Esophageal granular cell tumor (GCT) is a rare benign tumor with malignant potential. With wide application of endoscopic techniques, the esophageal GCT discovery rate and treatment strategy has changed. This study was to preliminarily evaluate outcomes of endoscopic diagnosis and treatment for esophageal GCT.Fourteen patients  with esophageal GCT diagnosed and treated by esophageal endoscopy. Esophagoscopy, endoscopic ultrasound (EUS), and endoscopic submucosal dissection (ESD) techniques were employed in diagnosis and resection.en bloc resection rate was 92.9% (13/14) pathologically. Operation time was 25 to 60 minutes, mean 38.2 ± 10.1 minutes. No recurrence was observed during a mean follow-up of 16.6 ± 12.7  months.Esophageal GCTs are tumors which arise from the submucosal layer, and vary in color but with a yellowish color on endoscopy being most common. On EUS, features were homogenous (ten cases) or mildly heterogeneous (four cases) hypoechoic solid pattern originating from the muscularis mucosa (six cases) or submucosal layer (eight cases) of the esophageal wall. Tumors ranged from 4 to 26 mm (mean 12.1 mm). ESD was performed in all patients without complication. Clinical diagnosis was confirmed by pathology and immunohistochemical examination (positive for S-100 and vimentin). The Esophagoscopy and EUS increased the esophageal GCT discovery rate, and its features were summarized. Minimally invasive ESD is feasible and safe for excisional biopsy, providing pathological diagnosis and treatment. Esophageal granular cell tumor (GCT) is a submucosal tumor (SMT) probably originating from the Schwann cell. It mainly affects the submucosal layer, and less commonly the mucosal layer and muscularis propria . DespiteIt has been suggested that asymptomatic esophageal GCTs smaller than 10 mm could be followed up with periodic endoscopy and/or EUS . AlthougWe reviewed the Zhongshan Hospital endoscopic therapy database. A total of 14 patients with confirmed histological diagnosis of esophageal GCTs had endoscopic diagnosis and ESD resection at our institute from 2006 to 2011. Patient medical records were reviewed for demographic data including age, gender, and indications for endoscopy. We obtained institutional review board approval for the study. Informed consent for all procedures, including gastroscopy, EUS, and ESD was obtained from each patient.Patient age from 18 to 75; esophageal submucosal lesion found by endoscopy with size evaluated as less than 30 mm and the patient willing to be treated by ESD technique. Patients with any abnormality in blood clotting, biochemistry or blood cell count were excluded. Patients with esophageal venous varication disease, esophageal stricture disease or history of esophageal surgery were also excluded.Endoscopic and EUS reports and images were reviewed for tumor size, appearance, location, and therapy.EUS was performed before treatment with a high-frequency miniprobe  to evaluate the tumor origin and size. All available EUS images were reviewed blindly by one proficient endoscopist.A transparent cap  was attached to the front of the endoscope. Additional equipment and accessories included a high-frequency generator , argon plasma coagulation (APC) unit , injection needle , hook knife , insulated-tip knife , hot biopsy forceps , and hemostatic clip . An insufflator (Olympus) was used for carbon dioxide gas insufflation during the procedure. A mixed solution of saline with indigo carmine (0.5%) and epinephrine (0.0005%) was prepared for submucosal injection.en bloc resection. The tumor was dissected outside the capsule, and the mixed saline solution was injected repeatedly during the dissection when necessary. Larger vessels or arteries with high bleeding risk were coagulated using hemostatic forceps. The resultant artificial ulcer was managed routinely with APC to prevent delayed bleeding, and hemoclips were used to close the deeply dissected areas when necessary. The time from the marking dots to complete resection of the tumor was recorded.Patients were treated under general anesthesia. The resection border was marked using argon plasma coagulation of the normal mucosa approximately 5 mm from the tumor margin. Submucosal injection of the mixed saline solution (5 to 10 ml) was then performed to lift the lesion. After sufficient lifting of the submucosal layer and mucosa, the hook-knife was used to cut open the mucosa in a circumferential incision around the lesion outside the marking dots and then extend into the submucosal layer. The circumferential incision around the lesion was made as deep as the muscularis propria if necessary and dissection was then performed under direct vision to achieve complete Routine H&E staining and additional immunohistochemistry (IHC) were performed and observed under an optical microscope for evaluation and differential diagnosis. IHC examination included smooth muscle actin, muscle-specific actin, desmin, CD34, S-100, vimentin, CD117, neuron-specific enolase, CD68, and Ki-67. Complete resection was defined as a resected specimen with tumor-free lateral and vertical margins. Incomplete resection was defined as a specimen with histologically positive margins.Esophagogastric endoscopy was performed three and six months after ESD, and then yearly thereafter. Endoscopic forceps biopsies of the operation site were not routinely performed except as necessary when suspicious lesions were observed, and then, additional EUS examination was performed to further diagnose suspected recurrence.P-value less than 0.05 was taken to indicate significant difference. Statistical analysis was performed using the SPSS statistical analysis program .The median of continuous variables was used to present data. The difference in tumor origin on EUS, esophageal location, and time required for resection were compared between the small tumor  and large tumor  groups using a nonparametric two-independent-samples test. A A total of 14 patients aged 29 to 75 years with GCTs of the esophagus were identified including 8 men and 6 women. The patient clinical data are listed in Table EUS was performed in all patients who were diagnosed with SMT in our institute. On EUS, esophageal GCT in our study appeared as a homogenous (ten cases) or mildly heterogeneous (four cases) hypoechoic solid pattern originating from the muscularis mucosa (six cases) or submucosal layer (eight cases) of the esophageal wall, with smooth edges despite the small tubercular aspect on endoscopy Figure c. The maEndoscopic forceps biopsy was not routinely performed in our institute when the lesion was recognized as a submucosal mass, because of the high risk of destroying the tumor integrity and causing bleeding. However, three tumors mimicked mucosal lesions because of their rough overlying mucosa, and thus endoscopic forceps biopsies were performed. Histological diagnosis of GCT was confirmed via biopsy in only one of these.P < 0.05) to remove the large tumors  than the small tumors . The procedure time for the tumors in the submucosal layer  was more than for tumors in the muscularis mucosa layer , but this was not statistically significant. Severe complications of ESD including perforation and delayed bleeding were not observed in all patients. The length of hospital stay was two to four days for each patient.Pathological diagnosis was confirmed in all cases after ESD  months. No recurrent disease was observed during follow-up, including the Rx margin case, within 18 months. A small protuberance of the mucosal scar was found at the operation site in two cases (negative margin) on follow-up examination. Endoscopic forceps biopsy and EUS examination were performed. EUS did not find any deep lesions below the mucosa and the pathologic diagnosis from the forceps biopsy proved that these were inflammatory granulomas.Esophageal GCT is a type of SMT which is thought to arise from Schwann cells of the submucosal neuronal plexus and is usually a benign neoplasm but has some malignant potential. Approximately 2 to 4% of esophageal GCTs reported in the literature were malignant , of whicEUS-guided fine needle biopsy or multiple forceps biopsy were employed in an attempt to obtain tissue diagnosis, but the results were not reliable because the tumors were small, underlying the mucosa layer, and biopsy tissue was not adequate. Moreover, there is sampling error when the biopsied tissue does not encompass the areas of malignant change . CompletOpen surgery or thoracoscopy is highly invasive and can lead to a decrease in patient quality of life compared to simple endoscopic resection. However, finding these lesions operatively was difficult because they were less than 20 mm in diameter. Endoscopic resection methods can prevent skin scars caused by incisions, reduce the inflammatory response and perceived pain associated with surgical trauma, and lower the risk of postoperative infection. Endoscopic methods are extremely minimally invasive, and are becoming the preferred treatment of benign esophageal tumors including leiomyoma, GCT, and gastrointestinal stromal tumor (GIST).Currently, traditional polypectomy and EMR are most commonly employed for GCTs. However, complete histological resection may not always be easy to achieve using EMR because most esophageal GCTs are not confined to the mucosa, but rather, involve the submucosa, which results in frequent involvement of the resection margin.To date, there are several reports showing successful removal of esophageal GCT by EMR, but most of these are small series or single case reports. The maximum number of cases of successful removal of esophageal GCT was ten from twenty-three patients reported from Mayo Clinic. However, EMR was not perfect for endoscopic resection of these lesions, especially for lesions exceeding 10 mm in diameter. There is a high risk of incomplete removal. An attempt to remove a 13-mm esophageal GCT by EMR was unsuccessful in a report from Mayo Clinic .en bloc excision and histologically complete resection even in large lesions because the technique involves dissection of the submucosal tissue beneath the lesion [en bloc and histologically complete resection, which may reduce local recurrent rate. In our study, the pathological en bloc resection rate was 92.9% (13/14). Only one was not recognizable because the cut edge had an eschar.ESD is a method of endoscopic resection that involves circumferential cutting of the mucosa surrounding the tumor followed by dissection of the submucosa beneath the lesion. ESD has the advantage of a high probability of e lesion . It has We demonstrated that ESD is a safe, effective, and minimally invasive procedure for treating esophageal GCT. Our experience with ESD in 14 esophageal GCTs validates the safety and efficacy in lesions less than 26 mm in size. Given the apparently low risk of this method, it is reasonable to consider ESD the treatment of choice for esophageal GCT.Bleeding and perforation are the two main complications of ESD of esophageal lesions. Other complications including pneumothorax and subcutaneous emphysema are secondary to perforation. In this study, none of the above complications were observed. Our measures in reducing the complications of ESD were: a) keep the operation field clear and perform all the actions under direct vision, b) prophylactic hemostasis of visualized vessels, c) well selected length and angle of the knife during the operation, and d) after the lesion was removed, hot biopsy forceps coagulation or APC were performed on the whole wound surface. This management strategy can reduce delayed hemorrhage and obliterate the residual lesion if it exists.The ESD procedure time in this study varied from 25 to 60 minutes, and there was a significant difference in operation time according to tumor size. It took more time to dissect the large tumors and tumors derived from the submucosal layer. The length of hospital stay was two to four days for post-operative observation. The ESD procedure caused little trauma and was relatively inexpensive.Follow-up ranged from 4 to 40 months (mean duration 16.6 ± 12.7), during which time local recurrence was not observed in any patient at gastroscopy. However, because of the limited number of patients and relatively short follow-up duration, we realize that much longer follow-up may be needed to evaluate long-term results of ESD of esophageal GCT.In summary, this study showed that esophageal GCTs did not appear uniform under endoscopy. EUS is an accurate imaging test for detecting the component of the esophageal wall from which the mass arises. Resection by ESD safely and entirely removed tumors without complications and should be the preferred procedure; this also helps to obtain an exact pathological diagnosis. Further investigation is required to assess the long-term outcomes of this method.APC: Argon plasma coagulation; EGD: Esophagogastroduodenoscopy; EMR: Endoscopic mucosal resection; ESD: Endoscopic submucosal dissection; EUS: Endoscopic ultrasound; GCT: Granular cell tumor; GIST: Gastrointestinal stromal tumor; H&E: Hematoxylin and eosin; IHC: Immunohistochemistry; SMT: Submucosal tumor.The authors declare that they have no competing interests.Conception and design: M-DX, L-QY. Provision of study materials or patients: WL, M-DX, P-HZ, L-QY, Y-QZ, Y-SZ, W-FC. Collection and assembly of data: WL, M-DX. Data analysis and interpretation: WL, M-DX. Manuscript writing: WL, M-DX. All authors read and approved the final manuscript.


