Hand macrodactyly is a very scarce deformity. It was first described over 200 years ago and was characterized as “local gigantism” of one or multiple digits. Benign bone overgrowth, massive increase of soft tissue volume, and nerve involvement are associated with hand macrodactyly have been consistently reported in the literature. Often, macrodactyly affects one or more digits and is further classified as static or progressive, depending on the growth pattern, and as sporadic or syndromic, according to its genetic predisposition. Surgical treatment for hand macrodactyly remains a complex issue even for expert hand surgeons. In most of the cases, macrodactyly is diagnosed during early childhood and can be appropriately managed with minimal and well affordable surgical approaches that stabilize its fast progression. However, adults with progressive hand macrodactyly develop advanced deformities leading to severe functional deterioration and aesthetic hand dysmorphia. The purpose of this report is to document the management and surgical approach of the oldest published case, a 60-year-old adult patient with neglected progressive hand macrodactyly despite previous surgical attempts for disease stabilization. A personalized preoperative planning was created, which included ray resection involving the fourth metacarpal and fourthfinger along with extensive debulking of the overgrown fatty soft tissue and carpal tunnel release. At six months’ follow-up, the patient reported an excellent aesthetic and functional outcome. The term “macrodactyly” is a descriptive term derived from the Greek words “macro” meaning long and “dactyl” meaning finger. Macrodactyly of the hand represents a very rare congenital deformity of unknown etiology, which constitutes less than 1% of congenital disorders in the upper extremity . There aA plethora of surgical interventions have been described mainly in young patients to cure static and prevent progressive hand macrodactyly, such as debulking procedures, epiphysiodesis, and osteotomies . The purA 60-year-old male individual was presented to our department to seek consultation for his hand macrodactyly. The patient’s condition was diagnosed in early childhood as a sporadic isolated anomaly affecting moderately the middle and severely the ring fingers. According to his medical records, he underwent two minimal soft tissue debulking surgeries on the third interdigital space when he was 4 and 37 years old, respectively. Initially, he reported that his hypertrophic left middle and ring fingers became more painful and less functional recently, albeit he could manage it until then. The thumb, index, and little fingers were normal. Despite his hand deformity, the patient has been a professional guitar player for at least 40 years. During the last three years, he was complaining of progressive disproportionate growth of his middle and ring fingers Figure . He was Under axillary block anesthesia and application of a pneumatic tourniquet, a racket-shaped incision was made around the base of the fourth metacarpal. The extensor and flexor tendons  as well as interosseous and lumbrical muscles of the fourth finger were detached and transected carefully to prevent tendon injuries of the remaining normal digits. Exposure of the digital neurovascular bundle of the third and fifth fingers was well visualized, mobilized, and preserved. Radial and ulnar digital nerves of the fourth finger were found enlarged and were easily recognized due to their increased thickness. Subsequently, nerve endings were carefully implanted within the surrounding soft tissue to avoid as much as possible the formation of painful neuromas. With a surgical oscillating saw, a transection at the base of the fourth metacarpal was performed. Subsequently, surgical debulking of the extensive soft tissue was implemented Figure , and hemPostoperatively, the patient was complaining of phantom pain, which was well tolerated with a two-week prescription of acetaminophen and NSAIDs. Eventually, the phantom pain was resolved entirely eight weeks after surgery . At the Until recently, pathogenesis of osseous and fibrofatty overgrowth in hand macrodactyly is not clearly identified and still no consensus exists on its treatment. Therefore, hand macrodactyly poses a significant surgical challenge. which often requires the expertise of experienced hand surgeons to manage effectively .It has been reported that significant nerve enlargement is usually observed during surgical approach of affected digits with morphological and neurophysiological impairment of the median nerve that requires carpal tunnel release . In the Genetic studies have demonstrated that hand macrodactyly can be a clinical manifestation of three major overgrowth syndromes: the Proteus syndrome (mosaic mutations in the AKT1 gene), the PIK3CA-related overgrowth syndrome (mutations in the PIK3CA oncogene), and the PTEN hamartoma tumor syndrome (mutations in somatic PTEN tumor suppressor gene). According to Cui et al., somatic mutations in PIK3CA oncogene were observed within bone marrow stem cells from patients diagnosed with hand macrodactyly . These sProgressive macrodactyly is an extremely challenging disease as no surgery is able to cure the underlying condition. Most patients, even if operated early in life, require multiple debulking procedures in accordance with the present case report. Concurring to the literature, only few published reports demonstrate potential strategies and surgical treatment options for progressive hand macrodactyly without a clear consensus on treatment guidelines, as shown in Table The largest case series study considering clinical characteristics and surgical management of 90 hand macrodactyly cases was conducted by Wu et al. . AccordiBased on recent bibliography, Jacobs et al. presented the most advanced case of a 55-year-old female patient diagnosed with Proteus syndrome and macrodactyly of her right-hand thumb, middle, and index fingers [Hand macrodactyly is usually visible at birth and patients experience overgrowth symptoms during early childhood. Consequently, an early and effective surgical management is strongly recommended to prevent chronic progression and development of severe secondary degenerative bone changes in macrodactyly fingers, such as ankyloses, narrowing of joints, and formation of osteophytes. Patients suffering from hand macrodactyly can significantly benefit from early surgical stabilization of the condition instead of late and more aggressive interventions such as amputation.In the present case, surgical interventions at an early stage proved ineffective, and the patient developed severe and disabling hand deformities due to the progressive subtype of hand macrodactyly. Most of the macrodactyly cases seem to stabilize at skeletal maturity, and it is unusual to see this degree of progressive bony overgrowth. Nevertheless, a personalized surgical technique including ray resection and debulking reconstruction surgery was proposed for this neglected case with great aesthetic and functional outcomes.


Staphylococcus hominis (S. hominis) is an opportunistic pathogen that is often highly resistant to antibiotics and is difficult to treat. In patients diagnosed with an adrenocorticotropic hormone (ACTH)-producing tumor that compromises the immune system due to hypercortisolemia, cancer treatment and infection control should be considered simultaneously. This report presents a case of refractory postoperative S. hominis bacteremia requiring the prolonged administration of several antibiotics in a patient with an ACTH-producing pancreatic neuroendocrine neoplasm (pNEN).S. hominis was identified and appeared sensitive to these antibiotics according to susceptibility testing. However, S. hominis was repeatedly positive in blood cultures for more than one month, despite treatment with several antibiotics. Eventually, with the combined use of three antibiotics  for more than 3 weeks, the S. hominis-associated bacteremia improved. He was discharged 79 days after surgery.A 35-year-old man visited a neighboring hospital for a thorough examination after experiencing weight gain and lower limb weakness for several months. Enhanced computed tomography revealed a pancreatic tail tumor and bilateral adrenal enlargement. Elevated plasma ACTH and serum cortisol were noted. Biopsy under endoscopic ultrasonography revealed the tumor as an ACTH-producing pNEN. The patient was transferred to our hospital for further treatment. Pneumocystis pneumonia was noted and treated with sulfamethoxazole and adjunctive glucocorticoids. Hypercortisolism was controlled with metyrapone and trilostane. Somatostatin receptor scintigraphy and ethoxybenzyl magnetic resonance imaging detected other lesions in the pancreatic head. A total pancreatectomy was performed given that the lesions were found in both the pancreatic head and tail. Plasma ACTH and serum cortisol levels decreased immediately after the resection. Pathological examination revealed that the pancreatic tail tumor was NEN G2 and T3N1aM0 Stage IIB and the pancreatic head lesions were SSTR-positive hyperplasia of the islet of Langerhans cells. On postoperative day 11, catheter-associated bacteremia occurred. Initially, meropenem hydrate and vancomycin hydrochloride were administered empirically. S. hominis bacteremia in such patients demands intensive treatments, such as with combinational antibiotics.Our patient with an ACTH-producing pNEN was immunocompromised and needed meticulous attention for infectious complications even after successful tumor removal. Specifically,  Staphylococcus hominis (S. hominis) is one of the most abundant microbial species on the human skin; it is an opportunistic pathogen that is found in the blood of neonates and patients on immunosuppressants [S. hominis infection that was refractory to even sensitive antibiotics.ressants , 2. An aressants , 4. We pd-glucose (FDG) positron emission tomography (PET)–CT revealed FDG deposition in the pancreatic tail tumor alone  was also administrated as adjunctive therapy for bacteremia during PODs 30–32 and 42–44. Specialized examinations, including enhanced CT, ultrasound cardiography, and FDG-PET–CT, were performed to investigate the source of the infection. Various bacterial cultures were performed using the bile, urine, and drainage fluid. However, except for the blood, no other apparent source was detected. Eventually, after more than 3 weeks of the combined administration of three antibiotics , the S. hominis-associated bacteremia improved. The patient was discharged 79 days after the surgery. Pathological examination revealed that the pancreatic tail tumor was NEN G2, T3N1aM0 Stage IIB with ACTH (+), somatostatin receptor (SSTR2) (+), and a Ki-67 of 15%. The pancreatic head lesion was identified as SSTR-positive hyperplasia of the islet of Langerhans cells  revealed a pancreatic tail tumor (55 mm in diameter) and bilateral adrenal enlargement Fig. , 3. Howeone Fig. . Contrasone Fig. . The cenS. hominis even after complete resection of the tumor; our observations indicated that meticulous attention should be paid to the management of S. hominis-associated bacteremia. Herein, we have discussed why the patient remained immunocompromised even after tumor resection and how S. hominis bacteremia can be treated in cases similar to the present one.We report the case of a patient with an ACTH-producing pNEN who suffered from postoperative infection with With a 5-year survival rate of 35%, ACTH-producing pNENs have a high metastatic capacity and high postoperative mortality rates; compared with other functioning and nonfunctioning pNENs, ACTH-producing pNENs have a poorer prognosis . AlthougEvidence on the systemic treatment of recurrent ACTH-producing pNENs is lacking due to their rarity , 13. We Staphylococcus hominis, a coagulase-negative staphylococcal strain, is a common organism that is involved in nosocomial bacteremia due to the increasing use of medical devices, such as intravenous catheters, vascular grafts, prosthetic heart valves, and devices used in the treatment of joint disease [S. hominis isolates carry the mecA gene, which resides within a mobile genetic element called the staphylococcal cassette chromosome mec [S. hominis is highly resistant to methicillin. Moreover, S. hominis often produces biofilms, which worsen antibiotic resistance and require high doses of antibacterial agents [S. hominis exhibited a cytotoxic effect on human epithelial cells, thereby inducing the destruction of host tissues and facilitating bacterial invasion [S. hominis sometimes requires the administration of multiple antibiotics, including clindamycin phosphate, over an extended period. In this case, there was no evidence of drug resistance to any of the administered antibiotics in the susceptibility testing. If multiple antibiotics, including clindamycin phosphate, had been administered consistently for an extended period, the S. hominis infection could have been resolved earlier. disease . Previousome mec , 17. As S. hominis-associated infectious complications even after a successful tumor removal. S. hominis bacteremia in such cases demands intensive treatments, such as combinational antibiotics.In this case, our patient with an ACTH-producing pNEN was immunocompromised and required meticulous monitoring for


