominal pain in the right iliac fossa ,12. The In order to implement OI treatment, it is essential to make a correct diagnosis. Today, OI is more frequently diagnosed, thanks to the development of imaging techniques, including ultrasound and computed tomography . CT is cThe initial diagnosis should be confirmed by imaging studies and be made preoperatively. There are clinical situations where the diagnosis of IO is made intraoperatively. Patients are initially diagnosed with acute appendicitis, but despite a lack of clear evidence of appendicitis on imaging, surgical treatment is implemented . SurgeonOmental infarction, as a rare cause of acute abdominal diseases in children, may be clinically challenging and requires an extension of diagnostic imaging to abdominal computed tomography. The diagnosis of OI based on symptoms, examination and imaging findings may allow for effective conservative treatment without burdening the patient with surgery. Due to the increasing prevalence of overweight and obesity in children, OI should be particularly considered in the differential diagnosis of acute abdominal diseases in patients from this risk group.


Multiple meningiomas or meningiomatosis are defined by the presence of at least 2 lesions that appear simultaneously or not, at different intracranial locations. Early classification of Cushing and Eisenhardt claimed that a diagnosis of multiple meningioma can only be made if the patient does not suffer neurofibromatosis type I . Majority of multiple meningiomas associate with neurofibromatosis type-II, multiple meningiomatosis refers to the association of at least 2 tumors in two different sites, in a patient who has no evidence of neurofibromatosis. The incidence of this condition varies between series between 1 and 3%, reaching a frequency of 8% with the onset of magnetic resonance imaging (MRI). This entity combines benign tumors of a different histological nature in 30% of cases. A 39-year-old woman patient, with no significant pathological history, who consulted for heaviness in both lower limbs having progressed for 6 months with paresthesias without genitosphincteric disorders, the examination found a spastic paraparesis rated at 3/5. The patient underwent a medullary MRI which revealed 4 lesions, the radiological semiology of which was suggestive of spinal meningiomas, the most compressive projecting in relation to T2. A cerebral computed tomography (CT) was systematically performed and objectified two intra cranial meningiomas, which until then were asymptomatic. Although its incidence is only 1 to 3%, the discovery of multiple meningiomatosis justifies morphological exploration (MRI) of the entire neurax, in which case any symptomatic location should suggest surgical management.


Colonic leiomyomas are rare. Their clinical presentation ranges from asymptomatic polyps detected on endoscopy to large symptomatic abdominopelvic masses. Imaging findings are usually non-specific, and percutaneous biopsy might help with differential diagnosis. However, radical surgery with negative margins is ultimately needed to rule out malignancy. We describe an uncommon presentation of a colonic leiomyoma mimicking a right hepatic lobe tumor on preoperative imaging. The robotic approach allowed a precise abdominal exploration with confirmation of colonic and hepatic infiltration and subsequent oncological en-block resection. Surgeons operating on hepatic tumors close to the right colic flexure should be aware of this diagnosis. Leiomyomas of the alimentary tract are usually located in the esophagus, stomach, or the small intestine and more rarely in the colon . Some pa2 and no previous abdominal surgeries. CT scan  scan ordered for non-specific abdominal pain. He had a past medical history of type 2 diabetes mellitus, glaucoma and deafness due to mumps. The physical examination was normal, with a body mass index of 22 kg/m CT scan  and magn CT scan  revealedAn ultrasound-guided liver biopsy indicated a spindle cell neoplasm with smooth muscle differentiation. Liver function tests were within normal limits. The case was extensively discussed by a multidisciplinary team, and there was consensus to proceed to surgery with the diagnosis of resectable peripheral liver tumor.The operation was performed using the Da Vinci Xi system. Initial exploration showed a large and bulky lesion in the liver’s segments V–VI completely fused to the right colonic flexure .The ascending colon and hepatic flexure were detached. The mesocolon was divided, and the ascending and proximal transverse colon were transected with the stapler leaving the right hepatic flexure attached to the tumor. Before the hepatic transection, intraoperative ultrasound was performed to confirm the tumor position, plan the resection line and rule out other nodules. Intestinal continuity restoration was achieved with a side-to-side anastomosis. Finally, the specimen was retrieved through a Pfannenstiel incision.The postoperative course was uneventful, and the patient was discharged on postoperative Day 5. At 2 weeks postoperatively, the patient presented with fever, and a CT scan demonstrated an infected subhepatic collection. The patient was readmitted, and the collection was successfully treated with antibiotics and percutaneous drainage. The surgical specimen showed a hard, round 10.8 × 8.9 × 7.2-cm mass bulging out from the liver’s surface and adhered to the right colic flexure . HistoloSmooth muscle tumors are the second most common mural mesenchymal neoplasia of the gastrointestinal tract after gastrointestinal stromal tumors . ColoniImaging findings are usually non-specific, and consist of tumors with lobulated margins, areas of calcification, focal necrosis, cystic changes and heterogeneous enhancement with contrast. These findings can be seen both in benign and malignant conditions . DespiteHistologically, the most important differential diagnosis of leiomyoma is GIST and malignant leiomyosarcoma. The distinction between leiomyoma and leiomyosarcoma is challenging and is based on the presence of necrosis, tumor size, mitotic count, atypia and nuclear pleomorphism . MoreoveDifferent techniques have been employed to treat these tumors. Small endoluminal leiomyomas could be endoscopically resected with excellent outcomes , 15. HowTo the best of our knowledge, this is the first reported case of a colonic leiomyoma presenting as a hepatic mass. Neoplasms involving the right colic flexure should be considered in the differential diagnosis of segments V–VI hepatic masses.This study was performed in accordance with the Declaration of Helsinki, the institutional review board (IRB) approval of our institution, and informed patient consent was obtained for case publication.NHD, SK, CB and PCG: Conception and design. NHD, AM, GA and VM: Data acquisition, analysis and interpretation of data. NHD, AM, GA, VM, SK, CB and PCG: Drafting of manuscript, and critical revision of manuscript. All authors read and approved the final manuscript.


