Introduction: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unknown etiology, which usually develops in the mediastinum. It can also occur in the cervical, retroperitoneal and axillary regions. Localized pancreatic CD is quite rare .Presentation of case: The authors herein present a case of a 34 years old female that was diagnosed during a symptomatic cholelithiasis evaluation. During the evaluation, an abdominal ultrasonography revealed a tumor at the head of the pancreas, which went on to generate a dilatation of the extrahepatic bile ducts. This finding was confirmed by abdominal magnetic resonance imaging (MRI). Subsequently, the patient underwent a laparotomy, where a capsulated tumor was found at the head of the pancreas with well-defined margins. The decision was made for tumor excision. The histopathology and immunohistochemistry established CD, hyaline vascular variation.Discussion: The authors of the present paper also performed a literature review concerning Pancreatic CD, where there were found only 33 cases until the time of the writing of this paper, and we have subsequently carried out a retrospective analysis of all cases.In a patient with atypical images, there might be a benefit from a preoperative diagnosis of CD, by using immunohistochemistry analysis in an image guided biopsy. Thus, avoiding unnecessary procedures and surgeries.Conclusion: Localized pancreatic CD is a very rare condition with good prognosis, but it can mimic many common diseases, such as gastrointestinal stromal tumor (GIST), pancreatic neuroendocrine tumor or pancreatic adenocarcinoma. InitialHowever, further case reports have gone on to describe a multicentric form of CD that often manifests a more malignant clinical course .There has been no evidence found of any age predominance, as the condition affects children as well as adults, and neither any significant sex predilection has been found . HoweverPancreatic localization of CD is very uncommon, with only a few reports in the literature. A list of the most common diagnoses of mass in the pancreas include adenocarcinoma, cystic tumors, and functioning or non-functioning neuroendocrine tumors.Our objective is to present another case of pancreatic CD that mimics a pancreatic malignant neoplasm and to make a review of the topic with a retrospective analysis of all 33 cases published until now, to the best of our knowledge. This work was reported in line with the SCARE criteria .2A 34 years old female presented herself to our service with a 3-year intermittent abdominal pain, associated with postprandial nausea and vomit. There was no history of fever, night sweats, decreased appetite, weight loss or bowel habit alterations. Family history was non-contributory. The physical examination was unremarkable.Due to the possibility of cholelithiasis, an abdominal ultrasonography (US) was ordered. Abdominal US revealed a retroperitoneal tumor at the head of pancreas, biliary tract dilatation and cholelithiasis.An abdominal magnetic resonance was ordered and showed an isointense smoothly marginated 4 cm mass in T1  and a siBased on the unspecific radiological findings and epidemiology, a provisional diagnosis of pancreatic adenocarcinoma, gastrointestinal stromal tumor (GIST) or pancreatic neuroendocrine tumor was made.Adenocarcinoma was not the likely cause, as no consumptive symptoms were reported, and usually a pancreatic adenocarcinoma in T1 window with contrast as a hypointense mass rather than a normal pancreatic parenchyma.Neither GIST showed signs of a strong diagnosis. Since it would appear as low signal intensity solid component in T1 and high signal intensity solid component in T2.Pancreatic neuroendocrine tumor is usually hypointense relative to the pancreas in T1 and hyperintense relative to the pancreas in T2, but there is a range of signal intensities. Therefore, a pancreatic neuroendocrine tumor became our principal hypothesis.The fact that the patient had a head pancreatic tumor with bile duct dilatation, associated with symptomatic cholelithiasis led to our preference for a surgical resection. Thus, a pancreatoduodenectomy was performed.On exploration, we found an encapsulated mass bulging in the retroperitoneum at the pancreatic head . There wBased on the macroscopic aspect of the tumor, we opted for excision. The mass was completely excised, while preserving its capsule , a featuAn anatomopathological examination showed chronic inflammation in the gallbladder and lymphoid proliferation in the excised tumor. Without immunohistochemistry, the lymphoid proliferation main possible diagnosis was a low grade B cells lymphoma or CD.Immunohistochemistry demonstrated a prominent vascular proliferation and hyalinization of the vessel walls with an onion skin appearance and normal B and T lymphocytes distribution. CD20, CD3, CD10 and KI 67 were positive. BCL1 and BCL2 were negative . Thus, wThe patient was seen on follow-up one month after the surgery. At that time, the patient was asymptomatic. The patient remained asymptomatic during all subsequent follow-ups.3Little is known about the cause of this disorder of unknown origin . The etiSeventy percent of cases present in the mediastinum and 20% occur in the axillary, cervical, inguinal and vulvar regions, while approximately 12% occur in the abdomen; mostly located in the pelvis, mesentery and perinephric regions . ExtrathIn the beginning, CD was classified histologically as two histopathological variants: hyaline vascular, , and plasma cell, (which is characterized by large follicles with intervening sheets of plasma cells) .A mixed type of plasma cell and hyaline vascular type was further reported , howeverClinically it can be classified as two types: localized and multicentric. The localized form is defined as a single, benign lesion, usually affecting young people .Patients with multicentric disease, either hyaline‐vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy .We only found one case of elevation of tumor markers in pancreatic CD. These results changed to normal 7 days after a pancreatic head mass excision. A possible explanation given by the author for the rise and fall of CA 19.9 could be intermittent compression of pancreatic ducts by the mass . HoweverThe current case to the best of our knowledge is the only case that demonstrates a biliary duct dilatation in a unicentric CD, documented by an MRI in a hyaline vascularh type, and no signs of tumor marker elevation either.Imaging techniques like ultrasonography, computed tomography (CT) and MRI have been proven to be helpful in diagnosing retroperitoneal tumors. However, they show conflicts in their findings concerning CD, this incongruence is probably due to multiples histological types.Therefore, imaging procedures should be considered for the differential diagnosis of a pancreatic mass . HoweverThe CD histological diagnostic is based on cell architecture, and therefore requires the study of the entire surgical specimen .The adhesion of the tumor to the surrounding tissue and hypervascularity in the mass are characteristic features of the hyaline vascular type ,20.Immunohistochemical stains for ĸ and λ chains, L26, and UCHL-l . Are curMultiple authors all have failed to establish CD as a diagnosis by endosonography controlled fine-needle aspiration biopsy (EUS-FNA) . In noneHowever, one case was successfully reported as a pancreatic CD preoperative diagnosis by EUS-FNA. The authors used flow cytometric analysis and a determined diagnosis. They claimed that occasional morphologic features on cytologic smears and on cell block section made the preoperative diagnoses possible .The collaborators in Rhee et al in 2008 used an endoscopic ultrasonography guided trucut biopsy, but they could not establish a CD diagnosis. They were unable to make a differentiation from a low grade B-cell lymphoma , based oAlthough there are no randomized studies, most published series agree that surgical complete resection is the best therapeutic option for the localized, unicentric form of CD including the plasma cell variant and mixed, with favorable long-term prognosis reports and no cases of malignant transformation .Table 1XOnly one case of death during treatment and follow-up was reported in all localized pancreatic CD cases. However, this was attributed to comorbid, once the diagnoses were made in intraoperative in light of a different pathology, and the elderly patient died due to postoperative complications .4CD affecting the pancreas is a very rare occurrence, even nowadays with a great complementary arsenal for performing a diagnosis. Therefore, this is one of the reasons that CD is not usually included in the list of possible diagnosis. However, pancreatic CD should be taken into consideration in the differential diagnosis of a pancreatic mass.These patients have a good prognosis in a majority of cases unlike malignant tumors. Accordingly, the possibility of a preoperative diagnosis in a patient with atypical findings in MRI or CT by image-guided biopsy associated with immunohistochemistry analysis would improve outcomes by avoiding useless tests, possible neoadjuvant chemotherapy, and finally would reduce surgical procedure morbidity. However, the definitive diagnosis will be performed based on the postoperative pathological findings.None.Authors did not receive any funding for this work.We do not require ethical approval to write a case report paper.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.1Edson Gonçalves Ferreira Junior: Conceptualization, Methodology, Resources, Writing the paper, Writing – Review & Editing, Project Administration, Final approval2Philippos Apolinario Costa: Conceptualization, Methodology, Data collection, Data analysis/interpretation, Writing – Review & Editing, Final approval3Larissa de Melo Freire Gouveia Silveira: Conceptualization, Methodology, Data collection, Resources, Writing – Review & Editing, Final approval4Rafael Valois Vieira: Conceptualization, Methodology, Investigation, Writing – Review & Editing, Data collection, Resources, Final approval5Hugo Alessi L M Soares: Conceptualization, Methodology, Data collection, Writing – Review & Editing, Supervision, Final approval6Bruna Menon Loureiro: Conceptualization, Methodology, Data collection, Resources, Writing – Review & Editing, Final approval7Nayane Carolina Pertile Salvioni: Conceptualization, Methodology, Data collection, Resources, Writing – Review & Editing, Final approval8Jose Roberto Coelho Ferreira Rocha: Conceptualization, Methodology, Data collection, Resources, Writing – Review & Editing, Final approvalCase reports don’t need to be registered.Edson Gonçalves Ferreira Junior.Not commissioned, externally peer reviewed.