Our patient displayed a decreased uptake of PET tracer 18F-florbetapir in the brain lesions and lower distribution volume ratio in the damaged white matter lesions compared to the normal-appearing white matter, indicating significant intracranial demyelination. After treatment, the 18F-florbetapir PET/MRI examination showed a significant increase in the uptake of 18F-florbetapir in the brain lesions, along with a reduced Expanded Disability Status Scale score. Although only a small number of patients have been validated, this case first reported 18F-florbetapir PET/MRI could quantitatively and non-invasively assess the myelin recovery in GFAP-A patients, which may lead to improvements in the early diagnosis and long-term prognosis.Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare autoimmune disease of the central nervous system that was newly reported in 2016. Previous studies have speculated that the pathological mechanism and clinical outcome of GFAP-A lie in the demyelination of the central nervous system, but due to the limitations of MR, this conclusion has not been further confirmed from the perspective of neuroimaging. A non-invasive, quantitative measurement of demyelination would be clinically valuable, given its critical role in mediating GFAP-A. Here, we report a case in which we use  GFAP-Aglial fibrillary acidic protein astrocytopathyIgGimmunoglobulin GCSFcerebrospinal fluidCNScentral nervous systemDVRdistribution volume ratioDWMdamaged white matterNAWMnormal-appearing white matterPETpositron emission tomographyMRImagnetic resonance imagingNGSnext generation sequencingEDSSExpanded Disability Status ScaleMSmultiple sclerosisNMOSDneuromyelitis optica spectrum disordersAQP4aquaporin-4anti-MBP antibodyanti-myelin basic protein antibodyADEMacute disseminated encephalomyelitisTSPOthe 18 kDa translocator protein1Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a rare inflammatory central nervous system (CNS) disorder first reported in 2016 . Therefo18F-Florbetapir, originally applied to the diagnosis of Alzheimer’s disease, was subsequently proved for their potential use in myelin imaging [18F-florbetapir bound to demyelinated lesions could monitor acute disseminated encephalomyelitis [18F-florbetapir PET for assessing GFAP-A has not yet been investigated. Compared to conventional MRI which could reliably locate the lesion, a hybrid PET/MRI could simultaneously obtain quantitative and locational information specifically for myelin states. Therefore, we investigated the potential use of hybrid PET/MRI with 18F-florbetapir for quantitative assessment of demyelination in the case of patients with GFAP-A.Magnetic resonance imaging (MRI) is a preferred radiological modality in the early diagnosis of GFAP-A, with a typical pattern of gadolinium enhancement in brain MRI, and longitudinally extensive T2 hyperintensities with central cord enhancement in spine MRI . Althoug imaging ,12,13. O2A patient with a definite diagnosis of GFAP-A was enrolled with follow-up. The enrolled patient underwent clinical assessments, including clinical disability through the expanded disability status scale (EDSS) before PET/MRI scan at baseline.18F-florbetapir PET/MRI was performed with a Biograph mMR system  with a National Electrical Manufacturers PET resolution of 4.2 mm [18F-florbetapir, dynamic PET acquisition in list mode over 60 min was started immediately, considering that it was cost-effective for most patients to reach a plateau [18F-florbetapir binding measured as the distribution volume ratio (DVR).Based on the method we used before, f 4.2 mm . After i plateau . Image s plateau . The LogEthical approval: The research related to human use has complied with all the relevant national regulations, institutional policies and in accordance with the tenets of the Helsinki Declaration. Human research was carried out with approval of the Ethics Committee of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine and with informed patient consent.3A 26-year-old female developed headache and fever up to 41°C, accompanied by rigors, chills and vomiting. She was treated with antibiotics and appropriate rehydration at a local hospital, but her symptoms were not alleviated. Subsequently, she experienced several instances of disturbance of consciousness, agitation and rambling. She was emergently referred to the department of infectious disease in our hospital. On admission, CSF examination showed normal intracranial pressure, elevated protein (1988.06 mg/L) and reduced glucose (2.18 mmol/L), but next-generation sequencing of bacteria and fungi was both negative. Screening for associated antibodies revealed positive CSF GFAP antibodies (titer of 1:32) and serum GFAP antibodies (1:10). Brain MRI showed multiple abnormal signals involving right temporal lobe, left paraventricular and left frontal lobe.18F-florbetapir PET/MRI. The decrease in the uptake of PET tracer 18F-florbetapir was found in the brain lesions on pre-treatment patient’s PET/MRI. Parallelly, T2 Flair MRI demonstrated multifocal hyperintense lesions , neuromyelitis optica spectrum disorders, and acute disseminated encephalomyelitis (ADEM) patients [18F-florbetapir uptake and myelin recovery in GFAP-A patients.Here, we report a hybrid PET/MRI imaging tool with ormality . Howeverpatients ,11,12,1318F-florbetapir DVR) and the shift in the clinical disability (as measured using EDSS). A previous study conducted by our group has demonstrated that the EDSS change and global myelin recovery were significantly correlated in patients with MS [18F-florbetapir DVR could provide additional valuable myelin state information in astrocytic damage-associated disease.In our study, we found the similar trend was identified for the myelin change (as measured by  with MS . Consist18F-florbetapir in ADEM and MS, our results for the first time indicate this technique could quantitatively and non-invasively assess the myelin recovery in GFAP-A patients and helps to understand the pathological process of the disease. This new approach should pave the way for real-time in situ myelin evaluations in clinical practice, and it offers the technical prerequisites for early diagnosis and long-term prognosis of demyelination-related disease.Based on the application of PET/MRI with


TREX1 is a gene that encodes an exonuclease on the C-terminal strand at the 3 ́ end for DNA repair. Multiple syndromes associated with the alteration of this gene have been described, focusing in this case on retinal vasculopathy with cerebral leukodystrophy (RVCL). We present the case of a 44-year-old female patient with a familial history of cerebral pseudotumors. At the time of diagnosis, the patient presented weakness in the lower limbs and dysesthesias of the right body at the beginning of the clinical picture, without visual alterations or retinal changes at fundus examination. A cranial magnetic resonance imaging (MRI) study showed a pseudotumoral lesion at the inferior frontal gyrus with a report of a choline peak in spectroscopy, ring enhancement in contrasted T1 sequence, and apparent central necrosis. A molecular study shows a mutation in c2136G>A, c.799dup, and c.5312A>G related to genes expressing PDE6A, TREX1, and VCAN proteins, respectively, mutations that have not been previously reported. Retinal vasculopathy with cerebral leukodystrophy (RVCL) was first reported in 1988 in a family with a familial history of unusual brain tumors ,3.Patients with RVCL exhibit a central constellation of neurological and visual symptoms . ClinicaIn the most severe cases, patients present with vision loss, seizures, hemiparesis, apraxia, dysarthria, or memory loss. There can be progression to blindness, a neurovegetative state and death within 5 to 10 years after the onset of symptoms . In thisWe present the case of a 44-year-old Mexican female, widow, high school graduate, housewife, and right-handed, with a family history of a mother with an unspecified brain tumor, as well as a maternal grandmother and cousin diagnosed with retinal vasculopathy with cerebral leukodystrophy due to TREX1 mutation. She smoked two cigarettes per day for the past 28 years while denying other drug addictions. Blood type O positive. History of systemic arterial hypertension diagnosed in 2020 in control and trigeminal neuralgia diagnosed in 2017 in treatment with pregabalin. Allergy to ketorolac and carbamazepine.She began in February 2021, five months prior to her first consultation, with weakness in the pelvic limb and right arm that made it difficult to ambulate; she was treated with analgesics and rehabilitation without improvement, which is why she requested medical evaluation.In her initial evaluation, she presented with hemiparesis and dysesthesia of the right body, predominantly in the distal lower limbs. The Ashworth scale scored 1 spasticity in the right arm and the Ashworth scale scored 2 in the right leg. Hoffman and Tromner's signs are present on the right body.The patient had a simple cranial computed tomography (CT) scan performed externally with left frontal periventricular digitiform edema Figure . A denseTwo months later, an oppressive biparietal headache of 4/10 was added. A contrasted cranial magnetic resonance imaging (MRI) was performed Figure , showingFour months later, in a follow-up visit, the patient presented with gait impossibility and difficulty in articulating words, in addition to a decrease in bilateral visual acuity, so an assessment was performed by the neuro-ophthalmology service (NO), which did not find any pathological alterations. An adjustment in steroid dose was made, achieving improvement in the symptoms. The histopathological study was deferred by NcxO since there was no benefit in performing the biopsy based on literature reports.In May 2022, a control MRI study was performed Figure , where aThe TREX1 gene, located on chromosome 3p21.31, encodes a 314 amino acid polypeptide with a molecular weight of ~33 kDa. Homologs of TREX1 are found in metazoans, are well conserved, and are expressed in most tissues, likely indicating an important maintenance function in most cells [Mutation of this gene has been consistently linked to a wide range of acronyms for several similar pathologic phenotypes Table  3]. The. The3]. In general, patients begin with vision problems, commonly reporting blurred vision or neurological symptoms such as paresthesias or plegia of a limb or hemibody. This disease becomes fatal 5-10 years after diagnosis ,10.The imaging study of choice is MRI, where a tumor-like lesion is revealed in about half of the patients, resembling a primary malignant neoplasm of the brain , while iPreviously, three types of lesions have been described on MRI; (1) white matter lesions (WML) without focal or confluent enhancement; (2) WML with punctate enhancement; and (3) lesions with rim enhancement with surrounding T2 hyperintensity (edema/gliosis) and/or diffusion restriction ,13. ThesThe treatment of these tumor-like or pseudotumoral lesions is not clear. This situation may lead to the erroneous behavior of surgical intervention, even if a biopsy is contraindicated since only a histopathological report of inflammatory cells would be found . SteroidThe present case is one of the few published reports of cerebral leukodystrophy without retinal damage. Our experience highlights the need to consider this pathology as a differential diagnosis in patients with tumefactive brain lesions, a family history of cerebral pseudotumors, and progressive visual involvement, which may help to avoid unnecessary invasive examinations. Likewise, contrary to the literature, the patient did not show clinical improvement with the use of steroids, but a decrease in the size of the pseudotumoral lesion was observed in MRI, probably due to the administration of steroids .Since previous findings and the results of this study show a clinical and imaging improvement just under oral steroid treatment, molecular testing for genetic mutations should be suggested in cases of vascular retinopathy, focal neurological deficits, and ring-enhancing lesions by long-standing imaging studies, as well as a family history of cerebral pseudotumors.Trex1 mutation is not completely studied and since we have discovered more cases with this mutation at our institution, it should be a crucial area of research for geneticists, neurosurgeons, and neurologists. Surgical operations that are unnecessary for the patient's health and would not make any difference in the outcome. The Medical treatment for this disease should be studied as well. Our patient was treated with steroids, but only to treat the neurological symptoms she was experiencing. However, as we have seen in other cases that have been reported in the literature, the cerebral pseudotumor recurs and worsens over time, eventually leading to the patient developing irreversible neurological changes.


