Esophageal Schwannoma is a rare tumor that represents the least frequent mesenchymal tumor of the esophagus and represents a condition with only a few cases reported in the literatureWe report a 40-year-old female with a 5 years history of gastroesophageal reflux, repeated history of pharyngitis, odynophagia that culminated in progressive oropharyngeal dysphagia to solids. A barium esophagogram revealed a filling defect in the superior and middle thirds of the esophagus. Upper gastrointestinal endoscopy showed a smooth elevated lesion in the upper third of the esophagus, impossible to resect by this mean. An open left cervical approach revealed an 80 × 45 × 20 mm large tumor, which was resected.In general, Schwanommas are rarely found in the gastrointestinal tract (GI), while the great majority occur in the stomach, esophagic is the least common GI form of presentation.The knowledge about a new case, impacts in obtaining more information about the clinical course and surgical treatment of this tumor. Approximately 2% of all esophageal tumors are benign primary tumors . Leiomyo2A 40-year-old female was admitted to our hospital with a 5 years history of gastroesophageal reflux, repeated history of pharyngitis, odynophagia that culminated in progressive oropharyngeal dysphagia to solids. Her medical and family histories were unremarkable.A barium esophagogram revealed a filling defect in the superior and middle thirds of the esophagus . A compuHistopathological examination revealed a benign mesenchymal neoplasm formed by spindle-shaped bundles organized in a palisading pattern with anucleated areas composed of fibrillar material in parallel deposition corresponding to Verocay bodies . There wThis symptomatic mass was decided to be resected with an open left cervical approach. A “J” incision was performed to access to the visceral compartment of the neck until complete exposure of the cervical esophagus was achieved. A 5-cm longitudinal incision was performed in the external lateral esophagus wall, exposing the esophagic tumor within the lumen. The tumor was then luxated and externalized . An 80 ×3Benign nerve cell tumors have been given a variety of names, like Schwannoma, Neurinoma, Neurofibroma, Neurilemmoma and spindle cell tumors. They are the most common type of neurogenic tumor, derived from proliferating Schwann cells. Their origin site is usually the mediastinum . TypicalEver since Chaterlin and Fissore described the first esophageal schwannoma case around 1967 only 30 cases had been described till 2010 .Table 1BThe most common presentation age is between 50 and 60 years, However, Giuseppe et al. reported the case of a plexiform esophageal schwannoma in a child of 11 years old with neurofibromatosis type 2. In addition, there is a mild female predominance, with a male to female ratios ranging from 1:1.6 and 1:2.8 .Diagnosis is made on a histological and immunohistochemical basis. Histologically, esophageal schwannomas are typically characterized by palisading spindle cells, few or absent mitotic figures, and a peripheral cuff of lymphoid cells containing germinal centers with anucleated areas composed of fibrillar material in parallel deposition corresponding to Verocay bodies. Immunohistochemically, tumor cells stain positive for S100, a characteristic marker of Schwann cells. Markers such as CD117 and CD34 will be negative, differentiating it from gastrointestinal stromal tumors (GIST). Smooth muscle cell markers (αSMA), actin and desmin, will also be negative, differentiating it from leiomyomas. Schwannomas are generally treated via surgical resection, but endoscopic resection can be done in lesion less than 2 cm. The surgical approach depends on the location of the lesion within the esophagus, surgeons can use the left cervical approach or the innovative video-assisted thoracoscopy to perform enucleation of the tumor .4We reported a rare case of a benign esophageal schwannoma. The knowledge about a new case, impacts in obtaining more information about the clinical course and surgical treatment of this tumor. The biological behavior of the esophageal schwannoma is in most cases benign, and the appropriate technique surgery depends on the location of the lesion.Authors have nothing to declareAuthors have nothing to declareThe ethical approval for the publication of this case was exempted by our institution because all of the data were collected from clinical records and imaging systems for routine perioperative planningInformed consent was obtained from the patient for publication of this case report and any accompanying images, the corresponding author has it if it is needed.Jorge Aguilar -Frasco Investigation, Writing- Reviewing and Editing. Alejandro Irigoyen, Frida Reyes VisulizationEmilio Sanchez-Garcia Ramos, Alejandro Alfaro-Goldaracena, Rubén Cortes: Conceptualization, Methodology, Writing -original draft preparation, Investigation, Supervision. Alexandra Rueda de Leon, Emmanuel Contreras- Jimenez: Data curation, Writing- Original draft preparation. Jorge Humberto Rodríguez-Quintero, Jesús Morales- Maza, This is not a ‘First in Man’ study and should not be registered.Emilio Sanchez-Garcia RamosNot commissioned, externally peer-reviewed.


In children, lymphangiomas are extremely rare pathologic entities that are characterized by unusual locations. The mesenteric localization is extremely rare in children, and the clinical signs usually mimic an acute abdominal syndrome. For most of the cases, their diagnosis is established by the radiologist, and the main therapeutic option is represented by surgery for lesion removal. We hereby describe the case of a 4 year old girl admitted to the pediatric emergency department for continuous abdominal pain, more intense in the orthostatic position, associated with abdominal distension, nausea, and vomiting. These symptoms raised the clinical suspicion of acute abdominal syndrome. The patient had no previous clinically significant events. Radiologic examination suggested a mesenteric multicystic lymphangioma certified by surgical and histopathological evaluation. No specific targeted therapy is currently available; moreover, no specific criteria for recurrences have been stated. A new approach of infantile lymphangiomas following surgery, regarding the use of specific lymphatic markers panel including D2-40, Prox-1, VEGFR-3, PDGFs, and Ki67 may improve the characterization of such lesions regarding their prognosis, recurrence rate and targeted therapy implementation especially for those with a more aggressive or recurrent behavior. Vascular tumors in children represent a challenge for both the pediatrician and the pathologist. Currently, no certified molecular classification is available, not even for more common vascular tumors such as congenital and infantile hemangiomas, and even less, a molecular characterization of other extremely rare vascular lesions that occur during childhood such as lymphangiomas with different locations. In children, lymphangiomas are extremely rare pathologic entities that are characterized by unusual locations. For most of the cases, their diagnosis is established by the radiologist, and the main therapeutic option is surgical removal of the lesion.Usually, lymphangiomas localization in children interests the head and neck area , althougThe mesenteric localization is extremely rare in children, and the clinical signs usually mimic an acute abdominal syndrome. The first mesenteric lymphangioma ever to be subject to surgical excision dates back to 1802, the procedure being applied by Tillaux , followeDue to the extremely rare occurrence of such lesions during childhood, almost no characterization has been made from a molecular point of view, and moreover, drug-based therapy is limited. Some childhood lymphangiomas are easily misdiagnosed for hemangiomas and are improperly treated using propranolol . Their lTargeted therapy represents the gold standard for several tumor conditions especially for those with malignant phenotype. This kind of therapy has several advantages to conventional ones because it acts specifically to tumor cells preserving the normal cells. It is well-known in that lymphatic endothelial cells (LECs) from tumors are different by molecular point of view compared with those from normal tissues. Prox-1 expression in LECs gives them an immature state. Immature LECs are more sensitive to targeted therapy compared to mature LECs because of their particular phenotype. Thus, specific therapies are needed to be developed. As a paradox, at the time being we do not dispose of a targeted therapy for specifically expressed markers of LECs, neither do we benefit from a large number of scientific studies that are focused on the characterization of lymphangiomas regarding LECs maturation markers as VEGFR-3, Prox-1, D2-40, PDGFR-β, or proliferation index such as Ki-67 , 13. BasWe hereby describe the case of a 4 year old girl admitted to the pediatric emergency department for continuous abdominal pain, more intense in the orthostatic position, associated with abdominal distension, nausea, and vomiting. These symptoms raised the clinical suspicion of acute abdominal syndrome. The patient had no previous clinically significant events.Based on the findings from initial clinical evaluation, the pediatrician recommended an ultrasound. The radiologist described an intraabdominal, multilocular cystic mass with liquid inside Figure , and subThe surgically removed tumor was sent for histopathologic evaluation. Macroscopically, the pathologists identified a 8.9 cm length intestinal fragment, with a 0.5–1.2 cm luminal diameter, that was incorporated in a soft, yellow-translucent mass. When sectioned, there were multiple cysts with a wall thickness that was less that 0.1 cm and a smooth inner and outer surface, filled with a white-milky content Figures .Microscopically, the small intestine fragment presented a lymphatic vessel proliferation containing vascular structures of variable size, diffusely disposed in the entire thickness of the wall, predominantly at the level of the serosa Figures . We consD2-40 staining Figure  certifieThe patient had a favorable postsurgical evolution, without presenting any complications or recurrences in the past year. The patient was evaluated from a genetic point of view and did not present any genetic abnormalities that could certify the occurrence of this lesion.We presented here a case of a multicystic mesenteric lymphangioma, an extremely rare and versatile vascular entity in children. The evaluation of such lymphangiomas in prior case reports has been similar to our own work, with focus given to LECs that line the multicystic lesions.The normal genetic profile of the patient challenged us to critically discuss other hypotheses regarding the origin of lymphangiomas, the LECs maturation status, the role of Prox-1 in lymphangioma pathogenesis, as well as the review of the literature considering the role of VEGFR-3 and PDGFR-β in such lesions. In the future, these two receptors will most probably be looked upon as potential specific therapeutic targets for recurrent lymphangioma treatment.Our results demonstrated the existence of active and immature LECs considering that most of them expressed Prox-1. Until now, the use of Prox-1 in multicystic mesenteric lymphangiomas is sparse in routine practice and this limited use of such marker may explain Prox-1 low impact on therapeutic management of patients diagnosed with lymphangioma . But ProProx-1 is expressed in the first stages of lymphatic differentiation . Prox-1 In vitro conditions showed that these CD133 positive cells, having the identity of progenitor cells, proved themselves to be multipotent, and capable of differentiating into adipose cells, bone cells, smooth muscle cells, and LECs. The CD133 negative cells that were isolated from lymphatic malformations did not express stem cell markers, however, they were characterized by an increased Podoplanin, VEGFR-3, and Prox-1 expression. These data are in concordance with the results obtained by us using immunohistochemical assay. The above mentioned authors conclude that LECs isolated from human lymphatic malformations are characterized by an active status and possess a lymphatic progenitor profile that altogether, could contribute to the clinically refractory behavior pattern that is associated with lymphatic malformations (In 2015, Wu et al. characterized LECs isolated from patients presenting lymphatic malformations as possessing a CD133 positive profile. rmations . ScatterVEGFR-3 is less studied than Prox-1, but plays a major role in LEC differentiation. VEGFR-3 is overexpressed in LECs belonging to lymphangiomas. Norgall et al.  concludeFew papers reported lymphangioma treatment using an intralesional injection of a steroid, bleomicin, and bevacizumab mixture; the duration of these therapeutic procedures was reported to be up to 1 year long and at 3 years follow-up no recurrences were detected . The intIn 2004, Esmaeli et al.  studied A recent study conducted on Prox-1, VEGFR-3, and Podoplanin co-expression demonstrated the phenotypic heterogeneity of Podoplanin positive cells that occurred in myocardial infarction. Interestingly, our study revealed that PDGFR-β and Prox-1 co-expression determines a profibrotic phenotype, a fact that may indirectly explain the development of lymphangiomas through a complex mechanism .We considered this extensive evaluation to be useful due to the rarity of the lesion during childhood, a rarity that resulted in the lack of a molecular analysis and in a limited and controversial therapeutic approach.We hereby described a case of a multicystic mesenteric infantile lymphangioma with an active, immature character that suggests the possible recurrence due to the presence of a cellular and molecular substrate. Until today, no recurrences have been detected for the present case.This study looked at markers of immaturity and active replication, that could help determine the prognostic value of these tumors and may guide the need for post surgical surveillance. Given the fact that these tumors can be in locations that are not amenable to surgery, pharmaceutical options that are targeted to the individual are needed. This paper identified some markers that can be used as targets for specific therapies. Our results support a review of the protocols evaluation applied in lymphangiomas.Written informed consent for the publication of this case report and figures was obtained from the parents. This case presentation carefully respects the statements and was carried out in accordance with the Declaration of Helsinki.SC was the radiologist who described the lesion by echography and MRI. CP performed surgery for tumor removal. RH gave the microscopic diagnosis and wrote the paper. AJ confirmed histopathology and revised the paper. AC and MR are involved in immunohistochemistry and its interpretation, built the discussions, and critically reviewed the final form of the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer MH and handling Editor declared their shared affiliation.


