Rectal neuroendocrine tumors (NETs) are rare, with an incidence of 0.17%, but they represent 12% to 27% of all NETs and 20% of gastrointestinal NETs. Although rectal NETs are uncommon tumors, their incidence has increased over the past few years, and this is probably due to the improvement in detection rates made by advanced endoscopic procedures. The biological behavior of rectal NETs may be different: factors predicting the risk of metastases have been identified, such as size and grade of differentiation. The tendency for metastatic diffusion generally depends on the tumor size, muscular and lymphovascular infiltration, and histopathological differentiation. According to the current European Neuroendocrine Tumor Society (ENETS) guidelines, tumors that are smaller than 10 mm and well differentiated are thought to have a low risk of lymphovascular invasion, and they should be completely removed endoscopically. Rectal NETs larger than 20 mm have a higher risk of involvement of muscularis propria and high metastatic risk and are candidates for surgical resection. There is controversy over rectal NETs of intermediate size, 10–19 mm, where the metastatic risk is considered to be 10–15%: assessment of tumors endoscopically and by endoanal ultrasound should guide treatment in these cases towards endoscopic, transanal, or surgical resection. Rectal neuroendocrine tumors (NETs), formerly known as “carcinoid tumors” because of their peculiar characteristics, are rare, with an incidence of 0.17% during screening colonoscopy .The gastrointestinal tract is the most frequent site for the onset of NETs, and the rectum is the second localization by frequency after the small intestine, even if these neoplasms represent only 1–2% of all rectal tumors .Although rectal NETs are uncommon tumors, their incidence has increased over the past few years, as reported in the surveillance, epidemiology, and end results (SEER) database , and thiWhen they are symptomatic, rectal NETs may occur with diarrhea, abdominal pain, weight loss, or gastrointestinal blood loss ; severe The biological behavior of rectal NETs may be different: factors predicting the risk of metastases have been identified, such as size and grade of differentiation. The tendency for metastatic diffusion generally depends on the tumor size, muscular and lymphovascular infiltration, and histopathological differentiation .Most of rectal NETs are diagnosed incidentally during endoscopic evaluation for colorectal cancer screening and they present as small lesions, usually less than 12 mm in diameter, and slightly protruding from the mucosa; these macroscopic features of rectal NETs resemble hyperplastic polyps, making them hard to differentiate from the other most common polypoid lesions. For this reasons, often the diagnosis is known afterwards, because a routine snare polypectomy or mucosectomy is mistakenly performed; therefore, the diagnostic pathway becomes relevant for recognizing these type of lesions and for avoiding mistakes leading to their mismanagement .Frequently, rectal NETs are located in the frontal or lateral wall of the mid-rectum, on average between 4 and 8 cm from the anal verge .Generally, these lesions appear on endoscopic examination as smooth, round, mobile, submucosal nodules, or focal areas of submucosal thickening, covered by a yellow-discolored mucosa (reflecting the presence of chromogranine) .Although this is the most frequent macroscopic manifestation, several studies have shown that rectal NETs can present with various endoscopic features, and the more the appearance of these lesions differs from the canonical one, the higher is the possibility that they are associated with a high risk of metastases. In particular, it is important for the endoscopist to pay attention to subtle surface changes, such as ulceration or erosion, which can be important indicators of aggressive disease . It is nThere are contrasting opinions on the management and diagnostic path that follows the incidental feedback of a neuroendocrine neoplasm of the rectum. One school of thought tends to practice biopsies during endoscopic examination, to proceed directly to the histological characterization of the lesion. This approach is not recommended for many reasons: first, a complete resection of neoplasm is only found in about 30% of cases, so a further operational procedure is still necessary , then roAccording to the current European Neuroendocrine Tumor Society (ENETS) consensus , an endoEUS is a good preoperative exam that defines accurately the tumor size, the depth of invasion, and the presence of pararectal lymph node metastases: this is important for choosing the appropriate therapeutic approach . MoreoveThe rectal wall appears on an EUS as a five-layer structure: the first two, hyper- and hypo-echogenic, respectively, correspond to the mucosa. The third layer is hyperechogenic and corresponds to the submucosa. In EUS, rectal NETs usually appear as hypoechoic lesions, located in both the second and third wall layer, and clearly demarcated from the surrounding tissue .If the rectal NET interrupts the upper two thirds of the third layer of the rectal wall with its hypoechogenic structure, it is classified as SM-D (−); if it extends to the lower third of the submucosa, it is classified as SM-D (+). This classification is useful for both the pre-operative assessment of the lesion and the post-operative assessment of resection margins .EUS is not always able to make a differential diagnosis with other non-carcinoid submucosal lesions and the most similar in appearance is leiomyoma, which also appears as a hypoechogenic submucosal nodule with a uniform internal echo . In thesAt the histological examination, rectal NETs usually show a trabecular growth pattern, with a nest-like or rose-like structure.The most common immunohistochemical markers for neuroendocrine neoplasms are synaptophisine, chromogranin A, and neuro-specific enolase , but theAfter EUS evaluation, which allows describing the primary lesion, patients should undergo computed tomography (CT) to discover regional and distant metastases. Magnetic resonance imaging (MRI) can be used when the nature of the injuries found at CT is uncertain. The ENETS consensus recommends CT/MRI when the diameter of the primary lesion is >10 mm or when, after resection, residual and metastatic diseases are suspected.In high-grade NETs (G3) it is important to perform functional tests, which can be useful for the management of rectal NETs and for distant metastasis detection.Octreoscan is the first approved radiopharmaceutical for carcinoid tumor imaging; it consists of somatostatin analogs radio-labelled with 111 Indium, used to detect somatostatin receptor positive tissue . PositroHowever, these functional tests cannot be used for poorly differentiated neoplasms that may not express somatostatin receptors; in these cases, PET-CT with 18 F-fluoro-deoxyglucose (18-FDG) may be more appropriate for metastasis detection  Scheme .Rectal NETs have an excellent prognosis compared to all other neuroendocrine gastrointestinal neoplasms, especially because they are often diagnosed incidentally as small and early neoplasms ; even ifData from the SEER database show that the 5-year survival rate is 88.3 %, depending on tumor size and invasion , and acc-Low Grade (G1): 2 mitotes/10 HPFs and <2% Ki67 index;-Intermediate Grade (G2): 2–20 mitotes/10 HPFs and 3–20% Ki67 index;-High grade (G3): 20 mitotes/10 HPFs and >20% Ki67 index.The grading is based on mitotic index per 10 high-power fields (HPFs) and the expression of Ki67, a tumor proliferation marker:The staging with TNM includes tumor size, depth/invasion of tumor, and metastatic spread  Table 1.As reported in the literature, the major criteria for assessing the invasiveness of rectal NETs are the size of the tumor, the depth of invasion, increased mitotic index, and lymphovascular invasion, but the only one that can be evaluated pre-operatively and that can guide the treatment approach is the size of the lesion .Rectal NETs less than 10 mm in size at diagnosis have a very low risk of distance metastasis (<3%), and local excision can be curative in most cases; long-term outcomes are excellent, with a 5-year survival of 98–100%, while rectal NETs diagnosed >20 mm and those with regional (N1) and distant metastases (M1) have a worse prognosis, with survivals of 54–74% and 15–37%, respectively [The choice of therapeutic intervention for rectal NETs depends on their features, especially on their size, grade of differentiation, muscolaris propria involvement, lymphatic and vascular invasion, increased tumor proliferative index, and risk of metastasis.The aim of the treatment is to achieve a complete oncological resection, with clear margins and no residual disease, therefore endoscopic resection is indicated if there is no evidence of invasion beyond the submucosa and presence of regional disease. In case of evidence of invasion involving the muscolaris propria and regional disease, surgery must be considered as the first option .According to the current European Neuroendocrine Tumor Society (ENETS) guidelines, tumors that are smaller than 10 mm and well differentiated are thought to have a low risk of lymphovascular and muscolaris invasion and rarely exhibit malignant potential, and should be completely removed endoscopically; the risk of metastases has been estimated at less than 3% for rectal NETs less than 10 mm in size. Rectal NETs larger than 20 mm are likely to have a higher risk of involvement of the muscularis propria and high metastatic risk (60–80%) and are candidates for surgical resection. There is controversy over rectal NETs of intermediate size 10–19 mm, where the metastatic risk is considered to be 10–15%: assessment of tumors endoscopically and by endoanal ultrasound should guide in these cases towards endoscopic, transanal, or surgical treatment ,32. Various endoscopic techniques can be used for treatment of rectal NETs, including endoscopic polypectomy, endoscopic mucosal resection (EMR), modified EMR (m-EMR), and endoscopic submucosal dissection (ESD).m-EMR includes: endoscopic mucosal resection with a ligation device (EMR-L), which in some studies is also called endoscopic submucosal resection with band ligation (ESMR-L); cap-assisted EMR (EMR-C); EMR after circumferential pre-cutting (EMR-P), which in some studies is also called endoscopic mucosal resection with circumferential incision (CIEMR) or circumferential submucosal incision prior to endoscopic mucosal dissection (CSI-EMR).The choice of optimal endoscopic treatment depends on the tumor characteristics, such as size, and mucosal and submucosal appearance .Endoscopic polypectomy is not usually used in rectal NETs resections, as it often does not provide adequate and complete resection of the lesion, and additional interventions may be needed . EMR is an endoscopic technique based on a submucosal injection of a saline solution to elevate the mucosal lesion away from the muscularis propria, followed by a snare cautery resection. EMR has been frequently used in the resection of small and superficial neoplasms confined to the mucosa or superficial submucosa because of its simplicity and lesser invasiveness, but its feasibility is still uncertain in cases of rectal NETs because of the potential problem of incomplete excision .Several studies report that complete resection rates of standard EMR are variable, ranging from 30% to 70% , and recEMR can also be performed using a dual-channel endoscope (EMR-D) and, by lifting the lesion with grasping forceps to identify the lower margin of the submucosal lesion and strangling the base with a snare, EMR-D enables deeper resection compared with standard EMR. Lee et al.  comparedm-EMR techniques are characterized by the use of special devices, which allow for a better resection of the tumor; EMR-C and EMR-L are commonly performed, and while EMR-P was recently introduced, several studies ,39,40,41EMR-P is performed by lifting the mucosa with a saline injection, making a circumferential incision (pre-cutting) using the tip of the snare or special endoknives and resecting the tumor with a snare; this technique presents advantages over other m-EMR procedures because it has no size limitation with respect to the tumor resection .EMR-L has some advantages over EMR, particularly due to the use of a ligation device; it is performed after an initial submucosal saline injection to elevate it from the muscle layer, by suctioning the lesion into the ligating device and cutting around the concerned