The authors report a case of a 69-year-old female with difficulty swallowing and neck swelling and review the clinical presentation, radiographic features and treatment of adult epiglottitis. Epiglottitis remains a medical emergency, with the potential for airway compromise. The authors present this case because it is a potentially life-threatening infection that warrants prompt diagnosis and management. This case is worth reporting because epiglottis in adults can have a milder and less classic presentation that sometimes results in delayed recognition. Acute epiglottitis, also known as supraglottitis, is an invasive cellulitis involving the epiglottis and the adjacent structures including the aryepiglottic folds and vallecula. Prior to widespread vaccination against haemophilus influenzae in the 1990s, it was primarily a bacterial illness seen in young children, with a peak age of three years . EpiglotThe definitive diagnosis of epiglottitis is made by visualization of an edematous, cherry red epiglottis by direct or indirect laryngoscopy or fiberoptic endoscopy . LateralAcute epiglottitis has the potential for causing abrupt, complete airway obstruction and is thus a medical emergency that requires prompt diagnosis and careful management of the airway. While less common in adults, it has more potential for poor outcome due to the often indolent nature of the clinical course. The authors present a case of acute epiglottitis in an adult.A 69-year-old female presented to our emergency department with chief complaint of neck swelling, sore throat and difficulty swallowing for the past two days. She also complained of fever and voice hoarseness. Flexion and extension of the neck made the pain worse. She denied difficulty breathing, nausea, vomiting, or chest pain. She reported a non-productive cough. She stated that her daughter and grandchildren were sick with a cough and cold. She took diphenhydramine, acetaminophen and some old amoxicillin at home. Her past medical history was significant for salivary gland cancer nine years prior that was treated with chemotherapy and radiation.3, and an elevated procalcitonin level of 23.67 ng/mL. Radiograph of the soft tissues of the neck revealed an enlarged epiglottis and aryepiglottic folds, consistent with epiglottitis. In addition, subglottic narrowing of the airway is demonstrated , possibly angioneurotic edema, retropharyngeal or peritonsillar abscesses, uvulitis, and diphtheria.Complications include epiglottic abscess and systemic bacteremia.In this case report the authors present a case of epiglottitis. If left untreated, epiglottitis can progress to life-threatening airway obstruction, and thus remains a diagnosis not to be missed.


Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and decreased libido. Unlike that of common pituitary adenoma, the association between pituicytoma and Cushing disease (CD) is extremely rare. There were only 6 reported cases till now. In the current study, we describe an unusual case of pituicytoma associated with severe CD with a recurrence-free follow-up period of 49 months.A 32-year-old woman was referred to our hospital with moon face, central obesity, and purple stripes on the lower limbs.The plasma cortisol level was 1122 nmol/L. The low-dose dexamethasone suppression test failed to suppress plasma cortisol. This test provided evidence of nonpituitary-dependent CD. However, magnetic resonance imaging demonstrated a sellar mass measuring approximately 7.6 × 5.7 mm. The patient was diagnosed with pituitary microadenoma. Histopathological analysis of the tissue sections based on the findings from the immunohistochemical staining diagnosed it as pituicytoma.Transsphenoidal surgery was performed to remove the pituitary mass.Within 2 months postoperatively, the patient's blood pressure and cortisol level decreased gradually and normalized on the 6th month when other symptoms of CD also disappeared. The patient is presently free from recurrence 49 months after the initial diagnosis.Based on the postoperative remission, CD was caused by pituitary disorders. A reasonable assumption is that an extremely small coexisting adenoma was not detected by the surgeon and washed out during the dissection. Another possible explanation might be the mass effect caused by this intrasellar lesion. The tumor mass is generally solid, well-circumscribed, and noninfiltrative. According to the location and tumor size, patients with pituicytoma usually exhibit clinical symptoms such as headache, visual disturbance, hypopituitarism, and decreased libido. To date, based on previous studies, the tumor develops mainly in adults rarely in children.–8 Radiological findings are nonspecific for pituicytoma; therefore, the most common radiological preoperative diagnosis was pituitary adenoma.,10 Currently, with the help of immunohistochemistry (IHC), histological study is still the most accurate examination for this tumor. The best choice of therapy is total resection.Pituicytoma, was previously known as “infundibuloma” or “posterior pituitary astrocytoma.”,6,11–14 In the current study, we report a 32-year-old woman with pituicytoma iassociated with severe CD.Unlike that of pituitary adenoma, the most common tumor of the sellar, and suprasellar regions, the association between pituicytoma and Cushing disease (CD) is extremely rare. There were only 6 reported cases were reported till now: 4 cases in female adults, 1 in male adult, and 1 in a 7-year-old girl.2A 32-year-old woman was referred to our hospital with chief complaint of back pain and kyphosis for 3 years. Three years ago, the patient was diagnosed with “osteoporosis and thoracic compression fracture” at a local hospital. After surgical treatment, the patient did not use regular medication. Last year, the patient experienced fatigue and severe back pain with lower extremity pain after lifting heavy objects, which was not relieved by rest but gradually increased. Upon examination in our clinic, moon face, central obesity, and purple stripes on the lower limbs were noted. The patient was 162 cm in height and weighed 70 kg, with a body mass index of 26.6. The resting blood pressure was 160/100 mm Hg. The patient also had type II diabetes. Laboratory tests showed that lutenizing hormone dramatically decreased to 0.17 mIU/mL; follicle-stimulating hormone level was 2.25 mIU/mL which was within the normal range; aldosterone level was 0.2 ng/mL, plasma renin activity was 4.4 ng/mL, and angiotensin II level was 42.55 pg/mL. Plasma adrenocorticotropic hormone level was within normal range, and plasma cortisol level was 1122 nmol/L . Only slight suppression of plasma cortisol was found during an overnight 8 mg dexamethasone suppression test, from 1291 to 1150 nmol/L. Low-dose dexamethasone suppression test failed to suppress plasma cortisol. This test provided evidence of nonpituitary-dependent CD. However, magnetic resonance imaging demonstrated a sellar mass measuring approximately 7.6 × 5.7 mm , pituicytoma is a benign spindle cell tumor that originates from pituicytes.,17,18 Pituitary-dependent CD was predominantly caused by pituitary adenoma. Cases of patients with pituicytoma associated with pituitary hyperfunction were reported but significantly limited, and those associated with CD were extremely rare. To date, there were only 7 reported cases, including this case, which was associated with CD or Cushingoid features, and one of these cases was reported in a 7-year-old girl. The details of all cases are summarized in Table  Among the other 5 cases with CD, 2 cases were histologically proven to be coexisting with corticotroph hyperplasia or corticotropin-secreting adenoma.,14 In 3 cases, no other central nervous lesions were evident besides pituicytoma,,12,13 similar to the current case. Then, what would be the cause of remission of CD in this patient? A reasonable assumption is that an extremely small coexisting adenoma was not detected by surgeon and washed out during dissection. Since pituicytomas are commonly quite hemorrhagic during surgery, more precise surgeries and findings are critical to explore small tumor tissues. Another possible explanation might be the mass effect caused by this intrasellar lesion since the compression of the anterior pituitary can also lead to hyperfunctional endocrinological disturbance, including hyperprolactinemia or hypercortisolism, which is similar to that observed in patients with intrasellar craniopharyngioma. In all cases in adults, including the current case, CD was in remission postoperatively, and no recurrence was reported. However, there was no postoperative remission in the case of the 7-year-old girl, and bilateral laparoscopic adrenalectomy was further performed.In most cases, the mass effect of pituicytoma mainly causes visual disturbance, headache, and pituitary insufficiency. In the current case, the controversial preoperative test results led to a dilemma for both the surgeon and patient. Based on postoperative remission, CD was caused by pituitary disorders. With the aid of IHC, the tissues sections of pituicytoma can provide the most important evidence in the differential diagnosis of more common pituitary adenoma or other rare pituitary tumors.,19 The tumor consisted of spindle-shaped cells arranged in a sheet, storiform patterns, or interlacing fascicles without Rosenthal fibers, eosinophilic granular bodies, or Herring bodies. Nuclear atypia was typically evident. The tumor cells are diffusively positive for vimentin and S-100, focally positive for glial fibrillary acidic protein, and generally negative for epithelial membrane antigen, synaptophysin, chromogranin A, and neurofilament. Unlike pituitary adenomas, pituicytoma tumor cells are negative for pituitary hormones. CD68 and PAS staining were generally negative or variably positive in pituicytoma but positive in granular cell tumor. Another rare pituitary tumor needs to be considered in the differential diagnosis. The Ki-67 index in pituicytoma is usually <2% to 3%. Several studies revealed that pituicytoma is diffusively positive for thyroid transcription factor-1 in the cell nucleus, and this also provides an important clue in differentiating it from pituitary adenoma.In pituicytoma, the radiological studies cannot provide accurate diagnosis in preoperative tests, especially when laboratory test results favor the diagnosis of pituitary adenoma.Therefore, this report of an extremely rare case demonstrates pituitcytoma in a 32-year-old woman presenting with severe CD. The microscopic features and IHC results support the diagnosis of pituicytoma.www.editage.cn) for English language editing.The authors thank Editage (Methodology: Jinping Wang.Writing – original draft: Xiaoman Li, Liang Wang.Writing – review & editing: Yang Liu, Yuan Miao, En-Hua Wang.Liang Wang orcid: 0000-0003-4015-9385.


