Neurofibromatosis comprises neurofibromatosis type 1 (NF1) and type 2 (NF2). Major tumor type of NF1 are neurofibroma recognized as benign peripheral nerve tumor, malignant peripheral nerve sheath tumor (MPNST), and glioma.We report a woman with a special condition, whose tumors in body surfaces were benign neurofibroma and tumors in posterior mediastinum are MPNST. The chest-enhanced CT suggested a round soft tissue density in posteriormediastium. The diagnosis was established by pathology and immunohistochemistry. A single-stage thoracoscopic mediastinal mass resection was performed. The whole operation went smoothly and the CT scan of lungs did not show relapse of tumor three months later.The appearance of neurofibroma should draw particular attention to the possibility of developing MPNST. More careful imaging examinations should be carried out, and pathological examination could diagnose it. Neurofibromatosis is a autosomal dominant disease that mainly implicates nervous system and causes tumor with distinct predisposition . AccordiFrom clinical experience, neurocutaneous tumors rarely associated with esophageal neurogenic tumors and related symptoms. The patient reported in this case is a 50-year-old woman with a special condition, whose tumors in body surfaces were benign neurofibroma Fig.  and tumoThis study was conducted under approval of the Ethics Committee of First Hospital of Jilin University, and the protocol was accorded with its standards.This patient was a 50-year-old woman admitted to our department due to a posterior mediastinal mass detected by computed tomography (CT) scan 7 days ago. The symptom of oppression in chest did not improve after anti-inflammatory treatment. At admission, her blood pressure, pulse rate, respiratory rate and body temperature were all in the normal range. She had no cough and expectoration, no fever, no significant chest and back pain, and no muscle weakness. She had no hypertension, heart disease, diabetes, any infectious disease or drug allergy history. At the age of 15 years old, she was diagnosed of multiple neurofibroma. At the age of 48 years old, she received the fibormectomy at a local hospital because of worsened symptoms of the left lower extremity. At day 1 after admission, the chest-enhanced CT revealed, a round soft tissue density sized 8.4 × 4.0 cm with unclear boundary with esophagus and heterogeneous enhancement in posteriormediastium, and multiple nodule-like soft tissue density shadows in subcutaneous chest and back Fig. . MeglumiBefore operation, the patient was treated with intravenous fluid resuscitation for a week and prepared for a surgery. A single-stage thoracoscopic mediastinal mass resection was performed. The patient was placed in the right supine position and anesthetized by double lumen tracheal intubation. A about 1.5 cm-long incision in the 9th intercostal axillary midline was made and thoracic exploration was performed by thoracoscope first. Then, a standard 24 cm-long lateral incision in the 7th intercostal space on the left was made. After each layer was cut, switch to single lung ventilation. No effusion or adhesions in the thoracic cavity and no space occupying lesion in pulmonary lobe were observed. The lower mediastinal pleural bulged and a mass adhered to surrounding tissue could be felt behind the esophagus and in front of the aorta. No obvious enlarged lymph nodes were found in the mediastinum. Mediastinal mass was diagnosed intraoperatively. Then, the posterior mediastinal pleura was open. After fully dissociating mass with surrounding tissues, the mass was completely resected. Rapid intraoperative pathology reported posterior mediastinal tumor. The tumor was measured about 11 × 7 × 5 cm in size with abundant blood supply and incomplete capsule Fig. a. The tuPostoperative histopathological examination showed the tumor was MPNST and measured 9.0 × 7.0 × 4.8 cm in size Fig. b. HematoNF1 with an global prevalence of approximately 1 in 3000, is more common than NF2 with a birth incidence from 1 in 25,000 to 40,000 by estimate , 7.Nf1. The result of Le et al. showed loss of Nf1 in skin-derived neural progenitors is required but not sufficient to induce tumors and suggested an essential role for the tumor microenvironment in neurofibroma development [This inherited disease is strongly associated with gene elopment .The clinical manifestations of NF1 include milk coffee spot, multiple neurofibroma, neurological symptoms, bone damage and visceral damage. As is known, neurofibromas are benign peripheral nerve sheath tumors arising from Schwann cell progenitors, of which cutaneous neurofibromas (cNF) are the most common type. The appearance of cNF varies, including nodular masses, peduncular lesions and diffuse plaques. They can be single or multiple, and are localized rather than encapsulated with no clear association with myelinated nerves . Generalvs. 30% of nerve sheath tumor) indicated the dramatic cell proliferation. The positive biomarkers of nerve sheath tumor showed a possibility of its metastasis. Neurofibromas are mixed, consisting of neoplastic Schwann cells and non-neoplastic elements [As for histology, S100 + Schwann cells were observed in surficial neurofibroma and part of posterior mediastinal MPNST. The significant difference between Ki67 expression in both tumor (1% of neurofibroma elements . Usuallyelements . Researcelements . Besideselements .MPNST is a rare neoplasm of the peripheral nervous system. Most of the tumors occur in young and middle-aged men, and most of them occur in limbs, scalp and neck . Atypica


Renal hemangioblastoma (RH) is a very rare benign tumour. Hemangioblastoma most commonly occurs in the central nervous system (CNS), and only few cases of RH have been reported as they occur most commonly as asymptomatic masses found incidentally. Mixed Mullerian tumour (MMT) of the uterus is a rarer and aggressive form of uterine malignancy. The detection of two primary rare tumours incidentally is a rare entity.A 50-year-old female presented with abnormal uterine bleeding which on endometrial sampling was diagnosed as a rare variety of endometrial cancer, i.e. MMT or uterine carcinosarcoma. On preoperative imaging, a renal mass was also detected which was highly vascular and was mimicking renal cell carcinoma (RCC). Fine needle aspiration cytology (FNAC) was done from the renal mass to differentiate between RCC and metastasis, but it showed only blood cells. Patient underwent staging laparotomy for endometrial cancer and frozen section examination of the renal mass which was inconclusive with few atypical cells, and thus, patient underwent radical nephrectomy too. Histopathological examination revealed it to be a RH which is a very rare benign tumour.RH is a rare benign tumour which does not require any treatment in majority of the patients. Only 26 cases of RH outside the CNS have been reported till date. MMT is a rare aggressive uterine tumour with an incidence of 1–2 % of all uterine neoplasms, which metastasizes early, and thus, early identification and treatment is the key. RH needs to be differentiated from RCC to avoid over treatment. Morphological findings are similar in RCC and RH; preoperative FNAC, PET scan, and intraoperative frozen section can be utilized to differentiate the two, in well-circumcised tumours and high index of suspicion. Occurrence of renal mass as an incidental finding in the preoperative work up of uterine malignancy directed us to the differentials of metastasis or another histologically distinct primary tumour. The presence of two rare primary tumours, i.e. RH and MMT in the same patient which are unrelated, is a rare entity. Hemangioblastoma, a rare benign tumour, is more commonly associated with Von Hippel-Lindau (VHL) syndrome, and its most common site is CNS. Hemangioblastoma can occur in other sites such as the kidney. To date, only 26 cases of a renal hemangioblastoma (RH) have been reported in literature; thus, it is a very rare benign tumour , 2 occurA 50-year-old woman presented to our Gynecology OPD with chief complaints of bleeding per vaginum for 4 months, pain lower abdomen, and dyspareunia for 2 months. Patient had regular menstrual cycles lasting for 6 days, occurring every 28 days till 4 months back when she developed vaginal bleeding, associated with passage of clots, pain, and sexual discomfort. She never used oral contraceptive pills or any other method of contraception. She has two children, both normal vaginal delivery with last childbirth 17 years back. Patient was detected with hypothyroidism 2 years ago and was maintaining euthyroid status on thyroxine 50 μg daily. There was no significant history of any genital, colonic, or renal malignancy in her family.On examination, patient was average built, afebrile, pulse 76/min, blood pressure 110/74 mmHg, and mild pallor was present. Abdomen was soft and did not reveal any mass. Per speculum examination revealed hypertrophied cervix and slight bleeding through external os. On per vaginal examination, cervix appeared firm and uterus was bulky, firm, and mobile with no palpable adnexal mass through fornices. Per rectal examination, she had normal rectal mucosa and soft parametrium.Patient had an endometrial sampling which was reported as a high-grade endometrial adenocarcinoma (villo-nodular