area with the ligation by using a round snare ; while iEMR-C is an effective technique for endoscopic resection of rectal NETs, as demonstrated in several studies ,44 and mMashimo et al. , in a reKim et al.  comparedA disadvantage of ESMR-L may be the fact that it is only applicable for tumors of 10 mm or less in size, due to the short diameter of the caps fitted to colonoscopies [In agreement with the superiority of ESMR-L over EMR, in terms of achieving complete resection, other studies have focused on the comparison between ESMR-L and ESD.ESD is an advanced endoscopic technique used for en bloc resection of slightly invasive gastrointestinal tumors or larger mucosal lesions, and it provides a precise histological staging. ESD is performed by submucosal dissection after a submucosal injection and a circumferential cutting of the mucosa around the lesion. It has been reported that ESD is an effective and safe technique for treating gastrointestinal tumors, including rectal NETs, even if it is associated with a high risk of complications (perforation and bleeding), a long procedure time, and required technical skills compared to other endoscopic resection techniques .Several studies ,47,48,49Another study, conducted by Niimi et al. , compareBang et al.  even demWang et al.  proposedYan et al.  in theirTALE is a surgical technique performed in T1 malignant rectal tumors, including rectal NETs, located in the lower rectum, less than 7 cm from the anal verge and less than 1/3 lumen diameter in size; it is not used in the higher rectum because the exposure is significantly limited. TALE is performed by introducing anal retractors into the anal canal to maintain exposure, lifting the mucosa with a saline injection to elevate the lesion and resecting the tumor with electrocautery under direct vision; the defect in the rectal wall is closed with an absorbable suture .An alternative to TALE is transanal endoscopic microsurgery (TEM), a minimally invasive technique that allows the complete resection of benign or T1 malignant rectal neoplasms, including rectal NETs, with a safety surgical margin and a continuous layer; TEM is also a feasible surgical option indicated for complete removal of residual tumor, in case of tumor-positive resection margins or recurrences .TEM is performed by using a multi-channel port positioned transanally that allows, at the same time, the use of a rigid rectoscope with magnified three-dimensional vision and endosurgical instruments: the scheduled resection area is previously marked by electrocautery dots, then a full-thickness resection down to the perirectal fat is performed, and finally the defect in the rectal wall is closed by a continuous running suture with clips or absorbable monofilament. As with TALE, TEM must be performed with the patient under local or general anesthesia. TEM shows several advantages over both endoscopic technique, by providing a full-thickness resection of large tumors, and over conventional transanal resection, because it provides improved operative visualization and access to lesions higher in the rectum .The morbidity of TEM reported in literature ranges from 4% to 29%, and the most common complications are bleeding, peritoneal entry with peritonitis, rectal wall perforation, fecal incontinence, and conversion to laparotomy .Moore et al.  comparedMcCarty et al.  in theirEMR and ESD achieved a complete microscopic resection in 46.3% to 65.5% and in 75% to 82.6% of cases, respectively. TEM allows achieving a 100% rate of free resection margins .Another endoscopic technique introduced recently is endoscopic full thickness resection (EFTR), which is performed in lesions that are difficult to resect, both for anatomic location and for negative lift sign after submucosal injection. EFTR is performed using a full thickness resection device (FTRD), that is an over-the-scope system, which allows a single step EFTR after placement of a modified over-the-scope-clip (OTSC). Limitations correlated with this technique are the resectable tumor size, due to diameter of the FTRD , but EFTR appears to be an effective and safe resection method for well-differentiated rectal NETs of smaller size; combining a high complete resection rate with low complication rate and short procedure time .In addition, Meier et al.  demonstrSurgery is recommended in case of tumors >20 mm or between 10 mm and 19 mm with high risk features, such as muscular or lymphovascular invasion, or if the margins are positive on endoscopic, or for local resection. Laparoscopic surgery has become the standard surgical approach because it provides better short- and long-term outcomes compared with open surgery and, often, low anterior resection or intersphincteric resection, depending on the localization of the tumor, with total mesorectal excision being performed. Many rectal NETs arise near the anus, and colectomy with lymphadenectomy often necessitates rectal transection with a colostomy, although anal preservation is an important outcome of radical surgery in terms of quality of life ,59.The prognosis after endoscopic resection of rectal NETs is generally good, but, although a macroscopically complete resection is achieved in most cases, microscopically remnant NETs may be present on resection margins, and this may be the cause of local tumor recurrence or distant metastasis. According to the ENETS guidelines, lesions presenting a histologic non-complete resection after endoscopic treatment must undergo additional salvage treatments, but this does not always happen, as the positive resection margins are not always predictive factors of local recurrence or metastasis. Sung et al.  analyzedCha et al.  evaluateIn addition Moon et al.  in theirAccording to ENETS guidelines , regularAlthough rectal NETSs are still uncommon tumors, their incidence has increased over the past few years, due both to the widespread use of colonoscopy for screening and due to improvements in detection rates made by advanced endoscopy. The choice of therapeutic intervention for rectal NETs depends on their features, especially on their size, grade of differentiation, muscolaris propria involvement, lymphatic and vascular invasion, increased tumor proliferative index, and risk of metastasis; and the assessment of tumors endoscopically and by endoanal ultrasound should guide treatment approach.Rectal NETs smaller than 10 mm in size are usually low grade and can be successfully treated endoscopically or by local excision, because they rarely metastasize and represent 80% of all rectal NETs diagnosed.EMR-L should be considered as the first-line of treatment for small rectal NETs, because it is a safe and effective technique, relatively simple, and less time-consuming compared with ESD. ESD should be left as a second-line therapy when EMR-L is not applicable. Rectal NETs that are 10–20 mm in diameter are associated with a poorer prognosis compared with those <10 mm, because they have a higher risk of muscolaris and lymphovascular invasion, and they can be treated by endoscopic or transanal resection or radical surgery. TEM is more likely to yield clear resection margins and lower recurrence rates compared to TALE.Rectal NETs > 20 mm in size have a high metastatic potential and they are candidates for radical rectal resection and lymph node dissection. The duration of follow-up remains uncertain, as data on long term outcomes of rectal NETs are scarce.


Level of Difficulty: Beginner.)A previously fit and well 30-year-old man presented with palpitations, fever, and pleuritic chest pain. Multimodality imaging and histopathology confirmed the diagnosis of primary cardiac angiosarcoma. We present the details of the presentation, diagnostic process using multimodality imaging, and clinical management. (  He also described an acute reduction in his exercise tolerance.On examination, a resting tachycardia with heart rate of 110 beats/min and low-grade pyrexia of 37.8°C were detected. Oxygen saturations were 97% on room air, and blood pressure was normal. There were no signs of congestive cardiac failure or audible murmurs on pre-cordial auscultation.The medical history was unremarkable.Given the history of pleuritic chest pain and fever and the timing of this presentation during the COVID-19 pandemic, coronavirus infection was the primary differential diagnosis. Venous pulmonary embolism and bacterial or other viral causes of pneumonia were also considered. Blood tests including D-dimer, electrocardiogram, and plain film chest x-ray film were ordered.Full blood count showed a normocytic anemia. Although C-reactive protein (158 mg/l) and D-dimer (840 ng/ml) levels were raised, troponin I level (27.3 ng/l) was not raised. Liver function test results were normal.COVID-19 swabs were negative.Electrocardiogram showed sinus tachycardia at 114 beats/min . Chest xGiven the presentation of pleuritic chest pain with sinus tachycardia and elevated D-dimer, computed tomography pulmonary angiogram was ordered. This excluded pulmonary embolism but demonstrated a large soft tissue mass in the anterior mediastinum .Figure 2Transthoracic echocardiogram confirmed the presence of a large hypoechoic right atrial (RA) mass. The patient was admitted to the ward for further assessment.1- and T2-weighted imaging (Cardiac magnetic resonance (CMR) confirmed the presence of an RA mass measuring 12 × 8 cm, filling >90% of the chamber lumen . The mas imaging  and 4 an imaging  and 6. F imaging . Left veNo functional tricuspid valve stenosis was detected on transesophageal echocardiography .Figure 7The RA mass demonstrated moderate uptake on fluorodeoxyglucose–positron emission tomography scan. Lymph node involvement was seen with low-grade hyperenhancement of the right internal mammary nodes. There was focal fluorodeoxyglucose uptake in the right iliac crest and left anterior iliac spine compatible with bony metastases  and 9.FiTransjugular core biopsy was performed for histopathologic analysis. This showed a spindle cell tumor, staining strongly with CD34 and ERG (erythroblast transformation-specific related gene), confirming the radiological suspicion of angiosarcoma.Coronary angiography was performed in preparation for debulking surgery. This showed a highly vascular tumor with supply from the right coronary artery (RCA) and proximal circumflex artery. There was also a mass effect compressing the RCA  and 4.Because of the presence of widespread metastatic disease and the extent of regional spread and size of the tumor, curative treatment was not considered to be possible by the multidisciplinary team.The patient underwent successful debulking surgery with reconstruction of the RA wall and grafting of the RCA. Post-operative transesophageal echocardiography showed preserved left ventricle systolic function with mildly dilated and impaired RV systolic function , and 9.Following surgery, the patient underwent palliative chemotherapy with doxorubicin and palliative radiotherapy at the sites of bony metastases.Angiosarcoma is the most common primary cardiac malignancy in adulthood, comprising 9% of all primary cardiac tumors . AngiosaThere are typically 2 distinct clinical presentations, reflecting the 2 common morphological variants of the tumor . The foc1- and T2- weighted images and LGE reflects areas of tumor tissue, necrosis, and hemorrhage , invasion of the RV base, variable tissue intensity on TThe prognosis of angiosarcoma is poor, with a median survival of 14 months, reducing to 6 months in metastatic disease . PalliatIn our case, the multidisciplinary team decision was for surgical debulking to provide the best option for palliative care, given the degree of RA cavity obstruction, and to allow time for chemotherapy administration.Because of the rare incidence of cardiac angiosarcomas, there is a dearth of evidenced-based guidelines, and currently, no standardized treatment algorithm for cardiac angiosarcoma exists .Following surgery and palliative chemoradiotherapy, repeat imaging showed significant metastatic disease progression within the lungs and pelvis. In addition to doxorubicin and single-slice radiotherapy, hospice services were engaged. The patient died 4 months following the initial diagnosis.This case demonstrates the typical presentation of primary cardiac angiosarcoma and its associated features on multimodality imaging. Multidisciplinary care of this relatively rare tumor is important, which reflects the lack of consensus guidelines on management.The authors have reported that they have no relationships relevant to the contents of this paper to disclose.


