Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. However, the core speech disorder subserving CMS is still unclear.This study investigates the speech and language symptoms following posterior fossa medulloblastoma surgery in a 12-year-old right-handed boy. An extensive battery of formal speech (DIAS = Diagnostic Instrument Apraxia of Speech) and language tests were administered during a follow-up of 6 weeks after surgery. Although the neurological and neuropsychological  symptoms of this patient are consistent with Schmahmann’s syndrome, the speech and language symptoms were markedly different from what is typically described in the literature. In-depth analyses of speech production revealed features consistent with a diagnosis of apraxia of speech (AoS) while ataxic dysarthria was completely absent. In addition, language assessments showed genuine aphasic deficits as reflected by distorted language production and perception, wordfinding difficulties, grammatical disturbances and verbal fluency deficits.To the best of our knowledge this case might be the first example that clearly demonstrates that a higher level motor planning disorder (apraxia) may be the origin of disrupted speech in CMS. In addition, identification of non-motor linguistic disturbances during follow-up add to the view that the cerebellum not only plays a crucial role in the planning and execution of speech but also in linguistic processing. Whether the cerebellum has a direct or indirect role in motor speech planning needs to be further investigated. Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) is characterized by a transient period of cerebellar mutism combined with a range of neurological (motor) disturbances, neuropsychological  abnormalities and neurogenic speech/language deficits. It is most commonly observed in children and has only been occasionally reported in adults –4. CMS iThe pathophysiological mechanisms subserving CMS remain to be clarified. The immediate onset of ataxia and dysmetria are likely due to surgical impact on the cerebellum. Secondary processes initiated by the tumor resection seem to play a role in the delayed onset of symptoms , 27 and Following remission of CM the cluster of motor speech characteristics are often defined as ataxic dysarthria, since this type of dysarthria is typically found in a context of cerebellar pathology . HoweverThis study investigates the speech and language symptoms following posterior fossa medulloblastoma surgery in a 12-year-old right-handed boy. A detailed description of the motor speech characteristics may help to unravel the puzzling nature and pathophysiology of the CMS.Following a 1-year period of frequent episodes of headaches, neck pain and progressive coordination and balancing problems, a 12-year-old right-handed boy (LD) was admitted to hospital. Medical history consisted of repeated falls with a brief loss of consciousness at the age of 2 years. A neurological and cardiological work-up including repeat EEG  and ECG (electrocardiogram) revealed no abnormalities. Growth and developmental milestones were reported normal. He was born at term after normal gestation and labor and there had been no perinatal or postnatal problems. His scholastic achievements had always been above average levels and there was no familial history of developmental disorder or learning disability. Both his parents and two brothers (14 and 16 years old) were healthy.The neurological examination on admission revealed mild hypotonia, dysmetria (finger-to-nose test) and balancing problems (Romberg test). No speech or language problems were observed but in-depth linguistic assessments were not performed. MRI (Magnetic Resonance Imaging) of the brain showed a tumoral mass lesion in the fourth ventricle extending to the foramen magnum, invading the vermis and cerebellar parenchyma bilaterally and exerting mass effect on the tentorium, pons and cerebellum Fig. -c. The tEarly postoperative MRI of the brain revealed subtotal tumor resection with tumoral residue located posteriorly right to the resection cavity, cranial to the vermis. Remission of the hydrocephalus was observed. However, postoperative sequelae included tissue damage at the level of both caudate nuclei, moderate dilation of the 4th ventricle, involvement of the cerebellar peduncles with a slight extension towards the corpora quadrigemina at the level of the inferior colliculi Fig. -i. ReducClinical neurological examination showed asymmetric peripheral muscle weakness (more severe at the right side than at the left side), but the peripheral reflexes were symmetrically preserved and cranial nerves were unimpaired. Ataxia, hypotonia, dysmetria and akinesia were found. He developed a general state of apathy during the first days after surgery.Due to leakage of cerebrospinal fluid at the insertion point of the drain and increased hydrocephalus, a new external drain was installed in combination with a ventriculostomy 2 weeks after surgery. The drain was removed one week later. On repeat CT 1 month after surgery (7/10) the ventricular volumes had diminished.At day 5, he developed akinetic mutism. Auditory comprehension for daily conversation seemed intact since he succeeded to accurately answer yes/no questions in a non-verbal but slightly delayed way using his hands. He had marked difficulties moving his mouth, tongue and eyelids on request (voluntary movements) although automatic movements were carried out flawlessly . He could not produce any sounds on command but when coughing or laughing, phonation was clearly audible. Marked presence of automatico-voluntary dissociations clearly indicated buccolabiolingual and eyelid apraxia. A bilateral paresis of the trochlear nerve (right > left) was present and a right paresis of the abducens nerve induced diplopia. After 1 week postsurgery, speech therapy was started to practice orofacial movements and to initiate speech. Under the guidance of the speech therapist peroral food was slowly built up successfully 2 weeks after surgery. Physiotherapy was started which resulted in increased movements of the arms and legs. Ataxia, hypotonia and akinesia were still present at 3 weeks postsurgery.Aside from apathy, he displayed emotional instability, infantile behavior and he was extremely frustrated and unhappy not being able to speak. A psychiatric evaluation three weeks after surgery confirmed that frustration occurred secondary to mutism.Four weeks after surgery the boy gradually started to speak again. Spontaneous output was severely adynamic (few words/sentences) at week four but became more fluent in the following weeks. The patient spoke with a clear dental articulatory setting: all alveolar sounds  were consistently pronounced with the tongue tip against or between the teeth. In addition, the patient had a falsetto voice quality with an average fundamental frequency of 490 Hz. The patient was able to lower his voice on command, but this sounded forced and unnatural. Speech was further characterized by the prolongation of speech sounds (e.g. ‘kammmm’ instead of ‘kam’ (comb)), the devoicing of consonants (e.g. ‘pank’ instead of ‘bank’ (bank), mostly in initial position), hypernasality and self-corrections. In addition, speech tempo was relatively slow (2.5 syllables per second). This is significantly slower than in a reference sample of adult male and female speech in Verhoeven et al.  in whichAside from staccato speech, none of the typical signs of (ataxic) dysarthria such as distorted vowels, imprecise consonants, excess and equal stress, and irregular articulatory breakdown were observed. By marked contrast, typical characteristics of apraxia of speech (AoS) were found including articulatory groping and segmentation. Formal investigation of motor speech by means of the Diagnostic Instrument Apraxia of Speech (DIAS)  was perfAdditional linguistic and cognitive assessments were carried out at 6 weeks postsurgery on the basis of the ScreeLing , the CliAlthough the right hemiparesis improved, the boy was bounded to a wheelchair. Hypotonia, ataxia and diplopia persisted. Affective/behavioral symptoms diminished but overall frontal behavioral disinhibition with infantile features , interdental articulatory setting) were still present 6 weeks after surgery. The infantile behavior decreased when the family was not present in the room.A grade IV medulloblastoma was treated by means of a program of radiotherapy (boost of 30.6Gy in 17 extra fractions of 1.8 Gy) and a preventive program of craniospinal radiotherapy (23.4Gy in 13 fractions of 1.8Gy). During the radiotherapy concomitant chemotherapy was given (Vincristine 1.5 mg/mt 1×/week * six cycli). During the entire period of radio and chemotherapy patient suffered from frequent headaches, nausea and infections which made extensive cognitive assessment impossible.He was admitted to a rehabilitation center in between the four cycli of chemotherapy where he entered a multidisciplinary therapy program . Clinical neurological investigations at 6 months postsurgery showed almost symmetrical strength with remission of the right hemiparesis. The patient started walking again but ataxia remained. Speech and language improved but slowed speech, articulatory groping, segmentation and wordfinding difficulties were still observed. No formal language tests were performed. He spoke with a normal tonal voice but the falsetto voice reappeared occasionally. After 8 months planned neurocognitive examinations were cancelled because of regrowth of the tumor.This patient with CMS not only shares a number of overt similarities but also some differences with the complex of symptoms that constitute CMS as generally described in the literature. As in most cases with CMS, this patient developed cerebellar mutism (CM) combined with neurological symptoms, neuropsychological disturbances and speech deficits following posterior fossa tumor surgery –4. PostoAlthough the neurological and neuropsychological  symptoms of this patient are typical for CMS –20, 22, In the posterior fossa literature motor speech symptoms following CM are typically defined as ataxic dysarthria in posterior fossa literature , 15–19. As a result it is hypothesized that the CM in this patient was more likely induced by an underlying speech planning and organization deficit (apraxia) and not by a pure motor disorder (dysarthria). AoS and ataxic dysarthria share some semiological similarities , but these may reflect more universal aspects (e.g. motor slowness) . The rolThis case might be the first example that clearly demonstrates AoS following posterior fossa tumor surgery. Therefore, the findings add to the view that the cerebellum plays a crucial role in the planning and execution of speech. Whether the cerebellum has a direct or indirect role in motor speech planning needs to be further investigated. Although residual speech symptoms following CMS are frequently defined as ataxic dysarthria, there is no consensus on the core speech characteristics that follow CM , 54, 55.No in-depth speech and language assessments were performed in the preoperative phase and after the 6 week follow-up period. No preoperative assessments were carried out because the presence of obstructive hydrocephalus required immediate surgical intervention. Longer-term postoperative assessments were missing due to referral to another hospital, time-consuming treatment plans and regrowth of the tumor. No motor scales were formally conducted but a close clinical neurological follow-up was performed. The use of functional neuroimaging might enable to further unravel the direct or indirect role of the cerebellum in motor speech planning.CMS consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. On the pathophysiolgical level the complex range of symptoms unambiguously reflects crucial involvement of the cerebellum and the cerebro-cerebellar pathways in disrupted cognitive and affective processes. However, the core speech characteristics of CMS and the exact role of the cerebellum in motor speech planning versus execution are still unclear. In this article, a detailed description is provided of the motor speech and language deficits following posterior fossa tumor surgery in a 12-year-old right-handed boy. In-depth analyses of motor speech production revealed features consistent with a diagnosis of AoS while ataxic dysarthria was absent. Future research is needed to confirm these findings and to explore the precise role of the cerebellum in motor speech planning processes.