type). Her haemoglobin was 8.9 gm%, and kidney and liver function tests were within normal limits. S.TSH was 2.6 uIU/ml, and Ca-125 was 165 IU/L. Pap smear was reported as negative for intraepithelial lesions or malignancy. On ultrasonography (USG) of pelvis, uterus was anteverted, bulky with increased endometrial thickness with a growth within the endometrial cavity. Computed tomography revealed bulky uterus showing heterogeneous attenuation of myometrium with foci of heterogeneous enhancement in central part and a heterogeneously enhancing mass lesion towards lower pole of left kidney suggestive of mitotic aetiology most likely RCC. Contrast-enhanced MRI of abdomen and pelvis was done, and it revealed a bulky uterus with an ill-defined myometrial-based lobulated mass lesion extending into the endometrium measuring 2.4 × 1.1 × 2.0 cm with distorted endometrial cavity with adenomyosis Fig. .Fig. 1MUSG-guided FNAC from the renal lesion was done (twice); however, it yielded only blood and no tissue in both attempts. The renal mass was radiologically enhanced and vascular, so it was presumed to be a highly vascular RCC. Patient did not agree for positron emission tomography-computed tomography (PET-CT) which could have been helpful and was thus planned for staging laparotomy for endometrial cancer and frozen section for renal mass with due counselling. She underwent extra-fascial hysterectomy and bilateral salpingo-oophorectomy with pelvic lymphadenectomy and infracolic omentectomy with frozen section from renal mass. There were no ascites or omental or peritoneal deposits. As frozen section report was doubtful and inconclusive, radical nephrectomy was performed; the counselling and consent was obtained prior for the same.On gross examination, the endometrial cavity was filled with papillary growth 2.2 × 2 × 1.5 cm which was grossly infiltrating the outer half of myometrium with areas of necrosis Fig. . There w2 and carboplatin AUC 5 once in 3 weeks for 6 cycles. Patient is healthy at present and is on regular follow-up for 15 months.The renal mass showed sheets of polygonal cells with haemorrhage with minimal atypia suggestive of RH Fig. . The tumCapillary hemangioblastoma is a benign tumour which consists of a network of small blood vessels along with lipid-laden stromal cells . The strMMT also known as uterine carcinosarcoma is a rare uterine malignancy occurring in postmenopausal females in 5th to 6th decade of life. It comprises of only 1–2% of all uterine neoplasms . It is aOn HPE, there are both epithelial and mesenchymal elements suggestive of MMT. PET scan is useful to identify unsuspected disease and extra-pelvic site or rule out metastasis –20. SurgBased on the GOG 161 study, ifosfamide/paclitaxel is considered the treatment of choice  and paclThe occurrence of rare tumours like sporadic RH and MMT in the same patient is a very rare entity. When a new tumour is found in a patient with one existing tumour, we need to differentiate it from metastasis to determine the further management and prognosis. To differentiate, a combination of history, clinical, radiological, and pathological investigation will be needed. The most appropriate way to distinguish is by HPE as it will show different cells of origin.On radiology and gross examination, RH mimics various renal neoplasms. Clear cell variety of RCC shares various morphological features with RH, making it the most common differential diagnosis. Thus, RCC may occasionally have a hemangioblastoma-like pattern making it nearly impossible to distinguish it from RH on morphological basis , 28.The clues to the diagnosis of RH are circumscribed borders, sheets of large polygonal cells with arborizing thin-walled blood vessels and pleomorphic nuclei, and paucity of mitotic figures despite prominence of atypical cells. The presence of peri-cytomatous growth patterns and intracytoplasmic lipid vacuoles strongly suggests hemangioblastoma although both tumour types have similar morphological features, such as clear cytoplasm and a vascular network , 27, 28.To confirm the diagnosis of hemangioblastoma, IHC must be done. The absence of immunostaining for cytokeratin and positive staining for α-inhibin, S100, and NSE is diagnostic of hemangioblastoma , 28.Since they can be sporadic or associated with VHL syndrome, a polymerase chain reaction sequencing analysis of the VHL gene is done to confirm the presence of mutation in exons.RCC needs to be differentiated from RH as the former is malignant and the latter is essentially benign. Each of them has a different prognosis, and differentiating the two will help avoid over-diagnosis and unnecessary treatment.RH needs to be correctly diagnosed as sporadic RH does not require further treatment, and it has a very good prognosis . AdjuvanSuch cases need a multidisciplinary approach with a team of gynaecologists, onco-surgeons, urologist, radiologist, pathologist, and medical oncologist. The key is to evaluate each tumour independently. They should be treated aggressively with the intent to cure each, so as to achieve maximum therapeutic benefit.RH is a rare benign tumour which does not require any treatment in majority of the patients. MMT is a rare aggressive uterine tumour which metastasizes early; henceforth, early identification and treatment is the key. RH needs to be differentiated from RCC to avoid over treatment. Morphological findings are similar in both; preoperative FNAC, PET scan, and intraoperative frozen section can be utilized to differentiate in between two in well-circumcised tumours and in high suspicion. The presence of two rare primary tumours, i.e. RH and MMT, in the same patient which are unrelated, is a rare entity.


Neurenteric cysts are rare, congenital lesions of the spinal axis composed of endodermal tissue arising from poor segmentation of the notochord. A 36-year-old patient presented with arm paresthesias and incontinence with imaging revealing a lesion in the C6-C7 region most consistent with neurenteric cyst. After partial resection of the lesion, the patient regained all neurological function. Here, we provide a brief overview of this rare neuropathologic entity and demonstrate surgical resection of neurenteric cyst through operative video. Neurenteric cysts are uncommon congenital spinal lesions which account for only 0.7%-1.3% of spinal axis tumors ,3. GrossA 36-year-old male presented with worsening left arm and leg paresthesias and issues with bladder control. Symptoms were noted shortly after a hospitalization for bacterial meningitis two months prior. Initially, the patient felt tingling and numbness in the left posterior arm, medial forearm, and left hand involving the third, fourth, and fifth digits. These symptoms eventually progressed to the left leg and were accompanied by episodes of urinary incontinence and ataxia.On physical exam, the patient had weakness of the left-hand intrinsic muscles (3/5), wrist extensors/flexors (4/5), and triceps (4/5); his other extremities had full strength. Sensation was diminished in the left C8 dermatome. Upper and lower extremity reflexes were normal, gait was normal, and Hoffman’s sign was negative. MRI cervical spine Figure  revealedThe lesion was most consistent with neurenteric cyst, though expanded differential could also include schwannoma, epidermoid cyst, and arachnoid cyst. Treatment of the cyst involved partial laminectomies at C5 and T1 and a C6-C7 laminectomy. This was followed by fenestration and excision of the cyst (Video Careful opening of the arachnoid was performed with sharp dissection using microscissors. Gentle dissection down the right aspect of the spinal cord, in between the C6 and C7 nerve roots, allowed for identification of the cyst. The cyst was then opened sharply and a significant amount of fluid was evacuated. At the dural entry of the right C7 nerve root, a small amount of mucinous material was resected and sent for frozen section, which confirmed neurenteric cyst. Given the ventral location of the cyst under the spinal cord, complete resection of the entire cyst was not possible.Postoperatively, the patient showed immediate improvement in left arm paresthesias and slight residual numbness in digits 3-5. The patient was discharged on postoperative day 3 and was ambulatory and voiding. Full strength was reached at two-week follow-up. On pathological analysis, the lesion demonstrated mucin-producing goblet cells surrounded by a central cystic cavity Figure  and was At six-month follow-up, the patient had complete resolution of left upper extremity dysesthesias, no residual weakness, and markedly improved numbness with residual numbness in only the distal fourth and fifth digits. Follow-up MRI Figure  shows reNeurenteric cysts are rare, congenital lesions that are usually ventral to the spinal cord in the intradural, extramedullary compartment of the cervical and thoracic regions of the spinal canal . ComposeRadiographically, neurenteric cysts are typically hypo- or isointense, non-enhancing lesions on T1 MRI and hyperintense on T2 MRI . SurgicaA 36-year-old patient presenting with arm paresthesias and incontinence was found to have a C6-C7 neurenteric cyst. We demonstrate partial surgical resection of this rare congenital tumor located in the ventral spinal cord in an accompanying surgical video.