Odontogenic myxoma (OM) is an infiltrative benign bone tumor that occurs almostexclusively in the facial skeleton. The radiographic characteristics of odontogenic myxomamay produce several patterns, making diagnosis difficult. Cone-beam computed tomography(CBCT) may prove extremely useful in clarifying the intraosseous extent of the tumor andits effects on surrounding structures. Here, we report a case of odontogenic myxoma of themandible in a 27-year-old female. The patient exhibited a slight swelling in the leftmandible. Surgical resection was performed. No recurrence was noted. In the CBCT sections,we observed perforation of the cortical plate and radiopaque line that extended from theperiosteum, resembling "sunray" appearance—a rare feature of OM—which could not beassessed by panoramic radiography. The posteriorregion of the jaw is the most frequent area where the neoplasia occurs.1 Pain, dysesthesia, ulceration, soft tissueinvasion and tooth mobility are the symptoms of patients, although the majority of them haveno clinical signs or symptoms.3Radiographically, OM exhibits several patterns such as unilocular, multilocular, pericoronaland radiolucent/radiopaque patterns.2 Mostreported OMs are multilocular with coarse or angular trabeculations.8 The aim of this report is to present a case ofOM in the mandibular left molar/ramus region of a 27-year-old female that had a rare CBCTappearance with periosteal reaction. Odontogenic myxoma (OM) is a relatively rare tumor of mesenchymal origin. A 27-year-old female patient who attended a dental clinic in Dezful presented for aserious 3-year complaint of a slight swelling in the left mandibular region, which haddeveloped over a course of one year. The patient initially underwent periodontal treatment for the involved teeth, whichincluded extraction of the third molar in the same involved side. After 6 months, thepatient returned to the clinic, reporting no improvement but progression of the disease,with slight facial asymmetry and numbness on the left side of the lower lip. Then, she wasreferred to the Department of Oral and Maxillofacial Radiology of Jundishapur University ofMedical Sciences, while complaining about dull, diffuse and chronic pain and feelingheaviness on the left side of the mandible. The physical examination showed expansion ofboth cortical plates in the left posterior mandibular alveolus. The swelling was bony hardin palpation with normal overlying mucosa and no ulceration. The medical and family historyrevealed nothing of note and she did not have any oral habits. The rotational panoramic and periapical radiography showed a mixed radiolucent/radiopaquelesion with a relatively well-defined border from the first molar to the ramus. Internally,the lesion exhibited a straight and sharp trabecular pattern . However9 The posterior region of thejaw is where the neoplasia most frequently occurs.6 Myxomas are slow-growing and locally destructive tumors that displaybehavior similar to malignant tumors in some cases but do not metastasize to lymph nodes.10 OM is slightly more common in females, with a male-to-female ratio of 1:1.5. Two-thirds ofOM cases are located in the mandible and the other one-third is located in themaxilla.11 such as pericoronal, unilocular, multilocularand radiolucent/radiopaque patterns with an impacted tooth; thus radiographic appearance isimportant to establish diagnosis. 6 In mostcases, the septa are curved and coarse, but straight and thin septa represent a tennisracket-like or stepladder-like pattern, which is a rare characteristic of OM.1 Myxomas have been reported to show a wide spectrum of radiographic appearances,12 Tooth displacement and rootresorption are more reliably observed by conventional radiography13 but CBCT appears more convenient and is useful for observingthe maxillofacial lesion in detail. These pieces of information include precise extension ofthe lesion, perforation, periosteal reaction and so on.14 Conventional radiography is usually the first imaging modality to be performed and mayreveal something from small periapical radiolucencies to large multilocularlesions. In this case, we observed the perforation of the cortical plate and radiopaque line thatextended from periosteum, resembling a “sunray” appearance in CBCT sections, which could notbe assessed by panoramic radiography. This “sunray” appearance suggested the diagnosis of anosteogenic sarcoma, but the histopathological examination after incisional biopsy wassuggestive of OM.15 but in our case, lingual and buccal corticalplates showed perforation and septa at the periphery of the lesion, presenting a true“sunray” spicular pattern that was not apparent on panoramic radiography. Thoma16 has described two types of myxoma: odontogenicmyxoma and osteogenic myxoma. The former is considered as benign while the latter is viewedas malignant and as a sarcoma with similar changes. On occasion, expansion of a small area with straight septa may project over an intactouter bony cortex and give a speculated appearance seen in osteogenic sarcoma. Carefulinspection of this expansion area reveals a thin but intact outer cortex that will not beseen in osteogenic sarcoma,20 The “sunray” appearance of OM is rare and has been reported in few studies.19 Some studies reported OM cases with unusual periapical cortical reaction on conventionalradiography, which mimicked osteogenic malignant disease.20 reportedlesions that displayed a “sunray” spicular pattern on CT. MacDonald-Jankowski et al In future, the application of three-dimensional CT or CBCT and full access to all sectionsmay reveal new information about the lesions and their impact on other structures.Furthermore, since myxoma has a high recurrence rate, CBCT can be the method of choice forthe diagnosis and follow-up of OM cases. The authors would like to thank Dr. Shahrokh Raeisian, Department of Oral andMaxillofacial Surgery, and Dr. Saedeh Attarbashi Moghaddam, Department of Oral andMaxillofacial Pathology, Ahvaz Jundishapur University of Medical Sciences Dental School. AD, NN, and SB performed the clinical and radiographic examinations. NN and AG drafted themanuscript. All authors contributed to critical revision of the manuscript, and have readand approved the final manuscript. The authors report no funding for this article. The authors declare that they have no competing interests with regards to authorshipand/or publication of this paper. The authors declare that the individual, whose data were reported in this article, hasgiven written consent to the authors and the Ethics Committee of Ahvaz JundishapurUniversity of Medical Sciences for the publication of this paper.


Nasopharyngeal carcinoma is one of the most difficult tumors to diagnose correctly at the initial phase because of the occasional lack of nasal symptoms. The perineural spread of the trigeminal nerve is one of the most common and important routes in the intracranial paracavernous extension of nasopharyngeal carcinoma, but visual loss is very rare.in situ hybridization. Although focal radiotherapy induced remarkable tumor shrinkage and relieved ocular motor disturbance and facial pain, his visual acuity did not improve.We report the case of a 54-year-old Japanese man with nasopharyngeal carcinoma, who presented with rapid and severe disturbance of left monocular visual acuity and eye movement with a 10-month history of ipsilateral otitis media and facial pain. Magnetic resonance imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to the orbital apex through the superior orbital fissure. The histopathological diagnosis was nonkeratinizing undifferentiated nasopharyngeal carcinoma. Epstein–Barr virus was detected by The awareness of cranial nerves in addition to intracranial and orbital apex involvement, as in this case, is important for appropriate diagnosis and treatment planning of nasopharyngeal carcinoma. Nasopharyngeal carcinoma (NPC) is a relatively uncommon cancer with an incidence of 0.29 per 100,000 people per year in Japan , but visWe report here the case of a Japanese man with undifferentiated nonkeratinizing NPC, who presented with acute visual loss following facial pain and serous otitis media. We discuss the clinical features and diagnosis of this rare clinical entity.in situ hybridization  imaging revealed a lesion in the left fossa of Rosenmüller, pterygopalatine fossa, sphenoid and ethmoid sinus, and the left cavernous sinus extending to orbital apex through the superior orbital fissure  was negative. Therefore, adjuvant chemotherapy was not strongly advised and was eventually suspended. However, cervical lymph node metastases were detected by a repeat FDG-PET scan 8 months later. The patient underwent cervical lymphadenectomy followed by chemoradiation therapy. Although three cycles of cisplatin were scheduled, only one cycle could be completed because of leukopenia. He then received additional radiation therapy (60Gy/30 fractions/43 days). There was no recurrence 18 months after the initial treatment. However, left temporal and frontal lobe dural metastasis and lung metastasis were detected by repeat MR imaging and FDG-PET. Subsequently, he has received stereotactic radiotherapy and adjuvant chemotherapy with docetaxel hydrate, cisplatin, and fluorouracil. At present, 39 months after the initial treatment, he is still able to perform his daily activities .He received focal radiotherapy at a dose of 46Gy in 23 fractions, which remarkably reduced the tumor size and relieved left facial pain, facial palsy, facial hypoesthesia, ocular motor disturbance, hearing loss, and hypoglossal palsy. However, his left visual acuity did not improve. After the radiation therapy, The frequency of cranial nerve paralysis in NPC has a range of 8.0 to 12.4% ; cranialIn the present case, the patient was treated for left serous otitis media and left facial pain mimicking trigeminal neuralgia for 10 months before the acute visual loss. These symptoms indicate that the tumor originally occurred at the fossa of Rosenmüller, then spread along the V3 and invaded the cavernous sinus and the intraorbita. Although he had serous otitis media and facial pain 10 months before the orbital symptoms, the main apparent cranial nerve paralysis in this case was left optic nerve paralysis with acute visual loss. Furthermore, there was no actual evidence of the cancer until the orbital symptoms occurred. We found only four cases in three case reports of initial optic nerve presentation of NPC -6, in whGadolinium-enhanced, fat-suppressed, T1-weighted MR imaging is very useful in detecting perineural tumor invasion and extracranial lesions . In the In the present case, we performed an endoscopic tumor biopsy from nasopharyngeal tissue with a tentative diagnosis of malignant skull base tumor, but could not reach the pathologic diagnosis. Although we subsequently performed open biopsy by craniotomy, a transnasal endoscopic biopsy could have easily and less invasively been performed instead of craniotomy .In our patient, EBV was detected by EBER-ISH. The association of EBV is well established in nonkeratinizing NPC, particularly the undifferentiated subtype, detected in virtually all cases irrespective of the geographic origin. It seems reasonable to assume that EBV infection somehow contributes to the pathogenesis of nonkeratinizing NPCs . RecentlAt present, radiation therapy is the first choice for NPC, and cisplatin therapy is also thought to be effective ,8. The aAlthough his left vision has not been recovered, he still lives independently. This clinical course suggests that we should have performed early nasal endoscopic biopsy and initiated adjuvant chemotherapy immediately after the initial high-dose radiation therapy, with continuing administration to control tumor recurrence.NPC is one of the most easily misdiagnosed tumors because it does not present with nasal symptoms; rather, the patient may initially have unspecific signs and symptoms such as headache, hearing loss, and facial pain. Although symptoms often appear after intracranial invasion, early optic nerve involvement is very rare. If NPC is suspected, a rapid pathological diagnosis and a high-dose radiation therapy with early adjuvant chemoradiotherapy are essential to ensure prolonged patient survival.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.in situ hybridization; EBV: Epstein–Barr virus; FDG-PET: 18F-fluoro-2-deoxy-d-glucose positron emission tomography; MR: Magnetic resonance; NPC: Nasopharyngeal carcinoma; V3: The third division of the trigeminal nerve.CT: Computed tomography; EBER-ISH: EBV-encoded ribonucleic acid The authors declare that they have no competing interests.YK and NS devised the study design and concept, obtained the data and figures, drafted the manuscript and references, and carried out a critical review. GT, SB and HN reviewed the manuscript and added comments for discussion. All authors read and approved the final manuscript.