A ureterocele is a congenital defect of the ureter. Based on available autopsy reports, the highest incidence of ureteroceles has been reported as 1 in 500, occurring four to six times more commonly in females than in males. It can occur in up to 95% of females with a duplex collecting system .Ureteroceles in adults usually arise within a single renal system with no or mild obstruction. They may present as recurrent urinary tract infections, flank pain or remain asymptomatic, only to be picked up incidentally via imaging. Stasis and infection may predispose to calculus formation in the ureterocele and upper urinary tract .The association of a ureterocele with a tumor is very uncommon. In this paper, we report a unique case of a tumor arising within one of bilateral ureteroceles.A 67-year-old Chinese male presented with painless gross hematuria after sexual intercourse. He was a non-smoker, and had no previous contact with anilines or other chemicals. Urine cytology and serum prostate specific antigen were normal. Computed tomography (CT) urography revealed bilateral simple ureteroceles with the left containing a slightly enhancing soft tissue nodule . FlexiblTransurethral unroofing of the left ureterocele containing the tumor together with right ureterocele was performed. There was a papillary lesion on the inner surface sparing the proximal end of the ureterocele and the ureteric orifice . This coThe pathology laboratory received multiple fragments of tissue, aggregating 1.5x1.5x0.3cm and weighing 0.5g. Histological examination of resected specimen revealed tumor cells displaying round to oval nuclei arranged to form a papillary architecture with fibrovascular cores, without stromal invasion. The tumor cells exhibited mild nuclear atypia and occasional mitoses, and there were no high grade nuclear features seen. These findings were consistent with a low-grade noninvasive papillary urothelial carcinoma . Post-opA combination of imaging techniques for ureteroceles has been employed in the past till present. Modalities include ultrasonography, intravenous pyelogram (IVP) and CT urography. Classical appearances of ureteroceles based on different imaging modalities are described below.The sonographic finding of a well-defined cystic intra-vesical mass within the posterior bladder wall is suggestive of a ureterocele and a classic description is that of a cyst within a cyst. Tumors arising from the ureterocele may bear features of irregular echogenicity without acoustic shadowing . HoweverThe classic finding on IVP is a round radiopacity in the bladder surrounded by a radiolucent rim. The characteristic appearance of a ureterocele on CT urography is an intra-vesical defect that is radiolucent and globular in nature manifesting as the “cobra-head sign” . The CT Magnetic resonance imaging (MRI) is usually not used, but it should be as effective as IVP and CT urogram for visualization of ureteroceles, especially when MR urography is performed with or without contrast . There iThere are fifteen reports of tumors arising from true ureteroceles , 7–12, hThis case highlights the need for upper tract imaging as an investigation for gross hematuria regardless of whether urine cytology reflects no evidence of malignancy. Common causes of gross hematuria include urinary tract infections, stones and malignancies. Whilst highly unusual, this patient was found to have bilateral ureteroceles, of which one harbored a tumor. There are no current guidelines on the management of urothelial tumors arising from ureteroceles. However, there is general consensus that when the tumor does not encroach the ureteric orifice or distal ureter, adjuvant therapy and intensity of surveillance can be guided by the histological grade and stage of the tumor .


Uterine leiomyomas presenting as incarcerated or strangulated hernias in surgical emergencies are extremely rare and should be considered in the differential diagnosis in patients with known uterine fibroids and an irreducible ventral abdominal wall hernia. Detailed history and multidisciplinary approach optimize the diagnosis and decision making toward surgical treatment. Uterine leiomyomas  are the commonest benign uterine lesions and can be histopathologically revealed in up to 70% of patients undergoing hysterectomy A midline abdominal incision is the commonest incision used in open general surgery procedures. It is mostly favorable because surgeons achieve access to the abdominal cavity with limited damage to the muscles, nerves, and blood supply of the abdominal wall, while it is quick and easy to perform However, an incarcerated giant leiomyoma within an incisional hernia is a very rare entity, surprising the surgeon, and raising multiple management queries A rare case of a giant uterine leiomyoma incarcerated into an incisional hernia is presented here, and management options and concerns are highlighted.A 56‐year‐old, nulliparous woman, with previous surgical history of an open paraumbilical hernia repair without the use of prosthetic mesh a year ago was referred due to diffuse abdominal pain. The patient was on antipsychotic medication for schizophrenia, a heavy current smoker (30 pack/years) and morbidly obese (body mass index 35). Patient's mental capacity was assessed using the legally relevant criteria, and she was found to be incompetent. Therefore, her brother acted as decision maker and consented for further investigation and treatment.The patient had normal pulse (85/min) and arterial blood pressure (130/82 mmHg), remaining apyrexial. She was initially assessed by the surgical team, and she was found to have an approximately 4‐cm irreducible incisional paraumbilical hernia. The content and texture of the hernia sac were round and firm clinically. Tenderness was elicited with deep palpation, and an incarcerated incisional hernia was the initial working diagnosis. Routine hematological and biochemical laboratory investigations were unremarkable, and abdominal X‐ray did not show any signs of small bowel obstruction. Lactate levels were within normal range.Digital rectal examination identified a large painful pelvic mass and no signs of bleeding. A gynecological examination was requested, but there were limitations for further assessment as the patient was virgin, and she could not undergo a per‐vagina examination. Considering all the above findings and body habitus, an urgent abdominal CT scan was performed. The latter showed a 15 cm solid pelvic mass with scattered calcifications originating from the uterus or adnexa suggesting a large uterine leiomyoma. The mass was extending up to the level of a sizable lower abdominal midline incisional hernia. The hernia sac was filled with small bowel loops on imaging. No free fluid or pneumoperitoneum was noted. The patient was discussed in our MDT with gynecological input, and a decision was taken to proceed with an exploratory laparotomy.Of note, she was postmenopausal and nulliparous, and a decision had to be made regarding the potential scenario of hysterectomy. Following an extensive discussion with her brother, consent was obtained to proceed with hysterectomy if needed.As a joint care with the Gynecologists, surgical exploration was decided and performed via a midline laparotomy incision along the previous abdominal wall scar. Cefuroxime 1.5 g was given at anesthetic induction for antimicrobial prophylaxis.Surprisingly, and against CT scan findings, a large incarcerated solid mass was seen within the hernia sac Fig. . There wFinal histopathology of the tumor showed a 19 × 14 × 9 cm uterine leiomyoma with ischemic and hemorrhagic necrosis, calcifications, and hyalinization with no evidence of dysplasia or malignancy Fig. .The patient had an uneventful postoperative course, and standard antipsychotic medication was promptly restarted. On the second postoperative day, she accidentally pulled her negative pressure drain off, and a small seroma was formed, which was gradually absorbed. She was discharged on the 7th postop day.Incisional umbilical and paraumbilical hernias, described for the first time back in 1968, are unusual entities, with an incidence of 21/100,000 in the Mintz study Uterine leiomyomas presenting as incarcerated or strangulated hernias in surgical emergencies are extremely rare and should be considered in the differential diagnosis in patients with known uterine fibroids and an irreducible ventral abdominal wall hernia. Detailed history and meticulous clinical examination in cooperation with other surgical disciplines when required as well as radiological evaluation with ultrasound scan and computed tomography are important adjunctive tools of a multidisciplinary team optimizing the diagnosis and decision making toward surgical treatment. In an era of extremely accurate imaging modalities, surgery can still reveal unexpected findings and requires high level of experience and teamwork to deal with complex clinical scenarios.GE: was the main author, wrote and reviewed manuscript and performed a literature review. NV: Provided the intra‐operative figures and the gynecological input in patient's management. DD: Contributed in edition of final manuscript. LM: Reviewed patient's CT imaging, contributed to study design and performed a literature review. TT: Involved in overall description of the manuscript and provided the intra‐operative pictures.None declared.