Situs inversus totalis is a relatively rare congenital anomaly. Performing the retrolaparoscopic adrenalectomy for the patient with situs inversus totalis is a skill-demanding and challenging surgical task, which has been even more rarely reported.We present a case with a large right adrenal mass (10.2 × 9.4 × 7.9 cm) complicated by situs inversus totalis. This 59-year-old female patient underwent the retrolaparoscopic adrenalectomy in our department. In order to facilitate the surgical orientation and improve the manipulating accuracy, the data from computed tomography images was extracted and the three-dimensional digital model was reconstructed. Under the assistance of preoperative planning and intraoperative navigation by the three-dimensional digital model, the retrolaparoscopic adrenalectomy was technically precise and successful. The targeted adrenal tumor was excised completely with final pathological diagnosis of adrenocortical adenoma.Retrolaparoscopic adrenalectomy can be performed safely in patients with situs inversus totalis. The assistance of preoperative planning and intraoperative navigation by the reconstructed three-dimensional digital model can facilitate the operation and lead to more precise vessel manipulation and accurate excision of tumor that is both effective and safe.The online version of this article (10.1186/s12957-018-1473-8) contains supplementary material, which is available to authorized users. Situs inversus totalis (SIT) is a relatively rare congenital anomaly characterized by the complete mirror-imaged visceral organs in the opposite anatomic positions , and itsA 59-year-old female patient was admitted into our hospital for the abnormal computed tomography (CT) image presentation of right adrenal mass revealed by occasional health examination, accompanied with the complaint of intermittent nausea and blurred vision. She had been diagnosed with SIT when she received the hysterectomy and near-total thyroidectomy for the uterus myoma and thyroid cancer in 2000. Besides, her past medical history also included the diabetes mellitus (type 2) for 6 years and significant hypertension for 5 years, ranging from 150 to 200/90 to 102 mmHg, fluctuated periodically.2, which was well correlated with her significant abdominal obesity. The laboratory tests presented the normal levels of the serum potassium concentration (4.2 mEq/L) and the renin activity (3.8 ng/mL/h). The plasma cortisol and aldosterone (217 pg/mL) concentrations were also within the reference range. Besides, there were no abnormalities found in the levels of the urinary catecholamines (59.2 mcg/24 h) and vanillylmandelic (4.3 mg/24 h) either.The patient’s height was 158.0 cm and the body weight was 70.0 kg. The BMI was 28.04 kg/mAs for the medical image examinations, the routine chest X-ray image revealed the dextrocardia; the dual-source 64-slice enhanced CT  scan including arterial phase, venous phase, and excretory phase after intravenous contrast administration confirmed the diagnosis of SIT and presented her round-shaped mass on the right adrenal gland with the size of 10.2 × 9.4 × 7.9 cm  was reconstructed: The original image data from CT scanning was set in the format of Digital Imaging and Communications in Medicine (DICOM). The copied information was analyzed and reconstructed into the 3D-DM by using a postprocessing software named three-dimensional medical image reconstructing and guiding system , which is a multifunctional workstation for the clinical application, whose functions included CT-based image reconstruction, preoperative planning, and intraoperative-assisted navigation . The retThe morphometric calculation and analysis of the reconstructed 3D-DM provided the surgeons with the valuable anatomic information such as the spatial locations of vital vessels, maximal diameter, and margins of the adrenal mass. The tumor can be shadowed and turned into a transparency, leaving the hollow-shaped crater. A more distinct spatial relationship between the adrenal mass and the nearby structures showed clearly via this intraparenchymal visualization. Based on these data, we tailored a specific surgical plan for this rare case. The surgeons can gain a full comprehension of the regional complexity which is mirrored to the normal anatomy.The retrolaparoscopic adrenalectomy was performed at the Department of Urology. The patient was placed on the operating table in the lateral decubitus position with the affected side upward. The general anesthesia and tracheal intubation were administrated. After padding the pressure points with beanbags and fixing the posture with the optimal table flexion, a longitudinal 1.5 cm incision for 12 mm trocar was made in the posterior axillary line below the 12th rib. In order to minimize the potential irritation to the stability of blood pressure, the retroperitoneal space was dilated in the blunt finger-dissecting style instead of the traditional ballooning way. Another two 12 mm trocars were located at the point of 2 cm above from iliac crest superior border in the mid-axillary line for the laparoscope, and at the point under the subcostal margin in the anterior axillary line for laparoscopic instruments respectively. After the routine insertion of three trocars and the establishment of pneumoperitoneum at a pressure of 10 mmHg, the retroperitoneal fat tissue was removed under the laparoscopic surveillance. Another assisted 5 mm trocar was inserted due to the patient’s obesity. A well-trained full-time surgical technician captured some typical screenshots to illustrate the anatomical landmarks. Then, the semitransparent 3D-DM were superimposed onto these screenshots with appropriate axis and size adjustments. The composite 3D-DM images provided the surgeon with information relative to the inverted anatomy, thereby acting as a kind of assisted navigation for the subsequent manipulations. All these speedy manual image fusions performed synchronously during operation and all the fused images were displayed on a separate screen. Under the assisted navigation of 3D-DM, the Gerota fascia was incised and the adrenal central vein was dissected carefully, following its ligation by using 5 mm Hem-o-lok clips. After that, the mass was excised completely with the careful preservation of normal adrenal tissue and the other adrenal vessels including the adrenal arteries were ligated and scissored up. Then, the retroperitoneal pressure was set down to the level of 5 mmHg and the hemostasis was achieved carefully. Given the size of the tumor mass and the skin elasticity, we extended the incision below the 12th rib along its axis for the total length about 7 cm. The specimen of adrenal tumor was packed into a homemade laparoscopic pouch with a string by graspers and then was withdrawn by holding and pulling the string vigorously. The trocar incisions were carefully closed, and a rubber drainage catheter was left in situ.The operative time was 1 h and 10 min, with no intraoperative complications happened. The estimated blood loss was about 10 mL. The size of resected tumor was 10.0 × 9.1 × 6.8 cm, and no gross extracapsular invasion was found. The final pathological diagnosis was adrenocortical adenoma Fig. . The posAfter more than two-decade global verification via massive clinical practice, laparoscopic adrenalectomy has been viewed as the gold standard  of surgiTo the dextromanual surgeons, the mirror-imaged anatomy of SIT patient always indicates the technical challenges in endoscopic orientation and maneuvers. The inefficient familiarity to the abnormal disposition of organs and the consequent reduced self-confidence in the surgical manipulations will lead to more cautious and repeated spatial confirmations, which can result in the prolonged operative course inevitably , 13. BesTo ensure a safe and smooth surgical procedure, accurate preoperative anatomic comprehension and adequate preoperative planning work are useful and pivotal. In this context, our surgeon team held a routine session to formulate a suitable surgical blueprint for this case based on the information gained from the detailed morphometry of the reconstructed 3D-DM preoperatively. The surgical plan consists of four parts: the planning of acquainting with the mirror-imaged retroperitoneal space, the planning of confirming and dissecting the central vein of the adrenal gland, the planning of accurate adrenal mass excision with maximal preservation of the normal adrenal tissue, and the planning of avoiding damage to the inverted proximal anatomical structures. The most accurate information about the mirror-imaged regional anatomy featuring the valuable warning landmarks where potential surgical injury may occur will be noticed and emphasized. It has been proven to be helpful and could achieve a more ample preparation by walking through the surgical procedure in this style prior to the operation.Compared with the conventional CT scanned images, 3D-DM helped the surgeon to generate an improved and more detailed surgical blueprint in his mind, which undoubtedly increased his comprehension and confidence on the upcoming operation and eventually resulted in the reducing of the tentative maneuvers in the vessel-controlling and tissue-dissecting procedures. Moreover, when it came to many surgical decision-making occasions in the operation, the rapid feedback according to the information provided by 3D-DM shortened the evaluating time efficiently on the premise of safety.Nevertheless, we still recommend that SIT-involved retrolaparoscopic adrenalectomy should be performed by the experienced laparoscopic surgeon, as the intraoperative fast and suitable solution to the SIT-initiated spatial orientation problems still mostly rely on the professional attainment and surgical skills of the surgeon.In our future clinical practice, we believe the robotic retrolaparoscopic adrenalectomy might be helpful to these rare cases. The centered robotic view of the surgical field can eliminate the difficulty of changing instruments in both hands .Retrolaparoscopic adrenalectomy can be performed safely in patients with SIT. The assistance of preoperative planning and intraoperative navigation by the reconstructed 3D-DM can facilitate the operation and lead to more precise vessel manipulation and accurate excision of tumor that is both effective and safe.Additional file 1:The 3D-DM guided preoperative planning and the intraoperative video. (WMV 57228 kb)


