Fibro-osseous lesions are common in the paranasal sinuses. The incidence of fibrous dysplasia (FD) in the ethmoid sinus is rare. Patients with such lesions are usually asymptomatic until the lesion is large enough to start compressing adjacent structures and organs. Common presentations include nasal obstruction, headache, eye swelling, and diplopia. Meanwhile, less common signs can include decreased visual acuity. We present a case of a 65-year-old male with comorbidities who presented to the clinic complaining of a chronic nasal obstruction, headache, and decreased visual acuity in the right eye. On endoscopic examination, a lesion was identified in the ethmoid sinus. Computed tomography was performed and confirmed the positioning of the lesion within the ethmoid sinus compressing the optic nerve. Total excision was performed through a direct nasal endoscopic approach. The lesion was excised completely with no recurrence. Histopathology report confirmed the lesion to be of cartilaginous nature, and a final diagnosis of cartilaginous fibrous osteoma was made. Such lesions are usually benign and symptomless. Excising the lesion completely is the best approach to decrease the chances of recurrence. The ethmoid sinus is one of the four paired paranasal sinuses in the human body. It is formed from air cells within the ethmoid bone between the eyes and nose . ParanasRadiologically, X-rays cannot provide sufficient details of the tumor. Meanwhile, magnetic resonance imaging (MRI) can give inaccurate results due to bone and tissue delineation . ComputeWe present a case report of a 65-year-old male patient with type 2 diabetes, hypertension, and ischemic heart disease. The patient presented to the otolaryngology clinic complaining of chronic nasal obstruction that was associated with right eye swelling, diplopia, and progressive decrease in visual acuity of the right eye. Endoscopic examination of the nose was done and revealed a lesion that was smooth on the surface reaching to the middle meatus, covered by mucosa and hard on palpation. The lesion has bounded the ethmoid sinus and the floor of the optical orbit with invasion of the lamina papyracea, extending to the inside of the orbit Figure .CT scan was done and showed calcified lesion and a glass ground appearance in the right ethmoid air cell eroding the medial wall of the right orbit, displacing the medial rectus muscle and globe laterally and originating from the ethmoid air cell and nasal cavity. In addition, the optic nerve was found to be compressed by the lesion Figure .The lesion was surgically removed using an endoscopic approach with drilling of the nasal part of the mass, and the orbital part was removed as one piece Figure . The patMicroscopically, the histological section showed fragments of sinonasal mucosa, with an underlying well-circumscribed bone-forming lesion composed of a mixture of lamellar and woven bone trabeculae with fibrous stroma. The stroma was surrounding and encasing the bone trabeculae, which were randomly arranged, irregular, and arising directly from the fibrous tissue. The fibrous area was composed of spindle cells in a storiform pattern showing bland, tapered nuclei. Other areas showed nodules of hyaline cartilage with disorganized growth, platelike ossification. A follow-up CT scan was performed after the operation and showed complete resolution with no signs of the lesion Figure .FD is an uncommon lesions that may occur as an isolated asymptomatic monostotic lesion or a severe polyostotic disease resulting in fractures and deformities . FD of tRadiologically, CT scans of FD often report a ground glass appearance. However, only a majority of CT scans show this resemblance, making postsurgical histopathology with CT scan the option of choice in the diagnosis of FD ,10. FurtThe main treatment of any paranasal fibroma is total excision of the lesion because the disease itself is usually symptomless until compression of adjacent structures occurs . EndoscoFibrous dysplasia is a fibro-osseous benign lesion of the paranasal sinuses. Its involvement in the ethmoid sinus is rare, but not fatal. Common presentations include headache, eye swelling, and diplopia. Compression of the optic nerve can lead to a progressive decrease in visual acuity unilaterally. Direct endoscopic examination is helpful, and computed tomography is the imaging modality of choice in diagnosing the lesion appearing as a glass ground mass. Total excision of the lesion through a nasal endoscopic approach has the least chances of recurrence with high chances of complete resolution.


Multinodular and vacuolating neuronal tumor (MVNT) is a rare benign brain lesion, commonly found in middle-aged adults. The patients experience a range of symptoms from being asymptomatic to epileptic seizures, with headache being the most common symptom. Here we report a case of an incidental diagnosis of MVNT in a young female. A 25-year-old female with a past medical history of occasional headaches without seizures or any focal neurological deficit presented after a motor vehicle rollover. The MRI brain revealed an incidental finding of a subcortical lesion in the right parietal lobe with T2-FLAIR (fluid-attenuated inversion recovery) hyperintensity between the cystic portions, indicative of a possible MVNT, with a less probable chance of dysembryoplastic neuroepithelial tumor based on the subcortical location of the lesion. No neurosurgical intervention was recommended. With one-year follow-up, no changes were noted on neuroimaging, and the patient remained stable without any neurological symptoms. The MVNT is a rare brain lesion that presents with benign features. In patients with epileptic symptoms, surgical resection of the lesion can be curative. However, in asymptomatic patients, careful monitoring may be sufficient, as described in this case. In 2013, the first documented case of multinodular and vacuolating neuronal tumors (MVNTs) was reported . SurgicaA 25-year-old female with a past medical history of hepatitis C, heroin abuse, and depression was admitted after a motor vehicle accident that resulted in multiple fractures. An MRI brain with and without contrast done at initial presentation revealed an incidental finding of a subcortical lesion in the right parietal lobe with T2-FLAIR (fluid-attenuated inversion recovery) hyperintensity between the cystic portions indicating a possible MVNT, with a less probable chance of dysembryoplastic neuroepithelial tumor based on lesion location Figure -1C.At the time of initial presentation, the patient did not report any visual disturbances, seizures, nausea, vomiting, or other neurological deficits. She had a past medical history of occasional headaches responding to over-the-counter pain medications. She had no past medical history of any neurological condition. The patient was identified to have abnormal brain lesion on neuroimaging as a child too; however, this could not be verified due to inability to obtain her childhood scans. Neuroimaging at her three-month, six-month, and one-year follow-ups showed no change in the appearance of the brain lesion; also, the patient continued to have no neurological symptoms except for occasional headaches. Surgical intervention was therefore not recommended.First reported in 2013, MVNTs are sporadic benign brain tumors . MVNTs hSurgical resection of MVNTs is discouraged for asymptomatic cases, only to be recommended when seizures are definitively linked to the MVNT ,3-6. A dHere we present a case that is particularly rare due to several factors. With our patient being 25 years old, she was much younger than the average patient diagnosed with MVNT ,3-6. MorMVNT is a rare, benign brain lesion that tends to remain unchanged over time. MVNTs have reliable radiologic findings; however, these tumors have varying presentation related to the location in the brain. Here we described a case of an asymptomatic MVNT in a 25-year-old female, younger than the widely reported age of MVNT diagnosis. Over the available follow-up period, the patient’s tumor remained unchanged since the initial incidental finding. The present report adds to the limited number of MVNT cases with radiological features reported in neurosurgical literature.


Objective: The aim was to report 4 patients with intra-articular knee synovial haemangioma (KSH) and to perform a systematic review to describe the patient characteristics, patterns of tumor location, clinical presentation, usefulness of imaging examinations, pros and cons of arthroscopic vs. open resection, and follow-up in the literature.Design: From 1996 to 2016, four patients with KSH were retrospectively reviewed. A literature search was conducted in PubMed from 2000/01 to 2020/06 using the search terms “synovial haemangioma” and “knee.” Fifty full-text articles that included a total of 92 patients were included for further discussion.Results: Four adults (20–40 years) were diagnosed with KSH. Three lesions located in the suprapatellar pouch, two eroding the patella and one the supratrochlear bone, and one in the posterior compartment. Persistent anterior knee pain was the main complain. MRI revealed a benign tumor mass in all cases except one. Open excisional biopsy and regional synovectomy were performed in three patients, and by arthroscopy of the posterior compartment in the fourth. Histological type was arteriovenous in three cases and capillary in one. A pain-free knee without recurrence was achieve in all cases except one, which was successfully reoperated. Average follow-up time was 3.5 years. A literature review showed that KSH appears most frequently in children and teenagers (64.6%) and does not differ by gender. The suprapatellar and patella-femoral joint compartment was the most frequent location (47.9%). The bony tissue of the knee was rarely affected (13.5%). Pain, swelling and haemarthrosis were frequently reported . MRI was the most commonly used imaging test (98%). Treatment consisted of regional synovectomy by open surgery or arthroscopy in 66.7 and 15.6% of cases, respectively.Conclusions: KSH should be considered in the differential diagnosis of adult patients with chronic low-intensity knee pain. MRI is the most useful exam because it establishes the location, extent and benign characteristics of the tumor. Definitive diagnosis requires histological examination. We believe excisional biopsy and regional synovectomy by arthroscopy should be the treatments of choice for intra-articular tumors, but we recommend open surgery when the lesion extends to the tendons, muscle or bone. Synovial haemangioma (SH) is a rare benign tumor accounting for 0.07% of all soft tissue tumors and 0.78% of all excised haemangiomas . While tKnee synovial haemangioma (KSH) can present as diffuse or localized intra-articular, juxta-articular or intermediate (intra- and juxta-articular) synovial tissue masses , 5–7. ExMost are located in the anterior compartment of the knee , 12. OnlThe clinical presentation is non-specific, with symptom onset usually occurring in childhood and young adulthood, although it has been described at later ages, probably due to delayed diagnosis. Various imaging modalities have been used, but all are non-specific, and there is no clear diagnostic algorithm , 57, 58.Once a benign synovial lesion is suspected, it is unsettled if a biopsy is needed or if excisional biopsy and regional synovectomy with histological examination can confirm the diagnosis and simultaneously accomplish definitive treatment. Whether open , 38, 57 Further investigation is warranted, particularly because the existing information is scattered and based mostly on isolated clinical cases. An understanding of the differences in patient characteristics and clinical presentation, along with knowledge of the real value of imaging examinations and follow-up results after arthroscopic or open surgery, is important for making informed decisions on the need for invasive imaging examinations and on the differential effectiveness of open vs. arthroscopic treatment in light of the evidence.The aim of the study was to describe four patients with intra-articular KSH, two with the previously undescribed phenomenon of patellar bone erosion and one with an unusual tumor location in the posterior compartment, and to perform a systematic review of PubMed publications on KSH from 2000 to 2020 to describe the characteristics of the patients , the patterns of tumor location, the non-specific clinical presentation, the usefulness of imaging examinations, the pros and cons of arthroscopic vs. open resection, and the follow-up time and recurrence after treatment.Based on hospital records, we identified four patients with KSH who were seen between 1996 and 2016. Demographic data, symptoms, imaging tests, treatment and follow-up were retrospectively reviewed. All patients underwent surgical treatment by one surgeon (JHH). Written informed consent was obtained from the patients in all cases, and the retrospective study was approved by the hospital ethical committee . The procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation  and with the Helsinki Declaration of 1975, as revised in 2000.A PubMed literature search was performed for full text publications between 2000/01 