Radiologic depiction of a sunburst pattern of new bone formation is characteristic.2 Early diagnosis and complete tumor resection are mandatory to improve the prognosis of jaw OS.Gnathic (mandibular) osteosarcomas (OSs) account for approximately 6.5% of all OS, and most of them are secondary in nature, occurring in patients with Paget disease or fibrous dysplasia or as a late sequela to craniofacial radiotherapy.A 56-year-old woman presented with left side cheek swelling of 1.5 months in duration. On clinical examination, expansile hard swelling of left hemimandible was noted that was seen to be both intraoral as well as in the neck. A reconstructed radiograph  showed aFour months later, the patient presented with rapid progression of the left mandibular mass. A repeat reconstructed radiograph  and CT iThe patient underwent left hemimandibulectomy with free fibula osteocutaneous flap reconstruction. The histopathology revealed OS with approximately 75% tumor necrosis. The left breast mass was excised and subjected to histopathology examination, which revealed infiltrating duct carcinoma with predominant ductal carcinoma in situ. Eight months after surgery, the patient presented with diffuse skeletal pain and dyspnea. Chest radiograph revealed pleural effusion, and technetium-99m–methylene diphosphonate bone scan showed multiple-site skeletal metastases with soft tissue tracer uptake in the left-sided pleural space with effusion suggestive of pleural involvement. Oral and neck examination did not show any evidence of recurrent or residual disease. The patient was managed for metastatic disease from the breast primary tumor.1 Two variants have been described in the literature, primary and secondary, of which secondary mandibular OS is more common. Secondary mandibular OS usually occurs in association with Paget disease, fibrous dysplasia, or Ollier disease or as late sequela to craniofacial radiotherapy. The exact cause of OS is unknown; however, a number of risk factors have been described. Rapid bone growth has been enumerated to be one of the predisposing factors in the etiopathogenesis of OS.4 The secondary variant suggests an association between OS in general and excessive bone cellular activity. The reported incidence of OS is significantly higher in patients with retinoblastoma, which suggests the role of the RB1 gene in tumorigenesis of both diseases. Other syndromic associations include Li-Fraumeni syndrome, Bloom syndrome, Werner syndrome, Rothmund-Thomson syndrome type 2, Diamond-Blackfan anemia, and RAPADILINO  syndrome.3OS of the jaw is rare and composes approximately 6.5% of all OSs and 1% of all malignant tumors of the head and neck. These tumors present later than extremity OS, are characterized by a higher propensity for local recurrence and absence of distant and pulmonary metastases, and are refractive to chemotherapy.4 The radiographic appearances are varied, ranging from osteolytic pattern to mixed osteogenic pattern. The osteogenic pattern almost always shows an area of the typical sunburst appearance, which on radiography is seen as stippled bone pattern with destruction of the cortical outlines and perpendicular striae (Sharpey’s fiber) of periosteal reaction. This pattern suggests the rapid tumoral growth that gives limited time for periosteum to lay down a new layer of protective bone. The common mimics of this radiologic pattern include odontomas, odontogenic myomas, solitary plasmacytomas, squamous cell carcinoma of the mandible, central hemangioma, fibrous dysplasia, and metastatic lesions from primary colonic malignancies. Other nonspecific features include widening of the periodontal ligament space and attenuation of the lamina dura.6 Valuable adjuncts for not only evaluating the extent of the tumor but also assessing its relationship with neighboring tissues include CT, as a result of its superior bone architectural detection, and MRI, as a result of its ability to image the soft tissues. On CT imaging, the mass shows thin irregular spicules of new bone formation that are perpendicular to the epicenter of the lesion, giving the classic sunburst appearance. Similar appearance can be appreciated on MRI as well, where the ossified newly formed periosteal bone reaction shows low signal on all of the sequences and the actively proliferating soft tissue mass is seen as enhancing intervening areas.7 Three-dimensional volume-rendered CT reconstruction and MRI play an important role in planning the surgical management.Males are more commonly affected than females. Swelling, loss and displacement of local teeth, spasm, and local parasthesia are common presenting complaints and are often misdiagnosed as chronic pulpitis or chronic periodontitis. The condition is usually not associated with pain. Most patients present after dental treatment and mostly relate the symptoms to previous tooth extraction.8 Histologically, OSs are composed of abnormal spindle cells that may produce osteoid or immature bone. In the jaw, the majority of the lesions reveal cartilaginous differentiation.9 Although OS of the jaw is better differentiated on histology than OS of the extremities, some tumors exhibit a deceptively bland histologic appearance, and hence, there is a need for appropriate clinicoradiopathologic correlation. Differentiation of OS and detection of any underlying bone lesion, such as odontogenic myomas, fibrous dysplasia, solitary plasmacytomas, Paget disease, and metastatic bone tumors, are based more on histopathology than radiologic evidence.9 Different levels of cell differentiation pose difficulty in distinguishing primary OS from reactive stromal proliferations that are commonly seen in callus formation in fractured bone and also in cellular forms of Paget disease.9 The patient reported here was diagnosed to have primary osteoblastic OS. Biochemical investigations that have been reported to have role in diagnosis are serum alkaline phosphatase and osteocalcin levels. Raised serum alkaline phosphatase may be seen in OS but is not a consistent finding. Osteocalcin, a bone-specific protein, has been used in differentiating OS from malignant fibrous histiocytoma.9The local aggressiveness of mandibular OS is greater than that of appendicular OS despite the same histology. The common histologic variants, on the basis of the type of extracellular matrix produced by the tumoral cells, are the same as those affecting the appendicular skeleton and include osteoblastic, fibroblastic, chondroblastic, and mixed OS. Other less common histologic variants are myxomatous, telangiectatic, epithelioid, fibrous histiocytoma–like, giant-cell, small-cell, and large-cell OS.RB and p53 gene heterozygosity, and overexpression of P-glycoprotein.3MDM2 overexpression and amplification are found to be molecular makers for low-grade and dedifferentiated mandibular OS and are not seen in lesions such as fibrous dysplasia and ossifying fibroma. GNAS mutations that are specific for fibrous dysplasia are absent in low-grade OS. Juvenile ossifying fibromas have the potential to evolve into a giant-cell–rich variant of high-grade OS and are shown to be associated with RASAL1 amplification or RASAL1/MDM2 coamplification.10Apart from necrosis, various other potential prognostic markers that have been identified include tumor cell ploidy, expression of HER2/CerbB2, gains and loss of specific chromosomes, loss of 12 Older age at onset carries a better prognosis because older age is associated with better resistance.12 The higher local recurrence rate  is attributed to the complex anatomy associated with the maxillofacial region that interferes with achieving adequate margins.1 In the patient presented here, the tumor showed an aggressive biologic course, an uncommon feature of gnathic lesions.Local recurrence and metastasis from jaw OS are less common than in OS of the long bones, and the proposed routes of metastatic spread include microscopically through the marrow spaces, via the mandibular canal or mental foramen and spread along the inferior alveolar nerve or mental nerve, and via the connective tissue that connects the intraosseous components and soft tissues such as the periodontal ligament. This may explain the facilitation of spread of an intraosseous lesion into the adjacent soft tissues. Metastases are relatively higher in postradiation OS of the jaws and tend to occur within 2 to 3 years. The 5-year survival rates of mandibular and maxillofacial OS are 34.8% and 25.8%, respectively, with a median survival time of 2.9 years for maxillary OS and 6.5 years for mandibular OS.13 Some studies have used radiotherapy and/or chemotherapy in conjunction with local radical surgery.14 Early diagnosis and appropriate surgery with adequate margins are the mainstay of treatment.14 In the patient presented here, no pulmonary or other distant metastases were present on whole-body scanning at presentation. Our patient’s disease was confirmed by histopathology and promptly treated by surgical resection. The patient was observed regularly at this center, and she responded well to the treatment; however, 8 months later, she developed skeletal and pulmonary metastases from the breast primary tumor.Unlike for extremity OS, for unknown reasons, chemotherapy has shown negligible impact on the outcome of craniofacial OS.In conclusion, gnathic OS is an uncommon mimic of common dental pathologies. Lack of awareness to consider OS in the maxillofacial region leads to delayed diagnosis that impedes early surgery. Timely radiopathologic diagnosis and depiction of extent of disease using three-dimensional volume-rendered CT and MRI are essential for prompt surgical resection and use of subsequent chemotherapy or radiotherapy.