In brain tumor surgery, an appropriate and careful surgical planning process is crucial for surgeons and can determine the success or failure of the surgery. A deep comprehension of spatial relationships between tumor borders and surrounding healthy tissues enables accurate surgical planning that leads to the identification of the optimal and patient-specific surgical strategy. A physical replica of the region of interest is a valuable aid for preoperative planning and simulation, allowing the physician to directly handle the patient’s anatomy and easily study the volumes involved in the surgery. In the literature, different anatomical models, produced with 3D technologies, are reported and several methodologies were proposed. Many of them share the idea that the employment of 3D printing technologies to produce anatomical models can be introduced into standard clinical practice since 3D printing is now considered to be a mature technology. Therefore, the main aim of the paper is to take into account the literature best practices and to describe the current workflow and methodology used to standardize the pre-operative virtual and physical simulation in neurosurgery. The main aim is also to introduce these practices and standards to neurosurgeons and clinical engineers interested in learning and implementing cost-effective in-house preoperative surgical planning processes. To assess the validity of the proposed scheme, four clinical cases of preoperative planning of brain cancer surgery are reported and discussed. Our preliminary results showed that the proposed methodology can be applied effectively in the neurosurgical clinical practice both in terms of affordability and in terms of simulation realism and efficacy. A brain tumor’s severity is mainly assessed by considering its grade and the originating tissue. Such an assessment is needed to provide the care team with an understanding of the tumor’s growth and how to match optimal treatments towards each individual . SurgeryAccordingly, the goal of this paper is to provide a methodology for applying 3D-based technology simulators  for aiding surgical planning of brain tumor interventions through an optimized repeatable process. In particular, we provide the reader with different fabrication approaches  using relatively inexpensive materials, commercial-grade printers, and open-source and freeware software with the aim of optimizing the costs and effectiveness of manufacturing. The fabrication strategy is determined based on the patient’s specific anatomy and the pre-surgical simulation goal. To explore the realism of the proposed methodology and evaluate its usefulness, we report four clinical cases that underwent neurosurgical treatment with the neurosurgery team of the Meyer Children’s Hospital in Florence. Our preliminary results showed that the proposed methodology can be employed efficiently in a neurosurgical clinical practice, ensuring increased affordability and simulation realism which in some cases enables surgeons to identify an alternative and minimally invasive surgical approach compared to the traditional one.As stated in the introductory section, brain cancer surgery is a complex procedure that is not risk-free, and possible complications can arise and even cause irreversible neurological deficits for the patient . The entBefore surgery, the time the surgeons devote a preoperative plan is essential and often determines the level of confidence they have during the intervention. During this step, the surgeon defines the surgical problem, first to fully identify all the anatomical and technical aspects of the procedure, and then to plan the approach. The first aim of the planning is to establish a correct diagnosis that starts from the visualization of multimodal data obtained from various imaging modalities. Such imaging modalities can be functional or structural such as magnetic resonance imaging (MRI), computed tomography (CT), functional magnetic resonance imaging (MRI) , computePreoperative planning is traditionally based on the identification of relevant anatomical reference points on tomographic data, on the measurement of the distance and angles between them, on the calculation of anatomical areas and volumes, and on the sketch of the possible trajectories for obtaining access to the tumor site. This process helps the surgeon to develop a correct 3D image of the problem in order to get a proprioceptive feel for the dynamics and complexity of the intervention to perform. Information on a surgical plan will ensure that the operating room staff has time to prepare for their patient and to identify and remedy potential difficulties . The surAn accurate and a high-resolution 3D reconstruction of the patient’s specific anatomy represents a major asset to the preoperative planning process. In neurosurgery, the development of virtual (VS) and physical simulators (PS) has allowed us to overcome some limitations of the traditional preoperative planning method (“in the mind” of the surgeon) . VirtualIn contrast, physical simulators can be directly palpated, easily modified according to the physician’s request, incised by real surgical instruments, and manipulated.Therefore, by using both physical and virtual simulators, it is possible to overcome the limits of traditional surgical planning, allowing the surgeon to understand the spatial relationships between vital structures and surgical targets. This in turn enables the surgeon to simulate different scenarios. In fact, the combination of virtual and physical simulation enables the identification of a set of promising surgical procedures that can be objectively compared to find the best intervention strategy. In addition, it makes the necessary clinical practices easier and safer for the treatment of patients, with the possibility for the clinicians to try procedures several times. In the state of the art research, there are some works dealing with the combination of virtual and physical simulators into a single mixed reality system, linking benefits coming from having a physical scenario to interact with and the potentialities offered by virtual reality . More spsimulation process developed in this work consists of the following phases (see (1)3D reconstruction of the patient’s anatomy;(2)Surgery virtual planning;(3)Fabrication of the bio-model;(4)Surgery simulation (the surgeon uses a hands-on bio-model to simulate the surgery).As mentioned in the introductory section, the present work provides a method for applying 3D technology for realistic surgical simulator fabrications for brain tumor treatment which integrate virtual and physical simulations. In particular, the ases see :(1)3D reWhile the 3D reconstruction is widely known, we focus on phases 2 and 3 in order to define a strategy which make the surgical procedure less invasive and provides a more realistic physical simulation.The remainder of the paper is as follows: in In the following subsections, the various phases of the simulation process are described in detail.The complete simulator manufacturing process includes some key steps represented in ®, Amira®, etc.), which has a price range of 4000 € to 6000 €, and with open source software . Within these software packages, once the segmentation of the anatomical element has been performed, the volume of the anatomical element is automatically obtained through embedded procedures, resulting in a three-dimensional configuration that can be stored in a 3D mesh file .An effective simulator for preoperative planning and simulation requires an exact patient specific geometry 3D reconstruction. The first step consists of the acquisition of patient medical images ) that provide anatomical information. More specifically, MRI better represents many soft tissues, thus enabling an accurate segmentation and 3D reconstruction of tissues such as the brain and blood vessels. Instead, the reconstruction of bones is performed from the segmentation of a CT scan, which provides a clear variation between calcium-based tissues and other types of tissues in gray values. Since information gained from different images acquired in the clinical track of events is usually of a complementary nature, a proper integration is often needed. The first step of the integration process is to find the spatial transformation that best aligns different datasets, a procedure referred to as registration . Such re®, Geomagic Design XTM, Geomagic Freeform, Materialise 3-Matic, etc., whose prices range from 12,000 € to 18,000 €) or open source . Such a process is needed to focus the simulation on a specific anatomical region, but could also be repeated to define the surgical lines needed to simulate the cranial resection process, thus leading the design to a partially operated simulator. In order to contain costs and to focus the simulator on an optimized head replica, a region of interest (ROI) can be first defined to specify the surgical interest 3D boundaries. Such an operation can be performed, using the surgeon’s instructions in a commercial 3D modeling software environment . In other words, the surgeon has to decide the position and orientation of a series of cutting planes to remove the bone and to easily reach the intervention area. The virtual simulation is carried out thanks to minimal cooperation between the surgeon and the CAD engineer.®, a mobile-based polygonal modeler running with an iOS operative system, demonstrated its effectiveness in dealing with virtual surgical planning [In detail, by using an information-sharing platform, the surgeon can autonomously manipulate 3D models and provide engineers with all the design constraints needed for the resection planes. In particular, the 3D modeler Forgerplanning . With an intuitive touch screen interface, the surgeon can quickly pan, zoom, and rotate the 3D models and can easily add resection planes to the virtual model. Once the planes are correctly positioned on the model, it is possible to simulate the surgery by cutting the bone and soft tissues defined by the planes themselves . More different surgical strategies can be simulated using such a tool, to define the best approach  to be further tested with the physical simulator. Moreover, the availability of the virtual model before and after the surgical simulation, allows us to manufacture the replica with and/or without the removed bone flap.A rigid simulator part is certainly appropriate to reproduce the bone tissue but also, when needed, to reproduce a rigid replica of the soft tissues. The latter is preferred when neurosurgeon does not need to interact with the simulator through real surgical instruments, but instead needs to simply train the proprioception and observe the spatial relationships between the anatomical elements. When the simulation requires distinguishing soft tissues from rigid tissues, the soft parts are fabricated by casting silicon materials in rigid shell molds, thus following the same fabrication process of other rigid parts.Once the virtual simulation has been carried out and the region of interest has been defined, the physical replica can be manufactured. This phase is divided into two different steps: rigid part fabrication and soft tissues fabrication.The manufacturing process of rigid parts can effectively be performed with low cost 3D printer (available starting from about 300 €). Materials like PLA (Polylactic Acid) or ABS (Acrylonitrile Butadiene Styrene)  are commFollowing negative feedback from some surgeons, wood-loaded PLA  was also tested to manufacture skull replicas. When PLA and ABS interact with the surgical drill, swarfs can be created adhering to the surface of the instrument, kneading it, and not allowing a correct and effective simulation of the cut. Wood-loaded PLA may represent a good choice to overcome this limit. In this case, the chip appears to be easily removable from the area of the tool, allowing us to easily perform the entire cutting procedure of the simulator.The separated structures need to be equipped by fixture joints to be assembled with accuracy after printing. CAD tools are employed to insert the anchoring systems between the various anatomical elements present in the ROI through the insertion of pins or other engineering/mechanical solutions. The 3D model