An 18-year-old male patient previously healthy and immunocompetent presented with a 6-months history of cough, sputum production, malaise, and weakness. The patient had fever, diffuse lymph node enlargement, multiple cutaneous lesions, bone pain, and a purulent collection of on the thoracic wall adjacent to the left clavicle. He reported a weight loss of about 10 kg in just one month.Physical examination revealed the presence of umbilicated skin-colored papules on the face A, neck, Contrast-enhanced computed tomography (CT) of the chest revealed lymph node enlargement and the presence of multiple osteolytic lesions on the sternum and both clavicles B. The puP. brasiliensis in the sample obtained from the chest aspirate , as observed in our case. Bone lesions in PCM are usually osteolytic, with no sclerotic rim or periosteal reaction


Staphylococcus aureus meningitis.Tumefactive demyelinating lesions belong to the rare variants of multiple sclerosis, posing a diagnostic challenge since it is difficult to distinguish them from a neoplasm or other brain lesions and they require a careful differential diagnosis. This contribution presents the case report of a young female with progressive tumefactive demyelinating brain and spinal cord lesions. An extensive diagnostic process including two brain biopsies and an autopsy did not reveal any explanatory diagnosis other than multiple sclerosis. The patient was treated by various disease-modifying treatments without significant effect and died from ascendent infection via ventriculoperitoneal shunt resulting in  Tumefactive demyelination belongs to the rare variants of multiple sclerosis (MS), posing a diagnostic challenge and therapeutic enigma as they can mimic other pathologies such as brain neoplasm, abscess, vasculitis, or granulomatous disease.Atypical features of MS plaques on MRI include size >2 cm, mass effect, edema, and/or the presence of ringlike or open-ring enhancement. Lesions with these characteristics are often described as tumefactive demyelinating lesions (TDLs) .The prevalence of TDLs is estimated to be one to three per 1,000 cases of MS , althougA 23-year-old female was admitted to the neurological department of a major university hospital presenting with a mild central paraparesis of the lower extremities; an MRI indicated T2-hyperintense lesions in a periventricular, infratentorial, and intramedullary localization; both contrast-enhancing and non-enhancing lesions were found . Five olThe MS diagnosis was determined according to the McDonald 2010 criteria . The patAt the age of 27, the patient exhibited a mild central paraparesis of the lower extremities (treated with high-dose steroids), and the chronic treatment began with dimethyl-fumarate. An MRI of the brain and spinal cord showed multiple TDLs . An exteA broad range of tests was undertaken during follow-up , all witAt the age of 28, the patient exhibited left-sided negative sensitive symptoms and was treated with high-dose steroids. An MRI of the brain and cervical spinal cord showed significant progression of TDLs, and the treatment with natalizumab commenced.At the age of 29, cognitive and gait problems together with a headache and papilledema occurred, and a diagnosis of obstructive hydrocephalus  was estaStaphylococcus aureus meningitis. The autopsy confirmed the central nervous system demyelination without any evidence of neoplasm or other chronic disease.At the age of 30, left-sided hemiparesis together with further progression of TDLs appeared. The patient was treated with high-dose steroids and an immunomodulatory dose of intravenous immunoglobulins resulting in a slight reduction of complaints to mild central paraparesis of the lower extremities and mild left-sided hemiparesis. From a chronic treatment point of view, hematopoietic stem-cell transplantation was considered. However, the patient died from circumscribed peritonitis complicated by ascendent infection via ventriculoperitoneal shunt resulting in This contribution presents the case report of a young female with TDLs. An extensive diagnostic work-up, including two biopsies and an autopsy, did not reveal any other explanatory diagnosis other than tumefactive MS. The report is unique due to subacute progressive TDLs of the brain and spinal cord, non-responsiveness to the high-efficacy drugs, and high diagnostic certainty due to multiple brain biopsies and an autopsy. Such a case report of a patient exhibiting similar TDLs has not been reported before.In distinguishing between TDLs and other pathologies on MRI, the features listed above can be helpful along with follow-up imaging since TDLs tend to resolve in response to steroid therapy . HoweverThe value of advanced MRI techniques such as diffusion, perfusion imaging, or magnetic resonance spectroscopy has been studied in distinguishing between TDLs and brain tumors; however, it appears that those techniques still cannot provide definite diagnosis, and further studies are required to determine their additional value .PET may also play some role in distinguishing TDLs from brain tumors . FLT traIn the presented case, we observed several imaging features considerably atypical for TDLs like mostly homogenous enhancement, spectroscopy, and FLT-PET findings. Also, considering the constant progression of the mass lesions during the therapy leading finally to the development of obstructive hydrocephalus, the coincidence of tumor infiltration was suspected, and the brain biopsy was required to provide a definite diagnosis.There is little comment on the effect of disease-modifying therapy on the evolution of TDLs. Some evidence supports that fingolimod should be avoided in MS patients with TDLs due to possible exacerbation . PatientThe original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Ethical approval was not provided for this study on human participants because The manuscript presents a case report study. Written informed consent was not provided because According to the local ethics committees, the written informed consent is not necessary for a post-mortem case report study. Written informed consent was not obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.All authors listed have made a substantial, direct and intellectual contribution to the work, and approved it for publication.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.


Synchronous multifocal osteosarcoma is a rare condition in which the osteosarcoma presents with multiple bone lesions at the time of diagnosis, usually without any visceral metastases. The first case was described in early 1930s by Silverman. To report a case of synchronous multifocal osteosarcoma in adolescent with pathologic fracture.An 18-year-old girl presented with a painful mass in the right thigh of 4 months’ duration and a history of thigh bone fracture a month ago. Patient’s medical records and family history was unremarkable. Physical examination showed angulation and shortening at right femoral region with tenderness and swelling. Initial radiograph and magnetic resonance (MR) images showed multiple lesions in right femoral shaft and pelvic bone with primary tumor in right distal femur with pathologic fracture and multiple bone marrow lesions found in the contralateral bones. Imaging and histopathological results supported the diagnosis of synchronous multifocal osteosarcoma. After following the chemotherapy as the treatment of choice, the radiograph and MRI evaluation were done and showed reduction of the mass size with union of the destructed part with the formation of callus. The advance MRI revealed reduction of the overall mass and the composition of the viable area compared to previous study. The patient had satisfying response to chemotherapy series and a better functional outcome on subsequent visits.Diagnosis of synchronous multifocal osteosarcoma was based on patient and family history and finding of multiple lesions in the MR images, meanwhile the plain radiograph only revealed the primary tumor. Amstutz described multifocal osteosarcoma as presence of one primary tumor and several smaller lesions. Most recent reviews concluded that multifocal osteosarcoma is bone-to-bone metastatic process rather than multicentric origin. The limitation in this case was absence of thoracic CT which is suggested to rule out any pulmonary metastases instead of routine chest radiograph.Although satisfying improvement was clinically achieved, further advanced MRI would be indicated to evaluate the progression of tumor and its respond to therapy. It occurs most commonly in patients between 5 years of age and early adulthood. The peak of incidence in the elderly has been associated with pre-existing Paget’s disease and prior radiation therapy. It is an osteoid-producing malignancy arising from mesenchymal origins.2Osteosarcoma of the adolescent most often develop at the metaphysis of the lower extremity long bones (~75% of cases), and the relationship between hormonal changes of puberty and/or physiologic bone growth and the pathogenesis of osteosarcoma was suggested.3 It may be synchronous, presenting with two or more bone lesions at time of diagnosis, or metachronous, which the new tumors develop after the initial treatment, involving more than one bone.4 The first case of multifocal osteosarcoma was described in 1930s by Silverman.5Multifocal osteosarcoma is defined as the presence of tumor at two or more skeletal sites without any evidence of pulmonary metastases (incidence around 1.5–5.4%).6 Many literatures stated the prognosis of synchronous multifocal osteosarcoma being poorer compared to the conventional osteosarcoma. Here, we present a case of synchronous multifocal osteosarcoma with complication of pathologic fracture at the main site of the tumor.Multifocal osteosarcoma typically presents with one primary large tumor and several smaller bone lesions.An 18-year-old girl presented with a painful mass in her right thigh for the past 4 months before admitted to the hospital. The patient also reported a history of thigh bone fracture due to accidentally dropped while being carried by his uncle