Fistulisation of Meckel's diverticulum in the top of an omphalocele sac is very rare. To our Knowledge, three cases were reported in the literature. We presente in this report a new case of this uncommon presentation. Minor degrees of omphalocele have been known to be associated with vitellointestinal duct (VID) anomalies. The most frequent association is Meckel's diverticulum (MD) . But theth intestinal loop before Bauhin Valve (th day after surgery and meconium was emitted by the anus. The newborn was discharged without complication. Histopathology of the excised specimen revealed wall of small intestine without ectopic mucosa.A one-day old, full term male, was referred with a swelling at umbilical region. Prenatal ultrasonography was not performed. Birth weight was 3500g. In physical examination he had normal morphological features. There was an omphalocele making 6cm with a collar of 4cm, covered with a thin, semi-transparent membrane with fistula draining meconium . The umbin Valve . There wet al. about 5 cases on 2006, there were two cases of a patent VID with a fistula, one case of ileal prolapse and in two cases there was a fistulous communication of the ileum directly with the exomphalos sac [Omphalocele is a midline abdominal wall defect in which a thin membrane surrounds the protruding organs that can include small intestine, liver, bladder, spleen, stomach, uterus and ovaries . It occualos sac . In thesIn summary, if fistula is noted in an omphalocele sac, a co-existing patent VID should be considered. The treatment involves a resection of small bowel including the VID anomalies with end-to-end anastomosis.The authors declare no competing interests.


A 3-year-old, male neutered domestic shorthair cat, presented for acute onset tail paresis. He was diagnosed with a spindle cell tumour at the level of L7-CD1 and treated with course fractionation radiation therapy. Three years following radiation therapy, the cat developed chondroblastic osteosarcoma of the pelvis, suspected to be secondary to radiation therapy. Hemipelvectomy was performed and the cat was treated with radiation therapy for remaining gross disease. The cat was euthanized 127 days post-operatively due to suspected metastatic disease. Development of radiation-induced tumours should be considered as a rare late complication in cats undergoing radiation therapy. Primary bone tumours are rare in cats, occurring with an incidence of 4.9 of 100,000 individuals, with osteosarcoma (OSA) comprising 70–80% of these tumours . These tProposed mechanisms for radiation-induced oncogenesis include genomic instability induced by free radicals and osteodysplasia secondary to osteoblast depletion and impaired vascular supply . RadiatiA 3-year-old castrated male domestic shorthair cat was initially referred to the Ontario Veterinary College Health Sciences Centre (OVCHSC) for evaluation of a 1-week history of progressive tail paresis. The patient was a previously stray cat and had tested positive for feline immunodeficiency virus (FIV). Physical examination performed at the time of presentation was unremarkable. Neurological evaluation revealed normal proprioceptive positioning of all four limbs, with absent cutaneous trunci reflexes on the right side, reduced perineal reflex, and markedly decreased tail tone. The lesion was localized between the lumbosacral junction and third sacral spinal cord segment.Magnetic resonance imaging (MRI) of the lumbosacral spine with contrast  using a 1.5 Tesla device  identified a homogenously contrast-enhancing mass within the spinal canal, which extended from the caudal third of the seventh lumbar vertebral body to the first caudal vertebral body. The iliosacral lymph nodes were mildly enlarged, more notably on the left. The region of the ischium and pubis was not included in the imaging. Fine needle aspirates of the spinal cord lesion were taken. The samples were of fair quality and had occasional clusters of plump monomorphic spindle cells with wispy grey indistinct cytoplasm. These cells had minimal anisocytosis and anisokaryosis up to two fold. Cytological interpretation was a spindle cell tumour with the two main differential diagnoses being a peripheral nerve sheath tumour and meningioma.Thoracic radiographs revealed no evidence of metastasis. Computed tomography (CT) scan of the pelvic region was performed for radiation planning with contrast using a 16-slice CT scanner and identified a healed malunion fracture of the left ischium. The spinal cord mass was not identified on CT. Palliative radiation therapy was elected and the patient was treated with a tapering dose of prednisolone  q24h) for 42 days. Course fractionation therapy was administered at five weekly doses of 6 Gy from L6-Cd7. Total dose administered was 30 Gy and the patient was treated with a 5 fields intensity modulated and image guided technic (IMRT/IGRT). Tail function returned to normal throughout the course of treatment and was fully functional by the third week.Thirty-six months after the end of radiation therapy, the patient returned to the OVCHSC for assessment of a 2-week history of left pelvic limb lameness and 5-day history of obstipation. Physical examination revealed mild discomfort on abdominal palpation and a moderate amount of firm faeces within the colon. A large, firm intrapelvic mass was identified rectally, with narrowing of the pelvic canal. Complete blood count (CBC) and biochemistry profile  were unremarkable. Three-view abdominal radiographs identified a moderate amount of faeces within the descending colon. The previously described oblique fracture of the left ischium was noted. The patient was deobstipated and radiographs were repeated. A mass effect was observed in the pelvic canal resulting in ventral displacement of the colon.A CT scan of the abdomen was performed under general anesthesia pre- and post- administration of iodinated contrast . The patCT imaging revealed a large mass with irregular margins and a hypodense nonenhancing central area . The masCT imaging of the thorax and pelvis pre and post contrast administration was performed 16 days later for staging and surgical planning. The aggressive mass was larger than previously noted with more obvious involvement of the sacroiliac joint. There was mild enlargement of the left medial iliac lymph node. The remainder of findings were unchanged. CT imaging of the thorax identified a focal area of soft tissue density, measuring approximately 0.7 × 0.6 cm, within the cranial sub-segment of the left cranial lung lobe, which was concerning for metastasic disease.A left total hemipelvectomy was elected for mass cytoreduction, and to relieve the obstipation caused by the mass. Under the same general anesthetic episode, epidural anesthesia was provided using a combination of hydromorphone (0.02 mg/kg) and bupivacaine(1 mg/kg). A femoral nerve block was also administered with bupivacaine (0.08 mg/kg). Cefazolin (22 mg/kg IV) was given 30 min before first skin incision and every 90 min thereafter.Sterile gauze was packed into the rectum and a purse string suture pattern was placed within the anus to reduce contamination and increase visualization of the rectum intra-operatively. A urinary catheter  was also inserted to make the urethra more readily identifiable. The patient was positioned in right lateral recumbency and a wide area around the left pelvic limb and pelvis was clipped and aseptically prepared.A semi-circular incision was made on the lateral aspect of the left pelvic limb overlying the middle third of the femur and extended medially. Musculature was transected using a combination of sharp dissection and electrosurgery . The cranial sartorius muscle, quadriceps muscles, and remainder of the lateral muscles of the femur were transected proximal to the patella. On the medial aspect of the limb, the pectineus, caudal belly of the sartorius, and gracilis were transected. The femoral artery was double ligated using 3-0 polydioxanone . The medial circumflex femoral vessels were isolated, ligated, and transected. The amputation was continued on the lateral limb by transecting the biceps femoris muscle. The biceps femoris was retracted, and the underlying sciatic nerve was identified and locally infiltrated with bupivacaine prior to transection. The iliopsoas muscle was transected at its insertion on the lesser trochanter. The semimembranosus, semitendinosus, and the adductor muscles were transected at the level of the proximal third of the femur. The adductor magnus was preserved for closure. The gluteal muscles were transected close to their insertion on the greater trochanter. The external rotator muscles and quadratus femoris muscle were severed at their attachments around the trochanteric fossa.A well-demarcated firm multilobulated mass was found invading into the ilium, crossing the sacroiliac joint, and extending into the surrounding musculature. An oscillating saw was used to cut through the pelvic symphysis. An osteotome and mallet were used to sever the sacroiliac joint and remove the entire hemipelvis. A small amount of gross disease was observed remaining in the sacrum. The surgery site was copiously lavaged with warm sterile saline. Ligating clips  were placed to denote the perimeter of the surgical site for future radiation treatment.The adductor and gluteal muscles were used to close the pelvic defect using simple interrupted sutures with 3-0 polydioxanone. The deep subcutaneous tissues were closed using a simple continuous pattern with 3-0 poliglecaprone . A wound diffusion catheter  was placed within the subcutaneous tissue, exiting dorsally to the incision, anchored with a finger trap suture using 3-0 polypropylene  and infused with bupivacaine(0.85 mg/kg). The skin was closed in a cruciate pattern using 3-0 polypropylene. An adherent dressing was applied over sterile gauzes to protect the surgical site. A soft rubber esophagostomy tube  was also placed.The patient made an uneventful recovery from surgery and was defecating and urinating normally within 2 days post-operatively. He was discharged to the care of his owners at day 4 post-operatively with buprenorphine  for 5 days. Amoxicillin-clavulanate potassium (10 mg/kg q12h) and meloxicam oral suspension (0.1 mg/kg q24h) were also administered through the esophagostomy tube for 8 days and 5 days respectively.The amputated limb, hemipelvis, and left popliteal lymph node were submitted for histopathological analysis. The mass on the pelvic section was microscopically multimodular, partially encapsulated, and well demarcated. Neoplastic spindle cells were arranged in streams producing osteoid and islands with dense central chondroid differentiation. Neoplastic cells had small amounts of eosinophilic cytoplasm, 1 to 2 nuclei, and up to 3 prominent nucleoli. There was three-fold anisocytosis and anisokaryosis with a mitotic figure of 27 per 10 400x fields. Approximately 50% of the mass was necrotic and characterized by hypereosinophilic streams of neoplastic cells with loss of cellular detail and lacunae containing hypereosinophilic cells. The left pelvis contained tissue histologically similar to the previously described mass and was comprised of neoplastic spindle cells producing regionally variable amounts of osteoid, chondroid, and fibrous connective tissue. These features were consistent with chondroblastic OSA. Analysis of the left popliteal lymph node revealed a suspected neoplastic cell embolus in a dilated lymphatic vessel indicative of lymphatic spread of the OSA.Due to the presence of residual disease in the pelvis and suspicion of lymphatic spread, the cat was treated with another