Spontaneous suburothelial hemorrhage is a rare process of unclear pathologic etiology, although it has been associated with bleeding diathesis and anticoagulation. The most common clinical presentation is acute onset flank pain and macroscopic hematuria. On imaging, there is a mural thickening of the renal pelvis and upper ureters leading to luminal narrowing. Despite luminal narrowing, hydronephrosis has only rarely been reported in the association. The imaging finding of mural thickening has led to the misdiagnosis as malignancy, resulting in unnecessary nephrectomy. Suburothelial hemorrhage can be unilateral or bilateral, although the majority of reported cases are unilateral. We present a case of a patient with bilateral spontaneous suburothelial hemorrhage with forniceal rupture and spontaneous retroperitoneal hemorrhage, a unique triad compared to prior cases presented in the literature. Spontaneous suburothelial hemorrhage is a rare pathologic process most commonly associated with anticoagulation, although other causes of bleeding diathesis such as hemophilia have been documented . PatientThe patient was an African American 72-year-old female with a history of chronic warfarin therapy for a combined history of deep venous thromboses of the lower extremities, strokes, and atrial fibrillation. She was found to have an international normalized ratio (INR) of 18  by her primary care provider, for which she was immediately sent to the emergency room. The patient reported no recent history of trauma and complained of some vague symptoms, most notable for lower abdominal and bilateral leg pain and macroscopic hematuria. She denied fever, chills, nausea, vomiting, dysuria, and dizziness, but complained of some generalized fatigue. At presentation, the patient demonstrated mild tachycardia with a heart rate of 106 beats per minute, systolic blood pressure of 113 mmHg, and diastolic blood pressure of 65 mmHg. The patient was otherwise afebrile with a normal respiratory rate and oxygen saturation. Physical examination of the patient revealed a nondistended abdomen without significant tenderness. There was no evidence of costovertebral angle tenderness. There was no evidence of guarding, rebound, or palpable/pulsatile mass. Despite the benign abdominal exam, the available clinical and laboratory findings at the time remained concerning for a source of bleeding, which prompted the emergency physician to order a CT scan. Laboratory and diagnostic tests obtained later in the emergency department revealed a decreased hemoglobin level at 6.5 grams per deciliter (g/dL), below the patient’s baseline ranging between 8.5 and 9 g/dL . The patient’s lactate was also mildly elevated at 3.5 mmol/L . The patient was given an intravenous bolus of normal saline and a blood transfusion was later initiated. The patient was admitted with conservative management, including further fluid resuscitation and discontinuation of her anticoagulation with correction of her INR. She was discharged in stable condition with a resolution of her symptoms and scheduled for a follow-up with her primary care doctor to consider the risk versus benefit discussion of continuing anticoagulation.An abdominal CT with intravenous contrast was performed in the portal venous phase, which revealed hyperdense fluid (Hounsfield units >30) of the bilateral perinephric regions extending along both sides of the retroperitoneum, with some fluid tracking down to the pelvis concerning retroperitoneal hemorrhage Figures -1C. No uAbdominal pain with hematuria is a common cause of presentation to the emergency department leading to CT evaluation. Hematuria and abdominal pain are most commonly related to urinary tract infections, or stones, with malignancy a less common but more serious etiology. Suburothelial hemorrhage is a rare, benign, reversible process with a similar clinical presentation to the previously mentioned processes and a similar imaging appearance to malignancy -4. In faUnderstanding the imaging features of suburothelial hemorrhage in various phases of CT imaging is essential to establishing the correct diagnosis. Unlike other etiologies of mural thickening or compromise of the lumen of the renal collecting system, suburothelial hemorrhage tends to be hyperdense on unenhanced CT with a more diffuse circumferential involvement ,5-8. On Once the appropriate diagnosis of suburothelial hemorrhage is established, treatment involves reversal of anticoagulation and/or treatment of the cause of coagulopathy , 11-13. Suburothelial hemorrhage is a rare process which can lead to the common clinical presentation of abdominal pain and hematuria. The most common etiologies for abdominal pain and hematuria are urinary tract infection or nephroliathiasis/urolithiasis. Laboratory values and imaging can often exclude these etiologies. The presence of hyperdense, circumferential mural thickening, either unilateral or bilateral, suggests suburothelial hemorrhage and should not be mistaken for a urothelial malignancy but instead lead to investigation for the presence of a bleeding diatheses. Although the process leads to luminal narrowing, only mild hydronephrosis has been rarely reported. Our case is unique in that forniceal rupture was present suggesting increase pressure in the renal collecting system. Although, at the time of imaging there was no hydronephrosis or hydroureter. The additional finding of retroperitoneal hemorrhage is a unique imaging triad  never before described in the literature. It is important for both clinicians and radiologists to be familiar with this diagnosis, and the spectrum of findings to ensure the appropriate diagnosis and treatment thereby eliminating unnecessary invasive treatment.


Instrument separation in the apical third of the tooth, which is associated with a large periapical lesion, presents an arduous task for the clinician. This case report presents nonsurgical endodontic management of a maxillary central incisor associated with a large periapical lesion and a separated instrument in the apical third of the root canal. A 28-year-old male patient presented with pain and labial swelling in the left maxillary central incisor region. Pulp sensibility testing showed no response. The radiograph revealed the presence of a separated instrument in the apical third of the root canal and periapical radiolucency. A diagnosis of previously initiated therapy with acute apical abscess was made. In the first visit, instrument retrieval was done using ProUltra Endo tips  under a dental operating microscope. In the subsequent visit, obturation was done as the patient was asymptomatic. The patient was recalled for follow-up at six, 12, 18, 24, and 36 months. Complete healing of the periapical tissues was evident on the radiograph, and the tooth remained functional for the entire follow-up period of three years. The successful outcome seen in this case shows that even large periapical lesions can be managed conservatively by nonsurgical endodontic treatment Instrument fracture can be a frustrating experience for the clinician and patient as it hinders the biomechanical preparation of the root canal system, thereby reducing the ultimate success of endodontic treatment . DespiteA 28-year-old male patient reported to the department of conservative dentistry and endodontics of a tertiary care hospital with a chief complaint of pain and swelling in the left maxillary incisor region. Extra-oral tissues appeared normal. On intra-oral examination, tooth #9 was tender on percussion, for which endodontic treatment was initiated by a private practitioner four months back. A supernumerary tooth (ST) was present between the left maxillary central and lateral incisor Figure . ST was On vitality testing using a cold test  and electric pulp test , no response was elicited wrt tooth #9 and ST. A normal response was seen wrt tooth #8 and tooth #10. Radiographic examination revealed a separated endodontic instrument measuring 5.5 cm in the left maxillary central incisor present in the apical third of the root canal. Large periapical radiolucency (dimensions 11 ×11mm approximately) was associated with maxillary central incisor and supernumerary tooth Figure . Based oIn the first appointment, access cavity preparation was modified wrt tooth #9 and the supernumerary tooth (ST) after rubber dam isolation under a surgical operating microscope . Modified Gates Glidden drill no. 3  was used at a speed of 800 rpm to prepare a staging platform wrt tooth #9. Gates Glidden drill was modified by cutting it perpendicular to its long axis at its greatest diameter. The fractured instrument was visible under the operating microscope at this stage. Ultrasonic tip ProUltra ENDO No 8  was positioned between the canal wall and fractured instrument and activated at the lowest power setting  by moving it in the counterclockwise direction in dry conditions. The instrument was loosened in the canal and moved in the coronal direction, as seen on the intraoperative radiograph Figure , 2B. HowWorking length was assessed radiographically for both teeth. Biomechanical preparation was done using hand K files using the step-back technique for tooth #9 and ST. Apical preparation was done till 60# K-file and 45# K-file for tooth #9 and ST, respectively. Irrigation was done using 5.25% sodium hypochlorite (NaOCl) , followed by 17% ethylenediaminetetraacetic acid . Canals were rinsed with normal saline in the end. The root canal was dried with sterile paper points, and calcium hydroxide dressing was placed in the canal space. The access cavity was sealed with a 3mm thick provisional restoration material , and the patient was recalled after two weeks. The patient was completely asymptomatic on the recall visit. Provisional restoration was removed using #2 diamond round bur. Copious irrigation with 5.25% NaOCl followed by 17% EDTA was done. A final rinse with normal saline was done, and the canal was dried with paper points. Obturation was done with AH Plus Sealer and Gutta-percha  using the lateral condensation technique. The access cavity was restored with nano-hybrid composite resin .The patient was regularly followed up. At the three-year recall visit, the patient was asymptomatic, and both teeth were functional. Osseous healing was evident peripherally on a six-month recall Figure . CompletTeeth presenting with concomitant large periapical lesion and a fractured instrument in the apical third fall in the category of complex endodontic cases and often requires surgical intervention. However, surgical intervention should be done only when conservative treatment options fail. It has been observed that up to 94.4% of periapical lesions show partial or complete healing when managed with a conservative approach using nonsurgical endodontic therapy ,6. In ouAnother major issue in the presented case was the presence of a separated instrument which is one of the most alarming complications occurring during endodontic treatment. The several attributing factors for instrument fracture are repeated usage of the instruments, instrument design, degree of canal curvature, and operator’s experience -9. HowevWhen a patient presents with a separated instrument, a skilled clinician should always weigh the advantages and disadvantages of attempting instrument retrieval. Lin et al. designed a statistical model to help clinicians in decision-making and concluded that the two most important factors determining the success of instrument retrieval are the depth of separated instrument, and canal curvature . In the Attempts to remove an apically located fragment can result in excessive removal of dentin and perforate or weaken the root making it susceptible to fracture . It has Proper illumination and magnification are the keys to successful instrument retrieval, particularly when the separated instrument is present in the apical third. The separated instrument needs to be carefully located under a dental operating microscope for precise removal of radicular dentin by ultrasonics. The instrument retrieval rate is reported to be as high as 95% with ultrasonics . While uOne of the potential drawbacks of the presented case is that cone beam computed tomography (CBCT) was not done postoperatively to confirm the healing of periapical lesion three-dimensionally. However, considering the socio-economic status as well as the clinical status of the patient, CBCT was not done.Instrument retrieval is critical for the success of endodontic treatment, especially when the tooth is associated with a periapical lesion. This case report demonstrates a successful retrieval of a separated instrument from the apical third of the root canal with minimal damage to the radicular dentine, which was possible due to the application of extremely fine ultrasonic tips under a dental operating microscope. Also, the use of an advanced armamentarium can reduce the risk of mishaps or procedural errors while managing such complicated cases.


