Intradural extramedullary epidermoid (EC) cysts are uncommon . Acquired tumors appear more frequently as a late complication of lumbar punctures (40%). The authors present three cases of epidermoid cysts of the cauda equina which were surgically treated in their department during the past five years. All three had suffered lumbar punctures for rachianesthesia 6–9 years prior to their presentation. The patients' ages ranged between 19 and 31. Surgical treatment was deemed necessary because of the space–occupying nature of this slow–growing lesion, and this indication was supported by the MRI findings. Two–level laminectomy and microsurgical total tumor ablation were performed in all three cases. There were no postoperative complications. Epidermoid cysts (EC) are benign tumors which take the form of nests of cutaneous tissues that migrate improperly along the line of neurocutaneous ontogenetic differentiation during neural tube closure ,2,3. Thecauda equina). Being dermoid tumors, EC can appear in the spinal cord, subdurally, extramedullarly [Due to the slow growth of compact keratin, there is a slow onset of symptoms caused by the space–occupying EC to the detriment of neighbouring nervous tissue  . Their strong subcapsular wall is generated when the local squamous epithelium spirals into pearly–white strata of brittle keratin on the inside of the subcapsule , 7.There are two main types of EC: congenital and iatrogenic . CongeniCongenital EC can be suspected in case of occult or obvious defects in the closure of the rachis, in the absence of local trauma and in symptomatic patients under the age of 20. The iatrogenic hypothesis comes into play when patients report having suffered a lumbar puncture  for rachCT scans depict EC as homogenous encapsulated masses with variable small calcifications inside their capsules. In medullary–CT and cysternography, the contrast medium surrounds the cyst and gives the impression of total arrest on sagittal reconstructions.2 [On MRI images the tumor appears slightly hyperintense in TWe shall proceed to the presentation of the three cases of EC, operated on in Sf. Pantelimon Hospital, Clinic of Neurosurgery, within a six–year interval (November 1999 – June 2005).The patient had been suffering from lumbalgia, which gave in at rest for approximately three years. This evolved into bilateral, predominantly left, lombocruralgias and ‘saddle’ paresthesias during the last two months. During the final three weeks of these months, the patient also complained of a retention–type sphincter malfunction. Her medical history was notable for an appendectomy with rachianesthesia six years prior to the current presentation. ‘Saddle’ paresthesias and bilateral L3 hypoesthesia and urinary retention due to sphincter malfunction were demonstrated by the neurological exam. 1 and L2 vertebral bodies, with an isosignal with marrow in T1 and hypersignal in T2, situated posteriorly to the spinal cord.Paraclinically, frontal and lateral lumbar spine X–rays did not show Elberst–Dicke changes , scalloping or dysraphism. Lumbar spine MRI showed an intradural and extramedullar oval tumoral formation of 3/1.7/2.7 cm at the site of the L1 and L2 laminectomy, longitudinal sectioning of the dura matter under x 2.5 magnification, with temporary suspension and the complete ablation of an ellipsoidal tumoral formation, with a long axis of 3.2 cm and a small one of 1.7 cm. The formation had a whitish, relatively transparent capsule, extremely poorly vascularised and contained a pearly–white, friable core surrounded by concentric strata. The right anterolateral portion of the tumor adhered rather laxly to two of the equinal roots, due to the presence of arachnoiditis. These adherences were separated under magnification.Surgery consisted of LHistopathologically :There were no postoperative complications, which generally take the form of chemical meningitis, hemorrhage or CSF fistulae. 90% of the patient's neurological complaints were gone immediately after the surgery, and the rest of her complaints faded within three months.The patient presented with hemi–caudal syndrome with a slow, progressive onset of approximately 6 months, consisting of pain in the right leg, on the anterior aspect of the thigh and knee, with paresthesias in the same territory and retention–type sphincter malfunction–listed in their order of appearance. The patient's medical history was positive for one lumbar puncture performed for rachianesthesia prior to an appendectomy. 2 and L3 territories on the right and the retention of urine was objectified.Neurologically, the patient adopted an antalgic decubitus, Lasegue's sign was positive at 50 degrees of angulation, algic hyperesthesia was demonstrated at the L2 to T12. Native CT showed a well–circumscribed mass inside the spinal canal with a density of 42 UH (isodense), surrounded by the remains of the opaque post–myellography contrast medium with a relatively cystic aspect. The mass was strictly median and did not compress adjacent roots. It blended in with the surrounding structures and seemed to extend cranially to the T12 vertebral body. Lumbar spine MRI showed a similar tumoral formation placed behind the medullary cone, displacing it anteriorly.Paraclinically, no abnormalities were seen on frontal and plane spinal X–Rays. Lumbar myellography described an absence of contrast medium extending caudocranially from L12 which was unclearly visualized on the CT was in fact an arachnoid cyst which adhered to the superior pole of the tumor.Surgery was performed under x 2.5 magnification and consisted of total ablation of a 3.7/2.7/2.2 cm tumor. The extension towards TThe histopathological exam described a cystic formation surrounded by keratinized pluristratified squamous epithelium with a core of keratin plates.No meningeal reactions were noted postoperatively. The patient's recovery was spectacular, and her symptoms disappeared within approximately two weeks.3 area of both thighs for approximately two months, muscular weakness in the same territory and retention–type urinary sphincter malfunction for approximately two weeks.The patient presented with ‘balancing’ cruralgias in the LThe patient had a lumbar puncture for rachianesthesia for appendectomy nine years prior to our neurosurgical consultation.3 territory. Urinary sphincter malfunction with urinary retention was objectified.The objective neurological exam showed a limping patient with predominantly rhizomyellic paraparesis. Mingazzini's sign was positive bilaterally and there was bilateral hypoesthesia in the L2 and L3 vertebral bodies, with a hyposignal in T1 and hypersignal in T2. The mass was situated posterolaterally to the left, displacing the anterior and right lateral medullary cones, with a total extramedular arrest on myellography.Paraclinically, the frontal and lateral spine X–Rays were devoid of any abnormality. MRI of the lumbar spine showed an oval mass of 4.5/2 cm at the site of the Lcauda equina. Surgery followed a similar protocol to those mentioned above. The tumor was considerably larger in vivo (6.5/3/2.5 cm), and a small portion of the inflamed arachnoid could not be completely removed as it adhered closely to one of the roots of the There were no postoperative complications.Intradural extramedulary iatrogenic epidermoid cysts (EC) are more frequent in the lumbar region, due to the preferred site for lumbar punctures, and form approximately 40% of all spinal EC ,5. PerfoIn the three cases we have previously described, we observed that the bigger the size of the tumors the  greater the time passed between the lumbar puncture and surgery:Case 1: 6 years and 3.2/1.7/1.7 cmCase 2: 7 years and 3.7/2.7/2.2 cmCase 3: 9 years and 6.5/3/2.5 cm The slowly–evolving neurological symptoms associated with this condition tend to be unsystematic and generally gravitate towards the ‘superior caudal syndrome’, sometimes with added elements that suggest the compression of the medullary cone. If a cyst were to rupture in the preoperative phase, the symptoms would appear rather abruptly and would be caused by granulomatous meningitis appearing as a defensive immune reaction to the presence of keratin cells in the CSF ,20. 1 sequences and hyperdense in T2 sequences . Staged laminectomy on two levels, with or without slighting of the supra– and subjacent plates, was radiologically oriented towards the tumor  and revealed the tumor prior to the incision of the dura . After a median incision of the dura extending at least 1 cm superiorly and inferiorly to the tumor and temporary suspension, the patient is placed in the Trendelenburg position and two gauze pads are placed above and below the tumor to limit the loss of CSF and prevent further dissemination of the cyst's cells. After exposing the tumor, the separation of its adherences to the pia mater, arachnoid, medullary cone and the roots of the cauda equina and the subsequent ablation of the tumor were performed under x 2.5 magnification with microsurgical instruments. Total ablation is never possible  because of the aforementioned adherences. The danger of placing traction of nervous and vascular elements and thus causing microthromboses and postoperative neurological deficits was avoided by debulking the tumor . In order to avoid simultaneous dissemination of the components of the cyst, continuous  irrigation and aspiration of the intradural space between the two gauze pads was performed. The ablation was total in all three of the cases, although a small portion of inflamed arachnoid was left in place in Case 3, as it adhered closely to a surrounding nervous root.The region was closed by using 3.0 Ford interlocking sutures, and anatomic parietorraphy was performed after epidural drainage. There were no early or late intraoperative or postoperative complications .Dexamethasone (8mg/8h IV) was administered in all three cases preoperatively, Solu–medrol was given intraoperatively and Dexamethasone was continued postoperatively at every 12 hours. Cases 2 and 3 also received Cerebrolysin (10 mL x 2/ day). The patients were mobilized on the second postoperative day with the help of a kinetotherapist. Almost 90% of their symptoms receded in the early postoperative period, and the rest disappeared within three months from surgery at most.The three surgeries were performed in 1999, June 2004 and June 2005 and there have been no reported recurrences thus far. These recurrences usually appear between five and nine years after surgery ,23.Lumbar puncture may be associated with EC, but more patients are needed in order to support this hypothesis. The presence of at least one spinal tap in the patient's history ,17,23, aDifferences between the MRI–predicted and real size of the tumor may be due to the decompression of the tumor. During staged laminectomy, the opening of the dura and the release of pressurized CSF were noted as preliminary observations resulted from these 3 interventons.The ideal goal of the surgeries we performed was total microsurgical ablation of the EC, paying great attention to avoiding contact between the interior of the tumor and CSF (secondary meningitis).