and 2020/06; these dates were selected because MRI was widely available in this period. The search terms were as follows:  AND (Knee) AND (2000/01:2020/06). Selection was restricted to single case reports or case series of KSH that described the diagnosis and at least four items in our study objectives. Only original reports or studies written in English, Spanish or French and available in full-text form were included.Each original manuscript was scrutinized, and search results on the number of cases described, the age and gender of the patients, the side of the affected knee, the tumor location, the history of previous trauma, the symptoms and signs, the presence of cutaneous haemangioma, the delay in diagnosis, the image examinations performed, the presence of bone involvement, the type of treatment, the histological type and the follow-up time were screened and collected in a database by two reviewers independently  to ensure accuracy and then stored on a Microsoft Excel spread sheet. Any discrepancy in the data collected was reviewed by both reviewers until an agreement was obtained. The review was limited to reporting variables that were obtained in each publication; when a variable was omitted, it was registered as not reported. The results are presented as the number of cases and the percentage with respect to the total number of cases, except for symptoms and signs and image examinations, which are presented as the number of manuscripts where they are reported and the percentage with respect to the total number of manuscripts reviewed. Age, delay in diagnosis and follow-up time are presented as the mean ± standard deviation of data available, and the percentage of cases not reporting those data was also specified. In order to avoid multiple reports of the same study, studies with the same authors were double-checked, and comparisons of case descriptions and treatments were performed. Neither of the review authors was blinded to the journal titles or the study authors or institutions.Results tabulated by age, gender, affected knee side, tumor location, history of previous trauma, clinical symptoms and signs, presence of cutaneous haemangioma, time in diagnosis delay, imaging test used, bone involvement, type of treatment, histology, follow-up time and recurrence are shown in A 20-year-old male suffering from sudden intense pain in the right knee with swelling presented to the emergency room. He had had occasional pain for 5 years, but never at that intensity. Palpation of the external aspect of the patella was painful. There was no patellar instability; meniscal, ligamentous and neurovascular examinations were normal.Plain radiographs were normal. Joint aspiration revealed a haemarthrosis. Anterior knee pain syndrome due to patella-femoral cartilage damage or synovial derangement was suspected. Bone scintigraphy with 99mTc revealed an increase in uptake in the anterior portion of the knee. MR revealed a suprapatellar and supratrochlear synovial tumor extending medially and eroding the supratrochlear cortical bone; the tumor was characterized by hyperintensity on T2-weighted images and hypo-/isointensity to muscles on T1 sequences .A non-specific benign synovial knee tumor, either SH or localized pigmented villonodular synovitis (LPVNS), was suspected. An open excisional biopsy, partial synovectomy and curettage of bone were performed. Histological examination reported proliferation of arterial and venous vessels in a fibrous stroma, consistent with the diagnosis of arteriovenous-type SH .Recurrence of the tumor was observed on MRI follow-up 5 years later. MRI revealed diffuse involvement of the sub-quadriceps synovial lining and tendon . An openA 22-year-old male was referred to our hospital for persistent anterior right knee pain lasting 6 years. He had moderate pain that did not limit activities of daily living but prevented him from practicing sports. He had no history of prior trauma, although he had experienced minor impacts during sports. On physical examination, he had lower limb varus alignment, no muscle atrophy, no effusion, normal range of motion, a stable knee, and normal meniscal and neurovascular examinations.Radiographic images were normal. MRI was reportedly normal. Due to the persistence of symptoms despite analgesics and muscle strength exercises, diagnostic arthroscopy was performed. We detected no evidence of joint damage. Histological and microbiological tests of the synovial lining from various areas were normal.However, owing to worsening symptoms 2 years later, another MRI was performed, revealing a tumor in the synovial lining just over the proximal pole of the patella; the tumor was isointense on T1 and hyperintense on T2, with intra-osseous invasion of the upper pole of the patella , suggestUpon open surgery, the synovial lining above the upper pole of the patella was swollen and bluish brown, with poorly defined margins, and there was invasion of the quadriceps tendon and the superior pole of the patella. We resected the tumor and half of the quadriceps tendon to healthy margins and curetted the superior patellar bone lesions back to normal trabecular bone. Histological examination revealed blood vessels with multiple arterial and venous vessels in the stroma, confirming the diagnosis of arteriovenous SH.At the 6-week follow-up, the patient had a stiff knee with a range of motion of 0–30°. At 10 weeks, we performed arthroscopic arthrolysis and obtained new biopsy samples. Histological examination showed no evidence of a tumor. At the 5-year follow-up, the patient had a pain-free knee and a normal range of motion. A repeat MRI showed no evidence of recurrence.A 31-year-old man presented with an 8-year history of diffuse right knee pain, which persisted at rest and was aggravated with activity. The pain limited his participation in sports but not his activities of daily living. On physical examination, the knee was well-aligned but had mild swelling and tenderness adjacent to the medial patellar facet. There was no muscle atrophy or warmth. The range of motion and stability were normal, as were the meniscal and neurovascular examinations.Plain radiographs were normal. On aspiration, the joint fluid was clear, and the cell count was normal with no microcrystals. We suspected anterior knee pain syndrome due to patella-femoral osteoarthritis. MR revealed an intra-articular synovial mass located superior and medial to the patella that had eroded into the superior patellar cortex. Heterogeneous lower signal intensity on T1-weighted imaging and isointensity on T2-weighted imaging suggested LPVNS .Open excisional biopsy and partial synovectomy were performed. Two small intra-osseous cystic lesions in the medial and superior poles of the patella were curetted until healthy trabecular bone was observed. Histological exam showed synovial proliferation with subjacent capillaries, consistent with capillary-type SH .The patient had an uneventful postoperative recovery, with restoration of full range of motion and good muscle strength. He resumed amateur sports. Four years after surgery, he had a normal pain-free knee, and there was no evidence of tumor recurrence on MR.A 40-year-old male presented with a 10-year history of progressive anterior and posterior right knee pain with insidious onset. He had no history of trauma. Pain occurred when he performed maximum knee flexion or intense cycling but not during activities of daily life.On clinical examination, both lower limbs were aligned in varus. There was no muscular atrophy, swelling, tenderness or joint effusion. The range of motion of the knee was normal, but the patient reported discomfort at maximum flexion. The knee was stable, and the meniscal and neurovascular examinations were normal.Plain radiographs were normal. MRI showed a well-defined intra-articular synovial mass in the posterior compartment of the knee, behind the PCL. On T1-weighted sequences, the mass was isointense to the surrounding muscle signal with peripheral heterogeneous areas, and on T2-weighted imaging, it was hyperintense . A benigThe patient opted for observation and regular follow-up. Four years later, his pain had increased and was more frequently limiting his activities of daily living. Repeat MRI showed features similar to what had been observed previously, but the patient elected to proceed with surgery. On arthroscopy, we observed a 1 × 0.5 × 0.5 cm pale bluish vascular lesion in the synovial lining of the posterior capsule just behind the PCL . An exciThree months after surgery, the patient progressively resumed recreational sports. One year after surgery, the patient was asymptomatic, even when cycling. There was no evidence of recurrence on repeat MRI.We identified 75 abstracts from articles located through the literature search. Eight articles on KSH case descriptions were not reviewed because six were in other languages , one full-text article from an Indian journal was not available and one was an open radiology case. Four articles described SH in other joints, and 13 did not focus on KSH clinical cases or case series. Ultimately, we reviewed 50 full texts that focused on case or case series descriptions, including a total of 92 clinical cases. A flow chart of the literature search is shown in The literature review showed that KSH appears more frequently in children and teenagers (64.6% of cases). The average age of presentation was 21.7 ± 14.7 years, and the prevalence was not related to gender . The right knee was more frequently affected (39.6%).The tumor mass could appear anywhere in the joint, but an anterior knee location in the suprapatellar and patella-femoral (47.9%) joint space was the most frequent, followed by the infrapatellar (14.6%), posterior (9.4%) and medial compartment (7.3%) and the intercondylar notch (4.2%). Usually, there was no previous trauma (39.6%), or it was not reported (46.9%).Pain, swelling and haemarthrosis were the most frequently described clinical presentation form , although the symptoms and signs associated with KSH were multiple and non-specific see . CutaneoThe most commonly reported image tests were plain XR (86.3%) and MRI (98%) alone or enhanced with gadolinium (MRGd). Ultrasound (US), echo Doppler (ED), arteriography, computed tomography (CT) or scintigraphy (Sc) were used less frequently (9.8–5.9%) In rare cases (3.9–2%), venography, CT arthrography (CTArt), CT angiography (CTA), multi-detector computed tomography angiography (MDCTA), red blood cell scintigraphy (RBISc) or single-positron emission computed tomography (SPECT) were reported. Knee bone erosion was occasionally observed (13.5%) on image exams.Synovectomy was the treatment of choice and was more frequently performed by open surgery (66.7%) than by arthroscopy (15.6%). Treatment options such as observation, pharmacological treatment (corticosteroids and beta blockers), and TKA were rarely recommended . Biopsy prior to treatment was infrequent (9.4% of cases). The most frequent histologic type was the cavernous type (28.1%), followed by the venous vascular malformation, capillary, arteriovenous and cavernous-capillary types.The average follow-up time was 2.7 ± 2.2 years, but the follow-up time was unreported in 36% of cases. Reported recurrence was low (3.1%), as it was the persistence of symptoms (11.4%).Synovial haemangioma have been described in various joints, but the knee is the most common . Up to 2This is the first study that performs a detailed revision of a large number of KSH (96 cases) and describes its more frequent location and clinical presentation forms. The major finding of this study is that plain radiographs and MRI image studies appear to be enough for the differential diagnosis, that will be confirmed after histology exam. Being a tumor mass of benign characteristics on image studies rarely a biopsy is indicated previous to treatment, an excisional biopsy and regional synovectomy can be performed for diagnosis and treatment purposes before diagnostic confirmation. Although open surgery has been the treatment of choice, nowadays arthroscopy seems to offer advantages in the treatment of intraarticular KSH without bone, tendon or muscle involvement. Recurrence after surgery is low (3.1%) but persistence of symptoms is more frequent (11.4%), especially in diffuse forms.In the last 20 years, only nine cases (9.4%), including ours, had localized lesions in the posterior compartment of the knee. One was an intermediate tumor  that extOur other three cases were local sessile intra-articular SH located near the superior pole of the patella, by far the most frequent location in the anterior compartment of the knee , 53–57, KSH usually occurs in children and teenagers , 8, 12, All four of our patients were male, but we found no differences in sex distribution, and the exclusively male composition of our sample is probably merely a matter of sample size. The right knee is more frequently affected than the left knee. Previous trauma appears to be rare and is often not reported. Symptoms and signs associated with SH of the knee are non-specific. Low-intensity and long-duration pain is the most frequent symptom , but it No clinical test or imaging procedures provide a definitive diagnosis, nor is there a