Haemangiomas of tongue are rare type of malformations. They can be treated mostly conservatively but in some cases they need more aggressive treatment with preoperative intra arterial embolization and surgical resection. Lesions of tongue that are localized superficially can also be treated with direct puncture and injection of sclerosing agent (absolute ethanol).We present a case of a 48 years old female patient, where we performed embolization of cavernous haemangioma with mixture of absolute ethanol and oil contrast. After the procedure the patient received analgetics and antioedematous therapy. After the sclerotization the planed surgery was abandoned. Control MRI examinations 6 and 12 months after the procedure showed only a small remnant of haemangioma and no signs of a larger relapse.In our case the direct puncture of haemangioma and sclerotherapy with ethanol proved to be a safe and effective method to achieve preoperative devascularization of the lesion. Direct puncture of the lesion is not limited by the anatomy of the vessels or vasospasm, which can occur during the intra-arterial approach. Vascular malformations of the tongue are relatively rare disorders. According to Mulliken and Glowacki they are divided into haemangiomas and vascular malformations .11The nomenclature in this field is very problematic, particularly in assessing benign vascular anomalies. Currently a variety of systems adapted for and by distinct clinical subgroups, like pathologists, clinicians, radiologist, are in use. In 1982, Mulliken and Glowacki proposed a binary classification system  for vasc2The ISSVA classification system divides vascular anomalies into 2 primary biological categories . The majHaemangiomas are the most common tumour of infancy and typically appear as a small reddish macule. About 80% occur within the first month of life. The macule quickly grows and becomes raised and lobulated. Majority of haemangiomas involve the skin, they can occur subcutaneously, appearing as a bluish patch under the skin. This can cause a rapidly developing swelling which then involutes as in the cutaneous lesion.The term vascular malformations (VM) refer to lesions where the anatomy and morphology of the vessels are abnormal although the vascular endothelium is normal. These lesions can either be high, low or mixed flow lesions.Classification that considers also clinical symptoms is named after Schobinger and has four stages, where stage 1 presents as a blue-skin blush, stage 2 as a mass associated with a bruit and a thrill, stage 3 as a mass associated with ulceration, bleeding and pain and stage 4 as a stage 3 lesions producing heart failure.1Each vascular abnormality requires a serious diagnostic approach. Based on the findings the most appropriate method of treatment is selected.The invasive treatment of the tongue vascular abnormalities is usually the preoperative endovascular embolization followed by the surgical resection of the lesion or partial resection of the tongue, depending on the size of the lesion.1A 48 years old female patient was admitted to our institution because of the difficulties in swallowing food and the swelling of the tongue. The otorhinolaryngology exam showed bluish, localized, approximately 35 × 25 mm big swelling on the upper side of tongue, with vessels shining through the glossal mucosa. The lesion was localised on the right side of the tongue in the middle part. The radix of the tongue was free. After the biopsy the histological examination of the tissue was performed and cavernous haemangioma was diagnosed.Contrast enhanced MRI with time resolved imaging of contrast kinetics sequence (TRICKS) was performed, so that the best method of treatment could be selected . The maxThe procedure was performed under general anaesthesia. This provided us the complete analgesia and prevention of tongue movement during the puncture and the injection of the ethanol and the contrast media.We performed parenchimography of the lesion using several projections to reach an optimal view of the lesion and to evaluate the potential extravasation of contrast from haemangioma . This enMost haemangiomas can be treated conservatively with an expectant strategy.Usually treatment of the tongue vascular abnormalities is performed by the intra-arterial catheterisation and embolization with particles or n-buthyl cianoacrilat (NBCA). Such a treatment has good results, but it is not suitable for all types of malformations.Direct puncture and the application of ethanol were initially described as an alternative method in cases where conventional intra-arterial catheter embolization was technically not possible.11Sclerotization must be carried out slowly so that the amount of ethanol entering the haemangioma vascular bed can be maximized. The movement of the sclerosing agent to avoid the passing to the venous side must be constantly monitored.13With our intervention we allowed a patient, which was intended to undergo a mutilant surgery with the resection of the tongue, a year and a half of normal life until now.Direct puncture of the cavernous haemangioma and sclerotherapy with alcohol proved to be a safe and effective method to achieve devascularization of the lesion and postpone or even avoid surgical resection.


Gallbladder rupture following blunt abdominal trauma is a rare event recognized on evaluation and treatment of other visceral injuries during laparotomy. Isolated gallbladder rupture secondary to blunt abdominal trauma is even more uncommon. The clinical presentation of gallbladder injury is variable, resulting in a delay in diagnosis and treatment. We report the case of a patient who suffered an isolated gallbladder rupture due to blunt abdominal trauma. Gallbladder injury arises from compressive and shearing forces, most commonly in motor vehicle accidents. Computed tomography is the best technique for diagnosing gallbladder injury. The treatment of choice is cholecystectomy.Isolated traumatic gallbladder injuries are uncommon and difficult to diagnoseHospital Geral do Grajaú complaining of abdominal pain for 3 weeks, which had worsened over the past 2 days. He reported no fever, vomiting or changes in bowel movements. He reported that 28 days prior he had fallen from a motorcycle and had been seen at another service where an abdominal computed tomography scan was performed due to abdominal pain. It revealed a small amount of free fluid. He remained hemodynamically stable and was discharged.AGF, a 27-year-old male, presented at the Emergency Room of On examination, he was in good general health conditions, afebrile, icteric and hemodynamically stable. The abdomen was tender on diffuse palpation and rebound tenderness.The laboratory test results were hemoglobin 14.2mg/dL, leukocytes 8,200 with no shifts, AST 65U/L, ALT 157U/L, alkaline phosphatase 371U/L, gamma GT 384U/L, total bilirubin 4.2mg/dL and amylase 104 U/L. Another abdominal CT was ordered and showed a large amount of free fluid .Exploratory laparotomy was recommended, which identified extensive bile peritonitis and an isolated perforation in the bottom of the gallbladder . Other hCholecystectomy was performed, followed by draining of the cavity.The patient recovered uneventfully and was discharged on the third postoperative day, with antibiotic therapy. At the time we reported this case, the patient was on postoperative day 20, asymptomatic, and followed on an outpatient basis.. Isolated perforation of the gallbladder is uncommon due to its protected anatomical position.Injuries in the gallbladder resulting from blunt abdominal trauma occur rarely and are usually associated with damage to other abdominal organs.Traumatic injury of the gallbladder is unusual. Its overall incidence varies from 0.8 to 2.1% in patients who suffered some type of abdominal trauma; 2% of patients undergoing laparotomy for traumatic injury are found to have a gallbladder injuryMost gallbladder injuries occur following motor vehicle accidents, significant falls, and direct blows in sports..Gallbladder perforation is more likely in cases when the gallbladder is distended and thin-walled at the time of injury. Although there has been an isolated case of injury secondary to a bull head-butting a patient's abdomen, there are no identifiable cases of damage occurring due to this mechanism.The presence of sterile bile in the peritoneal cavity causes a slight irritation, which explains the slow evolution. The patient may present with jaundice, which is due to the absorption of bile pigments by the peritoneum, as in this case.The diagnosis of the gallbladder lesion resulting from blunt abdominal trauma is usually confirmed during exploratory laparotomy. Frequently other more severe lesions in organs overlapping the gallbladder are observedWhen laparotomy is not done, there may be an interval of 1 to 6 weeks to diagnose a traumatic injury of the gallbladder, like in this case.. In this case, due to uncertain diagnosis, an exploratory laparotomy was chosen as the safest option.Cholecystectomy is the recommended treatment for gallbladder rupture and major tearing. Partial cholecystectomy has also been described in the literature and is an option in selected cases. Laparoscopic cholecystectomy is advocated as a safe and effective procedure for diagnosis and management of traumatic gallbladder rupture


Silent adrenocorticotrophic pituitary adenomas are nonfunctioning pituitary adenomas that express adrenocorticotrophic hormone (ACTH) but do not cause the clinical or laboratory features of hypercortisolemia. Primary central nervous system (CNS) melanoma is well documented, but rarely originates in the sellar region or pituitary gland. Here we report transformation of an aggressive silent adrenocorticotrophic pituitary adenoma that transformed into CNS melanoma and review other presentations of pituitary melanoma. A 37-year-old woman initially presented with apoplexy and an invasive nonfunctioning pituitary macroadenoma for which she underwent transphenoidal surgery. The patient underwent 3 subsequent surgeries as the tumor continued to progress. Pathology from the first 3 operations showed pituitary adenoma or carcinoma. Pathology from the final surgery showed melanoma and the magnetic resonance imaging characteristics of the tumor had changed to become consistent with CNS melanoma. Dermatologic and ophthalmologic examinations did not identify cutaneous or ocular melanoma. The patient’s disease progressed despite aggressive surgical, medical and radiologic treatment. To our knowledge, this is the first report demonstrating transformation of a primary pituitary tumor into melanoma. The mechanism of tumor transformation is unclear, but it is possible that a mutation in the original ACTH-producing tumor lead to increased cleavage of pro-opiomelanocortin or ACTH into α-melanocyte-stimulating hormone, which in turn stimulated the expression of microopthalmia transcription factor, leading to melanocytic phenotype transformation. A 37-year-old woman with no significant medical history presented to an outside hospital with acute headache and vision loss. Magnetic resonance imaging (MRI) showed a pituitary macroadenoma with invasion of the cavernous sinus, compression of the optic chiasm, and a cystic posterior component. Endocrine evaluation at the time of diagnosis was consistent with a nonfunctioning tumor. The patient underwent emergent transphenoidal surgery and subtotal resection. Pathological diagnosis was pituitary adenoma. Five years later, the patient underwent fractionated radiosurgery for tumor progression in the right cavernous sinus. Subsequently, her MRIs remained stable; however, the patient developed hypopituitarism.The patient was initially referred to our clinic at 45 years of age. At that time, MRI showed an enhancing mass in the right cerebellopontine angle and cavernous sinus, without residual tumor in the sella . On follAt age 49 years, MRI showed tumor progression in the posterior fossa, along the tentorium, right frontoparietal, and right temporal lobes. The patient underwent radiosurgery to the right temporal and right frontoparietal lesions, Six months after radiosurgery, the patient presented to our emergency room with left-sided weakness. MRI showed a contrast-enhancing right temporal dural-based lesion with extensive edema and mass effect . A crani1 One month later, MRI revealed further tumor progression, and cabergoline was discontinued. Temozolomide, which has also been shown to be effective against pituitary adenomas2 was then initiated. After 2 courses of temozolomide, the patient developed left lower extremity weakness, right facial weakness, and hearing loss. MRI showed extensive parasellar disease encasing the right internal carotid artery, entering the posterior fossa, jugular foramen, internal auditory canal, foramen magnum, and along the gyrus rectus . At age 51, she complained of double vision and facial numbness, and MRI showed progression of the tumor in the cavernous sinus and along the tentorium with compression of the right cerebral peduncle. She was initiated on carbergoline based on a case report of a silent corticotroph tumor responding to this dopamine agonist.s rectus . At thiss rectus . Imunohis rectus  and posis rectus . After ts rectus . After d3 in 1978 as pituitary adenomas that produce ACTH but do not cause hypercortisolemia. In our case, pathology evaluation initially supported the diagnosis of SCA. Based on the imaging characteristics during the 6 years of follow-up at our institution, the tumor transformed into a silent corticotroph carcinoma. Although this progression has been previously reported,4 there are no reports of SCAs transforming into melanoma or other tumor phenotypes.Silent adrenocorticotrophic pituitary adenomas (SCAs) were first described by Kovacs et al5 This patient had 3 pathological diagnoses all showing pituitary adenoma or carcinoma, 2 of which, when analyzed at our institution, showed positive ACTH immunoreactivity and no immunoreactivity for melanoma markers. Therefore, there is no doubt that the patient’s original CNS disease was of pituitary origin. Interestingly, as the patient underwent repeat MRI scans, lesions were detected at the foramen magnum and within the upper cervical spinal canal, which are sites of melanin production in the CNS. On the first MRI in which these lesions appeared, they were seen to be contrast enhancing but T1 dark, as was the patient’s original parasellar disease. On the patient’s final MRI, these lesions were T1 bright, as well as contrast enhancing, again, like the patient’s parasellar disease. Unfortunately, no specimens were available from these lesions, so the histology or possible histological change of these lesions is unknown. Regardless, the parasellar disease, which was