Fever of unknown origin is often a diagnostic dilemma for clinicians due to its extremely broad differential. One of the rarer categories of disease causing fever of unknown origin is malignancies; of these, soft tissue sarcoma is one of the least common. Soft tissue sarcomas make up < 1% of all adult malignancies and often do not present with any systemic manifestations or neoplastic fevers.A 73-year-old Caucasian woman presented with a 2-week history of fever and profound fatigue. The only other symptom she endorsed was a transient history of left knee pain, initially thought to be unrelated. There was no clear cause on initial examination and routine investigations, but her C-reactive protein was significantly elevated at 207 mg/L. Blood cultures and a urine culture were drawn. She was admitted to hospital for further investigation and placed on empiric antibiotics. Her blood cultures were negative, but she had one further fever in hospital. Computed tomography scans did not yield a cause of her fever. No vegetations were seen on echocardiography. Antibiotics were stopped as she did not seem to have an acute infectious cause of her fever. No new symptoms developed. She felt well enough to proceed with out-patient follow up and was discharged after 8 days in hospital. At 1-month post-discharge: no resolution of symptoms, but she endorsed a recurrence of her left knee pain. Ultrasound and magnetic resonance imaging revealed a 4.5 × 6.8 × 11.6 cm soft tissue mass, identified as a sarcoma on biopsy. She subsequently underwent a distal femur resection. Final staging was pT2bN0M0. She underwent adjuvant radiation therapy, but was found to have developed metastatic disease.This case revealed an atypical presentation of a rare soft tissue sarcoma as the cause of the illness. The etiology behind a fever of unknown origin can be difficult to elucidate, making the approach to investigation particularly important. Repeated history-taking and serial physical examinations can be crucial in guiding investigations and ultimately arriving at a diagnosis. In addition, we believe this case highlights the adage that no seemingly innocuous symptom should be left out when working up a condition with such an extensive and complex differential. Fever of unknown origin (FUO) commonly presents with atypical manifestations of the underlying cause. In these scenarios, no symptom should be deemed insignificant.Repeated examinations over time are often needed to elucidate the cause of FUO.FUO is an uncommon presentation for sarcoma but can occur.The case described is of a patient with non-specific constitutional symptoms, an initial history of transient left knee pain, and the development of a FUO. Workup eventually led to the diagnosis of an undifferentiated sarcoma of epithelioid morphology. This diagnosis is not only rare in terms of sarcoma incidence rates, but also because of its unusual systemic presentation .Sarcomas are a heterogeneous group of malignant tumors of mesenchymal origin, representing roughly < 1% of all adult malignancies. Approximately 90% of cases of sarcoma are defined as soft tissue sarcomas (STSs): developing in the muscles, deep tissues, blood vessels, nerves, fat, and joints, while the rest are classified as malignant bone tumors .The Surveillance, Epidemiology, and End Results (SEER) database indicates that the incidence rates of STS development are highest in children < 5 years of age and in adults > 50-years old, with the lowest incidence found in young adults . NumerouSTS can be divided extensively by histologic subtypes, for which the World Health Organization (WHO) currently lists more than 100. These malignancies can also be described in terms of location and specific histologic morphology. Site distribution favors the lower extremities 29%), with tumors less often involving the upper extremities (11%) and trunk (10%)  was 207 mg/L. On examination, she had a large, warm, left thigh mass. An urgent ultrasound revealed a 4.5 × 6.8 × 11.6 cm spindle-shaped, well-defined soft tissue mass with internal vascularity  reconstruction. Final pathology revealed a grade 3, pT2bN0M0 undifferentiated sarcoma with epithelioid morphology. She had no nodal involvement or distant metastases at this time. Her CRP fell to 28.42 mg/L within 8 days of surgical excision. She recovered well from her surgery with resolution of her constitutional symptoms. She subsequently was planned to receive radiation therapy.Prior to receiving radiation therapy, a follow-up CT scan was done a couple months after her surgery. This revealed the presence of a new 4 mm pulmonary nodule in the lower lobe of her left lung that was not felt to be a metastasis. There was no other evidence of distant metastases. Given these results, adjuvant radiation treatment was begun. She received 6600 cGy given in 33 fractions to her leg.Roughly 1 month following the end of her radiation therapy course, she re-presented to our emergency room with painless hematuria and a month-long history of non-productive cough associated with decreased energy. CT scans of her chest revealed 16 pulmonary masses, measuring up to 6.2 cm. A CT scan of her abdomen and pelvis revealed a solitary nonobstructive renal calculus, as well as a new 3.2 × 6.5 cm pelvic mass.She was subsequently referred to radiation and medical oncology where a shared decision was made to pursue palliative management.Figure The presence of FUO and neoplastic-based fevers with bone or STSs, as was the case with our patient, is not extensively reported in literature. In a cohort of 195 patients with bone and STSs who were admitted for treatment, 58 (30%) developed a fever while in hospital. Neoplastic fever was determined to be the cause in only 11 (6%) . This caInvestigation of FUO, guided by patient history and physical examination findings, should begin with a broad systematic approach to narrow the differential as efficiently as possible. Table Prognosis is variable in FUO, reflecting the multitude of potential etiologies . OverallIn the current era, limb sparing treatment procedures are often the preferred management option for STS. Along with surgical resection, adjuvant radiotherapy and chemotherapy can also be a key component to the management of STS. As was the case with our patient who had a grade 3 STS, most moderate to high-grade STSs receive adjuvant radiotherapy. The combination of local resection and radiotherapy has similar survival and recurrence rates as amputation, but with the added benefit of functional preservation . HoweverIn conclusion, determining an etiology in a patient with FUO can be challenging due to the breadth of possibilities. It is not only challenging, but FUO often represents an atypical presentation of a disease. This case provides such an example and aims to highlight the extensive workup required. In addition, it supports the adage that a full detailed history and examination is often a necessity when evaluating FUO; repeated history and examinations may be needed, with no symptom being viewed as irrelevant. Findings then must be used to direct further investigations. An investigatory approach is outlined in this case report and aims to provide clinicians a foundation from which to work from in the workup of similar cases.