Cutaneous tumors with adipocyte differentiation are frequently excised by surgeons in their daily clinical practice and sometimes less common histological diagnoses arise. Knowledge of different pathological entities and their natural history is essential for better patient management.Atypical spindle cell/Pleomorphic lipomatous tumor (ASPLT) is a recent group included in the WHO classification. We report a case of a middle-aged man with an atypical pleomorphic lipomatous tumor in an unusual location. Adipocytic tumors are the most common soft-tissue tumors, and surgical removal of these lesions is a procedure widely performed in general surgery. In 2020, WHO published a new classification of Soft Tissue and Bone Tumours, differentiating several entities . The atyWe report the case of an atypical pleomorphic lipomatous tumor that presented as a rapidly enlarging mass in the right groin region.A 58-year-old man, with no relevant medical history, presented with a history of an enlarging painless mass at his right groin region for the past three months Figure . The patThe patient was referred to the General Surgery department by a urologist, with suspicion of a soft-tissue tumor. An MRI described a “focal subcutaneous lesion with nodular morphology of 4.7 cm and no malignancy features”. Based on clinical and image findings, it was decided to perform an excisional biopsy.Despite the apparent benign characteristics, the lesion was surgically removed along with the surrounding adipose tissue, preserving the margins. There were no complications related to the procedure.Grossly, it was a subcutaneous nodular non-capsulated solid lesion, multilobulated, well-circumscribed, greyish-yellowish, without necrotic areas Figure . Microscth edition of soft-tissue tumors classification which included a new group, the atypical spindle cell/pleomorphic lipomatous tumor (ASPLT) [Cutaneous tumors with adipocyte differentiation are frequently excised by surgeons. Although they are mostly lipomas or angiolipomas, other rare entities can arise and raise doubts. Recently, WHO published the 5 (ASPLT) .Mentzel T et al. proposed the term “atypical spindle cell lipoma” for the first time in 2010 , suggestASPLT is a morphologic spectrum of benign adipocytic neoplasms . The celThis entity can affect both sexes, with a slight male predominance, including mostly middle-aged adults with a peak in the sixth decade , likewisFor palpable lesions, ultrasound can be used in the initial investigation, but MRI remains the imaging gold standard . Our patHistological differential diagnosis of ASPLT is highly dependent on the predominant histological features of each tumor. This entity can be considered a category between classic spindle cell/pleomorphic lipoma and liposarcoma, namely: WDL  or pleomorphic liposarcoma (PLS) in cases with significant pleomorphism . In dubiRegarding the differential diagnosis of malignancy, PLS can involve the dermis or subcutaneous tissue in 25% of cases. Most patients report a rapidly growing painless mass (median: 3-6 months) , similarRegarding benign differential diagnosis, classic spindle cell/pleomorphic lipoma are capsulated lesions with no atypia , that arComplete surgical excision  is the appropriate treatment for ASPLT. Incompletely removed lesions may locally recur in 10-15% of the cases [When a soft-tissue tumor is suspected, a diagnostic approach may include an ultrasound or an MRI and eventually a core needle biopsy. The diagnosis of ASPLT can be challenging and the morphological differential diagnostic range can be broad due to the wide variety of microscopic appearances. Recognition of morphologic clues and immunohistochemistry/molecular tests to confirm the diagnosis and exclude WDL and PLS are crucial. Although it is a benign adipocytic tumor, it carries a considerable risk of local recurrence.


Pindborg tumor is a benign expansile and slow growing odontogenic tumor that occurs mainly in adulthood. Limited management data exist for its treatment in young patients. We report the case of a 5-year-old patient and provide recommendations for the care of pediatric patients diagnosed with this rare odontogenic tumor. Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, was described by Danish oral pathologist Jens Pindborg in 1955 ,3. PrimaThere are approximately 350 cases of CEOT reported in the literature. None of the 350 cases have occurred in a patient younger than 8 years of age. A recent review by Chrcanovic et al. analyzed 339 cases of CEOT. The study found that the mean age of presentation of CEOT patients is 38.1 years and identified that the highest tumor recurrence rate was associated with treatment by enucleation and curettage . AlthougGiven the paucity of data that exists on CEOT in the pediatric population, there is no consensus on its diagnosis, surgical treatment, tumor surveillance or timing of reconstruction. Herein, we present a case of an intraosseous CEOT in a 5-year-old child, and share our algorithmic approach for its management in the pediatric population.The patient was an otherwise healthy 5-year-old female who presented to our institution for a second opinion regarding management of a mandibular mass. She reported 2 weeks of left lower mandibular pain, followed by a week of progressive left facial swelling. Laboratory studies  were unremarkable. Due to lack of response to empiric oral antibiotic treatment, a computed tomogram (CT) of the neck with contrast was obtained. Imaging demonstrated an expansile lesion of the left mandibular angle, measuring 2 × 2 × 3 cm. The intraosseous mandibular lesion was mixed radiolucent-radiopaque, with trabeculations –C).Clinical exam revealed mild left-sided facial swelling without signs of infection. Intraorally, bony expansion of the left posterior mandible was noted. There was no tooth mobility and her oral mucosa was intact. Her clinical findings were suggestive of an odontogenic tumor. Given the patient’s age, clinical findings of asymptomatic expansion, posterior mandibular location, and the CT findings, a benign odontogenic pathology was suspected. Given the patient’s age and the low risk of malignancy in the presentation, we decided to proceed directly to definitive treatment. Tumor enucleation and peripheral ostectomy was planned.The patient was taken to the operating room and the tumor was aspirated with no return of fluid. The tumor was then accessed intraorally via an incision along the left posterior mandible. Subperiosteal dissection was carried out, and a bony window was made, in an area where the tumor had thinned out the bone. The tumor was carefully enucleated and peripheral ostectomy  was performed with a round bur. Follicles for developing teeth #18 and 19 were embedded within the tumor and were therefore removed with the specimen. The mandibular cortices and inferior border were mostly intact, and therefore no immediate bony reconstruction was performed. The defect was packed with absorbable gelatin sponge and the incision was closed in a watertight fashion.Final pathology confirmed the diagnosis of CEOT. Histopathology showed large polygonal epithelial cells with ample granular and eosinophilic cytoplasm and well-defined cell borders. Nucleoli were focally present, as well as irregular calcifications and Liesegang rings .In our case, no reconstruction was performed at the time of resection. We opted to defer bone grafting to avoid obscuring the surveillance picture and to allow time for spontaneous bony regeneration. Given the low suspicion for malignancy and likely odontogenic tumor etiology in this case, we decided to pursue a semi-conservative approach. Strong consideration was given to the patient’s young age, excellent healing potential, and small bony defect size that could fill on its own. We did not bone graft any mandible defect after enucleation and peripheral ostectomy, but instead, take advantage of the pediatric healing potential. Our patient is now almost four years out from surgery, and she remains clinically and radiographically disease-free, with excellent bony regeneration and normal facial contours . No adjuThe classic presentation for CEOT involves an asymptomatic, slow growing, expansile lesion of the posterior mandible affecting individuals in their 3rd–6th decades of life. Although rare, the peak incidence of CEOT occurs in the 5th decade ,11,12. Gen bloc resection is unknown [Although CEOTs are frequently benign, there are reports that describe malignant transformation . Describ unknown ,16,17. Ien-bloc tumor resection and placement of a rigid reconstruction plate spanning the mandibular defect. Six months later, mandibular continuity is restored with secondary bone grafting. Dental prosthetic rehabilitation follows, with placement of osseointegrated dental implants at skeletal maturity.The main advantage of an immediate mandibular reconstruction is to provide a functional and aesthetically acceptable outcome at the time of tumor resection. A staged approach with delayed mandibular reconstruction has proven to be a viable alternative. Troulis et al. described a staged reconstructive protocol for pediatric patients undergoing treatment of mandibular tumors . Their aen bloc resection or hemi-mandibulectomy are advised. We do not recommend this approach when there is a high suspicion for a vascular tumor or a malignant neoplasm. This semi-conservative approach has proven efficacious in our practice over the past 7 years, as evidenced in our series of 15 pediatric patients with odontogenic tumors currently in preparation for publication.To date, the 5-year-old patient presented herein is the youngest person reported with an intraosseous CEOT. Such a young patient presents the surgeon with several management dilemmas, including how aggressively to treat a CEOT presenting in early childhood, and how to avoid multiple operations in a young patient. Determining the need for, and timing of bony reconstruction, as well as setting up an optimal tumor surveillance plan are critical in this population. Our differential diagnosis based on clinical presentation alone initially included dentigerous cyst, odontogenic keratocyst, central giant cell lesion, osteoblastoma, and ameloblastoma. When considering the imaging findings, our differential diagnosis expanded to include other mixed lesions like CEOT, odontoma, calcifying odontogenic cyst and dentinogenic ghost cell tumor. After an extensive discussion with the patient’s family, we decided to forego an incisional biopsy and instead proceed to definitive treatment due to the low suspicion for malignancy. This has been the approach at our children’s hospital in the management of pediatric odontogenic neoplasms, and it has its benefits and disadvantages. Benefits of foregoing an incisional biopsy prior to definitive excision includes mitigating the physical and emotional risks of repeated trips to the operating room, while decreasing surgical costs with fewer operations. Children and parents also benefit from fewer missed school days or workdays and decreased total recovery time. Disadvantages of this approach include the lack of a definitive histologic diagnosis at the time of tumor excision and the need to extract teeth involved in the lesion. It is important to stress that the decision of forgoing a diagnostic biopsy should not be taken lightly and is not advised to practitioners without vast experience treating these tumors. Importantly, this should be considered an option only if the tumor has clear signs of benign features. As many of these tumors may be malignant, it is paramount to determine whether the tumor can be treated conservatively or whether In other very large pediatric tumor centers, the groups also skip biopsies in about 85–90% of mandibular lesions that are ‘clear-cut’ and suspected to be benign based on appearance and relative size (3 cm in a pediatric mandible is much more significant than 3 cm in adult). Hence experience to make such determination is paramount and extreme caution is still warranted.Since the CEOT recurrence rate in the pediatric population is unknown, we can only extrapolate a recurrence rate based on long term adult data, that indicates a recurrence rate of 12–14% and the potential for malignant transformation ,16. GiveEven though a 4 year follow up is encouraging in our experience, the literature still reports a well- documented recurrence rate with enucleation of 14–15%, so vigilant observation should be maintained.CEOT is a rare odontogenic tumor with a slow growth pattern and a peak incidence in mid-adulthood. Intraosseous CEOT’s have been reported in the pediatric population, but to date never in a child as young as 5 years of age. Because of its rarity, no CEOT treatment consensus exists. We based our semi-conservative management on existing adult CEOT data, as well as on established treatment of other pediatric odontogenic tumors. To avoid multiple surgical procedures, we performed a single stage excision of the lesion with peripheral ostectomy. We defer immediate mandibular reconstruction to allow for adequate surveillance, as hardware and bone graft particles can obscure proper visualization of recurrent tumor foci. As CEOT recurrence in the pediatric population is unknown, close surveillance is paramount in recurrence identification. We recommend 5 years of surveillance, with postoperative imaging biannually for the first two years, then annually for the remainder three years.Due to the retrospective nature of this single case report, Ethics approval or exemption was not required by the Cincinnati Children’s Hospital IRB.