resected during the first and final operations at our institution, clearly underwent transformation between those surgeries. Imaging supports the hypothesis that all the patient’s tumor burden underwent similar transformation.Primary central nervous system (CNS) melanoma is rare, especially in the sellar region.7 the incidence of collision tumors in the sella is less than 2%.8 In the series by Koutourousiou et al,8 all the collision tumors were composed of pituitary adenomas with other tumors or lesions of the CNS such as another pituitary tumor, Rathke’s cleft cyst, sarcoid, or gangliocytoma. There is no report that we are aware of describing CNS melanoma and a pituitary tumor occurring together. The patient underwent complete dermatologic and ophthalmologic exams after her final surgery, neither of which revealed any melanotic lesions. Therefore, the primary melanotic lesion would have to have been extracutaneous, which is rare.9 Additionally, the radiographic transformation of the tumor from a T1 dark to T1 bright, and the loss of all pituitary adenoma markers in the final pathological specimen support a diagnosis of tumor transformation rather than collision.The possibility that this lesion was a collision tumor of 2 different pathologies at the same location is unlikely. Although there are reports of collision tumors in the pituitary region,10 glioblastomas,11 and ependymomas12 have all been shown to rarely express melanin. However, these tumors still retained their previous phenotype even as they expressed melanin. This is the first report of a pituitary tumor undergoing melanocytic differentiation and losing its expression of its previous antigens.There are previous reports of CNS tumors exhibiting melanocytic differentiation. Several CNS tumor types, including astrocytomas,7 Reports of primary melanoma of the sellar region are even rarer.1316 In these cases of isolated pituitary melanoma, the cellular site of origin has been hypothesized to be the meninges,14 a Rathke’s cyst,13 or the pituitary gland itself.17 In these cases, like ours, there were no melanotic lesions found outside the CNS. However, no prior description of primary pituitary melanoma involved transformation of one tumor phenotype to another, as was the case in the patient we describe.Cases of melanoma metastatic to the pituitary have been reported, though this is a rare occurrence.18 binds to extracellular receptors, and induces MITF expression, a key transcription factor for melanocyte differentiation and survival.19 It is possible that a mutation in the original ACTH-producing tumor lead to increased cleavage of POMC or ACTH into α-MSH, which in turn stimulated the expression of MITF, leading to melanocytic phenotype transformation. It has been shown that nonfunctional macroadenomas produce increased levels of ACTH precursors.20 This would magnify the effect of POMC being cleaved to form α-MSH. Regardless of the mechanism, the potential of a SCA to transform into melanoma demonstrates the aggressive behavior of SCAs and reveals a unique origin of pituitary melanoma.The exact mechanism of this tumor’s transformation is unclear. This patient received multiple rounds of radiation therapy, which may have predisposed the tumor to additional mutations. The mechanism of tumor transformation likely involves the processing of the ACTH. ACTH is derived from pro-opiomelanocortin (POMC) and is further processed in the pituitary into α-melanocyte stimulating hormone (α-MSH), which also results from POMC cleavage. α-MSH induces skin pigmentation,


An inflammatory myofibroblastic tumor (IMT) is an immunohistochemically diverse entity demonstrating neoplastic and nonneoplastic qualities. Although IMTs can arise in any area of the body, lesions arising in certain sites, namely, the nasal cavity, paranasal sinuses, and pterygopalatine fossa, demonstrate a heightened neoplastic and invasive potential. Despite case specific complete tumor regression and disease remission in response to pharmacotherapeutics, a subset of IMTs remain resistant to all forms of therapy. We present such a case, a 34-year-old female patient, with a highly resistant, maxillary sinus IMT. Her refractory, ALK-1 negative IMT has not responded well to novel therapies reported in current literature. This case suggests the role of zonal expressivity within a single lesion as a probable mechanism for its highly resistant nature and should promote determination of each IMT's cytogenetic profile to provide more effective targeted therapy. Paper includes a literature review of all maxillary sinus IMTs from 1985 to 2014 along with their immunohistochemical staining, treatments, and outcomes. An inflammatory myofibroblastic tumor (IMT) is an immunohistochemically diverse entity demonstrating neoplastic and nonneoplastic qualities first described by Brunn in 1939 . Until rAlthough the term inflammatory myofibroblastic tumor was coined by Umiker and Iverson in 1954 , the proTreatment, in like manner to pathological diagnosis, is often a quandary for surgeons and oncologists alike. To date, complete surgical excision or corticosteroid therapies of IMTs are the gold standards of treatment . HoweverA 34-year-old Hispanic female presented with complaints of progressive nasal congestion and mass in the right nasal cavity. Initial biopsy of the mass was felt to be benign. A year later, her right nasal mass recurred and again was reexcised with pathology reported as benign. A few months after her second surgery the mass had rapidly grown in size filling her mouth and causing right facial swelling . She hadThe lesion was biopsied and sent to two referral centers for assistance in making the diagnosis. The first referral center determined that the lesion consisted of a “polypoid sinonasal mucosa with ulceration, with acute and chronic inflammation and spindle cell proliferation, favoring reactive changes.” Immunohistochemical stains including smooth muscle antibody (SMA), desmin, S100, and keratin were all negative.The patient went to second referral center for further treatment. At this institution, the report was slightly different, determining the sample to be consistent with an IMT, but found it had scattered positivity for ALK-1 and SMA within spindled cells. This is significant, because a diagnosis of IMT is quite difficult; however, one would expect samples from the same tumor biopsy to show similar SMA staining. The complexity of this tumor is further noted in that another biopsy, three years following the initial pathology report, differed from both of the previous reads.Most recently, the second referral center reported immunohistochemistry stains demonstrating a profile different than the prior biopsies, with rare tumor positivity for SMA, and negativity for S100, pan-keratin, p63, and ALK-1. Next-Generation Sequencing- (NGS-) based analysis for detection of somatic mutations revealed TP53 and KRAS mutations. It was felt that pathology was consistent with inflammatory myofibroblastic tumor Figures . They deIn the beginning of July 2014, the patient was seen at our institution. At a multidisciplinary tumor board conference, it was decided that the best courses of action would be combination radiation and chemotherapy. She received a total dose of 60 Gy in 6 weeks using IMRT. She was also treated with ifosfamide, dacarbazine, and mesna as well as celecoxib. She recently completed four cycles of this regimen and a follow-up CT demonstrated overall tumor regression to a measurement of 4.9 cm at its greatest dimension as compared to 7.1 cm at initial presentation Figures .Since its first known appearance in medical literature in 1939, the inflammatory myofibroblastic tumor has garnered the attention of pathologists, surgeons, and oncologists, alike, due to its marked idiosyncrasies in immunohistology, pathophysiologic behavior, and therapeutic response . In pastThe definitive etiology of IMT has yet to be fully elucidated, but a great deal of proponents agree its development is largely multifactorial involving both inflammatory and chromosomal aberrations –23. UndeTo date, many cellular markers have been identified, including desmin, vimentin, smooth muscle actin, cytokeratin, and ALK-1 that aid in the pathologic diagnosis of IMT. Of particular interest is anaplastic lymphoma kinase or ALK-1 as well as its essential role in differentiating IMT from other spindle cell neoplasms that fall within the broad category of “inflammatory pseudotumors.” According to studies conducted by Lawrence in 2000 and Coffin in 2001, greater than 50% of soft tissue IMTs possess chromosomal translocations involving the short arm of chromosome 2 and the ALK tyrosine kinase receptor locus –19, 24.The diagnostic value of ALK-1 positivity is evident, considering most of the neoplastic counterparts of IMTs including desmoid fibromatosis, nodular fasciitis, calcifying fibrous tumor, myofibromatosis, and infantile fibrosarcoma are negative for ALK. Despite the obvious identification value of ALK-1 status in these neoplasms, the clinical and prognostic significance remains uncertain with some researchers suggesting unsubstantial value in ALK-1 status. However, adequate evidence exists to demonstrate the role of ALK-1 in conveying metastatic and invasive potential to IMTs. In fact, several recent retrospective studies have shown a marked increase in metastasis and recurrence in IMTs that are ALK-1 negative when compared to ALK-1 positive lesions , 25, 26.In light of such information, ALK-1 negativity alone, as seen in the case presented above, demands an aggressive therapeutic approach and increased vigilance for distant metastases or local recurrence. Additionally, as with any unregulated cell growth resulting in tumor formation, chromosomal aberrations leading to cellular atypia, nuclear pleomorphism, abnormal mitotic rate, DNA aneuploidy, and tumor suppressor inactivation have been shown to be beneficial in predicting IMTs  This heiDespite the IMT predilection for developing in the lung, abdominopelvic, retroperitoneal, and extremities in adolescent and pediatric populations, this rare tumor has been shown to occur in a vast age range and in a great number of locations , 24. TheOf particular interest regarding the etiology of IMT is the role both the immune system and its host of inflammatory mediators play in the development and persistence of these rare entities , 33–35. In the face of such early evidence and the relatively consistent presentation profile within pediatric intrapulmonary lesions, no surprise IMTs have struggled to shed their recurrent inclusion into the misnomer of inflammatory pseudotumor, even in recent years. However, due to the work of Meis and Enzinger in 1990 and Coffin in 1995 involving intra-abdominal and retroperitoneal tumors , 28, theSpindle cells within a vascularized myxoid stroma and an inflammatory infiltrate of neutrophils and eosinophils.Compact spindle cells within a collagenized stroma and storiform architecture and an inflammatory infiltrate of plasma cells and lymphocytes often forming germinal centers.Elongated spindle cells within a hypocellular highly collagenous stroma and a variable inflammatory infiltrate of lymphocytes, plasma cells, and eosinophils.Lymphohistiocytic variant consisting of myofibroblastic spindle cells and foamy histiocytes. This is thought to represent the most inflammatory variant.With regard to the case in question, it is important to point out the fact that significant overlap in cellular populations can occur and the phenomenon of maturation and zonation within a single tumor has been described: thus, adding to the complexity of affective histological classification and ultimate diagnosis [Just as erratic immunohistochemical characteristics seem to define IMT, differences in the cytological makeup of IMTs have been described and categorized into four cellular variants, largely modified from the three stromal classifications first described by Coffin et al. in 1995 , 4. The As for the case at hand, two prominent institutions obtained differing pathology reports and scattered reactivity of the previously discussed immunohistochemical markers within the same tumor sample before being seen at our institution. The occurrence of zonation and maturation explains this discrepancy. Additionally, and perhaps most importantly, the awareness of such a phenomenon within IMTs is paramount to successful diagnosis and treatment of inflammatory myofibroblastic tumors. It is likely that differential expression within the same IMT lesion explains disease resistance to some degree, as well.Although the mainstay for successful treatment of IMT has been complete surgical excision, cases like that presented above often prove problematic for surgeon and oncologist, alike. Despite the difficulty, standard and novel pharmacotherapies, including NSAIDs, COX inhibitors, corticosteroids, and kinase inhibitors, are readily available for treatment of refractory, recurring, or nonresectable disease as evidenced in the literature on IMTs . VariablRecall that crizotinib, an ALK-1 inhibitor, was used by second referral center as therapy after unsuccessful surgical excision. This, no doubt, was initiated in hopes the scattered ALK positivity initially present in histologic sections would be inhibited, leading to regression and death of the tumor. The resistance of this particular lesion to such treatment, again, sheds light on the tremendous zonal and maturative expressivity profiles IMTs can possess. A second unique and fascinating characteristic of IMT is the often astounding regression when COX inhibitors like celecoxib and systemic steroids have been administered, a fact of which oncologists involved in the above case were well aware . This hiIn the age of detailed cytogenetic analysis, refined imaging techniques, and precisely targeted therapeutic regimens a greater degree of time should be dedicated to discovering a unique cytogenetic “profile” for each IMT case.There is no denying this complex neoplasm demonstrates variation in the form of zonal expressivity, and overcoming this phenomenon will continue to be the challenge posed to all providers dealing with this rare tumor. Likewise, the role of specific cellular proteins such as ALK-1 will begin to serve as markers or the use of targeted therapy. Thus, physicians should emphasize effective determination of the cytogenetic profile of all IMTs, as well as a systematic and aggressive approach to IMTs presenting in areas shown to be refractory to many types of treatment, that is, paranasal sinuses. All things considered, the essential nature of taking a multidisciplinary approach with pathologist, surgeon, and medical and radiation oncologist providing concerted and comprehensive care is the foundation of proper IMT management.