Water-enema multidetector CT (WE-MDCT) provides a detailed multiplanar visualisation of mural, intra- and extraluminal abnormalities of the large bowel, relying on preliminary bowel cleansing, retrograde luminal distension, pharmacological hypotonisation and intravenous contrast enhancement. In patients with a history of colorectal surgery for either carcinoma or Crohn’s disease (CD), WE-MDCT may also be performed via a colostomy, which allows depicting the anatomy and position of the residual large bowel and evaluates the calibre, length, mural and extraluminal features of luminal strictures. Therefore, WE-MDCT may prove useful as a complementary technique after incomplete or inconclusive colonoscopy to assess features and suspected abnormalities of the surgical anastomosis, particularly when endoscopic or surgical interventions are being planned. This pictorial essay presents the WE-MDCT technique and pitfalls, the expected appearances after different colic surgeries and the imaging features of benign anastomotic disorders  and of locally recurrent tumours and CD.Water-enema multidetector CT (WE-MDCT) effectively visualises the operated colon• Complementary to endoscopy, WE-MDCT may helpfully depict abnormalities of surgical anastomoses• WE-MDCT allows assessment of strictures’ features and abnormalities of the upstream bowel• Technical pitfalls, normal postsurgical findings and benign anastomotic disorders are presented• WE-MDCT allows detecting relapsing Crohn’s disease, recurrent and metachronous tumours•  Since the first description by Gossios et al. , water-eFollowing colorectal surgery for either benign or malignant processes, optical colonoscopy remains the gold standard technique to assess the anastomotic site and residual large bowel and to identify recurrence of resected disease. However, in operated patients endoscopy is often hampered by postsurgical adhesions, sharp bowel angulations, bowel kinking, poor bowel preparation and anastomotic strictures (AS). WE-MDCT allows reliable measurement of the colonic wall thickness in normal and pathological conditions, and provides a detailed multiplanar assessment of mural, intra- and extraluminal abnormalities of the large bowel. Therefore, its use is appealing to investigate the operated colon, particularly after unsuccessful, incomplete or inconclusive endoscopy.Based upon personal experience at a tertiary hospital that performs IBD and oncologic surgery, this pictorial essay presents the WE-MDCT technique and pitfalls, the expected appearances after various colon surgeries and the imaging features of benign anastomotic disorders, recurrent tumours and Crohn’s disease (CD).Retrograde colonic distension is contraindicated in the presence of high-grade bowel obstruction, when a standard contrast-enhanced CT acquisition reliably investigates the site and cause of obstruction, thanks to the pre-existent bowel dilatation with intraluminal fluid. Before WE-MDCT, bowel cleansing is obtained using an iso-osmolar non-absorbable laxative solution  the day before the examination, in association with a low-fibre diet for 3 days. Patients fast for 12 h after a liquid dinner the evening before the scheduled examination .The patient is positioned on the CT scanner table and is instructed to turn to the left lateral decubitus. A lubricated enema tube is gently inserted into the rectum and connected to a bag that contains 1.5–2 l of warm tap water. Retrograde colonic distension through gravity is obtained within a few minutes and is stopped when the patient complains of abdominal distension. Compared with surgically naive patients, distension of the operated colon requires a reduced amount of water, depending on the extent of resection. Then, the patient is turned to his/her right side to improve water distribution in the colon and finally positioned supine for CT acquisition. During retrograde filling, pharmacological hypotonisation is induced with either 20 mg hyoscine buthylbromide i.v. or 1 mg glucagon i.m. to improve the patient’s comfort and obtain colonic wall distension. In patients with a colostomy WE-MDCT may be also performed using a Foley balloon catheter positioned and inflated into the stoma opening  after incomplete endoscopy to assess the colonic mucosa and pericolic tissues searching for local recurrence, metachronous polyps and tumours. Although the residual colon was always entirely visualised with 100% sensitivity for AS, in their experience CTC findings were not sufficiently reliable to differentiate fibrotic from neoplastic strictures. Furthermore, in patients with right hemicolectomy retrograde insufflation of the anastomosed ileum may cause suboptimal distension of the residual colon .Compared with full-dose contrast-enhanced CTC, WE-MDCT has a shorter learning curve for radiologists, is less cumbersome for the patient without the need for rotation from the prone to supine position and generates a lower radiation dose because it involves a single CT acquisition during contrast medium injection. Although no studies compared the comfort between the two different techniques, Ridereau-Zins et al. observed that the retrograde introduction of warm water was well, moderately and poorly tolerated by 86.2%, 12.2% and 1.7% of patients, respectively . At our Although prospective trials comparing accuracy for lesion detection between CTC and WE-MDCT are lacking, some authors have compared the degree of colonic distension between the two techniques, concluding that the sigmoid and left colon were better assessed using both WE-MDCT and prone CTC compared with the supine CTC acquisition . TherefoIn the setting of chronic IBD, WE-MDCT has been validated by the Genoa group, consistently provides superior distension of the large bowel compared with CTE and optimally reproduces the well-known mural and extraluminal features of CD –12. FurtSimilarly to the preoperative setting , interprInterpretation of WE-MDCT scans benefits from the excellent contrast between the intraluminal water, enhanced colonic wall and normal fat-attenuation perivisceral planes. In surgically treated patients, WE-MDCT allows depicting the anatomy and position of the residual large bowel in arbitrary planes, including the coronal orientation, which is most appealing for surgeons. Study interpretation benefits from precise knowledge of the type of resection and reconstruction performed and of recent endoscopic findings. Surgical anastomoses should be evaluated for site, configuration, patency and mural features. Ileo-colic and colo-colic anastomoses can be either manual (hand-sewn) or mechanical, with the latter clearly identified by the presence of hyperattenuating circular or linear staple lines. Focused maximum-intensity projection (MIP) images may be helpful to assess the configuration and integrity of a stapled anastomosis Fig. , image cIn the left-sided colon, colo-colic anastomoses are generally shaped end-to-end, since the bowel lumen has a similar diameter on both sides. In normal conditions, the colonic wall thickness at a surgical anastomosis should not exceed 3–4 mm Figs. , 3. In aEnd-to-side anastomoses are used when a smaller proximal lumen is connected to a larger one such as after standard Fig.  or extenFollowing ICR for CD, the ileocolic anastomosis is frequently of the end-to-side or side-to-side type with a distended, prominent colonic blind end (“cul-de-sac”) Fig. . FinallyThe development of a benign colorectal AS is an increasingly common postsurgical problem. Strictures may form after open or laparoscopic surgical treatment of either benign or malignant diseases, with a higher incidence in stapled compared with hand-sewn anastomoses. Although causes are still not clearly understood, AS may result from ischaemia, disruption or leakage at the anastomotic site. Symptoms such as pain, distension, nausea and vomiting are reported in up to one-third of patients after colorectal surgery and reflect the degree of luminal stenosis –23.The characteristic appearance of a benign, fibrotic AS includes an indistensible, often hourglass-shaped colo-colic anastomosis with normal mural thickness, without adjacent abnormalities Fig. . AlternaIn our experience, WE-MDCT is particularly helpful when repeated surgery or endoscopic treatment of AS is being contemplated. Balloon dilatation with or without endoscopic incision using laser or argon plasma is successful in 88% of benign AS cases. Alternatively, refractory ASs are increasingly treated with fully covered stents to achieve a prolonged clinical success and to obviate the high morbidity from revisional surgery , 25–28.Inflammatory anastomotic ulcers of unknown cause may occasionally develop at ileo-colic anastomoses months to years after the primary surgery. These rare conditions generally manifest with occult blood loss, do not respond to anti-inflammatory, antibiotic and immunosuppressive medications, and frequently recur after surgical treatment. At WE-MDCT, focal irregularities or unspecific intraluminal projections Fig.  may be sFollowing CRC resection, patients are at risk for local anastomotic or extraluminal tumour recurrence and may develop adenomatous polyps and metachronous tumours in the residual bowel. At endoscopic surveillance, abnormal findings are found within 2 years from surgery in up to 18.5% of patients , 32. RecThe hallmark of a local neoplastic recurrence is a solid, more or less homogeneously enhancing mass that projects in the lumen Fig.  or abutsFollowing ICR, CD recurrence at the ileo-colic anastomosis occurs in up to 70% of patients. In this situation, the use of CT has a complementary role to endoscopy to assess the transmural and longitudinal extent of transmural inflammation and detect possible extramural complications of penetrating CD. Like with peroral CT enterography, the hallmark of relapsing CD is stratified mural thickening at the neoterminal ileum with either mucosal or homogeneous transmural hyperenhancement compared with other adjacent bowel loops, with associated prominence of mesenteric vessels (“comb sign”) Figs. , 15A–B. failure to achieve retrograde colonic distension because of incontinence at either the rectum or colostomy;segmental non-distension or spasm, which most commonly occurs at the sigmoid or the nondependent transverse colon and generally mimics a segmental mural thickening Fig.A–C; thispresence of faecal residues from insufficient bowel cleansing Fig.D–E, whicThe three main pitfalls of this technique include:In our experience, WE-MDCT is a rapid, easy-to-perform and well-tolerated technique that provides multiplanar high-resolution visualisation of the operated large bowel, with the drawback of ionising radiation. In selected patients with a history of colorectal surgery, WE-MDCT may prove useful as a complementary technique to endoscopy to assess suspected abnormalities at the anastomosis. Furthermore, WE-MDCT allows assessing the calibre, length, mural and extraluminal features of luminal strictures and evaluating the bowel upstream to an endoscopically impassable tract and is therefore particularly helpful when endoscopic procedures or surgical interventions are being planned.


Amoebic colitis, caused by ingestion of water or food contaminated with the protozoan Entamoeba histolytica, can progress to a fulminant colitis. Computed tomography (CT) findings reported in the literature on this type of colitis are sparse. We present a 59-year-old male patient with a one-week history of progressive abdominal pain, abdominal distension and associated watery and bloody diarrhoea. A CT scan revealed deep ulcerations with submucosal and intramural tracking of contrast. Colonoscopy and biopsy confirmed a diagnosis of Amoebic colitis. The patient required a laparotomy and demised. Deep ulcerations with submucosal and intramural tracking of contrast on CT are diagnostic of fulminant amoebic colitis. Although not demonstrated at CT in this case, discontinuous bowel necrosis, omental wrapping (seen at laparotomy in our case) and neovascularisation of the bowel wall may be other features to look out for. Fulminant amoebic colitis (FAC) is the transmural extension of amoebic colitis, associated with a devastating outcome. Until a few decades ago, the diagnosis of FAC was made without the recourse to computed tomography (CT) scan; diagnosis was largely clinically supported by stool examination and sigmoidoscopic examination, with plain radiology and sonography having a subsidiary diagnostic role.In current practice, largely because of improvements in sanitation and primary health care access, FAC is uncommon; thus, the recognition of this entity may be found to be lacking, but should be suspected in those vulnerable to sexually transmitted disease, human immunodeficiency virus infection and those travelling from endemic areas. The difficulty in diagnosis is offset by improvements in diagnostic modalities and their availability, as prompted by our recent experience in managing a patient with FAC. To this end, the role of CT scan in the diagnosis of FAC merits review.9/L, haemoglobin 9 g/dL, urea 9 mmol/L and creatinine 100 µmol/L. An abdominal triple contrast-enhanced  CT scan revealed segments of grossly dilated large bowel with areas of thickening, deep ulceration, contrast tracking deep within the bowel wall and skip areas with normal bowel wall calibre . Once the ingested cysts reach the terminal ileum, they undergo excystation and release the trophozoite.1 The trophozoites may or may not penetrate the colonic mucosal barrier. If no penetration occurs, this is regarded as a non-invasive form and the patient has no luminal pathology and no symptoms; if mucosal penetration occurs, the patient will develop symptoms ranging from mild colitis  to the devastating FAC  which carries a high morbidity and mortality in the range of 55% – 100%.3Amoebic colitis is caused by ingestion of water or food contaminated with the protozoan E. hystolytica.4 Because of the low sensitivity of these non-invasive tests, they have been replaced by molecular detection using polymerase chain reaction that demonstrates excellent sensitivity in identification of symptomatic and asymptomatic stool carriers.Whereas mild amoebic colitis may be diagnosed and treated effectively utilising conservative measures, the confirmation of FAC on the basis of diagnostic modalities may be challenging. Historically, amoebiasis was confirmed on stool microscopy identifying trophozoites and cysts or stool antigens and the detection of antibodies specific for 5In addition to the demonstration of free intraperitoneal air or retroperitoneal mottling in patients with FAC, the plain abdominal radiograph may reveal features distinctive to amoebic colitis. Classically, the involved segments of the colon are not visualised (similar to toxic megacolon of ulcerative colitis) and gaseous distention occurs in the non-diseased colon. In addition, Luvuno described ‘fixed displacement’ of the colon as an indicator of the site of perforation, as the uninvolved colon acts as a patch over the perforated areas, to which it becomes adherent.Although generally not impacting considerably on the overall diagnosis of amoebic colitis, ultrasound examination of the liver is important as between 20% and 30% of patients with amoebic colitis have an associated amoebic liver abscess; recognition and treatment of the latter enhance the overall clinical outcome.E. histolytica and the pathognomonic flask-shaped ulcers. However, this carries the potential risk of perforation, especially in an acutely inflamed and possibly ischaemic colon. Furthermore, as in this case report, results of the various diagnostic tests may not be timeously forthcoming for a definitive diagnosis and a recourse to laparotomy may be required.Invariably, the diagnosis of amoebic colitis may have to be confirmed by a procto-colonoscopy with biopsy demonstrating 6 This phenomenon may be apparent on CT scan imaging.Although colitis caused by cytomegalovirus, salmonellosis, ulcerative colitis, pseudomembranous colitis and ischaemic colitis may present with pancolitis, discontinuous bowel necrosis is extremely rare in these types of colitis. Furthermore, as a result of the ischaemic phenomenon in amoebic colitis, the omentum forms adhesive ‘wraps’ that afford both mechanical protection from microperforation and neovascularisation of the diseased colon, a phenomenon described by Luvuno.Even if a diagnosis of FAC is made, the clinical challenge is whether to perform surgery on a severely ill patient, because there may be a role for effective conservative treatment when colonic perforations are contained by the omental ‘wraps’ and, less so, by the unaffected small bowel. The role of CT scan in identifying the latter category of patient would be appropriate.7 Thus, the literature describing the CT scan features of FAC is sparse.The decline in the incidence of FAC affords little opportunity to correlate this diagnostic modality with the features of FAC, although there are reports of non-specific CT scan features for FAC.8 This feature (noted in our patient) represents the underlying flask-shaped ulcer pathology typical of amoebiasis and excludes other infective, inflammatory or ischaemic bowel diseases . Furthermore, there is a target appearance of the rectum with proliferation of the perirectal fat.10 Crohn’s colitis is also associated with a greater colon wall thickness (> 10 mm) that is eccentric, preferentially mesenteric, and segmental with skip lesions. In addition, there is an association with several extraluminal features such as fistula and abscess formation and mesenteric abnormalities.10Crohn’s disease has transmural involvement that progresses to replacement of submucosal fat with fibrosis and loss of mural stratification.10 Typically, the mural thickening is 5 mm–15 mm.11 A misdiagnosis of FAC as Crohn’s disease can be deleterious for the patient if steroid treatment is implemented.3The role of CT in the evaluation of Crohn’s disease is well accepted. The ability of CT to depict bowel involvement and extraluminal pathology  makes it an essential imaging tool for patient care. The earliest CT finding of Crohn’s disease is bowel wall thickening, which usually involves the distal small bowel and colon, although any segment of the gastrointestinal tract can be affected.The challenges presented by FAC are confirming the diagnosis  and deciding on the appropriate management. Management depends on the severity of the colitis; both mild and severe colitis can be successfully treated conservatively; however, operative intervention is required when there is a free or a sealed perforation, contained by an omental wrap, which both progress to transmural disease, manifesting as FAC. Correlating the CT scan findings with the pathological manifestations of the FAC will prompt an appropriate management.Although a vast differential diagnosis of colitis should always be kept in mind, the majority of other colitis conditions such as ulcerative colitis, inflammatory colitis and ischaemic colitis can be excluded. Crohn’s colitis and cytomegalovirus (CMV) colitis may mimic amoebic colitis because of the skip lesions, but these conditions do not present with deep ulcerations, submucosal and intramural tracking of contrast, discontinuous bowel necrosis or omental wrapping and neovascularisation of the bowel wall.