Caroli's disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli's disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli's syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli's disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis. Magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed findings consistent with Caroli’s disease. Laboratory investigation disclosed a raised α-fetoprotein. Left hepatectomy was performed due to suspected cholangiocarcinoma. Morphological findings were compatible with Caroli’s disease and no evidence of malignancy was found. Case two was a 47-year-old man who presented with chronic epigastric pain and generalized abdominal discomfort. MRCP revealed findings compatible with Caroli’s disease. The patient was discharged with ursodeoxycholic acid treatment and was later admitted twice due to inaugural episodes of cholangitis that were medically managed. Bisegmentectomies II and III were performed for suspected neoplasia after a gradual rise in α-fetoprotein and CA19-9 values were noted during follow-up. The surgical specimen confirmed Caroli’s disease and there was no evidence of malignancy. Postoperative periods for both patients were favorable, and they remain asymptomatic and well to date. Caroli's disease is a rare congenital malformation of the intrahepatic bile ducts characterized by duct ectasia and dilation, which may involve the biliary tract in a focal or multifocal manner . It is cCaroli’s disease is less common than Caroli’s syndrome, and both are extremely rare with an approximate prevalence of less than one in 1,000,000 inhabitants . In CaroWe describe two cases of Caroli’s disease presenting with chronic, low-grade, abdominal pain. We believe the present reports are of medical significance since they relate to patients with atypical presentations and diagnosed at an unusually late age. It serves also as a reminder that although rarely, Caroli’s disease may present later in life, have atypical presentations and be masked by non-specific clinical findings. These reports hope to add to the existing knowledge base of this very rare congenital disease.Case oneA 76-year-old man consulted his primary care physician due to low-grade, chronic, epigastric pain with additional non-specific complaints. The pain was pressing in character, non-remitting and had been present for approximately eight months. The patient denied nausea, vomiting, weight loss, changes in bowel habits or fecal characteristics, fever, or urinary symptoms. He also denied alcohol or tobacco consumption. His history was positive for dyslipidemia, hypertension, obesity, orthopedic surgery in the right knee at age 39 due to trauma, and radical prostatectomy at age 69 following the diagnosis of a prostatic carcinoma. Abdominal ultrasonography revealed vesicular lithiasis, as well as a heterogeneous nodular lesion and atrophy of the left lobe of the liver. The patient was therefore referred for surgical consultation to assess the need for cholecystectomy and to further investigate the liver findings. A physical examination was remarkable for an overweight patient , blood pressure of 146/92 mmHg, and pulse of 68/min. An abdominal examination revealed a non-painful abdomen with positive bowel sounds, with no discernible organomegaly. The rest of the examination was unremarkable, as the patient presented systemically well with all observations within normal range. A magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed dilatation of the left intrahepatic bile ducts associated with parenchymal atrophy Figures , 1B.Three nodular images without phase enhancement in the dynamic study were also identified, the first with 20 mm in the left branch of the main bile duct at the level of the bifurcation, the second with 12 mm in the duct of segment II, and a third downstream of the previous one with 5 mm. These lesions were described as being compatible with a possible cholangiocarcinoma. The right bile duct presented a small ectasia with 4.3 mm without parenchymal or upstream bile duct changes. The extrahepatic bile ducts were unremarkable for changes in morphology or duct caliber. A cystic lesion of the head of the pancreas and several parapyelic and cortical renal cysts were also observed.Laboratory data of peripheral blood on admission are provided in Table Investigations disclosed an elevated α-fetoprotein level and due to the image and laboratory findings, a left hepatectomy was deemed appropriate. Histological examination of the excised portion Figure  revealedDiscrete cholestasis, foci of micro- and macro-vesicular steatosis, scattered lymphocytes, and intrahepatic lithiasis were also observed. The lymph nodes showed no histological changes nor did the surgical specimen present with histopathological findings of malignancy. The morphological findings described were compatible with Caroli’s disease with unilobar involvement and secondary periductal liver fibrosis.The postoperative period was favorable and without complications, and the patient was discharged seven days later. Post-operatively, due to age, the exclusion of neoplastic suspicion and a favorable clinical outcome, follow-up benefits, and a proposal of discharge from the outpatient clinic was discussed, which the patient agreed to, remaining asymptomatic, under surveillance in the primary care setting.Case twoA 47-year-old man presented to his primary care physician with low-grade, chronic epigastric abdominal pain, which had lasted for six months, and was referenced to our department for further evaluation. The pain was described as pressing in character, with no irradiation or associated factors. His history was positive for oligophrenia, congenital malformation of the upper limbs, bilateral cataracts, chronic hepatitis B, type I diabetes mellitus, and cholecystectomy at age 38. The patient was mildly tender, locally, over the epigastric region of the abdomen but rebound tenderness was absent. Abdominal ultrasonography exposed an apparent atrophy of the left lobe of the liver. MRCP revealed atrophy of the left lobe, dilation of the intrahepatic biliary tract and intrahepatic lithiasis, as well as a renal cyst on the left , or MRCP should be regularly performed given the high risk in this patient population . GeneticIn summary, Caroli’s disease is a rare congenital malformation of the intrahepatic bile ducts and may be associated with conditions affecting other organ systems, such as polycystic kidney disease. Despite the very low prevalence, Caroli’s disease should be present in the differential diagnosis of epigastric abdominal pain and recurrent cholangitis without risk factors or relevant history. After clinical suspicion, the diagnosis is always imagiologic, with ultrasound and MRCP being the tests of choice. Caroli’s disease may present with indolent abdominal pain instead of the more typical presentation of recurrent cholangitis. Medical therapy should be performed with ursodeoxycholic acid, but surgical intervention can be curative. Nevertheless, the management strategy should be adapted to individual cases and strongly depends on the location and extent of cystic dilations, history of the disease, complications, and comorbidities. Complications can be frequent and severe, and mortality from cholangiocarcinoma deserves early intervention and close follow-up.


Brunner gland hamartoma (brunneroma) is a rare benign tumor of the duodenum. It is usually asymptomatic and detected incidentally by endoscopy or other imaging modality. The definitive diagnosis is based on histopathological findings. These may mimic tumors of other natures, such as gastrointestinal stromal tumors (GIST), carcinoids, lipomas, and leiomyomas. Here, we present a case of duodenal polyp presenting with abdominal pain and obstructive symptoms that caused duodenal intussusception. It was surgically removed and found to be a brunneroma on histopathology.Patankar AM, Wadhwa AM, Bajaj A, Ingule A, Wagle P. Brunneroma: A Rare Cause of Duodeno-duodenal Intussusception. Euroasian J Hepato-Gastroenterol 2016;6(1):84-88. Brunner gland hamartoma, also known as brunneroma, is a rare benign tumor of the duodenum . These bA 31-year-old female of Indian ethnicity came to the gastrointestinal surgery department with complaints of epigastric pain for the last 15 to 20 days. The pain was moderate in intensity, intermittent, and aggravated by eating food (both solids and liquids). It was associated with bouts of nonbilious, non-projectile vomiting. There was no history of hematemesis, melena, jaundice, weight loss, or anorexia. There was no significant medical or surgical history of past illness. On examination, there was mild epigastric tenderness. However, there was no palpable mass, guarding, or rigidity. Her vital signs were stable. The blood parameters were within normal range.An initial ultrasound was performed and a 4.4 × 3 × 2.7 cm oval, well-defined, isoechoic, intraluminal solid mass was found in the region of distal duodenum extending up to the duodeno-jejunal flexure . On coloA multidetector triphasic CT scan of the abdomen and pelvis was performed on Siemens definition flash CT scanner with 5 mm slice thickness and 128 × 0.6 mm collimation. Retrospective 3D reconstruction of volumetric data was performed to obtain coronal and sagittal images. It revealed a 5 × 3.2 cm , well-defined, smooth marginated, and uniformly enhancing soft tissue lesion in the fourth part of the duodenum extending up to the duodeno-jejunal flexure. There was no evidence of any early arterial enhancement, necrosis, or fat density areas within.The lesion showed a long, slender stalk that appeared to arise from the first part of the duodenum. There was telescoping of the first part of the duodenum into the second part  to D. ThThe patient underwent upper gastrointestinal endoscopy with EUS, which revealed a giant polypoidal lesion arising from the junction of the first and second parts of the duodenum with ulcerations at its tip  to C. ThIn view of broad-based pedicle and possibility of GIST, a supra-ampullary duodenectomy was performed .The polypoidal duodenal mass lesion  and B wa3 These glands are present in the first and second parts of the duodenum, and their concentration decreases along with passage from pylorus. In 1835, Curveilheir described the first Brunner gland adenoma.4 The American Institute of Radiologic Pathology uses the terms “Brunner gland hyperplasia” for lesions < 5 mm in size and “Brunner’s gland hamartoma” for lesions > 5 mm in size. It has been suggested that Brunner gland hamartoma is better than adenoma because of the following facts: (1) Absence of dysplasia, (2) lack of encapsulation, and (3) admixture of tissues like ducts, acini, smooth muscle cells, adipose tissue, lymphoid tissue, and Brunner gland within a single pathologic lesion. The most common location for brunneroma is the posteromedial wall of the duodenal bulb (70%), the second part of the duodenum (26%), and the third part of the duodenum (4%). They can be pedunculated or sessile.56Although the small intestine constitutes 75% of the gastrointestinal tract, small intestinal tumors are extremely rare (5%). In the small intestine, duodenal tumors are more common than jejunal and ileal tumors. Adenomas are most common duodenal tumors including adenomatous polyps and Brunner’s adenomas.Helicobacter pylori is a postulated etiological agent.7 Smaller tumors can be asymptomatic; larger ones present with gastrointestinal bleed (45%) and small bowel obstruction (50%).8 The average size of hamartoma is around 2 cm, but a giant hamartoma up to 11 cm has also been reported. Intussusception is a rare presenting scenario for a brunneroma with less than 200 cases reported in the literature, as was in our case.67 Brunneromas are benign entities; however, there have been reports of malignant transformation in them.910The common age is the 5th to 6th decade of life without any gender predilection. Since Brunner gland secretions protect the duodenal mucosa from acidity of stomach, it is postulated that hyperacidity causes Brunner gland hyperplasia. Thus, 11 Hur et al have described the enhancement pattern in brunneroma as it is isodense to the pancreas on plain scan; it does not show any intense enhancement on arterial phase; thus differentiating it from a carcinoid, hypoattenuating to pancreas on portal phase and homogenous enhancement on venous phase.12 Upper GI endoscopy is performed to visualize the external morphology and ulcerations if any and to take biopsies. However, pinch biopsies are inconclusive at times as they fail to reach the submucosa. A EUS described by Weisselberg is the current modality of choice. Brunneroma appears hyperechoic with occasional cysts (due to dilated ducts) noted entirely in the submucosa, most importantly not involving the underlying muscularis mucosa.13 By demonstrating the benign appearance and the presence/absence of stalk, it can tell whether resection is possible endoscopically or laparoscopically.A high index of suspicion is required while diagnosing brunneroma on imaging. The standard imaging modalities employed include barium meal, ultrasound, CT, EUS, and upper gastroendoscopy. The findings on barium and ultrasound are that of nonspecific benign lesions like stromal tumors, carcinoids, or aberrant pancreatic tissue. A CT scan is done to see the extent of the lesion and determine its relationship to adjacent structures.14Endoscopic polypectomy is performed for smaller lesions. Laparoscopic removal is done in case of large pedunculated lesions. When the second part of the duodenum is involved or when malignancy is suspected, pancreaticoduodenectomy is performed.Although brunneroma is a rare entity, it should always be considered as a differential diagnosis of duodenal polypoidal lesions. Larger lesions can cause duodeno-duodenal intussusceptions and can present with obstructive symptoms. Imaging tells the extent of the lesion and its relation to adjacent structures and complications, and thus narrows down the differential diagnosis.