Atypical infratemporal fossa infections are rare and potentially fatal.A case of an aspergillosis localized in the infratemporal fossa and another case of tuberculosis of the infratemporal fossa originating from the maxillary sinus, is described. The first patient was immunocompromised and showed symptoms of facial numbness; whereas the other was an immunocompetent man who complained of trigeminal neuralgia type pain. It was difficult to differentiate between infection and tumour despite the utilization of computed tomography scans and magnetic resonance imaging.These cases illustrate the need for a high index of suspicion; in addition to endoscopic confirmation and histopathology to establish precise diagnosis and early intervention. Atypical infratemporal fossa (ITF) infections are extremely uncommon and can be potentially fatal. They have been known to arise from odontogenic infections, dental extractions, maxillary sinus wall fractures, and maxillary sinusitis -5. Two uCase 1: A 37–year-old immunocompro- mised post renal transplant man presented himself to the Otolaryngology Department with a four-month history of left facial numbness and left upper molar pain that persisted despite dental extraction. There were no other otorhinolaryngological symptoms. Past medical history revealed that the patient was on an immuno- suppressive regime and suffered from hypertension. Clinical examination and flexible nasoendoscopy findings were unremarkable. Computed tomography (CT) scan and magnetic resonance imaging (MRI) reported an irregular soft tissue infiltrating the temporalis and lateral pterygoid muscle . There wThe pre-operative diagnosis was a left maxillary sinus tumour. The patient underwent a left endoscopic excision of the tumour via maxillotomy. Peri-operatively, it was noted that the lesion had a light yellowish colour and was solely confined to the ITF with no involvement of the maxillary sinus. Histopathological examination reported granulation tissue with inflammatory cells and fungal hyphae. Apergillus fumigatus was confirmed during fungal culture of the specimen. The final diagnosis was localised aspergillosis of the ITF.Post-operative recovery was uneventful and the patient was commenced on a six-week course of voriconazole. As there was minimal improvement in the patient’s condition and slight reduction in the size of the mass, he underwent further debulking via an extended endoscopic medial maxillectomy. He was followed up for a year with no signs of recurrence.Case 2: A 56-year-old immunocompetent man presented himself to the Otolaryngology Department with a three-month history of left facial pain and swelling, limited mouth opening, and left upper molar pain, which persisted despite dental extraction. There were no other otorhinolaryngological symptoms. Past medical history was unremarkable with no apparent immune deficiencies.Clinical examination revealed marked trismus with interincisal opening of 4 mm, making intra-oral examination difficult with an indurated swelling over his left maxillary region. Flexible nasoendoscopy showed medialisation of the left medial wall of the maxilla. CT scan and MRI reported a soft tissue mass in the maxillary sinus with involvement of the left pterygoid muscles and left masseter muscle . There wPost-operative recovery was uneventful. The patient was started on an antitubercular regime and was followed up for a year with no recurrence.The ITF is defined as a space bound anteriorly by the posterior surface of the maxilla, posteriorly by the mastoid and tympanic portions of the temporal bone, superiorly by the greater wing of the sphenoid and the squamous portion of the temporal bone, medially by the pterygoid process of the sphenoid, laterally by the zygomatic arch and ascending ramus of the mandible, and inferiorly by the posterior belly of the digastric muscle and the angle of the mandible. Its contents are the temporalis muscle, medial and lateral pterygoid muscles, pterygoid venous plexus, the maxillary artery and its branches, the mandibular nerve and the chorda tympani.The presence of ITF infections is largely determined by which part of the anatomy is affected. Facial swelling can manifest as a result of an abscess occupying the ITF and fullness of this area is usually apparent on clinical examination. Trismus also occurs as a result of extension to the pterygoid muscles. Additionally, there may be trigeminal neuralgia type pain, hypothesia or paraesthesia that is indicative of the mandibular nerve involvement. Interestingly, both these patients presented different symptoms, with one complaining of facial numbness whereas the other had facial swelling and trigeminal neuralgia type pain. Hence, even if the clinical signs and symptoms are rational from an anatomic basis, the diagnosis of ITF infections remains a challenging task as they can present themselves in a variety of ways.Imaging studies are fundamental for diagnosis and surgical planning. CT can distinctly distinguish between cellulitis and abscess formation whereas MRI is better for assessing soft-tissue. Nevertheless, it is often difficult to differentiate between infection and tumour, particularly in these cases where radiological and clinical features are nonspecific. Chronic infections may mimic neoplasm by showing signs of trismus and swelling without fever; as opposed to acute infections where there is usually pain and fever. In fact, our initial impression was a maxillary sinus tumour. As such, endoscopic confirmation with histopathology and culture are necessary for a definitive diagnosis. Achieving the correct diagnosis is crucial so that effective therapy can be tailored according to the pathology. In the first case of aspergillosis, surgical treatment was necessary when medical therapy alone failed. It is concluded that endoscopic debridement combined with systemic antifungals is imperative to improve survival, especially in immunocompromised patients. The second patient with TB improved significantly once he was commenced on an antitubercular therapy. To the best of our knowledge, the first case of aspergillosis solely localized in the ITF is reported. Goyal et al reported four patients presenting invasive fungal sinusitis with infratemporal fossa extension . The rouOur second patient presented an exceedingly rare case of TB in the ITF with the primary involvement of the maxillary sinus. Very few cases, documented in literature, show the maxillary sinus as the primary site of infection -10. The The venous drainage of the ITF is done through the pterygoid plexus, which anastomoses with the cavernous sinus, ophthalmic veins, and pharyngeal venous plexus. As a result, infections involving the ITF can spread haematogenously into the cavernous sinus and orbit leading to ominous consequences . InfectiThese cases illustrate the need for a high index of suspicion to achieve a precise diagnosis so that the treatment can be tailored according to the pathology. We believe that prompt diagnosis of the disease based on radiological and pathological findings and early intervention are crucial in establishing a positive outcome.


Fungal infection of the orbit is rare especially among immunocompetent patients. We present a 9-year–old girl with peri-orbital, eyelid and internal canthus swelling of the left eye. Clinical impression was suggestive of malignant tumor such as rhabdomyosarcoma or lymphoma. Histopathological examination of biopsied tissue revealed necrotizing granulomatous inflammation confirmed as fungal infection. Complete response to antifungal therapy was noted after four months. Orbital lesions are relatively uncommon and caused by inflammatory and immunological disorders or secondary to systemic diseases such as thyroid ophtalmopathy or less likely due to idiopathic inflammatory pseudo tumors.[1] Orbit is also an organ which may be involved by primary or secondary malignant lesions such as lymphoma, metastatic carcinomas, or neoplasms of adjacent tissues such as conjunctiva, paranasal sinuses, eyelids, or even intracranial neoplasms.[2] Orbital inflammatory lesions are composed of infectious or non-infectious cellulitis. Orbital infectious cellulitis is divided into bacterial, non-bacterial and fungal types.[3] Fungal orbital infection is rare in immunocompetent patients especially in the childhood.[4] We report fungal orbital cellulitis in a 9-year-old immunocompetent female who presented with a large mass like lesion, mimicking neoplastic lesion.A 9-year-old girl was admitted with large mass like lesion in her left orbital area for a month. There was pain and swelling at the internal canthus and over nasal septum near left orbit. Past medical history showed no allergic rhino-sinusitis, trauma or surgery on her eyes. On inspection no red eye was detected but the mass was firm and had a violet to pinkish colored surface. Her visual acuity was not disturbed and she had no limitation of motion in her left eye. Her mother also pointed out that she used to like and play with vegetables in the garden.CT scan showed a large mass in the left ethmoidal air cells, left maxillary and frontal sinuses and extending to the medial aspect of extraconal causing mass effect on the left glob to the lateral side and also thinning of the medial bony wall of the left orbit . Obstruction of the left osteomeatal complex (OMC) was also depicted. The patient underwent biopsy of the lesion for possibility of malignancy. Histopathological examination revealed fibrovascular connective tissue infiltrated by inflammatory cells mostly eosinophils, neutrophils and lymphocytes, with areas of necrosis and granulomatous reaction composed of multinucleated giant cells. Giant cells engulfed fungal hyphae which were thin walled and non–septate that was confirmed by Periodic Acid Schiff (PAS) staining for Zygomycete infection .She was treated with itraconazole, 200 mg twice daily for one week and then 200 mg daily for four other months. After antifungal therapy, the orbital swelling disappeared.Fungal infections mostly spread from rhino-sinus involvement especially in the patients with history of allergic rhino sinusitis, diabetes mellitus and among those with immunosuppression states such as patients with lymphoma or leukemia undergoing chemotherapy. Other routes of orbital fungal infection could be transcutaneous penetration of fungus by trauma or hematogenous spread from primary infected sources such as pulmonary or CNS infection. Direct infection via globe, lacrimal sac, or facial structures has also been reported.[4] No such predisposing factor was found in the index case.Primary orbital tumors in childhood are mostly, congenital teratoma, lymphoma, rhabdomyosarcoma as a malignant lesions and epidermoid or dermoid cysts, neurofibroma and hemangioma as a benign lesions. Orbital masses have variable presentation such as optic neuritis, sub-periosteal abscess, optic neuropathy, orbital apex syndrome, ophtalmoplegia, epiphora and Duane syndrome.[6] Zafar et al reported fungal orbital infection in an 8-year-old male after non-penetrating trauma to the eye, but our case had no history of trauma to sinuses or orbit. CT scan and MRI are best imaging modalities for fungal infections to describe the soft tissue involvement and extension of inflammation via sinuses or other organs near the orbit.[7] To conclude, rare infections can mimic malignant lesions and effort should be made to diagnose them early.Source of Support: NilConflict of Interest: None declared