Sporadic spinal extramedullary hemangioblastomas of the conus medullaris are extremely rare. We present the case of a 40-year-old male with symptoms of severe back pain and monoradiculopathy. The magnetic resonance imaging (MRI) revealed an intradural extramedullary tumor attached to the conus medullaris. Total tumor removal was achieved via a typical posterior midline approach through laminectomy of L1 and L2 vertebrae, resulting in complete resolution of the preoperative symptoms. The histological examination was consistent with hemangioblastoma. To the best of our knowledge, this is the fifth case reported in the literature. We performed a brief literature review outlining the mainstay of diagnosis and therapeutic approach to these challenging lesions. Hemangioblastomas represent 1-3% of all central nervous system tumors and are most frequently located in the cerebellum, brain stem, and spinal cord -4.Hemangioblastoma can be sporadic or associated with the von Hippel-Lindau syndrome with autosomal dominant inheritance caused by the VHL gene mutation in 3p25-26 chromosomes. The syndrome is also linked with other types of tumors such as retinal angiomatosis, endolymphatic sac neoplasms, kidney or pancreatic cancer, pheochromocytoma, etc. [We present a case of sporadic hemangioblastoma in the area of the conus medullaris and cauda equina. We also conducted a brief literature review of the diagnostic and treatment approaches for this rare tumor location. To the best of our knowledge, this is the fifth reported case in the literature.We present the case of a 40-year-old male patient who presented with a gradual onset of progressive severe back pain for a duration of four months prior to hospital admission. The pain radiated towards the upper anterior surface of the right thigh. The symptoms were not relieved by conservative treatment with non-steroid anti-inflammatory drugs and were misinterpreted as a degenerative spinal disease. At hospital admission, the neurological examination revealed severe back pain, hyperalgesia, and hyperesthesia along the right L2 dermatome. The magnetic resonance imaging (MRI) revealed an intradural extramedullary tumor at the L1-L2 level, tightly adherent to the conus medullaris cranially and displacing the filum terminale and cauda equina roots to the right Figure -1C.The patient was operated on via typical midline posterior surgical access through laminectomy of L1 and L2 vertebrae. The dura mater was found to be tense. After midline durotomy and arachnoid incision, we evacuated xanthochromatous cerebrospinal fluid. A cherry-red intradural extramedullary tumor with a subtle capsule was visualized that originated from the area of the conus medullaris and ventrally displaced the filum terminale. The surrounding roots of the cauda equina as well as the caudal and cranial poles of the tumor were identified Figure . Using aThe postoperative period was uneventful, with complete resolution of the preoperative symptoms. The histological examination revealed a tumor formation rich in stromal cells with small nuclei, large vacuoles, scattered red blood cells, and thin-walled vessels and microhemorrhages consistent with hemangioblastoma Figure . Given tSporadic intradural extramedullary hemangioblastomas of the conus medullaris are extremely rare, with only four cases reported in the literature worldwide Table  1,4,6,7,6,74,6,7The age of the patients varied from 33 to 57 years, and our patient is no exception. Including the current case, the percentage of male patients prevails slightly, nearing 60%. Like others, our patient presented with pronounced lumbalgia, pain, and radiculopathy Table . In all Small-sized spinal cord hemangioblastomas in the area of the conus medullaris and cauda equina are usually asymptomatic. The neurological deficit occurs gradually with the increase in tumor size . The mosSporadic intradural extramedullary hemangioblastomas are usually amenable to total surgical removal. In such cases, complete patient recovery is observed in over 96% of the cases . RecurreThe diagnosis of spinal hemangioblastomas is difficult due to overlapping symptoms with degenerative spinal diseases as well as the variety of neoplasms and vascular malformations  that share similar MRI characteristics . The neuThe preoperative differentiation of spinal tumors from vascular abnormalities is of particular importance. In such cases, selective spinal angiography is a useful tool for the diagnosis of suspected vascular malformations. It can also be used to perform preoperative embolization, which reduces intraoperative bleeding ,11.In our case, we present the difficulties in the differential diagnosis of sporadic intradural extramedullary hemangioblastoma of the conus medullaris. In the same way as the case described by Shields et al., the suggested preoperative MRI diagnosis was ependymoma or schwannoma , while tThe differential diagnosis of tumors in the area of conus medullaris and/or cauda equina should include hemangioblastomas. Selective spinal angiography could facilitate the differentiation between spinal tumors and vascular pathology. In cases with proven spinal hemangioblastomas, a screening for von Hippel-Lindau syndrome should be performed. MRI is the imaging method of choice for accurate preoperative topical diagnosis and adequate surgical planning. The aim of the surgery must be total tumor removal, which provides permanent neurological recovery for these patients and reduces recurrence rates.


We present a case of a giant phyllodes tumor (PT) requiring simple mastectomy with en bloc pectoralis major resection and immediate deep inferior epigastric perforator (DIEP) flap reconstruction. This patient presented with a four-year history of an enlarging breast mass with ultrasound-guided biopsy results consistent with atypical fibroepithelial proliferation that was highly concerning for a borderline phyllodes tumor. In this large, rare breast tumor that required en bloc pectoralis major resection, we describe the novel use of an immediate single pedicled DIEP flap for the resulting chest wall defect. The patient’s postoperative course was uncomplicated, and she reported satisfactory cosmetic and functional outcomes at her initial postoperative follow-up visits. Our findings support the use of simple mastectomy with en bloc resection and immediate single-pedicled DIEP flap for the definitive treatment of giant phyllodes tumors. Our experience shows this is a safe and effective technique for achieving adequate oncologic resection while maintaining postoperative function and cosmesis, which are essential for patient quality of life. Phyllodes tumors (PTs) are fibroepithelial neoplasms that account for less than 1.0 % of all primary breast tumors . Giant PWe present a case of a giant phyllodes tumor requiring simple mastectomy and excision of the pectoralis major muscle with immediate deep inferior epigastric artery perforator (DIEP) flap reconstruction.A 51-year-old female was referred to the outpatient breast clinic for an enlarging and painful right breast mass. Four years ago, she underwent a screening mammogram that showed an obscured mass in the upper outer quadrant of the right breast (Figure A follow-up right breast ultrasound demonstrated a 2.8 cm hypoechoic mass at the 10:00 position, 5 cm from the nipple (Figure An ultrasound-guided biopsy was performed, and histopathology showed a fibroepithelial proliferative lesion consistent with a proliferating or juvenile fibroadenoma. The patient was recommended to have short-interval follow-up imaging. A six-month diagnostic mammogram demonstrated the lesion had increased to 3.6 x 2.2 x 3.0 cm from 2.8 x 2.3 x 1.9 cm (Figure Surgical excision was recommended at that time; however, she had not followed up for any breast imaging or surgical excision. Since then, she has noted the continued growth of the mass over the past three years, with a rapid increase in size over the last two to three months. Her personal and family history was unremarkable. Laboratory analysis was notable for anemia at a baseline of 8.8 g/dL. On examination, her right breast was markedly enlarged and tender with a firm, palpable mass occupying the breast, measuring approximately 15 cm. The overlying skin was shiny without significant ulceration, cellulitis, or abscesses Figure . There wA subsequent breast MRI revealed a right-sided 10.9 x 9.4 x 11.7 cm heterogenous T2-enhancing lobulated mass that occupied the entire right breast Figure . A metasThe case was discussed preoperatively at a multidisciplinary tumor board, and based on the large tumor size, proximity to the nipple-areolar complex on MRI, and patient preference of surgical options, a simple right mastectomy with immediate DIEP flap reconstruction and left excisional biopsy was performed Figure . The posThe left breast excisional biopsy found atypical ductal hyperplasia with microcalcifications and a complex fibroadenoma. The final pathology of the right mastectomy revealed malignant PT with marked ulceration Figure . MarginsPhyllodes tumors are uncommon breast neoplasms that Johannes Muller first described in 1838; he initially named them cystosarcoma phyllodes due to their leaf-like stromal projections . PhyllodPrior literature has demonstrated that giant PTs have a higher rate of malignancy compared to smaller tumors, at 42.5% and 21%, respectively. Likewise, the recurrence rate in giant tumors compared to smaller tumors is also increased, at 41% vs. 29%, respectively . AchieviFew case reports exist regarding autologous reconstruction for the large resultant soft tissue defect following resection of giant phyllodes tumors. Tsuruta et al. describe a similar single-pedicled DIEP-free flap; however, their case differs from ours in that there was not an en bloc resection of the pectoralis major . Fang etLimitations of the existing literature for autologous reconstruction following resection of giant PT include a direct comparison between various free flap options, the anecdotal nature of case reports, and small sample sizes. There is a need for prospective trials utilizing larger sample sizes to compare recurrence and complications. However, this is logistically challenging given the nature of such a rare disease.This case demonstrates that the resection of a giant phyllodes tumor with immediate single-pedicled DIEP flap reconstruction can be performed with satisfactory oncologic, functional, and cosmetic results.The giant phyllodes tumor is a rare breast neoplasm and is often challenging to manage from a resection and reconstruction standpoint. Previous case reports describe a simple mastectomy with DIEP flap reconstruction. We present the results of a mastectomy with en bloc pectoralis primary resection and a single pedicled DIEP free flap reconstruction.Our experience shows this is a safe and effective technique for achieving adequate oncologic resection while maintaining postoperative function and cosmesis, which are essential for patient quality of life. We propose this technique for the definitive management of giant phyllodes tumors of the breast.