course of coarse fractionation radiation therapy for remaining gross disease with 5 daily fractions of 4 Gy, with a total dose of 20 Gy with a 5 fields IMRT/IGRT. A soft opaque nodule superimposed with the cranial lung lobes was noted on thoracic radiographs 82 days post-operatively and was suspicious of progressive metastasis. The patient was euthanized 127 days post-operatively due to declining quality of life from oliguria, obstipation, and reduced pelvic limb function. Abdominal radiographs performed prior to euthanasia revealed ventral compression of the colon by a mass originating from the sacrum.This case report describes a unique occurrence of an OSA, which developed in the pelvis almost 3 years following radiation therapy for a primary spinal tumour. The young age of the patient, occurrence of two separate neoplasm types, site of development, and aggressive nature of the OSA make this case unusual.The pelvic OSA described in this case is suspected to be a result of irradiation of the underlying pelvis while targeting the L7-S1 spindle cell mass. The mass fulfills four of five described criteria of a radiation-induced OSA, namely, (i) it developed from an area within the irradiated field , 13 Fig, (ii) itConfirmation of this hypothesis would require histological assessment of the spinal tumour, however risk of iatrogenic neurological damage precluded biopsy and a necropsy was not elected. The most likely differential diagnoses for the spinal spindle cell tumour based on cytology were, peripheral nerve sheath tumour and meningioma. Both of these tumour types have a low propensity for metastasis and only peripheral nerve sheath tumours display locally invasive behaviour in cats –18. BaseFIV has been reported to increase likelihood of development of neoplastic disease five to six fold . The mosRadical surgical excision is the preferred treatment method for feline OSA and curative outcome can often be achieved with clean margins due to the low metastatic rate , 2, 22. Radiation-induced tumours are a well-recognized entity in humans, and some experimental and clinical reports exist in dogs , 23–25. Hemipelvectomy is indicated in cases necessitating an aggressive surgical approach, as in malignancies affecting the proximal femur, pelvis, or adjacent soft tissue structures . In tumoThis is the first known report of a suspected radiation-induced OSA in a cat. It additionally demonstrates hemipelvectomy as an effective and well-tolerated method for palliative management of tumours when complete resection is not feasible . Based o


Torsion or volvulus of the gallbladder is a rare situation that rapidly progresses to gangrene and linked with a poor prognosis, even death, if unrecognized and untreated. An interesting and rare case of gallbladder volvulus in which diagnosis was obtained by comparing CT images and HIDA scan with SPECT-CT is presented. Relevant literature is reviewed, and recommendations are outlined. Torsion or volvulus of the gallbladder, first described as early as 1898, is a rare entity that may be encountered only once in an entire practice –7.We present an interesting case of gallbladder volvulus in which diagnosis was obtained by comparing CT images and HIDA scan with SPECT-CT. We reviewed the literature and propose some recommendations.An 84-year-old thin and frail woman (BMI = 17.3) with rotoscoliosis, severe osteoporosis, and vertebral fractures presented to the emergency room with back pain. Spine X-ray showed two new vertebral fractures at T7 and L1 as well as a possible ileus. She was afebrile and had no abdominal pain. She was discharged with analgesic.3. Liver function parameters were within normal limits. An abdominal ultrasound showed distension of the gallbladder and lamellated wall thickening up to 9 mm, but no gallstones were seen  may occasionally be useful but invariably lead to further delayCholecystectomy is mandatory when facing clinical and radiologic features of severe and deteriorating cholecystitisLaparoscopy may be a useful and necessary diagnostic modality when the condition of patient deterioratesMost cases may be treated with a laparoscopic cholecystectomy, and good outcome is expected after surgeryConsidering this case and the review of the literature, we can draw the following conclusions and recommendations:Torsion of the gallbladder remains uncommon but its occurrence probably increases as the elderly population grows. Improved imaging techniques allow for more preoperative diagnoses. However, a high index of suspicion remains the mainstay in the diagnostic process. Prompt surgical intervention carries an excellent prognosis.


To present a patient with a diagnosis of pituitary adenoma and progressive visual pathway dysfunction detected in the electrophysiological tests in one-year follow-up. Patient is a 59-year-old male with a non-secreting pituitary macroadenoma.Routine ophthalmological evaluation, standard automatic perimetry (SAP), retinal nerve fibers layer and the ganglion cell complex thickness in optical coherent tomography (OCT), as well as electrophysiological examinations  were performed. The examination and additional tests were conducted 3 times  and 6 months after neurosurgery.Visual acuity, funduscopic examinations, SAP, OCT and electrophysiological test results at the first visit were all normal. In both eyes, the abnormalities were observed only in the multi-channel PVEP and PERG despite the absence of the changes in the routine ophthalmological examination and additional tests after 6- and 12-month follow-up. The tumor growth but without chiasmal compression was confirmed by magnetic resonance imaging. The progression of the optic pathway dysfunction in the electrophysiological tests was a cause of surgical removal of the pituitary tumor.This case highlights novel observations that in patients with pituitary tumor, detection of the early dysfunction of the visual pathway may lead to modification of the medical treatment regimen and reduce the incidence of irreversible optic nerve damage. The most common sellar tumors are adenomas of the pituitary gland. It is well known that pituitary tumors may cause a typical pattern of visual loss, primarily characterized by reduced visual acuity and bitemporal hemianopsia . ChiasmaUp to date, the surgical treatment of the pituitary tumors is usually prescribed when the visual vield is affected .The electrophysiological tests are sensitive enough to detect dysfunction of the visual pathway before the onset of chiasmal syndrome, follow-up of the course of the disease and the effect of a therapy. It is known from the literature that these examinations deliver a more comprehensive evaluation of the visual function and have been reported to be earlier and more severely affected than other ophthalmological tests –5, 7–14.There is still a need to search for diagnostic methods that can accelerate the diagnosis of pituitary tumors before appearance of a loss of nerve fibers of the retina and consequently irreversible changes in the visual field. The diagnosis and management of a sellar lesion involves a multidisciplinary effort, including detailed endocrinological, ophthalmological and neurologic procedures.In this study, we described an example of a patient with a history of macroadenoma without compression of the optic chiasm in MRI and without clinical evidence of the visual impairment in the routine ophthalmological examination and perimetry.A 59-year-old male with a known pituitary adenoma for the past 1 year was referred to ophthalmological examination after an endocrinological consultation with diagnosis of a non-secreting pituitary adenoma.In this case, macroadenoma located mostly on the right side of pituitary gland  without compression of the optic chiasm or optic nerves was revealed by MRI , normal anterior and posterior segment of the eye  and normal color perception (The Farnsworth-Munsell Dichotomous D-15 Test). Retinal sensitivity measured by standard static perimetry  Fig.  were witDue to the absence of ocular symptoms and without clinical evidence of the visual impairment in routine ophthalmological examination and additional testes , it was decided to perform the multi-channel PVEPs and PERG according to ISCEV standards  , 16. Obt2, mean luminance of the stimulus screen equal to 62 cd/m2, contrast equal to 97%; temporal frequency for the contrast reversals equal to 1875 rps (0.938 Hz); central fixation was used, with persistent monitoring. Unipolar recordings were performed; active gold disk electrodes  were placed on the skin at locations O1 and O2, reference electrode  was placed at Fz and ground (gold disk) electrode was placed on the forehead (Fpz). After cleaning the patient’s skin at the electrodes location and placing them using electrode gel , inter-electrode impedance was checked before the recordings were performed; values < 10 kΩ were accepted. Parameters of the recording system were as follows: filters: 1–100 Hz; notch filters: off; artifact reject threshold: 95% of the amplifiers range; sweep time: 300 ms; average 100 sweeps. Two consecutive waveforms were recorded, off-line averaged, and then analyzed. According to the standard, amplitudes of the obtained waveforms were analyzed and especially peak times/amplitude of P100-wave; manual correction was applied to the automatic cursors placement.The tests were performed in normal illumination conditions of the examination room. Patient’s pupils were not dilated, monocular stimulation was used, refraction correction was applied with respect to the eye–screen distance (1 m) and central fixation was applied; interruptions of the test were introduced when frequent blinking or fixation loss was observed (patient was monitored with a TV camera). Parameters of the pattern stimulation were as follows: 21″ CRT monitor with a frame rate equal to 70 fps (frames per second); aspect ratio between the width and height of the stimulus field (screen proportion H/V) equal to 4:3; black-and-white reversing checkerboard  presented to the patient, with a check size equal to 0°16′  and 1°4′ ; luminance for white elements equal to 120 cd/mMonocular stimulation was used, with appropriate refractive error correction in relation to the eye–screen distance. Examination was interrupted when frequent blinking or fixation losses were observed (patient was monitored with a TV camera). The patient’s pupils were not dilated, and central fixation was used. Parameters of the PERG stimulation were as follows: 21″ CRT monitor with a frame rate equal to 75 fps; black-and-white reversing checkerboard (30° FOV) presented to the patient, with a check size equal to 1°2′; temporal frequency equal to 4.6 rps (2.3 Hz), Michelson contrast equal to 97%, and luminance for white elements equal to 120 cd/m2. Ground (gold disk) electrode was placed on the forehead (Fpz), thread DTL electrode was used as active electrode, gold disk was placed at the outer position, canthus ipsilateral used as reference. Parameters of the recording system were amplifiers sensitivity: 20 µV/div, filters: 1–100 Hz, artifact reject threshold: 95% (for the amplifiers range ± 100 µV). Notch filters were off. Average was 200 sweeps. Sweep time was 250 ms (time base: 25 ms/div). Two consecutive waveforms were recorded and then they were off-line averaged and analyzed.According to the guidelines in the literature, for all measured parameters in PVEP and PERG, the intersession variability determined by calculating the coefficients of variation (CV) does not exceed 10% . The CV At the baseline, the results of both electrophysiological tests revealed no abnormalities in comparison to normal values from