Hepatocellular carcinoma is a major health issue, ranked the fifth most common tumor and currently being responsible for a third of the cancer-related deaths globally, with an ever-increasing number of fatalities. Current advances in contrast-enhanced imaging techniques such as contrast-enhanced ultrasonography, multi-detector computed tomography and diffusion-weighted magnetic resonance imaging are improving the rate of hepatocellular carcinoma diagnosis. Contrast-enhanced ultrasonography has widely become the first choice in liver tumor assessment, as it is faster, simpler and safer than other forms of diagnostic imaging. On the other hand, cross sectional computed tomography is frequently employed when a hepatic formation is suspected of malignancy and allows a more accurate characterization of lesions through multiphasic multi-detector computed tomography technology. Diffusion weighted magnetic resonance imaging represents another addition to the wide range of diagnostic and prognostic techniques available for patients with hepatocellular carcinoma and is currently regarded as one of the best tools for the characterization of these lesions. Furthermore, groundbreaking biomarkers for hepatocellular carcinoma are being discovered, although alpha-fetoprotein remains one of the most frequently used serum test in the early stages. Nonetheless, further advances are required for the detection of small liver carcinomas.Abbreviations : AASLD = American Association for the Study of Liver Diseases, AFP = Alpha-fetoprotein, AFP-L3 = Alpha-fetoprotein isoform 3, CEUS = Contrast-enhanced ultrasonography, DCP = Des-gamma-carboxy-prothrombin, DW-MRI = Diffusion weighted magnetic resonance imaging, FNA = Fine neddle aspiration, Gd-EOB-DTPA = Gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid, GGT-II = Gamma-glutamyl transpeptidase II, GP73 = Golgi protein 73, HCC = Hepatocellular carcinoma, HCCR-1 = Human cervical cancer proto-oncogene 1, IL-18 = Interleukin 18, MDCT = Multi-detector computed tomography, PET-CT = Positron emission tomography – computed tomography, SUV = Standardized Uptake Value Alternatively, advances in various imaging techniques (contrast-enhanced ultrasonography (CEUS), multi-detector computer tomography (MDCT), diffusion weighted magnetic resonance imaging (DW-MRI) have significantly improved the detection of hepatocellular carcinoma, with limitations of less than 2 cm in diameter in the case of liver tumors, where further studies are required [2]. An accurate screening method or a combination of methods for early HCC detection are often evaluated based on sensitivity and specificity rates, as these statistical indicators are not dependent on disease prevalence and offer the possibility of comparing studies performed in populations with varying characteristics [3].Hepatocellular carcinoma (HCC) is a major health issue, as recognized by the World Health Organization (WHO), being ranked the fifth most common tumor, currently responsible for a third of cancer-related deaths on a global scale, with an ever-increasing number of fatalities. As a result, there have been numerous attempts to establish a set of guidelines for an early liver cancer diagnosis and long-term prognosis based on different serum biomarkers such as alpha-fetoprotein (AFP), des-gamma-carboxy-prothrombin (DCP) or other novel markers that are currently being studied . The echographic contrast agent approved for use in Europe (SonoVue) is composed of small micro-bubbles, which can flow inside capillaries and, as a result, can generate a map of the intratumoral vascularization with a high degree of accuracy [5]. The evaluation of hepatic nodular lesions undergoes three vascular phases: the arterial and portal phases, which occur during the first 2 minutes after the contrast agent is injected, and a late phase that lasts until the contrast is eliminated. The typical appearance of HCC in contrast enhanced ultrasound is usually hyperenhancement in the arterial phase, with a chaotic vascular pattern . In the portal venous and late phases, HCC usually shows hypoenhancement apart from well-differentiated HCC that may be iso-enhancing  [6]Contrast-enhanced ultrasonography, a non-invasive diagnostic method for various types of hepatic tumors such as hemangioma, focal nodular hyperplasia or adenoma, has recently been introduced as an imaging technique useful for differentiating HCC from cirrhosis nodules based on the different vascularization patterns of the two pathologic entities . Hepatic carcinogenesis is a multistage process, which frequently has a cirrhosis regenerative nodule as a starting point. During CEUS, this type of lesion does not differ from the surrounding parenchyma and, consequently, is not visible in any vascular phase. For this reason, any change in behavior during the arterial phase, such as hypervascular images, can be suggestive for a dysplastic nodule, which can be very difficult to differentiate from early HCC Fig. 4). This method allows the acquisition of multiple cross sections simultaneously and at rapid speeds, which in combination with an intravenous contrast agent allows a multiphasic analysis of liver parenchyma: a pre-contrast phase, an arterial phase and a portal phase of approximately 30 seconds each and a delayed phase up to 3 minutes [12].Cross sectional computer tomography is frequently the first diagnostic imaging technique employed when a malignant hepatic lesion is suspected and, currently, a more accurate characterization of this type of lesions can be obtained by using multiphasic multi-detector CT technology , whereas a significant number of well differentiated together with poorly differentiated HCC showed hypoattenuation in the arterial phase. A possible explanation for these findings may be related to the gradual changes in tumor vascularization that occur during hepatic carcinogenesis, ranging from reduced blood flow during early angiogenesis in well differentiated tumors to increased vascularization for those moderately differentiated and continuing with a decrease in blood supply as the tumor becomes less differentiated. Furthermore, the correlation between the length of time necessary for contrast washout during portal phase and differentiation grade (faster washout as tumor grading increases) allows a better tumor characterization and is a valuable tool for prognosis and a correct treatment scheme [13]. Other studies suggest that further optimization can be achieved by assessing tumor washout both in the portal as well as in the delayed phase because certain tumors, especially the smaller ones, present late hypoattenuation and can remain unnoticed  .A 2011 retrospective study conducted over the course of 7 years on 243 patients with histologically confirmed HCC analyzed contrast enhancement patterns on MDCT and their correlation with tumor differentiation based on attenuation, washout and various other case specific factors such as necrosis, aneurysms or dysmorphic intratumoral vessels. According to the results, the typical pattern of hypervascularization normally seen in HCC was most frequent in moderately differentiated tumors and only partly present in well differentiated carcinomas  present a higher uptake in malignant cells, yet due to the normally increased glucose metabolism of hepatocytes, there are some challenges in successfully applying PET-CT as a diagnostic tool for liver carcinomas [17]. It has been proven that PET-CT is essential in establishing the extent of metastasis  in patients already diagnosed with HCC, therefore offering a reliable long-term prognosis.Currently, there is a growing interest in determining the feasibility and effectiveness of PET-CT in HCC detection and also in establishing which metabolic tracer offers the most accurate results. Due to the fact that this method focuses mainly on metabolism intensity, quantified through the standardized uptake value (SUV), and less on anatomical morphology, it is commonly combined with MDCT in order to optimally pinpoint the location of areas with atypical metabolic rates [18]. Numerous studies have been conducted on various techniques of enhancing PET-CT detection rate of primary HCC and secondary metastatic sites. One of them focuses on a dual-phase whole body scan. Phase one consists of a full body scan 90 minutes after the tracer is injected and is followed by phase two, a second scan is performed only in the abdominal region, in order to allow an increased ratio between the tumor and the normal