Abdominal apoplexy is one of the rare causes of non-traumatic intra-abdominal bleeding. This condition is usually seen in male patients in their 50s with history of hypertension. As soon as abdominal apoplexy is suspected, immediate resuscitation should be performed followed by emergent surgery. The patient's outcome depends entirely on the clinical condition and the time interval between diagnosis and treatment.Herein we present a 63-year-old man with idiopathic spontaneous intraperitoneal hemorrhage (ISIH) caused by spontaneous rupture of non-aneurysmal inferior pancreaticoduodenalartery (IPDA).In this report, a case of abdominal apoplexy has been presented caused by spontaneous rupture of non-aneurysmal inferior pancreaticoduodenalartery (IPDA) in a patient without any significant past medical history. Abdominal apoplexy or “idiopathic spontaneous intraperitoneal hemorrhage (ISIH) is a rare and often fatal clinical condition in which one or more of the major arteries in the abdomen rupture spontaneously, causing intra-abdominal hemorrhage , 2. Its A 63-year-old man was referred to Modarres Hospital, Tehran with gradual onset of obscure generalized abdominal pain six hours before admission. His pain was located in the epigastric and periumbilical region without subsequent radiation to anywhere else with one episode of vomiting. 9/L, neutrophils 84.2%, hemoglobin (Hb) 15.2 g/dL, C reactive protein +2 and amylase 63 U/L. abdominal x-ray and ultrasonography were normal.Other findings were moderate abdominal distension with periumbilical abdominal tenderness without guarding. Initial blood tests showed white cell count (WBC) of 21.7 × 109/L, neutrophils 76.5%, Hb 8.1 g/dL and abdominopelvic CT-scan showed evidence of hematoma near the head of pancreas and perihepatic and perisplenic fluid (About five hours after resuscitation and appropriate fluid therapy, he has not felt better and blood tests were deranged with WBC of 18.4×10ic fluid , 2. BecaAfter midline laparotomy and evacuating massive intraperitoneal hematoma about 2.5 liters, right medial visceral rotation, the Cattell-Braasch maneuver, was performed and an active bleeding in the base of IPDA was detected. IPDA was suture ligated at the base with 4/0 nonabsorbable stitch to achieve hemostasis. The post-operative period was uneventful. Oral fluids were started the day after the surgery and patient was discharged from the hospital two days after the surgery. One week after discharge, he had a normal follow-up without any concerning problem.Abdominal apoplexy is a clinical condition that can occur at any age or sex, but is most common in men between the ages of 50 and 60. Numerous causes can lead to spontaneous intra-abdominal bleeding without a history of trauma, including aneurysms, vasculitis, atherosclerosis, malignancies, and inflammatory diseases. Some patients have history of hypertension (Clinical manifestations of this condition present as the onset of sudden and often severe abdominal pain with or without nausea or vomiting, and sometimes hematochezia. On examination, these patients have abdominal tenderness that does not correlate with clinical condition . BleedinThe main treatment for ISIH is based on adequate resuscitation and control of bleeding. Usually, emergency laparotomy is performed in these patients but other treatments such as trans-arterial embolization in stable patients have recently been reported , 9.In this patient, because of stable condition, more diagnostic workups were performed. But because of deterioration of his condition, emergency laparotomy was decided for definitive diagnosis and treatment.Based on our research, this case is a second case of abdominal apoplexy due to spontaneous rupture of non-aneurysmal IPDA in a patient without any significant past medical history. The previous case of abdominal apoplexy due to IPDA was reported by Wang et al. in 2017 (Transcatheter embolization was performed . SpontanIn this case report, we emphasize that, in any patient with abdominal pain, even without a history of underlying disease, surgeon must consider abdominal apoplexy in differential diagnoses especially in clinical deterioration of the patient, laparotomy should be considered because in this disease, without immediate surgery, the patient's mortality rate would be very high.


Epidermoids are rare intracranial neoplasms that grow slowly and present in the third to fifth decade of life. Giant epidermoid cysts are infrequent, and their occurrence in the posterior fossa is rare. We describe a similar case, where a patient presented with a long-standing history of headache, imbalance, and progressive weakness in the arms. Imaging revealed a giant space-occupying lesion in the posterior fossa measuring 6.25 cm x 7.56 cm x 6.8 cm, which was confirmed on histopathology to be an epidermoid cyst. The patient underwent suboccipital craniotomy extending up to the rectosigmoid junction to remove the same and was on a follow-up to check for recurrences. Epidermoids are slow-growing and benign cystic lesions. These are rare intracranial congenital neoplasms that account for 1% of all brain tumors . Even thA 65-year-old female presented with the chief complaints of headache, which occurred intermittently for 1.5 years, difficulty in walking leading to imbalance since last six months, and gradually progressive weakness in both arms since last four months. MRI of the brain with contrast revealed a large smooth extra-axial lesion in the left posterior occipital convexity measuring 6.25 cm x 7.56 cm x 6.8 cm. The lesion was also extending into the jugular foramen, left CP angle reaching up to the floor of the middle ear , followed by the combined middle cranial fossa and rectosigmoid approach (20%) . In the We report a rare case of a giant epidermoid cyst of the posterior fossa of the brain extending into the jugular foramen, left CP angle reaching up to the floor of the middle ear which was removed via suboccipital craniotomy extending up to the rectosigmoid junction. There was no preoperative complication except the weakness in the arms. This tumor is exclusively extradural, during surgery no breach in dura was observed. Near-total excision was performed and specifically at CP angle, the dura was badly thinned and the capsule adhered tightly which made the separation impossible without dural breach. There were no postoperative complications. Intracranial epidermoids are slow-growing benign cystic lesions, whose symptoms for compression or irritation of vascular or neurological structures become apparent later on. Surgical resection is the treatment of the choice for the tumor and it depends on the extent of the tumor involvement. It's important to follow-up since recurrences are commonly reported in such cases.


SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. We present the case of a 44-year-old man who underwent a surgery for a perforated small intestine. Compued tomography scan revealed a tissular mediastino–pulmonary mass.Histopathological examination of the intestinal mass shown a malignant tumour with a typical epithelioid and rhabdoid cells, numerous mitoses and large necrosis. A large panel of immunohistochemistry revealed loss of SMARCA4 and SMARCA2 and allowed the diagnosis of SMARCA4-DTS. It is important to consider SMARCA4-deficient thoracic sarcoma in the differential diagnosis of tumours showing suggestive morphologic features in patients of all ages, especially in the case of metastasis associated with thoracic mass. SMARC4, a gene encoding the ATPase subunit of the switch/sucrose non-fermenting (SWI/SNF) chromatin remodelling complexes. It has an extremely poor prognosis with metastatic disease at the time of presentation and a median survival of 6–7 months [SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a distinct subset of intrathoracic tumors, recognised for the first time in 2015 by Le Loarer et al. [We report a case of a SMARCA4-DTS revealed by intestinal metastasis with an emphasis on differential diagnosis.A previously healthy 44-year-old man, with a 25-pack-year smoking history, underwent a surgery for acute peritonitis revealing a perforated small intestine mass with numerous peritoneal nodules and large lymph nodes.We received a 26-cm-long segment of the small intestine measuring, two lymph nodes and a peritoneal nodule. The intestine includes an ulcerating and infiltrating tumor of 6 cm. Several samples were taken. Histological examination showed a malignant tumor with poorly cohesive sheets of large cells with an epithelioid and rhabdoid appearance Fig. a. The tuBased on computed tomography scan data a complementary immunohistochemical investigations were performed and they revealed that tumor cells exhibited a loss of expression of both SMARCA4 and SMARCA2 while SMARCB1 was retained Fig. a, b.Fig.Thus morphology and immunophenotype, coupled with the computed tomography data and clinical history, were consistent with the diagnosis of SMARCA4-deficient thoracic sarcoma to the small bowel.The patient received two cycles of chemotherapy with gemcitabine and cisplatin. He died 6 months after the diagnosis from a digestive haemorrhage.SMARCA4-DTS is first described in 2015 by Le Loarer et al. [SMARCA4 gene, located on chromosome 19p13, encodes the BRG1 protein. The SMARCA2 gene, located on chromosome 9p24 encodes the BRM protein. These two proteins are mutually exclusive catalytic subunits of the SWI/SNF chromatin-remodelling complex which is formed by multiple other proteins, among which INI-1 (encoded by SMARCB1 gene). By regulating transcription, promoting cell differentiation, and repairing the DNA, the complex acts as a tumour suppressor [The Usually, clinical presentation of SMARCA4-DTS is a compressive and infiltrative large mediastinal mass, rarely found in the lung . They ocIn typical cases, SMARCA4-DTSs is a poorly differentiated neoplasm of round to epithelioid cells with prominent cytological atypia organized in a solid pattern and showing rhabdoïd differentiation . MitosisIn 2019, Perret and Le Loarer’s group in France proposed three criteria, deemed sufficiently significant in terms of specificity and sensitivity, based on which the diagnosis of SMARCA4-DTS intrathoracic malignancies ought to be carried: (1) rhabdoid or poorly differentiated phenotype, (2) complete loss of expression of SMARCA4 and SMARCA2, and (3) focal or diffuse expression of at least two of the following markers: SOX2, CD34, or SALL4 . But theThe prognosis for SMARCA4-DTS cases is poor because treatment strategies for these tumors have not been established. The estimated median overall survival duration is 7 months . Present