our laboratory. Table .Table 1MSix months after initial evaluation, the same examinations were performed. There were no significant changes in DBCVA, SAP or OCT results. Also, no abnormalities in PERG were observed. However, multi-channel PVEPs (1°4′ check size) revealed an increase in P100-wave peak time of the RE (O1 122.1/O2 122.8 ms) in reference to high limit of normative data of the laboratory (max. 116.6 ms). Additionally, the P100-wave peak time of the RE became delayed relative to initial values (O1 122.1 vs 108.6 ms and O2 122.8 vs 109.8 ms). The values of P100-wave peak time of the LE remain in normal range (O1 110.3–O2 111.5 ms). The multi-channel PVEPs (0°16′ check size) of the RE and LE revealed no changes. The results from the right eye at 6-month visit suggest dysfunction in the right pre-chiasmal optic nerve .After 12 months of follow- up, the visual acuity, perimetry, or OCT results were still normal, but progression in the electrophysiological tests were observed. The multi-channel PVEPs revealed characteristic crossed asymmetry. The PVEPs (1°4′ check size) detected continuous increase in P100-wave peak time in RE from crossed and uncrossed fibers (O1 128.6–O2 126.2 ms). Additionally, in the LE, the abnormal P100-wave peak time from crossed fibers (O2 129.3 ms) was detected. However, the LE uncrossed fibers recordings remain within the normal range (O1 110.2 ms) Fig. . The PERIn one-year follow-up, the results of the electrophysiological tests suggested the progressive dysfunction in the bioelectrical function of the RGCs and optic nerve. That is why the patient was referred for endocrine and neurosurgical consultation. The dimension of the tumor as noted on control MRI slightly increased (17 × 18 × 16 mm), with no visible compressive effect in tumor growth in relation to optic chiasm. Based only on the electrophysiological tests results, the current treatment was modified, and surgical tumor removal was performed.The ophthalmological examination 6 months after neurosurgery revealed no abnormalities in the routine ophthalmological tests . It is worth noting that the results of the PERG and multi-channel PVEP show improvement in the bioelectrical function of the RGCs and optic nerve relative to preoperative value Figs. , 5, 6. TThe case presented here indicated the validity of the electrophysiological tests in diagnosis and monitoring of the treatment in patients with pituitary tumor. It strongly confirms that the electrophysiological methods may be even superior to routine ophthalmological examination for detecting visual pathway disturbances. Our findings show the progressive significant visual pathway dysfunction was recorded only in electrophysiological tests, despite the changes in SAP and OCT. In this case, the proven progression of the visual pathway dysfunction detected in PERG and multi-channel PVEP was a cause of changes in the treatment strategies of the pituitary adenoma.Tumors compressing the optic chiasm can reduce visual acuity, affect the visual field and cause optic atrophy , 21. AnaBoth PERGs and VEPs have significant role in defining the visual pathway dysfunction in chiasmal compressive lesions. Tumor size and location, and the period of optic pathway compression, can result in a delay or block of nerve fiber conduction, or after long periods of gradual chiasmal compression can progress to nerve fiber loss and to retrograde degeneration of retinal ganglion cells , 23. An In tumors at the optic chiasm, VEPs can verify early chiasmal compression in patients with unreliable visual fields, as also in patients with normal perimetry result. VEPs are sensitive during follow-up, for improvements after medical or surgical therapy, or for deterioration. The recording of PERG is also important to detect possible ganglion cell dysfunction and to provide a prognostic indicator of visual outcome , 10, 11.It is sensible to hypothesize that in our case the improvement in visual pathway function after neurosurgery was probably associated with reduction in the tumor size and its influence on the optic chiasm unseen in MRI, local intracranial hypertension and/or ischemia.The early diagnosis and timely treatment prevent structural damage of visual pathway in this case.The electrophysiological diagnosis is fast, effective, pain-free, and recordable. These tests should be regarded as an integral part of diagnostic procedures in suspected compressive lesions of the chiasm.


Although gout is a common disease, the presence of gouty tophi outside joints is rare and in literature, there is to date only one report of hepatic tophaceous nodule. We would like to highlight here the difficult diagnostic workup in a patient with history of cancer and the presence of a tophus inside the liver. Moreover, we address the possible etiologic role of chronic inflammation related to tophi and liver cancer.We present the case of a 72-year-old man with a localization of gouty tophus in the liver, who thereafter developed a hepatocellular carcinoma (HCC) in the same site. The patient was followed up after surgery for left renal cancer from 1992 to 2011, when a hepatic nodule was discovered for the first time. After a detailed evaluation, the nodule was classified as a urate tophus of the liver. However, further follow-up showed that the nodule increased in size and changed its characteristics, bringing to the diagnosis of HCC.With the present case report, we would discuss the possible neoplastic degeneration of a gouty tophus and its etiologic role favouring cellular degeneration linked to chronic inflammation. We would also highlight the importance of histopathological evaluation of hepatic lesions in gouty patients at high risk of liver neoplasm, due to the difficulty in characterizing gouty tophi by imaging. At the best of our knowledge, there is only one other case in literature reporting the presence of gouty tophi in the liver. With this report, we would like to highlight the difficult preoperative diagnostic pattern in a patient with a previous history of neoplastic disease and at risk of liver cirrhosis, in whom a hepatic tophaceous nodule was found out. Moreover, we would like to investigate the possible relationship between gouty tophi in the liver with its associated chronic inflammatory reaction and the pathogenesis of hepatocellular carcinoma.In the presence of focal liver lesion, a detailed history, physical examination, radiological tests and pathology are required in order to obtain a diagnosis . In thisD.M., a 72-year-old man, was admitted to our surgical unit on June 2014 with a radiological diagnosis of a suspected malignant lesion of the liver. In the clinical history: arterial hypertension, chronic renal failure and gout. He was obese (BMI 30) with a history of chronic alcoholic abuse. Among the surgical antecedents, a subtotal gastrectomy for peptic ulcer and a complex surgery for left renal cancer  in 1992, at the pathological examination, it revealed to be a pT3N0 well-differentiated renal adenocarcinoma, with neoplastic caval thrombosis.After the surgery, he attended a regular follow-up, that was negative till April 2011, when the abdominal CT revealed the presence of a solid focal lesion in the eighth liver segment size 2.3 cm) characterized by poor vascularization and fatty component; alpha-fetoprotein was negative. According to CT, the hepatic lesion was classified as an indeterminate nodule . We took charge of the patient for the first time on May 2014. At this time, MRI showed exactly in the same area previously affected by the gouty lesion a solid nodule of 5.4 cm in size, characterized by irregular structure and rich in adipose tissue, suggestive for hepatocellular carcinoma with mosaic pattern; no evident cleavage was noted between the nodule and the middle and right hepatic veins  and the site (adhesion to two suprahepatic veins) of the nodule, only surgery was considered as a radical approach. We tested the liver function and we found out a liver remnant volume after right hepatectomy of 20% with indocyanine green test (ICG) at 15′ of 16%). For these reasons, we preferred a conservative approach, also considering the good biological features of the lesion (expansive nodule with a complete capsule and favourable grading).On June 4, 2014, we performed the resection of the VIII and the V hepatic segment; it was possible to spare both the hepatic veins Fig. . The posThe histological examination of surgical specimen confirmed the presence of encapsulated hepatocellular carcinoma with favourable grading. The non-neoplastic hepatic parenchyma was characterized by 10% of steatosis without evidence of alcoholic cirrhosis. The suspected satellite nodules observed at the macroscopic examination of the specimen just at the border of the lesion revealed to be necrotic tissue including many needle-shaped structures typical for gouty tophi Fig. .Fig. 6a–The patient has been followed up by our periodic checks since today and he is free from neoplastic disease; otherwise, no other systemic localization of tophi was discovered.Gout is a systemic disease that results from the deposition of monosodium urate crystals (MSU) in tissues. Increased serum uric acid above a specific threshold is a requirement for the formation of uric acid crystals, but other factors are needed, as only 5% of people with hyperuricemia above 9 mg/dL develop gout . The preThe presence of gouty tophus in the liver could represent a problem in patients at risk for cancer, when instrumental follow-up with ultrasound, CT scan or MRI is needed; the presence of a granuloma can actually give some difficulties in the differential diagnosis. Gout has been described as a “great mimicker” because it can deposit in unusual sites and mimic tumors and intraosseus gout can simulate metastatic disease –17. ConvOur case has a distinct peculiarity, linked to the growth of a primary cancer in the site of a tophus. No definitive proof arises from our case regarding a direct oncogenic effect of gouty tophus, even if we can suppose that the chronic inflammation related to the tophus pathogenesis could represent a factor involved in carcinogenesis. Even if the neoplastic transformation of a tophus into hepatocellular carcinoma has never been reported, we can find in literature cases of malignant transformation of gouty trophi. In all cases, it concerns tumors of mesenchymal tissues  but it dOn the other side in the specific case, we also have other risk factors that could have contributed to carcinogenesis: the metabolic syndrome together with the alcoholic liver disease could have favoured the aetiology of HCC. We can state that more than one etiologic pattern was involved in the specific case, but all these mechanisms are characterized by the presence of chronic inflammation inside the liver.The value of our observations in the clinical practice may be double. At first, we would highlight that a rapid enlargement and changes in the characteristics of a tophus may be considered with suspect for its possible neoplastic degeneration, as this is the morphologic evolution in almost all the cases reported in the literature , 22. At