a month ago. Her medical and family histories were unremarkable.Physical examination showed angulation and shortening at the femoral region with, tenderness and soft tissue swelling at the anteromedial aspect of the thigh above the right kneeInitial radiograph was presented, showed osteolytic lesion in one-third distal portion at the anteromedial aspect of right femur diaphysis with wide transition zone and periosteal elevation (Codman triangle) . Plain r–3 mm2/s in the solid area, which suggested a malignancy, and 2.05–2.54 × 10–3 mm2/s in the necrotic area  images; coronal T1 fast spin echo (FSE) and axial T2 fast relaxation fast spin echo (FRFSE) images; axial diffusion-weighted imaging (DWI) with apparent diffusion coefficient (ADC) followed by MR perfusion and MR spectroscopy; as well as post-Gadolinium contrast-enhanced 3D T1FS images. Bone destruction was found in one-third distal to the mid-femoral diaphysis with bulky soft tissue mass, about 6.044 × 3.662 × 13.254 cm, and pathologic fracture in distal femoral shaft. Intramedullary involvement was found to the mid-femoral shaft. Multiple skip lesions along fifth lumbar vertebra, right femoral shaft, left ischium bone, and left proximal femur were also detected. In the post-contrast images showed heterogeneous enhancement of the lesion with central necrotic area. The mass is shown to infiltrate the anterior compartment of the vastus muscles and displace the femoral and popliteal vessels and nerves to the posterior . DWI shotic area . MR perftic area . There wtic area . From MRFigure 7). Microscopic examination showed anaplastic cells proliferation with round oval to pleomorphic spindle, hyperchromatic nucleus. The cells were arranged diffusely in the osteoid matrix, forming lace-like appearance . This feature supported the diagnosis of synchronous multifocal osteosarcoma.After having several imaging procedures, then the open biopsy had been done and the specimen obtained during biopsy was several irregular grayish tissues sized 0.5–1.5 × 1.5×1 cm  and metachronous .8 In our case, unremarkable history of the patient and family supported one of multifocal osteosarcoma criteria.Several conditions are required to establish the origin of multifocal osteosarcoma: absence of previous systemic bone pathology, negative history of radiation exposure, simultaneous appearance of the lesions in the affected bones, and absence of pulmonary metastasis.6 described this multifocal osteosarcoma as the presence of one primary tumor and several skeletal lesions. He describd a synchronous form (Type I child/adolescent high grade tumor and Type II adult low grade tumor) and a metachronous form .6In 1969, Amstutz HC9–11In patients with synchronous multifocal osteosarcoma, the primary tumor has features suggestive of a primary osteosarcoma, usually presented as being more extensive than the other lesions. The smaller lesions, instead, mimic skeletal metastases on X-ray and MRI, presented as purely sclerotic or heavily mineralized metaphyseal lesion, with narrow transition zone, no cortical destruction or soft tissue mass, nor malignant periosteal reaction, in contrast to the metachronous group, which most of the lesions appeared similar to the presentation of primary osteosarcoma.1210 years later, Mahoney proposed an other classification of mutilple osteosarcoma in four groups (A–D) based on age, histology, and time of presentation of lesions. Group A included childhood and adolescent patients with multiple synchronous tumors; Group B comprised adults with low grade multiple simultaneous lesions. Groups C and D consisted of metachronous lesions with a time interval between the first and the second lesion of less than 24 months (Group C) and greater than 24 months (Group D).13 However, the synchronous lesions were detected after the first MR imaging was performed, instead of the plain radiograph, as mentioned by Yang,14 that skip metastases in many cases were possibly missed due to lacking of imaging modalities. Compared to the first MR images, in which the multiple lesions were already seen, the later MR images revealed the primary tumor being more extensive with pathological fracture and additional skip lesions in the pelvic bone.In the presented case, multiple lesions appeared at the time of diagnosis, with one primary tumor  which led to the presentation, and synchronous lesions in the several points surrounding the primary tumor with well-defined border and without tumor extension. The primary tumor was first shown in the plain radiograph in osteolytic-dominant pattern rather than the usual sunburst appearance, although the Codman triangle could be found.15 while the metachronous group prefers the axial skeleton, such as pelvis, spine, shoulder girdle, skull, synchronous type usually occurs in typical osteosarcoma location, such as distal femur, proximal tibia, or humerus.16 In the presented case, the main tumor was precisely located in the usual synchronous type location, however, multiple skip lesions could also be seen in the pelvic bone.Synchronous type principally involves long bones in symmetric distribution,16 Furthermore, there were three similar cases involving axial bones in 15, 16, and 17-year-old girls, respectively. The first case was a synchronous multifocal osteosarcoma occurred in the right ilium and left ramus of mandible.17 The second case were involving right ramus of mandible and left mid-shaft of the femur.18 The last case was a multiple metastatic osteosarcomas involving vertebrae, right zygomatic bone, right clavicle, sternum, right and left humerus, right tibia, right and left lungs, meninges, and mandible, with primary tumor in the left fibula.19Zhang reported a rare case of synchronous multifocal osteosarcoma which occurred in the maxilla and the mandible.20–22 One of the reason was the presence of one dominant tumor that led to presentation, which could be considered as a primary tumor.4 Enneking and Kagan23 described that the tumor cells may invade the marrow sinusoids and embolize both proximal and distal intraosseously, whereas the route for transarticular skips was less defined. They suggested the mechanism might be mimicking the vertebral spread of the prostatic tumor via Batson venous plexus. Alternatively, Hatori et al have demonstrated lymphatic spread to the lungs, giving another possible route.24Most recent reviews concluded the multifocal osteosarcoma being a metastatic process rather than multicentric origin.24–31Furthermore, the response of both primary and synchronous/metachronous lesions to chemotherapy was similar, and this evidence supports the bone-to-bone metastases theory.23The main limitation in the presented case was the absence of thoracic CT, which is necessary in every osteosarcoma case. Although the routine chest radiograph was appeared to be normal, it was not as sensitive as CT scan in demonstrating small pulmonary lesion.9,10 The presence of multiple bone lesions without any lesion in lung fields confirms the diagnosis of multifocal osteosarcoma differentiating it from primary osteosarcoma with multiple metastases. Furthermore, pre-treatment MDP scan can help assess the effectiveness of the therapy when compared to the post-treatment scan.9,10 This modality can be used for this purpose for further similar cases.In most settings, the imaging study for diagnosis and staging of osteosarcoma was mainly done using CT scan and MRI. These two modalities were superior for assessing the anatomic extent of the primary lesion. However, a radionuclide study is required for accurately assessing the metastatic disease and metabolic activity of the primary lesion. Whole body bone scintigraphy using 99m technetium-MDP has a high sensitivity in staging metastatic disease.24–31 However, in our case, good response had been established following chemotherapy. This might be proven in the second MR image which showed the large necrotic area in the center of the mass, furthermore, MR spectroscopy showed remarkable elevation of lipid lactate composition which indicated the necrotic area being extensive, without significant elevation of choline metabolite. Furthermore, after series of chemotherapy, latest follow-up plain radiograph showed union of the destructed bone with formation of callus and reduction of the mass. Advanced MRI obtained afterward demonstrated the reduction of viable tissue composition.Single lesion osteosarcoma and multifocal osteosarcoma have different disease courses and survival times, so it is important to make a differential diagnosis. Prior chemotherapy era, synchronous multifocal osteosarcoma was considered fatal within few months. Although in ordinary osteosarcoma without detectable metastases, surgery combined with chemotherapy significantly improves the success of treatment to 60–70%, the prognosis for synchronous multifocal osteosarcoma remains extremely poor in spite of the combined surgery and chemotherapy.The case of synchronous multifocal osteosarcoma in an Indonesian girl has been reported. This diagnosis was based on MRI finding, which showed multiple lesions and the primary tumor, and proven by the biopsy which supported osteosarcoma. Although many literatures stated the prognosis of synchronous multifocal osteosarcoma being poor, favorable response has been achieved following chemotherapy. The latest follow-up imaging showed reduction of the overall mass size and the cell viable composition.Synchronous multifocal osteosarcoma is a rare condition, with a reported incidence of 1–3%.Imaging evaluation using MRI is essential to make diagnosis and to evaluate the efficacy of chemotherapy.Advance MRI using DWI, ADC, MR perfusion and MR spectroscopy can give additional diagnostic information, providing a non-invasive assessment of tumor viability. Therefore, it may help monitor treatment response.Post-treatment changes cause decreased vascular permeability of the tumor vessels as well as viable tumor cell presentation.Synchronous multifocal osteosarcoma prognosis remains extremely poor in spite of the combined surgery and chemotherapy. In this case, there was a good response.