Gastric lipomas are rare, representing 2–3% of all benign tumours of the stomach. Most of these stomach neoplasms are small and detected incidentally during endoscopic or radiology evaluations. Computed tomography is highly specific imaging for lipoma diagnosis. Endoscopy and endoscopic ultrasound are other important diagnostic modalities to confirm the diagnosis. Identifying typical features can avoid biopsy or surgery in asymptomatic patients. In patients with larger lesions, usually more than 2 cm, clinical presentation may encompass haemorrhage, abdominal pain, pyloric obstruction and dyspepsia. As a result of its extreme low incidence, treatment is not standardized, though it is widely accepted that a symptomatic tumour mandates resection. Here, we present the case of a 60-year-old female presenting with abdominal pain and recurrent vomiting due to a giant gastric lipoma (80 × 35 × 35 mm). The patient underwent laparotomy and an enucleation was performed. Gastrointestinal (GI) lipomas are uncommon benign and slow-growing tumours composed of mature adipose tissue surrounded by a fibrous capsule. Although GI lipomas can occur in any part of the gastrointestinal tract, its most affected location is the colon, followed by the ileum and jejunum . GastricGiant gastric lipomas ≥4 cm) are even more unusual . The vas cm are eA 60-year-old female patient, with medical history of diabetes mellitus type 2, coronary artery disease, grade II obesity and dyslipidaemia, presented with complains of intermittent abdominal pain and postprandial vomiting over the past 6 months. The patient referred worsening symptoms in the last 3 months and an isolated episode of hematemesis, without hemodynamic repercussion or symptomatic anaemia. She denied weight loss, melena or change in bowel habits throughout this period. Clinical examination was unremarkable, and routine blood tests results showed values within normal range.An upper GI endoscopy revealed a large subepithelial mass arising from the posterior wall of the gastric antrum, occupying more than 50% of the lumen, soft when pressed with biopsy forceps. The biopsies were non-diagnostic. Additionally, a contrast-enhanced computed tomography (CT) demonstrated a well-defined lesion of the antrum, measuring 8 × 3 cm, non-contrast enhancing and with uniform adipose density, highly suggestive of lipoma . EndoscoWe decided to perform a midline supraumbilical laparotomy. During the procedure, a large and mobile soft globular mass was palpated in the antrum , and antGastric lipomas are usually solitary, originating in the posterior wall of the antrum and are typically found in patients in their fifth and sixth decade of life , 10. MalAn accurate preoperative diagnosis is based on endoscopic evaluation, EUS and CT examination , 10. TheAbdominal CT is the imaging study of choice and highly specific. In the case of gastric lipoma, it strongly suggests the diagnosis by visualization of a well-circumscribed homogeneous mass with fat density ranging between −80 and −120 Hounsfield units. These values are considered pathognomonic and suggest a diagnosis of gastric lipoma, by falling in the expected value range for adipose tissue , 5. EUS Because of pathognomonic appearance of gastric lipomas on endoscopy, EUS and CT scan, endoscopic biopsies is rarely needed. If a biopsy is needed, regular mucosa biopsies are usually inadequate to sampling because of the submucosal localization of the most lipomas, as it was the case on our patient. Alternatively, EUS guide-sampling can be used , 6, 10.The treatment for gastric lipomas is not standardized and is mostly based in case reports.Because of the benign nature and well-encapsulated feature of lipomas, gastric preserving can be a treatment option and related to tumour size: endoscopic resection when < 4 a 6 cm and surgery when > 6 cm, perhaps endoscopy has been used to submucosal resection of a giant lipoma with 9 cm , 9, 10. We believed that if an accurate preoperative diagnosis is established and malignancy is carefully ruled out, a gastric preserving surgery like enucleation should be the treatment of choice to avoid major morbidity associated to partial gastrectomy.None declared.


Gastrointestinal (GI) schwannomas are very rare. Among GI schwannoma, the most common site is the stomach. Very few cases of intestinal schwannoma have been reported in the literature. Moreover, intestinal schwannoma in the setting of inflammatory bowel disease has rarely been reported. We report a case of ileal schwannoma incidentally detected in a 52-year-old lady with long-lasting ulcerative colitis having intestinal obstruction. On laparotomy, the patient was found to have adhesive intestinal obstruction due to a previous abdominal hysterectomy. On careful inspection of the bowel loops, a 2cm ileal lesion on the mesenteric border was detected. Segmental ileal resection was performed. Histopathology revealed a mesenchymal tumor with immunohistochemical analysis suggestive of schwannoma . The patient had an uneventful recovery with no recurrence on follow-up. Schwannoma is a type of peripheral nerve sheath tumor. It is most commonly seen in the head, neck, and limbs. It is a rare mesenchymal tumor of the gastrointestinal (GI) tract. It accounts for 2.9% of all surgically removed mesenchymal tumors of the GI tract . Most ofA 52-year-old lady presented with abdominal pain, bilious vomiting, constipation, and abdominal distension for two days. She was diagnosed to have moderately active UC involving the whole colon (pancolitis) four months back and was treated with oral mesalamine therapy. She had undergone abdominal hysterectomy for uterine fibroids eight years back. On clinical examination, the patient was hemodynamically stable. Abdomen was distended with hyperperistaltic bowel sounds. Routine blood investigations were within normal limits. Computed tomography (CT) of the abdomen revealed dilated proximal ileal loops with air-fluid levels and collapsed terminal ileal loops Figure . There wOn laparotomy, the proximal ileal loops were grossly dilated and the terminal ileal loops were collapsed. The large bowel loops were also collapsed and appeared unremarkable. The ileal loops were densely adhered to the anterior abdominal wall at the previous scar site causing luminal obstruction. Extensive adhesiolysis was done and the obstruction was released. On careful examination, a 2 x 2 cm lesion was noted on the mesenteric side of the terminal ileum distal to the site of adhesive obstruction  is the most common tumor . In a laPreoperative diagnosis of GI schwannoma is difficult. Small lesions are often not visible on radiological investigations such as CT and magnetic resonance imaging (MRI) as seen in the present case. On CT abdomen, the schwannomas appear as exophytic or endophytic, round to oval lesions with heterogenous enhancement . Larger On histology, all mesenchymal tumors are typically composed of spindle cells. Immunohistochemistry is required to determine the origin of the tumor. Schwannomas are characterized by immunopositivity for S100, vimentin, and negative staining for CD34, CD117, actin, and desmin, as seen in the present case .There is an increased risk of colorectal cancer and small bowel adenocarcinoma in patients with inflammatory bowel disease ,13. HoweSchwannoma should be included in the differential diagnosis of small bowel tumors. Preoperative diagnosis of intestinal schwannoma is difficult. Surgical resection is the mainstay of treatment. It is a benign tumor and associated with a good prognosis. It should be differentiated from GIST on histology using immunohistochemical analysis.


Other abnormalities included pituitary lesions (n = 5), brain atrophy (n = 2), and one each of metabolic and traumatic conditions. Fourteen animals had a normal brain MRI study. The most common spinal abnormality was intervertebral disc disease (n = 7). Other disorders included vertebral dysplasia (n = 2), presumptive ischemic myelopathy (n = 1), subdural ossification causing spinal cord compression (n = 1), and multiple myeloma (n = 1). Spinal cord swelling of undetermined cause was suspected in two animals, and seven patients had a normal MRI study of the spine. MRI is a valuable tool in the diagnostic workup of non-domestic felids with presumptive neurologic disease.Magnetic resonance imaging (MRI) is the recognized gold standard for diagnostic imaging of the central nervous system in human and veterinary patients. Information on the use of this modality and possible imaging abnormalities in captive non-domestic felids is currently limited to individual case reports or small case series. This retrospective study provides information on technique and imaging findings in a cohort of cases undergoing MRI at an academic Veterinary Medical Center. The University of Tennessee College of Veterinary Medicine MRI database was searched for non-domestic felids undergoing MRI of the brain or spine from 2008 to 2021. Medical record data were recorded, and MRI studies were reviewed. Fifty animals met the inclusion criteria. The most common brain diseases were Chiari-like malformation ( With the exception of Chiari-like malformation and degenerative intervertebral disc disease, publications on diseases of the central nervous system in non-domestic felids are generally scarce and mostly consist of post mortem data, small case series, and individual case reports. An ante-mortem diagnosis of brain or spinal disease typically requires some form of diagnostic imaging. Radiography is readily accessible and may provide helpful information especially in the evaluation of osseous structures (skull and vertebrae), e.g., in cases of trauma .Computed tomography (CT) is increasingly available in zoological institutions and sanctuaries and has the advantage of rapid image acquisition resulting in short anesthesia times . CT is tAcinonyx jubatus) has been reported to cause bilaterally symmetric white matter changes best seen on T2-weighted images  is the recognized gold standard for diagnostic imaging of the central nervous system in human and veterinary patients. Information on the use of this modality and possible imaging abnormalities in captive non-domestic felids is currently limited to individual case reports or small case series. MRI findings reported in a Bengal tiger with hypoxic encephalopathy included bilaterally symmetric T2 hyperintense brain lesions with restricted diffusion and evidence of intralesional hemorrhage . MRI find images . The MRIty sella . MRI finty sella . Multifoty sella . InformaThe purpose of this retrospective study was to provide information on technique and imaging findings in captive non-domestic felids undergoing MRI at an academic Veterinary Medical Center.The University of Tennessee College of Veterinary Medicine MRI database was searched for captive non-domestic felids undergoing MRI of the brain and/or spine from 2008 to 2021. Inclusion criteria were a complete or partial ante- or postmortem MRI examination in felids suspected to have neurologic disease. Normal animals scanned in the frame of research projects or for comparison purposes were excluded. Animals included in a large retrospective case series on brain lesions in