Teratomas are very rare, originating from embryonal germ layers. The majority of them are mature, most common in the gonads, and with only 15% out of gonads. In particular, primary adrenal teratomas are extremely rare. The present study reported a case of a young female patient with right adrenal tumor who underwent intermittent pain in the right waist and abdomen and whose CT of adrenal gland showed an 88 mm × 79 mm × 69 mm mass. Besides, her adrenal gland-related hormones are not abnormal. Laparoscopic adrenal tumor resection was performed on her and the histopathological results confirmed that the mass was mature adrenal teratomas. As a newly diagnosed case, strict and regular follow-up is needed, and it is also necessary to detect her AFP and check her adrenal CT in the future. In addition, we have reviewed the literature from 1952 to the present, and a total of 49 cases of adrenal teratoma have been identified and analyzed. Teratomas are tumor stemming from embryonal germ layers that are mainly composed of pluripotent stem cells. Teratomas are very rare and uncommon, whose incidence is 0.9/100,000 among the population . Based oAccording to the differentiation degree of teratomas tissue, teratoma can be divided into two types, namely, mature teratoma and malignant teratoma, which consists of tissue derived from more than one kind of embryonic germ cell layer germ cell layers, including ectoderm, mesoderm, and endoderm . AlthougA 19-year-old female patient presented to the outpatient department of our hospital with the symptoms of intermittent pain in the right waist and abdomen for 4 months; she had no other symptoms. The patient had received no specific treatment prior to this, she follows a healthy daily diet and routine, and no relatives in her family had similar illnesses. After hospitalization, the patient’s temperature, heart rate, breath frequency, blood pressure, and oxygen saturation were normal. A mass was palpated on the upper pole of the right kidney area, about 10 cm × 8 cm in size, with mild percussion pain, but no bulge, no pressure, or percussion pain in both sides of the ureteral region.Cortisol (COR): 36.5 nmol/L, β-HCG < 0.1 MIU/ml (non-pregnant women 0–5 MIU/ml), AFP: 3.1 ng/ml (<9 ng/ml), CEA: 0.6 μg/L , CA19-9: 4 U/ml (<25 U/ml), renin: 1.09 ng/ml, angiotensin I: 1.62 ng/ml, and angiotensin II: 38.77 pg/ml; other indexes were normal and are shown in The results of chest CT scan and ECG were normal. An 88 mm × 72 mm mixed-echoic mass in the space between right kidney and liver were observed by color ultrasound of the urinary system, and CDFI showed no blood flow in the mass. CT scan + contrast-enhancement + 3D of adrenal gland showed an 88 mm × 79 mm × 69 mm mass with mixed density shadow, multiple nodular calcareous density, flake-like high density, and adipose density shadows but no obvious enhancement signals were monitored. Besides, the upper pole of right kidney was extruded by the huge mass, and there were no enlarged lymph nodes in the local area. The tumor was suspected to be a teratoma in the right adrenal gland area  and (teratoma or teratoma), and 296 results were retrieved. After removing duplicates and irrelevant studies, finally, 24 publications describing 49 cases were identified Table 2.The series of review comprised 49 patients (14 men and 35 women) with a mean age of 30.3 years. Adrenal-related hormones were normal in all patients and 27 (55%) patients were clinically asymptomatic. The majority of adrenal teratomas were unilateral, with only one case of bilateral adrenal involvement. In contrast to previous reports, the current data suggest that right-sided adrenal teratomas are more common. In most cases, mature fat and calcified tissue were observed. All cases were surgically resected and histopathologically confirmed as teratomas. Most of the adrenal teratomas were mature teratomas, two of which were within focal carcinomas and neuroblastomas, respectively, and one was a malignant transformation of a teratoma into an adenocarcinoma. Only one case was an undifferentiated malignant teratoma. Thirty-four of the 49 patients were followed up for 3 months to 10 years, and all had a good prognosis with no tumor recurrence or other discomfort during the follow-up period.The teratoma is a rare neoplasm; according to different original sites, teratomas can be categorized into gonadal and extragonadal teratomas, and retroperitoneal space is a site of the extragonadal location. When primary retroperitoneal neoplasms in adults were found that represent an infrequent mass , the priPrimary adrenal teratoma has no distinctive clinical presentation with normal adrenal-related hormones, presenting as “incidentalomas”, and they are not clearly distinguishable from other adrenal tumor with no endocrine function. In clinical practices, primary adrenal teratoma is usually found in medical examination and diagnosis of other functional adrenal tumors, such as adrenal cortical adenoma or pheochromocytoma . TherefoAdrenal teratomas are usually asymptomatic or have non-specific complaints, and laboratory tests are generally normal, thus posing a significant preoperative diagnostic challenge and relying primarily on imaging for diagnosis. Ultrasound usually shows a heterogeneously echogenic mass including hyperechoic calcifications and ossifications, as well as hypoechoic fatty and cystic areas . CT indiMost mature teratomas are benign tumors, and the surrounding organs can be compressed by the growing tumor, even with the possibility of tumor rupture, bleeding, and infection. Once diagnosed, surgery should be performed as soon as possible. Laparoscopic surgery has become the “gold standard” for the treatment of adrenal tumors smaller than 6 cm . For adrThe etiology of teratomas is not known and the pathological classification includes mature teratomas and immature teratomas, and the pathological criteria for benign lesions include the following four aspects: (I) absence of malignant or immature components in the tumor, (II) absence of other similar lesions elsewhere in the body, (III) normal levels of AFP and HCG, and (IV) absence of recurrence at follow-up . AlthougElevated AFP, β-HCG, and CEA were found in some immature teratomas, which were of great significance for preoperative diagnosis and prognosis . For theMy intermittent pain in my right lower back and abdomen was affecting my daily life and work, causing me great distress. After the doctor’s consultation, he helped me to make a correct diagnosis and opted for minimally invasive laparoscopic surgery to completely remove the tumor from my body and eliminate the pain in my upper right abdomen. My fears and worries about the tumor disappeared, especially after the pathology confirmed the diagnosis of a mature teratoma. I was able to achieve both physical and psychological healing. I consider that I have received a very successful treatment. I agreed to share my medical history and signed an informed consent form.We have described the features of imaging, pathology, and the treatment of the typical primary adrenal teratoma to further raise people’ awareness of this rare disease, and primary adrenal teratomas should be included in the differential diagnosis of adrenal masses.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The studies involving human participants were reviewed and approved by the Ethics Committee of the Affiliated Hospital of Zunyi Medical University. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.XW and XGL were the patient’s urologists, reviewed the literature, and contributed to manuscript drafting. HC, WX, XH and PS were responsible for the revision of the manuscript for important intellectual content. All authors contributed to the article and approved the submitted version.This study was supported by the National Natural Science Foundation of China (grant no. 81860524) and grants from the Department of Science and Technology of Guizhou Province .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.