clear algorithm for diagnosis. Plain radiographs are typically normal , 55– 57,MRI appears to be the examination of choice , 53–57. Ultrasound may , 24, 46 Diagnostic arthroscopy may be useful in localizing a synovial tumor and ruling out unrelated intra-articular lesions , 45, 47.Histological examination is essential to establish a definitive diagnosis, although it was not reported in 8.3% of reviewed cases. Haemangiomata are tumors derived from endothelial cells, and four types have been described . AlthougWe advise treating these lesions because they can cause joint destruction either by direct invasion of the surrounding tissues or by repeated haemarthrosis , 10, 31.As KSH appears as a synovial tumor mass with benign characteristics in imaging studies, an excisional biopsy and regional synovectomy can be performed until free resection margins are obtained for diagnosis and treatment purposes before diagnostic confirmation. Biopsy prior to treatment is rarely reported 9.4%) but should be indicated if there is doubt regarding the benignity of the tumor based on imaging examinations. There is controversy as to whether open or arthroscopic surgery is better. Most patients have been treated by open surgery because it has been argued that it allows a better view of the full extent of the lesion, mainly when it is located within the Hoffa fat pad, and therefore leads to a lower recurrence rate % but sho, 50, 55.Some authors recommend arthroscopic resection for localized intra-articular KSH , 59, 60.We note several limitations of our study. First, some clinical data were missing in our retrospective clinical review, although key data were available in the records. Second, the literature search was performed only in PubMed and was limited to English, French or Spanish; thus, there is likely additional literature on the subject. However, given the long period over which the papers were published and the large number of cases identified, the included studies are likely representative of the literature. We are unaware of any other such reviews at this level of detail.It is clinically relevant to know the frequent and unusual locations of KSH, its multiple clinical presentations, the real value of image examinations and the pros and cons of arthroscopic vs. open surgery to be able to make informed decisions on the need for invasive image examinations, previous biopsy or arthroscopic vs. open surgery in light of the evidence.In conclusion, KSH is a rare tumor. The superior pole of the patella is the most frequent location and only five intra-articular reported cases (including ours) in the posterior knee compartment. SH should be considered in the differential diagnosis of long-term knee pain in adults, particularly if there is no history of previous trauma. Plain radiography is usually normal. MRI is the imaging test of choice to localize a synovial mass, but the images are diagnostically inconclusive. MRA may be useful in diffuse or extended cases. The rare phenomenon of bone erosion, first time described in the patella, have to be bear in mind. Histological examination is necessary for definitive diagnosis. Biopsy prior to treatment should only be indicated if there is doubt regarding the benignity of the tumor. We believe arthroscopic resection should be the surgical technique of choice for treating localized intra-articular lesions or those in difficult areas such as the posterior compartment. We recommend open surgery for diffuse tumors and those that are localized in juxta-articular and intermediate regions with tendon, muscle or bone extension. Complete resection of the synovial lesion and the surrounding affected tissues is important to relieve symptoms and prevent recurrence.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.JH-H participated in the design of the study, participated in revision of clinical cases and search and revision of bibliography, and interpretation of the data and in drafting the manuscript. JM-B participated in revision of clinical cases and revision of bibliography. EG-O has participated in search and revision of bibliography. FC-S has been involved in search and revision of bibliography. SL-M has participated in search and revision of bibliography, interpretation of data, and drafting the manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Neuroendocrine tumors (NETs) are rare entities. Most common among them are gastroenteropancreatic neuroendocrine tumors (GEP-NETs) and pulmonary NETs. Most of them are indolent in nature. Colonic NETs are rare among GEP-NETs and mostly present with large size and with metastasis. Emergency presentation with hematochezia is rare in colonic NETs. This case report discusses a rare emergency presentation of colonic NETs and highlights their poor biological nature. Neuroendocrine tumors (NETs) are generally rare and associated with low virulent behavior ,2. AmongA 65-year-old male with good performance status  presented to the emergency department with episodes of haematochezia for two days. At the time of presentation, he had pallor and his vitals were within normal range except for tachycardia of 110 bpm. On abdominal examination, there was a vague lump in the left upper quadrant. On digital rectal examination, no growth was felt and the finger was stained with blood. The patient had already undergone a contrast-enhanced CT abdomen and thorax at the primary healthcare center, which was suggestive of transverse colon mass without any evidence of distant metastasis  level was 4 ng/ml, which was within the normal range (<5 ng/ml). The patient was resuscitated with six units of packed red blood cells along with fresh frozen plasma and platelets. After admission, as there was no further episode of bleeding and after achieving hemoglobin of 10.4 gm/dL, for tissue biopsy, the patient was taken for colonoscopy and also to rule out any synchronous lesions. Colonoscopy was suggestive of transverse colon mass and the scope was not further negotiable with no distal synchronous lesion. Although there was no new episode of haematochezia and the patient's vitals were stable, given the high risk of rebleeding and related complications, we decided to take the patient for urgent colectomy. For the same reason, we did not wait for the biopsy report. On exploration, we found a proximal transverse colon mass. Extended right hemicolectomy with handsewn side-to-side ileocolic anastomosis was performed Figures , 3 as thThe postoperative period was uneventful and the patient was discharged on postoperative day eight. The histopathological report was suggestive of a high-grade NET Figure  with staNETs are a rare condition with low malignant potential that arise from neuroendocrine cells, which primarily originate from neural crest cells. The most commonly involved sites are the gut, lungs, pancreas, thymus, testis, and ovaries . In CRC,According to the WHO criteria, all GI NETs are categorized into three grades based on the degree of differentiation and histology: grade 1 , grade 2 , and grade 3  . Less siColonic NECs are rare entities and associated with poor disease biology; they can present with hematochezia leading to emergency presentations. Although the surgical management is similar to that of adenocarcinoma of the colon, due to its aggressive nature and rarity, the therapeutic strategy depends on early detection along with timely surgical intervention followed by well-established adjuvant therapy, which is possible with a multidisciplinary team approach. As there has been an increase in the incidence of such cases, it is the need of the hour to study and establish adjuvant therapy protocols for these notorious tumors.


We report the finding of a rare diagnosis of a duodenal duplication cyst (DDC) resulting in malignancy. Duplication cysts are rare entities in itself but less than 5% arise from duodenum. Our case represents a rare case, but high suspicion and early resection may have prevented associated complications. Duplication cysts are rare congenital malformation of the gastrointestinal tract with an estimated occurrence of one in 100,000 live births. The exact etiology behind the occurrence of these cysts is unknown. Several theories have been proposed to explain their existence . When shA 69-year-old Caucasian woman presented to the ER with fevers and abdominal pain. A CT scan was performed, and identified diverticulitis with a small perforation Figure . IncidenAdditional imaging including a positron emission tomography (PET) scan, MRI of the abdomen and testing by endoscopic ultrasound (EUS) did not reveal any site of primary in the liver, pancreas. EUS was performed and found the tumor to be adjacent to the duodenum, but again, no primary was noted on this study. A repeat biopsy of lymph node was performed and additional testing was done, including tissue analysis from the CancerTypeID© test . This test suggested a high probability that this was a pancreaticobiliary tumor. Given the unusual nature of presentation and absence of a primary, it was decided to attempt surgical exploration. She then underwent an exploratory laparotomy at which point, it appears that the tumor had arisen in a previously unsuspected duodenal duplication cyst. She underwent resection of the porta hepatic lymph node, and Whipple's procedure with pylorus preservation. Multiple (16/21) lymph nodes were involved. Post-operatively alpha-fetoprotein (AFP) levels decreased down to 45, but did not normalize. Given concerns that she may have residual cancer, she was offered postoperative chemotherapy. She received therapy with FOLFOX, and had multiple toxicities so therapy was stopped after three cycles. With this therapy, her AFP finally decreased down to the normal range. This suggested that she perhaps indeed did have residual disease post operatively, and had benefited from cytro-duction from surgery. She was therefore treated with a combination of radiation and infusional 5-FU with curative intent to the tumor field. Within two months after completion of radiation, she developed two liver metastases with a corresponding increase in her AFP. No biopsy was done, as these lesions were very definitively noted to be malignant on CT scans and also were noted to be PET avid. Next Generation Sequencing of the surgical sample revealed that she had an inactivating mutation in the BRIP gene (exon 10 rearrangement). There is data that mutations of this gene may lead to sensitivity to PARP inhibitors, so the patient was initiated on Olaparib therapy. She had a complete response to therapy, which has been maintained for more than 14 months and is ongoing.The majority of duodenal duplication cysts (DDCs) are diagnosed in infancy or early childhood. Rarely they may remain asymptomatic and undiagnosed until adulthood and, depending upon their location, size and type, may present as bowel obstruction, bile duct obstruction, pancreatitis, bleeding or intussusception. Although DDCs are generally benign, malignant transformations inside them have already been reported -4. The fDDCs may present as a vague, often misleading clinical picture, thereby posing a diagnostic challenge for the clinician. Multiple case reports have reported an array of symptoms, including abdominal pain, nausea, anorexia, cachexia along with a clinical picture of recurrent acute pancreatitis or gastric outlet obstruction, or gastrointestinal bleeding, hemorrhagic ascites. Larger sized cysts result in compressive symptoms including malnutrition and weight loss, and may also result in compression of the pancreatic duct causing recurrent pancreatitis episodes with an unidentifiable cause -7.The best diagnostic tools available are endoscopic ultrasound (EUS) which helps differentiate the duplication cyst by the “gut signature” or “the double-layer wall” sign of its wall. Computed tomography of the abdomen and pelvis may help delineating the lesion and size as well. For further differentiation, a technetium scan may be helpful as well in detecting the heterotopic gastric mucosa in cases that present with bleeding . Other dThe appropriate treatment depends on the location, size, and type of the duodenal cyst, including taking into account patient comorbidities. Complete excision is indicated when the cyst is small and is in no relation with the pancreaticobiliary tree, or when the cyst is complicated with an ulcer, either in the cystic mucosa. Excision is considered the procedure of choice to avoid malignant transformation, as depicted in our patient.Our case report emphasizes a sudden change in bowel habits, early diagnosis and immediate complete resection of DDS to prevent malignant transformation and extensive surgery. Early treatment may have resulted in a conservative surgical approach, reduced health care cost, and shorter length of hospitalization.Duplication cysts in the duodenum are extremely rare, and intestinal duplication cysts are rarely diagnosed in adults. Preoperative diagnosis of primary malignancy in DDC is challenging. A high degree of suspicion is required to diagnose it. However, whenever DDC is suspected, its immediate resection is justified. We report a rare case of duodenal duplication cyst, resulting in malignant transformation which may have been prevented with early resection.