captive non-domestic felids identified on autopsy  were incMedical record data were recorded by an ACVIM neurology resident (DW) supervised by 2 ACVIM-Neurology diplomates , 2 ACZM diplomates , and an ACVR/ECVDI diplomate (SH) and included species, age, sex, weight, pertinent medical history, and, if available, information on cerebrospinal fluid (CSF) analysis, results of additional tests, final clinical diagnosis, and autopsy results. Additional information on clinical management and outcome was collected if possible by contacting the animals' caretakers.All MRI studies were reviewed by an ACVR/ECVDI board-certified radiologist (SH). The scan site and imaging diagnosis were recorded. Additional information captured included the MRI system used, if contrast medium was administered, and if any adverse effects to contrast medium administration were noted.Panthera leo) undergoing MRI examination of the brain, the foramen magnum height/skull width (FMH/SW) ratio was calculated as previously described , 11 lions (Panthera leo), 4 leopards (Panthera pardus), 3 each of bobcats (Lynx rufus), servals  and snow leopards (Pantera uncia), 2 each of cheetahs (Acinonyx jubatus) and caracals , and 1 each of clouded leopard (Neofelis nebulosa), cougar (Puma concolor), liger (Panthera leo x Panthera tigris), and lynx (Lynx canadensis) met inclusion criteria. The majority of cats (41) belonged to a single large cat sanctuary. One lion, one cheetah and one tiger belonged to zoological institutions, and the three servals, one caracal and one lion were privately owned.Eighteen tigers  and 31 male  cats. The median age was 8 years ; the age of one leopard was unknown. The median weight was 105 kg ; the weight of one tiger was not recorded.n = 28); brain and cervical spine (n = 9); cervical spine (n = 1); thoracolumbar spine (n = 3); lumbosacral spine (n = 4); cervical and thoracic/thoracolumbar spine (n = 2); and brain, cervical and thoracolumbar spine (n = 1). Scan regions post mortem included the brain (n = 1) and brain and cervical spine (n = 1). Eighteen scans were performed using a 1.0T MRI system , and 32 scans were performed using a 1.5T MRI system . Live animals were scanned under general anesthesia. Smaller felids undergoing brain MRI were positioned either in sternal , and post contrast T1-W images with or without fat saturation (FatSat) in 3 planes. Additional sequences acquired on a case-by-case basis included diffusion weighted imaging (DWI), transverse proton density (PD) weighted images, transverse “SPACE” (“Sampling Perfection with Application optimized Contrasts using different flip angle Evolution”), and transverse post contrast “VIBE” (“Volume Interpolated Breathhold Examination”) images. A full MRI examination of the spine generally included a dorsal STIR (short tau inversion recovery), sagittal T2-W, T1-W, STIR and “HASTE” , and transverse T1-W and T2-W images. Additional sequences acquired on a case-by-case basis included a transverse SPACE and post contrast T1-W FatSat images in 1-3 planes. The scan parameters used for small cats were identical to those used for similar sized canine patients. Protocols were modified for larger cats. Sample protocols for imaging the brain and spine of a large cat on a 1 and 1.5T MRI system are provided in MRI protocols varied between patients and were limited in some instances based on clinical suspicion, imaging findings, and number of scan areas. A full MRI examination of the brain generally included sagittal T2-weighted (T2-W), transverse T2-W, T1-weighted (T1-W), T2-FLAIR (fluid attenuated inversion recovery), T2A total of 39 felids had a partial or complete MR examination of the brain.Eight animals were included in this category. Six animals  had an MRI diagnosis of Chiari-like malformation  believed to be responsible for the clinical signs. Another lion was diagnosed with calvarial hyperostosis but also tested positive for toxoplasmosis. The clinical significance of calvarial hyperostosis was therefore undetermined. One additional lion had a diagnosis of “probable occipital hyperostosis” but had only mild clinical signs at presentation and was reported to be neurologically normal 8 years after the MRI study. Imaging abnormalities in affected cats included thickening of the occipital bone and osseous tentorium of the cerebellum, crowding of the caudal fossa, variable degree cerebellar compression and herniation, and syringomyelia of the cranial cervical spinal cord . Two aniInflammatory conditions of the intracranial and/or other structures of the head were diagnosed or suspected in eight patients.Three animals had a diagnosis of otitis media, with or without otitis interna. A 4-year-old snow leopard presented with recent vision loss, decreased mentation, and decreased response to stimuli had evidence of bilateral otitis media and questionable meningeal enhancement suggesting possible intracranial extension. The brain parenchyma was normal. On autopsy 3 weeks following the MRI study, the patient had meningoencephalitis with vasculitis, possibly of viral cause; likely unrelated to the MR diagnosis of otitis. A second patient, a 13-year-old tiger previously diagnosed with chronic otitis media based on CT examination, underwent MRI due to the recent onset of seizures. The MRI examination confirmed otitis media and interna with extension into the adjacent soft tissues of the caudal head but without evidence of involvement of the intracranial structures . RegionaA 17-year-old tiger that had been treated for blastomycosis 18 months prior presented for neurologic deficits and was found to have asymmetric hydrocephalus associated with marked contrast enhancement of the ventricular lining . CSF anaA 1-year-old tiger was presented with acute onset seizures, ataxia, and nystagmus. MRI examination revealed ill-defined and indistinct multifocal intra-axial brain lesions as well as marked meningeal enhancement especially affecting the leptomeninges . Based oA 12-year-old bobcat with head shaking, ataxia, and hyporeflexia of one-week duration was found to have a tract-like cerebellar hemorrhage on MRI, suggestive of possible parasite migration . The CSFA 5-year-old bobcat was presented with a variety of clinical and neurologic deficits consistent with cerebellar and/or brainstem neuroanatomic localization. The MRI examination of the brain was normal, however, based on CSF analysis the patient was diagnosed with neutrophilic meningoencephalitis. No infectious organisms were identified. The animal was successfully medically managed and remained normal for more than 10 years following the MRI examination until its death without recurrence of the previous clinical signs.A 6-year-old lynx presented for disorientation, opisthotonos, ataxia and protrusion of the eyelid. The MRI examination did not show any abnormalities of the intracranial structures; however, contrast enhancement of the cornea and uvea was noted . A subseA 16-year-old snow leopard presented with acute onset of ataxia and blindness. Following slow recovery from anesthesia he sustained a seizure during recovery and was euthanized. A limited MRI examination of the brain was performed postmortem. There was marked diffuse bilaterally symmetric white matter disease , consistA 15-year-old lion developed ataxia in both front limbs approximately 3 weeks prior to presentation which was attributed to complications of a recent declaw repair and was managed medically. Immediately prior to presentation, the animal was found in sternal recumbency unable to sit or stand up. The MRI examination revealed a fracture of the occipital condyle that was subsequently confirmed on autopsy . The lesMarked diffuse brain atrophy  was docuLesser degree brain atrophy was also seen in a 15-year-old cheetah with staggering, moving slowly, falling, stiff gait, somnolence and disorientation. The animal was euthanized 2 years following the MRI examination for progressive likely age-related deficits. The brain was normal on gross inspection postmortem; histopathological examination was not performed.Pituitary abnormalities were noted in five animals.A large cyst-like lesion was noted associated with the pituitary fossa in a 14-year-old cougar  presentiAn empty sella was identified in a 5-year-old tiger presented with a suspected focal seizure and was considered an incidental finding unrelated to the clinical signs . The samA 12-year-old leopard with obtundation, ataxia and anisocoria had a mildly nodular and heterogeneous pituitary gland  confirmeFinally, a 10-year-old tiger presenting with vague neurologic signs including ataxia and subsequently diagnosed with intervertebral disc disease (see below) had an enlarged and cystic pituitary gland with a suspected small focus of hemorrhage. A limited postmortem examination was performed and no pituitary abnormalities were reported.n = 4; see section on spine below), outside the CNS (n = 2), or remained undetermined (n = 7).In addition to the bobcat with neutrophilic meningoencephalitis mentioned above, 14 felids had a normal MRI examination of the brain. In one of these, a 0.5-year-old caracal, a diagnosis of presumptive idiopathic epilepsy was made, and the patient's seizures were controlled with medical management. For the 13 remaining animals, a reason for the presenting signs was either found in the spine  or to the lumbar spine (n = 2) (n = 2) or lumbosacral spine (n = 1). All three animals were medically managed. Two were euthanized 1 week and 4 months following MRI, respectively, due to lack of clinical improvement. One leopard remained stable neurologically and died of unrelated causes 5 years following the MRI examination.The evaluation and classification of intervertebral disc disease was performed using previously published criteria , 18. A d (n = 2) . All aniTwo young animals, a 0.8-year-old female leopard and a 0.75-year-old male liger, were diagnosed with congenital vertebral anomalies ultimately confirmed to be vertebral dysplasia.The leopard presented with an acute onset of neurologic deficits after a fall. A possible episode of ataxia and lethargy may have occurred the week prior. MRI findings included foreshortening and abnormal shape of the C2-C5 vertebral bodies, irregular endplate margination of these vertebrae, loss of normal T2 hyperintensity of the C2-C5 intervertebral discs, and flattening and compression of the spinal cord. The imaging findings were consistent with congenital vertebral dysplasia. There was also the impression of diffuse cervicothoracic spinal cord swelling and meningeal enhancement, possibly suggesting concurrent meningomyelitis. This animal was euthanized 6 weeks following MRI due to progressive decline in mentation and mobility. Cervical vertebral dysplasia with spinal cord compression was confirmed on autopsy.sella turcica and tentorium cerebelli dysplasia).The liger presented with chronic paraparesis and proprioceptive ataxia of the pelvic limbs. On neurologic