Retroperitoneal liposarcoma frequently recurs within 2 years of the initial surgical resection. For the early detection of recurrent retroperitoneal liposarcomas, a shorter follow‐up interval with CT or MRI would be helpful. Retroperitoneal liposarcoma frequently recurs within 2 years of the initial surgical resection. For the early detection of recurrent retroperitoneal liposarcomas, a shorter follow‐up interval with CT or MRI would be helpful. Magnetic resonance imaging of the abdomen was used to evaluate the tumor. The recurrent mass was about 19 cm and the patient underwent reoperation.Soft‐tissue sarcomas (STS) are less than 1% of all malignant tumors in adults. Liposarcoma is the most common variant and accounts for approximately 15% of adult soft‐tissue tumors.2A 62‐year‐old man was admitted to our department in June 2017 with abdominal pain and urinary difficulties. A retroperitoneal mass measuring 22 × 15 × 12 cm was observed in an abdominal computed tomography (CT). However, the tumor has not spread to the lymph nodes or other parts of the body. The patient underwent an excision of the tumor. The histopathological examination presented a well‐differentiated (grade I) liposarcoma that weighed 1172 g and measured 25 × 18 × 13 cm (stage IIIB according to American Joint Committee on Cancer TNM system). Because of microscopic positive margins, the patient received adjuvant chemotherapy  for eight cycles and complete remission was demonstrated by an abdominal CT follow‐up after the 8th cycle. The patient's clinical follow‐up by oncology group and imaging scans showed no evidence of recurrence 12 months after the 8th cycle of chemotherapy. After treatment, routine follow‐up continued on regular basis with physical examination, abdominal ultrasound (US) every 3 months, and CT every 12 months. In October 2019, the patient had the abdominal US, but no mass was detected. In December 2019, the 62‐year‐old man got readmitted because of a complaint of pain in the back of the left hip. The pain was moderate in severity. On examination, it was also revealed mild back pain as well as a palpable abdominal mass. Bowel sounds were audible, and rectal examination was normal. His vital signs were unremarkable. His routine blood tests including hemogram, C‐reactive protein level, liver, and renal function test, serum amylase, and lipase were normal. Chest and abdominal radiography showed no abnormalities. He was submitted to a CT  is the most common histological subtype of soft‐tissue sarcomas (STS), a family of mesenchymal‐derived malignancies that measures over 50 different histological subtypes.Treatment of primary LPS, according to the latest guidelines, must always involve a multidisciplinary team of practitioners, and all the patients should be referred to physicians with experience in STS management, if possible. According to the NICE recommendations for the treatment of sarcoma patients, centralizing the treatment of such patients was deemed so necessary that led to the formation of 15 specialized centers for the management of sarcoma.Recurrence in liposarcoma patients is frequent. Approximately 50% of patients with grade II/III liposarcomas will develop either local disease recurrence or distant metastatic disease, with a median survival of 12 months.For the treatment of liposarcoma recurrence, the primary contributing factor is the type of recurrency: local relapse of the disease, or distant metastatic lesions. Whenever a patient presents with local recurrent liposarcoma, the attending team of physicians must first thoroughly evaluate for the presence of concurrent distant metastasis, usually utilizing PET‐CT scans, or MRI, before further deciding upon treatment.Histological subtypes must once again be incorporated in the decision‐making, about patients with recurrent liposarcoma. Myxoid and clear cell histology have greater metastatic potential than that of the WD or DD and therefore should be monitored in tissues other than the chest region alone, for example, spine, pelvis, and fat pads.4The retroperitoneal liposarcoma is a rare type of malignancy, and surgery is considered the first‐line treatment. The diagnosis of well‐differentiated retroperitoneal liposarcoma and postoperative follow‐up of patients is very difficult. Relapse after surgery is very frequent and shorter follow‐up interval with CT or MRI would be helpful, in order to detect the tumor earlier.None declared.FM, G‐IV, EL, LT, DK, IP, and CK contributed to the clinical data collection and prepared the case report. FM and IK contributed to the design of the case report presentation and performed the final revision of the manuscript.


Bezoars have different compositions and can be subdivided into trichobezoar, phytobezoar, pharmacobezoar, lactobezoar and food bolus. The reported incidence of bezoar is 0.4% with phytobezoar being the commonest. Rapunzel syndrome is an extremely rare complication when trichobezoar crosses the pylorus to enter the duodenum, ileum and colon. We present the case of a 29-year-old female with a one-week history of abdominal pain, anorexia, nausea, vomiting, constipation, lethargy and a one-year history of increasing abdominal mass. Physical examination revealed a 20 cm palpable mass extending from the left upper quadrant to the umbilicus. Laboratory investigations demonstrated iron deficiency anemia and CT showed two well-defined foci within the gastric lumen consistent with trichobezoars. She was managed conservatively during her hospital stay and discharged home with a plan for elective laparotomy. We present this case to discuss the management of trichobezoars and to highlight the importance of early recognition of recurrence to avoid severe complications. Bezoar, derived from the Arabic word ‘Bazahr’, refers to an agglutination of indigestible content in the gastrointestinal tract -3. BezoaA 29-year-old female presented to the emergency department with a one-week history of worsening left upper quadrant (LUQ) pain, anorexia, nausea, vomiting, constipation and lethargy. She had a four-year history of ongoing abdominal pain and a one-year history of an increasing abdominal mass. Her past medical history was significant for trichotillomania, trichophagia and anxiety which started four years prior in the context of relationship stressors. She reported no surgical history. Vital signs were stable. No obvious alopecia or halitosis were noted. Physical examination revealed a soft abdomen with LUQ tenderness and a firm palpable mass (approximately 20 cm) extending from the LUQ to the umbilicus.Laboratory investigations showed a reduced haemoglobin level of 63 g/L, mean corpuscular volume of 78 fL and an elevated white cell count of 17.7 × 109/L. Urea, electrolytes and liver function tests were normal. C-reactive protein was elevated at 104 mg/L. Iron studies demonstrated marked iron deficiency with an iron level of less than 2 umol/L, ferritin 11 ug/L and transferrin saturation of <4%. Vitamin B12 and folate levels were within the normal range but a low albumin level of 26 g/L was noted.Computed tomography imaging of the abdomen and pelvis (CTAP) showed two well-defined foci of heterogeneous density but predominantly hyperdense foreign material within the gastric lumen consistent with trichobezoars. The larger focus (18 x 6 x 5.6 cm) is seen in the distended gastric body while the second focus (12 x 4.7 x 4.5 cm) extends from the distal gastric body into the pyloric antrum. There were no signs of gastric outflow obstruction and perforation  and oral thiamine (100 mg daily) were prescribed for malnutrition. She was managed conservatively with Coca-Cola consumption twice a day for at least four days  and was commenced on a free fluid diet. Regular Macrogol-3350 and Coloxyl-Senna were used to aid with bowel motions while her abdominal pain was well managed with intravenous hyoscine butylbromide. Abdominal X-ray taken four days later demonstrated progression of the trichobezoars into the caecum and ascending colon (Figure She was discharged home with oral ferrous fumarate tablets and a plan for elective laparotomy when she is medically and mentally optimized. She was scheduled for outpatient follow-up with the general surgeons, dietician and psychiatrist for ongoing review.Trichobezoars resist peristalsis and retain in the gastric rugae due to their smooth surface and poorly digested keratinaceous substance . GastricThe clinical presentation of trichobezoars is associated with its size and presence of complications . 33 to 3Rapunzel Syndrome is an extremely rare complication where trichobezoars cross the pylorus to enter the duodenum, ileum and colon , 5. FeweConservative management of trichobezoars includes hydration, Coca-Cola dissolution, meat tenderizers and prokinetic agents , 10. CocExploratory laparotomy is the standard treatment for trichobezoar and remains the only valid treatment for Rapunzel Syndrome as it has a shorter operative duration, allows feasible examination of the gastrointestinal tract and enables definitive management when complications arise , 7. The As patients with trichobezoars may have vague presentations, recurrence features such as anemia, weight loss, abdominal mass, malnutrition and parental concern should prompt early consideration of endoscopic evaluation , 4. TherTrichobezoars and Rapunzel Syndrome are rare in clinical practice and require urgent intervention to avoid severe complications. Coca-Cola or meat tenderizers may be considered as an option for conservative management of trichobezoars, however, exploratory laparotomy is the standard management and remains the only valid treatment for Rapunzel Syndrome. As patients with trichobezoar may have a vague presentation and are prone to recurrence, a multidisciplinary approach with dietetics, long-term psychiatric follow-up for trichotillomania management, a good support network and close monitoring are important for early detection and recurrence prevention. Further research on the follow-up appointments required for post-operative patients may be considered to reduce recurrence and facilitate early detection.