A bezoar is a mass found trapped in the gastrointestinal system. The condition may be associated with pica, especially in developmentally retarded children. Clinical manifestations are usually nonspecific. Endoscopic diagnosis and removal of the foreign materials is often indicated. Occasionally, severe complications may occur. We report two cases to illustrate the clinical features and complications in these children. In the first case, a reliable history was not obtained in the developmentally delayed girl which precluded prompt diagnosis, but the grossly dilated stomach on plain abdominal radiograph gave clues to an underlying insidious mechanical obstruction of upper gastrointestinal tract. In the second case of a normal child, the unrelenting symptoms and weight loss prompt further investigations which revealed the diagnosis. Literature on pediatric bezoar is reviewed. Oesophagoduodenoscopy is the investigation of choice for diagnostic confirmation, but surgical facilities must be available to deal with acute complications. A bezoar  is a mass found trapped in the gastrointestinal system , though it can occur in other locations –4. A bezstreptococcus pneumoniae.A developmentally delayed girl from Mainland China was abandoned at the age of 3 years in the streets of Hong Kong and subsequently institutionalized. She had multiple abnormalities including surgery for presumed Hirsprung disease in Mainland China. She was confirmed to have 46XX del (11) chromosomal abnormality in Hong Kong. She resided at an institution, and reportedly, she had pica and chronic constipation. At age of 9 years, she was admitted with painless abdominal distension. Abdominal radiography showed dilated gastrium . The pathelicobacter pylori infection. Subsequently, a 16 cm long trichobezoar was removed piecemeal via gasrtrostomy following laparoscopy and minilaparotomy. The clinical course was complicated by abdominal wound dehiscence which necessitated delayed secondary suturing of wound one month later. Child psychiatry assessment revealed no evidence of psychiatric illness or social problems.A 9-year-old girl with good past health presented with unrelenting left-sided abdominal pain, nausea, and vomiting undigested foods (4-5 times per day) for 3 weeks. There were no symptoms of acid reflux, altered bowel motility, blood, or mucus in stool. Her body weight dropped from 27.3 kg to 25.8 kg (10–25% centile) over 6 weeks as a result of poor oral intake and vomiting. Left upper quadrant and epigastric fullness were noted. Blood tests showed iron deficiency anaemia with hemoglobin of 9.2 g/dL. Abdominal radiography showed nonspecific dilatation of gastrum and small bowel loop . OesophaMany cases of bezoars have been reported in children who are mentally retarded or having psychosocial problems –10. NeveA literature search using the keyword “bezoar” and with limits activated (humans and ages from birth to 18 years) was performed using PubMed in August 2013. Journal impact factor (IF) is in accordance with Journal Citation Report (JCR), a product of Thomson (ISI) Institute for Scientific Information. 635 publications were retrieved. The majority of these publications were single case reports with relatively low impact factors. With additional filter activated (reviews), 48 publications were obtained, most are case reports with review of the subject matter. With filter “clinical trials” activated, 3 publications were retrieved, but only one was relevant . The autBezoar is an easily missed diagnosis especially in the mentally retarded young patients , 10. SymOesophagogastroduodenoscopy is indicated for definitive diagnostic and therapeutic purposes –10. The Complications of bezoar and its treatment include weight loss, malnutrition, anemia, gastric ulcer, bowel obstruction, and surgical complications , 10, 13.In summary, bezoar should be considered as a diagnostic differential when a developmentally delayed child with history of pica presents with acute abdominal symptoms. The symptoms might be confused with gastroenteritis. A high index of suspicion with prompt diagnosis is pivotal to avoid unnecessary nonspecific treatment and delay in surgery for this disease. Oesophagogastroduodenoscopy allows definitive diagnosis to be made in a timely manner.


The syndrome of isosexual precocious puberty associated with primary malignant hepatic tumors is rare. All previously reported cases in the literature are old and prognosis was grim.We present the case of a 15-month-old Asian male baby who presented with precocious puberty associated with hepatoblastoma. Serum concentrations of alpha-fetoprotein and free testosterone were elevated, as was beta human chorionic gonadotropin hormone. He was treated with six courses of chemotherapy and underwent surgery. His surface markers as well as free testosterone level returned to normal during therapy. The child has now been off therapy for 18 months with no evidence of tumor recurrence at follow-up.Virilizing hepatoblastoma is rare and reported with poor outcome, but the development of new chemotherapeutic agents and complete surgical resection are promising. Hepatoblastoma is the most common pediatric hepatic tumor. Using the current modalities of treatment, non-metastatic hepatoblastoma usually carries a favorable prognosis if completely resected. The tumor typically secretes α-fetoprotein (AFP) which is a useful marker for management and follow-up. On rare occasions, hepatoblastoma is associated with beta human chorionic gonadotropin hormone (β-hCG) secretion .Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include thrombocytosis and increased alkaline phosphatase . Occasioth centile). At the age of eight months his mother noticed enlarging genitalia with sparse pubic hair and changes in his voice. These symptoms progressed over time and six months later, he started to have persistent fever and abdominal distension. A right upper quadrant mass was palpable at that time, so he was referred for evaluation. His family history was significant for an older sibling who died after being diagnosed with Wilms' tumor at the age of ten years and for a grandparent who died of lung cancer. There was no family history of overgrowth syndrome or other pediatric tumors. His parents were not related by blood.A 15-month-old Asian male baby presented to our center with precocious puberty and a hepatic mass. He was a product of cesarean section at 37 weeks of gestation. He had meconium aspiration at birth but had no history of hypoglycemia. His birth weight was 3.75 kg (in the 75th centile for age) and his weight was 12.45 kg (in the 95th centile). There was no evidence of hemihypertrophy or dysmorphic features. He had a large-for-age, uncircumcised penis that measured 10 cm from the base with a large thick scrotum and scarce suprapubic hair . An abdominal ultrasound revealed a right hepatic lobe mass. A computed tomography (CT) scan  and his weight was 14.2 kg (at 50th centile).Our patient was treated with four cycles of preoperative chemotherapy . The tumor showed a good partial response with more than 50% reduction in the size of the mass and a resolved IVC thrombus. Doppler US confirmed patency of the hepatic, portal and IVC veins. During therapy, we observed a decline in the level of free testosterone Table  with an Isosexual precocious puberty due to a virilizing hepatoblastoma is a rare but well documented occurrence ,3-15. CaTwo hypotheses explaining androgen secretion are suggested in the literature: ectopic testosterone secretion and secondary testosterone secretion. The first theory suggests excess secretion of testosterone by neoplastic cells , while tThe majority of the previous cases were reported more than two decades ago, making it difficult to judge the outcome of virilizing hepatoblastoma, taking into consideration the inferior quality of imaging techniques, surgical techniques and chemotherapeutic regimens. As a matter of fact, it was suggested that virilizing hepatoblastoma carried a worse outcome when compared to nonvirilizing tumors, alluding to a unique biological setup . Our patAlthough isosexual precocious puberty with hepatoblastoma is rare and carries a poor outcome (as reported in most cases), results due to developments in chemotherapeutic agents and complete surgical resection are promising.Written informed consent was obtained from the patient's legal guardian (father) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.UA interpreted the patient data and was a major contributor to the writing of the manuscript. IS and FAM collected the clinical data. FA obtained and interpreted radiological studies. UA and IS reviewed the literature. IS gave approval for the final manuscript. All authors read and approved the final manuscript.


The most common benign tumors of the mesenchyme are the lipomas. Benign fatty tumors can arise in any location in which fat is present. Fibrolipomas are characterised by fat modules. Most patients affected by such tumors are in the fifth or sixth decade of life. When very close to vital structures such as joints, they may cause functional limitations as well as pain. Osseous and chondroid metaplasia can infrequently manifest after chronic persistence. Given the rarity of this condition, a case of a big fibrolipoma of Hoffa's fat pad with osseous and cartilaginous metaplasia is reported. A 44-year-old woman presented with an enlarging soft mass on the right knee in the infrapatellar fat pad. After a thorough preoperative clinical and imaging examination, the mass was removed and sent to laboratory where the diagnosis was put. One year after surgery, both local and general condition of the patient were good and no signs of recurrence were found. The most common  benign tA 44-year-old woman presented with an enlarging soft mass on the right knee in infrapatellar fat pad; the mass had first been noticed 1 year ago. A gradual increase of the size of the mass had been observed by the patient during this year. Upon admission the mass could be easily seen on the right knee right below the patella in the infrapatellar region .Physical examination revealed a palpable mass in Hoffa's fat pad. Flexion of the knee appeared to be limited and pain also occurred when trying to mobilize the joint. The patient complained for these limitations especially during the last few months when the mass increased in size. A standard X-ray examination revealed osseous structure in the infrapatellar site .Furthermore, a CT, MRI, and a soft tissue ultrasonography were undertaken. According to the CT  and MRI Additionally, the ultrasound showed a well-defined mature adipose tissue with the overlying patellar tendon intact. The patient was then operated and a mass of 6 cm approximately in diameter was excised under general anaesthesia along with its pedicle base Figures  and 6.Histopathological evaluation revealed that the lesion was composed of mature adipocytes with myxoid areas as well as areas with osseous and chondroid metaplasia. The result of this histological examination suggested a fibrolipoma with osseous and cartilagenous metaplasia Figures  and 8.To date, one year after operation, both local and general condition of the patient are good, the knee has no function limitations or pain, and there are no signs of recurrence of the lesion. Followup will continue with clinical examination and imaging (radiographs and if necessary MRI or CT) annually.The aetiology of lipomas is unclear. They have been reported to be both sporadic and inherited , 11. Lip