A poorly feeding neonate presents the clinician with a diagnostic challenge. Feeding difficulties and irritability may be due to sepsis, congenital heart disease, inborn errors of metabolism, non-accidental head trauma, as well as a vast variety of other pathologies. Teratomas are rare pediatric tumors that can occasionally present in the immediate neonatal period and can manifest in the infant's central nervous system (CNS) with non-specific symptoms of poor feeding, lethargy, and somnolence. Operative resection remains the cornerstone of treatment; however, there is no well-defined role for adjuvant chemotherapy or radiation in these treatments. We report a case of a four-week-old female presenting with progressive feeding intolerance secondary to a near holocord thoracic spinal teratoma. Her tumor was surgically resected and she was treated with adjuvant chemotherapy and radiation for 13 months and is now in clinical remission. While rare, intramedullary spinal cord lesions should be considered in the differential diagnosis of infants presenting with poor feeding and hypotonia. A poorly feeding neonate presents the clinician with a diagnostic challenge. Frequently, feeding difficulties and irritability are attributed to sepsis, a common presentation in the immediate neonatal period that can present without other classic signs such as fever or temperature instability ,2. HowevA four-week-old female born at term via unremarkable spontaneous vaginal delivery presented with a one-week history of irritability, poor feeding, and progressive somnolence. Prior to the onset of symptoms, her newborn period was unremarkable with good appetite, growth, voiding, stooling, and weight gain. Per parental report, she had a normal neurological exam in the nursery and at her newborn and two-week well child evaluations. She then began to have progressive feeding difficulty, becoming very irritable with feeds. She also became irritable with any attempted movement of her upper extremities. There were no fevers or hypothermia noted at home. Family and social histories were noncontributory.On physical examination, she was afebrile with a heart rate of 130 beats/minute, respiratory rate of 40 breaths/minute, and irritable with any attempts at examination. Her head was normocephalic and her fontanel was soft and non-bulging. Her cardiac exam was without murmurs, her lungs were clear bilaterally, and her capillary refill was less than two seconds. Neurological examination was notable for absent bilateral Moro reflexes and decreased bilateral upper extremity grasp reflexes. Emergent computed tomography of her head was negative for an acute intracranial process. She was admitted to the inpatient ward where a lumbar puncture yielded slow-flowing, grossly xanthochromic fluid containing 132 nucleated cells with a normal differential. Ampicillin and cefotaxime were started. Blood, urine, and spinal fluid cultures were negative. She remained irritable and, over the next 12 hours, developed progressive hypotonia and areflexia of her bilateral lower extremities. Magnetic resonance imaging (MRI) of her brain was subsequently performed, which was also negative for acute intracranial pathology but demonstrated signal enhancement in the proximal cervical spinal cord  teratomas are exceedingly rare. Teratomas make up approximately 3% of all pediatric tumors and 0.1% of all CNS tumors ,5. Of thIntramedullary spinal cord lesions should be considered in the differential diagnosis of infants presenting with poor feeding, irritability, and hypotonia. A high index of suspicion is necessary for their diagnosis. Given that focal neurological findings can be subtle in infants, a thorough initial and repeated neurological examination should be a cornerstone of physician evaluation.


Gastrointestinal schwannomas are submucosal tumors accounting for 2–7% of mesenchymal gastro-intestinal neoplasms; the stomach being the most common site. Esophageal schwannomas are more frequent in women, and are usually located in the upper to mid portion. Dysphagia is the main presenting symptom. A definitive diagnosis requires confirmation by histopathological and immunohistochemical studies.A 50-year-old healthy lady, presented with gradual increasing onset of dyspnea, with minimal dysphagia to solid food, over a period of several years. Enhanced CT scan of the chest revealed a well-defined soft tissue mass arising from the proximal third of the esophagus, measuring 7.8 × 5.4 x 10.5 cm. Esophagogastric endoscopy with ultrasonography showed an elevated, smooth surface lesion, arising from the submucosal layer of the esophagus, with a hypervascular mucosa. Enucleation of this large tumor, with preservation of the overlying mucosa, was difficult to accomplish due to its large size.Making use of a dilated proximal esophageal segment, total en-bloc excision of the mass rendered a 15 cm esophagotomy gap, which was easily closed, in two layers, without affecting the overall caliber thus achieving a good esophagoplasty result.Histologically, abundance of spindle-shaped cells with positive S-100 proteins, confirmed the diagnosis of esophageal schwannoma.Variations in mesenchymal gastrointestinal tumors is vast, rendering diagnosis by radiology alone difficult. As such, characteristic histologic and immunostaining features are cornerstones in precise diagnosis of esophageal schwannomas. Despite being rare in incidence, symptomatic esophageal schwannoma lesions can be excised entirely, with low rate of recurrence and favorable overall outcomes. Esophag2A 50-year-old healthy lady, case of bilateral partial mastectomy for high grade ductal carcinoma in situ, presented in 2014 with progressive exertional dyspnea, with minimal dysphagia to solid food, over a period of several years.An enhanced CT scan of the chest revealed a well-defined soft tissue mass arising from the proximal third of the esophagus, measuring 7.8*5.4*10.5 cm in its maximal transverse, AP, and CC dimensions, respectively, with prominent superior mediastinal lymph nodes, the largest measuring 7 mm . An uppeThrough a right posterolateral thoracotomy incision, the pathological portion of the esophagus was identified. The proximal portion of the esophagus was dilated due to the chronic partial obstruction caused by the mass.Enucleation of the mass was impossible as the layer between the mucosa and submucosa could not be developed safely. Taking advantage of a dilated esophageal segment proximally, en bloc excision of the tumor was performed preserving enough width of esophageal wall. The width of the remaining esophagus measured 8 cm uniformly along the 15 cm longitudinal defect caused by the tumor excision. The 8 cm width allowed the reconstruction of the 15 cm longitudinal defect of the esophagus without undue narrowing of the lumen . The esoGrossly, a 9.5*7.0*3.0 cm firm mass, covered with normal mucosa having a focal ulceration, was identified . The masGastrograffin swallow, performed five days later, revealed no evidence of contrast leak. Diet resumed, gradually thereafter, and advanced as tolerated. She was discharged home after a smooth postoperative course. The patient has been followed up for approximately 4 years, without any evidence of recurrence nor complications.3Esophageal schwannomas are categorized under neurogenic tumors. The latter are classified according to Ranson’s histopathological classification, published in 1940, as either nerve sheath tumors or neuroblastic tumors of the sympathetic system . SchwannAmong the most common malignancies in the esophagus are the squamous cell carcinomas. Tumors that originate from mesenchymal cells, such as leiomyomas, leiomyosarcomas, gastrointestinal stromal tumors, and schwannomas, are uncommon inside the esophagus ,9.Schwannomas of the esophagus are frequently developed more in females than males, with a ratio of 4 to 1 , especiaSchwannoma of the gastrointestinal tract are submucosal tumors, commonly covered by normal mucosa and principally involving the submucosa and the muscularis propria . HistoloA preoperative including radiologically-alone diagnosis of this entity is difficult to achieve, and often established, after surgical resection .As a rule, most benign mesenchymal tumors of the esophagus arise from the submucosa layer, and can be enucleated without destruction of the internal mucosa, and with preservation of good swallowing function. On the contrary, malignant neoplasms generally require more extensive resection and reconstruction; so the pre-emptive diagnosis is important in decision making, before any definitive treatment is undertaken .Enucleation, using either video-assisted thoracoscopic surgery or endoscopically, is becoming the approach of choice for small submucosal tumors, measuring less than 2 cm in length ,14. ReceEsophageal schwannomas can significantly increase in size, reaching up to 15 cm, as reported in the literature, without severe symptoms of dysphagia and dyspnea. In such extreme cases, there isn’t significant narrowing of the esophageal lumen or compression on the trachea. Variation in surgical approach is evident in the literature and depends mainly on the size and extent of the lesion. Simple lesion enucleation, segmental esophagectomy with primary anastomosis, or even subtotal esophagectomy with gastric pull-up or jejunal/colonic interposition are some of the surgical options. The originality in our report is that esophagoplasty as an option for repair of a large esophageal defect after en bloc resection of an esophageal schwannoma should be considered if the esophagus is dilated along the length of the tumor. The width of the remaining esophageal wall at the site of the tumor excision should be at least 7–8 cm to allow a longitudinal closure that should restore the normal caliber of the esophagus which is around 1.5–2.5 cm in diameter .Malignant transformation, albeit rare in esophageal schwannomas, is indicated by the more frequent mitotic figures, necrosis, and cytological irregularities . When maFinally, the prognosis with benign schwannomas of the gastrointestinal tract, after complete resection, is usually excellent , with di4Esophageal schwannomas are rare submucosal lesions that carry very low malignant potentials. While small lesions are amenable for enucleation using minimal invasive procedures, larger tumors, especially those greater than 8 cm, may request en-bloc esophagectomy. In cases where the proximal esophageal segment is dilated, resection followed by esophagoplasty for defect closure should be considered.The authors declare that they have no competing interests.The authors declare any source of external funding for conducting this manuscript.The study such as this case report was exempted from ethical approval by the Institutional Review Board of the American University of Beirut-Medical Center.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.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on requestJD, PS, and AH performed the surgery for the patient. The manuscript was prepared by JD under the supervision of AH. Literature review was performed by JD, PS, and AH. IK performed the histological and immunological staining analysis of the gross specimen and provided images for the case report. All authors have approved the final version of the manuscript prior to submission.This is a case report not research study.Dr. Jad DegheiliNot commissioned, externally peer-reviewed