Myxopapillary ependymomas rarely present as a primary intralumbosacral lesion, and extensive sacral osteolysis is unusual. We report a case of a 57-year-old man presented with a 10-year history of low back pain. Two months before presentation, he had bladder dysfunction. On examination, there was a reduced range of movement of the lumbar spine without other neurologic deficits. Computed tomographic images of the lumbosacral spine showed lysis of whole sacrum. Magnetic resonance imaging demonstrated a giant lumbosacral mass. Because of the extent of bone destruction, only decompression and subtotal removal of the tumor could be performed, and the patient was referred for local radiotherapy. Histological findings from the tissue removed during surgery showed myxopapillary ependymoma with a positive margin. He had no low back pain at the last follow-up examination and has resumed full activities with normal bowel, bladder, and sexual function.


Intracranial meningeal convexity chondroma is a rare benign lesion hypothesized to stem from remnant chondrocyte precursors of embryonic origin. This lesion often masquerades as meningioma given the similar dural-based attachment and pattern of calcification. We describe the case of a 26-year-old female with incidentally discovered convexity meningeal chondroma, originally presumed to be a meningioma. In this case, we share our diagnostic and operative intervention and outcome and discuss the unique pathologic findings in this lesion that differentiate it from similar appearing lesions. To the authors’ knowledge, there are fewer than 20 cases of convexity meningeal chondroma in the literature; thus, we also provide a brief review of the literature regarding this rare pathology. Chondromas are rare and indolent tumors originating from mature chondrocytes producing cartilaginous matrix . They reIntracranial chondromas most often present extradural to the skull base, in either a sellar or parasellar location . ChondroWe present the case of a 26-year-old with an incidentally discovered left frontal convexity lesion initially concerning for meningioma, and later histologically found to be a chondroma. We seek to share our diagnostic approach, surgical management, and operative outcome of this patient in order to add to the paucity of literature available on this rare neoplasm.The patient is a 26-year-old female with poorly controlled type II diabetes and Crohn's disease who initially presented to her primary care physician (PCP) with vague neurological symptoms of dizziness, headache, and memory loss one week following a motor vehicle collision. The patient also mentioned symptoms resembling absence-type seizures. Her PCP ordered a CT scan of the head (CTH) to assess for intracranial pathology, which demonstrated a left frontal hyperdense extra-axial lesion with minimal surrounding edema, suggestive of a meningioma. Calcification present within the lesion suggested an indolent, long-standing mass Figure .Subsequent MRI brain re-demonstrated the dural-based extra-axial mass lesion along the left frontal convexity, but with less enhancement as expected with a meningioma. The lesion measured 15 x 25 mm in the axial plane  signal changes around the lesion, reducing the concern for pathologic edema Figure .She was referred to our institution for further diagnostic workup. Her neurological examination was non-focal and unremarkable for any signs of increased intracranial pressure. Repeat MRI three months later showed no change of the lesion. The patient continued to have symptoms likely attributable to her recent concussion. However, given the atypical imaging features of the left frontal convexity lesion that were not wholly consistent with meningioma, surgical intervention was offered to both definitively diagnose and treat the lesion. At this time, craniotomy with intraoperative neuro-navigation was planned to excise the lesion, as her blood glucose was slightly under control over this interval.The patient underwent a left frontal craniotomy with a partial pterional incision. The tumor was found to extend further anteriorly than suggested by neuro-navigation. The dura surrounding the tumor was excised circumferentially with a clean margin. The tumor was found to reflect off the cortical surface with no evidence of adhesion, and gross total resection was achieved. An intraoperative frozen section histologically revealed atypical mature cartilage, and abundant cartilaginous material with scattered, bland cells, inconsistent with a meningioma Figure . The perHer postoperative course was mostly uneventful, and while she was discharged on her fourth postoperative day, she returned for admission ten days later complaining of a frontal headache. She was discharged the next day after the resolution of her symptoms, and an unremarkable MRI revealing no infection, hemorrhage, or hydrocephalus. No adjuvant therapy was performed. At one year follow-up, the patient was free of any recurrence and doing well. She has not required follow-up visits since then.The etiology of chondroma is proposed to stem from the remnants of embryonic chondrocyte precursors that are housed within synchondroses, as these are joints composed entirely of hyaline cartilage. This leads to the most common location of intracranial chondroma being the skull base, specifically along the spheno-petrosal, spheno-occipital, and petro-clival synchondroses, this is where the majority of remnant chondrocytes would be found ,6. AnothGiven the rare nature of intracranial chondroma, few studies have looked specifically at the unique radiographic features of this tumor. However, a case series of six patients by Duan F et al. describes that all their cases presented as a well-demarcated lesion with irregular lobulation, variable density, and calcification on CT. Calcification is explained by the slow-growing and indolent course of this tumor. It is likely to be more present in chondroma than in meningioma, where it occurs in approximately 25% of cases, although more data is needed to support this fully ,19. DuanTissue diagnosis is the only reliable method to distinguish intracranial chondroma from other mimics, like meningioma. The literature consistently describes chondroma as comprising bland chondrocytes in a hyaline cartilage matrix ,12,16,18Because intracranial chondroma is a benign and slow-growing tumor, resection alone is usually sufficient, although occasionally adjuvant radiotherapy is required. However, there are no outcomes data specifically on meningeal chondroma, a case series of 66 patients with intracranial chondroma by Weng JC et al. details the difference in outcome between total vs. partial resection and the use of adjuvant radiotherapy . In their cohort, overall survival was significantly improved with gross total resection as compared to partial resection, but not subtotal resection. Factors significantly associated with greater progression-free survival included tumor size <3.1 cm, age >33 years, histology without atypia/mitotic activity, and surgery with adjuvant radiation therapy [Intracranial chondroma is a rare, indolent tumor that infrequently presents as a convexity lesion with dural attachment. This case report highlights the key clinical features of this rare pathology. The clinical presentation of this tumor is variable, but in most cases, will be a slow-growing, surreptitious mass of the skull base. Diagnosis of this lesion is a multimodal effort, with key radiographic findings including heterogeneous enhancement and density on CT, and mixed hyper- and hypo-intensity on T2 MRI. This lesion can be distinguished from meningioma, which tends to have more homogenous enhancement on T1- and T2-weighted imaging, even though both lesions may show calcification and the appearance of dural tails. Histologic diagnosis is definitive, as chondroma will show characteristic chondrocytes in a matrix of benign hyaline cartilage. With respect to clinical relevance, care must be taken while distinguishing the more common lesion of meningioma from convexity chondroma. This may occasionally be difficult due to similar  radiographic findings as delineated earlier. The treatment of choice when possible  is total resection of the mass. When achieved, favorable outcomes are generally the rule. Should total resection not be amenable, or atypical histology found, adjuvant radiotherapy is a useful strategy to consider.