Spontaneous intracranial hypotension is a rare clinical entity caused in most cases by a cerebrospinal fluid leak occurring at the level of the spinal cord. Cranial dural leaks have been previously reported as a cause of orthostatic headaches but, as opposed to spinal dural leaks, were not associated with other findings characteristic of spontaneous intracranial hypotension. We present the case of a male admitted for severe orthostatic headache. The patient had a history of intermittent postural headaches, dizziness, and symptoms consistent with post-nasal drip, which appeared several years after head trauma. Brain imaging showed signs consistent with intracranial hypotension: bilateral hygromas, subarachnoid hemorrhage, superficial siderosis, diffuse contrast enhancement of the pachymeninges, and superior sagittal sinus engorgement. No spinal leak could be identified by magnetic resonance imaging, and the patient had a rapid remission of symptoms with conservative management. Further work-up identified an old temporal bone fracture which created a route of egress between the posterior fossa and the mastoid cells. Otorhinolaryngology examination showed pulsatile bloody discharge and liquorrhea at the level of the left pharyngeal opening of the Eustachian tube. The orthostatic character of the headache, as well as the brain imaging findings, were consistent with intracranial hypotension syndrome caused by a cranial dural leak. Clinical signs and imaging findings consistent with the diagnosis of apparently “spontaneous” intracranial hypotension should prompt the search for a cranial dural leak if a spinal leak is not identified. Orthostatic headache is the hallmark finding of spontaneous intracranial hypotension (SIH). The headache is usually severe, bilateral, appears a few moments after standing up, and improves by lying down. It may be associated with nausea, vomiting, neck pain, vertigo, tinnitus, and other signs and symptoms related to dysfunction of cranial nerves .Leakage of cerebrospinal fluid (CSF) through a defect in the cervicothoracic thecal sac is the most frequently identified cause of SIH . In 44–5We report the case of a 76-year-old Caucasian man who presented to our hospital for acute onset epistaxis associated with headache and dizziness. He had a history of arterial hypertension, left bundle branch block, dyslipidemia and heart failure, for which he was taking antihypertensive and antiplatelet drugs. He also reported a traumatic head injury in the context of a road traffic accident seventeen years before, after which he had otorhinolaryngology (ENT) surgery twice: the first time for traumatic fracture of the nasal pyramid and second time to correct a nasal septal defect.Brain computed tomography (CT) showed small, subtle areas of subarachnoid hemorrhage located in both frontal and left temporal lobes and bilateral 5mm hygromas . A subseGeneral clinical examination was unremarkable. Blood pressure was 155/80 mmHg and his heart rate was regular at 80 beats per minute (bpm). Neurological examination showed a left-sided peripheral facial palsy (sequela from his car accident) without any other pathological findings. Headache and dizziness occurred within minutes of adopting an orthostatic position and significantly improved when lying supine. His epistaxis resolved within hours from the onset and the otorhinolaryngology exam showed evidence of hemotympanum, considered to be a consequence of epistaxis. A thorough anamnesis revealed that he had a few episodes of isolated headache attacks sometimes associated with dizziness over the previous years, all presenting a strictly orthostatic character. These episodes were sometimes associated with symptomatic episodes consistent with post-nasal drip.The brain magnetic resonance imaging (MRI) was consistent with the diagnosis of SIH, showing bilateral subdural hygromas, diffuse contrast enhancement of the pachymeninges, superior sagittal sinus engorgement, and small areas of cortical and subarachnoid hemorrhage in the left temporal and right frontal regions. Susceptibility weighted imaging showed evidence of superficial siderosis at the level of the frontal lobes bilaterally, which could be due to past episodes of subarachnoid hemorrhage or a consequence of his past head trauma .The patient was confined to bed rest and received an increased hydration regime, generous caffeine intake, and low dose dexamethasone (8 mg/day). Two spinal cord MRI scans aiming to identify a potential CSF leak were performed on separate days, but no signs of dural leakage were identified. His headache and dizziness progressively improved, but two days after admission, he complained of left ear fullness. A high-resolution CT scan of the temporal bone showed an oblique fracture involving the left temporal bone and communicating with the posterior superior marginal mastoid cell. Liquid density was visualized in the left mastoid cells, and therefore, a new brain MRI with fine T2-weighted sequences was performed, confirming this finding .Repeated and more elaborate ENT examination showed left hemotympanum with intermittent pulsatile discharge from the pharyngeal opening of the Eustachian tube, and nasal endoscopy revealed oto-liquorrhea. However, repeated nasal endoscopy performed two days later, intending to obtain a sample for b2-transferrin testing, was unsuccessful because the discharge had stopped. Besides ear fullness, the patient remained symptom-free and was discharged home. He was further referred for ENT and neurosurgical follow–up in an experienced center for further assessment of the opportunity of surgical exploration aimed to clearly localize and seal the cranial dural defect and the temporal bone fracture. He did not report any other symptoms in the first month after discharge and refused surgery proposed by a joint neurosurgical-otorhinolaryngology team.This is a rare case of intracranial hypotension syndrome associated with a cranial dural leak. We hypothesize that our patient developed intermittent slow-flow CSF leak, which led to atypical symptoms and an episodic occurrence of orthostatic headaches after the road accident. Episodic and intermittent orthostatic headaches have been described in patients with slow-flow CSF leaks before , 4. MoreEar fullness, symptoms of post-nasal drip (due to liquorrhea) as well as headaches have been reported in patients with temporal bone fractures, and these symptoms might sometimes be present for years before diagnosis . It is eThe first otorhinolaryngology examination showed evidence of haemotympanum, which was interpreted in the context of epistaxis. The bleeding gradually abated, and clear liquorrhea could be seen at a follow-up examination. Epistaxis has been previously reported in patients with headaches, and the presumed mechanism might be an aberrant activation of the trigeminovascular system or a transient increase of venous pressure, which could also explain bleeding from superficial bridging veins with subsequent subarachnoid hemorrhage .Remission of the patient’s symptoms with conservative therapy was concordant with the abating of oto-liquorrhea on follow-up ENT examination and is in general line with his clinical history of infrequent spells of orthostatic headache and dizziness. We considered this clinical course suggestive of the intermittent nature of the dural leakage. Dynamic optic nerve sheath diameter measurements by ultrasound can be used to prove the opening or closing of dural fistulas responsible for CSF leaks, but it could not be performed at that moment in our hospital .Although we did not perform dynamic myelography to rule out a spinal CSF leak, the brain MRI changes together with the findings of the otorhinolaryngology examination strongly point towards a cranial CSF leak leading to intracranial hypotension syndrome in our patient.We report the case of a patient with intermittent cranial cerebrospinal fluid leak and intracranial hypotension syndrome. The traumatic nature of his old temporal bone fracture makes the interpretation of this case as “spontaneous” intracranial hypotension disputable. However, the onset of symptoms years after the head trauma and their paroxysmal nature would have classified this syndrome as “spontaneous” if no cranial dural leak would have been found. Therefore, we believe that cranial cerebrospinal fluid leaks should be searched for in selected patients with spontaneous intracranial hypotension syndrome.This case report was written in accordance with the legal and ethical requirements of the University Emergency Hospital Bucharest, Romania.Written informed consent was obtained from the patient.Further data are available from the corresponding author on reasonable request.The authors declare that there is no conflict of interest.