Recently, endovascular repair has become the first-line treatment for internal iliac artery aneurysm (IIAA). However, rectal necrosis due to the compression of the residual IIAA early after endovascular repair is rare.We present a rare case of a huge, isolated left IIAA that severely compressed the rectum and ureter. The patient underwent emergency endovascular repair; however, rectal necrosis occurred 10 days later because the repair failed to shrink the size of the aneurismal sac.We hypothesize that the compression of the residual IIAA caused rectal necrosis. During open surgery, endovascular repair disrupted blood flow within the IIAA, which probably allowed for aneurysm dissection and residual hematoma removal.Endovascular repair alone could not immediately release compression on the surrounding organs; however, open surgical removal of aneurysms after successful endovascular repair may be a useful option for IIAAs with compression of surrounding organs. The cHerein, we present the case of a large, isolated IIAA complicated by bowel and bilateral ureteral obstructions. Although we treated the IIAA using endovascular repair, residual aneurysm compressed the rectum, resulting in necrotic perforation in the rectum early during the postoperative period. Colonic ischemia is known to occur after interruption of internal iliac artery at 1.12%–2.5% ; however2A 76-year-old man was referred to a local hospital with a 2-week history of dysuria and constipation. He had a medical history of hypertension and atrial fibrillation, for which he had been prescribed antihypertensives and anticoagulants. When the initial abdominal computed tomography (CT) revealed a large IIAA, the patient was transferred to our hospital for emergency treatment. At our hospital, the biochemical test indicated renal dysfunction  sheath catheter. A 4-Fr catheter was used to select the left internal iliac artery and two outflow vessels. As there was no evidence of rupture, the cardiologist deployed a 10-mm Amplatzer Vascular Plug (AVP) II in each outflow vessel and a 16-mm AVP II in the inflow vessel. Hemodynamic stability was maintained throughout the procedure.After successful completion of the endovascular repair, the patient underwent continuous renal replacement therapy. After 3 days, a urologist inserted a ureteral stent into the right ureter; however, he was not able to rescue the left ureter as the deformation was highly severe. The patient also had persistent abdominal distention and limited defecation. Ten days after the endovascular repair, he suddenly developed severe abdominal pain. Abdominal CT at this point revealed intestinal perforation. Emergency open surgery was recommended, and after obtaining written informed consent from the patient and his family, emergency surgery was performed by median laparotomy under general anesthesia. This revealed necrosis and perforation of the anterior wall of the rectum. Compression caused by the aneurysm, which was firmly adherent to the posterior wall of the rectum, had severely damaged the mesorectum. Therefore, the surgeons decided to resect the lesion; however, owing to strong adhesion between the aneurysm and the rectum, incising through the IIAA wall was deemed necessary . AlthougRenal replacement therapy proved unnecessary 5 days after the open surgery. Although a vesicorectal fistula developed 18 days after the open surgery , there w3Most IIAAs are associated with abdominal aortic aneurysms, making isolated IIAAs relatively rare . One revSurgical intervention has been universally recommended for IIAAs exceeding 30 mm in diameter because of the possibility of spontaneous rupture . In the In this case, we considered initial open surgical repair to be technically challenging and instead preferred endovascular repair. AVP is a versatile embolization agent that allows the operator to treat various conditions including highly challenging vascular lesions . DespitePrevious studies have suggested that endovascular repair does not result in rapid aneurysm shrinkage ,14. In aThe intraoperative findings in this case revealed that compression of the aneurysm caused the mesorectum to lose its structure; moreover, the aneurysm and the rectum were firmly adherent to each other. We therefore had to remove the aneurysm wall and residual hematoma. This procedure was successfully completed as endovascular repair had disrupted blood flow within the aneurysm. In addition to our case, even in the case of ruptured IIAA to the rectum reported by Kato et al. , an openThe limitation of this study is that it is a single-case report. Besides that reported in this case, it has also been reported that an IIAA after endovascular repair can form a fistula within the sigmoid colon ,17. Tana4We presented a case in which compression by a residual IIAA caused rectal necrosis 10 days after endovascular repair. Endovascular repair alone could not immediately release compression on the surrounding organs. However, open surgical removal of aneurysms after successful endovascular repair is considered a useful option for IIAAs with compression of surrounding organs. Further accumulation of cases, both successful and complicated, will be our best attempt for improving the treatment for huge IIAAs.This study did not receive any specific grant from funding agencies in the pubic, commercial, or not-for-profit sectors.This study was approved by the ethics committee in Nagoya Tokushukai General Hospital.Written informed consent was obtained from patient for publication of this case report.TI, TH and RM performed open surgery for rectal necrosis. AT performed endovascular repair. SK was responsible for urological examination. TI drafted the manuscript. ST and HK participated in the correction of the manuscript. All authors approved the final manuscript.No research study involved in this case report. Not applicable.Toru ImagamiNot commissioned, externally peer-reviewed.The authors have no conflict of interest to declare.


A case report of chronic ileocecal intussusceptions in 3-years old Sudanese boy diagnosed as abdominal Burkett's lymphoma as leading point, who presented to his local hospital severely wasted with prolonged abdominal symptoms. Ultra sound and computed tomographic scan of his abdomen and pelvis with oral contrast confirmed intussusception. He was referred to pediatric surgical department and underwent laparotomy confirming ileocecal intussusception with resection of gangrenous part of his large and small bowel with end-to-end anastomosis. Histopathology of resected part showed infiltration of small bowel with cells of Burkett's lymphoma. This case highlights the importance of considering chronic intussusception, though rare, as a cause of faltering growth in young children with prolonged abdominal symptoms. The multidisplinary approach was highly appreciated and the outcome was satisfactory. Intussusceptions, defined as insertion of proximal part of intestine into distal one, it is the second most common acute abdomen pathology after appendicitis . Acute ith centile for his age and sex. Abdominal examination revealed distended abdomen with visible peristalsis. Digital rectal examination revealed soft tissue mass 5 cm from the anal margin. He showed no response to nutritional supplementation. Complete blood count showed mild norm chromic anemia. Blood urea, serum keratinize and electrolytes were within the normal range. Abdominal and pelvic Ultra sound scan showed picture of intussusception with mesenteric lymph nodes enlargement. Computed tomography scan of his abdomen and pelvis with oral contrast showed large mass in the right iliac fossa indicating intussusception. Stool analysis showed hookworm infestation. He was screened for HIV, tuberculosis and celiac disease and all were excluded. Broad-spectrum antibiotics was started for suspected sepsis on admission. The patient was initially managed conservatively, given oral and injectable analgesics and nutritional supplementations with no or little response. Then referred to pediatric surgery after the confirmation of diagnosis and underwent laparotomy, eight cm of his terminal ileum, appendix and right hemi colon up to proximal part of his transverse colon was found to be gangrenous and was resected with end to end anastomosis. Histopathology of resected part showed infiltration of small bowel wall with starry sky patterned cells reaching serosal margins going with Burkitt's lymphoma. The boy did well post operatively, gain three-kilogram weight in the next four weeks. He was referred to the Radioisotopes center and subjected to further investigations for staging. Chemotherapy started with poor compliant was observed.A 3-years old male was admitted to his local hospital with history of vomiting associated with periods of constipation (for few days) followed by passage of formed stools or loose motions, those never contained blood. There was also colicky abdominal pain that referred to his penis with visible central bowel movements and abdominal distension that worsened with meals. He lost much of his weight although he was a healthy child before. He was exclusively breast fed only for the first six weeks of his life after which he depended mainly on boiled camel milk. Physical examination showed severely wasted boy with WHO z-scoring of his weight for length equal to-4SD, his length and head circumference on the 25Chronic intussusceptions (CI) are mostly present with episodes of non-specific abdominal pain of prolonged duration of more than two weeks. Around 3% of all reported CI cases occur in infant less than one year, while about 10% in those over one year of age7 as in our case. A striking feature of chronic intussusception is emaciation and a marked loss of weight, which could be attributed to prolonged anorexia and vomiting and some reported cases have been misdiagnosed and investigated thoroughly for malabsorption including our case . Due to This case highlights the importance of considering chronic intussusception, though rare, as a cause of faltering growth in young children with prolonged abdominal symptoms. The multidisplinary approach was highly appreciated and the outcome was satisfactory.The authors declare no competing interests.