of the parts prior to being printed, must be first processed and optimized by setting some printing parameters , which cThe last step of the bio-model fabrication consists of the post-processing of the 3D printing output. When the prototype presents a poor surface finish, high porosity, the presence of appendices, supporting materials, and unfinished surfaces, an improvement can be obtained through a sandblasting process. The next step can involve the application of a resin coating to finish the surface of the final product. The resin is brushed on the object to fill all the model’s cavities or indentations and to improve surface quality, thereby smoothing the roughness and reducing the stair stepping effect typical of a 3D printed object ,30.The low-cost soft tissues fabrication process combines 3D printing and some tissue-mimicking material casting. Soft tissue structures can be obtained by casting silicone rubbers in 3D printed molds. Indeed, the ultra-soft nature of some human tissues, such as the brain, can be replicated thanks to silicone rubber with different hardness ranging from the ultra-soft scale  to the soft scale . Other materials such as alginate, agarose, poly  (PVA), phytagel (PHY), Poly (ethylene glycol) (PEG), and polyurethanes are widely used in the literature , as theiDuring this work, four case studies were carried out, which involved the fabrication of preoperative simulators for neurosurgical interventions involving the resection of meningioma. Written informed consent was obtained from the four patients, including for publication of both subjects’ data and all accompanying images. All methods were carried out in accordance with the guidelines laid down in the Declaration of Helsinki.The first clinical case involved a sixteen-year-old girl suffering from a benign tumor at the base of the skull. The tumor was slightly compressing the optic nerve, thus making to accessing the tumor intracerebrally a hazardous process. The simulation objective was the identification of an alternative optimal surgical access option to preserve the optic nerve integrity. CT images  and MRI images  were acquired and saved in a DICOM  format. After obtaining the 3D reconstruction with Materialise Mimics, using first phase of the pipeline shown in In similar clinical cases, the pterional craniotomy is the traditional approach for accessing the tumor through the brain by opening the dura mater. Thanks to careful preoperative planning, a neurosurgeon has been able to identify a transorbital route for the resection of the tumor that enabled the removal of the bone of the orbit and provided access to the tumor, making the surgery minimally invasive and providing a low risk of brain damage.This case study focused on the fabrication of a simulator for the intervention of a 67 years old patient with a meningioma at the tentorium level. The position of the tumor required an accurate study of the geometry and spatial location of the meningioma placed near a venous sinus. The anatomical parts involved in the construction of the simulator were the skull, the tumor, the brain, the tentorium, and the falx. CT images  and MRI images  were acquired and saved in a DICOM format. Starting from the reconstructed digital 3D model obtained from CT segmentation with Materialise Mimics, the physician indicated that a cut on the skull was needed to access the tumor area. The models have been studied with the neurosurgical microscope to simulate the surgical approach with the aim of understanding the volumes, depth and spatial relationships between the tumor, brain, and tentorium in detail . Thanks The third simulator involved planning the removal of a meningioma during the clinoid process in a 55 years old patient. The simulator’s aim was to increase the neurosurgeon awareness of the spatial relationships between the patient’s brain, the cancer, and the big intracranial vessels. CT images  and MRI images  were saved in a DICOM format. A different view of the simulator is shown in This case study concerned the manufacturing of a simulator used in the case of a 76 years old patient with a meningioma located in the temporal lobe closely related to the meninges. The surgical operation involved separating the meninges from the tumor mass without affecting any tissue involved in the operation. In this case, therefore, the simulator consisted of four parts: a skull printed in PLA loaded with wood fiber, a brain, a meningioma made with different silicones as tissues with different hardness levels (the first with Eco-flex 00-50 and the second with Dragonskin 10), and meninges reproduced with a thin rubber sheet. In this case, the cut was made physically on the simulator by the surgeon, using real surgical instruments. The simulator allows resident physicians to practice craniotomy centered on the lesion and therefore primarily has an educational purpose. In the present section, an assessment of production times and costs is reported for applying the proposed method. Overall, the simulation process with the five phases described above requires about one week from the acquisition of diagnostic images to the actual surgery. The duration of each phase is closely linked to the simulator produced  and to the complexity of the surgery. Therefore, it is not possible to define a precise time for each phase, but an average time range could be evaluated based on our experience. The production costs of 3D printed parts can be evaluated according to the following equation :(1)Cp=Ced labor. When the simulators are composed of both soft and rigid parts, the material costs of the silicone rubbers and additives must be added (see Equation (2), Preoperative planning is a crucial step for any entire surgical procedure. A deep knowledge of the anatomical geometry of the patient is necessary to plan the best surgical strategy and to reduce the risk of surgical errors. Within this context, it is clear how important it is to use surgical simulators that allow the physician to simulate the operation before entering the surgical room, improving self-confidence and reducing stress during actual surgery . Specifi


Intestinal duplications are rare congenital anomaly found in the pediatric age group. Although, the ileum is the most common site, there are cases of colonic duplications even in the adult. Colonoscopy is a good investigation tool for the diagnosis of colonic duplications; however, it may not be true in all cases. We report a case of 43 years woman presented with chronic constipation, intermittent colicky abdomen pain, and a cystic lump in the left abdomen diagnosed as tubular duplication cyst of descending colon. She was managed with left hemicolectomy and excision of the cyst with uneventful postoperative days. This case has been reported as it is a rare condition. Ileum is the most common site (60%).A 43 years woman presented to gastro-surgery OPD in Blue Cross Hospital with painful cystic swelling on the left upper and lumbar region extending to the central and right upper abdomen associated with chronic constipation for 2 years. The pain was colicky, intermittent, mild, which was increasing in the recent months associated with a gradual increase in the size of the swelling. On examination, there was 12 cm x 8 cm, cystic swelling in the left hypochondrium, and the lumbar region extending up to the umbilical and right lumbar regions.Routine hematological and biochemical parameters were within normal limits. However, the ultrasonogram showed a 12 cm x 8 cm cystic lesion in the left lumbar region. Contrast-enhanced computed tomography (CECT) scan showed, large cystic dilation of large bowel involving transverse colon with gas and faces indicating a possible diagnosis of mega-colon. Colonoscopy showed normal findings. Barium enema revealed, duplication cyst arising from the distal transverse colon with the blind end extending up to the right upper quadrant .With the clinical diagnosis of the duplication cyst of the colon, she underwent left hemicolectomy and excision of the duplication cyst. Intraoperative findings revealed 35cm of tubular duplication cyst of the mid-transverse colon and the descending colon with the distal blind end attached to the retroperitoneum behind the second part of the duodenum  and 3.The cyst and the normal functioning colon shared common vascular supply. There was a small communicating ostium between the main bowel and the cyst at the mid transverse colon.The final pathological diagnosis was a tubular duplication cyst of descending colon with atrophic mucosa and few areas of strictures. Sections from the saccular duplicated colon show colonic tissue composed of mucosa, submucosa, muscularis propia, and serosa without any dysplasia or metaplasia. Muscularis propia is irregular and thickened .The postoperative days were uneventful. The patient started on a liquid diet on the third postoperative day and was discharged on the sixth postoperative day. The patient was followed up after two weeks and 2 months on OPD, which were unremarkable.4 It is in the cystic form in more than 80% of cases and tubular in the rest. Colorectal duplication represents only 13% of all gastrointestinal duplications.5 Tubular duplication cysts are commonly associated with genitourinary abnormalities. There has been found an association of colonic duplication and colo-vesical fistula.6Enteric duplication cysts are rare congenital abnormalities commonly seen in pediatric populations, which occurs commonly in the small bowel. It has a smooth muscle coat and a mucosal lining.7 However, in our case colonoscopy has failed to diagnose the pathology possibly due to small communication between the cyst and normal colon.It presents with a wide variety of clinical signs and symptoms causing difficulty in clinical and radiological diagnosis. The common presentation is colicky abdominal pain with or without abdominal distension or a lump and long-standing constipation. Now a day, the liberal use of newer diagnostic modalities like CT scan, MRI, and colonoscopy have helped to come to the diagnosis. Colonoscopy has reported being helpful in the case of larger ostium between the normal bowel and cyst.8 Type I: pseudo diverticulum with a wall consisting mainly of granulation tissue. Type II: inflammatory diverticulum, secondary to a perforation of the mucosa and submucosa communicating with the lumen. Type III: true diverticulum, all bowel layers, and communicating with the main lumen. Type III diverticulum is similar to a communicating duplication cyst. According to Choong and Frizelle, Type I diverticulum is a pseudo diverticulum with a fibrous wall with no muscular layer and type II is a true diverticulum with a muscular wall.9 Regardless of the classifications, giant colonic diverticulum may be difficult to distinguish from duplication cyst, when it becomes elongated. Our case was a tubular duplication cyst of the colon, as it was very elongated, not associated with other diverticular diseases of the colon, and lined by normal colonic mucosa free from dysplasia and metaplasia.Duplication cysts, if not associated with other abnormalities, may go undiagnosed until complication develops in adult life. It may be commonly mistaken for giant diverticulum, as its blind end appears large air-filled cysts as in our case. According to McNutt et al, the diverticulum is divided into three types:Recommended treatment for duplication cyst is resection of the part and anastomosis between the normal bowel either laparoscopic or open as it eliminates the risk of future malignancies in it. In our case, we performed left hemicolectomy due to the predominant part of the blood supply to the normal colon was from mesentery supplying to the cyst.Our aim of reporting this case is to highlight the rarity of the condition, vague clinical presentations, and its difficulty in diagnosis with other entities. Clinical suspicion is necessary for an adult presenting with cystic abdominal lump with chronic constipation and colicky abdominal pain.In a patient with chronic constipation, colicky abdominal pain, and the cystic lump in the abdomen, duplication cyst of the colon should be considered in the absence of other diverticular diseases as it presents with a wide spectrum of clinical signs and symptoms.