The gastrointestinal location is very rare being the stomach the most affected organ. However, gastric schwannoma represents only 0,2% of all gastric tumors. Usually they have benign prognosis, but malignant transformation has been described. Symptoms are usually vauge and depend on the localizationIn this case report we present a patient with and antral submucosal gastric tumor that recived totally laparoscopic resection. A 56-year-old woman, with hypertension, hypothyroidism, clinical history of six months of epigastric pain, without vomits, dysphagia, jaundice, fever or weight loss, and no specific founding at physical examination, presented for consultation. The upper endoscopy shown an anterior antral submucosal tumor of 5 cm, with no ulceration . The abdominopelvic CT scan showed an anterior antral submucosal exophytic solid tumor of 5.2x4.8 cm, without celomic, lymphatic or visceral metastasis .With the diagnosis of an antral GIST, the patient underwent laparoscopic exploration and resection of the lesion. A 15-mmHg pneumoperitoneum and four trocars (one of 5 mm and three of 12 mm) were used. A 6 cm exophytic gastric tumor was found in the same position describe by the endoscopy and CT. Dissection of the grater curvature was performed to access the lesser omentum and posterior wall of the stomach. After passing a 36F bogie throw the pylorus, a total resection of the tumor was performed with two staplers , and neurogenic tumors . In the neurogenic group 91% are scwhannomas, and the most frequents locations are stomach (60-70%) and colorectal. Gastric schwannoma represents 5% of nonepithelial gastric tumorsth decade of life. Usually they are asymptomatic, but when they require surgery, 42-88% have abdominal pain, 25-65% gastrointestinal bleeding and 10-25% weight loss. The localization is usually at the body (50%) or antrum (32%) and the size is less than 10 cm in 88% of cases, and less than 5 cm in 48% of them. In our case report, this demographic, clinical and anatomical characteristics are present. As described by BrunetonEndoscopic and tomographic study are the first line approach, but they cannot differentiate accurately between GIST, myogenic and neurogenic tumors,,,,,,Surgical therapy is the cure in most cases without any other adjuvant therapy. The surgical approach depends on the tumor size, localization, surgeon experience and preference. The most important thing is to achieve negative margins, and to do so, resections could be from an economic enucleation and suture, to a partial or total gastrectomy. Because of the nature and frequency of this tumor, the literature available consist in case reportsThe pathologic examination is the key to differentiate between all subtypes of submucosal gastric tumors. Before the advances in immunohistochemical stain, the majority of this tumors were diagnosed as leiomyoma and leiomyosarcoma, but now with the advent of c-Kit, DOG1 and CD34 which are usually positive in GIST, SMA and desmin positive in leiomyoma, S100 positive in schwannoma.,,,,After free margin surgery the prognosis is excellent, some argue that the risk of malignant transformation is theorical, and that histologic findings such as tumor size and mitotic rate have no prognostic significance


Carcinoid tumors are one of the most common types of small intestinal neuroendocrine tumors (SI-NETs). However, SI-NETs that manifest as subacute intestinal obstruction are extremely rare. The annual occurrence rate of jejuno-ileal NETs is 0.28-0.8 per 100,000 people. In this report, we describe a case of subacute intestinal obstruction due to a mid-ileal stricture. The patient underwent laparotomy after evaluation and investigation. Mid-ileal growth was noted, and small bowel resection was performed with primary end-to-end anastomosis. Postoperative histopathology revealed the growth to be a well-differentiated NET. Neuroendocrine tumors (NETs) are slow-growing, heterogeneous tumors that develop from neuroendocrine cells distributed all over the body. The lung, small intestine, rectum, and anus are the prominent organs affected by them . PrimaryCarcinoid tumors are the most common type of SI-NETs, even though they are a rare condition. Carcinoid tumors have an annual incidence of four to five cases per 100,000 individuals . UsuallyWe present a case of multiple SI-NETs with clinical and radiological findings of acute intestinal obstruction treated at King Abdulaziz University Hospital (KAUH), Jeddah.The patient was a 75-year-old male with a medical history of diabetes mellitus, hypertension, and benign prostatic hyperplasia. He presented to the Emergency Department (ED) complaining of recurrent, non-radiating paraumbilical colicky pain for six months. The pain was exacerbated by lying and relieved by sitting and bowel movements. It was associated with vomiting, abdominal distension, and constipation with alternating diarrhea. The patient had a history of hemorrhoidectomy 15 years ago. The abdominal examination was unremarkable, except for the presence of significant abdominal distention and mild umbilical tenderness.An abdominal CT scan with IV contrast was done, which showed a dilatation in the small bowel reaching up to 4.8 centimeters with multiple air-fluid levels and transition zone at the distal ileal loops. There was circumferential mural thickening of the ileal loops in the right lower quadrant with surrounding fat strandings and prominent mesenteric lymph nodes. The rest of the ileal loops and the large bowel was collapsed. The constellation of findings was assumed to represent a neoplastic or inflammatory process. In addition, a plain abdominal radiograph was done, showing dilated small bowel loops, suggestive of small bowel obstruction Figure . The CT The patient underwent laparoscopic exploration, which revealed two mid ileal masses. The proximal part related to the masses was dilated, where the distal part was collapsed. The mid ileal masses were attached to the small bowel mesentery, and hence the procedure was converted to the midline laparotomy. The affected bowel was found 2.5 meters distal to the duodenojejunal (DJ) junction. Oncological resection of the masses was excluded to avoid superior mesenteric artery injury. Therefore, a small bowel resection accompanied by multiple intermediate size lymph node resection was made. At that time, side-to-side stapled anastomosis was also done. Regarding histopathology, the measurement of the obstruction site was 11 x 5 cm. There were nine well-circumscribed, firm submucosal nodules, and the largest nodule measured 2.2 cm in its maximum dimension. The mesenteric lymph node specimens consisted of multiple fragments of fibrofatty tissue measuring 8 x 4 x 3 cm in aggregate  cells, which are multipotent stem cells that migrate from the neural crest cells at the crypts of Lieberkuhn base to the gut ectoderm ,8. The oHowever, in primary tumors, the patient may be asymptomatic, and the tumor is slow-growing and relatively small; other manifestations include abdominal pain, diarrhea, weight loss, and bleeding. Moreover, intestinal obstruction caused by small bowel tumors is relatively rare. In fact, it may represent only 15-25 of the total cases . In spitDiagnosis of NETs in the early stages is challenging, and it may be accomplished through the grade of the mitotic count and Ki-67 index, which is considered the best marker for the cell proliferation state ,11. The According to multiple studies of research and observations, the most common site of metastasis for GI carcinoid tumors is the liver . As repoRegarding the treatment of SI-NETs, the stages play a crucial role. In stages I, II, and III, radical resection is performed to achieve the R0 stage. Besides, in the case of stage IV metastatic SI-NETs, the surgical approach must be preferred to achieve the least risk of local complications. In palliative settings, medical therapy is mandatory . The goaThe prognosis of SI-NETs depends on both stage and grade. According to the WHO classification of 2010, the five-year survival rate for stages I and II is 100%, while that for stage III is 97.1%, and for stage IV, it is 84.8%. According to grading, patients with G1 SI-NETs have a 93.8% five-year survival rate, while those with G2 have 83.0%, and patients with the relatively rare G3 have only about 50% [We presented the case of a patient with SI-NETs, particularly in the mid-ilium, which manifested as intestinal obstruction. Our case highlights the importance of keeping a high index of suspicion for NETs in patients with small bowel obstruction, especially if the risk factors we mentioned above are present. Finally, the treatment approach and management that were employed in this case represent the optimum approach toward any case where SI-NETs are suspected.


Intraneural ganglion cysts are a rare occurrence. They are most commonly found originating from the common peroneal nerve but are also frequently reported on the radial, ulnar, median, sciatic, tibial, and posterior interosseous nerves. A typical clinical presentation is posterior knee and calf pain resulting from tibial neuropathy with preferential degeneration of the popliteus muscle. Symptoms include pain, paresthesias, and decreased strength that originates in the knee and commonly extends to the plantar surface of the foot. These findings can be mistaken for lumbar neuropathies and compression of the sacral nerve roots. Differential diagnosis includes peripheral nerve sheath tumors, Baker’s cysts, extraneural ganglion cysts, and atypical vascular or lymphatic malformations. In this case report, the patient was a 61-year-old male, previously in good health, who presented with progressive pain in his medial left hamstring as well as weakness in left foot plantar flexion and paresthesias in the plantar aspect of his left foot. He first noticed impairments with his ability to push off with his left foot when running. His electromyogram (EMG) was abnormal and subsequent MRI of the left leg showed a complex intraneural ganglion cyst arising from the tibiofibular joint and ascending into the tibial nerve. He underwent indirect decompression through joint resection. Unfortunately, he did not have clinical improvement on one-year follow-up. Overall, symptomatic treatment of intraneural ganglion cyst includes decompression, surgical excision, or minimally invasive decompression by percutaneous aspiration of the ganglion under ultrasound guidance. Ganglion cysts are non-neoplastic synovial or gelatinous cysts primarily consisting of hyaluronic acid, which originate within the epineurium of peripheral nerves. Although most ganglion cysts are asymptomatic, cysts arising in the peripheral nerves can lead to pain, paresthesias, pressure, and decreased mobility -2. CystsThis study was conducted in accordance with the ethical standards of the Institutional Review Board of our institution, Des Moines University College of Osteopathic Medicine.A 61-year-old male patient presented with progressive pain in his medial left hamstring as well as weakness with left-sided foot drop and paresthesias in the plantar aspect of his foot. Symptoms first presented as impairments in his ability to push off with his left foot during moderate exercise. He did not present with radicular symptoms but did have a previous X-ray of the lumbar spine, which showed no signs of stenosis or compression.Upon initial physical examination, the patient was found to have no tenderness to palpation of his lumbar spine. Tenderness was appreciated in the medial left glute and left posterior medial thigh down to the knee. No reproducible pain was noted below the knee, and no obvious foot deformity, erythema, or edema were present. He did endorse decreased sensation in the distal third of the plantar foot including the first metatarsal. The patient was unable to flex or extend his left first metatarsal. Left Achilles tendon reflex was 0/5, with a contralateral reflex of 5/5.The patient was subsequently evaluated by a physical medicine and rehabilitation specialist, who noted diminished left S1 reflex. He was able to stand in plantarflexion on the right, but was unable to flex to full height on the left. Also, weakness of the left flexor hallucis longus and digitorum longus with absent sensation on the plantar surface of the first metatarsal to pinprick and light touch was noted. He was then referred for osteopathic manipulative therapy (OMT). Initial treatments with OMT did offer some relief, but his symptoms persisted. An electromyogram (EMG) was performed, which showed evidence of peroneal nerve injury in the absence of a superficial peroneal sensory response, temporal dispersion when stimulating across the knee, and tibial nerve injury.Magnetic resonance imaging (MRI) of the lumbar spine was obtained and showed no evidence of disc herniation, spinal stenosis, or foraminal stenosis. Subsequent MRI of the left knee showed markedly complex cysts at the level of the superior tibiofibular joint. The cysts appeared to connect to the posteromedial superior tibiofibular joint and descend in close proximity to the anterior tibial artery and anterior tibial veins, as appears on the axial and coronal T2-weighted fat-saturated images Figures , 2. SagiGiven the findings, the patient was further referred for both orthopedic and neurosurgical consultations. Surgical intervention was discussed, and options included cyst decompression, joint connection-disconnection, and joint resection. The patient opted for indirect decompression through joint resection. He subsequently underwent arthrotomy of the proximal tibiofibular joint with excision of the medial aspect of the proximal fibula including the articular surface of the proximal fibula. The lateral aspect of the articular surface of the tibia was also removed. Resection of the left superior tibiofibular joint was performed with reconstruction of the proximal tibiofibular joint to increase stability. Exploration and mobilization of the left peroneal nerve at the fibular neck region and drainage of anterior compartment cyst were also performed.On postoperative three-month follow-up, the patient noted improvement of motor strength with minimal footdrop. There was no improvement of sensation on the distal plantar surface of the left foot. Denervation of the posterior tibialis and popliteus muscle was still present. On 12-month follow-up, he was noted to have no additional improvement of motor strength. No improvement of sensation or innervation of the posterior tibialis or popliteus muscles.Intraneural ganglion cysts are a rare occurrence. They are typically found on the radial, ulnar, median, tibial, and posterior interosseous nerves, most frequently involving the common peroneal nerve . Common The origin of ganglion cysts is mostly unknown, but many theories exist. A few theories have tried to explain the origin of ganglion cyst citing chronic stress or joint stress causing a rent in the capsular space or mucoid degeneration leading to fluid accumulation . CurrentFor the diagnostic process, it has been shown that MRI and ultrasonography are the most useful tools to identify intraneural ganglion cysts. On ultrasonography, an intraneural ganglion cyst will appear as a large, well-circumscribed hypoechogenic lesion. On MRI, it appears as a multilobulated lesion with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, oriented longitudinally along the course of the affected nerve . MRI finTreatment of an intraneural ganglion cyst includes surgical excision, decompression, or minimally invasive decompression through percutaneous aspiration of the ganglion under ultrasound guidance ,8, althoWe presented a rare case of intraneural ganglion cyst arising from the tibiofibular joint in a healthy 61-year-old male who was previously in good health and ran frequently. He underwent indirect decompression through joint resection. Unfortunately, he did not have clinical improvement on one-year follow-up. Overall, symptomatic treatment of intraneural ganglion cyst includes decompression, surgical excision, or minimally invasive decompression by percutaneous aspiration of the ganglion under ultrasound guidance.