To the Editor,Pneumolabyrinth is defined as the presence of air within the inner ear including cochlea, vestibule or semicircular canals. This condition is a rare complication of temporal bone fractures, barotrauma and surgeries, such as stapes surgery and cochlear implantation in patients with malformed cochlea . We do nTM electrode . The patient had severe vertigo after surgery in the early period. We suspected that the electrode was located in the scala vestibuli. Therefore, a computed tomography scan was conducted to check the electrode place postoperatively. The cochlear implant electrode was found in the cochlea. Air shadow was detected in the inner ear, including cochlea, vestibule and semicircular canals (A 34-year-old woman had progressive profound sensorineural hearing loss in both ears. The patient had no vestibular symptoms and had normal otologic examination. Cochlear implantation was performed on the left ear. The computed tomography scan indicated normal middle and inner ear structures with well pneumatized mastoid cells in both ears preoperatively . Althougr canals . The patr canals . The amoThe presence of the air in the cochlea and vestibule is typically observed in patients with large vestibular aqueduct syndrome and vestibular schwannoma after cochlear implantation ,3,4. PneIn conclusion, acute pneumolabyrinth should be considered when patients suffer from severe vertigo after cochlear implantation in the early postoperative period. Such condition could be due to non-malformed cochlea, and promontory cochloestomy is necessary. We should not drill the promontory deeply for cochleostomy and we should not cause possible basilar membrane damage to avoid pneumolabyrinth.


Primary hepatic tuberculosis is a rare presentation and sporadically reported in the literature, mostly from our part of the world. Sometimes the presentation can be atypical and mimics hepatic tumor and poses diagnostic challenge. We, herein, present a case of a 58-year-old man who presented to us with abdominal pain and weight loss. Raised serum alkaline phosphatase (ALP) and imaging raised a suspicion of gall bladder carcinoma with hepatic invasion. Peroperative frozen section revealed hepatic chronic granulomatous inflammation with caseous necrosis consistent with the diagnosis of hepatic tuberculosis. Surgery was postponed and he was put on antituberculous treatment. It is important to consider tuberculosis in the differential diagnosis of the space occupying lesion of liver in a patient with vague symptoms and abnormal findings on imaging. Tuberculosis (TB) is a worldwide health problem with particularly high prevalence in developing countries , 2. The sth; while complete blood count (CBC), renal function tests, coagulation profile and CA 19-9, 18.9u/ml  were within normal limits. Ultrasonography (US) of the abdomen revealed distended and irregular thick walled GB. Computed tomography (CT) of the abdomen revealed GB with irregularly thickened wall in the region of the fundus. There was an illness defined hypodense area extending from the body of GB to adjacent hepatic parenchyma measuring 3.0x1.5 cm and an enlarged lymph node at porta hepatis measuring 2.0x1.5 cm. The above constellation of findings raised a radiological suspicion of malignant lesion of GB  also returned to normal levels. He is still on treatment and on regular follow-up.A 58-year-old man presented to our hepatogastroenterology clinic with non-colicky pain in right hypochondrium and vomiting for 1 month. The pain was mild in intensity, dull, aching in nature, gradual in onset and progressive in course. It was non-radiating and not associated with meals. There was history of weight loss of 3 kg over a period of 1 month. There was no history of jaundice and tuberculosis or exposure to tuberculosis in the past. The general physical examination was unremarkable. There was no lymphadenopathy or abdominal visceromegaly. The laboratory investigations revealed raised ALP, 111 IU/L, Gamma-glutamyl transpeptidase (GGT), 61 IU/L and erythrocyte sedimentation rate (ESR), 70 mm/1on of GB . Bone scon of GB . The secHepatic TB as a part of disseminated TB is seen in 50-80% of cases. However, isolated or primary localized hepatic TB is very uncommon even in countries with high prevalence of the disease. The isolated liver TB without active pulmonary or miliary TB, or other clinical evidence of TB can easily be misdiagnosed in clinical practice, and only few cases have been reported in the literature . HepatobClassically, there are three patterns of hepatic involvement in TB i.e. miliary hepatic, nodular hepatic and biliary tract TB . The firet al., reported the sensitivity of mycobacterial antigen PCR up to 57% for hepatic granuloma [In our case, the patient presented with abdominal pain and weight loss. His ALP was raised. The imaging studies showed distended and irregularly thick walled GB on ultrasound, while contrast enhanced CT revealed thickening of the GB wall at the fundus region with a soft tissue density and reported as GB carcinoma by the radiologist. The definitive diagnosis of hepatic TB requires pathological and bacteriological evidence. Due to the low yield of culture and acid fast bacilli (AFB) staining, CT/US guided percutaneous biopsy or peroperative biopsy is the preferred approach for the diagnosis. Diaz Regarding management of the condition, most often surgical intervention is done due to pre-operative diagnosis of malignancy rather than tuberculosis. As in our case, on the basis of clinicoradiological diagnosis of GB carcinoma, the patient was planned for extended cholecystectomy. Peroperatively, we found multiple whitish spots on the surface of liver, whose frozen section revealed epithelioid granulomata with central necrosis. Two lymph nodes were also sent for histopathology, which revealed reactive changes. The frozen section histopathology spared the patient an unnecessary radical surgery.Once the diagnosis of hepatic TB is established, conventional ATT for 1 year will cure the disease nearly in 100% of cases. In biliary TB causing obstruction of the biliary tree, there may be a need for ERCP (Endoscopic Retrograde Cholangio-Pancreatography) to relieve the obstruction. Tuberculosis should be kept in the differential diagnosis of space occupying lesions of the liver, especially in endemic areas. Our patient has been given ATT and is on follow up; he was found to be afebrile and had recent weight gain of 4 kg in a period of 4 months of therapy.Isolated hepaticobililary TB is a rare entity with potentially curable outcome, but presents a diagnostic challenge due to its atypical presentation. It is necessary, to consider it in the differential diagnosis of space occupying lesions of the liver in endemic areas.The authors declare no competing interests.