Introduction. Restoring lateral ankle stability following distal resection of the fibula is a difficult procedure for which several surgical techniques have been proposed. Each of these techniques has potential drawbacks. This report presents a new option for fibular reconstruction. Case Study. We report the case of a 68-year-old male with evolving pain in the left ankle throughout the past 3 months. Three years prior to consultation, he underwent left nephrectomy for clear-cell adenocarcinoma. A swelling on the external side of the left ankle was noticed upon clinical examination, with no signs of inflammation. The ankle was stable with normal mobility. Radiographic examination revealed a 4 cm lytic lesion on the lateral malleolus with internal and external cortical damages as well as invasion of the soft tissues. Neither lower peroneotibial nor tibiotarsial joints were invaded. Needle biopsy confirmed the presence of metastatic renal clear-cell adenocarcinoma. Consequently, large exeresis of this single metastasis was indicated while preserving functional integrity of the ankle. Following block resection of the distal fibula including the lower tibioperoneal joint, a bicortical autograft was positioned to abut against the external side of the talus. Emslie-Vidal's ligamentoplasty procedure was performed with half of the short peroneal passed under the pedal flexor, then in the bone abutment, and finally through a calcaneal bone tunnel. Peroneus muscles were stabilized using a fragment sampled from the Achilles tendon. Pain decreased in 3 months, and the ankle was stable with normal functionality at a 5-year follow-up. Discussion. Reconstruction of the lateral ankle following fibular resection is possible by reconstructing the external facet of the malleolus using an autograft associated with Emslie-Vidal's ligamentoplasty procedure, hence stabilizing both tibiotalar and subtalar joints. This surgical procedure allowed the patient to return to his daily activities with neither instability nor evolution towards short-term tibiotalar arthrosis. Metastatic renal-cell carcinomas localized on the fibula are rare but occasionally require total exeresis , 2. ReseA 68-year-old male in 2012 consulted for pain in the left ankle evolving throughout the past 3 months. Three years prior to consultation, he underwent left nephrectomy for T1N0M0 clear-cell adenocarcinoma. A swelling on the external side of the left ankle was noticed upon clinical examination, with no signs of inflammation. Articular motions were not limited, with the dorsiflexion angle measured at 10° and the plantar flexion angle measured at 15°. The ankle was stable. Radiographic examination showed a 4 cm lytic lesion on the lateral malleolus. Scan images revealed osteolytic lesions along with internal and external cortical damages as well as invasion of the soft tissues .Neither lower peroneotibial nor tibiotarsal joints showed signs of invasion. Scintigraphy revealed hyperfixation on the lateral malleolus. PET scan showed hypermetabolism localized on the left lateral malleolus as well as on the 7th thoracic vertebra and on the left iliac wing. MRI did not find any spinal anomalies and revealed a benign infarct on the iliac wing.Needle biopsy confirmed the presence of a metastatic renal clear-cell adenocarcinoma. Consequently, a large exeresis of this single metastasis was required while preserving the functional integrity of the ankle.The patient was positioned in dorsal decubitus. The operation was carried out with a lateral approach centered on the lateral malleolus. En bloc resection of the tumor was performed, carrying the lower tibioperoneal joint. A bicortical graft was sampled from the anterior iliac crest and positioned to abut against the external side of the talus. Emslie-Vidal's ligamentoplasty procedure was performed with half of the short peroneal passed under the pedal flexor, then in the bone abutment, and finally through a calcaneal bone tunnel . PeroneuPostoperative follow-ups were simple and the patient progressively regained support over 3 months. He was helped by physiotherapy. Anatomopathology confirmed metastasis of a clear-cell renal carcinoma with nonlesional tissue edges.At one and a half months postoperative, mobility of the ankle was slightly limited (dorsiflexion +10° and plantar flexion at 15°) and control scan showed consolidation of the graft .At seven months postoperative, the patient walked with a good stepping motion. The ankle was painless. Tiptoeing and walking on heels was possible. Motions were similar to the ones displayed one month postoperative with a 5° less angle for plantar flexion compared to the right ankle. The ankle was stable during clinical evaluation. Radiographic images showed integration of the graft. There was no reduction of the graft.At one year postoperative, good stepping motion was found at clinical evaluation; the trophic state is good with no signs of inflammation. Ankle mobility remains unchanged with a +15° dorsiflexion during knee extension and +15° plantar flexion. There was no ankle laxity; palpation of the bone graft was slightly sensitive. There was no pain during palpation of the peroneus longus tendon or during contraction of this muscle. Palpation of the Achilles tendon was painless .At 5 years postoperative, there was excellent functional result . There wTumors located on the lateral malleolus are rare, including renal adenocarninoma metastases . Which tSchuurman and Willems  describeA few cases of distal fibular reconstruction using an allograft can be found in the literature. In 1985, Lublinar et al. [Another case was described in 2004 by Sirveaux et al.  in NancyFinally, Jamshidi et al.  reconstrLeibner et al.  proposedCapanna et al.  also pubDe Gauzy et al.  used a vAnkle prosthetic reconstruction was performed by Lee et al.  in a 36-More recently, Vaseenon et al.  describeMonson et al.  also desHere, Emslie-Vidal's ligamentoplasty procedure associated with an iliac graft was chosen in order to reconstruct the ankle while preserving mobility and stability while allowing total exeresis of this single metastatic lesion. This procedure was the most suitable in our opinion for several reasons.It has the advantage of using an autologous graft allowing the reconstruction of the external malleolus with a little osteosynthetic material, contrary to allograft reconstruction using osteosynthesis plates, which is nonnegligible given the cutaneous risks associated with every surgery centered on the external malleolus. The use of an autologous graft also eases the reconstruction by allowing the sampling and grafting of a piece, whose size perfectly corresponds to the morphology of the patient's talar articular surface, whereas the allograft would require a compromise on the size in cases of up- or downsizing.Moreover, this tumoral surgery requires a rapid scheduling of the patient in order to prevent evolution of the tumor. The availability of the allograft in France is very variable and not centralized, which does not allow for carcinological surgery within short time periods . MoreoveFinally, Emslie-Vidal's ligamentoplasty associated with this autograft allows for a perfect stabilization of both tibiotalar and subtalar joints. The first part of the transplant ensures good control over stability in an equinovarus position and the second part, of which the direction is perpendicular to the subtalar plan, controls the pure varus with the ankle at a right angle at the level of the tibiotalar and subtalar joints.Another solution would have been ankle arthrodesis with a risk of nonconsolidation and at the cost of complete limitation of motions.At a 5-year follow-up, the 30/30 ISOLS score is representative of the very good result obtained with this technique which seemed the safest to us and the most suitable in order to obtain a satisfying functional result.Block resection of the distal fibula leads to ankle dysfunctions and long-term valgus deformation as well as laxity in the frontal side with degenerative arthrosis. Our autograft technique allowed the reconstruction of this lateral malleolus along with the stabilization system of the ankle using Emslie-Vidal's ligamentoplasty with a very satisfying functional result in terms of mobility. Thanks to our procedure, this T1N0M1 patient was thus able to recover his former quality of life with a painless and functional ankle.