parenchyma SUV and a better contrast. Although analyzed only in a limited number of studies, this approach appears to be promising, as it may improve primary HCC detection by up to 20%, and allow a more exact delimitation of tumors in relation to unaffected surrounding liver tissue .However, a recent study conducted on 138 patients with confirmed liver carcinomas revealed that, when compared to other ultrasonic or tomographic diagnostic methods, PET-CT is significantly less sensitive as a detection tool [21]. Despite the increase in the sensitivity of up to approximately 80%, a major drawback is that both FDG and 11C-Acetate PET-CT reach this sensitivity only for tumors of over 5 cm, whereas small tumor detection rate (1-2 cm) is less than 40% [22]. An alternative option is the association of dual tracers such as FDG and 11C-acetate, a substance involved in lipid metabolism, which increases PET-CT sensitivity. Certain tumors that remain undetected with one tracer may become visible when using another [Magnetic Resonance Imaging (MRI)23]. DW-MRI has proven itself a more accurate tool than the conventional T1 or T2–weighted MRI [24]. The use of a 3T (Tesla) MRI in conjunction with subsequent Dynamic Contrast Enhanced and Diffusion are regarded as the best diagnostic tool for HCC , although some limitations still exist for tumors under 10 mm [25].Diffusion weighted MRI (DW-MRI) represents another addition to the wide range of diagnostic and prognostic techniques available for patients with HCC. The functioning principle of this method is based on the diffusion capacity of water molecules, which varies depending on the density of cells in different areas of the studied tissue. As a result of the increased cellularity characteristic to tumors, liquid particles are displaced over shorter distances under the action of the magnetic field in comparison to normal cellular architecture [26]. From a different perspective, two studies that assessed 162 tumor nodules and 83 respectively showed that, although DW-MRI is more susceptible to artifacts than MDCT, its greater contrast offers comparable or even superior sensibility in liver carcinoma diagnosis. Furthermore, for small HCC (<10 mm), this novel MRI detection instrument is twice as accurate as the 64-row multidetector CT [27]. With the development of hepatocyte-specific magnetic resonance imaging contrast agents such as gadoxetic acid disodium (Gd-EOB-DTPA), there is an interest in combining multiple MRI techniques in order to obtain the best possible sensibility without affecting specificity, especially in hipovascular tumor .A 2011 study revealed that contrast-enhanced imaging such as Dynamic Contrast Enhanced MRI offers reduced sensibility for small liver tumors due to the difficulty in distinguishing venous washout phase on images less than 1 cm. Although for larger tumors, DW-MRI and Dynamic Contrast Enhanced MRI offer similar results, for lesions of this size sensibility increases from 72% to 92%, thus providing a viable option for early HCC detection [Fine Needle Aspiration Liver Biopsy28]. This is opposed to the Asian Pacific Association for the Study of the Liver and EFSUMB (European Federation of Societies for Ultrasound in Medicine and Biology) guidelines  which consider CEUS comparable to MDCT as previously discussed studies have shown [30]. For tumors between 1 and 2 cm, there are various approaches ranging from noninvasive use of one or two imaging techniques in conjunction with invasive liver biopsy, which remains the option of choice in case of uncertainty. Lesions under 1 cm remain under periodic surveillance (every 6 months) until changes in size are observed, although the Korean Liver Cancer Study Group guidelines currently consider that combining MDCT with Gadoxetic Acid Enhanced MRI is successful in detecting even infracentimetric HCCs [31].Current diagnostic guidelines of the American Association for the Study of Liver Diseases (AASLD) refer to liver biopsy only after other less invasive methods have been exhausted. According to a 2010 update, these methods now solely include imaging methods, as the use of AFP as a diagnostic criterion has been abandoned due to its reduced specificity and no other serum markers have yet been proven a viable replacement. There is a consensus that for hepatic tumors larger than 2 cm, one contrast enhanced imaging diagnostic tool, either MDCT or Dynamic Contrast Enhanced MRI, presenting a typical vascularization pattern is sufficient for diagnosis. The AASLD and European Association for the Study of the Liver guidelines have excluded CEUS from this list due to false positive results in cases with cholangiocarcinoma [32].As previously stated, fine needle aspiration (FNA) liver biopsy is reserved only for atypical HCC due to the inherent risks involved in any invasive procedure such as intraperitoneal or needle tract-seeding and possible bleeding. Percutaneous FNA accuracy has been reported to be up to 100%, granted that the biopsy has been taken from the suspected tissue and not from normal liver parenchyma. These errors can be avoided by performing a CT or ultrasound guided FNA. The development of endoscopic ultrasound guided FNA has largely increased the area of the liver available for biopsy and has offered a fast and relatively safe method for the histopathological diagnosis of HCC [Prognostic Markers33]. A recent study that proposed the associating AFP with human cervical cancer proto-oncogene 1 (HCCR-1) determined that the latter is actually more accurate to some extent and that a large number of subjects were positive for at least one of the two markers, which lead to a significant increase in early stage HCC diagnosis [34]. Furthermore, another diagnostic option for patients with AFP < 20ng/ ml is the alpha-fetoprotein isoform 3 (AFP-L3) fraction, which presents a higher specificity (80%-97%) and has proven to be useful in clinical practice [35]. Regarding the prognostic value of serum markers, Survivin, an apoptosis inhibitor and a promising therapeutic target, although significantly correlated with the clinical stage, portal thrombi and lymph node metastasis, has not proven to be more reliable than AFP [36].Among the earliest discovered oncofetal proteins, AFP is currently one of the most frequently used serum tests for early stage HCC and, although groundbreaking biomarkers are being discovered, to this date, it still plays an important role in terms of HCC detection. The introduction of new serum markers in order to improve or replace AFP is encouraged by its poor specificity and sensibility coupled with the fact that recent studies have yet to provide sufficient unbiased data necessary for establishing it alone as an efficient surveillance, diagnostic and prognostic tool [37], des-gamma-carboxy-prothrombin  has been suggested as a measurement for cancer progression. Tumor size, as well as vascular invasiveness and metastasis frequency, have been reported to correlate with serum DCP levels [38]. Moreover, increased DCP values are an indicator for the reoccurrence and low survival probability. The applicability of this marker is limited by the fact that it has a low positivity rate for small HCC with a current sensitivity of around 50%, despite the introduction of more accurate detection techniques [39]. A case-control study conducted on 1031 patients concluded that the sensitivity of DCP and AFP increases with HCC progression and proposed further studies regarding the use of a combination of the two markers in early diagnosis and surveillance of HCC’s response to treatment [40].Despite its modest sensitivity in preclinical tumor detection at its current cut-off values . A novel study has indicated that the use of GP73 in conjunction with gamma-glutamyl transpeptidase II (GGT-II) alone did not lead to any conclusive results; however, a triple marker assay including AFP yielded promising results [43]. A 2007 study adopted a new approach by establishing a prognostic correlation between HCC tumor stage and aggressiveness and interleukin 18 (IL-18), which is speculated to be part of an antitumor response by adjacent healthy hepatocytes [44]. According to another recent study, one option would be an extensive serum and urine metabolite profiling technique, which would take into consideration various alterations of metabolic pathways determined by HCC evolution, which unfortunately may also occur in hepatitis and cirrhosis. Although a promising screening tool, this approach, as well as most of the before mentioned markers, requires further research and refining in order to be useful in clinical settings [45].Numerous other biomarkers have been also studied and compared to AFP. One of these is Golgi protein 73 (GP73), a transmembrane protein, which has yielded contradictory data in several studies and thus research is required into whether it is more accurate than the traditional serum markers [In conclusion, the multitude of current imaging techniques available for HCC assessment allows a higher rate of detection and significantly contributes to a more accurate long-term prognosis. Contrast-enhanced ultrasonography has proven to be a cheaper, faster, simpler, non-invasive and safer method for the patient, which offers high accuracy for the characterization of focal hepatic lesions. In order to obtain a precise characterization of HCC, MRI and CT capabilities are continuously evolving and offering better results from both a morphological point of view through DW-MRI and MDCT, as well as from a functional perspective through PET-CT.Nevertheless, there is hope that future constant research for a rapid and specific screening serum marker would lead to an early detection of small liver tumors and thus to a better chance of a successful treatment.AcknowledgmentsThis paper was published under the frame of European Social Fund, Human Resources Development Operational Program 2007-2013, project no. POSDRU/159/1.5/136893 and UEFISCDI Partnership project VIP SYSTEM, ID: 2011–3,2-0503.