Surgical correction was performed with resolution of the symptoms. MRI and CT are complementary modalities for evaluating this entity, the first being the method of choice for meningoencephalcele diagnosis although bone defects are best depicted on CT scan. CT cisternography identifies the specific site of leak and confirms benign intracranial hypertension, consistently reported in meningoencephaloceles. Obesity and benign intracranial hypertension have been reported as a combined mechanism allegedly contributing to meningoencephaloceles through congenital skull base defects, by increasing intraabdominal pressure thus decreasing venous return, with augmented intracranial pressure and subsequent reduced absorption of the CSF.Spontaneous transsphenoidal meningoencephalcele is a rare entity, even rarer through the Sternberg’s canal, a congenital defect on the lateral wall of the sphenoid sinus. We report such a case in an obese 52-year-old female with spontaneous cerebrospinal fluid (CSF) rhinorrhoea and recurrent meningitis. Brain CT, MRI and CT cisternography were performed. Surgical correction and short-term follow-up were recorded. CT scan showed a defect on the lateral wall of the right sphenoid sinus filled with a soft tissue mass extending to the nasal cavity. MRI scan revealed brain parenchyma from the right temporal lobe herniated through the sphenoid bone defect. CT cisternography showed 270 mmH A positive glucose test confirmed CSF rhinorrhoea.A 52-year-old female presented with an 8-year history of rhinorrhoea clinically consistent with cerebrospinal fluid (CSF) leakage and two episodes of meningitis. She also complained of mild headache worsening with flexion posture of the head, dizziness and, since last occurrance of meningitis, anosmia. No previous mild to severe head trauma or surgeries were reported. Other clinically relevant data were morbid obesity  showed a sphenoid sinus bony defect on its posterior and lateral aspects, medial to foramen rotundum, through which a soft tissue density mass could be observed, connecting the temporal fossa with the right sphenoid sinus and nasal cavity, protruding into its roof  and 2. I2O).MRI study performed  showed gliotic temporal brain parenchyma and a surrounding meningeal sac, without contrast enhancement, herniating through the previously identified sphenoid bone defect, with extension to the nasal cavity through the sphenoid ostium, consistent with meningoencephalocele of true transsphenoidal type . AdditioThe defect was corrected surgically , and the1 the last being the most common.2 The basal type, accounting for 10% of all meningoencephaloceles,3 includes transethmoidals, sphenoethmoidals, frontosphenoidals and finally those involving exclusively the sphenoid sinus—transsphenoidals, intrasphenoidals, sphenoidal lateral and transalar. Depending on whether the meningoencephalocele is contained by the sinus or surpasses its wall protruding into the nasal cavity it is classified as intrasphenoidal or transsphenoidal, respectively.4 They may be congenital or acquired,5 the latter being more common and often due to traumatic skull base fracture, rarer after a neurosurgical intervention. Congenital meningoencephaloceles may occur isolated or in association with other congenital defects when an error occurs early during neural tube formation, most commonly associated to frontoethmoidal type, accompanied by facial malformation, hypertelorism and nasal obstruction.7 When no underlying cause is identified they are referred to as spontaneous meningoencephaloceles.Meningoencephaloceles consist of cerebral parenchyma and meninges herniating into the extracranial compartment through a cranial defect. They are divided into three main groups according to the defect location: basal, sincipital or occipital,8 as the result of incomplete fusion of greater wings of the sphenoid bone with basisphenoid by the 50th gestational day.9 In adults, it can be patent or vestigial up to 4% and 30%, respectively.10 By definition and according to literature two locations are hypothesized, either medial to foramen rotundum, defended by Barañano in its thousand patients series,10 or lateral to it, as stated by Tomazic and Stammberger.11 To our knowledge to date only 36 cases of meningoencephaloceles through the Sternberg’s canal were reported,14 but fewer of true transsphenoidal type with nasal cavity extension. Some involving the sphenoid sinus have been related to laterally extensive pneumatization in large sinuses; therefore contact between the Sternberg’s canal and the lateral recess of the sphenoid sinus can occur.15The sphenoid sinus is rarely a site of meningoencephaloceles and even rarer through the Sternberg’s canal, a congenital anomaly also called lateral craniopharyngeal canal, first described by Maximilian Sternberg in 198816The most common clinical presentation of meningoencephaloceles includes CSF rhinorrhoea; however, this entity is sometimes misdiagnosed and meningitis can be the first alarming clinical sign. When recurrent meningitis occurs it is highly suspicious of a cranial defect.−2) females are consistent among previously published cases, many of them presenting with benign increased intracranial pressure and empty sella, as well as focal arachnoid granulations along the defect. An explanation could be the augmented intraabdominal and thoracic pressures leading to benign intracranial hypertension, obesity being the main risk factor.11,17 Another theoretical mechanism emphasizes the adipose tissue hormonal production in women, with relative hypoadrenalism inducing altered resistance to CSF dynamics and higher pressures at arachnoid villi, thus causing focal meningeal herniation.10 These mechanisms suggest the persistent benign intracranial hypertension upon an already weak structure of the cranium to be a relevant prognostic factor. Also, the altered CSF dynamics seems to be associated with empty or partially empty sella.5As in our case, middle-aged obese , which was completely solved after surgery. Also, there was no worsening on imaging suggesting hypertension . It is not clear which patients with evidence of elevated intracranial pressure may undergo isolated repair and which require the use of CSF diversion techniques, such as lumbar drainage, ventriculoperitoneal shunting and medical treatment;CSF spontaneous fistula should prompt investigation in order to exclude meningoencephaloceles.The true transsphenoidal type is rarely reported, particularly through the Sternberg’s canal.The clinical picture more often includes persistent CSF rhinorrhoea and recurrent meningitis. Obese middle-aged women seem to be more commonly affected and the suggested underlying mechanism hypothesizes benign intracranial hypertension to be a relevant factor.MRI seems to be the most valuable imaging modality for an accurate diagnosis, although both CT and MRI are complementary modalities, especially for surgical planning.We, the authors, confirm that a patient’s relative has signed an informed consent regarding the submission of the CT, MRI and pathology images without identification.


The giant multilocular prostatic cystadenoma is a very rare benign tumor of the prostate gland. It is composed of predominantly cystic enlarged prostatic glands in a fibrous stroma and spreads extensively into the pelvis. Because of the large size at the time of diagnosis, it is not always possible to determine the exact point of origin for these multilocular cystic neoplasms. Thus, diagnosis before histological examination of a surgical specimen is often difficult. Here, we present a case involving one of the largest giant multilocular prostatic cystadenomas reported in the literature and discuss preoperative diagnoses and appropriate surgical approaches for this rare retroperitoneal tumor.A 50-year-old man presented with a 2-year history of abdominal distension and lower urinary symptoms. Enhanced CT showed a large retroperitoneal mass with multiple septations in the pelvis and lower abdomen, measuring 30 cm in size, surrounding the rectum and displacing the bladder, prostate, and seminal vesicle to the right anterior side. MRI showed multiple cysts with a simple fluid appearance on T2-weighted images and enhanced solid components on gadolinium-enhanced fat-saturated T1-weighted images, suggesting the retroperitoneal mass as leiomyoma with cystic degeneration or perivascular epithelioid cell tumor. Biopsy of the mass showed a spindle cell tumor with focal smooth muscle differentiation. Differential diagnosis comprising leiomyoma, low-grade leiomyosarcoma, and perivascular epithelioid cell tumor was made. Complete resection of the tumor with low anterior resection of the rectum was performed. The tumor was solid with multilocular cavities containing blackish-brown fluid and measured 33 × 23 × 10 cm. Histologically, the tumor was composed of variously sized dilated glandular structures lined by prostatic epithelia surrounded by fibromuscular stroma. The prostatic nature of the lesions was confirmed by immunohistochemical staining of the epithelium for prostate-specific antigen. Thus, pathological diagnosis was a giant multilocular prostatic cystadenoma.We present our experiences with one of the largest giant multilocular prostatic cystadenomas. When a retroperitoneal huge lesion with locular cavities fills the pelvis in a male patient, the possibility of giant multilocular prostatic cystadenoma should be considered before planning for retroperitoneal tumor treatment. Retroperitoneal tumors often present diagnostic and therapeutic challenges. Although computed tomography (CT) and magnetic resonance imaging (MRI) may be useful for diagnosis and understanding the extent of disease, a definitive diagnosis can be established only by histopathologic analysis after surgery. Knowledge of the tumor characteristics may help to narrow down the differential diagnosis. Here, we report a case of a giant multilocular prostatic cystadenoma, an extremely rare benign neoplasm that originates in the prostate glands. Fewer than 30 cases have been reported thus far –21. Our A 50-year-old man presented with a 2-year history of abdominal distension. He also had lower urinary symptoms such as the sensation of incomplete voiding and increased frequency. He had no symptoms of bowel obstruction. Physical examination revealed a palpable mass occupying the lower abdomen up to the level of the navel, but there was no tenderness. Digital rectal examination revealed an elastic hard mass on the anterior side of the rectum without palpable intraluminal mass. Total colonoscopy showed no masses or stenosis in the rectum. We evaluated the urinary symptoms were due to the compression of the bladder by the tumor.The results of laboratory tests were normal. Serum prostate-specific antigen (PSA) was not available preoperatively. Urinalysis was normal, with no evidence of hematuria. Enhanced CT showed a large retroperitoneal mass measuring 30 cm in size with multiple septations surrounding the rectum and displacing the bladder, prostate, and seminal vesicle to the right anterior side Fig. . MRI shoAs the biopsy findings did not reveal obvious evidence of malignancy and because the outer wall of the tumor was relatively smooth, we chose to forego total pelvic exenteration and instead attempt complete macroscopic resection of the tumor with minimal combined resection of adjacent organs.Laparotomy was performed through a midline incision, which revealed a huge mass from the pelvic floor up to the level of the navel Fig. . During The gross pathologic specimen was a 33 × 23 × 10 cm solid mass containing multilocular cavities. Sectioning revealed a multicystic cut surface Fig. , and blaFinal histology indicated a giant multilocular prostatic cystadenoma.Postoperatively, whereas the patient developed a pelvic abscess due to urine leakage from the prostatic urethra, he recovered conservatively and was discharged on postoperative day 37. Since there is no positive evidence of adjuvant therapy of this prostatic cystadenoma, the patient is now under follow-up by blood tests and CT scan every 3–6 months.At 2 months after surgery, the patient had a PSA of 0.365 ng/ml, which was within the normal ranges. Since then, we have observed no signs of tumor recurrence. The patient was completely asymptomatic at the 6-month follow-up visit and made a full recovery, with no complaints of any urinary disorders or sexual dysfunction.A giant multilocular prostatic cystadenoma is an extremely rare benign neoplasm that originates in the prostate glands. In 1991, Maluf et al. became the first to describe and designate this rare entity ; since tClinical presentation and radiographic features are helpful for preoperative diagnosis. As shown in Table Imaging findings of retroperitoneal tumors are often overlapping, and the first step to understanding the tumors is to divide them into solid or cystic tumors. In addition, their content, precise localization, extent of local invasion, and vascularity may help to define a specific differential diagnosis . CT and Differential diagnoses of a multicystic retroperitoneal tumor can include liposarcoma, leiomyoma with cystic degeneration, lymphangioma, multilocular peritoneal inclusion cyst, phyllodes variant of atypical prostatic hyperplasia, prostatic abscess, and teratoma. Cysts of the lower male genitourinary tract are uncommon. There are also differential diagnoses for cystic lesions in the pelvis, including Müllerian cysts, utricle cysts, and seminal vesicle cysts. However, these are typically smaller than the giant multilocular prostatic cystadenoma and not “multilocular” .Although imaging studies may provide useful information about the extent of the lesion and invasion to adjacent organs, a clear and definitive diagnosis is possible only through histological means. Histologically, a giant multilocular prostatic cystadenoma is composed of multiple cysts in hypocellular fibrous stromal tissue and dense fibrous stroma that corresponds to a focal solid area. It is characterized by cuboidal cells lining the prostate glands and cyst, with positive immunohistochemical staining for PSA in epithelial cells . No prevTreatment for giant multilocular prostatic cystadenoma is complete surgical excision. However, adherence to surrounding structures often makes complete resection difficult, and clinical suspicion of malignancy may lead to unnecessarily aggressive surgery and no preservation of urogenital or digestive organs. Despite its benign nature, incomplete resection can result in recurrence , 12. To One recent report found that a gonadotropin-releasing hormone antagonist is effective for recurrent giant multilocular prostatic cystadenoma , and thiIn conclusion, when a retroperitoneal huge lesion with locular cavities fills the pelvis in men, giant multilocular prostatic cystadenoma should be considered as a differential diagnosis.