Pericardial lipomas are rare and mostly asymptomatic tumors, which are usually detected incidentally during physical examination. The present study describes a case of giant pericardial lipoma that was diagnosed by surgical pathology. The study also describe the X-ray, magnetic resonance imaging, and the distinguish of the pericardial lipomas. The study also describes the ultrasonography, X-ray, computed tomography and magnetic resonance imaging findings of the tumor, and a review of the literature of cardiac lipoma, to help increase awareness of the tumor and selection of the correct imaging method for diagnosis. Pericardial lipomas are rare and mostly asymptomatic tumors, which are usually detected incidentally during physical examination . The preA 45-year-old female suffered from post-exercise pressure in the chest for one year. The patient was diagnosed at Henan Provincial People’s Hospital . An electrocardiogram revealed a normal sinus rhythm without any remarkable abnormality.3 and the left ventricular ejection fraction was 66%. Multi-detector computed tomography  plain scan revealed a large mass along the right, anterior and left epicardial surface. The density of the mass was equal to that of subcutaneous adipose tissue  revealed a marked enlargement of the cardiac silhouette without any sign of pulmonary congestion and the cardiothoracic ratio was 64% . A transe tissue . 3D reco3 in size. Histological examination revealed the nature of the tumor as mature adipose tissue with inflammatory infiltration in the lipocyte and envelope, confirming the diagnosis of lipoma (The patient underwent surgery to remove the mass under the diagnosis of epicardial lipoma. During surgery, a large, yellow, soft, encapsulated tumor was identified at the surface of the heart. The tumor had a 1-cm pedicle that was connected to the anterior exterior epicardial surface of the left ventricle, with no invasion to the myocardium and pericardium. The mass was completely removed, weighed 1,550 g and was 16×14×4 cmf lipoma .The patient was discharged on the tenth postoperative day and remained asymptomatic in the following three months. The patient has demonstrated no sign of recurrence by echocardiography during every three-month follow-up for approximately one year following the surgery .Primary cardiac lipomas are rare benign tumors, which account for 10% of all primary cardiac tumors and 14% of benign cardiac tumors . The tumGenerally, cardiac lipomas originate from the subendocardium, subpericardium or myocardium. A subendocardium lipoma may appear hemodynamically abnormal or display other symptoms depending on the location and the size. The tumor may cause valve regurgitation if the location is near the valve . SubperiIn the diagnosis of cardiac lipoma, X-ray alone is not sufficient to reach an exact diagnosis, as it is easily misdiagnosed. The anatomical definitions, including the location, size, activity and hemodynamic consequences of the tumor may be assessed by transthoracic echocardiography, which is usually the initial examination for patients with a suspected cardiac mass. Sonography is able to identify an echogenic mass that is similar to the adipose tissue. When the adipose tissue causes liquefactive necrosis, it may appear as a low-level echo in the mass .However, an accurate and reliable diagnosis must be obtained by CT  or MRI  with an Lipoma should be distinguished from liposarcoma. CT or MRI are good tools for a differential diagnosis . As for Pericardial lipoma should also be distinguished from mesothelioma of the pericardium, constrictive pericarditis, other secondary tumors of the pericardium, pericardial cysts and diaphragmatocele . MesotheIn summary, although pericardiac lipoma is rare and seldom encountered, it is not difficult to obtain a correct diagnosis using the typical imaging findings. The present case aimed to aid the diagnosis of a cardiac tumor and propose the initial examination method of ultrasound, location, morphology, size, activity, internal echo, the association with the surrounding tissues and the hemodynamic changes. The method also has the advantage that the technique is cheap and convenient. CT and MRI have more ideal tissue resolution and a broader visual field than ultrasound, and are preferred in the imaging of associations with the mediastinum, lungs, diaphragm and other surrounding tissues. However, CT and MRI are more expensive than ultrasound and ultrasound is more convenient for a bedside examination.


Isolated facial nerve palsy usually manifests as Bell's palsy. Lacunar infarct involving the lower pons is a rare cause of solitary infranuclear facial paralysis. The present unusual case is one in which the patient appeared to have Bell's palsy but turned out to have a pontine infarct.A 47-year-old Asian Indian man with a medical history of hypertension presented to our institution with nausea, vomiting, generalized weakness, facial droop, and slurred speech of 14 hours' duration. His physical examination revealed that he was conscious, lethargic, and had mildly slurred speech. His blood pressure was 216/142 mmHg. His neurologic examination showed that he had loss of left-sided forehead creases, inability to close his left eye, left facial muscle weakness, rightward deviation of the angle of the mouth on smiling, and loss of the left nasolabial fold. Afferent corneal reflexes were present bilaterally. MRI of the head was initially read as negative for acute stroke. Bell's palsy appeared less likely because of the acuity of his presentation, encephalopathy-like imaging, and hypertension. The MRI was re-evaluated with a neurologist's assistance, which revealed a tiny 4 mm infarct involving the left dorsal aspect of the pons. The final diagnosis was isolated facial nerve palsy due to lacunar infarct of dorsal pons and hypertensive encephalopathy.The facial nerve has a predominant motor component which supplies all muscles concerned with unilateral facial expression. Anatomic knowledge is crucial for clinical localization. Bell's palsy accounts for around 72% of facial palsies. Other causes such as tumors and pontine infarcts can also present as facial palsy. Isolated dorsal infarct presenting as isolated facial palsy is very rare. Our case emphasizes that isolated facial palsy should not always be attributed to Bell's palsy. It can be a presentation of a rare dorsal pontine infarct as observed in our patient. Isolated facial nerve palsy usually manifests as Bell's palsy, which is commonly described as an acute peripheral facial palsy of unknown cause . Bell's A 47-year-old Asian Indian man with a medical history of hypertension presented with nausea, vomiting, generalized weakness, facial droop, and slurred speech of 14 hours' duration. His physical examination revealed that he was conscious, lethargic, and had mildly slurred speech. His blood pressure was 216/142 mmHg. Tachycardia was present.His neurologic exam showed that he had loss of left-sided forehead creases, inability to close his left eye, left facial muscle weakness, rightward deviation of the angle of the mouth on smiling, and loss of the left nasolabial fold. His afferent corneal reflexes were present bilaterally. All but seven cranial nerves were intact.Computed tomography (CT) of the head without contrast enhancement and a CT angiogram of the head and neck showed no evidence of intra-cranial hemorrhage or ischemia. MRI of the head was initially read as negative for acute stroke. Bell's palsy was considered in the initial differential diagnosis but appeared less likely because of the acuity of the patient's presentation, encephalopathy-like imaging, and hypertension.The MRI was reevaluated with a neurologist's assistance, which revealed a tiny 4 mm focus of restricted diffusion involving the left dorsal aspect of the pons, consistent with a tiny ischemic infarct Figure .The final diagnosis was isolated facial nerve palsy due to lacunar infarction of the dorsal pons and hypertensive encephalopathy. A transesophageal echocardiogram showed an atrial septal defect with bi-directional shunt. The patient was kept in the intensive care unit for blood pressure management and was started on aspirin, heparin, and coumadin therapy because of the remote possibility of a paradoxical embolism. There was no evidence of deep vein thrombosis in his legs.The facial nerve is a mixed cranial nerve with a predominant motor component which supplies all muscles concerned with unilateral facial expression. Knowledge of its course is vital for anatomic localization and clinical correlation Figure  4]. Bel. Bel4]. Pontine infarcts form around 7% of all ischemic strokes, and isolated pontine strokes contribute to around 15% of all posterior circulation infarcts , with hyet al. [MRI diffusion-weighted imaging (DWI) has been shown to have an advantage over CT in the detection of acute ischemic stroke, but even DWI can lead to false-negative diagnoses within the first 24 hours after presentation. Such false-negative findings are more common in strokes involving the posterior circulation, including the brainstem. In a study of 139 stroke patients, Oppenheim et al.  found thet al. .Isolated dorsal pontine ischemia presenting as isolated facial palsy is very rare, and a review of the literature disclosed only one previously reported case . Our casCT: computed tomography; DWI: diffusion-weighted imaging; FLAIR: fluid-attenuated inversion recovery; MRI: magnetic resonance imaging.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.RA cared for the patient, did the literature search, and wrote the manuscript. LM and PS helped in the literature search and manuscript writing. BG was the attending physician and helped in the manuscript writing. All authors read and approved the final manuscript.