Signs and symptoms of a rapidly enlarging breast mass are not only important for all clinicians to recognize and assess, but also are not uncommon occurrences. We describe a similar but unique case that developed into an enormous, 36 cm exophytic mass.A 51-year-old woman with history of psychiatric conditions presented for signs and symptoms of sepsis. It was determined that the source was an enormous 36 cm mass originating from the breast/chest wall. After stabilizing the patient with antibiotics, she underwent successful resection. Surgical margins were positive, and histopathology demonstrated bland spindle cells with stromal overgrowth. Together with clinical and histopathological information, the patient was diagnosed with a phyllodes tumor.Differential diagnosis of rapidly growing breast masses is discussed, which are not uncommon occurrences in clinical medicine. One etiology, phyllodes tumors, can grow into large, exophytic masses as described. Oncologic treatment is discussed, usually consisting of surgery with postoperative radiotherapy for high-risk features. The differential diagnosis of a rapidly growing breast mass is very important for not only oncologists, but any health care provider, owing to the relative ubiquity of the symptoms and need for further workup and treatment. In this report, we describe the exceptional case of a woman who noticed a rapidly growing breast mass that became extremely large. We discuss the difficulties of diagnosis as well as differential diagnoses of which clinicians should be aware.A 51-year-old woman with history of multiple psychiatric conditions including uncontrolled anxiety and depression presented to the intensive care unit with tachycardia and hypotension. She had initially felt a left breast mass five years ago but not sought medical attention, and the mass continued growing. Over the past sixteen weeks, the mass had nearly tripled in size and started oozing purulent fluid. Visual inspection revealed a 36 cm mass composed of several different colored, shaped, and textured tissues. Fluid drainage and necrotic debris was present Fig.  without After stability in clinical status was achieved, due to great concern of ongoing sepsis from the necrotic/infected tumor, emergent resection was done. Thus, pre-surgical biopsy could not be performed, although differential diagnoses included abscess/necrosis, sarcoma, phyllodes tumor, and fibroadenoma. Radical mastectomy was performed owing to intraoperative tumor involvement of the pectoralis and intercostal muscles. Owing to the emergent circumstances, large tumor size with necrosis, and no prior tissue diagnosis, nodal sampling was performed; lymph nodes were grossly nonenlarged. Owing to no clinical nodal disease as well as clinical suspicion for the aforementioned diagnoses, there was no indication for complete axillary dissection. Postoperatively a clean base was present Fig.  with skiPathological diagnosis of the mass was difficult. There was significant (over 50 %) necrosis with bland spindle cells being the only detectable cells Fig. . There wWhereas the pathological diagnosis was initially an unspecified spindle cell neoplasm, adding together the clinical history and presentation, the patient was diagnosed with a phyllodes tumor.th decade of life and vastly more commonly in females [Phyllodes tumors most often arise in patients in the 5 females . They cl females . Spindle females . Phyllod females . Much of females , this tuThis patient was treated with postoperative radiotherapy, in part due to positive surgical margins, malignant phenotype, and stromal overgrowth. A large retrospective study of 478 patients with malignant phyllodes tumors demonstrated that tumors over 10 cm have recurrence rates of 15 % after mastectomy and worse survival, which was a primary consideration in proceeding with radiotherapy  and distThese are in contrast to the NCCN guidelines , which dThis patient completed radiotherapy without wound healing complications but owing to psychiatric conditions and past issues of self-neglect, has failed to follow up.Written consent to participate and publish this case was obtained from the patient and is available on request. This case report was not required to be reviewed by the Institutional Review Board committee at the University of Nebraska Medical Center.


Tumefactive demyelinating lesions are a rare manifestation of multiple sclerosis (MS). Differential diagnosis of such space occupying lesions may not be straightforward and sometimes necessitate brain biopsy. Impaired cognition is the second most common clinical manifestation of tumefactive MS; however complex cognitive syndromes are unusual.We report the case of a 30 year old woman who presented with Gerstmann’s syndrome. MRI revealed a large heterogeneous contrast enhancing lesion in the left cerebral hemisphere. Intravenous corticosteroids did not stop disease progression. A tumour or cerebral lymphoma was suspected, however brain biopsy confirmed inflammatory demyelination. Following diagnosis of tumefactive MS treatment with natalizumab effectively suppressed disease activity.The case highlights the need for clinicians, radiologists and surgeons to appreciate the heterogeneous presentation of tumefactive MS. Early brain biopsy facilitates rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS. Gerstmann’s syndrome is a rare disorder resulting from damage to the angular gyrus of the dominant parietal lobe leading to dysgraphia, dyscalculia, finger agnosia and left-right disorientation . In adulA 30 year old, right-handed woman was admitted with a two week history of word finding difficulties. There was subtle expressive and receptive dysphasia alongside acalculia, agraphia, finger agnosia and left-right disorientation  of the head demonstrated a large heterogeneous lesion associated with focal cystic changes in the left occipito-temporal lobes with partial restriction on diffusion weighted MRI (DWI) and heterogeneous gadolinium enhancement Figure A-G. No sShe was treated with intravenous (IV) methylprednisolone (MP), 1 g daily for three consecutive days with no clinical effect. Two weeks later the patient developed severe headache with intractable vomiting. Repeat MRI head revealed increase in size of the previously detected lesion and three new lesions  maps . Howeverancement . ClinicaThe findings on proton MRS of an NAA/Cr ratio well below normal may be indicative of axonal dysfunction or loss whilst the significantly increased Cho/Cr ratio suggested increased cell-membrane metabolism. However, these findings, as well as the ‘inverted doublet’ shape of the lactate curve may occur in tumefactive demyelinating lesions and tumours alike . Thus, lThe evidence regarding prognosis and disease course of tumefactive MS remains controversial. Lesions larger than 5cm have been reported as being associated with a higher disability at follow-up . On the et al. proposed an algorithm for the acute management using IVMP and/or PLEX followed by disease modification using immunomodulatory agents [There is no standard immunomodulatory treatment for people with tumefactive MS. Treatment choices include IVMP, β-Interferons, plasma exchange (PLEX), rituximab and natalizumab ,11-14. Iy agents . The disy agents . Howevery agents  and IFN y agents , respecty agents .In summary, we present video, MRI and pathological evidence of a case of tumefactive MS which posed a diagnostic dilemma due to its atypical clinical and radiological presentation, and lack of response to steroid treatment. Clinicians, radiologists and surgeons need to appreciate the heterogeneous presentation of this disorder, and an early brain biopsy is often necessary for rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS.We confirm that a signed consent from the patient has been obtained for the publication of this report, the images and videos.KS is a PI on trials sponsored by Novartis and Roche. He has received speaking honoraria from Merck-Serono and Novartis, served on advisory boards for Biogen, Merck Inc, Merck-Serono, Novartis and Teva, and has received grant support from Novartis.SG wrote the draft manuscript and prepared the videos. ZJ provided the histological analysis, prepared pathology images and was involved in drafting of the manuscript. KPB and SaG prepared MR images and were involved in drafting the manuscript. KS initiated the study, reviewed and edited the manuscript, and approved the final version. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/14/68/prepubCognitive assessment of the patient before treatment.Click here for fileCognitive assessment of the patient after three months treatment. Dysphasia, dysgraphia, finger agnosia and left-right disorientation are shown (video 1), which have subsided on subsequent assessment (video 2).Click here for file