The authors present the case of a 34-year-old patient with neurofibromatosis type 2 (NF-2) who underwent a left translabyrinthine approach for resection a meningioma, vestibular schwannoma, and placement of an auditory brainstem implant (ABI). They review the preoperative workup, technical nuances of the surgery, and cadaveric dissections with anatomical diagrams, and provide a review on ABIs. The patient remained neurologically intact and had improvement in lip reading when using the ABI device in the postoperative period.https://stream.cadmore.media/r10.3171/2021.7.FOCVID2163The video can be found here:  A 34-year-old female with NF-2 and multiple tumors, losing hearing on the left side that has failed Avastin as well as twice Gamma Knife to the left tumor.Here is the audiogram showing anacusis on the left side.One can appreciate on MRI the multiple meningiomas and the bilateral acoustic neuromas. The lesion of interest is on the left side.The rationale for doing the procedure is the complete loss of hearing on the left side, tumor growth, failure of previous radiosurgery twice and her young age with an opportunity to train her on the ABI before she loses hearing in the right hear.Risks of the procedure include infection, hematoma, facial nerve weakness, incorrect placement of the ABI, occasionally death. The benefits are of course tumor control and possibly hearing restoration to the left ear.The alternatives do not include radiosurgery anymore because she has already had the procedure performed twice.The setup includes supine position with a shoulder bump for a translabyrinthine approach with the head turned 45°. The necessary equipment includes the usual neuro-otologic and neurosurgical equipment as well as intraoperative monitoring.The translabyrinthine approach is shown here having been well underway on the left side. The neuro-otologist performs this part of the procedure.The sigmoid sinus can be decompressed further both presigmoid and retrosigmoid.The sinodural angle is being exposed here at the junction between the middle and the posterior fossa. Diamond drilling is being used close to the dura after using the cutting drill earlier in the drilling.The retrosigmoid decompression is important particularly when there are large tumors.The labyrinthectomy now is underway particularly with the superior semicircular canal here being delineated in a circular fashion. The fallopian canal is delineated. The lateral semicircular canal, the lumen of which is well seen. The sigmoid sinus is mobilized posteriorly and here is the presigmoid dura with the endolymphatic duct and endolymphatic sac visualized. One can appreciate the location of Donaldson’s line.Now as we are approaching the internal auditory canal the inferior trough is being delineated and drilled. The jugular bulb is noted and is not particularly high in this case.This is the superior trough of the internal auditory canal being delineated with the diamond drill bit.Here is a dissection and a schematic from Rhoton to remind us where choroid plexus, flocculus, and lateral recess of the fourth ventricle are.We are here placing the ABI receiver in a subcutaneous pocket.Here is the electrode end of the ABI.Again, the goal is to enter the lateral recess of the fourth ventricle to place it on top of the cochlear nuclei area through the foramen of Luschka. Naturally the correct surface of the electrode has to be placed down on the brainstem floor.Here is a schematic of where the various nuclei are in the floor of the fourth ventricle overlaid on a Rhoton dissection.The audiology team will then confirm correct placement by interrogating a pair of electrodes and to make sure we are on top of the cochlear area.ABI is the most common placed surface stimulator in the CNS. It was first developed in the 1970s at the House Ear Institute.The patient remained neurologically intact. She currently has 15 out of the 21 electrodes. Two of the electrodes are turned off because of a sensation in her throat. Four of them are turned off because they are stimulating probably the medial longitudinal fasciculus. She has improved significantly in her lip-reading ability when her implant is turned on. A postoperative MRI showed essentially gross-total resection of the acoustic neuroma.The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication.Primary surgeon: Morcos, Angeli. Assistant surgeon: Khan, Brown. Editing and drafting the video and abstract: Morcos, Brown. Critically revising the work: Morcos, Brown, Angeli. Reviewed submitted version of the work: Morcos, Brown. Approved the final version of the work on behalf of all authors: Morcos. Supervision: Morcos.


Metastasis or recurrence was not detected during the 7-year follow-up.Inflammatory pseudotumor (IPT)-like follicular dendritic cell sarcoma (FDCS) is a rare neoplasm referred to as the FDCS variant. Here we report a 66-year-old female patient suffering from hepatic IPT-like FDCS and summarize IPT-like FDCS reported in the literature. The patient presented with obvious abdominal pain without significant laboratory abnormalities and subsequently underwent surgical resection of a hepatic lesion. Postoperative pathological results demonstrated a vascular tissue-rich neoplasm (7.0-cm maximum diameter). The tumor cells expressed CD21 and CD35, and  Follicular dendritic cells (FDCs) develop from perivascular precursors of stromal cell origin that are essential for the organization and maintenance of lymphoid architecture, induction of the germinal center reaction, production of B memory cells, and protection from autoimmune disorders . FDC sarThe cause of IPT-like FDCS is unknown, and the diagnostic criteria are not definitive. However, Epstein–Barr virus (EBV) infection is considered one of the most important etiologies of this sarcoma . DistingHerein, we report the case of a 66-year-old female with an intraabdominal IPT-like FDCS in the hepatic right lobe. By analyzing the distinctive clinicopathologic features of this rare case combined with reviewing the related literature, here we summarize the current clinical features and diagnosis of IPT-like FDCS and discuss the treatment and prognosis of this tumor subtype.We systematically searched the PubMed, EMBASE, and MEDLINE databases using the search terms “inflammatory pseudotumor-like” combined with “follicular dendritic cell sarcoma,” or “follicular dendritic cell tumor,” or “fibroblastic dendritic cell sarcoma,” or “fibroblastic dendritic cell tumor” in research published from 2000 to 2022. We collated demographic, clinicopathological, and follow-up information .In 2015, a 66-year-old woman who suffered from right upper abdominal pain was admitted to the Department of Hepatic Surgery and Liver Transplantation Center at the Third Affiliated Hospital of Sun Yat-sen University because of a liver mass detected using abdominal ultrasound at a local hospital. Based on ultrasound examination and medical history, the preliminary diagnosis was liver-occupying lesions and diabetes mellitus type 2. The patient did not complain of vomiting, nausea, fever, or diarrhea. After admission, the values of routine tests including liver and kidney function and routine blood tests were almost within their normal limits. Liver function according to the Child–Pugh classification was class A. Serological analyses to detect hepatitis virus, syphilis, and human immunodeficiency virus were negative. Furthermore, the levels of common female-specific tumor markers, particularly α-fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen (CA)-199, and CA-125 were normal. The patient had a long history of diabetes mellitus type 2 and achieved good fasting plasma-glycemic control with acarbose combined with metformin.1WI. Therefore, the primary diagnosis highly suggested a hepatic fat-poor angioleiomyolipoma (Preoperative enhanced magnetic resonance imaging (MRI) showed a mass approximately 94 × 74 mm with clear borders in hepatic segments VI and VII. On enhanced phase, the images showed progressive enhancement of the lesion, while enhancement was not seen in the necrotic region. Significantly, the lesion demonstrated slightly hypointense speckled signals on in-phase and out-of-phase Tyolipoma .Although the diagnosis was not definitive, the large liver mass caused significant clinical symptoms  in the absence of concurrent systematic disease. Our multidisciplinary hepatic surgery team therefore proposed surgical resection as the most appropriate procedure to confirm a diagnosis and further formulate the treatment strategy. Subsequently, the patient underwent resection of hepatic segments VI and VII, and minor complications occurred postoperation. During surgery, lesions were not observed in the gastrointestinal tract, spleen, mesentery, or other abdominal organs. The operation lasted 135 min, and the estimated intraoperative blood loss was approximately 150 mL.in situ hybridization detection of Epstein–Barr virus-encoded RNA (EBER), the morphological and immunophenotypic results were consistent with a diagnosis of IPT-like FDCS  is a rare mesenchymal tumor of follicular dendritic cell origin originally identified by Monda et al. in 1986 . FDCS isInflammatory pseudotumor (IPT)-like FDCS possesses morphological and clinical features intermediate between inflammatory pseudotumors and FDC tumors and was first classified in 2001 as a distinct variant . CompareInflammatory pseudotumor (IPT)-like FDCS predominantly occurs in middle-aged adults , with marked female predominance . Patients are mainly asymptomatic or present with abdominal distension or pain, occasionally accompanied by systemic symptoms such as back pain, waist soreness, significant weight loss, fever, and weakness. In rare cases, IPT-like FDCS exhibits paraneoplastic arthritis .CDKN2A and RB1), and immune evasion genes (CD274 and PDCD1LG2) may be pathologically associated with IPT-like FDCS  may play an essential role in etiology. Stimulation by EBV may induce FDCs derived from mesenchymal cells to undergo neoplastic transformation and express CD21 and CD35 , 12. Intike FDCS .in situ hybridization to detect EBER.The morphology of IPT-like FDCS is similar to that of the conventional type. Gross examination reveals that most tumors exhibit a well-marginated, thin-walled, yellowish, soft tissue mass (maximum diameter = 7.54 ± 4.93 cm). In particular, localized hemorrhage or necrosis is observed within the tumor. The neoplastic cells, which may exhibit mild atypia, are usually spindle, ovoid, or polygonal and form storiform, fascicles, or trabecular arrays, which exhibit sparsely vesicular chromatin and distinct nucleoli . In partAlthough limited reports are available on the imaging features of IPT-like FDCS, they aid in making correct diagnoses before treatment when a neoplasm is detected . Most unThe diagnosis of IPT-like FDCS is invariably supported by immunohistochemistry, and multiple FDC markers are often necessary, including CD21, CD23, CD35, CXCL-13, D2-40, Clusterin, Fascin, epidermal growth factor receptor, and CNA42 . In partn = 11) experienced recurrence or metastasis during follow-up. Yet, PNP-associated IPT-like FDCS predominantly occurs in intraabdominal sites, indicating poor prognosis (Inflammatory pseudotumor (IPT)-like FDCS is a low-grade malignant tumor with good prognosis. Unlike FDCS, IPT-like FDCS is apparently indolent, with few instances of recurrence and metastasis . Diseaserognosis –10. Surgrognosis . NotablyInflammatory pseudotumor (IPT)-like FDCS is an extremely rare neoplasms that mainly occurs in the intraabdominal region. EBV probably plays an essential role in the etiology of IPT-like FDCS. The diagnosis of IPT-like FDCS is complex and usually relies on fine-needle aspiration biopsy or postoperative pathological diagnosis. Surgical resection is the most effective treatment, although the efficacy and safety of adjuvant chemotherapy, radiotherapy, or targeted therapy for postoperative management are unknown. IPT-like FDCS presents a certain risk of recurrence or metastasis after initial treatment. Thus, regular follow-up visits are strongly recommended.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The studies involving human participants were reviewed and approved by the Institutional Review Board review ([2021]12-114) at The Third Affiliated Hospital of Sun Yat-sen University in Guangzhou, China. The patients/participants provided their written informed consent to participate in this study.CX was the patient’s physician and responsible for the revision of the manuscript for important intellectual content. FD reviewed the literature and contributed to drafting the manuscript. CW performed the radiographic analysis. FD and HT conceptualized and designed the study, coordinated and supervised data collection, and critically reviewed the manuscript for important intellectual content. All authors issued final approval for the version to be submitted for publication.