This study aims to present a case of giant cell tumors of the tendon sheath on the big toe of a 5-year-old child.A 5-year-old girl presented with a painless swelling over the dorsal aspect of right big toe for 2 weeks. Physical examination revealed non-tender rubbery like swelling over the dorsal aspect of the right big toe. Ultrasound scan of the swelling showed a 17 × 7 mm oval-shaped subcutaneous hypoechoic lesion. Magnetic resonance imaging showed evidence of 20× 8 mm well-defined fusiform soft tissue lesion scalloping the bone. Under general anesthesia, the mass was totally excised. Microscopic sectioning showed a mixture of fibroblasts and histiocyte like cells associated with multinucleated giant cells in the vascular connective tissue stroma with the definite diagnosis of the giant cell tumor of the tendon sheath.These tumors mostly compose of several types of cell like synovial, siderophages, foam, inflammatory and multinucleate giant cells. The major etiological factors that induce development of this tumor could be traumatic, inflammatory, metabolic or neoplastic disease.although it is a sporadic finding, giant cell tumors of the tendon sheath might affect the lower limb in children. Complete excision is the main modality of treatment. •A giant cell tumor of tendon sheath is a painless benign soft tissue tumor.•There are only a few cases that have been reported in the feet of children.•In this study a case of giant cell tumor of tendon sheath in a 5-year-old child discussed. This enThis study aims to present a case of GCTS on big toe in a 5-year-old child with a brief literature review. The report has been arranged in line with SCARE guidelines .Patient Information: A 5-year-old girl presented with a painless swelling over the dorsal aspect of right big toe for 2 weeks. There was no history of traumatic events. Past medical, surgical, drug and family history was negative.Clinical Findings: Physical examination revealed non-tender rubbery like swelling over the dorsal aspect of the right big toe  with normal overlying skin and normal movements of the metatarsophalangeal and interphalangeal joints  with intravenous gadolinium contrast showed evidence of 20× 8 mm well defined fusiform soft tissue lesion scalloping the bone with no obvious destruction, intermediate signal in T2 with homogenous contrast enhancement , the possibility of existing satellite lesions and the relationship of the tumor to the surrounding tissue . GCTS usComplete resection is the main therapeutic strategy. It has been encouraged to use postoperative adjuvant radiotherapy as well to decrease the chance of recurrence .There are a lot of controversies regarding the factors involving the recurrence of GCTS. These are; hypercellularity and mitotic activity, interphalangeal joint lesion, previous joint diseases and incomplete excision ,16. In aIn conclusion; although it is a sporadic finding, GCTS might affect the lower limb in children. Complete excision is the main modality of treatment.Written informed consent was obtained from the patient's family for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.None is found.Not commissioned, externally peer-reviewed.None.


A 58-year-old male who underwent left total hip arthroplasty in 1988 for post-traumatic arthritis after an operatively-treated acetabular fracture presented with progressive left hip pain, lower extremity swelling, urinary urgency, constipation, and tenesmus (the feeling of needing to pass stool despite having an empty colon). Imaging demonstrated massive pseudotumor causing iliac vein compression and significant displacement of the rectum and bladder requiring decompression in combination with general surgery followed by revision hip arthroplasty four months later. This case highlights a unique constellation of symptoms due to pseudotumor after total hip arthroplasty, as well as the severity to which pseudotumor can progress, requiring staged decompression with general surgery before revision. Pseudotumor occurs in the setting of inflammation due to total hip arthroplasty (THA) wear debris ,2. TheseTo our knowledge, compression of the rectum causing constipation and tenesmus due to pseudotumor after THA has not been documented in the literature. This case report details a complex case of pseudotumor 27 years after THA, which required coordination with general surgery for multiple abdominal surgeries followed by THA revision four months later.The patient was informed that data concerning the case would be submitted for publication, and he provided informed consent for publication.A 58-year-old male presented to our clinic in May 2015 with worsening left hip pain, urinary urgency, constipation, and tenesmus in the setting of prior left metal-on-polyethylene THA in 1988 for post-traumatic arthritis after an operatively treated acetabular fracture sustained in 1986. The patient had done well after his total hip arthroplasty until March 2013, when he began having progressively worsening left hip pain, followed by intermittent left leg swelling, urinary urgency, constipation, and tenesmus. The patient had undergone multiple workups at outside hospitals, including hip aspirations, MRI, CT scan, and X-rays. X-rays demonstrated prior left acetabulum fixation with broken plate along the superior pubic ramus, heterotopic ossification, and prior left THA with large lytic lesions consistent with osteolysis  and C-reactive protein (CRP) were normal. The initial surgical plan included revision of the total hip arthroplasty with possible second surgery by general surgery for decompression of the abdominal mass if needed. The patient subsequently developed progressively worsening left lower extremity swelling and was referred to the general surgery clinic before revision THA. He underwent a lower extremity arterial and venous duplex ultrasound that was negative for thrombosis. At his appointment with general surgery in October 2015, he was noted to have discoloration of the left ankle and foot due to venous stasis secondary to left external iliac vein occlusion. His case was discussed with the orthopedics, general surgery, and vascular surgery services, and a plan for decompression and staged hip revision was developed. In November 2015, he underwent a combined procedure with orthopedics and general surgery for exploratory laparotomy, debulking, and decompression of the retroperitoneal mass. A standard laparotomy incision was utilized. Intraoperatively, the mass was noted to be significantly displacing the rectum and iliac vessels to the right. A screw and broken plate were removed from the left superior pubic ramus. The large cyst was then entered from a retroperitoneal extension through a preexisting defect in the quadrilateral surface Figure .A portion of the cyst wall was excised, and intra-abdominal and intra-cystic irrigation and debridement were performed. The mass contained a large volume of wear debris with a clay-like consistency Figure .Samples were sent for histopathology, which demonstrated a non-specific inflammatory reaction. Complete intraperitoneal excision was not undertaken due to the significant risk of adhesions associated with this approach. Two drains were placed, and the abdomen was closed with retention sutures and supra-fascial wound vac. The patient returned to the operating room the following day with general surgery after developing increasing left lower quadrant abdominal pain and increased sanguineous output from the drains and wound vac. A CT scan was obtained before he was taken to the operating room, which was unremarkable aside from the notable decompression of the pseudotumor Figure .Intraoperatively he was noted to have significant pelvic bleeding and received six units of packed red blood cells. His abdomen and pelvis were explored, and the bleeding was believed to be associated with the left anterior branch of the obturator artery. This branch was unable to be found intraoperatively, therefore the pelvis was packed with lap pads and temporarily closed with ABThera open abdomen negative pressure therapy . The patient was taken for angiography and underwent embolization of the anterior division of the left internal iliac artery and placement of an inferior vena cava (IVC) filter. He subsequently stabilized and did not require further blood transfusions. He returned to the operating room the following day for removal of pelvic packing and secondary abdominal closure with retention sutures and supra-fascial wound vac. He was discharged from the hospital with the supra-fascial wound vac, which was maintained and changed every 3-5 days until secondary healing was complete. In December 2015, the patient’s incision had completely healed, and he was noted to be voiding normally and having normal, consistent bowel movements with a resolution of his pre-operative symptoms. In March 2016, he underwent left THA revision consisting of head and liner exchange through the central aspect of the prior Kocher-Langenbeck incision. His prior acetabular shell and femoral stem were noted to have excellent fixation without loosening. The size 28 head and polyethylene liner were removed, and significant wear was noted centrally in the polyethylene liner. The trunnion was inspected, and no evidence of trunnionosis was present. Extensive scar tissue was noted around the joint and required debridement. Clay-like wear debris, similar to that encountered in the initial surgery, was again noted and debulked. The most proximal cable from the prior proximal femur cerclage was removed. The acetabular screw holes were curetted, and the anteroinferior screw holes were noted to enter the cyst. Upon entry of the cyst, approximately one liter of coffee-colored fluid was evacuated. This fluid was culture-negative and associated with polyethylene wear debris. A new size 32 metal head and highly cross-linked polyethylene liner were placed following thorough debridement.The patient’s most recent follow-up was in July 2020, and he was noted to have no left hip pain with the resolution of prior urinary and bowel symptoms. X-rays demonstrated left THA components in good alignment without evidence of ongoing osteolytic changes or hardware complications Figure .His IVC filter was removed in June 2016. He continues to have occasional, intermittent left lower extremity swelling, however not to the degree that was noted pre-operatively in 2015.The case presented details, the course, and treatment of a large pseudotumor secondary to polyethylene acetabular liner wear with a unique constellation of presenting symptoms not previously described in the literature. In the case presented, several symptoms were present secondary to the mass effect. In some patients, pseudotumor may be asymptomatic. Patients with symptomatic pseudotumor typically undergo revision arthroplasty, while asymptomatic patients can be closely observed for clinical signs or symptoms as well as metal ion levels if metallic wear is suspected ,2,10. InMultiple reports detail compression of the iliac vein secondary to pseudotumor, resulting in lower extremity swelling and/or deep vein thrombosis (DVT) -5. A comPseudotumor secondary to THA can present in a variety of ways, and as such, it is critical for clinicians in various specialties to be aware of this when evaluating patients with prior THA. Our case demonstrates a patient with a unique constellation of symptoms due to intrapelvic compression secondary to pseudotumor. The severity and complexity of these cases are noteworthy and often require a combined approach for treatment with orthopedics, general surgery, and vascular surgery, as in the case presented. The ultimate goal of treatment is to relieve compression on intra-abdominal or intrapelvic structures and ultimately perform THA revision to prevent worsening of the pseudotumor.