Desmoplastic fibroblastoma (collagenous fibroma) is a recently described tumor thought to arise predominantly from subcutaneous tissue or skeletal muscle. The natural evolution of this tumor on magnetic resonance imaging has never been described, to the best of our knowledge. We herein report a case of desmoplastic fibroblastoma arising in the thigh and show the longitudinal magnetic resonance imaging findings.A 60-year-old Japanese man presented with swelling of the medial side of his right thigh, and he complained of nighttime pain and slight tenderness. Magnetic resonance imaging demonstrated a 4 × 4 cm mass in the right thigh. Open biopsy was performed. The mass was diagnosed histologically as a benign fibrous tumor, and we maintained follow-up without surgical therapy. After one year, magnetic resonance imaging showed an increase in tumor size to 4 × 5 cm, but the histologic findings were the same as those obtained one year earlier. Resection was performed with narrow surgical margins. Pathologic diagnosis was desmoplastic fibroblastoma. Two years after surgery, the patient is free from pain and shows no signs or symptoms of recurrence.The natural evolution of desmoplastic fibroblastoma is characterized by no changes in patterns on magnetic resonance imaging despite increasing size. This finding is clinically helpful for distinguishing desmoplastic fibroblastoma with increasing pain from the desmoid tumor. Palpation disclosed a hard, well-circumscribed, mobile tumor. Otherwise healthy, he had no history of trauma to the thigh. The results of routine laboratory studies were normal.MRI demonstrated a 4 × 4 cm mass in the right thigh, occupying a space between the vastus medialis, sartorius and semimembranosus muscles Figure . Open biPathologically, the tumor showed well-circumscribed borders and low vascularity Figure . SpindleAt the most recent follow-up examination two years after surgery, the patient was free from pain and showed no signs or symptoms of recurrence.desmoplastic fibroblastoma when he described seven patients having masses with distinct morphologic characteristics. Subsequently, Nielsen et al. [collagenous fibroma, arguing that desmoplastic fibroblastoma misleads the reader into believing that the lesion consists of immature tumor cells inducing a desmoplastic response in host tissues.Evans  used then et al.  reportedThese neoplasms typically occur in the fifth or sixth decades of life and occur two and a half times more often in men than women. Clinically, desmoplastic fibroblastoma presents as a firm, mobile, slowly growing mass located in subcutaneous tissue or near the deep aspect of skeletal muscles. Common locations include the arm, shoulder, posterior neck and upper back -8. Few pPrevious authors recommended treatment of desmoplastic fibroblastoma by total surgical excision. No tumor recurrence during follow-up has been reported, including the present case.On MRI, T1-weighted images of the mass depicted a mixture of low SI and isointensity. With T2-weighting, the mass showed scattered high-SI areas within a zone of low SI. Contrast T1-weighted images demonstrated inhomogeneous enhancement after contrast administration, and nonenhanced areas corresponding to regions showing low SI on noncontrast T1-weighted images. The size of the tumor increased over the course of one year, but the MRI findings of the tumor showed no changes.Several reports have described the MRI appearance of this tumor, but in only three cases have MRI findings been described in detail ,4, and tIn previous reports, the relationship between MRI findings and histologic findings has been described. On T2-weighted images, low-SI areas correspond to abundant collagen fibers ,4,9, andThese reports indicate that MRI findings vary among and within individual tumors because of the variable cellularity.Evans  suggesteet al. [On the other hand, Marco et al.  reportedet al. [Details of imaging studies of desmoid tumors have been well documented in previous reports -13, and et al.  reportedet al. [The natural evolution of desmoid tumor has been documented , but thaet al.  describeThis case study has described the MRI findings of desmoplastic fibroblastoma in relation to its natural evolution. The natural evolution of desmoplastic fibroblastoma is characterized by no change in patterns on MRI despite increasing size. This finding is clinically helpful for distinguishing desmoplastic fibroblastoma with increasing pain from desmoid tumor.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.YK drafted the manuscript and reviewed the literature. UA supervised treatment of the patient, carried out the patient's surgery and revised the manuscript. MM performed the histopathological analysis and helped draft the manuscript. HM, TM, KM, HY and TY helped draft the manuscript. All authors have read and approved the final manuscript.


Abundant mucin deposition is an unusual finding for melanocytic lesions. This notion, however, should be toned down since mucin may be present in distinct melanocytic lesions. Indeed, following the first description of mucin depositions in neural differentiated naevi by Maize and Ackerman in 1987 In 2003, Rongioletti and Innocenzi described the first two examples of sclerosing blue naevi with an abundant mucinous stroma. Herein, we describe the third case of this rare entity. It should be noted, that particularly this uncommon variant of a blue naevus is easily mistaken as desmoplastic-neurotropic melanoma in which the present stromal deposition of mucin is a more typical finding.A specimen from the right lower leg of a 53-year old man was referred to our dermatopathology department with the clinical diagnosis of a dermatofibroma. Clinically, the tumor appeared as a brown lesion with a regular and sharp border.The histopathological work up revealed a symmetrical, well circumscribed papule; the fibro-mucinous stroma contained spindle-shaped and dendritic melanocytes, which sometimes were arranged in whorls . OrderlyThe cause of mucin deposition in melanocytic lesions remains elusive. It was attributed to a partial involution of the respective lesions. Interestingly, the dermoscopy of the presented sclerotic blue nevus revealed a hypopigmented center; a notion which has been described before.8With the present case report we like to raise the awareness for this rare entity because of the momentous differential diagnosis. Further reports of such cases would help to learn more about its clinical, dermoscopic and histological features.


Radiofrequency ablation (RFA) therapy is recognised as a safe and effective treatment option for osteoid osteoma. This case report describes a 27-year-old man who underwent computed tomography (CT)-guided percutaneous RFA for a femoral osteoid osteoma, which was diagnosed based on his clinical presentation and CT findings. The patient developed worsening symptoms complicated by osteomyelitis after the procedure. His clinical progression and subsequent MRI findings had led to a revised diagnosis of a Brodie’s abscess, which was further supported by the eventual resolution of his symptoms following a combination of antibiotics treatment and surgical irrigations. This case report illustrates the unusual MRI features of osteomyelitis mimicking soft tissue tumours following RFA of a misdiagnosed Brodie’s abscess and highlights the importance of a confirmatory histopathological diagnosis for an osteoid osteoma prior to treatment. Brodie’s abscess, a localised subacute or chronic osteomyelitis  is a greA 27-year-old previously healthy man presented with one-month history of left thigh pain which was worse at night. There was deep tenderness at his upper left thigh but no swelling or skin changes. Computed tomography (CT) revealed a small calcified nidus surrounded by a radiolucent rim within the thickened anterior cortex in the shaft of the left femur, with periosteal reaction . The patThree weeks later, he developed progressive left thigh pain with erythema and induration over the quadriceps despite anti-inflammatory medications and physiotherapy. Radiographs demonstrated periosteal reaction over the site of previous RFA , which rHe underwent an open biopsy and surgical irrigation. Pockets of blood-stained soft tissue were discovered along the femoral shaft. These corresponded to the intramuscular masses seen on MRI and were proven to be granulation tissue on histopathological examination. There was inflammation of the adjacent muscles but no abscess collection. His symptoms improved following the surgical irrigation and oral Cloxacillin therapy. Over the subsequent months, he developed several episodes of recurrent left thigh swelling with purulent sinus discharge and was treated with multiple courses of oral antibiotics. His symptoms eventually resolved after a second surgical irrigation and an extended course of Cefoperazone.Follow-up MRI performed four months after the second surgery demonstrated a small area of low T1signal, high T2 and high STIR signal within the marrow cavity, which was enhanced post-gadolinium, and was consistent with residual marrow oedema . There wOsteomyelitis is known as the great masquerader. It mimics various benign and malignant conditions, especially in the subacute state. Up to 90% of cases are initially misdiagnosed, with a mean delay of 3 months to the correct diagnosis, with 50% of them wrongly diagnosed as tumours [First described by Sir Benjamin Brodie in 1832 [Radiographically, Brodie’s abscess is typically a lucent medullary lesion varying in size from less than 1 cm to more than 4 cm in diameter with marginal sclerosis and cortical thickening in the diaphysis or metaphysis of the long bones of the lower extremities .A wide range of anatomical and radiographic variation has been described in both Brodie’s abscess and osteoid osteoma , with maet al. classified Brodie’s abscess based on its anatomic location and morphology which simulates other lesions [Gledhill and Roberts  lesions , 3. A ty lesions . A type  lesions . A type et al. described an enhancing penumbra of marginally higher signal intensity than the abscess cavity but of lower signal intensity than the fatty marrow on T1-weighted images of a Brodie’s abscess [This patient did not have an MRI prior to RFA. MRI can be of use in distinguishing tumour from infection. Grey  abscess . A “targ abscess . From th abscess . These M abscess , 5. The  abscess .The treatment for a Brodie’s abscess and osteoid osteoma is different. For Brodie’s abscess, surgery often offers rapid symptom relief, as in this patient . For ostA diagnosis of osteoid osteoma was made in this patient based on the age of the patient, symptoms and imaging features of the lesion, the absence of a history of trauma, local or systemic symptoms or signs of infection .Distinguishing neoplasm from osteomyelitis in general can be challenging. Osseous destruction, aggressive periosteal reaction and enhancing intra-osseous or extra-osseous granulation tissue may be demonstrated in both. In osteomyelitis, cortical destruction tends to be more focal and is commonly associated with exuberant and infiltrative soft tissue enhancement which represents oedema and granulation tissue. In bone tumour, cortical destruction tends to be more diffuse and is often associated with less dominant soft tissue oedema. The appearance of intra-osseous or juxta-cortical soft tissue abscesses, though not seen in all, are almost diagnostic of an infective process. In the absence of a pathological fracture, loculated fluid collections are unusual in bone tumour .The differential diagnoses of intramuscular mass lesions on MRI include abscess, haematoma, tumour, myositis ossifican, sarcoidosis, parasitic infection and focal myositis . GranulaIn summary, this case report illustrated a young man who was suffering from a type III Brodie’s abscess in the left femoral diaphysis which was misdiagnosed as an osteoid osteoma and underwent RFA which resulted in an unusual MRI appearance of osteomyelitis, with well-circumscribed, mass-like granulation tissue. This case highlighted the importance of a confirmatory histopathological diagnosis in cases of clinically and radiologically suspected osteoid osteoma prior to the initiation of treatment. As the old dictum emphasises “culture every tumour, biopsy every infection” , a wrong