Peritoneal lymphomatosis (PL) is defined as intraperitoneal dissemination of lymphoma. Although rare, it is associated with high-grade lymphomas and can be easily mistaken for peritoneal carcinomatosis (PC) on imaging, which is a common condition associated with gastrointestinal and gynecological malignancies. Both PL and PC share similar radiographic features, however, they differ considerably in terms of prognosis and management. We present a case of a 58-year-old male with abdominal distention and ascites, initially reported as having PC on imaging. A subsequent peritoneal biopsy revealed PL secondary to a low-grade follicular lymphoma. Since PL responds well to chemotherapy, its prompt diagnosis and differentiation from PC helps avoid unnecessary surgery. Neoplasms that involve the peritoneal cavity are secondary to the spread of primary malignancies from elsewhere. The peritoneum may become affected by three malignant cell lines: epithelial (carcinomatosis), mesenchymal (sarcomatosis), or lymphoid (lymphomatosis) .Around 40% of lymphomas may have extra-nodal involvement (outside of the lymph nodes) and can involve almost any organ . PeritonOn imaging, PL can be easily mistaken for peritoneal carcinomatosis (PC), a common condition associated with disseminated malignancies, including gastrointestinal and gynecological malignancies. Peritoneal lymphomatosis responds well to chemotherapy and hence early diagnosis and differentiation from PC are imperative to avoid surgery. We present our experience of a patient who presented to our institution with abdominal pain and distention. Imaging revealed a pancreatic head mass with findings concerning for peritoneal carcinomatosis, however, a subsequent peritoneal biopsy revealed PL secondary to a low-grade follicular lymphoma.A 58-year-old male with no prior medical problems, who was leading an active lifestyle, presented to our emergency department with progressively worsening shortness of breath and abdominal distension for one month. His breathlessness had gradually worsened to the point where he felt winded even upon getting out of bed. He also reported intermittent abdominal discomfort associated with a decrease in appetite for a similar duration. Initially, the patient sought medical attention from his primary care physician and was started on treatment for community-acquired pneumonia without any significant improvement. He denied any fever, chills, night sweats, or weight loss. Review of systems was unremarkable including bowel habits, which were regular.The patient had been a nonsmoker his entire life but had secondhand exposure to smoking from his wife. His alcohol consumption was limited only to social occasions and he denied any illicit drug use. He had no known allergies, nor did he take any daily medications. Family history was not significant for any malignancies. No recent travel was reported and by occupation, he was a tailor.On examination, the patient was found lying comfortably in bed. Vital signs were significant for a pulse rate of 106 beats per minute, respiratory rate of 17 breaths per minute, and oxygen saturation of 98% on 2 liters of oxygen via nasal cannula. He was normotensive and afebrile. No cyanosis, clubbing, rash, or lymphadenopathy was noted. Respiratory examination revealed decreased air entry and dullness on percussion on the right side of the chest. His abdomen was distended, non-tender with a palpable liver and spleen, and he was noted to have shifting dullness. The rest of the examination was within normal limits. Laboratory data are summarized in Table Upon admission, a chest x-ray showed a near-complete opacity of the right hemithorax secondary to a large pleural effusion Figure . A bedsiPeritoneal lymphomatosis and carcinomatosis are among a host of pathologies that may involve the peritoneum. These include peritoneal sarcomatosis , tuberculosis, and pseudomyxoma peritonei .Peritoneal involvement from the secondary spread of tumors is much more common than a primary peritoneal tumor. Primary tumors such as peritoneal mesothelioma and mesenteric sarcomas are rare, whereas diffuse peritoneal and omental seeding due to malignant diseases of the ovaries, colon, and stomach is a very common phenomenon . PC has Owing to its rare nature, PL receives significantly less attention than PC, and only a handful of reports are found in the literature ,8. In a Clinical manifestations of PL include abdominal distension, abdominal pain, fever, emaciation, or malaise. Smooth thickening of the peritoneum and a diffuse mass involving the peritoneal layer of the bowel due to lymphoma is reported. Peritoneal lymphomatosis can manifest as discrete nodules involving the peritoneum and as exudative ascites that is high in protein ,9,10. ThPL has common imaging (CT or MRI) features with PC including pleural effusion, ascites, anasarca, omental caking, peritoneal thickening, and serosal disease. Omental caking is the appearance of fine nodular or large bulky soft tissue deposits involving the peritoneum secondary to malignancy. The large subtype is associated with lymphomatous spread .Findings distinctive to PL include a greater burden of lymphadenopathy with a higher number of involved nodes, larger associated nodules, and more frequent hepatosplenomegaly . AnotherFluorodeoxyglucose-positron emission tomography (FDG-PET)/CT helps in better staging and monitoring the treatment response and aids in the early detection of disease recurrence. In subtle cases, the sensitivity of detection of peritoneal involvement and extranodal disease is better than that of CT. This imaging also helps in choosing an appropriate biopsy site and increases the chance of detection of early disease. Its sensitivity and specificity for detecting peritoneal seeding reach as high as 100% and 97%, respectively . Diagnosing lymphomas in effusions and/or ascites can be challenging, especially if the lymph node architecture is not available for analyses. Studies like flow cytometry and immunohistochemical staining aid in making an accurate diagnosis and help in identifying the molecular characteristics of the lymphoma and its immunophenotype, which dictate treatment decisions . NeverthThe patient described in the case initially presented with shortness of breath. Chest imaging revealed bilateral pleural effusions. The pleural fluid analysis showed elevated cholesterol and triglyceride levels, suggestive of chylothorax. The major cause of non-traumatic chylothorax is malignancy. Most cases of malignancy-associated chylothorax occur in patients with advanced disease ,17. CT sUpon further review of this patient’s CT imaging, soft tissue deposits in the anterior pelvis with peritoneal enhancement concerning for PC were present. A diagnosis of PL based on imaging can be difficult to make considering its rarity and its similarity to peritoneal carcinomatosis on imaging Figure , hence aPatients with a pancreatic mass and peritoneal metastasis are often ceded to palliative care, however, a diagnosis of lymphoma will change the management. Treatment of PL is non-surgical as dramatic improvement can be seen with chemotherapy. Upon suspicion, early communication with a specialist regarding the possibility of lymphoma may facilitate earlier diagnosis and treatment, thereby preventing unnecessary surgery.Our patient's imaging findings had a presumptive diagnosis of peritoneal carcinomatosis; however, biopsy results were consistent with a mature B cell neoplasm characterized as a grade 2 follicular lymphoma. The patient was transferred to a specialized lymphoma center for further evaluation and treatment. As peritoneal lymphomatosis responds well to chemotherapy, we advocate for earlier detection based on subtle yet distinct imaging findings, allowing for its evaluation and treatment in a timely manner, while avoiding any unnecessary abdominal surgery.