Radical surgical resection and oncologic follow-up is warranted.•When compared to ductal adenocarcinoma, patients with ITPN reveal less lymph node metastasis and a better overall survival. Intraductal tubular papillary neoplasm (ITPN) displays a very rare subtype of epithelial neoplasms of the pancreas. ITPN is characterized by intraductal tubulopapillary growth and cellular dysplasia. In contrast to intraductal papillary neoplasm (IPMN) no overt epithelial mucin production is observed. To date, little is known about ITPN and particularly about pancreatic cancer arising in this tumor entity.A 68-year-old male presented at our hospital with a distal bile duct occlusion suspicious for adenocarcinoma of the pancreatic head. Preoperative staging revealed no signs of distant metastasis. The patient was surgically explored and pylorus preserving duodenopancreatectomy was performed for a solid pancreatic head tumor. Final histopathology surprisingly revealed an ITPN with an associated invasive carcinoma pT3, pN0 (0/12), R0, G2.Patients with ITPN frequently present with jaundice suspicious for a bile duct stenosis or a malignant tumor of the pancreatic head. Although, it is possible to diagnose ITPN by endoscopic retrograde cholangiopancreaticography, many tumors are found not before histopathological examination. Differential diagnosis includes ductal adenocarcinoma of the pancreas, neuroendocrine tumors, IPMN, distal bile duct tumors, and solid pseudopapillary neoplasms. Using immunohistochemistry, other entities of pancreatic tumors can be ruled out. In case of R0 resection oncological prognosis is described to be more favorable when compared to regular ductal adenocarcinoma.ITPN displays a rare entity of pancreatic neoplasms. As shown in the present case report, there is a relevant potential of malignant transformation and therefore radical surgical resection and oncologic follow-up is warranted. They were included into the subgroup of premalignant epithelial tumors. Two different types of intraductal neoplasms of the pancreas were noted in the 2010 WHO classification: intraductal tubular papillary neoplasm (ITPN) and intraductal papillary mucinous neoplasm (IPMN) [Intraductal tubular papillary neoplasm (ITPN) displays a rare subtype of epithelial neoplasms of the pancreas. This entity has been described for the first time in 2009 by Yamaguchi et al. . In the While IPMN has become a clinically well described and established entity of cystic pancreatic neoplasms ,4, very Due to its rarity, data about the natural history of ITPN and particularly about pancreatic cancer arising in ITPN is very limited . Moreove2A 68-year-old male presented at an external hospital with painless obstructive jaundice and a loss of weight of 5 kg over the last two months. Except for a Billroth II gastric resection in 2011 for ulcer disease, no previous abdominal surgery was noted. Secondary diagnoses included a smoking history of 10 pack years, arterial hypertension, and a mild type 2 diabetes treated with metformin. The diabetes lasted for several years and was not new onset. Using contrast-enhanced multi-phase computed tomography (CT), a tumor in the head of the pancreas associated with a distal biliary obstruction was diagnosed suspicious for pancreatic cancer . The Ca Subsequently, the patient presented at the Katharinenhospital Stuttgart as a referral center for hepatobiliary and pancreatic surgery seeking a second medical opinion. The patient again underwent staging, which revealed no signs of liver or lung metastasis . Upon anFinal histopathology of the surgical specimen surprisingly revealed a pT3, pN0 (0/12), R0, G2 ductal adenocarcinoma arising in the background of an ITPN . The tumA follow-up re-staging CT in March 2018 revealed no signs of recurrent disease or distant metastasis. The patient therefore underwent restoration of intestinal continuity by an ascendodescendostomy. The postoperative course was uneventful and the patient was discharged on postoperative day six. To date the patient is in a good clinical condition without signs of recurrent disease and with a non-insulin dependent diabetes mellitus treated orally with metformin as preexisting prior to surgery.3Clinical data about ITPN remains very limited. ITPN is a rare entity and was not recognized in literature before 2009. It is estimated that ITPN accounts for less than 1% of all pancreatic exocrine neoplasms and only approximately 3% of intraductal neoplasms . In 2016ITPN is frequently compared with IPMN ,4. Both Only few articles have focused on imaging findings for ITPN. Oh et al. and Ishigami et al. have emphasized upstream dilation of the main pancreatic duct due to a mass effect with subsequent obstruction in ITPN whereas IPMN typically leads to a downstream dilation of the main pancreatic duct due to hypersecretion of mucin . HoweverIn summary, it seems very difficult to identify ITPN preoperatively because no specific characteristics can be used to differentiate it reliable from other cystic or solid neoplasms of the pancreas . In the Histologically, ITPN shows a tubulopapillary growth with entirely high-grade atypical cells with no or only minimal cytoplastic mucin and no visible mucin secretion in the pancreatic ducts. In order to further distinguish ITPN from IPMN, immunohistochemistry may be helpful. ITPNs are typically positive for cytokeratin (CK) 7, CK19, MUC-1, and MUC-6, and they are negative for MUC-2, MUC-5AC, trypsin, and b-catenin. This is in contrast to IPMNs, which are usually positive for MUC-5AC but often negative for MUC-1 . In contOncological surgical resection of ITPN remains the only curative management option . In caseThe presented patient received adjuvant chemotherapy using gemcitabine and capecitabine (Xeloda). Since cancer arising in ITPN is a very rare tumor entity, no clinical studies are available on this topic. Kuscher et al. for example reported that in a comparable case with a pT2, pN0 0/25), R0 tumor an interdisciplinary tumor conference decided for short time follow-up (three months) without adjuvant chemotherapy [, R0 tumo4ITPN displays a rare entity of pancreatic neoplasms. As shown in the present case report, there is a relevant potential of malignant transformation and therefore radical surgical resection is warranted and displays the only curative treatment option. When compared to regular ductal adenocarcinoma, patients with ITPN reveal a lower incidence of lymph node metastasis and a better overall survival.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Contributions of the authors:Study concept and design: SF, RH, JK.Data collection and interpretation: SF, RKS, CMS.Writing: SF, RKS, CMS, RH, JK.Proof reading: SF, RKS, CMS, RH, JK, KF, GR, AB.Stefan Fritz, MD accepts full responsibility for the work, conducted the study, had access to the data, and controlled the decision to publish.Commissioned, externally peer-reviewed.This is a case report without personal data or personal figures of the patient. Therefore no ethical approval is required.The case report was written without any funding.The case report does not contain data of human studies.The authors declare that there are no conflicts of interest.


Heterotopic pancreas is a rare congenital anomaly. We report a case of esophageal heterotopic pancreas complicated by recurrent mediastinal abscess and treated by minimally invasive resection.A 31-year-old woman was admitted with a history of recurrent chest pain, dysphagia, and heartburn. CT scan revealed focal confined collection in the lower mediastinum surrounding esophagus. Endoscopic ultrasound revealed a subepithelial lesion. The patient was treated by minimally invasive esophagectomy and made an uneventful postoperative recovery.The management of subepithelial lesions would depend on their size, ability to exclude other etiologies and their associated symptoms. The patient, in this case, was obviously symptomatic and accurate differentiation from malignant etiologies could not be accurately made.Although pancreatic heterotopia is rare, it should be remembered in the differential diagnosis of various gastrointestinal lesions. It is qComplications of the heterotopic pancreas are pancreatitis of the heterotopic tissue, pseudocyst formation, abscess, endocrine dysfunction, malignant degeneration, mechanical obstruction, and bleeding .We report an exceedingly rare case of symptomatic esophageal heterotopic pancreas appearing as recurrent mediastinal abscess, and treated as minimally invasive esophagectomy. The work has been reported in line with the SCARE criteria .22.A 31-year-old black woman was admitted with a history of recurrent chest pain, dysphagia, and heartburn, no complaint of fever or weight loss, and no significant past medical history. Patient denied swallowing any foreign body. She denied alcohol consumption. On admission, physical examination revealed normal sinus rhythm, normal pulse and blood pressure, normal temperature, and mild abdominal pain, without peritonitis. BMI: 22.1 kg/mLab results: Hgb: 14.4 g/dL; WBC: 9.3 × 103/μL; serum amylase: 653 U/L; serum lipase: 544 U/L; liver enzymes were within normal limits. CT scan revealed distal esophageal wall thickening and focal confined collection with air in the lower mediastinum surrounding esophagus, and pancreas showed neither parenchymal enlargement nor changes in density . EndoscoThe main differential diagnoses of subepithelial esophageal lesions are leiomyoma, lipoma, varices, neural tumors , granular cell tumor, inflammatory fibroid polyp, duplication cyst, lymphangioma, Brunner’s gland hyperplasia, GIST, carcinoid, and metastatic carcinoma . After cAlong two-year follow-up, the patient had four readmissions with a similar clinical presentation , all of The patient made an uneventful postoperative recovery, being discharged from hospital 10 days after surgery. Pathological analysis of specimens revealed a heterotopic pancreatic tissue, containing acini, ducts, without islet cells .Fig. 5HaAfter a two-year follow-up, the patient had no complaints, with no recurrent symptoms.3Esophageal heterotopic pancreas with recurrent inflammation and abscess formation treated by minimally invasive esophagectomy has not been previously reported. Lowry et al.  and TakeIn this case report, an extra-mucosal resection would be risky due to the high level of surrounded fibrotic tissue, besides the fact that the possibility for malignant lesion was not ruled out, yet unlikely. Also, short-segment distal esophageal resection may be associated with severe gastroesophageal reflux . AnotherThe management of subepithelial lesions would depend on their size, ability to exclude other etiologies, and their associated symptoms. The patient, in this case, was obviously symptomatic and accurate differentiation from malignant etiologies could not be accurately made. Endoscopic ultrasound is the most accurate study to differentiate submucosal lesions, especially with ultrasound-guided fine-needle aspiration . In this4Heterotopic pancreas is an uncommon congenital anomaly. While the majority of patients are asymptomatic, patients may show clinical complications such as inflammation and abscess. We report a case of esophageal heterotopic pancreas complicated by recurrent abscess treated by minimally invasive resection. Although pancreatic heterotopia is rare, it should be remembered in the differential diagnosis of various gastrointestinal lesions.The authors received no specific funding for this work.Ethical approval exemption was given for this study.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not applicable.Francisco Tustumi.Edno Tales Bianchi.Not commissioned, externally peer-reviewed.The authors declare no conflict of interest.Edno Tales Bianchi: Conceptualization. Francisco Tustumi: Writing - original draft. André Fonseca Duarte: Writing - review & editing. Evelin Sánchez Ortiz: Methodology, Writing - original draft, Writing - review & editing. Sérgio Szachnowicz: Methodology. Francisco Carlos Bernal da Costa Seguro: Formal analysis, Investigation. Rubens Antônio Aissar Sallum: Validation, Supervision. Ivan Cecconello: Supervision.