We report a case of mucinous cystic neoplasm of pancreas with sarcomatous stroma metastasizing to the liver. The tumor occurred in a male patient aged 46 years. Symptoms included persistent epigastric and right upper quadrant pain. Radiographically, the pancreas contained four large cystic masses located in the neck, body, and tail. Histologically, the cysts were lined with benign, mucinous epithelium with underlying bland, storiform, ovarian-like stroma. An undifferentiated focally hyalinized, sarcomatous stroma composed of bland spindle cells showing short fascicular growth pattern and focal nuclear palisading was associated with the epithelial component in one of the cysts. These cells showed strong immunoreactivity with vimentin and inhibin (weak), they were negative for CD34, estrogen receptor, progesterone receptor, androgen, calretinin, S-100, CD117, melan A, chromogranin, and synaptophysin. A morphologically and immunohistochemically identical metastatic sarcomatous focus was identified in the liver without any glandular component. This case is unique in its clinically malignant behaviour and metastatic nature despite its morphologically benign epithelial and stromal components. Mucinous cystic neoplasms (MCNs) represent one of the major cystic neoplasms of the pancreas. They have distinct clinicopathological features. They are seen in women in the fifth or sixth decades of life; only rarely are examples documented in men. The tumor is usually located in the tail of the pancreas. Macroscopically, the cysts are multilocular and they do not communicate with the pancreatic ductal system. Histologically, the lining epithelium consists of tall, columnar cells with abundant apical mucin, although cuboidal cells that lack obvious mucin may also be present. The epithelium may be bland in appearance or might range from mild to severe atypia resembling ovarian counterparts and include mucinous cystadenomas, borderline mucinous cystic tumors, and mucinous cystadenocarcinomas. Another distinct histologic feature is the presence of subepithelial hypercellular, ovarian-like spindle cell stroma, and its presence has become a requirement for the diagnosis of this entity ,2. Here A 46-year-old man presented with persistent epigastric and right upper quadrant pain. Endoscopic ultrasound studies revealed multiple cysts in the pancreas Figures A,B. CompA partial pancreatectomy removed four cysts ranging in size from 1.5 × 1.5 × 0.8 cm to 7.0 × 5.0 × 3.5 cm, located in the neck, body, and tail of the pancreas. All the cysts had a green-tan to red, smooth, glistening lining. The parenchyma separating the cysts was tan-yellow and focally indurated Figure A.Histologically, the cysts were lined with columnar cells with mucinous laden cytoplasm and basally located, bland nucleus. Beneath the epithelial lining, a hypercellular ovarian-like stroma composed of spindle-shaped cells in a storiform growth pattern was observed Figure B. In somThe epithelial component of the cysts was positive for CAM 5.2 antibody Figure A. The stSarcomatous neoplastic lesions arising from the stroma of MCNs of the pancreas have been described in the literature Table . The stret al. [In three cases reported by Wenig et al. ,12, stroOur case differs from the previously reported literature by distant metastasis of only the stromal component, despite its morphologically benign features with bland spindle cells showing mild atypia, a low number of mitotic counts and a low Ki67 index. Both the sarcomatous stroma in the pancreas and the metastatic foci in the liver were strongly positive for vimentin and variably positive for inhibin. Estrogen receptor and progesterone receptor expression was not detected; this is not unlikely in MCN of pancreas arising in men.To the best of our knowledge, this is the first clinically MCN of the pancreas reported with distant metastasis of sarcomatous stromal component despite the relatively benign histologic features of the lesion. Although both epithelial and stromal components of the lesion are morphologically benign, the presence of a metastatic stromal focus in the liver renews the discussion that all pancreatic MCNs should be regarded as potentially malignant and that histology alone is not a definitive prospective indicator of behaviour ,13. AccoThe patient has given his consent for this case report to be published.H & E: Hematoxylin and eosin; MCN: Mucinous cystic neoplasm; MFH: Malignant fibrous histiocytoma.The authors declare that they have no competing interests.M W performed the surgery and completed the final paper. D G and V G are pathologists who evaluated the specimen. G A-gastroenterologist who diagnosed the tumor and helped in article writing. B A article writing and literature review. All author read and approved the final manuscript.


Imaging appearance of cyst-like changes is most frequently described in primary neuroendocrine lesions, especially pancreatic NETs.The imaging finding of a pseudocystic lesion of the liver puts in differential diagnosis many pathologies such as infectious diseases, simple biliary cysts up to biliary cystadenomas and eventually to primary or metastatic malignancies.Primary or metastatic hepatic malignancies with pseudocystic aspects are rare, and a pseudocystic aspect is reported only after neo-adjuvant treatment.Liver metastasis of untreated neuroendocrine tumors are usually solid and, to our knowledge, only two cases of neuroendocrine cystic hepatic metastases of ileal atypical carcinoids have been reported so far.We present a case of a 67 years old man with synchronous finding of an untreated hepatic pseudocystic lesion and an ileal mass histologically diagnosed as a well differentiated (G1) neuroendocrine tumor.http://www.diagnosticpathology.diagnomx.eu/vs/1443883503102967.The virtual slides for this article can be found here:  Gastro-entero-pancreatic neuroendocrine tumors (NETs) are uncommon, accounting for around 2% of all gastrointestinal tumors -3, and aThis family of heterogeneous neoplasms is thought to derive from the gastrointestinal diffuse endocrine system, and includes functioning tumors, which secrete a variety of peptide hormones with the resulting clinical syndromes, and non-functioning tumors. The lattest are often metastatic at the time of diagnosis. Even if the majority of NETs are well-differentiated, low-grade tumors, others may show an aggressive, frankly malignant behavior. Histopathological grading of these lesions has been recently reviewed: the 2010 WHO classification acknowledges and emphasizes the malignant potential of neuroendocrine neoplasms . AccordiKi67 labeling index cut off of 3% allows the division of NETs in well-differentiated and moderately differentiated and it predicts metatasis or recurrence .The most frequent site of metastases of gastrointestinal NETs, apart from regional lymphnodes, is the liver: hepatic metastases are found at the time of diagnosis in up to 40% of ileal and 80% of caecal lesions . FurtherAn aggressive surgical management of neuroendocrine hepatic metastases has been demonstrated to improve 5-years survival rates , hence tLiver metastases are usually solid with a dense capillary network; thereby, computerized tomography (CT) and magnetic resonance (MR) scans reveal hypervascularization with arterial phase enhancement.A small minority of hepatic NET metastases have a cystic appearance at standard cross-sectional scans, and may be mistaken for benign lesions. Cystic changes, due to central tumor necrosis, are described in NET hepatic metastases as a result of chemotherapic treatment.A primary cystic appearance is exceedingly rare in untreated cases. At the best of our knowledge, only two other cases of cystic hepatic metastases of untreated ileal NETs have been reported ,13.We present a case of a 67 years old man with synchronous finding of a hepatic pseudocystic lesion and an ileal mass, histologically diagnosed as a well differentiated (G1) NET.A 67 years old man was referred to our attention for an incidental finding  of four hepatic lesions. He had a history of hypertension, ischemic cardiomyopathy, chronic obstructive pulmonary disease and prostatic nodular hyperplasia.Ultrasonography showed that three lesions, in segments III and VII, were solid, less than 2 cm in diameter; the fourth one, in segment VII, was a 9 cm multilocular cyst with mixed echostructure (hyperechogenic with fluid content).An abdominal contrast-enhanced CT scan Figure  confirmeThe cyst showed a portal-phase enhancement in walls and inner septa. Moreover, a 3 cm thickening of terminal ileum wall, and an enlarged (cm 1.5) mesenteric lymphnode were highlighted.A pan-colonoscopy confirmed the ileo-caecal mass. Histological examination of the endoscopic biopsy specimens was consistent with a well differentiated (G1) NET. Immunostaining for both Synaptophysin and Chromogranin A turned out positive. Afterwards, a fine-needle biopsy of a III segment nodular lesion was performed: histological diagnosis was of well-differentiated (G1) NET hepatic metastasis.111In-pentetreotide somatostatin receptor scintigraphy, which displayed intestinal and plurifocal hepatic hypercaptation , as well as 5-indolacetic acid urinary excretion . Clinical staging was concluded with a The patient underwent a synchronous right hemicolectomy, left hepatic lobectomy, VII segment resection and cholecystectomy with concurrent radiofrequency ablation of the subcentimetric hepatic nodules. Postoperative course was regular, and a medical regimen of somatostatin analogs (SSAs) was started.Gross examination of the resected specimens revealed a 2.5 cm peduncolated mass in the terminal ileum, a 14 cm thick-wall pseudocystic lesion with bloody content in VII hepatic segment Figure , and sevMicroscopically, the ileal tumor was composed of rounded nests of closely packed, monomorphous cells lacking atypia, and with a peripheral-palisading pattern of growth. The tumor exhibited infiltration of perivisceral adipose tissue, along with two mesenteric nodal metastases Figure A. The psMitotic count was lower than 2/10 HPF, and Ki67 proliferative index  turned out 1% in both primary and metastatic lesions. All the described lesions showed a positive reaction to Synaptophysin  and Chromogranin A .Moreover, immunohistochemical stainings for Insulin , Glucagon , VIP  and Serotonin  were performed: only Serotonin stained weakly positive in the ileal mass and in the cystic lesion Figure C and 4D.According to the 2010 World Health Organization (WHO) Classification of Tumors of the Digestive System , the finThe patient was discharged without complications after fifteen days of recovery. Nine months later he is in good health, with no evidence of residual disease and under a medical regimen of long-acting SSAs.Hepatic neoplasms with cystic features are rare .The imaging finding of a pseudocystic lesion of the liver, in absence of clinical symptoms, implies several differential diagnoses, ranging from infectious diseases such as echinococcal cysts , benign Two cases of pseudocystic GEP/NETs liver metastases ,13 and tHowever, this is the first case of a G1 NET that provoked pseudocystic hepatic metastasis. Hepatic pseudocystic secondarisms are also reported from uterine cervical carcinoma  and nasoNeuroendocrine tumors can undergo pseudocystic metastases in different sites: a cerebral cystic metastasis of neuroendocrine carcinoma, with unknown primary tumor, has been reported .Cystic changes are often reported in liver malignancies after a neo-adjuvant treatment. This is thought to be due to therapy-induced tumor necrosis .A pseudocystic appearance with a peculiar peripheral contrast enhancement are described in pancreatic NETs  and in aPrimary cystic NETs display a less frequent tendency to undergo nodal and hepatic spread when compared to solid counterparts.We observed an aggressive behaviour, with multiple hepatic and nodal metastases in a well differentiated (G1) ileal NET at the time of diagnosis.111In-pentetreotide somatostatin receptor scintigraphy revealed the same capitation pattern in the hepatic cyst, suggesting its secondary nature. Taking account on these acknowledgements, a complete resection of the cyst was performed. Neo-adjuvant chemotherapy was not performed, despite the advanced clinical stage [In our case, once the neuroendocrine nature of the primary ileal mass was histologically confirmed, the al stage .Both macroscopic and microscopic examination showed that the pseudocystic appearance of this metastasis was not due to the central necrosis, but to intratumoral haemorrhage.All the described findings were incidental, and patient did not complain for pain, flushing, diarrhea or other symptoms related to a ‘’hypersecretion syndrome”. For this reason, the hepatic pseudocyst could have been misdiagnosed, unless an ileal mass was identificated and raised the suspicion for a metastatic NET.A complete resection of NET hepatic metastases leads to an improved prognostic outcome: 5-years overall survival rates higher than 70%  are repoWritten informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors read approved the manuscript. SFiori, ADG and FC analysed, interpreted the patient’s data and drafted the manuscript. GG performed immunohistochemistry. LC and SR revised the clinical data and supervised the case report. SF and SB revised the pathology data and supervised the case report. All authors read and approved the final manuscript.