Xanthogranuloma is a rare benign tumour, part of the non-Langerhans cell histiocytosis group, uncommon in adults and even less common in the paranasal sinuses. Despite its benign nature, it mimics neoplasm due to its local effects which can have serious functional consequences depending on the anatomical location. We present the rare case of a young lady who presented insidiously with a maxillary sinus xanthogranuloma and was treated with endoscopic resection. Tissue diagnosis is of paramount importance to guide correct further investigations and management, and we discuss the potential challenge in identifying such a rarely seen pathology. Histiocytes are a type of phagocyte derived from dendritic cells or macrophages and are produced in the bone marrow. These cells can travel haematologically to distant tissues with the potential to cause pathological proliferation and deposition, resulting in local tumour formation . SeveralDue to the variable anatomical involvement, clinical presentation and diagnosis can be challenging and is largely based on detailed specialist histological analysis.We present the rare case of a young lady who presented with nasal symptoms and a unilateral sinonasal mass, which was found to be an adult-onset xanthogranuloma.A thirty-five-year-old Caucasian lady was referred by her GP to General ENT clinic after presenting with a mechanical fall and head injury, who then developed a sudden onset anosmia, ageusia, and worsening right-sided nasal obstruction and discharge, which was occasionally blood-stained. This was associated with right-sided facial pain which radiated to her right ear and perioral region. She was otherwise systemically well. She had no significant past medical history, took no regular medication, was a nonsmoker, and worked as a journalist with travel to Rio de Janeiro and rural Uganda in the preceding two years.Flexible nasendoscopy revealed what appeared to be an enlarged and oedematous right middle turbinate obstructing the middle meatus, with surrounding mucopus tracking back to the postnasal space. Remaining head and neck examination including cranial nerves was unremarkable.An urgent contrast-enhanced CT scan of the sinuses was performed  which shFurther evaluation with a contrast-enhanced MRI scan of the sinuses  demonstrThe histology  showed aShe remained well and asymptomatic one year on from her excision, and follow-up imaging was reassuring of no disease recurrence .Non-Langerhans histiocytic diseases are poorly understood and diagnosis is challenging due to the multiple inconsistent definitions of the disease in the literature. The Histiocyte Society provided the clearest classification system in 1987, which was subsequently updated in 1997 by the WHO  2]. The. The2]. Differentiation between the various classes and subtypes is made histologically with immunohistochemistry as first identified by Weitzman and Jaffe . Class 1Xanthogranuloma, a non-LCH, is typically a disease of childhood and adolescence, known as juvenile xanthogranuloma (JXG), manifesting as cutaneous lesions predominantly in the head and neck, but less commonly the trunk and extremities. Compared to other non-LCH variants, JXG lesions are often solitary . AlthougAdult-onset JXG was first reported in 1963 by Gartmann and Tritsch, and since then, it is estimated that 10% of JXG is found in adults with an equal sex distribution and median age of 35 years , 14. DesThe lesions of adult-onset xanthogranuloma are similar to JXG; however, they may be larger and do not present with hyperlipidaemia, in contrast to JXG, as was the case in this patient. Whilst histiocytosis can be multicentric, this is not typically seen in adult xanthogranuloma, and therefore systemic medical management with immunosuppressive or corticosteroid therapy is usually not indicated. Furthermore, the rate of disease regression is reportedly 54% in adult disease compared to 83% in JXG, though the prognosis is remarkably favourable .Xanthogranuloma in the paranasal sinuses is not a widely reported phenomenon with only a handful of cases in the English literature. Symptomology depends on the sinonasal site involved, but typical clinical features include nasal obstruction, epistaxis, and focal pain. It is sometimes regarded as part of a disease group known as “inflammatory pseudotumours” . Though Given the benign nature of the disease, management is usually symptom-driven, and the gold standard is surgical resection; however, complete resection may not be achieved if key anatomical structures are at risk such as the orbit or skull base and such cases require the review of a head and neck multidisciplinary team. Whilst chemo- and radiotherapy have reportedly been used in LCH, the role of this is not fully established in non-LCH .Klebsiella rhinoscleromatis infection [On reflection of the presentation of this case, it is likely that her previous head injury coincided with progression of the mass lesion. Nevertheless, the diagnostic process proved to be a challenge to both surgeons and histopathologists requiring exclusion of other more common conditions affecting this anatomical region such as Rosai-Dorfmann disease or rhinoscleroma through specialist immunohistochemistry, microbiology, and mutational analysis. Rosai-Dorfmann disease is a benign histiocytic disorder, derived from a macrophage lineage, and may clinically present in a similar fashion to xanthogranuloma but is typically associated with massive lymphadenopathy and “B” symptoms. It also expresses positivity to S-100, CD68, and CD163 but is negative for CD1a . Rhinoscnfection , 6. It iXanthogranuloma is a benign type of histiocytosis rarely seen in adults and rarely affects the sinonasal cavitiesIts presentation within the sinonasal cavity often clinically and radiologically mimics that of a neoplastic process which should be ruled out in the first instanceHistiocytic disease encompasses a spectrum of disorders which have variable clinical presentation and course, and hence subtype diagnosis requires detailed histopathological analysis with immunohistochemistry staining.Surgical resection is the mainstay of treatment and symptom relief but with careful consideration to surrounding anatomical structures.


In North Africa which is an endemic region for Hydatid Cyst, Tunisia is considered as an endemic country. The liver and lungs are common locations for Hydatid Cysts, whereas the Adrenal Glands are unusual and rare locations.Here is a report of primary Hydatid Cyst in a 55-year old patient, with left hypochondrium pain as chief complain. No remarkable findings were revealed by physical examination and blood analysis showed normal range. Hydatid serology was negative. The diagnosis of Hydatid Cyst was suspected based on CT Scan results which showed a well-circumscribed, non-enhanced, multi-cystic, 12 cm mass with scattered calcifications located in the left adrenal gland. Therefore, the patient underwent an open surgery with resection of the protruding dome of the cyst as it was attached to the renal pedicle, the pancreatic tail, the spleen and the jejunum. The final pathological examination of the specimen led to a Hydatid Cyst.Throughout an extensive literature review that we have made, we have analyzed 54 reported cases, with their clinical presentations, biological exams, radiological features and surgical managements. The treatment should be surgical and has to be as conservative as possible. The prevention of the parasite transmission has to be the cornerstone of the disease management.The hydatid cyst of the adrenal gland remains a rare diagnosis that has to be evoked in case of an adrenal gland cyst, especially in an endemic country. It is c2A 55-year-old patient, living in an endemic area, was referred to our institution for pain in the left hypochondrium evolving for 6 months, with no other symptoms such as: nausea and vomiting. Arterial blood pressure was within normal limits. No past medical history was reported. No anomalies were detected in the physical examination. Blood analysis including complete blood count, 24-h urinary VMA and metanephrine were normal. Serological analysis of the blood was negative for Echinococcus IgM and IgG. An abdominal sonography showed a large hypoechoic retroperitoneal mass in the upper pole of the left kidney containing internal cystic component. The CT scan showed a well-circumscribed non-enhanced multi-cystic 12 cm mass with scattered calcifications located above the left kidney and the left adrenal gland which seemed shadowed . The mas3Hydatid cysts are usually found in the liver or the lungs. It occurs when humans ingest accidentally echinococcus eggs found in the dogs’ stools. After passing through the digestive system, the larvae are released; they pass through hepatic filter to the lungs. If they are not destroyed by the immune system or trapped in the filtration system; they can spread anywhere in the body, giving unusual locations of the hydatid cyst as spleen, kidneys, brain, bones, heart, muscle tissue, pancreas, retroperitoneum, thyroid and adrenal glands, through the arterial circulation. Other mechanisms of the dissemination of the larvae imply entering the systemic circulation through lymphatic vessels, or by adjacent contact.The adrenal location of the hydatid cyst is rare and accounts for less than 0.5% off all hydatid cysts ,4. It geBased on a systematic Pubmed search using the keywords ‘Adrenal Gland’ and ‘Hydatid Cyst’, we have found 76 cases published in the literature. We have excluded those who could not be opened. We have selected therefore 54 cases for review. All the cases are summarized in . The datAdrenal hydatid is more likely to be discovered during the 5th decade. The mean age in our review was 50 years. It occurred equally in men and women. It is most frequently discovered incidentally during surgery or on radiology, as autopsies reports shows an incidence between 0.06 and 0.18% with 92% of the lesions being unilateral . In our The radiological findings can orient the diagnosis. Ultrasound remains the first exam performed for this localization. The depth of the adrenal glands and sometimes peripheral calcifications make this exploration difficult. The computed tomography scan then allows for a better understanding of the location and relationships with the surrounding organs . But oftThe most recommended surgical procedure is the resection of the protruding dome with drainage of residual cavity. A pericystectomy of the hydatid cyst, or, if this is not possible, total excision of the adrenal gland can also be performed. Either laparoscopic or open procedure can be used ,13, depeLaparotomy can be performed either by intercostal lumbar access with or without resection of the rib; or transperitonal anterior access allowing a sufficient view on the liver in case of associated localization. In our case, we performed a left subcostal incision, finding an accolated cyst to the pancreas, jejunum and renal pedicle. It is important to protect the operative field with compresses soaked in hypertonic (20%) sodium chloride solution to prevent larvae dissemination in case of accidental opening of the cyst intraoperatively. Conservative management of the adrenal gland is the standard attitude, except in the case of adrenal gland destruction by the cyst. In most of the cases, post-operative course is uneventful. Surgical removal of the hydatid cyst may normalise the hypertension if it was associated to the hydatid cyst preoperatively. We found normalization of the hypertension in 63% in our review. Among the published cases, no recurrence has been reported, as the case of our patient 10 months after the surgery. Post-operative albendazole prophylaxis is still controversial, even if some authors recommend it. The prevention of hydatic transmission remains an indispensable measure to avoid hydatid disease whatever his location is.4The hydatid cyst of the adrenal gland remains a rare diagnosis that has to be evoked in case of an adrenal gland cyst, especially in an endemic country. Clinical presentation and physical examination remain unspecific. CT Scan in combination with hydatid serology help raise the diagnosis, that is confirmed by the final pathological examination of the specimen. The treatment is surgical and has to be as conservative as possible. The prevention of the parasite transmission has to be the cornerstone of the disease management.The authors have no conflict of interest to declare.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Given the nature of the article, a case report, no ethical approval was required.Written informed consent was obtained from the patient for publication of this case and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.-Skander Zouari: Writing - original draft-Marouene Chakroun: Writing - review & editing.-Hana Bibani: Writing - original draft.-Ahmed Saadi: Writing - review & editing.-Anis Sellami: Data interpretation of the radiological findings.-Linda Haj Kacem: Data interpretation of the pathological findings.-Ahlem Blel: Data interpretation of the pathological findings.-Bouzouita Abderrazek: Project administration.-Amine Derouiche: Supervision.-Riadh Ben Slama: Study concept and design, data collection-Soumaya Rammeh: Data interpretation of the pathological findings-Haroun Ayed: Supervision; Reviewing and editing-Mohamed Chebil: Supervision; Reviewing and editingThis does not apply as it is a case report of a patient who has given written consent and has been de-identified. It is therefore not prospective research involving human participant.Dr. Skander Zouari.Not commissioned, externally peer-reviewed.