Glomus tumors are usually benign tumors of the glomus cells with the immunocytochemical and structural features of smooth muscle cells. The majority of the cases of glomus tumors are benign but, rarely, they demonstrate malignant features both clinically and histologically . Although glomangiosarcoma involving extracutaneous sites is uncommon, a few cases have been reported. A glomangiosarcoma of the heart is extremely rare due to the rarity of glomus bodies in the myocardium. In this case report, we present the case of a 31-year-old female with glomangiosarcoma involving the heart with an unknown primary lesion. Glomangiosarcoma usually arises from a primary glomus tumor. These tumors commonly involve the skin, where an abundance of glomus bodies is present for temperature regulation in the dermis . An exhaA 31-year-old Caucasian female presented with progressive dyspnea and palpitations for three months. The patient reported gradual onset, intermittent dyspnea aggravated with walking, relieved with rest, and associated with palpitations. Her past medical, surgical, and family history were non-contributory. The patient denied fever, cough, chest pain, orthopnea, diaphoresis, excessive caffeine intake, or smoking/alcohol/illicit drug use. The relevant findings on physical examination included a pulse rate of 96/minutes and a blood pressure of 135/89 mmHg. On cardiac auscultation, a diastolic murmur of grade 3/6 over the left fifth intercostal space in the midclavicular line was appreciated. The electrocardiogram (ECG) showed atrial flutter at the rate of 122 beats per minute with a rapid ventricular response.Laboratory investigations, including complete blood cell count, electrolytes, thyroid profile, and liver enzymes were within the normal range. The erythrocyte sedimentation rate (ESR) was 56 mm/hr, c-reactive protein (CRP) was 12.6 mg/dl, lactate dehydrogenase was 587 U/l, and the N-terminal-pro-B-type natriuretic peptide was 1654 pg/ml.Transthoracic echocardiography revealed an irregular heterogeneous mass in the left atrium, adhering to the posterior leaflet of the mitral valve, leading to mild-moderate mitral stenosis. Cardiac computed tomography (CT) confirmed the presence of an irregular solid mass attached to the posterior wall of the left atrium Figure . SystemiMacroscopically, an irregular solid mass of 2.1×1.7×1.6 cm was found. The non-encapsulated tumor consisted of highly vascularized beige-to-pink tissue. Microscopically, the tumor was composed of polygonal cells with centrally placed round to oval nuclei and a prominent nucleolus. The cells were surrounded by thin-walled capillary-sized vascular structures and areas of coagulative necrosis. Mitotic activity was approximately 7-8/50 high-power field  ,6.Imaging studies can also be helpful in the localization and diagnosis of glomangiosarcomas of deep visceral organs, especially the heart. A computed tomography (CT) scan usually shows an irregular solid mass that can invade surrounding tissue. An echocardiogram (EKG) shows a heterogeneous mass. Hypervascularity is a typical feature of glomangiosarcoma leading to contrast enhancement on myocardial perfusion imaging.The most effective treatment of glomangiosarcomas is wide surgical excision with good prognosis. There is no recommendation for the use of radiotherapy or chemotherapy even in the presence of metastasis. However, the local recurrence rate of glomangiosarcomas is high, so long-term follow-up is generally recommended .Glomangiosarcomas are often misdiagnosed as other soft tissue tumors. Despite being rare, glomangiosarcomas should be considered in the differential diagnosis of extracutaneous tumors. Immunohistochemistry and pathology are essential in diagnosing glomangiosarcomas and should be performed in all suspicious cases of soft tissue tumors. Glomangiosarcomas metastasize frequently and have a high local recurrence rate, so a vigilant follow-up is recommended even after complete resection.


We report a case of acute pancreatitis after an elective screening colonoscopy. A 51-year-old male with a left ventricular assist device for end-stage nonischemic cardiomyopathy and a family history of colorectal cancer was admitted for an expedited heart transplant evaluation. He underwent screening colonoscopy during this admission which was technically uncomplicated apart from requiring slight maneuvering at the splenic flexure. The following day, the patient developed acute epigastric pain and one episode of emesis. Subsequent laboratory evaluation revealed a significantly elevated lipase level and cross-sectional imaging consistent with acute pancreatitis. With no evidence of gallstones, alcohol use, and hypertriglyceridemia, the acute pancreatitis was deemed to be a complication of colonoscopy. The presumed mechanism of the pancreatitis was due to mechanical trauma from insufflation and abdominal pressure, applied to at the splenic flexure, which is in close proximity to the pancreatic tail. The patient was treated with supportive care  and improved significantly over a three-day period. Colonoscopy is a common procedure for the diagnosis and management of a range of conditions and symptoms including colonic polyps, colon adenocarcinoma, colitis, and gastrointestinal (GI) bleeding. It is generally a well-tolerated and safe procedure with a low rate of serious complications. While up to 33% of patients report at least one mild, transient GI symptom, such as abdominal pain, bloating, or nausea, the rate of a serious adverse event is about 2.8 per 1000 procedures . We presA 51-year-old male with a history of nonischemic cardiomyopathy with a left ventricular assist device was admitted for expedited heart transplant evaluation. The evaluation included an elective colonoscopy in light of a family history of colorectal cancer in his mother who died at age 61 from the disease. The patient had his first screening colonoscopy at age 45 and was diagnosed with benign polyps, which were removed, and left-sided diverticulosis. The procedure was uncomplicated and he was advised to repeat a colonoscopy in five years.The patient was without GI symptoms at the time of his colonoscopy. He denied tobacco, alcohol, or illicit drug use. His medications included amiodarone, aspirin, famotidine, levothyroxine, lisinopril, metoprolol, sildenafil, and intravenous heparin as well as torsemide, acetaminophen, docusate sodium, and melatonin as needed. On examination, he had a left ventricular assist device port entering at the upper abdomen, but otherwise the abdomen was soft and nontender to palpation with normal bowel sounds and no appreciable masses or ascites.The patient underwent a standard bowel preparation which included a clear liquid diet the day prior to the procedure and 20mg of Dulcolax with 4 liters of polyethylene glycol the night prior to the procedure. Monitored anesthesia care sedation was administered with propofol. The colonoscopy was performed at night without difficulty with good bowel preparation. Abdominal pressure was briefly required to maneuver around the splenic flexure. The colonoscope was advanced to the cecum with identification of the appendiceal orifice and ileocecal valve. Findings included multiple sigmoid and descending colon diverticula and two, small (<5mm) sessile polyps that were removed using cold forceps.μmol/L . An abdominal X-ray did not reveal an obstructive bowel gas pattern or evidence of free air. A computed tomography (CT) scan of the abdomen/pelvis revealed diffuse edematous changes of the pancreas with surrounding inflammatory stranding in the bilateral paracolic gutters, extending superiorly to the perihepatic region and inferiorly to the pelvis (The night of the procedure the patient had no pain or nausea and ate dinner and breakfast the following morning without incident. He then developed epigastric abdominal pain in the midmorning approximately 12-14 hours after the procedure and had one episode of nonbloody, nonbilious emesis following lunch. On physical examination, he was afebrile with a blood pressure of 104/89mmHg, heart rate of 68 beats per minute, and oxygen saturation of 100% on room air. Abdominal examination was notable for mild distension and moderate tenderness to palpation in the epigastric region without guarding or rebound tenderness and decreased bowel sounds. Laboratory examination revealed an elevated lipase of 2275 U/L and amylase of 1141 U/L. Additional abnormal laboratory findings included an elevated aspartate aminotransferase of 105 U/L, alanine aminotransferase of 94 U/L, and total bilirubin of 1.4 e pelvis . The conThe patient was treated conservatively with bowel rest, intravenous fluids, and analgesics as needed. Over the next three days his symptoms and abdominal examination improved and his diet was advanced to a regular diet. The lipase normalized to 15 U/L.Acute pancreatitis is the leading cause of hospital admissions for GI disorders in the United States and the fifth-leading cause of in-hospital deaths . The patObstruction of the common bile duct by gallstones and alcohol abuse are responsible for 75% of acute pancreatitis cases . The patAcute pancreatitis as a complication of colonoscopy is rare: there are only five reports describing this entity to date. The reported cases and patient characteristics of acute pancreatitis following colonoscopy are summarized in Conversely, there are cases of postcolonoscopy acute pancreatitis that did not note technical difficulty. In one report, CT imaging demonstrated hemorrhage around the tail of the pancreas in close proximity to the splenic flexure that the authors hypothesized could have been due to trauma , while iWe agree with the proposed mechanism of mechanical trauma at the splenic flexure leading to pancreatic injury. Carbon dioxide is the preferred gas used for insufflation during colonoscopy in our practice and was utilized during this patient's procedure. The previously reported cases do not specify the type of gas insufflation used. Of note, our patient developed abdominal pain approximately 12-14 hours after the colonoscopy. This timeline is atypical as a few case reports have reported development of abdominal pain and acute pancreatitis within a few hours after procedure . It is pIt is possible that acute pancreatitis after colonoscopy, including mild episodes, is more common than reported in the literature. Abdominal pain and nausea after colonoscopy are common occurrences that occur in nearly one-third of patients and are typically benign. However, life-threatening complications such as bowel perforation are typically ruled out with diagnostic testing. The series of cases discussed here suggest that acute pancreatitis should be in the differential diagnosis in patients presenting with these symptoms in the appropriate clinical context. For example, in patients with predominantly epigastric abdominal pain or symptoms of nausea and vomiting, the diagnosis of acute pancreatitis should merit additional consideration. This is especially important as early diagnosis and management of acute pancreatitis are key to better outcomes .In conclusion, we present a case of acute pancreatitis following elective colonoscopy and review the literature on previously reported cases. This complication is exceedingly rare; however, it may be more common than previously noted. It is important for endoscopists to keep this complication in mind, especially in technically challenging procedures.