Gall bladder cancer (GBC) is associated with abdominal pain, lump, nausea, vomiting, and jaundice due to either gall bladder mass or the involved adjacent peritoneal structures. Gall bladder cancer presenting as refractory epilepsy is rare. Here we report a young female GBC patient who presented with an atypical and refractory frontal lobe seizures as the first manifestation of gall bladder cancer.A 46 years young female presented first time to the hospital with uncontrolled seizures and headache in 5 months duration. Seizures were very atypical in semiology with ptosis and mydriasis to either side along with ipsilateral ocular deviation. The episodes were bilateral but right eyelid ptosis, mydriasis and right horizontal conjugate deviation were frequent. MRI brain showed encephalomalacia in the left frontal region on axial T2 and coronal T1 weighted images without any enhancement on gadolinium contrast. CECT abdomen revealed a heterogeneously enhancing gall bladder mass with the evidence of lung metastasis from chest CT scan. CSF for malignant cytology was negative. Seizures were refractory to the treatment.Though CNS involvement is uncommon but it can be the only presentation in gall bladder cancer.The online version of this article (10.1186/s12883-019-1318-2) contains supplementary material, which is available to authorized users. The neurological manifestation in cancer appears because of the spread of the tumor to brain parenchyma or leptomeninges. CNS spread is uncommon in gall bladder cancer and only a few cases are reported till now , 2. StroA 46 years old, previously healthy female presented with headache, fits and decreased vision for last 5 months. Headache was diffuse, non-throbbing, moderate intensity and occasionally associated with nausea and vomiting. The patient was suffering from headache every day without diurnal fluctuations. She was on daily analgesics for relieving the pain. A few days later she developed fits with eyes deviating to either side along with drooping of the eyelid and pupillary dilatation to the side where the eye would deviate.  During the episodes, the patient responded partially and become very irritable afterward. The episode used to last for few minutes followed by irritable mood and crying for the next few hours . The episodes were occurring daily. In most of the episodes forced eye deviation was to right with right eye ptosis. There was no history of fever, rashes and myoclonic jerks except significant loss of weight in the last 3 months.Additional file 2:Movie S1. Video showing the ictus in which patient is having left eye-lid ptosis, mydriasis and conjugate eye deviation to left side. Patient is being asked to move her eyes to right but she is not following it. She is partially responding to commands with saying single word “YES” only. The episode is not associated with any other motor manifestation. (MP4 8970 kb)Additional file 3: Movie S2. Video showing postictal state during which is patient is conscious, following commands and is having normal ocular movements without any ptosis. (MP4 6213 kb)At admission, she was conscious, alert, higher mental functions were normal. Cranial nerves were normal. Bulk, tone, power and deep tendon reflexes were normal in both upper and lower limbs. There was no evidence of limb or gait ataxia. Bilateral plantar reflex was extensor.Mycobacterium tuberculosis was also negative.Haemoglobin was 9.2 g% with microcytosis and anisocytosis. Total leucocyte count was 14,200/cu mm with neutrophil dominance. Liver functions were deranged with raised liver enzymes  and alkaline phosphatase (ALP-507 IU/L). Kidney functions, serum electrolytes and thyroid profile were normal. Her Serum ANA and ENA screen were negative. Chest X-ray showed haziness over the right lower zone. MRI brain axial T2 and coronal T1 weighted images revealed encephalomalacia left frontal area  is a very aggressive tumor with poor survival. It usually presents with local symptoms pertaining to the primary tumor and it spreads to liver, peritoneum, and lungs. Rarely, CNS involvement in GBC can be in the form of carcinomatous meningitis with features of raised intracranial pressure or space occupying parenchymal lesion with stroke-like features and/or focal seizures . SeizureAnother possibility for this seizure semiology could be the spread of the seizure from frontal to the insular region resulting in ptosis and mydriasis as a part of autonomic manifestations of seizure.The focal seizures in our patient probably resulted from the encephalomalacic zone in the left frontal area near frontal eye-field Fig. . As the Neurological manifestations in a patient with gall bladder cancer although rare but can be the only presentation. Refractory de novo seizures in an adult do require workup for an underlying malignancy. Ptosis as a part of seizure semiology is not much described in the literature and further research is warranted in this area for clear anatomical localization.Additional file 1:Figure S1. Lymph node biopsy: Hematoxylin & Eosin stain (400 X), showing tumor cells in cluster and sheets. Tumor cells are having pleomorphic nuclei, conspicuous nucleoli, and moderate amount of eosinophilic cytoplasm. Inset: Acini like arrangement of tumor cells (arrow). (PNG 2599 kb)


Hemangiomas are defined as giant if theirsize exceeds 4 cm.2 The most commonsite is the right lobe of the liver (subcapsular region). Small, asymptomatichemangiomas do not show changes during long-term follow-up; hence they can beobserved without any treatment.3Giant liver hemangiomas are symptomatic and patients present with mild pain,abdominal mass, abdominal fullness and rarely with jaundice, consumptivecoagulopathy (Kasabach-Merritt syndrome), or intra-abdominal bleeding as the resultof rupture. Congestive heart failure and intraperitoneal bleeding can sometimes befatal.4 Surgical resectionis the only treatment option for symptomatic hemangiomas. Diagnosis is not easy toobtain because percutaneous biopsy is risky and the role of other treatment optionssuch as steroids, hepatic artery ligation, and radiotherapy iscontroversial.5Hemangiomas are the most common benign tumors of the liver, with a frequency of 0.4%to 7.3%.Herein, we report an unusual case of a giant symptomatic hepatic hemangioma, whichwas initially diagnosed as an inoperable hepatocellular carcinoma (HCC) and treatedwith an oral tyrosine kinase inhibitor .A 65-year-old woman visited our outpatient department with a complaint of weight lossfor 1 year. She was in good general condition and had an Eastern CooperativeOncology Group performance status of 1. Abdominal examination revealed an enlargedliver that was palpable 6 cm below the right costal margin; it was not tender and noother mass was felt. A complete hemogram as well as liver and renal function testswere normal. The coagulation profile was normal. Levels of carcinoembryonic antigen(1.94 ng/mL) and alpha-fetoprotein (2.13 ng/mL) were within the normal range.Contrast computed tomography (CT) of the abdomen at presentation  revealedUltrasound-guided fine-needle aspiration cytology of the liver was done twice andboth times it showed blood and tiny fragments of the liver with few dilated vascularchannels. Because biopsy was unsuccessful, a provisional diagnosis of HCC wasestablished on the basis of radiologic findings.The mass was considered inoperable because it involved all three hepatic veins andthe right branch of the portal vein. Therefore, with a presumptive diagnosis of HCC,the decision was made to start sorafenib at a dosage of 800 mg per day. The patientdeveloped grade 2 hand-foot syndrome, which was observed approximately 1 month afteradministration of sorafenib and subsequently settled down after tapering andadjusting the dosage to 200 mg per day. This was the dose that the patient couldtolerate. Sequential ultrasonography of the abdomen after 5 months of treatment withsorafenib revealed a 40% size reduction; hence the same dose of sorafenib wasmaintained.A repeat CT scan after 18 months of regular treatment with sorafenib revealedremarkable tumor size reduction with atrophy of the right lobe of the liver . The tum18F]fluorodeoxyglucose-avid simple cyst in theleft lobe of the liver. There was no uptake elsewhere in the body.A positron emission tomography–CT scan was performed, which confirmed anirregular lobulated mass in the right lobe of the liver involving segments 5, 6, and7 , with increased metabolic activity and a tiny non–[The general health of the patient was fit on the basis of other routineinvestigations required for the surgery. Right hepatectomy was performed. Theintraoperative findings were that there was a necrotic friable tumor in the rightlobe of the liver with autodemarcation of the right and left lobes. The portal triadwas free of tumor.The postoperative surgically resected specimen was later reported as a hemangioma. SorafenLiver hemangiomas are found in approximately 7% of the general population. Most ofthe time they are asymptomatic, and they affect women more than men. Approximately40% of giant hepatic hemangiomas (> 4 cm) produce symptoms that include earlysatiety, abdominal pain, and sometimes nausea and anorexia.6 Atypicalenhancement patterns of hemangiomas are also observed because of the changes invascularity.7 An atypicalhemangioma can sometimes mimic a malignant hepatic tumor and cause diagnosticconfusion.Hepatic hemangiomas on a dynamic CT scan show an initial intense peripheral nodularenhancement with a gradual central fill-in. Because of this typical radiologicappearance, hemangiomas can be differentiated from other tumors. Sometimeshemangiomas can present with atypical enhancement patterns because of the presenceof intralesional nonenhanced thrombosis, degenerated, fibrotic, or calcifiedcomponents.In the case reported herein, a patient presented with atypical radiologic featuresand was initially diagnosed with HCC, which after surgery was found to be ahemangioma.10 Chemoembolization was alsoreported to be an effective treatment option for giant hepatic hemangioma.11 However, it is still not awell-established option because in some cases it causes an increase in tumormass.12 High morbidityrates of 10% to 27% and a mortality rate of 2% after resection or enucleation of thehepatic hemangioma have been reported in various studies.14Treatment options for symptomatic hemangiomas include resection, ligation of thehepatic artery, radiation therapy, and rarely, in selected cases, livertransplantation can be performed.15 Itworks on the growing hemangioma by the following three mechanisms: vasoconstriction,induction of apoptosis, and downregulation of angiogenic factors.16 Furthermore, propranolol exerts an inhibitory effect onmatrix metalloproteinase 9, which is involved in upregulation of the angiogenesisprocess. It has been used for the treatment of infantile hemangioma, with effectsranging from significant reduction in size to complete resolution of the hepatichemangioma.17Propranolol (a nonselective beta blocker) is an effective treatment option forproliferative hemangioma.18 Although the pathogenesis ofcavernous hemangioma is not known, it is hypothesized that they are formed becauseof upregulation of angiogenic factors  and downregulation of antiangiogenesis.20 Compared withhepatic sinusoidal epithelial cells, VEGF-A is overexpressed in cavernous hepatichemangiomas and leads to increased angiogenesis.11 Bevacizumab exerts its effect by blocking VEGF-A.18Some studies have shown activity of bevacizumab in hepatic hemangiomas.21Sorafenib is effective against hepatocellular carcinoma24 and softtissue sarcomas.25 This drug hasalso been tried for the treatment of patients with vascular tumors, includingangiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitaryfibrous tumor.26In this case report, after sorafenib was administered to a patient with a diagnosisof HCC, there was an impressive response in size of the hepatic mass. Sorafenib is amultikinase inhibitor that works by inhibiting epidermal growth factor receptor;VEGF receptors 1, 2, and 3; and platelet-derived growth factorreceptor-β.In conclusion, we report an unusual case of a woman with a giant hepatic hemangioma,for whom sorafenib was found to be beneficial with tolerable adverse effects.Additional clinical trials should be performed to prove the efficacy of sorafenib inadult hepatic hemangiomas.