examination, he also failed to demonstrate deep pain perception in the pelvic limbs and tail. This animal was reported to have a sibling with similar clinical signs. MRI findings included foreshortening and abnormal shape of the L4 through L6 lumbar vertebrae, with decrease in intervertebral disc space width, decrease in the normal T2 hyperintense signal of the intervertebral discs, dorsal protrusion of the intervertebral discs, lumbar kyphosis, and spinal cord compression . ComputeA 16-year-old tiger was presented with a two-week history of progressive paraparesis. MRI examination of the lumbosacral spine showed evidence of multifocal vertebral lesions (involving at least L2-L4). The lesions were T1, T2 and STIR hyperintense, strongly contrast enhancing, centered on the medullary cavities of the affected vertebrae, and for the most part exhibited cortical sparing . The lesAn 11-year-old clouded leopard presented for ataxia of unknown duration and possible vestibular disease. On MRI there was a T2 moderately hyperintense intramedullary lesion associated with the dorsal aspect of the spinal cord at the level of C2 which was symmetric and wedge-shaped on transverse image . This leA 7-year-old serval was presented with a several week history of ataxia and hind limb paresis unresponsive to medical treatment. The MRI examination of the thoracolumbar spine showed extensive attenuation of the subarachnoid space throughout the thoracic spine especially on the MR myelogram, with a suspected intradural extramedullary lesion at T11-12 . There wA 2-year-old snow leopard and an 8-year-old caracal had MRI evidence of spinal cord swelling/hyperintensity and diffuse attenuation of the subarachnoid space, respectively, suggesting possible meningomyelitis. CSF analysis in the snow leopard was normal, and the caracal had suspected, mild mixed pleocytosis. The snow leopard died 3.5 weeks following MRI, after developing systemic illness. Autopsy yielded a diagnosis of multifocal moderate Wallerian-type degeneration, and a primary axonopathy with myelin degeneration was suspected. The cause of death was attributed to adrenal insufficiency. The caracal died of presumptive septicemia 5 days after MRI. Autopsy yielded a diagnosis of mild to moderate Wallerian degeneration of the brain and spinal cord. There was no meningomyelitis in either animal, and the reason for the spinal cord and subarachnoid space changes suspected on MRI remains undetermined.The MRI examination of the spine was considered unremarkable in seven felids.A 0.7-year-old serval with a 3-month history of progressive paraparesis, weakness, ataxia and decreased postural reflexes in the pelvic legs had a normal MRI examination of the thoracolumbar spine. Electromyography (EMG) and nerve conduction velocity studies were performed. Muscular activity was normal, but peripheral nerve conduction recordings were decreased, consistent with a probable diagnosis of demyelinating/remyelinating polyneuropathy. The animal was treated medically, improved initially, and then had recurrent clinical signs 1.5 years after the MRI. Medical treatment was repeated and the patient improved, again.A 6-year-old serval was presented for a 6-month history of self-mutilation of the tail and hyperesthesia. MRI of the lumbosacral spine was within normal limits, and a diagnosis of behavioral self-mutilation was made.A 0.75-year-old cheetah was presented with progressive neurological deficits. MRI of the cervical spine (and brain) were normal. The CSF tap was consistent with hemorrhage, likely iatrogenic. Infectious disease testing was negative. Medical management was instituted at a referral institution. The animal was initially stable but then deteriorated and died <1 year following MRI. Autopsy findings were consistent with degenerative myelopathy.A 10-year-old tiger became acutely non-ambulatory 2 weeks prior to presentation. CT of the thoracolumbar spine was performed and was within normal limits. The patient had minimal response to empiric treatment and was euthanized. A limited postmortem MRI examination of the cervical spine was performed and was unremarkable. On autopsy there was severe subacute myelomalacia of the lumbar spine (not included in the MRI field of view) attributed to fibrocartilaginous embolism although no emboli were detected in the examined sections.n = 2) or remained undetermined (n = 1).In the remaining three animals the reason for neurologic deficits were found in the head/brain  intravenously at a dose of 0.1 mmol/kg and not exceeding 20 mL total volume. No adverse effects or other problems were noted in 43 cats. One patient, a white tiger, developed urticaria shortly after contrast medium administration , and an This manuscript presents the largest case series of captive non-domestic felids undergoing MRI of the CNS to date. Diagnostic quality MRI scans were acquired using a standard 1.0 or 1.5T MRI system and using modified standard brain and spine MRI protocols , 22. IntThe most common disorder was Chiari-like malformation which has previously been described in several feline species and has been associated with hypovitaminosis A in lions , 26–28. Cuterebra, Capillaria, Taenia, Angiostrongylus and Baylisascaris species  in one patient was unexpected, and the decision to pursue advanced imaging in this patient may in part have been related to the inability to perform a thorough physical examination with the patient awake and attribution of ocular signs to CNS disease.Clostridium perfringens neurotoxicity  were diagnosed with a novel leukoencephalomyelopathy of undetermined cause . An enviDiffuse brain atrophy was the main finding in two geriatric cats. Even though there is clear evidence of age-associated brain pathology in domestic cats , reportsOne patient was diagnosed with a skull fracture. The diagnosis was challenging, and the lesion was originally missed when the images were first evaluated. CT is generally considered the gold standard in the evaluation of acute traumatic head injury. MRI has been shown to have fairly high accuracy in fracture in identification in a canine and feline cadaver model . HoweverNeoplastic brain lesions  are common in domestic felids  and wereThe most common spinal disease in the current study was intervertebral disc disease. This is in line with several prior studies describing this condition as being common in non-domestic felids –6, 54.Two animals were diagnosed with vertebral dysplasia resulting in spinal cord compression. This is surprising as this type of congenital vertebral anomaly is rather rare in animals. Spinal cord compression due to atlantal vertebral malformation has previously been reported in two African lions . It is cThe only patient with spinal neoplasia in this patient cohort was diagnosed with multiple myeloma, which is surprising [this animal was included in a prior case series on hypergammaglobulinemia and myeloma in 5 tigers ]. SpinalA presumptive diagnosis of an ischemic vascular accident was made in a 20-year-old clouded leopard. The location and appearance of the intramedullary lesion are consistent with an ischemic event. Even though the lesion resembled that of feline ischemic myelopathy , 20 in oExtensive subdural ossification resulting in spinal cord compression was diagnosed in one serval, an uncommon condition with only few similar cases reported in the veterinary literature , 62. TheThe MRI examination was normal in several patients as expected with certain CNS diseases  or due to lesion location outside the image field of view or outside the CNS. While a normal MRI examination may be frustrating to the attending clinician when trying to identify the reason for an animal's presumed neurologic deficits, it is important to understand that even a normal study has value as it helps to rule out many diseases which would be expected to result in MRI abnormalities.There are several limitations to this study. The information on clinical and neurologic examination findings in the medical records was limited in many cases, inherent to any retrospective study and complicated by the inherent difficulty in performing a clinical/neurologic exam in non-domestic felids. The decision which site to image was usually based on clinical signs reported by the caretakers and/or abnormalities observed by attending veterinarians. A decision to pursue brain MRI was based on abnormalities indicative of intracranial disease including seizures, head tilt, vision loss, and behavioral abnormalities. Imaging of the cervical spine was performed when gait abnormalities of all four legs were evident but there was no obvious evidence of concurrent abnormalities pertaining to the brain or cranial nerves. A decision to pursue MRI of the thoracolumbar/lumbosacral spine was based on abnormalities limited to the pelvic limbs or tail. Even though this resulted in imaging of the inappropriate area in some instances, and in some unnecessary MRI studies in animals with disease localization outside the CNS, the authors believe that this is representative of the veterinarians' and caretakers' situation when dealing with non-domestic felids. It is important to provide information on utility and limitations of performing MRI in the frame of the diagnostic workup for presumptive neurologic disease. Another limitation of this study is that a definitive diagnosis was not obtained in all animals to confirm the MRI diagnosis. Autopsy was performed in almost all animals that died during the evaluation period, and follow-up information was available for many others. However, there were a few cases for which either only an imaging diagnosis was obtained, or in which the diagnosis remained open. This is unfortunately not uncommon in retrospective studies. Another limitation is that the MRI protocols were not standardized. However, most studies included a minimum number of standard MRI sequences, and when performing an MRI examination in a clinical patient the protocol had to be tailored to the individual animal's situation. Sample MRI protocols are provided as appendices to this paper to aid veterinarians interested in performing an MRI study in a large non-domestic cat.In summary, MRI is a valuable tool in the diagnostic workup of non-domestic felids with presumptive neurologic disease. This manuscript provides a summary of recommended MRI techniques and describes a variety of possible diagnoses including several previously unreported conditions.The original contributions presented in the study are included in the article/SH devised the study, assisted with recording of the medical record data, interpreted the MRI studies, and prepared the manuscript. DW-H, KA, WT, AC, and ER recorded the medical record data, provided follow-up, and revised the manuscript. LC reviewed the histopathology for select cases and revised the manuscript. Final approval of the completed article was done by all authors.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.