Belly dancer's dyskinesia (BDD) is an extremely rare manifestation consisting of involuntary and repetitive rhythmic movements of the abdominal wall. These movements cannot be voluntarily suppressed but may be influenced by respiratory maneuvers. Investigations such as spinal cord and abdominal imaging usually fail to reveal any local abnormalities to explain the movement disorder.A 23-year-old male presented with sudden onset of undulating movements of the abdominal wall for the last two months after he took domperidone. There was no associated pain or effect of respiration. The movements used to subside during sleep. His radiological and hematological evaluations were inconclusive. The movements, however, subsided after administration of promethazine and clonazepam.The cause of BDD varies, making diagnosis difficult. One of the causes being drug induced but it has never been reported earlier by domperidone. Also, our report provides a possible way to manage BDD by clonazepam and promethazine. Dyskinesia limited to the axial musculature is an extremely rare phenomenon . When suA 23-year-old male presented to the Movement Disorder Clinic of Institute of Human Behaviour and Allied Sciences, Delhi, India with the complaint of undulating movements of his anterior abdominal wall for the last two months. These movements were sudden in onset and non-progressive in nature. On detailed history, the patient had acute gastroenteritis at onset, for which he took antibiotics and domperidone for two days. These symptoms started three days after the medication.The patient was admitted to the neurology ward for indoor observation, detailed examination and evaluation. These abdominal movements were fast, continuous and voluntarily uncontrollable. He was examined both in supine and sitting position respectively Videos -2. ThereHis brain and spinal cord imaging as well as routine blood investigations were negative for any abnormality or pathological conditions. Chest X-ray and ultrasound abdomen were also negative Figure -2. DiaphBelly dancer’s dyskinesia is an extremely rare phenomenon described as various case reports in the literature, initially described by Iliceto G, et al. . A thoroThe clinical manifestations of BDD include fluttering or rolling of anterior abdominal wall. The movements in BDD are involuntary, repetitive, semi-continuous, sometimes painful, often rhythmic and slow writhing . The movThe onset of BDD is usually gradual and the movements are due to the variable combination of contractions of the rectus abdominis, oblique muscles, paraspinal, and perineal muscles. The pathophysiology of this condition is still unclear. It has been postulated that these abnormal movements are due to a dysfunction of inhibitory spinal interneurons or structural reorganization of local neuronal circuits .BDD has a long list of causes spanning a gamut from post-operative to tumor (Table-1). When the syndrome is caused by irritation of the phrenic nerve, the heart is the most common source of irritation. Such cases result in diaphragmatic flutter synchronous with the systole. When diaphragmatic flutter is present bilaterally, a central origin is more likely. BDD arising from a central origin are usually present during sleep whereas the movements originating from peripheral or spinal origin invariably would subside . PsychogCertain pharmacological agents were found to induce the BDD. In one case, an 80-year-old male developed diaphragmatic flutter after beginning a course of galantamine for the treatment of Alzheimer's disease. This medication was identified as the source of his affliction upon cessation of medication . LinazasDiagnosing BDD is mainly clinical but may be supplemented by fluoroscopy and electromyography. But as the tests are not being standardized, using them for diagnosis is still debatable. Brain and spinal cord imaging, in suspected cases be utilized to rule out the secondary causes.No clinical studies have addressed the treatment options of BDD. Current treatment relies on expert opinion and case reports. Several pharmaceuticals have been utilized in the treatment of BDD. These are mainly of cosmetic concern and are difficult to treat despite numerous pharmacological agents. Diphenylhydantoin is frequently used to treat this disorder, but it is not effective in all the cases of BDD .Diazepam has also shown to be beneficial in treatment of BDD. Haloperidol has also been tried successfully in one case . ClonazeWhen pharmacologic therapies prove ineffective, phrenic nerve block or crushing can alleviate symptoms of unilateral diaphragmatic flutter. Relief of symptoms is instantaneous. It can be ascertained that the intact phrenic nerve, enabling the full function of one hemisphere of the diaphragm, can maintain oxygenation at adequate levels during the patient undergoing either rest or exercise. Interestingly, after diaphragmatic function returns to normal, the symptoms of BDD do not return . RecentlAlthough the horizon of both symptomatology and treatment is expanding, success is not a guarantee in afflicted patients. In summary, due to the varying causes of BDD, many methods of treatments have been tried. Our case provides a possible way to manage the BDD.Belly dancer's dyskinesia (BDD) is difficult to diagnose and often refractory to medical therapy. The exact mechanism is inconclusive. It may also be induced following administration of domperidone. Administration of oral clonazepam and injectable promethazine is suggestive to control the symptoms of BDD.


A 47-year-old patient presented from outside the hospital for evaluation of iron deficiency anemia (IDA). The endoscopic workup suggested a gastric antral subepithelial lesion with an overlying arteriovenous malformation (AVM). Endoscopic ultrasound (EUS) revealed the lesion to be a lipoma. Given the patient’s anemia and blood transfusion requirements, the AVM was treated with argon plasma coagulation (APC). During this treatment, desiccation of fat was noted with a significant decrease in the size of the subepithelial lesion (the gastric lipoma). While the APC therapy was intended for management of the overlying AVM, it resulted in the partial dissolution of the gastric lipoma, proving to be a potential diagnostic and therapeutic tool. Lipomas constitute about 3% of gastrointestinal tumors with a predominant location in the colon 60-75%) and the small intestine (30%) . The occ% and theA 47-year-old man was referred to our facility for evaluation and management of iron deficiency anemia (IDA). He had no history of melena, hematemesis, or bright red blood per rectum. He denied any recent diarrhea and intolerance to gluten. No family history of celiac disease or colon cancer was reported. There was no history of shortness of breath or chest pain. He was recently started on iron supplements. There was no report of non-steroidal anti-inflammatory drugs (NSAID) use, alcohol or any illicit drugs. He denied a history of previous abdominal surgeries and a history of bleeding diathesis. On physical exam, this middle-aged Caucasian male appeared very pale. Oxygen saturation on pulse oximetry was 97%, blood pressure (BP) was 112/75, heart rate was 82/min, and temperature was 98.4 degrees Fahrenheit. He was alert and oriented times three.  Conjunctival pallor was noted. Pupils were about 4 millimeters (mm) bilaterally and reactive to light. On auscultation, he had dual heart sounds with no murmurs noted. Breath sounds were bilaterally equal with no crackles or rhonchi. The abdomen was soft, non-distended with no masses noted. On laboratory testing, his white blood cell count (WBC) was within normal limits at 9.6 g/dL, a hemoglobin of 9.5 g/dL, and a platelet count of 162,000. Electrolytes were within normal limits. He had an endoscopic workup done outside the hospital before being referred to us. Upper endoscopy (EGD) performed at the outside facility showed a gastric antral subepithelial lesion with overlying erosions, which were considered to be the source of his occult bleeding.  Gastric biopsies were negative for heliobacterium pylori and showed features of chronic gastritis with no features of atrophy or intestinal metaplasia. The colonoscopy and capsule endoscopy were reported as normal from the same facility.Video To our knowledge, this is the first case describing the simultaneous treatment of a lipoma and an AVM with APC in the gastrointestinal tract. Endoscopically, gastric lipomas are seen as subepithelial lesions among other causes, such as gastrointestinal stromal tumors (GIST) and leiomyomas . They haArgon plasma coagulation (APC) has been available in flexible endoscopy since 1991 and used in the treatment of various conditions, such as hemorrhage, malignant and benign tumors, tissue ingrowth, overgrowth of stents, and angiodysplasia . The supThough this case showed resolution of the lipoma due to the application of APC, there are potential limitations. Due to the thermal nature of APC, aggressive application for lipomas could potentially lead to deep thermal injury to walls and perforation . The useThrough this case, we demonstrate that APC could be used in the treatment of AVMs and gastric lipomas when they co-exist. APC could be used as a possible diagnostic and therapeutic adjunctive management strategy for symptomatic gastric lipomas when endoscopic removal is not feasible. The risk of thermal injury and potential perforation is a possibility. Debulking with APC can also be a considered in symptomatic lipomas without co-existing AVM's in high-risk surgical candidates, although future studies are needed to confirm this.