Neuroendocrine tumors are aggressive and rare tumors which can occur almost everywhere in the body. The annual incidence of neuroendocrine tumors is 2.5-5 per 100000. We report seven cases of gastrointestinal neuroendocrine tumors which were diagnosed and treated at our hospital from the time period of 2016-2018 knowing that the total number of our hospital tumor board cases registry during the same period was 444 cases. Generally, Neuroendocrine tumors (NETs) are rare tumors comprising ~2% of all malignancies  with theNeuroendocrine tumors (NETs) are a heterogeneous group of epithelial neoplastic proliferations arising in many body organs. Irrespective of their primary site and of their grade of differentiation, neoplastic cells share features of neural and endocrine differentiation: the “neuro” property is based on the identification of dense core granules that are similar to dense core granules (DCGs) present in serotonergic neurons, which store monoamines, and the “endocrine” property refers to the synthesis and secretion of these monoamines which is about two-thirds of NETs that arise in the gastrointestinal tract and pancreas , 3.This case series describes seven cases of gastrointestinal NETs diagnosed and presented in the tumor board at our hospital from the time period starting from 01/01/2016 till 31/12/2018 as shown in A 60-year-old male presented with abdominal pain and vomiting with constipation for 4 days; CT of the abdomen showed an enhancing mass at the ileocecal area including the appendix; he was diagnosed as having complete intestinal obstruction and underwent right hemicolectomy; histopathology report showed goblet cell carcinoid of the appendix. The patient is doing well with follow-up with an oncologist.A 20-year-old female presented with right iliac fossa abdominal pain for 2 days, was diagnosed as having acute appendicitis, and underwent appendectomy; histopathology report showed a well-differentiated neuroendocrine tumor of the appendix. Patient is doing well with follow-up with an oncologist.A19-year-old female presented with right iliac fossa abdominal pain for 1 day, was diagnosed as having acute appendicitis, and underwent appendectomy; histopathology report showed a well-differentiated neuroendocrine tumor of the appendix grade 1. Patient is doing well with follow-up with an oncologist.A 52-year-old male presented with abdominal pain and vomiting with constipation for 3 days; CT of the abdomen showed an enhancing mass at the ileocecal area including the appendix as shown in An 80-year-old female presented with abdominal pain and weight loss for 4 months; ultrasound of the abdomen showed multiple liver lesions. CT of the abdomen showed multiple liver lesions with an enlarged appendix as shown in A 22-year-old male presented with right iliac fossa abdominal pain for 2 days, was diagnosed as having acute appendicitis, and underwent appendectomy; histopathology report showed a low-grade neuroendocrine tumor of the appendix. Patient is doing well with follow-up with an oncologist and planned for right hemicolectomy.An 86-year-old male presented with vomiting of blood for 2 days, underwent gastroscopy, and showed ugly-looking gastric ulcer; histopathology report showed a low-grade neuroendocrine tumor. Patient is following up with an oncologist.As neuroendocrine cells are distributed among the whole body, so NETs have been described in multiple organs such as the central nervous system, respiratory tract, the larynx, gastrointestinal (GI) tract, thyroid, skin, breast, and urogenital system. The GIT and lung are the most common primary sites for NET , 2. ThisThe pathologic classifications of neuroendocrine tumors across different organ systems use a range of site-specific terminologies and criteria, creating significant confusion among pathologists and treating clinicians . The mosWe have to mention that the term carcinoid has been criticized because it was used to describe different tumors distinct in their etiology, prognosis, and management, leading to terminological confusion and diagnostic unreliability , and alsThe improvement of the current diagnostic techniques has led to an increased number of patients diagnosed with GIT NET, and, in contrast with the past, most of the tumors belong to the so called “nonfunctioning” category and not associated with symptoms and signs of hormone hypersecretion . The diath edition [Commonly measured tumor markers in NETs include serum CgA and 5-HIAA, the final secreted product of serotonin, levels in a 24-hour urine sample , and act edition .At our hospital, every case with a histopathological diagnosis of NET should be presented within the weekly tumor board  after completion of the workup—radiological and laboratory—and treatment plans are constructed for each case specifically, based on multiple parameters including the stage, performance status, and the clinical picture as shown in There are different treatment modalities that can be used in the management of NET as shown in Tumor characterization Patient's clinical status Defining the objectives of careActually, the optimal therapeutic sequence should be based on the evaluation of at least three major issues :Tumor cFortunately, 5 of our patients were diagnosed in early stages and thus, these 5 patients were treated radically aiming for cure and they underwent radical surgeries; 2 cases underwent upfront right hemicolectomy, and 2 cases underwent appendectomy, whereas the last case underwent appendectomy, but based on the histopathological findings and the discussion of the case within the tumor board completion, right hemicolectomy was done. Based on the clinical picture and staging of these 5 cases, no adjuvant treatment was required. One case presented with a metastatic disease to the liver, and she presented in poor general condition, and unfortunately, she died without starting any treatment. All patients who did radical surgery were following in surgery and oncology clinics; follow-up consists of clinical examination and abdominal imaging .Despite the increasing number of patients diagnosed with GIT NET and also the increasing knowledge within this field, still, most of the cases are diagnosed in advanced stages; thus, a higher incidence of suspicion is required. Also, there are still some controversies regarding the sequence of systemic treatments used in the management of GIT NET that needs more clarification.


Myxofibrosarcoma is a soft tissue neoplasm that usually affects the extremities of the elderly. It usually presents as a slow-growing painless mass that can metastasize to the lung and bone. However, the reported incidence of primary osseous myxofibrosarcoma is very low. Moreover, the metastatic pattern of these bone tumors is largely unknown. We describe a unique case of a young Caucasian male with symptomatic low-grade myxofibrosarcoma arising in the left clavicle, who presented with multiple bone metastases. Myxofibrosarcoma, previously known as “myxoid variant of malignant fibrous histiocytoma”, is a spindle cell neoplasm with a wide spectrum of nuclear atypia and cellularity, embedded in a variably myxoid stroma with typical curvilinear blood vessels . AlthougA 32-year-old Caucasian male with a history of diabetes mellitus noted mild discomfort and swelling of the left shoulder in November 2016. Despite analgesics and six weeks of physical therapy, his pain continued to worsen. Due to persistent symptoms, a CT of the neck was obtained that showed a 3.3-cm expansile lytic lesion at the left clavicular head  stain for MUC-4, which is a sensitive and specific marker for low-grade fibromyxoid sarcoma, appeared negative in the scant tissue available. Differential diagnosis at this time included a low-grade fibrosarcoma, desmoplastic fibroma and possibly a low-grade fibromyxoid sarcoma.A positron emission tomography CT (PET-CT) scan was then performed, which demonstrated hypermetabolic (F-18 fluorodeoxyglucose [FDG]-avid), lytic lesions involving the left clavicular head , S100, MUC4 and CD117 was negative with a patchy positivity for CD34. These non-diagnostic needle biopsies prompted an open biopsy of the left clavicular head in May 2017 Figures , 6, whicDue to the presence of multiple bone lesions, a potentially curative surgical resection to remove all the lesions was not feasible. In October 2017, he underwent palliative resection of the left clavicular mass with left proximal humerus lesion curettage, cementation and plating. Repeat CT scans demonstrated progressive disease with a marked increase in tumor burden with new skeletal lesions, involving the sacrum, lumbar and thoracic spine, iliac bones, sternum, right clavicle and scapula. Due to the widespread nature of the disease, radiation therapy was not a feasible treatment option. In November 2017, he was started on systemic chemotherapy consisting of cisplatin plus adriamycin alternated with methotrexate (MAP regimen) for metastatic primary myxofibrosarcoma of the bone. However, he developed acute kidney injury and elevation of liver transaminases after cycle 1, requiring interruption of chemotherapy. He would then go on to require prophylactic nailing of the left femur for impending fracture in April 2018. In June 2018, he was found to have new pulmonary metastases, and he eventually succumbed to his progressive disease in December 2018. Myxofibrosarcoma is a soft tissue neoplasm accounting for approximately 20% of all soft tissue sarcomas . In 1977Myxofibrosarcoma shows a wide spectrum of cellularity, atypia and proliferative activity; however, distinctive histomorphological features such as multinodular growth with incomplete fibrous septa and a myxoid stroma are present in all cases ,10. ThisMyxofibrosarcoma is one of the most common sarcomas of the elderly, usually presenting as a slow-growing painless mass on the extremities ,10,12. OMyxofibrosarcoma is known to metastasize to the bone , but theMyxofibrosarcoma is a myxoid soft tissue neoplasm, with an extremely rare incidence of a primary osseous origin. To the best of our knowledge, this is the first reported case of primary myxofibrosarcoma of the bone presenting with multiple skeletal metastases (bone to bone metastases).Due to the use of a variable nomenclature in relation to the tumor in the past, the true incidence of this entity may have been underestimated. The absence of a specific immunostaining pattern with no known diagnostic molecular markers may have further contributed to the low reported incidence of this neoplasm.