Approximately 0.17-2% of mature cystic teratomas undergo malignant transformation, of which squamous cell carcinoma (SCC) is the most common, accounting for 80% of these cases. Urothelial malignant transformation is extremely rare. The present study involves a 58-year-old patient who visited the hospital with discomfort in the lower abdomen. USG and pelvic MRI showed a left ovarian mature cystic teratoma. Left salpingo-oophorectomy was performed, and pathological examination revealed urothelial carcinoma transformation of the mature cystic teratoma morphologically and immunohistochemically. No metastasis to other organs was identified by CT after the surgery. Additional surgery, including total hysterectomy, right salpingo-oophorectomy, omentectomy, and dissection of pelvic and para-aortic lymph nodes, was performed without complications. No tumors were identified elsewhere, and the patient's stage was confirmed as IA. She had an uneventful postoperative course and was discharged 10 days later. CT showed no metastasis or recurrence six months later. Mature cystic teratoma (MCT) of the ovary is the most common type of ovarian germ cell neoplasm, occurring in approximately 20% of all ovarian neoplasms , 2. The A 58-year-old postmenopausal woman, gravida 1, para 1, presented at our hospital, with complaints of lower abdominal discomfort. Her initial pelvic examination revealed a soft mass in the pelvic cavity. Transvaginal USG revealed a 15-cm cystic mass, with a smooth surface and a fatty component, within the left ovary. Pelvic MRI revealed that the ventral side of the left ovarian cyst showed high signal intensity on T1-weighted images (T1WIs) and low signal intensity on fat-suppressed T1WIs, suggesting an accumulation of fluid with a fatty component and hairball-like floating material inside. These imaging results were indicative of MCT Figures -1B.As none of the mass was found to be solid, malignancy was not suggested. The tumor markers were as follows: SCC antigen, 3.6 ng/mL ; carcinoembryonic antigen (CEA), 1.3 ng/mL ; carbohydrate antigen (CA)-125, 7.7 IU/mL ; and CA19-9, 72.0 IU/mL . Other laboratory tests revealed no abnormalities.The patient underwent a left salpingo-oophorectomy via open abdominal surgery. Gross examination of the left ovary revealed a smooth surface cyst, which was 16 cm in diameter. The right ovary and uterus were grossly normal. Tumor rupture was not observed during surgery. On the cut surface, the cyst was filled with yellowish, creamy fluid and hairs, which are classic features of MCT.Microscopically, the left ovary showed various mature tissues, including skin appendages, glandular epithelium covering the bronchi, and fatty tissue. The epithelium covering the inner surface of the cyst had areas of urothelium and squamous epithelium Figure .Atypical epithelial cells were found only in the area of the uroepithelium, and grew as papillary-like structures, while the urothelium had nuclear pleomorphism, hyperchromatism, and increased mitotic activity, and there was evidence of microinvasion of nests of malignant epithelial cells into the ovarian stroma Figure . ImmunohPostoperative CT revealed no metastasis to other organs. The patient underwent a secondary operation, which included a total abdominal hysterectomy, right salpingo-oophorectomy, omentectomy, and dissection of pelvic and para-aortic lymph nodes, without complications. Pathologically, no tumors were identified in other organs, and therefore, the patient was diagnosed as stage IA . She had an uneventful postoperative stay and was discharged 10 days later, without sequelae. At her six-month follow-up, CT did not show distant metastasis or local recurrence, and no elevation of SCC or CA19-9 was observed.MCT is one of the most common ovarian tumors, accounting for approximately 20% . MalignaAn overview of the salient features of the previously mentioned eight cases, as well as the present patient, is presented in Table The mean age of the patients from all nine cases was 52 years. Of the patients for which data were available, four were postmenopausal and only two were premenopausal, which is compatible with previous reports on malignant transformation of MCTs . In mostSome studies have reported various risk factors for the malignant transformation of MCT. Age greater than 45 years, preoperative tumor size >9.9 cm, and elevated levels of serum tumor markers  were noted as being risk factors . Of the GATA3 is a dual-zinc finger transcription factor that regulates gene expression in many developing tissues, which is positive in 76% of urothelial carcinomas . It can The pathogenesis of malignant transformation of MCTs is not yet completely known. Iwasa A et al.  suggesteMRI is useful in the preoperative diagnosis of the malignant transformation of MCT, but sometimes no indication of malignant transformation is noted, as in the present case. Most mural nodules in MCTs without malignant transformation are called Rokitansky protuberances. However, because MCTs are composed of various components of the tritubular lobe, it has been reported that even without malignant transformation, they form mural nodules that differ from the Rokitansky protuberances, which are composed of neural tissue, thyroid tissue, and fibrous stroma . In the When it is difficult to differentiate malignant transformation using MRI, F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography/CT (PET/CT) may be considered. It has been reported that MCT with malignant transformation can be differentiated from MCT without malignant transformation by FDG-PET/CT, as the former has higher FDG accumulation when using the maximum standardized uptake value (SUVmax) 2.9 as a cutoff value .Urothelial carcinoma arising from MCT is extremely rare. The conditions for considering the possibility of urothelial malignant transformation may be the same as those for other malignant transformations; if the size is large, if there is a transmural soft tissue component inside the teratoma, if the patient is older than 45 years, or if tumor markers are high, the possibility of malignant transformation, including that of the urothelium, should be considered. FDG-PET and MRI may both be considered for the diagnosis of malignant transformation of MCT.