Median arcuate ligament syndrome (MALS) can cause severe complications after pancreaticoduodenectomy (PD). Most of the reported cases of MALS have been diagnosed perioperatively and can be treated efficiently by interventional radiology or division of the median acute ligament (MAL) fibers.A 69-year-old woman underwent PD with resection of the SMV for pancreatic head cancer. Intraoperative exploration showed normal anatomy of the celiac trunk. Intraoperative digital palpation revealed normal pulsation of the common hepatic artery after resection of the gastroduodenal artery. On postoperative day (POD) 3, her liver function tests were abnormal, and bloody fluids were found in the drain. Abdominal CT showed necrosis of the pancreatic body and ischemia in the liver secondary to MALS which was not detected in the preoperative CT. Interventional radiology was tried first but failed. Division of the MAL fibers markedly increased the blood flow in the hepatic artery. Resection of the remnant pancreas and spleen was also performed simultaneously. Abdominal CT on POD 20 showed re-occlusion of the celiac artery. She experienced rupture of the gastrojejunostomy site, severe hepatic cytolysis, and choledochojejunostomy stricture thereafter.This is the third case of MALS that has developed acutely after PD. MALS can cause refractory complications even after MAL release. The incidence of celiac axis stenosis caused by median arcuate ligament syndrome (MALS) is reported in around 7.3% of asymptomatic individuals . MALS haThe median arcuate ligament (MAL) is a normally occurring tendinous band spanning both diaphragmatic crura anterior to the aorta . In MALSMost of the reported cases of MALS have been diagnosed perioperatively, either by radiologic findings or by intraoperative digital palpation or Doppler sonography . When MAHerein, we report a case of MALS that developed acutely after PD that was refractory to interventional radiology and MAL division, leading to serious ischemic complications.A 69-year-old female presented to our hospital with a one-month history of epigastric discomfort. Physical examination was unremarkable, and laboratory examination was normal other than slightly elevated amylase levels. Abdominal contrast-enhanced computed tomography (CT) revealed a low-density mass with a diameter of 30 mm at the pancreatic head, with segmental superior mesenteric vein (SMV) attachment. There was neither stenosis nor arteriosclerosis around the celiac axis at that time Fig. . EndoscoThe patient then underwent PD with superior mesenteric vein resection and reconstruction, and a lymphadenectomy including right half dissection of the lymph nodes without the nerve plexus around the celiac trunk. Intraoperative exploration ruled out latent peritoneal or liver metastasis and showed normal anatomy of the celiac trunk, mesenteric vessels, and related branches. A clamp test of the GDA showed normal hepatic artery pulsation. The pancreatic body needed to be mobilized more than usual for the pancreaticojejunostomy because it was a hard pancreas. The duration of surgery was 549 min, and the blood loss was 863 mL; blood transfusion was not performed.On POD 3, her liver function tests were still abnormal and bloody fluids were found in the drain. Abdominal CT showed a characteristic hook-pattern on the anterior proximal celiac axis from compression of the MAL, which had not been detected in the preoperative CT Fig. a. In addAbdominal CT on POD 20 showed re-occlusion of the celiac artery at its root, which was almost same as the first stenotic site Fig. . HoweverCeliac artery patency is vital to successful outcome after PD; impairment of celiac flow results in potentially catastrophic hepatic, biliary, gastric, splenic, and pancreatic ischemia . The preIntraoperative assessment of the flow through the hepatic artery should be performed in pancreatic resection, even if preoperative CT does not demonstrate MALS . HoweverMALS can develop acutely after PD even if it has not been diagnosed pre- or peri-operatively. This is the third case which showed no evidence of hepatic artery flow impairment before PD or during GDA clamping or pancreatic transection , 13. The


Malignant rhabdoid tumor of the kidney (MRTK) is a rare neoplasm of infancy. We report a case of a nine-month-old male infant who presented to the pediatrics outpatient department with the history of fever, lethargy, and abnormal head movements. On gross examination, the patient had a firm, non-tender, intra-abdominal mass at the right lumbar region with irregular margins. Computed tomography scan of the abdomen revealed a lobulated soft tissue arising from the kidney with areas of necrosis. Brain magnetic resonance imaging was also performed, which showed a large heterogeneous lesion in the posterior fossa. Histopathologic study revealed loss of INI1 protein. Since MRTK and atypical teratoid rhabdoid tumor (ATRT) of the brain share a common mutation in the gene (hSNF5/INI1), hence a diagnosis of MRTK with co-existent ATRT of the brain was established. Actinomycin-D and vincristine failed to show any improvement and the condition of the patient deteriorated progressively, resulting in his death within 15 days of hospital admission. Rhabdoid tumor of the kidney (RTK), a rare and highly malignant neoplasm of infancy , constitA nine-month-old male infant presented with the complaints of fever and lethargy for the past one month and abnormal head movements since the last fifteen days. A right-sided abdominal mass was also observed by the mother five days prior to the admission. The head movements were oscillatory, not associated with fits or unconsciousness. Gross examination revealed a firm, non-tender, intra-abdominal mass (measuring approximately 6 x 7 cm) at the right lumbar region with irregular margins and smooth non-erythematous overlying skin. The mass was immobile and abutted on the underlying viscera. The decreased tone in all four limbs and loss of neck holding was observed on central nervous system (CNS) examination. There was no positive history of hematuria.Initial laboratory investigations revealed leukocytosis. Abdominal ultrasound (US) demonstrated a solid 6 x 5.3 cm mass at the upper pole of the right kidney with thickened renal parenchyma. On performing computed tomography (CT) scan of the abdomen, a lobulated soft tissue arising from the kidney was seen with areas of necrosis Figure .There was an extension of the mass to the right paracolic region, encasing the right renal vessels along with the invasion of the lymph nodes in the left para-aortic, interaortocaval, and mesentry. The findings were suggestive of Wilms tumor with neuroblastoma being considered as a differential diagnosis. However, the microscopic analysis of the neoplasm displayed sheets of large monomorphic cells with eosinophilic cytoplasm and eccentric, prominent nucleoli. The immunohistochemical study revealed positive immunomarkers for epithelial membrane antigen (EMA), Wilm's tumor transcription factor-1 (WT-1), periodic acid-schiff (PAS), and vimentin. Moreover, histopathological study revealed loss of SMARCB1/INI1 protein expression. Thus, the right MRTK was confirmed. The results of echocardiogram and CT of the chest were unremarkable. Brain magnetic resonance imaging (MRI) displayed a large heterogeneous lesion, measuring approximately 4.7 x 4.2 x 3.7 cm in the posterior fossa  has been recommended as a novel therapy for malignant rhabdoid tumors (MRTs) . TargetiRTs have the worst prognosis among all the renal tumors. It depends on the age of the patient at the time of diagnosis and also the stage of the disease. A four-year survival of 8.8% was reported for infants less than six months at the age of diagnosis as compared to 41.1% for children of 2 years [RTs in the pediatric population have a dismal prognosis, independent from localization. The presence of metastasis at the time of diagnosis appears to be the only prognostic factor. Moreover, due to its significant association with brain tumors or early brain metastases, concurrent brain imaging is essential for all patients.