Severe constipation is a frequent presentation but progression into a life-threatening acute compartment syndrome (ACS) is few and far between. This case highlights the typical physiological manifestations of ACS and the immediate benefits of correcting these physiological imbalances through the disimpaction of a massive faecaloma. Furthermore, in adult patients with a history of colonic dysmotility, adult Hirschsprung’s disease should be considered. Abdominal compartment syndrome (ACS) from a large faecaloma is rare; however, it has been previously documented . We pres2 and a creatinine count of 125μmol/L The computed tomography imaging of his abdomen revealed a massively dilated rectum and sigmoid colon extending to his splenic flexure without evidence of a perforation  of 68 mL/min/1.73 mforation  and 2. Pforation .The patient has a background history of two prior episodes of severe constipation requiring manual disimpaction. He had one attempted colonoscopy in 2017; however, the procedure was aborted due to inadequate bowel preparation; he subsequently failed to attend the follow up colonoscopies.He suddenly became hypotensive 6 hours postreview in emergency and required the vasopressin, metaraminol support despite 4 L of intravenous fluid resuscitation. His tachypnea progressively worsened and supplemental oxygen was started. The colorectal team was consulted upon, and the decision was made to evacuate the faecaloma in theatre.Upon manual evacuation of large faecaloma in theatre by the surgical fellow, the patient’s hemodynamics immediately improved and vasopressors were ceased. He was placed on a diet upgrade plan and was placed on an aggressive aperient and enema regime. He was discharged on day 4. He was rebooked for a sigmoidoscopy and biopsy to exclude Hirschsprung’s; unfortunately, he failed to attend the follow-up. On reflection, an opportunistic rectal biopsy during his disimpaction should have been considered..In all recorded cases of fecal impacted ACS, the diagnosis was made clinically. ACS occurs when intraabdominal pressure exceeds 20 mmHg with end organ dysfunction. Normal physiological abdominal pressures should remain between 0 and 4 mmHg in the adult population . Intraab2 expulsion. This may lead to a ventilation-perfusion mismatch hypoxemia. Cardiac shock may result from compression of the inferior vena cava resulting in decreased cardiac venous return.ACS can manifest vital signs as tachypnea, hypotension and tachycardia. Clinical examination will often reveal a tense abdomen and poor urine output . ExpansiMedical management in ACS can include nasogastric decompression, prokinetic agents, enemas and rectal decompression. The patient’s fluid balance should be closely monitored; and over resuscitation should be avoided to minimize third spacing. In some cases, diuretics may be considered .Measurements of intra-abdominal pressures can be helpful and can be taken either directly with a transducer via an intraperitoneal catheter or indirectly via intravesical transducers.Adult Hirschsprung disease (AHD) is the most likely etiology for our patient. AHD is a rare gastrointestinal motility disorder characterized by chronic constipation and abdominal distension secondary to the absence of ganglionic cells of the myenteric and submucosal nervous plexus, within segments of bowel. It is commonly identified in neonates and infants, but milder cases can evade diagnosis until acute presentations later in life. The gold standard diagnostic test is a full rectal biopsy but adjunctive tests such as anorectal manometry and a barium enema may be helpful. The common ‘pull-through’ elective surgical management involves removing aganglionic bowel and mobilizing normoganglionic bowel to create an anastomosis above the dentate line.All general surgeons should familiarize themselves with signs and symptoms of ACS and be able to understand the adjunctive resuscitative measures to manage ACS related physiology. However, prompt manual decompression is often the most important and curative treatment for patients with a large faecaloma.None declared.None.


This rare case vignette describes hypoglycemic, hyperinsulinemic nesidioblastosis in a female patient with prior Roux-en-Y gastric bypass. The patient presented with severe symptomatic hypoglycemia resistant to IV dextrose and diazoxide, requiring surgical resection. Traditional imaging found nonspecific findings, and biochemical analysis was inconsistent with insulinoma. A gallium-68 dotatate PET scan was utilized to successfully localize the tumor in the distal pancreas. She underwent laparoscopic resection of the distal pancreatic lesion with resolution of her symptoms and return to euglycemia. The histological evaluation confirmed the diagnosis of nesidioblastosis. Nesidioblastosis is a rare complication of bariatric surgery that may be more clinically relevant with rising prevalence of obesity. Diagnosis with conventional imaging modalities may be challenging; however, the dotatate PET scan may have high utility in detecting lesions. It is essential for clinicians to consider nesidioblastosis in the differential diagnosis of hyperinsulinemic hypoglycemic conditions and recognize there may be a link with increasing rates of bariatric surgery. Hyperinsulinemic hypoglycemia (HH) refers to uncontrolled insulin release from neoplastic beta cells or functionally defective pancreatic beta cells that results in hypoglycemia. This broad term is used to describe a number of disease pathologies with similar mechanisms and clinical presentation. Adult-onset, hyperinsulinemic hypoglycemic syndromes have a wide differential that usually necessitates systematic evaluation. Disease syndromes from endogenous insulin production include insulinoma, nesidioblastosis, and dumping syndrome. The remaining syndromes include surreptitious sulfonylurea or insulin use which needs a biochemical analysis for differentiation.Although rare, insulinomas are the most common cause of hyperinsulinemic hypoglycemia . InsulinNesidioblastosis is likely to be an increasing etiology for HH conditions, with rising bariatric rates. Nesidioblastosis was first identified in 1938 by Dr. Laidlaw who described the diffuse proliferation of pancreatic duct epithelium . Over tiβ-cell mass [β-cell hypertrophy in response to presurgical obesity [β-cell area compared to that of obese controls [The acquired form of nesidioblastosis most often occurs following an Roux-en-Y bypass, and pathophysiology remains controversial . Variousell mass , 13. In ell mass . The pre obesity . Meire econtrols , 15. Durcontrols . This macontrols . Althougcontrols . It is acontrols .It is essential to recognize that hypoglycemia after gastric bypass surgery does not strictly indicate altered pathology but is a side effect of altered anatomy and function. Meier and colleagues examined histopathology from 53 specimens, comparing patients with prior gastric bypass to obese patients and lean controls from autopsies . Not surIn this case vignette, we describe a rare hypoglycemic hyperinsulinemic nesidioblastosis in a 37-year-old female patient with prior gastric bypass and review the relevant diagnostic evaluation. As our population continues to have an increased prevalence of obesity, it is also likely that there will be an increase in rates of bariatric surgery and nesidioblastosis . We presA 37-year-old woman with a history of hypothyroidism, remote amphetamine use, and gastric bypass surgery presented with neuroglycemic episodes. She had gastric bypass surgery with Roux-en-Y 10 years ago and initially lost 120 pounds. In the last four months, she has had recurring bouts of syncope, diaphoresis, and unintentional weight loss of 60 pounds. She reported a history of hypoglycemia and stated she was told a pancreatic mass was found in her previous hospitalization four months earlier. She had no history of diabetes but had self-measured blood glucose levels of less than 50 mg/dL despite using over-the-counter glucose tablets. At the time of her presentation, she had taken up to six glucose tablets daily for low blood sugar. She had severe hypoglycemia of 43 mg/dL, non-anion gap metabolic acidosis, and hypokalemia at 2.8 mmol/L. She required ICU level of care for hourly blood glucose monitoring. A 3-day fast was attempted but her persistent and symptomatic hypoglycemia necessitated dextrose infusion, and her fasting was discontinued before the end of the first day. Diazoxide and continuous intravenous dextrose 5% in water (D5W) were given to attempt to normalize her blood glucose but with poor response to treatment and persistent hypoglycemia.An endocrinology consult was obtained and a systematic biochemical evaluation was initiated . Her bloShe had multiple imaging studies, including abdominal MRI and CT abdomen and pelvis, which had no significant findings except for a contour abnormality near the pancreatic tail identified by MRI imaging . She theGeneral surgery was consulted, and she subsequently underwent laparoscopic resection of the distal pancreas and splenectomy. A 0.6 × 0.6 × 0.3 cm pancreatic mass was identified . Patholo3The patient had a resolution of symptoms following the distal pancreatectomy and was safely discharged after the appropriate vaccination series for splenectomy.Our patient presented with severe symptomatic hypoglycemia resistant to diazoxide, requiring dextrose infusion with TPN. Her resistant hypoglycemia suggested insulinoma, but her biochemical analysis was not consistent with diagnosis. Her C-peptide and insulin levels were inappropriately normal, yet the proinsulin levels were not as elevated as expected. Routine imaging with CT suggested a possible lesion near the pancreas tail but overall was nondiagnostic. Endoscopic Ultrasound was unable to identify any lesion. A gallium-68 dotatate PET scan showed a focal radiotracer concentration in the distal pancreas suggesting a possible neuroendocrine tumor. She underwent laparoscopic resection of the distal pancreas mass, which confirmed nesidioblastosis . In a siDiagnosing nesidioblastosis in adults is challenging because traditional imaging techniques lack the sensitivity to differentiate between focal and diffuse hyperinsulinism . Common In our literature review, previous cases used selective arterial calcium-stimulation tests to localize and guide partial pancreatectomy , 17. We Treatment of nesidioblastosis differs for adults and children. Isolate disease in children can present throughout the pancreas and can be cured with partial pancreatectomy of the focal lesion , 9. The In many cases, surgery can confirm the diagnosis and provide treatment for severe cases , 20, 23.We propose that with increasing rates of obesity and correlating gastric bypass surgeries, more providers will be faced with elucidating the various causes of postprandial and fasting hypoglycemia , 30. We