Chronic traumatic encephalopathy (CTE) has been receiving increasing attention due to press coverage of professional football players. The devastating sequelae of CTE compel us to aim for early diagnosis and treatment. However, by current standards, CTE is challenging to diagnose. Clear clinical diagnostic criteria for CTE have not been established. Only recently, pathological diagnostic criteria have been recognized, but postmortem diagnosis is too late. Reliable biomarkers are not available. By imaging criteria, cavum septum pellucidum has been the only consistent identifiable MRI finding. Because of the imprecise nature of diagnosis based on clinical suspicion, physicians must become cognizant of the broad spectrum of presentations of CTE. With this awareness, appropriate workup can be initiated. CTE can present with early symptoms of emotional changes or late symptoms with memory decline and dementia. Here we present an unusual case of a patient with Alzheimer's disease secondary to suspected CTE that stems from subconcussive head impacts presenting with severe memory and MRI changes. Clinicians should be aware of this presentation and consider CTE in their differential diagnoses while undergoing workup of memory disorders. Chronic traumatic encephalopathy (CTE) is the long-term consequence of brain injury, due to repetitive brain trauma. It is associated with collision sport athletes, military personnel, domestic violence, and head banging behavior. The incidence for concussion is high, affecting between 1.6 and 3.8 million people per year . The preCTE was first described as “punch drunk” syndrome in retired boxers . Later iThere is no reliable biomarker for CTE. Imaging of CTE shows numerous varied findings, with only cavum septum pellucidum being a consistent finding. In a study of boxers, brain MRI showed hippocampal atrophy and cavum septum pellucidum, periventricular white matter changes, and cortical atrophy , 11. StuEmerging evidence shows that repetitive subconcussive head impacts correlate best with CTE –19, suggCurrently management of CTE is symptomatic treatment of the neuropsychiatric or cognitive symptoms. Because many of the patients present at a younger age, while they are still working, diagnosis of CTE is critical for treatment, counseling, and prognosticating. Unless physicians consider CTE in their differential diagnosis, these younger patients cannot obtain their necessary early treatment interventions. In addition, the proper diagnosis is particularly important for caregivers as they will require guidance in terms of planning and coping with this devastating disease.Increasing the awareness of these clinical presentations for the clinicians is key to facilitating the appropriate workup and management. Here we present a case of presumed CTE with a former football player, who suffered from repetitive subconcussive brain impact. He presented with severe memory, cognitive deficits, and significant MRI changes and thus was diagnosed with Alzheimer's disease secondary to suspected CTE.Patient was a 54-year-old right-handed male, former professional football player. He first developed memory problems at the age of 46. Initially, he seemed more forgetful. The onset and the progression of the short-term memory problem were gradual over about eight years. He always did his own finances in the past. However at the age of 46, he started spending money more irrationally and was not paying the bills on-time. He repeated questions, sometimes even just a few minutes later. He had trouble learning new information. He could not manage his own calendar. He has become dependent on the GPS to get around. Patient had become less social. He did not have depressed mood; however, he had become more irritable and more easily angered. He had no behavioral issues. His activities of daily living (ADLs) were intact.Patient started playing football when he was age 7 or 8. He played football in high school and college and then professionally. He played football for total of 23 years. Although he never lost consciousness, he experienced brief moment of flashes. This type of head injury averaged 3-4 times per game. There is no family history of dementia.His mini-mental status exam (MMSE) was 24/30, and the clinical dementia rating (CDR) was 1. On neuropsychological testing, he had significant impaired verbal and nonverbal learning, recall, and recognition with rapid forgetting (more than two standard deviations). Patient's MRI showed cortical and subcortical atrophy, enlarged ventricles, and cavum septum pellucidum . The hipThis case was diagnosed as early onset Alzheimer's dementia with presumed CTE, for insidious gradual onset of memory impairment. Findings in the neuropsychological testing are consistent with AD. However, history of playing professional football and no other risk factors for AD suggest that CTE is the underlying cause of his memory impairment and MRI findings. Because there are no established clinical diagnostic guidelines for CTE, the diagnosis for CTE can only be proposed. Patient had low hippocampal volume, which fits the profile for AD . The hisIn contrast to early presentation of neuropsychological symptoms , this paThe anatomical distribution of hyperphosphorylated tau deposition suggests possible breakdown of blood brain barrier and inflammatory response during traumatic brain injury. Phosphorylated tau appearance later in disease in area other than the perisulcal space suggests that possible tau propagation. Mechanism for CTE has been proposed to involve three stages of pathological changes, including an acute/subacute state, followed by chronic/static state and then neurodegeneration .In conclusion, we suggest that the clinicians should be aware of subconcussion contributing to CTE. They should include CTE in their differential diagnosis when evaluating patients with memory impairment and dementia and recognize that Alzheimer's and CTE can coexist.


T1 weighted MRI and demonstrates partial fat suppression onfat-saturated sequences. Large intratumoral vessels likely support the diagnosisof hibernoma but are not invariably present. Fludeoxyglucose avidity on PET scanis not beneficial in distinguishing hibernoma from soft tissue malignancybecause of its inherent, metabolically active property. Owing to theradiographic heterogeneity of hibernoma, it is currently not possible todiagnose hibernoma based on imaging characteristics alone. Given the excellentprognosis of hibernoma with marginal excision alone, an appreciation of theradiographic features is helpful in the appropriate pre-operative workup of softtissue tumours.Hibernoma is a rare benign soft tissue tumour that can mimic a liposarcoma onradiographic imaging. Our case series review illustrates the clinicalpresentation and radiographic appearances of four patients with histologicallyconfirmed hibernoma. Hibernoma is usually hypointense relative to subcutaneousfat on  A 21-year-old male was admitted for a pre-operative work-up of a tensor fascia lataeperforator-free flap to a defect over the left ankle following a motorbike accident.During the clinical examination, he was noted to have an incidental finding of awarm mass over the adductor musculature of the left thigh. On further questioning,the patient complained of 2 months of mild pain over the thigh mass. Subsequently,an MRI of the left thigh showed findings that were reported as a low gradeintramuscular liposarcoma .A combiFollowing a discussion at a multidisciplinary meeting, patient underwent a CT-guidedcore biopsy of the most FDG avid part of the left thigh mass and anultrasound-guided left inguinal lymph node core biopsy. The thigh core biopsies weresuggestive of hibernoma; the histology showed brown fat composed of adipocytes withvacuolated and granular cytoplasm and small, round, bland nuclei. No cytologicalatypia was identified. The left inguinal lymph node biopsies revealed featuressuggestive of only reactive changes, with no evidence of neoplasia.The patient subsequently underwent an elective wide local excision of the left thighmass. He had an uneventful post-operative course and was discharged from thehospital on Day 5. On clinic reviews, the patient had no complaints and was able toreturn back to work with full function and good cosmesis of the treated leg. Thehistopathology confirmed an intramuscular hibernoma. The excised mass measured 17× 13 × 6.5 cm and weighed 800 g . The vasA 26-year-old male was referred for the management of a large lump on his leftanterolateral thigh. The patient noticed the lump after the area was traumatized,whilst playing rugby. An ultrasound scan was performed as an initial investigationshowing a non-specific intramuscular lesion . An MRI A 53-year-old male presented following an incidental finding of a mass on an MRI scanof the neck. The lesion was asymptomatic and there were no palpable masses onphysical examination. An MRI scan of the neck revealed a 2.5 × 3.8 ×4.2 cm lobulated mass within the right semispinalis capitis muscle . An exciA 52-year-old male presented with an asymptomatic right shoulder mass forinvestigations. MRI revealed a well-circumscribed mass that measured 6.0 ×7.2 × 8 cm . An inci1 The primary function of these fat cells is thermogenesis. Brown fat is alsophysiologically present in non-hibernating species, including newborn human, but ittypically regresses with age comprising less than 0.1% of total body weightby the age of 70 years.2 Hibernomas arise from the vestiges of fetal brown fat; according to thelargest series published to date, the thigh was the most commonly affected site.3 Other common sites include neck, back, axilla and shoulder. Hibernomas aremost common in the third or fourth decades of life.3Hibernoma is a rare benign soft tissue tumour that consists of brown fat, immaturefat cells. The term hibernoma was first described in 1914, owing to its resemblanceto the brown fat in hibernating animals, where large quantities were found.4 that patients should be made aware of the potential effect of weight gainfollowing a removal of a large hibernoma. Owing to the insidious onset of hibernoma,the true incidence is unknown as most lesions often go undiagnosed.They are generally asymptomatic, slow-growing and warm to touch owing to itshypervascularity. However, they can sometimes be associated with pain and weightloss. In our case series, pain was reported in only the first case owing to itssignificant size and the subsequent mass effect. It has been postulated that weightloss is attributed to the hypermetabolism of brown fat, but further studies arerequired to elucidate the underlying pathophysiology. It is recommended6 In our case series, preoperative core needle biopsies and incisional biopsywere performed without any bleeding complications. Hibernomas are benign tumours andcomplete excision results in cure in all reported cases. Recurrence has not beenreported in the literature, except in a case where complete excision was notpossible owing to its location impinging on the brachial plexus and axillary vessels.7Previous reports discourage preoperative core needle biopsy in suspected cases ofhibernoma owing to the potential risk of bleeding resulting from its hypervascularity.8 which is consistent with our finding in Case 2. However, ultrasound is alsoof limited value, as it is generally unable to characterize tissue componentsaccurately, and is limited in its ability to assess the full depth and extent of thelesion. CT performs better in terms of assessing the extent of the lesion, thepresence of fat and hypervascularity, manifesting as visible vessels, andenhancement of parts of the lesion after intravenous iodine contrastadministration.The imaging features of hibernomas are unfortunately, largely non-specific. 