Background: Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies, which arise in children and adolescents, with only 9 cases reported in patients over 30 years of age. Due to its rarity, MRI features and treatment strategies for primary intracranial ES/pPNETs remain unclear. The purpose of this study was to explore the clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in adults.Case Description: A 55-year-old female was admitted to the hospital with memory decline over 1 month, which aggravated in the last 2 weeks. MRI showed a 4.3 × 6.5 × 3.5 cm heterogeneous large mass in the left frontal lobe with mild peritumoral edema. The mass was successfully removed under neuronavigation and electrophysiological monitoring. The entire mass was removed, and postoperative pathology indicated an ES pPNET diagnosis, with an EWSR1 gene rearrangement. Subsequently, the patient underwent disciplinary radiotherapy.Conclusion: The diagnosis of primary intracranial ES/pPNETs depends on the comprehensive consideration of histological examination, immunohistochemical analysis, and genetic detection. Gross tumor resection combined with radiotherapy and chemotherapy might be the most beneficial treatment. EWSR1 gene with a member of the ETS gene family is thought to be the primary cause of the ES/pPNETs (in situ hybridization (FISH). Moreover, we have concluded the patients over 30 years of age, and summarized the typical pathological and radiological features of this rare tumor entity, with surgery, adjuvant therapy, prognosis, and important differential diagnoses discussed in detail.Primary intracranial ES/pPNETs are a group of poorly differentiated, highly malignant, and aggressive small round cell neoplasms, generally originating from bone and soft tissue, which are common among children and adolescents –4. ES/pPS/pPNETs –15. HereA 55-year-old female patient was admitted to the hospital due to progressive memory decline for over 1 month, which aggravated in the last 2 weeks. Physical examination showed normal higher mental functions. Left limb muscle strength was normal; right limbs were slightly weaker than left limbs.Magnetic resonance imaging (MRI) showed a 4.3 × 6.5 × 3.5 cm large irregular mass located in the left frontal lobe with mixed isointense-to-hypointense signals on T1-weighted imaging (T1WI), uneven hypointense-to-hyperintense signals on T2-weighted imaging (T2WI), and T2 dark-fluid. After gadolinium administration, obvious heterogenous enhancement was observed . ComputeA preoperative clinical diagnosis of meningioma was made, and surgery was performed under preoperative neuronavigation and intraoperative electrophysiological monitoring of somatosensory, and muscle-evoked potentials. The tumor mass was soft, rich in blood supply, and invaded into the sagittal sinus from the left side. The tumor and eroded dura were completely removed.in situ hybridization (FISH) with a EWSR1 break apart probe showed 41.0% of split signals, thereby confirming the diagnosis of ES/pPNETs , hemiplegia and muscle strength decrease , facial palsy , deafness and hearing disturbance , drowsiness and fatigue , epilepsy , memory decline , and ataxia . The mean duration of clinical symptoms was 5.9 months (range: 1–18 months), and the duration in our results is longer than what has been described in the literature (with a median of 9 weeks) . This miWe extensively reviewed previous reports and found that primary intracranial ES/pPNETs mostly showed mixed isointense-to-hypointense signals on T1WI, and isointense-to-hyperintense signals on T2WI . Our casES/pPNETs are highly aggressive, malignant tumors with focal necrosis . They arEWSR1 gene rearrangement is a golden standard for diagnosing ES/pPNET (t(EWS-FLI-1 (friend leukemia integration 1 transcription factor) in 85–90% of ES/pPNETs, being the in-frame fusion of the 5' end of EWSR1 gene with the 3' portion of the FLI-1 gene with abnormal transcription regulator properties , t(EWS-ETV1), t(EWS-EIAF) , t, 22 (q22-ETV1), t, 22 (q21WS-EIAF) –28. None-EIAF)  , malignant meningioma, and melanoma, among others , 20, 30.for CD99 . As for r CD99  for the publication of any potentially identifiable images or data included in this article.YJ, LZ, and YW made study design, data collection, data analysis and interpretation, and composed the manuscript and literature review. YL and XL was the surgeon that performed the surgery and did data collection, data analysis, and interpretation. XW made English and grammar corrections, critical revisions, and approved final version. YL had the acquisition, analysis or interpretation of data for the work, revising it critically for important intellectual content, final approval of the version to be published, and agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy, or integrity of any part of the work are appropriately investigated and resolved. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Epipericardial fat necrosis (EFN) is an inflammatory process that occurs in the mediastinal fat surrounding the heart. It is a rare cause of acute chest pain and mimics more ominous clinical conditions such as acute coronary syndrome, aortic dissection, and pulmonary embolism. Clinicians are often not familiar with this condition due to its infrequent occurrence, and traditional textbooks of medicine and cardiology have not covered this topic adequately. In the past, EFN had been managed primarily with thoracotomy and surgical excision. This has changed with advances in imaging techniques and their more frequent utilization. Computed tomography (CT) of the chest is essential for the diagnosis of EFN as it allows for the evaluation of the nature and precise location of the lesion. Magnetic resonance imaging helps to differentiate EFN from other mediastinal fatty lesions such as lipomas or liposarcomas. The clinical presentation of acute chest pain along with CT findings of the encapsulated fatty pericardial lesion is adequate for diagnosis. Our review describes the emerging role of imaging in diagnosis and change in management over the last few years. Fat necrosis in systemic adipose tissue can occur at various sites: in the breast and subcutaneous fat after trauma, peripancreatic fat in pancreatitis, and epiploic appendagitis ,2. RarelThere has been some discrepancy in the literature regarding the nomenclature of EFN. Initially, Jackson reported it as pericardial fat necrosis in 1957, and multiple reports have used the same terminology in the following years , have usEtiologyThe pathophysiology of EFN is not well established, and two probable mechanisms may describe the underlying disease process. In some cases, the mass consisting of fatty tissue attached to the heart via a vascular pedicle, suggesting that acute torsion of it might cause the necrosis ,10. The Clinical presentationEFN classically presents as chest pain in previously healthy individuals. The pain is pleuritic, and moderate to severe in intensity. The pain is predominantly left sided and some patients also experience dyspnea on exertion. Other systemic symptoms such as fever, myalgia, or cough have not been reported. The presentation is often similar to other common causes of chest pain, including myocardial infarction, pulmonary embolism, and pericarditis . The paiImage findingsThe workup usually begins with a chest x-ray. X-rays are suggestive but do not establish with certainty the diagnosis of EFN. The most common finding on X-rays is indistinct opacity, which usually develops along with the cardiomediastinal silhouette, raising concern for a mass lesion ,19,20. CDifferential diagnosis includes lesions such as thymoma, lipomas, liposarcoma, pericardial mesotheliomas, teratomas, and diaphragmatic hernias with abdominal fat occupying the cardiophrenic space ,22. FollHistopathologyExploratory thoracotomy has been performed frequently in the past for diagnosis of EFN and to rule out a neoplasm. Macroscopically, the lesion consists of circumscribed, lobulated adipose tissue fragments attached to the pericardium. The cut surface of the mass showed a white fibrous capsule enclosing degenerated fatty tissue ,31. MicrTreatmentEFN had been managed primarily with thoracotomy and surgical excision in the past. This treatment led to the resolution of symptoms, with no significant postoperative complications or short-term mortality reported. A total of 20 cases of EFN, managed with thoracotomy and surgical excision, have been reported between 1957 and 2005. Inoue et al. suggested that surgery remains the first-line option to diagnose and manage EFN and rule out a thoracic neoplasm . HoweverEFN is an uncommon cause of acute chest pain and mimics other potentially life-threatening conditions presenting as chest pain. Our review highlights the recently emerging role of imaging in diagnosis and subsequent change in the management. The incidence of EFN will continue to rise due to the increasing quality and use of imaging techniques. Clinicians should be aware of this condition, which helps them to establish the diagnosis non-invasively and avoid unnecessary interventions.