Adenomatoid odontogenic tumor (AOT)-a benign (hamartomatous) lesion of odontogenic origin-is an uncommon tumor which affects young individuals with a female predominance, mainly in the second decade. This lesion is most commonly located in the anterior maxilla and is usually associated with an impacted canine tooth. This is a case report of a 39-year-old female patient presented with a large AOT of the posterior maxilla associated with an impacted second molar – a very rare situation. In 1969,et al. indicatiThis is a report of a large follicular AOT associated with a dentigerous cyst in the posterior maxilla in association with an impacted second molar- a very rare occurrence, which was mistaken for a dentigerous cyst clinically and radiographically.A 39-year-old female patient reported to my dental clinic with a chief complaint of pain and swelling over left side of upper jaw of 1month duration with mobility of her upper last tooth. On examination of the patient, she had a diffuse extra-oral swelling measuring approximately 1.5×1.5cm extending from left infraorbital region superiorly to left corner of mouth, obliterating the nasolabial crease. There was no paraesthesia over infraorbital region. Intraoral examination revealed a soft fluctuant swelling on left side maxilla extending from left upper premolar to third molar region, obliterating the buccal vestibule. Left upper first and second molars were missing, left upper third molar showed grade 3 mobility. Mucosa overlying the swelling was normal. On aspiration, straw-colored fluid was obtained. Orthopantomogram was taken which revealed a well-defined, unilocular corticated radiolucency extending from left upper first premolar to third molar region, associated with an impacted second molar and involving the left maxillary antrum Figures . On the The patient underwent surgery under general anesthesia. A mucoperiosteal flap was raised from the left canine to third molar region in the buccal vestibule. The buccal cortex was resorbed completely with thin shells of bone in between. The lining of the cystic lesion was carefully separated from the mucoperiosteam and the lesion was enucleated along with the impacted second molar and the mobile third molar Figures  and 4. THistopathological examination revealed thin nonkeratinized lining epithelium and shows proliferation into the lumen with spindle cells and calcifications . The spiAdenomatoid odontogenic tumor is a slow growing lesion, constituting only 3% of all odontogenic tumors and 0.1%et al.[et al.[18et al. reported a case of AOT associated with dentigerous cyst. The tumor in association with a dentigerous cyst is reported to occur in the anterior maxilla and other areas of the jaw such as the angle of the mandible, and in our case – the posterior maxilla.Radiographically, this lesion usually surrounds an unerupted tooth and is seen as a corticate radiolucency with small radiopacities, but there are cases where the lesion has no radiopaque component, as in our case, and in such a case – a dentigerous cyst is the preferred differential diagnosis. However, an AOT often appears to envelop the crown as well as the root as shown in the picture of our case, where we divided the specimen to show the relation of the lesion to the tooth; unlike the dentigerous cyst which does not envelop the roots.14–16 Wheet al.18 report[et al.18 describeTherefore, dental laminar remnants could probably be the progenitor cells for this benign odontogenic tumor. According to this hypothesis, the lesion grows next to or into a nearby dental follicle leading to the “envelopmental theory”.20 In our2et al.., a percentage similar to that recently indicated by Leon et al.[Immunohistochemical studies report that the slow growth, its benign character and low tendency to recur are clearly related to the low cellular proliferation observed on carrying out immunostaining for the Ki67 antigen. Only 2-3% of components of tumor demonstrated reactivity for Ki67 in a study done by Francisco Jose Vera Sempere on et al. This poson et al.Much of the above data  would seem to reinforce the idea of the hamartomatous character of thin infrequent form of benign, odontogenic tumor, as previously indicated by other authors, and indicating that this is probably not a true neoplastic growth. Given itThis is a case report of AOT in association with a dentigerous cyst in the posterior maxilla associated with an impacted second molar, which is extremely rare. The article also provides a review of literature on the clinical, histopathological, radiographical details and immunohistochemical studies of the lesion.


Keratocystic odontogenic tumour (KCOT) is a benign cystic intraosseous tumour of odontogenic origin that is usually solitary except when syndromic. It rarely occurs in the maxilla; therefore a rapidly progressive, nonsyndromic bimaxillary KCOT with locoregional extension poses significant diagnostic and management challenges. To the best of the authors' knowledge, documentation of a nonsyndromic bimaxillary KCOT is nonexistent in the English literature. We therefore present the case of an extensive bimaxillary KCOT in a 38-year-old Nigerian male. Phillipsen was the first to report the entity odontogenic keratocyst (OKC) in 1956 . It alsoKCOT typically occurs in the 2nd to 3rd decades of life with a slight male predilection . KCOT grKCOT lacks pathognomonic clinical and radiologic features and thus can mimic cystic, inflammatory, and neoplastic lesions affecting the jaw. These make clinical diagnosis challenging. The most common location in the jaw is the posterior mandible with occasional occurrence in the maxilla , 9. TreaWe therefore report the case of a massive bimaxillary KCOT which proved challenging in its clinical diagnosis and management.A 38-year-old male presented to our clinic on account of a diffuse bilateral maxillary swelling of 3 years duration. The swelling had progressively increased in size from a peanut-sized growth in the upper right quadrant to involve the right infraorbital region resulting in epiphora but without visual disturbances. Two years after the onset of the right maxillary swelling, he noticed a painless left maxillary swelling. The growth of both swellings resulted in mobility of adjacent teeth and recurrent infection evidenced by pus discharge. His medical history was noncontributory. Clinical examination revealed a nodular projection covered by darkly pigmented skin over the right maxillary swelling and a central area of ulceration over the left maxillary swelling . There wClinical impressions were a deep mycotic infection, a non-Hodgkin's lymphoma, or an epithelial malignancy originating from the maxillary sinuses. Radiology, histology, and microbiology investigations were conducted. Craniofacial computerized tomography revealed complete destruction of the maxillae, palate, nasal septum, and nasal bone by a mass occupying the whole of the maxillae and maxillary antrum Figures  and 3. TSurgical decompression was carried out under general anaesthesia using nasotracheal intubation. A modified Trotter-Weber Ferguson incision was made in the right maxillary region, while a Caldwell-Luc incision was made in the left maxilla to expose the lesion. The exposed cavities contained masses of friable necrotic tissue that were curetted and sent for histology. The cavities were well irrigated with normal saline and packed with dry sterile gauze. After shielding adjacent vital structures with Sofra-Tulle dressing, chemical cautery of the tumour bed was achieved with Carnoy's solution. The cavities were then packed with argotone soaked gauze to allow for proper haemostasis; this was left in place for 3 days. The patient had regular saline irrigation and dressing, initially every other day and after discharge on a weekly basis. Perceptible reduction in tumour size was achieved after a month of review, but complete resolution of the mass was not achieved before the patient defaulted from review visits and was lost to follow up.Appropriate therapy for any disease first requires correct diagnosis, which is formed by a combination of clinical details with or without supporting investigations. Our patient's pattern and site of presentation for KCOT were quite unusual, leading to a myriad of confusing clinical opinions and it was only following histologic examination that a working diagnosis of KCOT was reached. Unlike other jaw cysts with unremarkable histological features, the histology of the KCOT is rather unique and even though clinical features may not be clearly defined, the histology is distinctive. It is typified by a parakeratinized or orthokeratinized stratified squamous epithelial lining (6–8 cell layers thick) which is corrugated, a prominent and palisaded basal layer which may be cuboidal or columnar, lumen containing keratin, and a connective tissue wall without inflammation as well as absent rete pegs , 13. TheAlthough there is no universally accepted treatment yet for KCOT, the primary aim of treatment is to achieve total eradication utilizing an appropriate technique, taking into consideration site, size, location, invasion of the surrounding tissues, and previous treatments , 16. TreStudies have shown that decompression and marsupialization relieves the pressure within the cavity of KCOT leading to a reduction in size and formation of new bone . FollowiRecurrence rates following decompression and marsupialization of KCOT vary and recent studies have reported recurrence rates of 0%–18% , 18–20. Strength. To the authors' knowledge this is the first case report, in the English literature, of a bimaxillary OKC that is not syndrome associated. Characteristically, multiple KCOT of the jaws is associated with the nevus basal cell carcinoma syndrome [syndrome , 25; ourLimitation. A more comprehensive follow-up process would have improved knowledge about the tumour biology and its response to the adopted treatment protocol, but the patient's right to decline sustained treatment is acknowledged.The perplexing clinical presentation of a bimaxillary tumour and its challenging therapeutic intervention has been described for the benefit of awareness and early suspicion.