Lymphoma presenting as a scalp mass is a rare but serious medical condition mandating aggressive treatment and neurosurgical intervention. We report a case of 53-year-old male who presented with a large right sided frontal scalp mass and a smaller mass located on the left frontal scalp. After discussion with the patient, it was decided to resect the larger mass for definitive diagnosis. After subtotal resection of the mass, biopsy revealed WHO grade 1 follicular lymphoma (FL), diffuse pattern stage IV. The patient was subsequently treated with 4 grays (Gy) of palliative radiotherapy over 2 fractions to the right frontal scalp and systemic chemo-immunotherapy (6 cycles) followed by rituximab maintenance. Lumbar puncture to obtain cerebrospinal fluid was done a month after therapy began and the results were negative for spread of malignant cells. Approximately 3 months after initiation of therapy, PET/CT showed no evidence of active malignancy and MRI revealed a complete internal resolution of the enlarged right frontal scalp mass. We use this case to provide a detailed discussion regarding disease pathophysiology, early diagnosis, and management Non-Hodgkin’s lymphoma (NHL) is a broad class of malignant neoplasms originating from B-cell progenitors, T-cell progenitors, mature B-cells, mature T-cells, and in rare cases natural killer cells (NK-cells) . The secOur patient is a 53-year-old male who worked as a coal miner. In spring of 2017, he noticed enlarging scalp nodules and right hip pain. He also complained of fatigue but was otherwise asymptomatic. Because of the hip pain and nodules, he came to the emergency department where a CT scan showed an enlarged right frontal scalp mass that measured about 4 cm . MRI alsIGH (immunoglobulin heavy chain locus)-Bcl-2 fusion. These findings support the definitive diagnosis of follicular lymphoma, WHO grade 1, diffuse pattern stage IV.He was taken to the operating room where a right frontal scalp incision was performed for subtotal resection of likely metastatic cancer. The mass was primarily located within the right frontal scalp but extended through the skull into the right frontal cortex. It did not however appear to be involved with the brain parenchyma. The resection consisted of removing scalp mass and taking a core biopsy from the specimen. Histopathology of the biopsy showed a diffuse and monotonous infiltrate composed of small cells with irregular nuclei, condensed chromatin and inconspicuous nucleoli . On immuAfter definitive diagnosis was made, the patient was then referred to radiation oncology for follow-up. By time of presentation to radiation oncology, the patient was showing symptoms of lower extremity numbness, personality changes, and some memory loss. The radiation oncologist discussed the diagnosis with the patient and his family and came up with a treatment plan to combat the unusual presentation of follicular lymphoma with the intracranial extension from the calvarium. The treatment plan consisted of repeat MRI’s; local  XRT (4 Grays over two fractions) and systemic chemo-immunotherapy (6 cycles) followed by rituximab maintenance. Lumbar puncture was done a month after resolution of vasogenic edema to assess leptomeningeal spread later proven negative for malignant cells. MRI showed complete resolution of the right scalp mass post-radiotherapy and after the patient had already underwent 3 cycles of chemo-immunotherapy. PET/CT done approximately 3 months since therapy initiation showed no suspicion for abnormal hypermetabolic activity, which suggested the absence of active malignancy.In the United States, FL represents 35% of all NHL diagnoses with the majority being sporadic . Other fIGH enhancer, causing constitutive expression of the antiapoptotic protein BCL2 [Our patient was unique in that he presented with a rare type of FL that had spread to the scalp by time of symptom onset. An extensive case series of FL patients showed that of 93 cases only one patient had metastatic spread to the neck with none to the scalp highlighting the rarity of our case . The reaein BCL2 . Dissemiein BCL2 . The keyein BCL2 .In summary, our patient had an aggressive metastatic FL that spread to his scalp. While initially asymptomatic he quickly progressed to B symptoms. Surgical resection, radiation, chemotherapy, and rituximab were used successfully to manage the FL. 3 months since therapy the patient is doing well. The key points going forward are related to the need for future study. First, understanding FL pathophysiology is critical for developing disease classification criteria. Second, prospective studies are needed to map survival once scalp metastasis has occurred. Finally, experimental immune modulator therapies should be explored further as adjuvant treatment options. With these three approaches improved management for patients with aggressively expanding FLs can be obtained.


We present a case of a papillary tumour at the cerebellopontine angle in a 54-year-old man. He presented with right-sided ear pain associated with dizziness and hearing loss. The radiological diagnosis was in favor of acoustic neurinoma. Surgical excision was performed and the diagnosis of the endolymphatic sac tumour was made. Endolymphatic tumour is a low grade adenocarcinoma that originates from the endolymphatic sac. The definitive diagnosis requires a combination of clinical features, radiological finding and pathological correlation. Endolymphatic sac tumor (ELST) is a rare neoplasm with benign histopathological appearance and clinically destructive behavior which occurs in the skull base and frequently invades the posterior petrous bone, the mastoid and cerebellopontine angle (CPA). It is synonymous with Heffner tumor, low grade adenocarcinoma of endolymphatic sac origin, and aggressive papillary middle ear tumor according to the recently published World Health Organization tumor classification . It is eA 54-year-old man presented with a chief complaints of progressive hearing loss of approximately 4-year duration. In the two past months, the symptom increased accompanied by headaches, tinnitus and dizziness. Otological examination revealed hearing loss in his right ear associated with incomplete right-sided facial paralysis. There was no history of trauma or surgeries. The left ear was normal. Neither the symptoms nor a family history of VHL disease were found in the patient. On magnetic resonance imaging (MRI), a 3.2×4.1 cm sized multi-lobulated extra-axial mass was detected in the right CPA, compressing the cerebellum. The cranial nerve complexes VII and VIII could not be differentiated from the mass and the radiological diagnosis at that time was acoustic neurinoma . MicroscNeoplasm arising from the endolymphatic sac was first described by Hassard . SubsequThe majority of ELST are sporadic while around 15% of patients have an autosomal dominant inherited disorder, von Hippel-Lindau disease. Patients with VHL disease have a germline mutation in the VHL tumor suppressor gene that is responsible for their genetic susceptibility to various neoplasms. Apart from ELST, VHL disease is associated with haemangioblastoma of the central nervous system, choroid plexus papilloma, renal cell carcinoma, phaeochromocytoma and papillary cystadenoma of the epidydimis . Our patThe present case showed a similar appearance in magnetic resonance imaging. Concerning treatment of this tumor, radical surgical local excision remains the mainstay of current therapy. The best treatment choice is the total removal of the lesion, which may sometimes necessitate sacrifice of cranial nerves, because total resection of the advanced tumors may be impossible due to the anatomic complexity , so postELST should be taken into consideration for differential diagnosis of CPA tumors. Detailed clinical and radiographic evaluation is required to direct an appropriate management in every case. Radical excision is feasible using appropriate surgical approach. Early diagnosis, surgical excision and long-term regular follow-up may constitute an efficacious management.The authors declare no competing interests.


Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). We report a patient with this rare condition who was referred to radiology department for CT abdomen examination. A 40-year-old female patient presented with abdominal pain, distension, fever and diarrhea for the past 1 week. Antinuclear antibody was strongly positive showing speckled pattern. The double stranded DNA was positive. Clinically she gave history of joint pain and on examination oral ulcers and malar rash were present. Hence a definitive diagnosis of systemic lupus erythematosus was considered. CT abdomen was done which showed presence of bowel wall thickening, dilatation of intestinal segments, engorgement of mesenteric vessels, increased attenuation of mesenteric fat, ascites, peritoneal enhancement, retroperitoneal lymphadenopathy and target sign which is due to submucosal edema. In view of the patient's medical history of systemic lupus erythematosis (SLE) and the appearance of the bowel, lupus enteritis from vasculitis was diagnosed. Patient was treated with steroids and the patient subsequently improved. Mesenteric vasculitis is uncommon (2.2-9.7%) and highly lethal among gastrointestinal complications of SLE, if it is not carefully diagnosed and promptly treated. CT scanning has become the gold standard for diagnosis of lupus enteritis. The most frequent findings described were the bowel, mesenteric changes, ascites and retroperitoneal lymphadenopathy which our patient had. Lupus enteritis is a rare manifestation of the disease, but is important to suggest as the vasculitis requires control of the disease by high doses of steroids.