Endotracheal tumors are rare in the respiratory system. Myoepitheliomas are benign tumors, which are rarely reported in the respiratory system. Herein, we report a rare case of endotracheal myoepithelioma, which was resected by rigid bronchoscopy.A 36-year-old man, presenting with chest pain, dyspnea, stridor, and hemoptysis, was referred to our center with radiological features of near-total tracheal obstruction due to mass. Fiberoptic bronchoscopy with argon plasma coagulation and rigid bronchoscopy with grasper forceps was utilized to resect the mass. Pathological evaluation of the mass demonstrated myoepithelioma. The patient was discharged in good condition. Now, after 6 months, the patient is symptom-free with no evidence of tumor recurrence or re-growth.Despite being extremely rare, myoepithelioma should be considered a possible differential diagnosis for endotracheal tumors. Fiberoptic and rigid bronchoscopy management is an effective method for the resection of endotracheal tumors. Myoepitheliomas are benign neoplasms derived from myoepithelial cells accounting for 1–1.5% of salivary gland tumors  in 1943,Tracheal tumors include less than 0.2% of all tumors in the respiratory tract , while eHerein, we report a case of endotracheal myoepithelioma, resected with fiberoptic bronchoscopy with argon plasma coagulation and rigid bronchoscopy with grasper forceps. We also reviewed the literature with regard to the myoepitheliomas of the trachea and surgical interventions, including rigid bronchoscopy, to excise endotracheal tumors.The patient is a 36-year-old man without any significant past medical history, who came to our center due to dyspnea, chest pain, stridor, and hemoptysis. His symptoms had started about 8 months before his admission with dyspnea and stridor after exercise. The initial symptoms deteriorated over time, till 10 days before admission; he developed chest pain and hemoptysis, which led to his admission to the local hospital. Computed tomography (CT) scan, lung spirometry, and bronchoscopy were performed, respectively, which revealed a large tracheal mass in the middle part of the trachea. No biopsy was taken at that time due to a shortage of necessary equipment in the local hospital. The patient was referred to our center for further investigation and treatment.Upon his arrival, the patient was stable, and the only significant finding in his physical examination was mild stridor in lung auscultation. A high-resolution CT scan was done, which revealed a tracheal mass that nearly completely obstructed the airway Fig. . Then, tThe patient was taken to the operating room for general anesthesia; then, an endotracheal tube exchanger, connected to jet ventilation, was inserted for proper ventilation. Surgical resection was planned with a bronchoscope. First, to control the bleeding, flexible fiberoptic bronchoscopy with argon plasma coagulation was utilized for partial resection of the tumor with laryngeal mask airway assist Fig. .Fig. 3UtAfterward, for complete resection of the remaining mass, since the endotracheal mass was exceptionally large and interfered with the patient’s breathing, rigid bronchoscope no. 8.5 was inserted to remove the mass. By utilizing grasper forceps and the bevel of the rigid bronchoscope, debulking and the resection of the tumor were attempted. After crushing the base of the mass, again, fiberoptic bronchoscopy with argon plasma coagulation was used via the rigid bronchoscope. In the same way, multiple attempts by using forceps graspers and fiberoptic bronchoscopy, and in the meanwhile, utilizing cautery and suction with the help of rigid bronchoscope, resulted in successful complete resection of the tumor with good hemostasis and bleeding control. Also, the sample of the mass was sent for pathology evaluation.After the surgery, the patient was transferred to the intensive care unit for postoperative recovery, which was uneventful. In the follow-up, a CT scan and bronchoscopy were performed 1 month following the surgery, which showed no remnants of tumor remaining or regrowth with no bleeding or complications Fig. . Also, hThe gross description of the sample showed multiple fragments of tissue with creamy gray color and soft consistency, measuring 2X2 cm. Histopathologic sections showed solid, myxoid and acinar patterns of myoepithelial cells with clear and plasmacytoid morphology, as well as a few ductal differentiations. Stroma showed mucoid and hyaline material. Also, no capsule was identified Fig. . FurtherTo the best of our knowledge, to date, only four cases of benign myoepitheliomas in the trachea have been reported in the literature, excluding our case –10. GeneRegarding the resection of tracheal tumors, bronchoscopy can be a valuable tool , 12. Hermyoepithelioma has been used for both benign and malignant neoplasms in the literature [benign myoepithelioma has been suggested to differentiate benign neoplasms from malignant ones [Although myoepithelioma is defined as the benign neoplasm of myoepithelial cells, and on the other hand, myoepithelial carcinoma and malignant myoepithelioma are defined as malignant , myoepitterature . Therefoant ones .Since Strickler et al.  presenteFor benign myoepitheliomas, a risk–benefit evaluation, like other nodules, is needed based on the clinical presentations, age, change in the lesion size, etc., to determine the need for treatment . RegardiAn important differential diagnosis for benign myoepithelioma is its malignant form . In soft tissue, atypia has been reported to be the only predictor for the malignant behavior of myoepithelial tumors; besides, high mitotic rates and necrosis are commonly seen in myoepithelial carcinoma . In addiIn conclusion, myoepithelioma of the trachea is reported extremely rare in the literature; however, it should be considered a differential diagnosis for respiratory tract tumors. For complete resection of endotracheal masses, fiberoptic bronchoscopy with argon plasma coagulation and rigid bronchoscopy with grasper forceps is an effective procedure.


Pilomatrixoma, or calcifying epithelioma of Malherbe, is a benign tumor with differentiation toward the hair matrix cells and is one of childhood's most common epithelial tumors. Bullous pilomatrixoma has an extremely low incidence of occurrence, usually appears in the upper extremities, and is frequently associated with trauma. We report the case of a bullous pilomatrixoma in a patient with a rapid-growing neoformation one month after receiving a coronavirus disease 2019 (COVID-19) vaccine in his left upper arm, and we discuss whether the bullous appearance is part of the biology of the tumor or a secondary anetoderma. Pilomatrixoma is a benign tumor with differentiation toward the hair matrix cells that presents as a slow-growing dermal mass. Multiple variants have been reported, including bullous, anetodermal, perforating, and giant . BullousCoronavirus disease 2019 (COVID-19) and its vaccines have been associated with multiple cutaneous manifestations, including local hypersensitivity reactions to generalize cutaneous vasculitis. Some case reports describe the association between bullous pilomatrixoma and influenza, hepatitis A, and COVID-19 vaccines . To our A 28-year-old male presented to our clinic complaining of a five-month-old asymptomatic tumor in the left upper arm that has rapidly grown and changed in consistency. He received the AstraZeneca COVID-19 vaccine in the left upper arm four months prior. Physical examination revealed a hemispherical nodule measuring 4 cm x 2 cm x 2 cm, pink-colored, bullous appearance, soft consistency, and with an indurated zone beneath  Figure , charactCTNNB1 gene), a downstream effector of the Wnt signaling pathway that influences multiple cellular processes, including hair follicle differentiation, exists in 26 to 100% of pilomatrixomas. Also, a high level of B-cell lymphoma antigen 2 (BCL-2) proto-oncogene is present, which is associated with the activation of neoplastic cell proliferation [The exact etiology of pilomatrixoma is unknown. Trauma, insect bites, or surgical procedures sometimes precede the tumor . Mutatioferation .The etiology of the bullous appearance of some pilomatrixoma is a topic of discussion . One theOther tumors, such as schwannoma, xanthogranuloma, lymphoma, and plasmacytoma, have been reported to develop a bullous appearance. Several authors hypothesized that this "bullous appearance" is secondary anetoderma because of its distinctive histological features, including dermis with atrophy, edema, fragmented collagen, and disappearance of elastic fibers ,9. FurthLee et al. hypothesized that a skin tumor may be predisposed to secondary anetoderma if mechanical stress is applied. Mechanical stress on highly indurated tumors like pilomatrixoma can lead to a more significant inflammatory response with catabolic enzyme release . The neeWe propose a two-hit model in which pilomatrixoma neoplastic cells have higher expression of metalloproteinases, and mechanical stress causes a secondary inflammatory reaction, leading to an inflammatory cascade in which metalloproteinases play an essential role. Finally, secondary anetoderma develops.On the other hand, due to the nature of the vaccine itself, namely, AZD1222/ChAdOx1 nCoV-19 Vaxzevria, Covishield (AstraZeneca), an adenovirus based-vector could cause this inflammatory cascade. Nestic et al. demonstrated that after receiving an intramuscular adenovirus-based vaccine, infected epithelial cells augmented the expression of the proinflammatory cytokines IL-6, IL-8, IL-1β, and TNF-α . WhetherThere are a few reported cases of pilomatrixoma that develop a bullous appearance. Multiple reports of bullous pilomatrixoma are associated with trauma or vaccination, including our case report. Further research should be conducted to determine whether mechanical stress or a particular component in some vaccines is the inciting event. It is important to note that observations and studies on this matter indicate that the bullous appearance of some pilomatrixoma is, in fact, a secondary anetoderma developed on them.


Sclerosing encapsulating peritonitis is a rare chronic inflammatory condition often with unknown origins. We report a case of an abdominal cocoon or sclerosing encapsulating peritonitis, which was suspected to be a result of bowel obstruction. Tuberculosis peritonitis was also suspected. However, the exact diagnosis was unclear, and it was diagnosed as an idiopathic abdominal cocoon. The patient’s history is of clear relevance in this diagnosis, and this report will be of interest to clinicians attending to cases of bowel obstruction. Sclerosing encapsulating peritonitis (SEP) is a rare disease of uncertain cause characterized by intestinal obstruction followed by encapsulation of the small intestine with a fibro-collagen membrane . VariousAn 82-year-old man presented with a three-day history of abdominal distention and nausea. His family had a history of tuberculosis, and his past surgical history included strangulated small bowel obstruction 35 days prior. Then, he underwent release surgery without bowel resection Figure .Abdominal ultrasound sonography revealed ascites around the small intestine in the encapsulated area. Abdominal computed tomography further showed a mass of jejunum in the encapsulated area with ascites and calcified nodules Figure .We aspirated ascites comprising serous blood. Cytology, culture, including Mycobacterium tuberculosis, and other ascites laboratory data did not indicate malignancy or tuberculosis. A blood test did not confirm tuberculosis nor other malignancy; interferon-gamma release assays, CA125, and other tumor markers were unremarkable. Then, serum albumin and C-reactive protein were 2.5 g/dL and 6.4mg/dL, respectively. The nasogastric tube did not improve the ileus, and he underwent surgery. The small intestine was enveloped in dense fibrous tissue with calcified nodules and became a contiguous mass like sclerosing encapsulating peritonitis (SEP), also known as an abdominal cocoon Figure .This section, which lacked peristalsis without signs of obstruction, was resected. Histopathological evaluation of the capsule, nodules, and resected bowel did not reveal an apparent cause of abdominal cocoon; chronic dense fibrous tissue without caseous granuloma. Thus, we diagnosed an idiopathic abdominal cocoon. While tuberculosis peritonitis was most likely, we did not confirm the cause of the abdominal cocoon.SEP is a rare disease causing bowel obstruction. Our radiographic findings are similar in tuberculosis, peritoneal carcinomatosis, Crohn's disease, and sarcoidosis . Thus, iSEP is a rare condition that can present in patients with bowel obstruction. Even if past surgical history of bowel obstruction is within about 30 days, we must consider SEP as a differential diagnosis.


Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumours(EWS/pPNETs) of the kidney are rare. Signs and symptoms are atypical in EWS patients. Presenting symptoms include haematuria, abdominal pain, or a palpable mass. A comprehensive review of the literature shows that it is difficult to make an accurate diagnosis based on physical examination alone. The imaging findings of EWS/pPNETs are nonspecific. We used contrast-enhanced ultrasound (CEUS) to diagnose an EWS/pPNET in our patient, which had never been reported previously to our knowledge.This article reports the case of a 20-year-old female with an abdominal mass and gross haematuria for 1 month. The ultrasound revealed a hypoechoic mass with a clear margin at the lower pole in the left kidney. CEUS demonstrated signs of annular enhancement and heterogeneous enhancement of the tumour, and simultaneous wash-in was predominant. Computed tomography images showed an elliptical low-density tumour. The patient underwent a left kidney resection, and the pathological diagnosis was an EWS/pPNET. Twenty-one days after the kidney operation, the patient underwent 8 cycles of a CAV  + IE  chemotherapy regimen. Subsequently, radiotherapy  was administered for nearly 30 days. The patient had no signs of local recurrence or metastasis within a follow-up of 4 years.As a radiation-free, inexpensive, convenient, and repeatable examination method, ultrasound was the primary choice for kidney examination. Early CEUS was helpful to make an accurate diagnosis. Surgery and adjuvant radiation or chemotherapy administered in a timely manner can prevent further deterioration.The online version contains supplementary material available at 10.1186/s12894-022-01146-w. Ewing's family of tumours (ESFTs), the rarest of oncologic disorders , are higA 20-year-old female presented to the hospital with an abdominal mass and gross haematuria for 1 month. She recently complained of left abdominal pain, nausea and vomiting, and noticeable weight loss. The patient did not have a family history of malignant tumours. The physical examination revealed abdominal distention with a large mass in the left upper quadrant. The mass was irregular, hard, immovable, nontender and without overlying skin changes. The laboratory test results for tumour markers were as follows: carbohydrate antigen 125 (CA-125), 136.89 U/mL (reference range: 0.00–35.00 U/ml); the levels of other tumour markers, such as carcinoembryonic antigen (CEA), carbohydrate antigen 199 (CA-199), and alpha-fetoprotein (AFP), were normal. Urine analysis showed that the red blood cell (RBC) count was 56/µl (reference range: 0.0–25.0), and the white blood cell (WBC) count was 59/µl (reference range: 0.0–25.0). Liver and kidney function test results and the complete blood count were within normal levels. Conventional ultrasound (US) examination showed a 4.5 × 3.2 cm irregular lesion at the lower pole of the left kidney. To further clarify the diagnosis of the tumour, contrast-enhanced ultrasound (CEUS) was performed, which presented signs of annular enhancement and heterogeneous enhancement of the tumour, and simultaneous wash-in was predominant. The possibility of a tumour lesion was considered  , and 5–20% of patients often present with mutations in the EWS-ETS gene –13. A coImmunohistochemical and molecular diagnostic methods are critical to diagnose EWS/pPNETs. Pathological examination showed that the tumour cells were small, round, short spindle-shaped, and densely arranged. Homer Wright rosettes were found. Immunohistochemical results showed that the glycoprotein CD99 was expressed on the cell surface. The chromosomal translocation T 11;22)  can be observed in 95% of EWS/pPNETs. However, despite the availability of immunohistochemical and molecular diagnostic methods, these tumours were also misdiagnosed on biopsy [2 (q24; QCurrently, there is no universally accepted treatment regimens for EWS/pPNETs of the kidney based on treatment guidelines , 29, andIn conclusion, EWS/pPNETs of the kidney are very rare tumours that can be diagnosed by CEUS. However, multimodal imaging combined with pathological examination is necessary. Total surgical resection and auxiliary treatment can improve the prognosis of patients with EWS/pPNETs.Additional file 1. Supplement of immunohistochemical staining.


EWSR1 gene, with t  EWSR1-ATF1 or t  EWSR1-CREB1 fusions. We present a case of a 71-year-old man with an incidentally discovered GNET in the gastric cardia and fundus.Gastrointestinal neuroectodermal tumors (GNETs) are malignant tumors frequently arising within the muscularis propria of the gastrointestinal tract and often extend into the submucosa and subserosa. The stomach is the second most common site of incidence of GNETs after the small intestine. The most important differential of GNET is the clear cell sarcoma-gastrointestinal (CCS-GI). Both share similar morphological as well as molecular features and show S100 positivity; however, the lack of melanocytic differentiation in GNET distinguishes it from CCS-GI. Both typically show rearrangements of the  A malignant gastrointestinal neuroectodermal tumor (GNET) is a rare and aggressive tumor that typically occurs in adults, most frequently occurring in the stomach and the small intestine. Other common sites are the esophagus, colon, and anal canal . GNETs tWe report the case of a 71-year-old man with a past medical history of hypertension, hyperlipidemia, and benign prostatic hyperplasia who presented with complaints of dyspnea, fatigue, black stools, and a recent syncopal episode. Laboratory testing revealed anemia (HB 5.4 g/dl). A subsequent esophagogastroduodenoscopy was performed, and a submucosal gastric mass was detected   with the conventional treatment modalities (Surgery plus chemotherapy or radiotherapy). It hypothesized that chemotherapy might be a potential trigger for the onset of GNET . GNET isThe clinical presentation varies from symptoms of anemia, weight loss, and abdominal pain to an abdominal mass that can cause intestinal obstruction and is usually detected by a CT scan ,8. GrossEWSR1 gene, with t  EWSR1-ATF1 or t  EWSR1-CREB1 fusions with no specific impact on prognosis or clinical management [Different pathologic entities exist in the differential diagnosis of GNET. This poses a diagnostic challenge for distinguishing GNET. Gastrointestinal stromal tumors (GIST) and GNET share clinical and histomorphology features as both arise in the wall of the gastrointestinal tract and microscopically show spindle or epithelioid cells. Also, GIST is frequently positive for S100. It is important to exclude metastatic melanoma or clear cell sarcoma of the gastrointestinal tract (CCS-GI). Immunohistochemical staining would aid in the differential diagnosis since GISTs are negative for melanocytic markers such as HMB45, Melan A, and tyrosinase and are also negative for CD34, CD117, and DOG-1. Molecular characterization of the tumor nature will conclude the final diagnosis in the context of the clinical and histologic picture. GNET and GIST share some characteristics where both tumors arise in the gastrointestinal tract wall and microscopically show spindle and/or epithelioid cells. However, GIST does not typically show osteoclast-like multinucleated giant cells . Malignanagement ,16. GNETGNETs pose a diagnostic challenge both clinically and microscopically, where the careful distinction of the histologic and immunohistochemical patterns is warranted. Pathologists show be aware of this clinical entity when considering the differential diagnosis of a gastric mass showing spindle or epitheliod morphology.


The juvenile Nasopharyngeal Angiofibroma (JNA) is a benign vascular tumor of the skull base, which affects almost only male adolescents. It is a rare tumor, making up less than 0.05% of all head and neck tumors. Although histologically benign, it may have a very aggressive behavior, extending to adjacent tissues and causing bone destruction by compressionWLS, male, 16 years old, soccer goalie, with a past of epistaxis episodes and left nasal obstruction for 5 months; and a nasopharyngeal lesion seen upon nasal endoscopy. The CT scan showed signs suggestive of an Andrews/Fisch IIAfter 1 year of follow up, the patient had symptom recurrence, and a nasopharyngeal lesion was seen upon nasal endoscopy. CT scan and MRI showed an Andrews/Fisch IVbConsidering the aforementioned aspects, in October of 2007 we decided for a craniotomy approach through the left frontotemporal zygomatic via for tumor ressection, since the tumor may be detached from the ICA. Under microscopic view, the duramater was dissected from the middle fossa floor, which was opened by a high speed pneumatic burr, exposing the orbit, the round foramen with the maxillary nerve (V2), the oval foramen with the mandibular nerve (V3) and the cavernous sinus. The vascular connections between the ICA and the angiofibroma were coagulated with the bipolar cautery. Following that, we noticed that the angiofibroma was invading beyond the duramater, and after opening it we saw that the tumor was in close contact with the temporal lobe, with a clivage plane. After removing its intradural portion, the dural gap through which the tumor had passed was closed with a temporal fascia graft. The nasopharyngeal, sphenoidal and infratemporal portions were digitally dissected and removed superiorly through the middle fossa floor gap, which was later rebuilt by rotating the temporal muscle. The histopathology analysis of the intradural specimen showed a close relation between the JNA and the dura mater, with invasion of the latter .Figure 1The patient developed left abducent nerve paralysis in the post-operative, which lasted for 1 month. After 1 month of follow up, there were no signs of recurrence upon nasal endoscopy and MRI, or neurologic deficit, and the patient resumed his regular activities.JNA with skull base erosion and intracranial extension courses with an increase in operatory risks, as well as the likelihood of significant vascular contributions of the ICA, which pre-operative embolization is not feasible. For these reasons, it is associated with higher hemorrhage rates of difficult control, neurologic deficits, subtotal resection and recurrence. Previous pre-operative embolization is also associated with recurrence, since the reduction of tumors with deep invasion of the sphenoid makes it difficult to completely excise it, with quick revascularization of the residual tumor in the immediate postop, especially by ICA branchesBased on the assumption that the bone destruction mechanism by the angiofibroma results from a compressive growth pattern instead of an infiltrative oneDura penetration is a rare phenomenon, with very few reports in the literature. Upon MRI, the transdural lesion suggestive signs are an absence of the cleavage plane between the tumor and the duramater, or the circunferential involvement of the ICA; nonetheless, in some cases the MRI is unable to distinguish the invasion as extradural or intradural. The presence of colateral branches of cerebral parenchima vassels irrigating the tumor, seen upon angiography, may be a sign of duramater involvement.Jafek et al.According to Danesi et al.Although intracranial invasion by JNA is relatively common, its intradural extension is very rare, happening mainly in tumor recurrences. Usually the cleavage plane between the JNA and the cerebral tissue is well defined. Total tumor resection is possible with minimum neurological deficits, but dura reconstruction is necessary.