Peritoneal lipomatosis is a rare disease in childhood with only two cases previously described in children. We report a further case of a 12-year-old boy diagnosed with peritoneal lipomatosis. His main symptoms were abdominal pain, alternating bowel habit, abdominal distension, and melaena. His diagnostic work up included an abdominal MRI, wireless capsule endoscopy and single-balloon enteroscopy. Peritoneal lipomatosis although rare can be diagnosed in childhood. It is a benign clinical entity with variable manifestations. Lipomatosis of the peritoneum is a rare disorder with less than 50 cases published to date . There aA two-year-old boy initially presented to his local hospital prominent gastrocolic reflex, bloating, and intermittent changing shape of his abdomen. At the age of 4.5 years he had an abdominal U/S due to ongoing symptoms, which was suggestive of peritoneal lipomatosis. As he continued to complain about abdominal pain, urgency and alternating bowel habit he was referred for further evaluation to our specialist centre at 12 years of age. At the time other symptoms were noted, including intermittent abdominal distention and occasional blood in the stools. Radiological imaging was initially done by MRI scan which revealed widespread peritoneal lipomatosis, encasing the intraperitoneal contents with the lipomatous tissue lying anterior to the liver and stomach. It extended between the right lobe of liver and right kidney and down to the peritoneal cavity into the pelvis. The lipomatosis displaced the bowel loops centrally. The liver spleen, kidneys, and pancreas appeared normal Figures . As the Lipomatosis is a distinct clinicopathologic entity characterized. By the development of nonencapsulated lipomas in subcutaneous tissues . LipomasPelvic, abdominal, and intestinal lipomatosis are distinct clinical entities. They are characterised by abdominal distension as a consequence of intraperitoneal and retroperitoneal fat and respiratory distress due to mediastinal airway compression . Pelvic Our patient is the first reported case of peritoneal lipomatosis investigated by panenteroscopy using WCE and enteroscopy. The patient suffered from altered stool habit, occasional blood in stools, pain, and abdominal distension. Despite panendoscopic examination and pangastrointestinal tract biopsy assessment, no mucosal pathology could be identified, and despite extensive radiological and blood work up his symptoms could not be explained. Therefore, by the elimination of all other causes, we attributed the symptoms to direct mass effects of the lipomatosis. Peritoneal lipomatosis would be responsible for the mechanical symptoms of distension, causing obstruction intermittently and altering bowel transit time. Nevertheless, this would not explain the symptom of blood, albeit a very occasional symptom at best reported by the family.This we believe is the first WCE description of this condition with the addition of the rarely performed SBE that examined the entire small bowel of this child along with histological specimens excluding infiltration of the intestinal wall by the lesions.Our case should not be misinterpreted with intestinal lipomatosis, which is the presence of numerous circumscribed lipomas of the intestine . Less th


Torsion of the gallbladder is an uncommon condition that may present as an acute abdomen. Its preoperative diagnosis can often be challenging due to its variable presentation, with specific sonographic signs seen infrequently. We describe, to our knowledge, the first case of torsion of a wandering gallbladder following a colonoscopy in a 69-year-old female who presented with acute abdominal pain after procedure. This was discovered intraoperatively, and after a subsequent cholecystectomy, she had an uncomplicated recovery. Torsion of the gallbladder is an uncommon condition that may present as an acute abdomen. Its preoperative diagnosis can often be challenging due to its variable presentation, with specific sonographic signs seen infrequently. We present the case of a 69-year-old female who presented with an acute abdomen, three hours after colonoscopy. To our best knowledge this is the first published case of torsion of a gallbladder caused by colonoscopy.A 69-year-old female presented to our surgical unit with acute onset epigastric pain and nausea three hours after an elective colonoscopy. The colonoscopy was performed to investigate altered bowel habit and left iliac fossa pain. No cause for the pain was demonstrated, but a long redundant, tortuous, and highly spastic colon with some haemorrhoids was noted. Physical examination revealed mild tenderness in the right upper quadrant. Initial investigations included normal liver function tests and inflammatory markers. A chest radiograph did not demonstrate any free gas under the diaphragm. The patient's pain worsened markedly over a 12-hour period of observation. Computerized tomography (CT) of the abdomen was obtained which showed a grossly distended gallbladder and stomach with hepatic duct dilatation, but no cholelithiasis . Transabμmol/L, alanine transaminase 68 (NR = 0–55) units/L, alkaline phosphatase 76 (NR = 35–110) units/L, and gamma-glutamyl transferase 47 (NR = 0–50) units/L) and serum C-reactive protein was elevated to 177 mg/L. A presumptive diagnosis of acute acalculous cholecystitis was made, and the patient proceeded to theatre for a laparoscopic cholecystectomy. Repeat liver function tests demonstrated a new cholestatic picture (bilirubin 49 (NR = 0–20) At laparoscopy, a gangrenous hypermobile gallbladder was seen. This was found to be completely free from the liver and connected to the biliary tree by a cystic duct within a narrow mesentery around which it had undergone 360° of torsion . The galIntraoperative cholangiography demonstrated dilated intrahepatic ducts and common hepatic duct down to the level of the cystic duct and gallbladder. Cholecstectomy was performed without difficulty after the cystic duct and vessels were isolated, clipped, and divided. Histopathological examination of the specimen confirmed acute gangrenous cholecystitis and the absence of cholelithiasis. The patient had an uncomplicated recovery and was discharged after two days. Over 300 cases of gallbladder torsion have been reported in the literature since its initial description in 1898 , but theThe earliest description of torsion of a wandering gallbladder was by Ziegler in 1952 , and fewTo our knowledge, this is the first reported case of wandering gallbladder torsion occurring in the context of colonoscopy. Although causality between colonoscopy and gallbladder torsion cannot be proven, the development of acute right-upper-quadrant pain just hours after colonoscopy leads us to postulate that the colonoscope mechanically rotated the gallbladder during the procedure, which lead to the torsion of the wandering gallbladder. It is also conceivable that gas insufflation of the colon may have resulted in torsion of a hypermobile gallbladder. Interestingly, the colonoscopy was originally performed to investigate long-standing intermittent abdominal pain, perhaps due to intermittent incomplete torsion of the wandering gallbladder. In conclusion, torsion of the gallbladder is a rare but important cause of the acute abdomen. Preoperative identification of gallbladder torsion is challenging, but specific ultrasound findings can be used to assist with diagnosis. Management of the condition is relatively easy with laparoscopic cholecystectomy.