Aggressive pelvic angiomyxoma is a very rare mesenchymal tumor that is usually diagnosed in premenopausal female patients. The current mainly reported treatment is wide surgical excision. Other treatment options, such as radiotherapy and hormonal therapy, have been suggested as potential alternatives. A 61-year-old postmenopausal female patient presented with hematuria that led to the identification of a perirectal mass on abdominopelvic imaging. A 46-year-old female patient presented with a perineal mass of unknown etiology. Despite extensive investigations, the diagnosis could not be confirmed before surgical resection in both patients. Surgical excisions were performed and revealed the presence of an aggressive angiomyxoma with positive estrogen and progesterone tumoral receptors in both cases. Radiological and clinical recurrence was noted in one patient. Tumor regression was noted in this patient after treatment with a luteinizing hormone-releasing hormone (LHRH) agonist with long-term remission. The diagnosis of a perirectal aggressive angiomyxoma is an exceedingly rare event. Preoperative biopsy and pathological diagnosis are challenging and often yields poor results. Its slow growth and expression of hormonal receptors make noninvasive therapeutic strategies, such as radiotherapy, gonadotropin-releasing hormone agonists, or even watchful waiting, valid options in selected patients. Due to the lack of reported cases, the best treatment has yet to be elucidated. Aggressive angiomyxoma (AA) is a rare mesenchymal tumor usually found in the pelvis of young female patients -2. SteepCase 1A 61-year-old female patient was evaluated at our university hospital for a perirectal tumor of unknown origin. Her past medical history included dyslipidemia and non-neoplastic postmenopausal vaginal bleeding. She had undergone a hysterectomy and bilateral salpingo-oophorectomy two years prior to the actual episode. Her medication included hormone replacement therapy (HRT). The patient presented initially with macroscopic hematuria. A urological assessment, including a cystoscopy, did not reveal any identifiable cause for her complaint. An abdominopelvic computed tomography (CT) scan was performed as part of the investigation and showed a hypodense left perirectal mass with enhancing borders and ischiorectal extension Figure . An abscPelvic magnetic resonance imaging (MRI) revealed a left perirectal mass of 10.6 x 10.7 x 4.9 cm, which was in contact with the left posterolateral vaginal wall Figure . TransreA fine needle biopsy was performed but was inconclusive. A positron-emission tomography (PET) scan showed a mild hypermetabolic state in the mass, but it could not differentiate between a benign or malignant condition. No metastases were objectified. Our tumor board recommended a surgical resection, and the patient consequently underwent an open uncomplicated tumoral excision. The mass was not visible intraoperatively until the pouch of Douglas was opened. The rectum was left in place.The pathological examination found a myxoid tumor without atypia or significant mitotic activity. Expression of estrogen (ER) and progesterone receptors (PR) was positive. Histological and immunohistochemical (IHC) features were consistent with an AA with positive microscopic margins.The patient recovered uneventfully, except for a local pelvic abscess which was treated with antibiotics and percutaneous drainage. An abdominopelvic CT scan performed three months after the surgery showed no signs of recurrence.Case 2A 46-year-old healthy female patient was evaluated at our institution for a perirectal mass of unknown etiology. Her past medical history was unremarkable. The patient reported a perineal mass in the standing position. Her only other symptom consisted of mild occasional dysuria. Rectal and vaginal examinations were normal.An endovaginal ultrasound performed one year before the present events to assess a known voluminous fibroma made no mention of a pelvic mass. An ultrasound of the perineal soft tissues found a hypoechogenic and heterogeneous mass of 4 x 5 cm beneath the paramedian region of the buttock. Endoscopic ultrasound showed a pelvic heterogenous mass that did not seem to originate from the rectal wall. A pelvic MRI and scan were performed and revealed a tubular structure of 13 x 7.5 x 3.5 cm in the right parametrium. It was centered around the right adnexal region, crossing the pelvic floor, reaching the ischiorectal fossae, and continuing as digital extensions in the soft tissue of the buttock (Figure This unusual case was discussed at the institutional tumor board meeting and the decision was to proceed with surgical excision. The patient underwent an open surgical resection of the tumor. Due to a voluminous uterine fibroma that limited access to the pelvic cavity, an abdominal hysterectomy was performed. The pathological examination found a lesion with a proliferation of paucicellular spindle cells in a myxoid stroma and medium to thick-walled vessels of variable caliber. The stroma was without atypia. No mitoses or necrosis were identified. The histological aspect and immunohistochemical profile were consistent with the diagnosis of AA. The evaluation of the uterine specimen revealed two leiomyomas of 1.5 cm and 5.5 cm.The patient’s evolution was satisfactory one month after the surgery with no symptoms suggesting a possible recurrence. An MRI performed four months after the procedure showed a right perirectal longitudinal tissular density with a length of 6 cm Figure . ImagingThree months later, a repeat MRI found a considerable improvement with a sharp decline in the lesion’s extent, now measuring 3.1 x 2.3 x 1.6 cm. One year after the surgical excision, the lesion’s dimensions had further decreased to a length of 2.6 cm. An MRI 32 months after the surgery found a small residual centimetric lesion. After four years, an MRI revealed a thin image of residual fibrocicatricial tissue. After five and eight years, respectively, a repeat MRI showed no signs of recurrence and stability of a small residual local scar Figure .Herein, we present two very rare cases of perirectal AA incidentally diagnosed in a postmenopausal woman and in a premenopausal woman.The majority of AA cases in women are diagnosed in premenopausal patients . ReporteAlthough much scarcer, some cases of AA have been reported in male patients as paratesticular masses -6. In feAA is usually diagnosed in premenopausal patients. The positivity of tumoral hormonal receptors may influence its development in women of childbearing age. The diagnosis of AA in our first postmenopausal patient is very unusual. One may wonder if the HRT the patient was receiving might have influenced the tumor’s growth.Macroscopically, AA has been described as having both smooth and adherent margins that infiltrate the host’s tissues , 4. The The best treatment for AA remains unknown. Wide surgical excision is the main reported therapeutic approach , 8-10. ARadiation therapy is a potential option that has been described as an alternative to extensive resection in advanced disease or as an adjuvant treatment for recurrent tumors , 7-8. ItFollow-up after surgery includes clinical and radiological examinations . HoweverIn conclusion, surgical resection of AA seems to be a valid option, but the local and slow-growing features of this tumor make other noninvasive approaches reasonable in some patients. Nonetheless, note that the scarcity of data on the specific pathophysiology of AA and the inability to accurately predict its exact evolution pose serious challenges to the decision-making process. The perirectal location of AA poses an additional challenge to its diagnosis and treatment, as a surgical intervention may put several major anatomic pelvic structures at risk, especially since the role of surgical excision has been repeatedly challenged in favor of noninvasive alternatives.


Calcification of the lateral collateral ligament of the metacarpophalangeal thumb is a rare pathology. It’s may be due to a deposit of hydroxyapatite crystals. We report the case of a 36-year-old man with chronic pain of MCPP. X-ray and Ultrasound finds a calcium deposit of LCL. Acute calcific tendinitis (ACT), also known as peritendinitis or calcified periarthritis, is an acute inflammatory condition of unknown aetiology commonly occurring in the pericapsular region of the shoulder and less commonly in the hand or wrist. Calcification of the lateral collateral ligament (LCL) of the metacarpophalangeal thumb (MCPP) is a very rare pathology. This calcification may be due to a deposit of hydroxyapatite crystals falling within the framework of apatitic rheumatism of the hand. Through an observation of calcific periarthritis with apatite of the hand and the review of the literature, we present the clinical, radiological and therapeutic aspects of this pathology.A 36-year-old man with chronic pain of MCPP. The examination found an inflammatory appearance with oedema, redness and pain on palpation of LCL of MCPP. The mild mobilisation of MCPP was painless. The inflammatory and phosphocalcic balance was normal. Standard X-ray showed LPC calcification of MCPP . UltrasoPeriarticular calcifications with hydroxyapatite deposits were described in 1966 by Mac Carthy and Gatter  Uhthoff Hydroxyapatite calcifications affect men and women equally and are particularly common between 40 and 60 years of age [The disease is usually mono-articular and most often asymptomatic of chance discovery. They can be revealed by acute periarticular inflammation with intense pain preventing mobilisation of the joint and sometimes accompanied by swelling and general signs (fever) with non-specific biological inflammatory syndrome, and this for a few days . Rarely,Localizations of apatitic calcifications in the hand are rare. They sit near the tendon of the carpi-ulnar flexor muscle, in the vicinity of the pisiform bone.They also concern the tendon of the carpal radial flexor opposite the palmar face of the radiocarpal spacing. Hydroxyapatite deposits also have a predilection for the metacarpophalangeal and interphalangeal proximal joints of the fingers ,9.LCL of MCPP is a rare localisation of apatitic rheumatism.The diagnosis is established by a hand radiography showing the image of amorphous, homogeneous calcium density cluster. Their contours are smooth without cortical and their forms are often ovoid, sometimes triangular, rarely linear . These cComputed tomography is necessary, not for the positive diagnosis, but to specify the topography of calcification most often in a zone of tendinous insertion, and for the exploration of cortical erosions.MRI retains its place in the presence of such calcifications on standard radiographs to diagnose this entity and avoid imaging traps. In addition, it provides a precise description of the morphology and location of calcification and its effect on surrounding structures that may be useful in guiding therapeutic management .The main differential diagnoses to be eliminated are septic arthritis, gouty arthropathies, calcified circumscribed myositis, metabolic abnormalities such as hypervitaminosis D, chronic renal failure, hyperparathyroidism .Symptomatic treatment with nonsteroidal anti-inflammatory drugs and colchicine is sufficient for recovery. After some painful breakouts, these calcifications can disappear spontaneously. Their persistence indicates surgical excision.An appropriate diagnosis with a correct therapeutic behaviour is the only guarantors of the good results. Any periarticular pain or ligament insertion in the hand should encourage practitioners to request a standard radiological assessment for calcification in favour of an apatitic deposit.