Fibroepithelial polyps of the urinary tract are rare tumors, and their occurrence in the upper urinary tract is highly unusual.This study reports a 9-year-old boy who presented to our clinic with complaints of unilateral flank pain and macroscopic hematuria. The direct urinary system graph did not show stone formation; therefore, magnetic resonance urography was performed. This revealed a filling defect in the left proximal ureter. On cystoscopy, a polyp was seen in the orifice of the left ureter, extending along the ureter. The polyp was resected by laser ablation and removed from the ureter. Histopathologic examination revealed a fibroepithelial polyp comprising fibrovascular stroma covered with transitional epithelium.Although extremely rare, a fibroepithelial polyp should be considered in the differential diagnosis when a young patient presents with flank pain and macroscopic hematuria. Endoscopic procedures may be the treatment of choice for polyps located in the upper ureter. Fibroepithelial polyps (FEPs) are rare, benign, non-epithelial tumors of mesodermal origin that are located in the urinary system. They originate in the stroma, which consists of mesodermal and normal transitional epithelial cells. Typically, FEPs are found in the upper urinary system of adolescent patients. The mean diameter of all reported FEPs is less than 5 cm, and they occur as plain, mobile, and pedunculated masses . AlthougA 9-year-old boy presented to our clinic with fever, nausea, vomiting, and colic pain on his left side. Physical examination revealed sensitivity in the costovertebral angle and ureteral points. Urinalysis indicated macroscopic hematuria. The results of the biochemical examination were normal; however, ultrasonography (USG) revealed grade 2 dilatation in the pelvicalyceal system of the left kidney. The patient was suspected with ureteric stone, and a direct urinary system graph was obtained during pain control with analgesia. However, this did not reveal any stone formation. Therefore, magnetic resonance urography was performed; this revealed a filling defect in the left proximal ureter , which wFEPs are rare tumors and are generally located in the distal ureter. Their occurrence in the UPJ is highly unusual . The treThe etiology of benign ureteral polyps is uncertain. They are thought to be a congenital abnormality acquired or caused by an infection, chronic irritation, obstruction, or trauma . Althoug


We present a patient with giant bilateral perinephric masses favored to represent liposarcoma preoperatively. Bilateral renal involvement posed a clinical challenge; careful histologic assessment and surgical planning allowed preservation of renal function. A 52‐year‐old Caucasian man with multiple sclerosis presented in August 2014 for worsening chronic abdominal distension with associated lower extremity edema. These symptoms began in 2010 at which time radiologic work‐up revealed bilateral, large retroperitoneal masses. An initial biopsy of these masses was reportedly benign, and hence, the patient did not pursue further intervention. Over time, he developed complications related to progressive enlargement of the lesions, including multiple hospital visits for lower extremity cellulitis in the setting of chronic venous stasis ulcers. In 2014, he presented to our institution with progressive abdominal distension with associated severe dyspnea and limited mobility .A CT of the abdomen and pelvis revealed massive bilateral, well‐encapsulated, fatty retroperitoneal masses encasing both kidneys, compressing the vena cava, and measuring 38.1 × 24.4 × 18.6 cm on the left and 35.7 × 23.5 × 14 cm on the right  would be the most frequent diagnosis. A dedifferentiated liposarcoma with inflammatory myofibroblastic tumor‐like features is a variant wherein the dedifferentiated component is virtually identical to an inflammatory myofibroblastic tumor The increase in IgG4 plasma cells raised the possibility of an IgG4‐related sclerosing process. On histologic grounds, such diseases are characterized by both an increase in IgG4 plasma cells, storiform fibrosis, and obliterative phlebitis Retroperitoneal lipomas are an extremely rare finding and only diagnosed after thoroughly excluding liposarcoma on morphologic and molecular genetic grounds. There have been at least 40 cases in the English literature; some presented with giant abdominal tumors Due to the presentation of bilateral massive perinephric masses with a bland adipocytic component, the lesion warranted a diagnosis of a well‐differentiated adipocytic neoplasm. This diagnosis conveys that the lesion is not malignant but also implies the possibility of local recurrence.Given the benign pathology of the left‐sided mass, the right perinephric mass was not resected initially. Six months after his first resection, the patient reported recurrent symptoms of lower extremity edema and dyspnea. Repeat CT scan at that time revealed mild progression of the right‐sided mass with worsening hydronephrosis. The patient was scheduled for resection with the intent of kidney preservation or autotransplantation, if the kidney could not be safely dissected from the mass in situ. At operation, the kidney was successfully mobilized from the mass, which was removed intact Fig. . Final pNone declared.AGPA: involved in manuscript write‐up  and contributed gross photographs and microscopic pictures; EKB: involved in manuscript write‐up  and contributed radiologic images; PJZ and KC: performed manuscript review, differential diagnosis, revisions, and editing; AN and RER: performed manuscript review and editing.


Epithelioid trophoblastic tumor (ETT) is a rare gestational trophoblastic neoplasm of chorionic-type intermediate trophoblasts, and it is most frequently located in the lower uterine segment and endocervix. Due to the epithelial-growth pattern with geographic necrosis exhibited by the neoplastic cells, ETT is commonly confused, both clinically and pathologically, with squamous cell carcinoma. Although there have been no previous reports of ETT focusing on computed tomography (CT) or magnetic resonance imaging (MRI) findings, we report a case of uterine ETT with special attention to the MRI findings referring to the pathological findings and MR images of previous reports. A 42-year-old Japanese woman  presented with uterus enlargement during screening, and complained of recent-onset lower abdominal pain. The MRI showed a solid tumor throughout the entire myometrium of the lower uterine segment, with the hemorrhagic cystic portion extending to the posterior subserosal space. Following hysterectomy, the final pathological diagnosis was ETT. An ETT is essentially a solid tumor composed of intermediate trophoblasts that exhibit an epithelial-like growth pattern and contain geographic necrosis with calcification. In our case, MRI revealed a non-specific-intensity solid tumor in the lower uterine segment with massive necrosis and hemorrhage extending to the subserosa. While it is difficult to distinguish between ETT and uterine carcinomas, recognition of certain tumor shapes and necrosis could enable more accurate diagnosis before treatment. As the neoplastic cells in ETT exhibit an epithelial-like growth pattern with geographic necrosis, ETT is commonly confused, both clinically and pathologically, with squamous cell carcinoma.4Epithelioid trophoblastic tumor (ETT) is a rare gestational trophoblastic neoplasm of chorionic-type intermediate trophoblasts.An ETT is generally a slow-growing malignant tumor, though some cases have had aggressive and fatal clinical courses involving multiple metastases. Over 100 cases have been reported so far, many of them in clinical or pathological reports. No previous report has focused on the CT or MRI findings associated with ETT. Referring to pathological findings and MR images of previous reports, we report a case of uterine ETT with special attention to the MRI findings.A 42-year-old Japanese woman, gravid 1, para 1 , presented uterine enlargement in screening. She complained of recent-onset lower abdominal pain and a few months’ history of urinary incontinence and constipation.In a pelvic examination, the uterus and left ovary presented as a fist-size lump. There were no abnormal findings in the vulva or vagina. Because the portio could not be clearly identified, endometrial smear test and uteroscopy were not possible. Serum tumor markers including carcinoembryonic antigen (CEA), CA125, CA19-9, and squamous cell carcinoma (SCC) were all within the normal range. Human chorionic gonadotropin (hCG) was not measured prior to operation.2-weighted images (T2WIs) [repetition time (TR)/echo time (TE) = 1300/90 ms], isointense on T1-weighted images (T1WIs) (TR/TE = 500/12) and heterogeneously enhanced on gadolinium-diethylene triamine pentaacetic acid (DTPA)-enhanced T1WI. It appeared hyperintense in diffusion-weighted images (DWIs) (b = 1000), with an apparent diffusion coefficient (ADC) value of 0.9 × 10−3 mm2/s. The subserosal cystic lesion had an irregular wall and septum, and protruded into the solid lesion of the posterior wall like an ulceration. The contents of the cystic lesion appeared hyperintense on T2WI, T1WI, and fat suppression T1WI, and included T2WI-hypointense precipitated material. Slightly solid components were present on the inner surface of the cystic wall. There were no findings indicating adenomyosis in the myometrium of the uterus, and no abnormal findings in the endometrium and left ovary. The right ovary was not detected. To summarize, the tumor was a solid mass of non-specific intensity replacing the myometrium of the lower uterine segment with a large subserosal hemorrhagic cystic lesion; unfortunately, however, the solid mass had not been observed initially. The operation was performed as for an endometriotic cyst of the ovary or peritoneum.Ultrasound revealed a cystic mass 125 × 75 mm in diameter behind the uterus; this was interpreted as an endometrioid cyst. The MRI showed a solid lesion extending throughout the myometrium of the lower uterine segment and an irregular large cystic lesion filling the pelvic cavity behind the uterus . The solIntraoperative observation revealed a subserosal large mass , and a hPathologically, the carcinoma specimen was a solid yellow mass involving the entire thickness of the myometrium in the lower uterine segment . There wA follow-up CT 3 months after the operation revealed multiple lung metastases. Chemotherapy and CyberKnife were administered to treat these. Today, more than 4 years after the operation, the patient is alive and still undergoing treatment.5 there are three clinical or pathological reports that contained MR images.7 This report aimed to identify some keys to the presurgical differential diagnosis of ETT, namely, CT or MRI findings that would be incompatible with a diagnosis of uterine carcinoma, referring to pathological findings and MR