3 These lesions are rarely seen in the retroperitoneum, despite brown fat beingcommonly found in the peripancreatic and suprarenal retroperitoneum.9 On T1 weighted imaging, all four cases demonstrated hyperintensity comparedto skeletal muscle, but hypointense relative to subcutaneous fat in three cases, andisointense to fat in one patient. These findings are concordant with the literature.11 Incomplete fat suppression, as in all our cases, precludes the diagnosis of asimple lipoma. On fat-suppressed MRI images, signal suppression in a hibernoma maybe incomplete because of the nature and amount of the lipid content.9 After gadolinium contrast, enhancement is variable but generally present and heterogeneous.10 In our patients, three lesions showed significant gadolinium enhancement, butone lesion showed negligible enhancement. Large flow voids indicating intratumoralvessels with fast flowing blood are common and offer some degree of specificity forthe diagnosis of hibernomas as opposed to angiolipomas, haemangiomas, liposarcomasand other soft tissue sarcomas.12 The latter lesions typically lack large intratumoral blood vessels.12,13 In our patient with a massive 800-g hibernoma, large flow voids weredetected, which strongly supported the pre-operative diagnosis of hibernoma.Furthermore, there is evidence to suggest that internal septation is one of thefeatures of hibernoma.14 Internal septations were present in all our cases. The heterogeneous imagingappearances of hibernomas, including the frequent demonstration of non-uniform fatsignal on MRI means that a firm diagnosis of this entity is very seldom made on MRIprior to biopsy, liposarcoma being the main imaging diagnosis requiringconsideration.MRI is the imaging modality of choice, as it provides superior characterization oftissue types and greater delineation of the margins of the lesion. In general,hibernomas are well-circumscribed, encapsulated masses, most frequently seen inareas where there is a preponderance of brown fat, such as thigh, shoulder, back,neck and mediastinum.15There is overlap in imaging features among fat-containing lesions, such ashibernomas, myolipomas, lipomas and liposarcomas. The overlap is made morecomplicated with the variable degree of differentiation of liposarcomas. Forexample, internal septations are seen in most of hibernomas but also in lipomas andliposarcomas. The thickness (>2 mm) and nodularity of internal septations hasbeen suggested as a possible feature favouring liposarcomas over less aggressivelesions. However, fine septations, which are seen in hibernomas and lipomas, arenon-specific and can still be seen in well-differentiated liposarcomas.T1 and T2 signal on MRI, which helps in differentiations from hibernomas. Theycan also demonstrate cystic changes, which are typically not evident in hibernomas.16 Another type of soft tissue sarcoma is clear cell sarcoma, which can exhibitintense enhancement similar to hibernomas. However, the signal characteristics ofT1 intensity being similar or slightly higher than muscle intensity shoulddifferentiate this entity from hibernomas.17Another differential diagnosis for hibernomas is the myxoid type of liposarcomas.This entity, which is more common in lower extremities, typically shows low18 Brown fat cell expresses unique mitochondrial uncoupling protein (UCP1),which functions to generate heat instead of adenosine triphosphate production.19 A landmark study based on 3640 consecutive FDG PET and PET/CT scansdemonstrated that the biopsies of the FDG-avid regions have the histological andmolecular characteristics of brown fat remnants, including UCP1. The lack of FDGenhancement helps in differentiating lipomas from other lesions, such as hibernomas,liposarcomas and myolipomas. Hibernomas show increased FDG avidity more thanexpected for other lipomatous lesions, which can suggest the diagnosis.20 In general, an FDG-PET SUV in excess of 2.0–2.5 is consideredconcerning for malignancy. As demonstrated in our first case with SUV-max 18.4,FDG-PET lacks specificity as FDG uptake occurs in any regions of high utilization ofglucose regardless of the underlying pathology, including benign, malignant orinflammatory processes. This extremely high level of FDG-PET avidity may beconsidered even excessive for sarcomas and may be suggestive of hypermetabolic brownfat, i.e. hibernoma. Hoshi et al21 and Charest et al22 concluded that FDG PET/CT lacks the specificity required to differentiatebenign from malignant soft tissue tumours. In addition, FDG-PET may not besufficiently sensitive in detecting myxoid liposarcoma owing to the limitation indiscerning metabolically active cells within the myxoid matrix.23 The value of FDG-PET in soft tissue tumours may lie in its accuracy indiscriminating low- and high-grade sarcomas.22 If an FDG-PET imaging is obtained, we recommend that hibernomas should beconsidered prospectively in the work-up of lipomatous tumours with high SUV levels,since an excision without adjuvant therapy is curative owing to their benign nature.Previous studies demonstrated that a single dose of oral propranolol (anon-selective beta-blocker) can reduce FDG avidity in brown fat, since brown fatcontains beta-adrenergic receptors.26 In the future, we plan to investigate whether oral propranolol administeredprior to FDG-PET can be used to help differentiate hibernoma from soft tissuemalignancy.Fluoro-labelled 2-deoxyglucose PET scan (FDG-PET) is commonly utilized as adiagnostic imaging tool to detect metabolically active tumours based on their FDGuptake. It has been proven that brown fat has a high level of FDG uptake.T1 hyperintense lesion are suggestive of the diagnosis ofhibernoma.Hibernoma is a rare, slow-growing, benign tumour that demonstratescharacteristic but non-specific imaging features. MRI is the superiorimaging modality of choice, compared to plain radiography, ultrasonographyand CT. If present, large arterial flow voids within a FDG avidity offers poor specificity in the discrimination between soft tissuemalignancy and benign lesions. Hibernoma should be included in thedifferential diagnoses of an FDG avid lipomatous tumour.Owing to the current lack of definitive imaging modality for diagnosinghibernoma, a histopathological confirmation is required to exclude softtissue malignancy.Marginal excision of hibernoma offers excellent prognosis.


Afferent loop syndrome is a rare complication following a Billroth II procedure and is seen in 3/1000 cases. This case report shows the importance of radiological imaging in a patient with a past history of abdominal surgery, for which no records were available. Imaging showed incomplete annular pancreas, leading to narrowing of the second part of the duodenum, which was the indication for gastrojejunostomy in the past, now presenting with features of afferent loop syndrome. Most cases of ALS are caused by obstruction owing to adhesions, kinking at the anastomosis site, internal hernia, stomal stenosis, malignancy or inflammation surrounding the anastomosis. Clinically, ALS is often difficult to diagnose because its presentation may be vague and non-specific. Delayed diagnosis may result in life-threatening events such as bowel ischaemia or perforation.A 70-year-old male presented with multiple episodes of loose stools and vomiting for the past 3 months, which was managed conservatively. The patient gave a history of having undergone an abdominal surgery 20 years ago for clinical features suggestive of gastric outlet obstruction. No surgical records were available. No other significant history was noted.–1 and without signs of dehydration. A right paramedian abdominal scar and mild abdominal distension were noted. There was no tenderness or organomegaly. Bowel sounds were normal. Laboratory investigations revealed haemoglobin 10.2 g/dl, normal blood counts, serum Na+ 131 mEq l−1 and K+ 4 mEq l−1, serum creatinine 0.6 mg dl−1 and serum albumin 2.4 g dl−1. Liver parameters were within normal limits.On general examination, the patient was conscious, afebrile, normotensive, with a pulse rate of 76  minThe initial differential diagnosis was broad and included abdominal abscess, peptic ulcer disease, gastric outlet obstruction, ALS, strictures, hernias, mesenteric ischaemia and bacterial overgrowth syndrome.Upper gastrointestinal endoscopy showed normal appearance of the gastrojejunal stoma. The afferent loop of the bowel was dilated proximal to the stoma and distended with fluid residue.On barium meal follow through, a gastrojejunostomy stoma was demonstrated. Additionally, abrupt narrowing of the D2 segment of the duodenum was noted with no mucosal irregularity. The contrast was seen to pass through the stoma and retrogradely fill the dilated D3, D4 and jejunal loops proximal to the stoma opening. The contrast was seen to pass into the rest of the jejunal loops .Contrast-enhanced CT (CECT) scan of the abdomen was performed for further evaluation of the narrowing in the D2 segment of the duodenum and the stoma site. On CECT of the abdomen, the cause of narrowing of the D2 segment was seen to be the incomplete annular pancreas. Narrowing of a short segment of the jejunum  with dilatation of the intervening D2, D3 and D4 segments of the duodenum and the proximal jejunal loop was noted, suggesting afferent loop dilatation  and 3a.The patient was initially managed with nasogastric tube drainage with temporary relief of symptoms, partial parenteral nutrition and probiotics, following which his general health improved. Surgical correction or luminal stent placement was offered to the patient, but the patient deferred the procedure. Subsequently, the patient was lost to follow-up.2 ALS is caused by three different mechanisms, which include mechanical obstruction of the afferent loop, preferential gastric emptying into the afferent loop and obstruction of the efferent loop resulting in preferential filling of the afferent loop. This condition occurs infrequently following gastroenterotomy reconstruction.1 ALS can be acute or chronic. The clinical features include abdominal pain, nausea and vomiting, postprandial fullness and, rarely, post-obstructive jaundice.3 In the case presented, the clinical presentation had multiple differentials, including peptic ulcer disease, gastritis, gastric outlet obstruction, small bowel obstruction, mesenteric ischaemia and ALS. Radiographic barium examination findings of ALS include non-filling of the afferent limb or preferential filling and retention of barium in a dilated afferent limb for at least 60 min.3 Fluid-filled C-shaped afferent loop (C-loop appearance) on coronal plane in combination with valvulae conniventes projecting into the lumen (keyboard sign) is the most common multidetector CT feature of ALS.1 Complications of ALS such as a dilated gallbladder, biliary dilatation and pancreatitis also are identified readily on CT and ultrasound imaging.3Proximal or afferent loop consists of the oversewn duodenal stump and the most proximal jejunum, which carry the biliopancreatic secretions. The distal or efferent loop extends downstream, carrying gastric contents distally.4Annular pancreas occurs in 1/20,000 of the population and results from failure of the ventral bud to rotate with the duodenum, causing encasement of the duodenum. This encasement of the duodenum by a band of pancreatic tissue can be complete (pancreatic parenchyma or annular duct completely surrounds the duodenum) or incomplete . In a patient with gastric outlet obstruction, pancreatic tissue extending in a posterolateral or anterolateral direction to the second part of the duodenum or pancreatic tissue anterior and posterior to the duodenum should raise the suspicion of annular pancreas.In the case discussed above, the patient had narrowing of the efferent loop of the jejunum, leading to preferential filling of the afferent loop. Since there is narrowing of the D2 segment of the duodenum (incomplete annular pancreas) proximal to the afferent loop, it leads to progressive dilatation of the afferent loop, leading to ALS.In a patient with past history of gastrojejunostomy presenting with abdominal pain, distension and vomiting, ALS should be considered in the differential diagnosis.Prompt imaging with conventional barium meal follow through and CECT of the abdomen with oral contrast is required, as surgery is usually necessary to relieve the mechanical obstruction and possibly revise the gastrojejunostomy anastomosis.Informed consent to publish this case was obtained and is held on record. Patient's identity has not been disclosed in the case report.