Paratesticular liposarcomas (PLS) is a very uncommon pathology type of paratesticular sarcomas, with less 200 similar cases reported to date in the English literature. There are a few cases regarding giant paratesticular liposarcoma measuring over 10 cm. We present an unusual case with a giant well differentiated PLS of the left testis extended to the pelvic cavity. We report the case of a 55-year-old man who presented with large left groin mass. The patient underwent left orchiectomy following a cure of a scrotal hernia. Histological and immunohistochemical findings were suggestive of a well-differentiated liposarcoma of spermatic cord. The surgical margins were positive. Metastatic work-up, which included CT of the thorax, abdomen and pelvis, did not reveal any distant metastasis in thorax but there is a left pathological external iliac lymph nodes and a left lateroplevic lipomatous mass extended to the iliac fossa and left parietocolic gutter up to the umbilicus measuring 15x7x17 cm. Our patient refused treatment. A review of the literature revealed that there are fewer cases of giant well differentiated paratesticular liposarcoma extended to the pelvic cavity were reported. This study focuses on the clinical characteristics and treatment of this rare type of tumours. Paratesticular liposarcomas (PLS) are rare variant of genitourinary malignancies, occurring more often in the spermatic cord where it accounts for less than 12% of all liposarcomas . Due to A previously healthy 55-year-old man presented with an increasing scrotal swelling lasting for 2-years. Examination revealed a left hard hemiscrotal mass, distinct from the left testis, measuring approximately 15 cm × 12.5 cm. A scrotal exploration was performed under general anaesthetic where a large left groin mass was found separate to the testis; however, with suspicions for underlying mass, a left orchidectomy was performed. Microscopically, the tumor had lipoblasts with atypical nucleus indented on its surfaces by fat vacuoles and stromal cells with hyperchromatic nuclei . The hiset al. found that the 3 year local recurrence free survival was 100% for negative margins compared with 29% for positive margins [et al. [Paratesticular liposarcomas, which account for 7% to 10% of all intrascrotal tumors, have their mesodermal origin derived from adipose tissue. The most common site of origin is the spermatic cord accounting for 90% of cases . There a margins . Retrope margins . Indicat margins -17. Cerd [et al. . Due to  [et al. .Often misdiagnosed preoperatively, paratesticular liposarcoma must be considered in the differential diagnosis of groin mass. Regardless of tumor size, radical orchidectomy with free surgical margins is recommended in order to avoid recurrence. The role of adjuvant therapies represented by radiotherapy and chemotherapy is controversial.The authors declare no competing interests.


We present the case of a male with an asymptomatic abdominal mass, where imaging-guided biopsy confirmed the lesion as a solitary fibrous tumour arising from the mesentery. This is a notably rare location for solitary fibrous tumours with only a few reported cases in the literature. A 71-year-old male was referred by his general practitioner in view of a palpable pulsatile lower abdominal mass noted on routine examination. The patient denied abdominal pain, weight loss, fever, change in appetite or bowel habit and passage of blood per rectum.An abdominal ultrasound was performed, which demonstrated a large heterogeneous, significantly vascularized mass anterior to the lower aorta . This waAn ultrasound-guided biopsy was performed and histopathology confirmed the mass to be a solitary fibrous tumour with no features of malignancy. The patient was admitted for two separate sessions of transarterial embolization of the tumour  in an at2 They comprise less than 2% of all soft tissue neoplasms, usually occur between the 4th and 6th decade of life and show no sex or ethnic predominance.3 Clinical presentation varies according to the site of involvement and includes pain, an asymptomatic palpable mass and symptoms secondary to pressure effects. A rare but well-described phenomenon is the occurrence of hypoglycaemia secondary to the production of insulin-like growth factor by some SFTs, known as Doege–Potter syndrome.4Solitary fibrous tumours (SFTs), first described in 1931, are rare mesenchymal tumours of fibroblastic or myofibroblastic origin .7 The mesentery is a very rare location for SFTs, with only a few cases being reported in the literature.8Although SFTs were initially thought to only involve the pleura, a higher incidence of extrapleural SFTs is now recognised. In fact, SFTs have been found to arise from a myriad of sites including somatic structures (soft tissues and bone), head and neck , as well as abdominal and pelvic structures .2 However, even SFTs originally deemed histologically benign have demonstrated recurrence and metastasis after several years.9 The behaviour of malignant SFTs is closely related to mesothelial tumours with spread via seeding. In a study, metastases occurred in the liver, lung, mediastinum, peritoneum, omentum, colonic mesentery and subcutaneous tissue. According to some studies, extrathoracic SFTs seem to be more indolent than their intrathoracic counterparts with regard to metastasis; however, they confer a higher risk of local recurrence. Overall, these two classes are considered to be a single disease entity.10While the majority of SFTs are benign, all have malignant potential. Indicators of malignancy include large tumour size (>10 cm) and histological findings such as high mitotic index, necrosis, cellular atypia and hypercellularity.11 SFTs are usually isointense on T1 weighed MRI and isointense or hypointense on T2 weighting owing to the presence of fibrous tissue, with intense enhancement following gadolinium administration.12 Positron-emission tomography with fludeoxyglucose has shown accuracy in distinguishing SFTs from malignant disease. SFTs typically show a low level of fludeoxyglucose uptake (SUV < 2.5) if at all.14Imaging features of SFTs have been described; though these are not entirely specific and vary depending on the tumour cellularity, vascularity and stromal density. Suggestive features on CT include a well-defined, hypervascular mass, with possible pressure effects on adjacent structures, subject to tumour size and location. In our case, the mass demonstrated organised hypervascularity with large feeding arteries and draining veins. Small masses are typically heterogeneous; however, in larger masses areas of central hypodensity may be present indicating necrosis or cystic change. While it has been reported that hypodense areas within the tumour may indicate the presence of malignancy, there are no pathognomonic CT features to confirm this.16 Differential diagnoses to be considered in our case include lymphoma, primary adenocarcinoma of the mesentery, mesenteric fibromatosis, inflammatory pseudotumour, solitary vascular metastatic lesion and extra-adrenal phaeochromocytoma of the organ of Zuckerkandl. In the case of lymphoma, the mass typically appears very homogeneous on CT (heterogeneous density is seen in very aggressive subtypes) with minimal to no enhancement due to the poor vascularity of the lesion. The presence of large collateral feeding vessels may be a useful imaging feature to distinguish SFTs from other masses; however, this is not specific.en bloc surgical resection with negative margins. The role of neoadjuvant chemo- or radiotherapy is controversial, producing mixed results.17 Antiangiogenic therapy for unresectable SFTs may be promising.18Treatment of SFTs involves 19 There are no surveillance guidelines; though it is generally recommended to perform a CT scan every 6 months for the first 2 years and then annually.20The prognosis of these tumours is not well defined; however, the high recurrence rate and the variability at histology necessitate long-term follow-up.Solitary fibrous tumours (SFTs) are rare mesenchymal tumours comprising less than 2% of all soft tissue neoplasms.While SFTs were initially thought to only involve the pleura, a higher incidence of extrapleural SFTs is now recognised.Despite the large size of SFTs, patients are often asymptomatic at the time of diagnosis.Management involves surgical resection with long-term follow-up.Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Amyloid is an abnormal insoluble protein that can deposit in extracellular space. It can involve nearly any organ system and may manifest as a systemic process or focal lesion (amyloidoma). We present a rare case of localized amyloidosis with trigeminal nerve being the only site of involvement and no evidence of systemic disease. We also review literature relevant to trigeminal amyloidoma and make recommendations for diagnosis and treatment. Amyloidosis is a generic term that refers to the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins, many of which circulate as constituents of plasma . These sA 34-year-old woman with no significant past medical history presented with the chief complaint of left facial numbness, left ear pain and decreased hearing in the left ear of three years duration. The patient had also been experiencing sharp and shooting pain in different areas of her left thigh. The pain was not associated with any weakness, tingling or numbness. Besides mild fatigue she denied having any fevers, night sweats or weight loss. Neurological physical examination was grossly intact except for sensory loss in the V2 (maxillary) and V3 (mandibular) distribution of trigeminal nerve . Abdominal exam was without evidence of lymphadenopathy and hepatosplenomegaly. Given deficits in the sensory distribution of trigeminal nerve, she was initially evaluated by ear, nose and throat (ENT) and underwent two sequential minimally invasive surgeries for nasal polyps without significant resolution of her symptoms. She was subsequently referred to a neurologist and had magnetic resonance imaging (MRI) of the brain performed. Brain MRI revealed a soft tissue mass with expansion in the left Meckel’s cave, measuring 22 x 16 x 12 mm  or as a secondary disease associated with an underlying chronic inflammation, non-inflammatory conditions such as plasmacytomas, chronic renal failure and rheumatoid arthritis . AmyloidPathogenesisThe reasons for amyloid deposition in the central nervous system are unclear. Laeng et al. have theorized that amyloidomas are neoplasms of AL (primary) lambda producing B cell clone capable of terminal differentiation in reaction to unique antigens . Cohen ePresentationReported cases of Gasserian ganglion amyloidomas predominantly occurred at a mean age of 48 years with slight female predominance , 10, 17.ImagingCentral nervous system amyloidomas typically appear on computed tomography (CT) scan as hypodense patchy lesions with intense enhancement following contrast administration. These lesions have been predominantly described as hyper to hypo intense lesions on T1 weighted MRI and heterogeneous on T2 weighted MRI . GasseriPrognosis and treatmentThe clinical course of trigeminal ganglion amyloidoma is thought to be benign. These lesions grow slowly and symptoms are typically present for years before the diagnosis. The natural history of this rare lesion is poorly known due to low incidence of the disease and few published reports. Overall there have been 16 reports of patients with trigeminal amyloidomas since the first case reported in 1957  of these patients underwent a craniotomy with surgical resection of the lesion , urine protein electrophoresis (UPEP), serum kappa and lambda light chains, urine kappa and lambda light chains, chest X-ray, electrocardiogram, CT scans of the chest and abdomen, X-rays of the skeleton, nuclear bone scan and echocardiography . In a feThis case demonstrates that in addition to meningiomas, schwannomas, malignant cavernous sinus tumors, and inflammatory disorders, amyloidomas should be considered as a possibility in the differential diagnosis of a cavernous sinus lesion or of a lesion arising from the Gasserian ganglion or within Meckel's cave. Once the diagnosis is established, the workup should be done to rule out the presence of systemic amyloidosis including fat pad and/or bone marrow biopsy. In the absence of systemic involvement, localized amyloidosis in the form of trigeminal amyloidoma can be effectively treated with surgical resection with relief of symptoms.