Craniopharyngiomas are rare, benign primary brain tumors that arise from remnants of the craniopharyngeal duct epithelium within the sellar and suprasellar region. Despite their benign biology, they may cause significant morbidity, secondary to involvement of nearby eloquent neural structures, such as the pituitary gland, hypothalamus, and optic apparatus. Historically, aggressive surgical resection was the treatment goal to minimize risk of tumor recurrence via open transcranial midline, anterolateral, and lateral approaches, but could lead to clinical sequela of visual, endocrine, and hypothalamic dysfunction. However, recent advances in the endoscopic endonasal approach over the last decade have mostly supplanted transcranial surgery as the optimal surgical approach for these tumors. With viable options for adjuvant radiation therapy, targeted medical treatment, and alternative minimally invasive surgical approaches, the management paradigm for craniopharyngiomas has shifted from aggressive open resection to more minimally invasive but maximally safe resection, emphasizing quality of life issues, particularly in regards to visual, endocrine, and hypothalamic function. This review provides an update on current multi-modal approaches for craniopharyngiomas, highlighting the modern surgical treatment paradigm for this disease entity. Craniopharyngiomas are uncommon, benign central nervous system (CNS) tumors that arise from the remnants of the craniopharyngeal duct epithelium in the sellar and suprasellar region . They reThe incidence of craniopharyngiomas is between 1.3 to 1.7 cases per 1,000,000 person-years with a bimodal age distribution ,3. ApproThere are two histological subtypes of craniopharyngioma: adamantinomatous and papillary. While adamantinomatous craniopharyngiomas can occur in children and adults, the papillary subtype is mainly observed in adults ,6. The aOn imaging, most craniopharyngiomas measure between 2–4 cm at time of diagnosis and demonstrate a cystic component in approximately half of cases, more prevalent in the adamantinomatous subtype ,13. On mDespite being a benign tumor, craniopharyngiomas can cause significant morbidity, related to visual, endocrine, hypothalamic, and cognitive dysfunction. As such, the primary goal of surgery is to decompress involved structures, while achieving maximally safe resection. If possible, gross total resection (GTR) is the best surgical outcome, as recurrence rates are significantly lower in cases of GTR . Recent Historically, craniopharyngiomas were resected via open transcranial techniques, later improved by the introduction of the surgical microscope ,32. The The supraorbital keyhole craniotomy is an alternative minimally invasive surgical approach to craniopharyngiomas in the suprasellar region and around the third ventricle. This approach may afford wider visualization of the internal carotid arteries, optic chiasm and nerves, optico-carotid recess, lamina terminalis, and pituitary stalk, while minimizing the risk of cerebrospinal fluid leak, associated with an expanded endoscopic endonasal approach . In geneHere, we provide a succinct overview of our surgical technique for the endoscopic endonasal approach for craniopharyngiomas. With the ability to expand the endoscopic endonasal approach beyond the transphenoidal approach into the transtuberculum and transplanum region, we have found that the majority of craniopharyngiomas are amenable to the endoscopic endonasal approach alone. In cases where there is significant lateral expansion of the tumor, limiting endoscopic visualization, we consider adjunct surgical approaches after initial endoscopic endonasal debulking.All patients undergo standard pre-operative evaluation to assess visual and endocrine function by our neuro-opthalmology and endocrine colleagues. This includes formal visual field testing and measurement of pituitary hormone levels. A dedicated pituitary protocol MRI and CT angiography (CTA) of the head are obtained to understand the locations of the pituitary gland/stalk, optic nerves, internal carotid arteries, and anterior cerebral and middle cerebral arteries in relationship to the tumor a–c.The technique of the endoscopic endonasal approach has been extensively described in the literature ,45,46. HSubsequently, a bimanual approach is performed through each nasal cavity during the neurosurgical portion of the operation, aided by endoscopic visualization by otolaryngology. Afer routine craniotomy and dural opening, the corridor between the optic chiasm and pituitary gland is dissected to approach the tumor capsule. a. The tuAfter the tumor is maximally resected d, we typWe have utilized the supraorbital approach as an ideal surgical corridor for the majority of craniopharyngiomas with significant antero-lateral extension in the suprasellar region beyond the optic chiasm and supraclinoid internal carotid arteries, respectively. In contrast, more midline and sellar-based craniopharyngiomas can be readily removed with the endoscopic endonasal approach alone. Given the versatility of an expanded endoscopic endonasal approach, we resect the vast majority of craniopharyngiomas endoscopically as the initial approach and reserve the supraorbital craniotomy as a surgical adjunct for those with significant laterally located residual tumors, causing persistent compression of eloquent structures. A brief description of our surgical technique is described below.The supraorbital approach has been described in detail previously in the literature ,49,50. KA subset of craniopharyngiomas cause symptoms related to mass effect from the cystic component of the tumor, either at time of diagnosis or tumor recurrence. In these cases, alternative strategies aimed at reducing the cyst via stereotactic or neuroendoscopic cyst aspiration may be a viable strategy with less morbidity than surgical resection ,52. Most32P), yttrium-90 (90Y), resulting in radiation dosages, ranging between 150–500 Gy, delivered to the cyst wall [In the pediatric population, the use of the Ommaya reservoir system has been described to treat mass effect-related symptoms and delay the need for aggressive surgical surgery and/or radiation to allow for full maturation of the hypothalamic-pituitary axis . This apyst wall ,58. A reyst wall ,61,62,63In the adult population, the use of Ommaya reservoirs for cystic craniopharyngiomas as an isolated therapy has been mainly limited to smaller and heterogeneous case series with relatively low recurrence rates of 0–27.3% over a follow-up period up to 56 months ,64,65. TThe Ommaya catheter can be inserted under neuroendoscopic visualization, stereotactically, or under direct visualization during open surgery. A recent case series of 11 adult patients by Frio et al. did not find any significant differences in local cyst growth control with either endoscopic or stereotactic reservoir insertion techniques . StereotThe addition of adjuvant radiotherapy may facilitate local tumor control after Ommaya catheter placement for cystic craniopharyngiomas ,74,75,76Radiotherapy is an alternative treatment for craniopharyngiomas, either used alone or in combination with surgery. Traditionally, conventional external beam radiotherapy using X-rays was used but at the expense of collateral radiation exposure to normal tissues from the entering and exiting traversing beams ,79. ProtIn general, radiotherapy in craniopharyngiomas has been utilized as an adjuvant therapy after STR, leading to similar tumor recurrence rates of 9.1–37.5%, compared to those following GTR alone ,92,93,94At our institution, we routinely utilize the endoscopic endonasal approach as the first surgical intervention for patients with suspected craniopharyngioma diagnosis. The surgical goal is to decompress the optic nerves through a maximally safe resection, given that inadequate separation from the optic nerves may limit adjuvant radiation post-operatively. While GTR is the most desired outcome, it should not be pursued where the tumor cannot be safely removed from the optic nerves, hypothalamus, and pituitary stalk, the latter particularly in patients with normal pre-operative endocrine function. In cases where there is residual tumor, patients are referred routinely for adjuvant radiation therapy, typically with stereotactic radiosurgery. Rarely, the optic nerves are inadequately decompressed after endoscopic surgery, precluding adjuvant radiation. These patients typically have significant lateral extension of their tumors and require a second operation, typically performed during the same admission usually via a supraorbital craniotomy, with the goal of decompressing the optic nerves enough to facilitate post-operative radiation. Other open transcranial approaches are also utilized if the supraorbital approach is not feasible. Patients with papillary craniopharyngiomas harboring BRAF mutations are also evaluated by neuro-oncology for consideration of treatment with oral BRAF inhibitors. Subsequently, after GTR or STR with adjuvant radiation, patients undergo periodic surveillance MRI to monitor for tumor recurrence, as well as routine endocrine evaluations to manage ongoing or delayed endocrine deficits. In patients with tumor recurrence and progression, a repeat resection can be performed for most cases, followed by a combination of re-irradiation and/or chemotherapy. In instances of purely cystic recurrence, stereotactic placement of an Ommaya reservoir into the cyst is preferred, followed by adjuvant radiation during the same hospitalization. While each case of craniopharyngioma is evaluated individually for the most optimal upfront surgical approach or alternative treatment modalities, we have found that the vast majority of operative cases are amenable to an endoscopic endonasal approach. As such, a simplified schematic describing our treatment paradigm for adult craniopharyngiomas undergoing endoscopic resection is shown in The management of craniopharyngiomas requires a multi-disciplinary approach involving neurosurgery, otolaryngology, endocrinology, ophthalmology, radiation oncology and neuro-oncology. As highlighted in this review, the introduction and refinement of the endoscopic endonasal approach has revolutionized surgery for craniopharyngiomas, such that the vast majority of these tumors can be resected through this approach. Adjuvant radiation and novel targeted therapies represent viable, effective treatment options for residual tumors after surgery. Likewise, adjunctive open or minimally invasive surgical approaches like a supraorbital craniotomy and/or stereotactic cyst decompression can be utilized to address residual or recurrent tumors. With the success of this multi-modal treatment approach, the goal of surgery remains maximally safe resection, emphasizing preservation of visual and hypothalamic function to maintain quality of life in these patients.