Ewing sarcoma (ES), which is described as diffuse endothelioma of the bone, is divided into osseous and extraosseous Ewing sarcoma (EES) mostly affecting children and adolescents. It is a rare, aggressive, and poorly differentiated small blue round cell tumor that seldom affects the head and neck regions.Herein, we reported a 46-year-old man presenting with right nasal block, epistaxis, and epiphora from the right eye for one month. The nasal endoscopy revealed a friable mass arising from the anterior half of the right nasal cavity. Histological findings were suggestive of Ewing sarcoma. A contrast-enhanced computed tomography (CT) scan of the paranasal sinuses showed a soft tissue mass in the right anterior nasal cavity with mucosal thickening in the right maxillary sinus, without any bony erosion or distant metastasis. The patient underwent endoscopic medial maxillectomy with modified Denker’s procedure, followed by a 6-cycle course of chemotherapy. He was clinically well after chemotherapy; however, the recent bone scans were suggestive of bone involvement with the tumor. The EES of paranasal sinus in the head and neck regions is extremely rare and requires exceptional attention due to their adjacent vital structures. The ES diagnosis-related dilemma arises from the numerous differential diagnoses of small round blue cell tumors. In this regard, accurate diagnosis is important, since ES requires a multi-modality approach. Furthermore, early diagnosis and aggressive intervention are crucial to obtain good prognosis and function. Ewing sarcoma (ES) was first described as the diffuse endothelioma of bone by James Ewing in 1921 that most often affects children and adolescents. The EES is a rare, aggressive, and poorly differentiated small blue round cell tumor, primarily in the soft tissues of the lower extremity and the paravertebral region. It seldom affects the head and neck region, accounting only for 1-4% of all ES . Herein,A 46-year-old Chinese man referred to us with one-month experience of gradually deteriorating right nasal block, epistaxis, and epiphora from the right eye. Nasoendoscopy revealed a reddish, friable mass arising from the right inferior meatus, extending over the anterior half of the right nasal cavity bleeding on touch . The histology showed a malignant blue cell tumor infiltrating into the subepithelial stroma. Neoplastic cells displayed round to oval nuclei with fine chromatin and indistinct cytoplasmic membrane . Fig’s.3a,3b). The patient underwent right endoscopic medial maxillectomy with modified Denker’s procedure.A final diagnosis of ES/primitive neuroectodermal tumor (PNET) was made. The contrast-enhanced computed tomography (CT) scan showed a polypoidal soft tissue mass at the right anterior nasal cavity floor, measuring 0.7×1.0×0.7 cm with mucosal thickening observed in the right maxillary sinus, without any bony erosion and no evidence of distant metastasis , and completed six cycles. Repeated rigid nasendoscopy revealed no observed tumor relapse. After a month of post-chemotherapy, a bone scan was performed and revealed an increased tracer uptake over the right maxillary and nasal bone extending to the right orbital floor. This indicated sclerotic changes of the bone which were suggestive of bone involvement with tumor. In this regard, he was advised to refer to local radiation therapy; however, he refused to continue his treatment. He was last seen at our clinic 11 months post-operation, with no sign of local recurrence. He defaulted the follow-up since then. Involvement of the paranasal sinus (PNS) in the head and neck region EES is extremely rare, the majority of these cases were mentioned in the mandible, maxilla, maxillary sinus, ethmoid sinus and nasal cavity. It has a male to female ratio as high as 2.4:1, with a median age range of 11-20 years and prevalent among Caucasians (up to 95%). More than 90% of patients refer with a rapidly growing painful mass with the signs of early central nervous system extension. The tumor of the maxillary sinus presentation might be delayed until the lesion protrudes into the nasal or oral cavity and causes obvious obstructive or nasal symptoms , as presented in our patient. Other unspecific symptoms can be paraesthesia, ulceration, pyrexia, anemia, and weight loss -5. ThereThe cytoplasm of tumor cells frequently contains perioidic acid Schiff-positive glycogen. Hallmark translocation of ES involving the fusion of the ES gene on chromosome 22 with the friend leukaemia virus integration site 1 (FLI1) gene on chromosome 11 shows the characteristic translocation t  which is present in 85% and > 90% of PNETs/ES and extraosseous ES, respectively  (6). It There are various differential diagnoses for small round blue cell tumors (SRBCTs), such as epithelial tumors (including poorly differentiated squamous cell carcinoma), mesenchymal tumors (including rhabdomyosarcoma), lymphoproliferative disorder (including lymphoma), and neuroendocrine/ neuroectodermal tumors . The extra panel of immunohistochemical stains and markers will be performed in order to establish final diagnosis and precise treatment. They include cytokeratin and anti-endomysial antibody , Desmin, myogenin (rhabdomyosarcoma), CD3, CD20, CD45, Tdt (lymphoma including lymphoblastic lymphoma markers), HMB 45, S100 protein, Melan-A (melanoma), neuron-specific enolase, chromogranin A, synaptophysin, and CD56  -9. In thThe choice of local treatment depends on the size and resectability of the tumor, as well as the primary site and critical surrounding structures with possible complications. Some prefer radiotherapy to surgery, due to difficulties in obtaining clear surgical margins, as well as the possible disfigurement and destruction caused by surgery . Siegal Our patient was subjected to endoscopic medial maxillectomy with modified Denker’s procedure. In this regard, he underwent endoscopic transnasal inferior turbinectomy and uncinectomy, followed by wide middle meatal antrostomy performed in the usual manner. Subsequently, incision was made inferiorly at the junction of nasal floor and lateral nasal wall (down to the periosteum), and superiorly along the lateral nasal wall extending to anterior-inferiorly up to the anterior end of inferior turbinate, overlying the edge of pyriform aperture. Subperiosteal flap was raised with a freer suction to expose the anterior aspect of the maxilla up to the infraorbital foramen and lateral nasal wall. Osteotomy was performed at the anterior wall of maxilla and connected to the inferior bony cut of medial maxillectomy. The osteotomy was bounded superiorly by the roof of the maxillary sinus, inferiorly by the junction of nasal floor and medial maxillary wall, and posteriorly by the posterior wall of maxillary, preserving the infra-orbital neurovascular bundle. Thereafter, the lesion was resected and removed en bloc. Nasolacrimal duct was identified and marsupialized with sickle knife to prevent stenosis.An analysis conducted by Bivas et al. showed that baseline high white blood cell count  was an independent predictor of the worst event-free survival, due to possible micrometastatic disease without overt metastasis. Histologic filigree pattern carried poorer prognosis, based on the evaluation of the Intergroup Ewing Sarcoma Study (IESS) data in 1983 .AccordinThe ES is extremely rare to occur as a primary tumor in the head and neck region, especially at PNS; however, it has better prognosis and lower mortality. Early and accurate diagnosis, as well as aggressive intervention with multimodality approaches, are crucial to obtain good prognosis and functionality after the treatment. However, patient still needs to be followed up closely since local recurrence and distant metastasis are common.