Oncologic patients who are extreme responders to molecularly targeted therapy provide an important opportunity to better understand the biologic basis of response and, in turn, inform clinical decision making. Malignant neoplasms with an uncertain histologic and immunohistochemical characterization present challenges both on initial diagnostic workups and then later in management, as current treatment algorithms are based on a morphologic diagnosis. Herein, we report a case of a difficult to characterize sarcoma-like lesion for which genomic profiling with clinical next generation sequencing (NGS) identified the molecular underpinnings of arrested progression(stable disease) under combination targeted therapy within a phase I clinical trial.Genomic profiling with clinical next generation sequencing was performed on the FoundationOne™ platform . Histopathology and immunohistochemical studies were performed in the Department of Pathology, MD Anderson Cancer Center . Treatment was administered in the context of a phase I clinical trial ClinicalTrials.gov Identifier: (NCT01187199).KIAA1549-BRAF gene fusion resulting from a tandem duplication event, a homozygous deletion of PTEN, and frameshift insertion/deletions in CDKN2A A68fs*51, SUFU E283fs*3, and MAP3K1 N325fs*3. The patient had a 25% reduction in tumor (RECIST v1.1) following combination therapy consisting of sorafenib, temsirolimus, and bevazicumab within a phase I clinical trial.The histology of the tumor was that of a spindle cell neoplasm, grade 2 by FNCLCC standards. Immunohistochemical staining was positive for S100 and CD34. Genomic profiling identified the following alterations: a KIAA1549-BRAF and PTEN loss within a spindle cell neoplasm, as revealed by genomic profiling based on NGS. This is the first report of a tumor driven by a KIAA1549-BRAF fusion responding to sorafenib-based combination therapy.The patient responded to combination targeted therapy that fortuitously targeted  KIAA1549-BRAF fusion in a PTEN null background as a driving genomic alteration susceptible to targeted therapy.Tumors that defy ready histopathologic characterization are difficult to rationally approach in the setting of treatment algorithms which are based on the anatomic origin of the neoplasm. Spindle cell neoplasms are such tumors that defy characterization even with ancillary methods such as immunohistochemistry. In such cases, genomic profiling based on next generation sequencing (NGS) can identify the genomic alterations (GA) that drive oncogenesis and thus prospectively suggest aberrantly activated pathways for pharmacologic inhibition independent of tumor site origin. Conversely, in the case of exceptional responders to targeted therapy, diagnostic genomic profiling may uncover the linked underlying alterations retrospectively. We report one such case of a patient with a malignant spindle cell neoplasm who exhibited clinical and radiographic improvement with simultaneous RAF kinase inhibition (sorafenib/Nexavar®), mTOR inhibition (temsirolimus/Torisel®) and VEGF (bevacizumab/Avastin®) targeted therapy who had been refractory to standard cyototoxic chemotherapy. A Clinical Laboratory Improvement Amendments (CLIA) laboratory performed NGS-based diagnostic genomic profiling which identified this tumor as the first reported case of the We reviewed the medical records of a patient with spindle cell neoplasm who presented to the Department of Investigational Cancer Therapeutics at The University of Texas MD Anderson Cancer Center after failing standard of care therapy. Treatment and consent on investigational trial, and data collection were performed in accordance with the guidelines of the University of Texas MD Anderson Cancer Center Institutional Review Board (IRB). Tumor response was determined using RECIST (version 1.1) by CT scans obtained about every six to eight weeks. Clinical evaluation and assessments were performed per protocol.3.Next-generation sequencing was performed by using the Clinical Laboratory Improvement Amendments (CLIA)-approved FoundationOne™ platform . FoundationOne™ is a targeted assay utilizing next generation sequencing in routine cancer specimens. The assay simultaneously sequences the entire coding sequence of 236 cancer-related genes  plus 47 introns of 19 genes frequently rearranged in cancer to a minimum coverage depth of 250X. The assay detects all class of genomic alterations  using routine FFPE tissue samples that may be as small as 0.6 mmA 55 year old female presented to the clinical center for targeted therapy to discuss treatment options for a progressive metastatic spindle cell neoplasm. Disease at presentation included a left chest wall mass measuring more than 6 cm in greatest dimension. Extent of disease evaluation also revealed a lytic lesion in the left seventh rib and a second smaller mass centered in the pleura.Pathologic examination of formalin fixed paraffin embedded (FFPE) biopsied tissue from the presumed primary tumor site revealed a spindle cell proliferation, which was diagnosed to be a malignant spindle cell neoplasm, favor sarcoma, which is akin to a diagnosis of exclusion. The diagnosis of a malignant solitary fibrous tumor was also entertained, but the features were not typical for such a diagnosis  75 mg/m2 and ifosfamide (Ifex®) 10 g/m2. She progressed during two cycles of this treatment, and was then started on gemcitabine (Gemzar®) 900 mg/m2 and docetaxel (Taxotere®) 100 mg/m2. The patient then progressed after two cycles of her second regimen.The patient was initially treated with doxorubicin  of bevacizumab and temsirolimus in combination with sorafenib for the treatment of advanced cancer . The patContemporaneously with the entrance of patient into the clinical trial, FFPE tumor cell-predominant tissue from the biopsy was submitted to a commercial CLIA-certified, CAP-accredited laboratory for genomic profiling based on next generation sequencing (NGS) based.At the time of this report, the patient’s overall response was stable disease for eleven cycles. She had the following ongoing toxicities that were felt to be at least possibly related to the study drug: grade 1 altered taste, grade 2 proteinuria, grade 1 hand-foot syndrome, grade 2 hypoalbuminemia, and grade 2 mylagias. The patient did not experience a dose limiting toxicity from the combination therapy. Unfortunately, the patient developed an acute myocardial infarction, hypotension, clinically deteriorated and died because of this co-morbidity.KIAA1549-BRAF fusion gene  due to the positive S100 immunohistochemical stain and histologic phenotype of spindle cells, although that diagnosis was not made here. Interestingly, the known activating V600E ST cases . The ideKIAA1549-BRAF fusion protein, BRAF is a cytosolic serine/threonine kinase that is activated by binding of RAS-GTP. The function of KIAA1549 remains unknown. This fusion joins intron 15 of KIAA1549 and intron 8 of BRAF, although several other configurations of these fused genes have been observed [Of the two components of the KIAA1549-BRAF as the BRAF kinase domain is completely conserved. Interestingly successful targeting by sorafenib of BRAF-fusion bearing cells specifically the AGK-BRAF fusion that contains the first 33 amino acids of AGK and the C-terminal portion of BRAF starting from position 328 of BRAF with loss of the CR1 domain (not KIAA1549-BRAF) has been recently reported [AGK-BRAF expressing melanoma cell line demonstrated an increased sensitivity to sorafenib [RAF fusion proteins reported in prostate cancer [Sorafenib is a small molecule inhibitor of WT BRAF and other kinases including VEGFR 1/2/3, PDGFRB, and RAF family, that has an IC50 for WT BRAF at 25 nm  and bindreported . In the orafenib . RemarkaBRAF fusion is insufficient for tumor response. An analogy can be made to non-melanoma solid tumors expressing the activating V600E mutant BRAF, where vemurafenib treatment is sometimes ineffective, ie as in the colon [KIAA1549-BRAF fusion hyperactivates the mTOR pathway [PTEN [BRAF fusion protein by sorafenib and of the downstream mTOR pathway by temsirolimus synergizes, although the specific mechanism of tumor regression awaits further investigation. Bevacizumab was also administered, but the contribution of this therapy to the patient response is difficult to ascertain. As has been theorized for other tumor types, it is possible that bevacizumab enhanced delivery of the other therapeutics by normalizing the tumoral vasculature [Notably, pediatric astrocytoma patients expressing this fusion progressed on sorafenib monotherapy in a small phase II trial . This obhe colon . In suchhe colon -12. Thishe colon -16 to BRhe colon . Thus, t pathway , as doesay [PTEN . In thisculature .KIAA1549-BRAF fusion such as pediatric pilocytic astrocytomas?What are the implications of this case for treating tumors that express the in vitro and in vivo studies well outside the scope of this report are required. Sorafenib is indeed not a specific inhibitor of BRAF and as such the effects may not be mediated by targeting KIAA1549-BRAF in this tumor.As patients with such tumors on sorafenib monotherapy progressed rapidly in a very small Phase II study , sorafenPTEN loss or other GAs activating the mTOR pathway are present alongside the BRAF fusion in a pilocytic astrocytoma of patient, thus suggesting a potential responsiveness to combined sorafenib/mTOR targeted therapy. As a possible therapeutic alternative, a recent in vitro study suggested that second generation BRAF inhibitors may be capable of effectively inhibiting this fusion protein [Specifically, genomic profiling based on clinical-grade NGS could identify whether  protein .KIAA1549-BRAF fusions typically coincide with alterations in the mTOR pathway, and whether such patients can be effectively treated with targeted therapy on that basis. However, this case already indicates such treatment possibilities exist for patients possessing KIAA1549-BRAF fusions, whether in pediatric astrocytomas or difficult to characterize sarcoma like lesions. In general, genomic profiling based on NGS may be of great relevance in the management of patient with solid tumors that defy pathologic definition, as this case demonstrates that such diagnostics can reveal unexpected but effective avenues of targeted treatment.A prospective clinical trial with prospective diagnostic genomic profiling will best answer whether tumors driven by KIAA1549-BRAF fusion, a recurrent oncogenic driver in sporadic pilocytic astrocytoma, is described here in a spindle cell neoplasm for the first time.• KIAA1549-BRAF fusion with PTEN loss to be successfully treated by targeted therapy.• This case is the first report any tumor expressing • Genomic profiling based on Next-generation sequencing (NGS) may be of great relevance in the management of patient with solid tumors that defy pathologic definition, as this case demonstrates that such diagnostics can reveal unexpected but effective avenues of targeted treatment.KW, SMA, VAM, PJS, GAP, and JSR are all employees of and own stock in Foundation Medicine.All authors contributed to writing the manuscript. VS, VAM and SMA conceived the manuscript. VS and DA provided clinical expertise. SNW is the primary investigator for the clinical trial and VS is the co-primary investigator for the clinical trial. VS, SNW, KW, WW, VAM, JR, PJS, GAP and SMA analyzed the data. WW, JR, SMA provided pathology expertise. VS and SMA wrote the paper. VS and DA provided sarcoma expertise. VS and SMA provided cellular, molecular and targeted therapy expertise. All authors read and approved the final manuscript.


The prevalence of carcinoma in main-duct intraductal papillary mucinous neoplasm (IPMN) is high, and surgical resection is recommended for all patients with a main-duct IPMN.A main-duct IPMN with typical imagings including protruding lesions in the dilated main pancreatic duct was resected, but the histology was intraductal papillary mucinous adenoma of the pancreas.It has been reported that the presence of mural nodules and dilatation of MPD are significantly higher in malignant IPMNs. The presented case had protruding lesions in the dilated main pancreatic duct on endoscopic ultrasonography, but the histology was adenoma.Preoperative distinction between benign and malignant IPMNs is difficult. Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a distinct entity characterized by intraductal papillary growth and thick mucus secretion. Copious mucous fills the main and branch pancreatic ducts and causes ductal dilation. Thus, IPMNs of the pancreas are being diagnosed with increasing frequency by radiologic findings.IPMNs are pathologically classified into two types based on site of tumor involvement, that is, main-duct type or branch type. The histology is classified into intraductal adenocarcinoma, intraductal adenoma, and hyperplasia. It has been suggested that these lesions have the capacity to progress from hyperplasia to adenoma, to noninvasive carcinoma, and ultimately to invasive carcinoma. The prevalence of carcinoma in main-duct IPMN is as high as 60%  to 92%  recommenA 56-year-old man presented with mild abdominal pain. Physical examination and laboratory data were unremarkable. Abdominal symptoms were relieved with conservative medical management within a few days. Abdominal ultrasonography showed dilation of the main pancreatic duct (MPD). Subsequent computed tomography scan showed dilatation of the MPD and several small cysts in the pancreatic tail, with no findings of mural nodules, mass formation, metastasis, or lymph node swelling. Magnetic resonance cholangiopancreatography (MRCP) showed an irregular dilatation 10 mm) of the MPD of the tail [Figure 0 mm of tIPMN is an increasingly recognized entity representing a spectrum of benign and malignant neoplasms of the pancreas. International consensus guidelines  recommenIt has been reported that the presence of mural nodules and dilatation of MPD are significantly higher in malignant IPMNs . We alsoEUS is one of the most useful modalities to evaluation local changes in the pancreas. Intraductal papillary adenocarcinoma is characterized by papillary protrusions and thick septum like structures in a dilated duct as delineated by EUS. When the thickness of the septum structure is more than 4 mm on EUS, neoplastic change should be suspected. Finding of solid mass or a mass showing a mixed-echo pattern in the pancreatic parenchyma are characteristic of the invasive type of IPMN .The present case was a typical main-duct IPMN, and EUS showed its entire picture. Although the case had mural nodules and the dilated MPD which were indicators of possible malignancy, the histology was intraductal papillary mucinous adenoma. For the high prevalence of carcinoma in main-duct IPMNs, international consensus guidelines that recommend surgical resection for all patients with a main-duct IPMN are considered reasonable and proper. As it stands now, although the histology was adenoma, resection was considerable based on the patient' s age and adenoma-carcinoma sequence. Preoperative distinction between benign and malignant IPMNs remains difficult.Preoperative distinction between benign and malignant IPMNs remains difficult.Written informed consent was obtained from the patient for analysis of the resected specimen and imaging, and publication. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KT, TK, and TT did the examination, KT and TK wrote the paper, MK and GH did the surgery, and SH did the pathology. All the authors reviewed and approved the end version.