We report a case of an intraabdominal desmoid tumor that occurred at a gastro-pancreatic lesion with spontaneous cystic features, and present the successful laparoscopic resection of the tumor.A 20-mm retroperitoneal cystic mass with a solid component was found adjacent to the stomach and pancreatic body in a 52-year-old woman with no history of familial adenomatous polyposis. Laparoscopic spleen-preserving distal pancreatectomy with wedge resection of the stomach was performed, and complete resection was achieved without intraoperative and postoperative complications. Histopathological examination by immunohistochemistry enabled diagnosis of a desmoid tumor that had originated from the stomach and invaded the pancreatic parenchyma with a spontaneous cystic change. We herein report an extremely rare case of an intraabdominal desmoid tumor with a spontaneous cystic change.Regardless of its rarity, desmoid tumor should be included in the preoperative differential diagnosis of a cystic intraabdominal mass, and laparoscopic function-preserving surgery may be an optimal treatment option. Desmoid tumors  are locally aggressive tumors with no potential for metastasis or dedifferentiation. Radiologically, they are usually non-specific, slow-growing solid masses that are difficult to distinguish from other soft tissue tumors, but they rarely show cystic lesions. Sporadic desmoid tumors can occur at any site in the body, and anatomic locations are classified into 3 regions: trunk/extremity, abdominal wall, and intraabdominal. Complete surgical resection is the only curative option, but intraabdominal desmoid tumors are often unresectable, because they often infiltrate the mesentery. Here, we report a case of an intraabdominal desmoid tumor that occurred at a gastro-pancreatic lesion with cystic features, and presents the successful laparoscopic resection of the tumor.An intraabdominal cystic mass was incidentally detected between the stomach and the pancreas in a 52-year-old woman undergoing postoperative follow-up computed tomography (CT) for chondrosarcoma of a rib. Her family history was unremarkable. Blood counts and serum biochemistry results were within normal limits. An abdominal CT scan revealed a 20 × 18-mm well-defined cystic mass with a solid component  gene [Desmoid tumors are rare, and their estimated incidence in the general population is 2–4 per million per year . Individli) gene .Desmoid tumors potentially show cystic changes with medical treatment or abscess formation , but a sThe mesentery and retroperitoneum are common anatomic locations of intraabdominal desmoid tumors. In the current case, the tumor originated from the stomach, and locally invaded the retroperitoneum and pancreas body, based on the histopathology of the resected specimen. Desmoid tumors can theoretically arise from any part of the body, but those arising from the stomach are extremely rare ; thus, mIntraabdominal desmoid tumors rarely metastasize, but may show local invasion and recurrence . The recIn conclusion, we encountered an extremely rare cystic desmoid tumor at a gastro-pancreatic retroperitoneal site that was difficult to diagnose preoperatively. Laparoscopic SPDP with wedge resection of the stomach was performed safely; such procedures could offer an optimal method to achieve both curability and preservation of function.


When patients present with transient loss of consciousness without headache and head computed tomography is performed, clinicians should pay attention to cortical high densities as convexity subarachnoid hemorrhage can be a differential diagnosis. A 91‐year‐old woman with a history of hypertension and diabetes mellitus presented to our emergency department with transient loss of consciousness. The patient was taking a bath alone and there was no witness. There was no sign of seizure when her family found her. She reported no headache or episode of trauma. Her blood pressure was 139/66 mm Hg, heart rate 90 bpm, body temperature 36.5°, and SpO2 97% (room air). The level was clear, and the symptom had disappeared before presentation. Physical examination revealed no neurologic finding. Her vital monitoring during examination kept sinus rhythm. The laboratory findings were significant for leukocytosis at 11 800/μL and hyperglycemia at 170 mg/dL. Head computed tomography (CT) revealed high densities and obscurations in left high‐convexity frontal sulci (Figure cSAH is rarely reported.None declared.SY: drafted the article. All authors participated in critical review and the revision of the article. All authors gave the final approval of the article. All authors have accountability for all aspects of the work.


Nasal septoplasty is a common procedure performed in plastic surgery and otorhinolaryngology. Many complications after septoplasty have been reported. Palatal perforation is one of the rarest complications with only a few cases reported in the literature. The reported cases had misdiagnosed submucousal cleft palate or high arched palate, but a patient with palatal perforation after septoplasty is presented here which have had neither evidence of submucousal cleft palate nor high arched palate. Nasal septoplasty is a common and low-risk procedure being performed in plastic surgery and otorhinolaryngology. Many complications after septoplasty have been reported. The most common complications are bleeding, adhesions, septal hematoma, saddle nose deformity and nasal septal perforation. Palatal perforation is one of the rarest complications. A few cases have been reported in the literature which regardless of their size and location they have various symptoms such as nasal regurgitation and nasal emission. The reported cases had misdiagnosed submucousal cleft palate or high arched palate.A 31 year-old female patient referred to our clinic due to a palatal perforation secondary to septorhinoplasty performed 2 years before, with minimal regurgitation and hypernasal speech. The patient had history of 2 failed fistula closure operations. On examination, a small perforation in the hard palate was observed . There w1-3 Palatal perforation is a very rare complication and literature review shows very few reported cases.4 Ersoy et al. reported a case of post-septoplasty palatal fistula that the diagnosis of submucous cleft palate as the underlying cause was made after septoplasty.4 Gökdemir and Bal reported a case similar to our case but, during the examination of the patient they figured out that his palatine vault was high and its concavity was more than normal.5Following septoplasty, many complications such as breathing difficulty, toxic shock syndrome, hemorrhage, vestibulitis, hematoma, adhesions, septal hematoma, septal perforation , etc. were reported.et al. also reported a case of the renal cell carcinoma, receiving biophosphanate treatment. The patient had sleep apnea and recurrent bleeding due to nasal polyp. Nasal surgery was performed for a nasal polyp and the patient developed palatal perforation after the surgery. It was postulated that the palatal fistula was caused by the osteonecrosis which developed due to the biophosphanate treatment.6 In the literature, it was emphasized that the local flaps could be sufficient for closure in small fistula, while in larger defects, a multidisciplinary approach should be used.7Balmor In our case, the palatal perforation developed as a complication after the septal surgery in an otherwise normal palate. In contrary to previous case reports no submucousal cleft palate or high arched palate were observed on midface CT scan of our patient. Aggressive and incautious osteotomy of septal bony part  resulted in this complication. To prevent such complications, we suggest to perform a precise and detailed examination prior to surgery to avoid such complications. Also, surgeons should be cautious in excising the bony part of septum and avoid uncontrolled osteotomy in all septoplasty patients, especially in high arched palate and submucousal cleft palate patients. Palatal perforation is a rare complication of septoplasty. Precise pre-operative examination of the patient and cautious gradual osteotomy under direct vision are key points to avoid this annoying complication.The authors declare no conflict of interest.


Chordomas are rare, slow-growing, and locally aggressive malignant neoplasms derived from primitive notochord remnants. The chondroid variety represents 14% of all chordomas mainly developing in the spheno-occipital region and presenting between the third and fifth decades of life. When developing intracranially, symptoms can range from headaches and neck pain to cranial nerve neuropathies and facial numbness. We illustrate a case of an adolescent woman who presented with excruciating facial pain, otalgia, decreased visual acuity, quadriparesis, headache, nausea, and dysphagia. Radiological studies revealed a large heterogeneous mass in the spheno-occipital region with clivus destruction. Biopsy and histopathology confirmed the diagnosis. Proper identification with prompt surgical resection and adjuvant radiotherapy remains critical to prevent complications. Chordomas are rare, slow-growing, and locally aggressive malignant neoplasms derived from primitive notochord remnants that develop longitudinally along the axial axis. They represent 1%-4% of all primary bone tumors, with 35% of cases presenting at the base of the skull. The chondroid variety represents 14% of all chordomas and is generally found in patients between the third and fifth decades of life , 5. BiopA 15-year-old female with no past medical history presented to the emergency department complaining of five months of severe pain in nasal region and associated cutaneous hypersensitivity, headache, nausea, dysphagia, otalgia, strabismus of left eye with decreased visual acuity, and quadriparesis. On physical exam, she presented with horizontal nystagmus, dysarthria, quadriparesis, and a palpable mass on the right side of the neck with neck stiffness. An MRI of the brain and neck revealed an enhanced broad and destructive mass in the infrasellar region with complete destruction of the clivus, C1-C2 infiltration with compression of the occipital foramen, and a displaced pons and medulla . Microscopically polygonal cells with hyperchromatic nuclei, fine cytoplasmic vacuoles, and chondroid tissue corresponding to phisaliferous cells constituting nests and lobules infiltrated and replaced the hyaline bone cartilage and part of the soft tissue Figure .The second procedure was performed one month later and consisted of maxillectomy Le-fort 1. Partial excision of residual tumor mass was successfully achieved. After three days of postoperative recovery, the patient showed signs of partial improvement. She was discharged to follow up with imaging monthly Figure .Chordomas are rare malignant neoplasms that develop from the primitive notochord and present as local, slowly infiltrative lesions. Chordomas can occur at any age but are usually seen in Caucasian adults between the ages of thirty and seventy years. Those located in the sacrococcygeal region constitute 50% of all cases and are typically seen in a slightly older age group with highest frequency within the fifth decade of life . In contThe most common locations involve the head and neck, specifically the clivus, spinal-occipital synchondrosis of the clivus, and sella turcica. Per histopathology and immunohistochemistry techniques, they can be further classified into classic, chondroid, and undifferentiated chordomas , 5, 13. Imaging studies such as MRI and CT of the skull are essential for proper diagnosis and surgical planning. Biopsy and immunohistochemistry contribute to final diagnosis.Chordomas appear as destructive lytic lesions at the base of the skull and soft tissue masses with occasional dystrophic calcifications -9. MRI sWhile chordomas are resistant to chemotherapy, the treatment of choice lies in complete surgical resection in blocks usually followed by adjuvant radiotherapy -8. Of thTreatment of intracranial chordomas can be restricted by their common location near sensitive structures such as the brain stem, optic nerve, optic chiasm, cochlea, pituitary gland, and temporal lobes , 14, 15.The probability of recurrence remains elevated even after total resection, with recurrence rate reported as high as 68% and associated with a poor prognosis. The natural history of chordomas remains unpredictable. The rate of distant metastasis is reported between 5% and 29%. Five-year survival rates vary from 51% to 82% according to histological grade and absence of positive surgical margins [Chordoma cases remain extremely rare in adolescents, as they tend to occur after the third decade of life. These tumors mostly manifest sporadically, hence posing a clinical challenge to general practitioners. Our main goal was to briefly review primary characteristics of chondroid chordomas, diagnostic tools needed, and management. Imaging studies, including MRI of the brain are essential for surgical planning, assessment of growth pattern, and integrity of critical anatomical landmarks. Chondroid chordomas usually have a better prognosis when compared to other types. Surgical resection followed by postoperative radiotherapy remains the treatment of choice.