images of previous reports.ETT is a rare disease, so few studies have reported on the radiographic findings seen in uterine ETT. Although there is no previous report focused on CT or MRI findings, and only PET-CT findings has been reported,The present case consisted of a solid tumor in the lower uterine segment containing massive necrosis and hemorrhagic matter and extending to the subserosa. The solid tumor had nonspecific signal intensity on MRI.2WI was available for two cases and enhanced image was available for one case. A case reported by Noh et al. showed T2WI-hyperintense solid tumor with cystic hemorrhage into subserosa.6 It was quite similar findings to those in the present case. Kara et al. have reported a case of uterine ETT diagnosed by fractional curettage, it was difficult to detect tumor as its intensity was similar to that of a normal endometrium.5 Last case was well-circumscribed tumor containing hypoenhanced area reported by Okumura et al., and it was diagnosed ETT with necrotic cell debris.7In three cases containing MR images, T2WI as a non-specific malignant tumor.6 Generally, in its outward appearance, ETT is essentially a white solid mass. Histologically, it is composed of nests and cords of intermediate trophoblasts, shows an epithelial-like growth pattern, and contains characteristic geographic necrosis with calcification.2 This MRI finding is consistent that ETT does not contain specific-intensity component such as fibrous tissue and abnormal finding. Accordingly, it was difficult in this case, as in similar cases, to distinguish between ETT and uterine carcinoma based on signal intensity data only.In three cases, including the present case, the signal intensity of ETT shows hyperintense on T14 Shen et al. have reported nine cases of ETT, seven of which had extensive massive necrosis and two of which had focal necrosis in the absence of chemotherapy.8 Lo et al. reported a 9-cm ETT extending to the right pelvic side wall, with an extensive area containing necrotic matter.9 Liang et al., Noh et al., and Fadare et al. have reported four cases of large cystic lesions containing hemorrhagic material and necrosis, as in this case, and some of their cases had fistulas in the uterine cavity.11 In the present case, the fistula was narrow and was associated with no inflammation or foreign body reaction along its length; an inflow of menstrual blood through it was therefore considered unlikely.In addition, the present case had massive necrosis and hemorrhage. Since the first report on ETT by Shih and Kurman, many cases have been reported to contain various amounts of necrosis and hemorrhage, which appear to be independent of tumor size. In fact, necrosis and hemorrhage seem to have fairly common characteristics of ETT.Retrospectively, the differential diagnosis of uterine ETT is similar to that of subserosal cystic lesions as well as endometriotic cyst on an ovary or the peritoneum and cystic adenomyosis. The existence of uterine tumors and the absence of adenomyosis in the myometrium, however, are not typically associated with these other diagnoses.Although it does contain some calcifications in the geographic necrosis, these are apparently too small and too low in density to be recognized on CT. In our case, pelvic CT was not performed before the operation, but we were unable to find any calcifications in the metastatic lung tumor on follow-up CT.17 but sometimes presents as an invasive growth. Several shapes of ETT have been reported, including solitary nodules of the myometrium,12 a solid mass replacing the myometrium,6 polypoid lesions extending into the uterine cavity,11 and irregular lesions along cesarean section scars.3 Accordingly, it might be difficult to distinguish ETT from uterine carcinomas and intramural uterine myomas based on shape alone. Especially in the case of a tumor extending into the uterine cavity, a differential diagnosis cannot be successfully made. A circumscribed intramural uterine lesion with malignant tumor intensity on MRI, on the other hand, is unlikely to be a common uterine carcinoma, excepting those based upon ectopic endometrium. Though it is not a specific finding, the frequent appearance of necrosis and hemorrhage in conjunction with ETT merit their consideration in the differential diagnosis of uterine carcinomas.Turning to the shape and macroscopic growth pattern of uterine ETT, it usually presents as a circumscribed mass of various size, the maximum tumor diameter ranged from 0.5 to 14.8 cm,ETT is a rare disease that is not often seen in clinical practice. There is as yet no standard treatment for it; rather, it is treated as a gestational trophoblastic neoplasm. The ability to diagnose ETT accurately prior to treatment would be useful. Though ETT’s non-specific intensity on MRI contributes nothing to the diagnostic process, gross necrosis and hemorrhage are not uncommon, to the best of our knowledge. Therefore, whereas it may be difficult to diagnosis ETT, if there is an intramural lower uterine mass with massive necrosis and hemorrhage, ETT is considered to one of the differential diagnoses of uterine carcinoma. This is only one case report referencing previous clinical reports, and further reports in more patients are needed.


An insulinoma is a functional neuroendocrine pancreatic tumor, and surgical resection is indicated. Robot-assisted laparoscopic surgeries have been shown to be generally safe and feasible for treatment of pediatric cases of urologic and digestive disease.In July 2016, a 9-year-old girl  was admitted with a pancreatic tail insulinoma and underwent robot-assisted spleen-preserving laparoscopic distal pancreatectomy. The total procedure time was 155 min, and the blood loss was about 10 ml. The patient recovered without complications.This case supports that robot-assisted spleen-preserving laparoscopic distal pancreatectomy may be safe and feasible in pediatric insulinoma patients. Insulinomas are functional neuroendocrine pancreatic tumors that are mainly treated via surgical resection. The traditional open surgeries have been largely replaced by minimally invasive surgeries, including laparoscopic surgery and robot-assisted laparoscopic surgery (RAS). The Da Vinci surgical robot  offers the advantages of a three-dimensional (3D) surgical field and high-resolution viewing, which allow it to overcome difficulties in complicated surgeries better than conventional laparoscopic surgery. RAS is increasingly being practiced in the treatment of pediatric urologic conditions and digestive disease . HoweverA 9-year-old Chinese girl was admitted to our unit due to episodic fainting attacks associated with sweatiness and decreased consciousness persisting for a year with aggravation for the previous 1 month. Hypoglycemia with a serum glucose level of 2–3 mmol/L was confirmed when the symptoms appeared and was resolved with intravenous glucose infusion. The fasting plasma blood-glucose level (FBG) was 1.84 mmol/L, insulin level was 8.2 mU/L, and insulin/glucose ratio was 4.46. An abdominal dynamic contrast-enhanced magnetic resonance imaging (MRI) scan revealed a tumor at the tail of the pancreas  was inserted 5 cm below the umbilicus. The other three trocars were introduced under laparoscopic watch into the left anterior axillary line at the umbilical level (8 mm), the right anterior axillary line 2 cm below umbilical level (8 mm), and the left midclavicular line 3 cm below the umbilical level , CgA (+), Syn (3+), SSTR2 (−), CK (+), CD56 (+), SSTR3 (+), SSTR1 (+), β-catenin (+), CD117 (-), and Grade 2, with local nerve involvement.Pancreatic insulinoma is an uncommon neuroendocrine tumor with an estimated incidence of 4 per 1,000,000 people worldwide . Most inMost pediatric insulinomas are curable by surgical removal of the lesion . Most inDistal pancreatectomy includes pancreatosplenectomy, spleen-preserving distal pancreatectomy, and vessels-sparing spleen-preserving distal pancreatectomy. The vessels-sparing spleen-preserving distal pancreatectomy can fully retain the physiological structure and function of the spleen to prevent postoperative thrombocytosis, thrombosis, pneumonia diplococcus infection, spleen infarction, spleen abscess, gastric varices, and other complications. It is applicable to benign, small, or well-differentiated tumors on the pancreas tail. However, the pancreas is adjacent to many important organs and vessels, and thus, the practice of this surgery is difficult and high risk.The Da Vinci surgical robot can provide a 3D, clear, and wide surgical field, with a magnification of ×10–15. It can also complete surgical clamping, grasping, cutting, and suturing effectively and accurately with a great degree of simulation and jittering filtration, which shows an obvious advantage of limiting the surgical field during pediatric abdominal surgeries . This teWith the accumulation of experience, robot-assisted laparoscopic proximal pancreatic resection, central pancreatic resection, and pancreatic tumor enucleation are feasible . CompareFor adult patients, trocars are mainly inserted in the upper abdomen with conventional 5-trocar placements as the abdominal wall area is larger. Children are shorter and the area of the abdominal wall is smaller. Thus, the trocars are placed below the umbilical level. For the present case, we placed the trocars below the umbilicus, and the surgical field was in the left upper quadrant. We arranged the auxiliary holes in the left lower abdomen, with arm 1 and arm 2 in left and right lower abdomen, respectively. The cold light source lens was placed in the middle abdomen pubic symphysis. All the procedures were completed through the four holes without unwanted contact of the equipment.We believe that the precondition for successful robot-assisted laparoscopic, spleen-preserving distal pancreatectomy for pediatric insulinoma is an accurate surgical approach, which minimizes the interference of tissue around the tumor. In this case, the tumor was located in the pancreatic body tail, which was about 3.0 cm in diameter, and there was a certain border to the pancreatic tissue. The body and tail of the pancreas lost their normal shape, and the tumor was a localized bulge-like shape and with hyperemia on surface. Thus, local enucleation was not suitable, and the distal pancreatectomy was appropriate.We began using the Da Vinci Robot 5 years ago in 2011. To date, we have accumulated a large number of surgical experiences, more than 500 cases, of which more than 300 cases require pancreatic surgery. The success of this initial experience revealed that the robot-assisted laparoscopic surgeries have the advantages of 3D visual field and flexible operation over conventional laparoscopic surgeries, which lead to a higher chance of surgical success. This case showed that robot-assisted spleen-preserving distal pancreatectomy is safe and feasible for children with insulinoma.