Primary biliary non-Hodgkin's lymphoma (PBNHL) is a rare disease with only 41 cases reported since 1982. The incidence of PBNHL in patients with malignant cholangiocarcinoma was 0.6%, and PBNHL accounted for 0.4% of extranodal non-Hodgkin's lymphoma, and only 0.016% of all non-Hodgkin's lymphoma cases.We present a rare case of PBNHL in a 59-year-old female who had jaundice for 3 days with weight loss and Epstein-Barr virus infection. Initial computed tomography and magnetic resonance imaging showed diffuse thickening wall of bile ducts with corresponding lumen stenosis, blurred fat space around the portal vein, lymphadenopathy, and a normal spleen. These manifestations and images were similar to hilar cholangiocarcinoma. So, the diagnosis of hilar cholangiocarcinoma was initially considered.Postoperative pathology confirmed the final diagnosis was PBNHL.The patient and her family requested to clarify the histologic diagnosis by laparotomy biopsy. Because the biopsy result could not be defined during operation, then right hemihepatectomy and choledochojejunostomy were performed. She did not receive any antitumor treatment.One month after the patient's first examination, both computed tomography and magnetic resonance images showed diminished stenosis of common bile duct and left hepatic duct, but a new mass in segment IV of liver was observed. Unfortunately, the patient died due to disease progression.This case reminds us that although PBNHL is rare, making accurate diagnosis difficult preoperatively, PBNHL should be considered when encountering a case with Epstein-Barr virus infection and those typical imaging findings. Hepatitis B virus, hepatitis C virus, human immunodeficiency virus Epstein-Barr virus infection, elevated lactate dehydrogenase (LDH), or low immunity are associated with the development of PBNHL.–5 This paper reports a case of PBNHL that appeared like hilar cholangiocarcinoma.Primary biliary non-Hodgkin's lymphoma (PBNHL) is a rare disease with only 41 cases reported since 1982.2A 59-year-old woman was admitted to our hospital with a 3-day history of jaundice associated with generalized itching, pale stools, and dark urine. There was no history of nausea or vomiting. Oil aversion, anorexia, abdominal pain, or fever was also absent. History of weight loss was present which was about 5 kg in the past 3 months. Past medical history was unremarkable. Laboratory tests showed elevated total bilirubin, direct bilirubin, alkaline phosphatase, and LDH. Routine blood tests were regular. Immunoglobulin M antibody to Epstein-Barr virus (EBV-IgM) was positive. Immune-related laboratory tests revealed immunoglobulin G4 level was average. Tumor marker showed elevated carbohydrate antigen 19–9 (CA 19–9) and normal alpha-fetoprotein and carcinoembryonic antigen.Initial computed tomography (CT) and magnetic resonance imaging images showed diffuse thickening wall of bile ducts  with corresponding lumen stenosis and blurred fat space around portal vein. Thickening of the gallbladder wall was also noted. All of these lesions above exhibited significantly diffusion restricted and moderately homogeneous enhancement. Lymphadenopathy was found around the celiac trunk and its branches. The spleen was normal Fig.  A–D. OneThe diagnosis of hilar cholangiocarcinoma was first considered because it was combined with elevated tumor marker CA19–9 and images showing gallstones with cholecystitis.One month later, the patient and her family requested to clarify the histologic diagnosis by laparotomy biopsy. Because the biopsy result could not be defined during operation, then right hemihepatectomy and choledochojejunostomy were performed. During the operation, significant contracture of the first porta hepatis, thickening of the hepatoduodenal ligament and the wall of the right hepatic duct, stenosis of the right posterior bile duct was observed. Moreover, lymphadenopathy at the portal vein, antrum, and proper hepatic artery could be found. The postoperative pathology report showed atypical cells in group 8 of the lymph nodes were observed. Furthermore, the surgical margin was negative. The immunohistochemistry of bile duct tissue suggested that FOXPI (80%), Ki-67 (70%), Bcl-6 (60%), MUM-1 (70%), c-myc (3%), CD38 Kappa, CD43, CD20, and CD79a were positive, and Lambda and Bcl-2 were negative .PBNHL is a rare type of diffuse large B-cell lymphoma. Clinical manifestations are jaundice, epigastric pain, fever, weight loss, and abdominal mass. Biliary obstruction is a sign of poor prognosis.,11 Cholangiocarcinoma presents that the tumor marker CA19–9 increases significantly, and the extent of lesion is more localized, and bile duct stenosis is severe and stiff. The biliary stricture in lymphoma is softer than cholangiocarcinoma. The patient without any antitumor treatment, the imaging showed relief of some biliary strictures, which has been reported in lymphoma, but it has not been reported in cholangiocarcinoma.–14IAC patients have elevated serum and bile immunoglobulin G4 levels with the imaging feature of homogeneous progressive enhancement of the symmetric thickening wall (including the stenotic area and nonstenotic area). The most common location of IAC is inferior portion of the common bile duct. IAC is frequently accompanied by pancreatic inflammation.,16 At the same time, due to the rarity of PBNHL, unified treatment plan is uncertain. But it is believed that once the diagnosis is confirmed, chemotherapy is the basic and necessary treatment. Immunochemotherapy with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone or similar regimens is generally used.,17 And if biliary obstruction occurs, the biliary diversion is required. Radiotherapy has been used in some patients and can be used for patients with residual disease after primary chemotherapy, or to relieve pain. However, whether radiation therapy is an essential treatment remains to be further studied because of the rarity of the disease.,18 Besides PBNHL also lacks studies to definite prognosis. The overall survival can be less than 1 year due to disease progression caused by adverse effects of surgery or the lack of chemotherapy.,19However, establishing the diagnosis of PBNHL can be challenging by relying only on imaging and clinical manifestations. Even for biopsy, the results are often misdiagnosed as chronic nonspecific inflammation and other malignant tumors. So, surgery is inevitable to obtain enough tissue and achieve an accurate diagnosis.4Although it is difficult to diagnose PBNHL preoperatively, diagnosis of PBNHL should be considered when the bile duct is diffusely thickened without stenosis and the surrounding fat space is blurred. At the same time, factors such as infection with Epstein-Barr virus, elevated LDH, typical imaging findings, and spontaneous remission of the patient's condition are more suggestive of this disease.The authors wish to thank Jannatul Maoya Bashanti for the language polishing and Paperpal Preflight Service for writing assistance.Conceptualization: Yun Mao.Data curation: Jiamei Wu, Jing Zhang.Project administration: Yun Mao, Yin Zhou.Resources: Yin Zhou, Qingshu Li.Supervision: Yun Mao, Yin Zhou, Qingshu Li.Writing – original draft: Jiamei Wu.Writing – review & editing: Jiamei Wu, Yun Mao.


It is diagnosed by clinical features that include an orthostatic headache combined with imaging findings demonstrating intracranial hypotension and a CSF leak. We present the case of a 45-year-old woman with an orthostatic headache who was found to have a sagging brain with a downward-displaced cerebellum and pachymeningeal enhancement with gadolinium contrast. This was initially misidentified as a Chiari I malformation, but the constellation of symptoms and MRI findings were later recognized as characteristic of SIH. Diagnosis of SIH and a CSF leak was confirmed with CT myelography. She was treated with a nontarget epidural blood patch, and her symptoms resolved. An orthostatic headache, a sagging brain, and pachymeningeal enhancement on MRI are highly specific for SIH, raising suspicion for this uncommon and often missed diagnosis.Spontaneous intracranial hypotension (SIH) is a rare condition caused by a  Spontaneous intracranial hypotension (SIH) is an uncommon condition characterized by a low volume of CSF. The most common presenting symptom is an orthostatic headache, although rare symptoms can include cranial nerve palsies, behavior changes, reversible dementia, and movement disorders –5. The eA 45-year-old Hispanic woman with a history of hypertension and chronic migraines presented to a neurology clinic with a worsening headache, dizziness, tinnitus, and gait imbalance. She was also experiencing associated nausea, vomiting, and photophobia. The only things that improved her symptoms were lying supine, on her side, or taking over-the-counter pain medications containing caffeine. Her symptoms began two months prior to presentation and had gradually worsened over that period. At the time of presentation, she was afebrile and reported no recent illness or sick contacts. She reported no recent trauma. No focal neurological deficits were noted on her physical exam. A brain MRI with and without contrast was performed due to the acute worsening of her symptoms. MRI demonstrated sagging of the brain  and pachAs previously mentioned, this case was initially misdiagnosed as a Chiari I malformation. This can be common due to the overlap of clinical presentations and similarity that can be seen upon imaging. SIH has even been described as a “pseudo-Chiari I malformation” due to the striking similarities . The patDiagnosis of SIH is based on a clinical presentation including an orthostatic headache and radiological evidence of a CSF leak, usually by an MRI or CT myelography . Recent Additional MRI findings associated with SIH are subdural effusions, closure of the midbrain-pons angle, enlargement of the pituitary, flattening of the pons, and obliteration of the prepontine cistern , 17 Fig. These fCT myelography is often considered the gold standard for diagnosis, as it offers direct visualization of the spinal canal and potential CSF leaks. This involves intrathecal contrast injection and visualization of the spinal canal. This can offer further insight and can often localize tears in the dura or arachnoid. Diagnosis in this case was confirmed using this method . Lumbar Patients have been found to have improvement with hydration and caffeine, but an epidural blood patch of leak is often warranted. This procedure involves a blood draw of 20 mL or greater that is then injected into the epidural space . This isSIH is a rare and often missed diagnosis that requires careful examination of radiological signs for accurate diagnosis and distinction from differential diagnosis. The presented case highlights a constellation of MRI findings demonstrating brain sagging unique to SIH that includes downward cerebellar displacement, enlarged pituitary, closure of the midbrain-pons angle, and flattening of the pons. These findings, in combination with pachymeningeal gadolinium contrast enhancement, are specific to SIH and can provide an accurate diagnosis to guide proper management.


Primary amenorrhea is generally because of congenital defects in the uterine anatomy or because of gonadal dysgenesis. Here we are presenting a rare case of primary amenorrhea due to the uterine transection caused by a blunt trauma secondary to a road traffic accident at the age of 2 years. To the best of our knowledge, this is the only case reported.This patient presented to us at 17 years of age with the complaints of cyclical pain abdomen every month since 2 years, and it was increasing in intensity. Thorough history revealed that she had a history of blunt trauma at 2 years of age due to a road traffic accident leading to a pelvic fracture and bladder injury. She had undergone bladder repair through an emergency laparotomy and she was given conservative management for pelvis fracture. Details of the surgical procedure were not available with them. She developed secondary sexual characteristics at the age of 12 years and at the age of 15 years she consulted a doctor outside for primary amenorrhea.The doctor released labial adhesions and did hymenoplasty, after which she had spotting for 2 days. Surgical notes of that procedure were not available. At 16 years of age, she presented to us with primary amenorrhea and cyclical pain in abdomen. On clinical examination, she had tanners stage 2 thelarche with good axillary and pubic hair development. Per abdomen was soft with a midline scar. Local pelvic examination revealed normal labia and introitus.USG and MRI revealed normal retroverted uterus with normal cervix and ovaries with a small 1.5 × 1.6 cm hemorrhagic cyst in left ovary. Serum FSH was 14.5 micro-IU/ml with normal female karyotype. Diagnostic laparoscopy and hysteroscopy was suggested to the patient but they got it done outside which revealed old blood in the pelvis, bulky left ovary with a cyst and a normal right ovary with bilateral normal tubes. Uterus was seen completely transected at the lower uterine segment and seen separately from the cervix. Uterus was retroverted with posterior uterine wall adherent to the rectum with flimsy adhesions with normally placed tubes and round ligaments. Hysteroscopy revealed normal vagina and ecto cervix. Endocervical canal was normal with a small endometriotic tissue seen at the upper blind end. She came back to us for reconstructive uterine surgery. After proper counselling and pre operative evaluation she was posted for laparoscopic reconstructive surgery. Posterior wall of uterine adhesions to rectum were released and the edges of the lower uterine segment and cervix were freshened after the bladder was pushed down slightly Fig. . ThroughBladder in small children is mostly intraabdominal and is less protected by pubic symphysis. If any blunt injury occurs to the abdomen with full bladder, the bladder is most likely to rupture intraperitoneally . Blunt aEvaluation of primary amenorrhea should always start with secondary sexual characteristics development which indicates that her ovaries are normal. In this case, ovaries are well developed along with uterus and cervix as visualised on the scan. As her Ser FSH was 14.5, karyotype was also done which showed normal female karyotype. Generally we suspect genital tuberculosis  in casesFollowing basic surgical principles with good anastomoses of uterus to the cervix helped the patient to have a good approximation leading to regular periods without cyclical pain and preventing endometriosis. She was advised to go for an elective cesarean section in the future.Uterine injury as a cause of primary amenorrhea must be suspected when the girl presents with primary amenorrhea with a background of road traffic accident leading to bladder repair in childhood specially when the secondary sexual characters and uterus are normal. It is important to look at the genital structures specially uterus in a female child, while repairing the bladder injury caused due to blunt trauma.Systematic evaluation and good surgical principles are needed to treat the patient at the right time and retain her future fertility.