Background: Enteric duplication cysts are congenital malformations that typically affect children in infancy, but can also affect adults. Rarely, these cysts can be complicated by malignancy. We present the first case of retroperitoneal duplication cyst that was complicated by malignancy transformation and managed by robot-assisted excision.Case presentation: A 64-year-old female with a history of a left-sided renal cyst presented with a 4-month history of abdominal pain and fatigue. MRI revealed a bilobed cyst, with components measuring 6.9 × 6.6 and 6.1 × 6.9 cm, which had grown since previous imaging, and hemorrhage in some portions of the cysts, as well as cystic wall enhancement, suggesting a possible malignancy. The patient consented to a robot-assisted partial  nephrectomy. During the procedure, the cystic structure appeared to have grown since imaging, was intimately associated with the hilum, and had a complex vasculature, which prompted us to perform a radical nephrectomy. Grossly, the specimen consisted of a 14.8 cm cystic structure at the superior portion of the kidney, but was not contained within the renal parenchyma. Histologically, the internal mucosa of the cyst showed columnar epithelium with high-grade dysplasia and carcinoma in situ with focal individual cell infiltration into the superficial portion of the inferior part of the cyst. The patient saw a medical oncologist and was instructed to follow up with quarterly imaging to assess for disease progression.Conclusion: Enteric duplication cysts are uncommon entities that can occur in various locations in the body, causing a wide spectrum of symptoms, and are rarely complicated by malignancy transformation. Robot-assisted surgical resection is an option that we have shown to be effective in managing these patients. Presentation can include asymptomatic, abdominal pain, gastrointestinal bleeding, and intestinal obstruction.1 Duplication cysts typically present in children; however, cases affecting adults have also been reported.2 These cysts can also be completely separate from the bowel and have their own blood supply and rarely can be found near retroperitoneal structures.3 A rare complication of these cysts is malignancy transformation, whereby adenocarcinoma has been reported.4 Herein, we present the first case to our knowledge of a retroperitoneal enteric duplication cyst complicated by mucinous carcinoma that was managed through robot-assisted radical nephrectomy.EA 64-year-old female with a history of kidney stones and renal cysts presented with a 4-month history of abdominal pain and fatigue. Her pain was vague and localized to the left lower quadrant. A CT scan without contrast was initially performed, which showed a bilobed cystic structure, with components measuring 6.9 × 6.6 and 6.1 × 6.9 cm associated with the left kidney. These structures had shown interval growth since the last study, 5 years prior. Subsequent MRI  revealedAfter obtaining the patient's informed consent, general anesthesia was induced and the patient was placed in the right lateral decubitus position. Four total robotic ports were placed: 8-mm camera port lateral to the umbilicus, 8-mm left-handed arm several fingerbreadths subcostally just lateral to the midline, 8-mm right-handed arm mid way between the camera port and the left anterior superior iliac spine, and 12-mm assistant port in the midline infraumbilical location. Once the robot was docked, the line of Toldt was incised and the colonic mesentery was reflected off the kidney. The large mass was immediately observed and appeared to have grown since imaging was done 5 months prior. A plane was then made between the mass and colon. The hilum was identified, as was the gonadal, which inserted into the renal vein. One portion of the mass appeared to abut the hilum. A plane was then developed between the lower pole of the kidney and the psoas fascia. We then skeletonized the renal vein, as well as two renal arteries (one of which inserted into the cystic mass). Gerota's fascia was incised and the kidney was then defatted. A fair amount of aberrant vasculature between the kidney and mass was apparent. The mass was then separated from the tail of the pancreas and splenic vasculature, although several parasitic vessels were encountered, which were cauterized. Owing to a combination of the mass size, broad association with the renal hilum, and aberrant vasculature, a radical nephrectomy was decided upon. A 45-mm vascular staple load was first used to ligate the renal artery associated with the mass. The remaining renal artery and vein were then ligated using a 65-mm load. The mass was freed from the surrounding structures and the specimen was removed by using a large EndoCatch bag. The adrenal was not identified during the procedure. Estimated blood loss was 50 mL. The patient did well postoperatively and was discharged on postoperative day 3.in situ with focal individual cell infiltration into the superficial portion of the inferior part of the cyst. These neoplastic components stained positively for PAX8, CDX2, CK7, CK20, P504s, p53, and KIM1. These findings were consistent with a diagnosis of a completely isolated enteric duplication cyst with mucinous adenocarcinoma.Grossly, the specimen consisted of a 14.8 cm cystic structure at the superior portion of the kidney, but was not contained within the renal parenchyma. The cyst was separated by a septum into the superior portion up to 8.2 cm. The lower portion had a size up to 8 cm. Histologically , the intThe patient followed up with a medical oncologist, who advised quarterly abdominal CT scans for the next 2 years. Pathologic specimens and subsequent imaging (CT scan of the chest) suggested no regional or distant spread of disease.1 These cysts typically cause symptoms, some of the more common being abdominal pain, vomiting, and constipation. However, because these cysts can occur in various locations in the body, they may also cause respiratory distress or hematuria, when associated with the thorax or the genitourinary system, respectively.3 Although most cysts present early in childhood, cases occurring in adults have also been reported. Interestingly, in the rare scenario that there is malignancy transformation of the cyst, these cases are known to occur solely in adults.4Enteric duplication cysts are uncommon congenital entities that most often occur in children. These cysts often present in infancy, as larger studies have consistently reported that affected patients are typically around 2 years of age or younger.1 For patients with cysts that have been complicated by malignancy transformation, there is not a standard treatment approach, as some patients have been solely managed by surgical resection, whereas others have, in addition, received chemotherapy.4 Although reported outcomes have been generally favorable across these groups, no standard of care has been established for these patients because of its rarity and variable extent of disease. The patient described in this report did not receive chemotherapy and has been instructed to follow up with quarterly imaging to assess for disease progression.Our patient was known to have a “renal cyst” at least 5 years prior, which was interpreted as a benign finding on imaging. It was not until she presented with pain and fatigue that a follow-up MRI was able to demonstrate some growth in the cyst and cyst wall enhancement, suggesting malignancy. This is the first case to describe a duplication cyst in the retroperitoneum that was complicated by malignancy transformation. It is also the first nonesophageal duplication cyst that has been managed through robotic assistance. In symptomatic cases reported in the literature, surgical resection has been effective in alleviating symptoms.Enteric duplication cysts are uncommon entities that typically occur in children, but can affect adults. These cysts can occur in various locations in the body, causing a wide spectrum of symptoms, and are rarely complicated by malignancy transformation. Management of these patients typically includes surgical resection and potentially adjuvant chemotherapy. Robot-assisted surgical resection is an option that we have shown to be effective in managing these patients.