Gliomatosis peritonei (GP) is a rare disease, usually associated with immature ovarian teratoma. GP may be rarely associated with mature ovarian teratoma. GP is composed of mature glial tissue elements, which histopathological examination can further confirm. Benign glial implants usually involve the omentum, peritoneum and lymph nodes. Many benign and malignant peritoneal diseases may mimic GP on clinical examination. GP may be confused with peritoneal carcinomatosis on computed tomography (CT) scan. A microscopic examination from peritoneal mass biopsy helps to rule out differential diagnosis. GP consists of mature glial tissue and is regarded as grade 0 according to the WHO grading of immature teratoma (IT). GP corresponds to a good prognosis with occasional cases showing malignant evolution. Gliomatosis peritonei (GP) is identified by multiple nodules affecting the omentum and peritoneum, and it shows mature glial tissue on histological examination. GP may be associated with immature or mature ovarian teratoma -3. CasesClinical and radiological featuresIT clinically presents as a large pelvic mass, primarily turning up in the first three decades. Serum tumour markers like alpha-fetoprotein (AFP) are within normal limits . On CT, MT is usually well encapsulated with regular margins and foci of bony tissue within cystic elements. Compared to MT, IT is larger 14-25 cm) and shows predominantly solid components. IT usually presents with capsule perforation and irregular margins on a CT scan. Foci of punctate fatty tissue and scattered calcifications in a solid ovarian mass on CT scan discriminate IT from MT  and CT gives the additional benefit of providing morphologic and functional details in the case of IT .GP on CT scan can present as multiple nodules involving the peritoneum and omentum with free fluid in the peritoneal cavity. The size of the peritoneal nodule varies from 0.3 to 1.2 cm in diameter . It is cRadiology provides details concerning tumour staging and further surgery planning .Gross and morphologyIntraoperatively GP may mimic peritoneal carcinomatosis. Grossly, GP presents multiple nodules having size ranging from 1 to 10 mm in diameter. They are small, greyish and well-circumscribed involving parietal and visceral peritoneum and omentum. Grossly it is challenging to differentiate GP from tuberculosis. IT presents as a large ovarian mass having an irregular outer surface. Tumour is mostly unilateral with cut surface showing mainly solid areas with focal cystic regions along with necrosis and haemorrhage ,10.Microscopically, IT is defined by immature tissue components, mainly immature neuroepithelium in the form of rosettes and primitive neuroectodermal tissue in the background of mature teratomatous elements. Histology shows mitotically active primitive cells with dark hyperchromatic nuclei and glial tissue foci in the backdrop Figures -3D.IT may present with immature mesodermal tissue in the form of immature cartilage. Very rarely, IT may show immature endodermal tissue. The case of IT should not show components of a yolk sac tumour; otherwise, the case will be labelled as a mixed germ cell tumour. If a histological picture of IT and serum AFP levels are raised, further additional sampling must be carried out to rule out any component of the yolk sac tumour ,10.According to WHO classification of Female Genital Tumours, 5th Edition; grading is decided by examining immature neuroepithelium under a low-power microscopic view (40×). If the aggregated amount of neuroepithelium is present in one or less than one 40× field, it is grade 1; if present in one to three 40× fields, it is grade 2 and if present in more than three 40× fields, it is classified as grade 3 IT. Here, 40× power is equivalent to a 4.5 mm diameter view. Interestingly, pure GP is regarded as a grade 0 (zero) mature component. It shows multiple nodules of mature glial tissue beneath the surface  Figures-4D.On immunohistochemistry, GP cells show characteristic positivity with SOX2 because SOX2 is necessary for preserving stem cell pluripotency and induction of neural cells origin from stem cells .Genetic profileBest et al. presented molecular studies in patients of ovarian mass diagnosed as IT with GP. Results showed that genetic changes in both lesions were mutually exclusive, indicating that neoplasms of IT and GP are genetically discrete. So, both are independent tumours, and GP does not represent tumour metastasis or tumour recurrence from IT mass . AnotherDifferential diagnosisGrowing teratoma syndrome (GTS) is a distinct entity characterised by a growing mass of MT in a known case of malignant germ cell tumour undergoing or having completed chemotherapy. GTS may contain mature tissue from any of three lineages, whilst GP shows only mature glial tissue. Unlike GTS, GP is not associated with chemotherapy treatment of malignant germ cell tumours .In some cases of ovarian teratoma, predominantly MT components are seen. If clinically, radiologically and grossly suspicious of immature or malignant nature, then extensive sampling should be conducted to detect any immature neuroepithelium and also need for accurate grading of the immature component. Peritoneal carcinomatosis and tuberculous peritonitis can be easily differentiated from glial nodules on histology ,10.Prognosis and predictive factorsThe patient is staged according to TNM staging of the ovary, fallopian tube and primary peritoneal carcinoma. Although GP is considered a stage III disease, its behaviour is mainly observed as benign .GP is considered a grade 0 (zero), so it usually bears an overall favourable prognosis and is treated conservatively accordingly . As GP lGP is an uncommon disease usually associated with IT of the ovary. In the case of ovarian teratoma, radio-imaging may help predict the immature or malignant nature of teratoma based on the identification of speckles of fatty tissue. In such suspected cases of IT, peritoneal and omental lesions should be searched, if any and should be excised along with ovarian mass to be sent for histopathological examination.Grossly, throughout sampling should be carried out from ovarian mass to detect any immature neuroepithelium or other immature mesodermal components. A combined clinical, radiological and histopathological diagnostic approach is required to confirm the GP associated with IT and to differentiate it from its mimickers. It is also vital to acknowledge the benign mature nature of GP in most cases which helps to avert any avoidable surgical procedure in young patients.


Key molecular alterations found in the diagnosis and prognosis of brain tumours have been revealed by the latest advances in transcriptomic and genome-wide analysis. In-depth studies revealed that alterations of the V-Raf murine sarcoma viral oncogene homolog B (BRAF) could be shared by different brain tumour types. The identification of BRAF p.V600E mutations in gliomas is nowadays of more importance regarding the development of BRAF-targeted inhibitors.This report presents the case of a 37-year-old female with a voluminous expansive neoplastic lesion, extending from the lenticulocapsular region to the medial aspect of the temporal lobe on the left. Pathological examination revealed an astrocytic neoplasm without high-grade histological features in small biopsy fragments. The molecular study revealed the presence of a mutation in the BRAF V600E gene and CDKN2A/2B homozygous deletion. The lesion was partially removed and irradiated. The patient has been on treatment with dabrafenib plus trametinib for 10 months. In addition to reasonable tolerance, she obtained an impressive tumour reduction, which was manifested in the complete resolution of neurological deficits and in the full acquisition of autonomy.The remarkable results reported in this clinical case justify the pressing need to identify new therapeutic targets in gliomas in the current era of precision medicine. Gliomas and glioneuronal tumours are the most prevalent group of intrinsic central nervous system (CNS) neoplasms. This category of tumours comprises a huge diversity of pathological types with diverse prognoses. Moreover, molecular studies, namely wide genome sequencing and methylation profiling, have been updating the knowledge about these CNS tumours and the recent revised fifth edition of the World Health Organization (WHO) classification included new, well-established molecular parameters in the glioma classification. Some specific molecular characteristics have been introduced in the definition of tumour types (i.e. IDH1/2 and histone H3 mutations) and others in grading criteria (i.e. CDKN2A/2B homozygous deletion) [This new integrative approach to the classification and grading of gliomas significantly improved the prediction of prognosis. In specific cases, it is also dependent on patient factors, such as the age of the patient and the location and size of the tumour. However, the therapeutic options for gliomas treatment remain limited , and onlV-Raf murine sarcoma viral oncogene homolog B1 (BRAF) gene alterations may be held in primary brain tumours . The BRAThe BRAF p.V600E mutation is not specific to a tumour type. It is commonly held in the category of pleomorphic xanthoastrocytoma and ganglioglioma and can also be found in a subset of pilocytic astrocytomas (PA) and glioblastomas (namely with epithelioid morphology) . Adult pBRAF inhibitors, which are small kinase inhibitors, bind specifically to V600E-mutated B-Raf proteins and do not allow them to activate MEK, consequently stopping the MAPK/ERK signalling cascade, inhibiting aberrant cell signalling. MEK inhibitors applied during therapy delay developing resistance to BRAF inhibitors and increase the time of tumour progression . So far,We present an adult-onset case of BRAF V600E-mutated extensive astrocytoma, conditioning a much higher burden of symptoms, partially resected and treated with a combination of BRAF and MEK inhibitors (BRAF+MEKi).A 37-year-old female with a history of papillary thyroid carcinoma operated in 2014 and currently in surveillance, was admitted to the emergency department for occipital headache and vomiting. A spontaneous subarachnoid haemorrhage was diagnosed.Brain CT showed a diffuse subarachnoid haemorrhage in the basal cistern, predominantly on the left side of the pentagonal cistern and with an important hematic component in the left Sylvian trench and grooves of the hemilateral temporo-frontal convexity Figure . AngiogrAfter three months, the patient was admitted to the emergency room due to loss of consciousness and right hemiplegia. Brain CT showed an acute-phase haemorrhagic lesion with an epicentre in the left lenticulocapsular region, where a hematoma, about 25 mm in diameter, was observed with dissection into the ventricular system, showing tetraventricular haemorrhage. Left uncal hernia and left lateral mesencephalic obliteration were seen. Ectasia of the left ventricular system with moderate hydrocephalus was visualised . It presented perilesional oedema and a clear mass effect on the third ventricle and the left lateral ventricle with a deviation of the median structures to the right of about 5.5 mm  were observed in neoplastic cells. No expression of H3 p.K27M or IDH-1 p.R132H was identified; ATRX expression was preserved; p53 expression was wildtype. The proliferative index assessed by the ki-67 index was up to 10% Figures , 4B, 4C.The next-generation sequencing (NGS) of the tumour revealed a mutation in the BRAF Val600Glu (V600E) gene. Homozygous deletion of CDKN2A/B genes was also detected. No evidence of alternative IDH1 or IDH2 mutations, EGFR amplification, or TERT promoter mutation was identified.The case was presented at the multidisciplinary meeting. Given the aggressiveness of the lesion and the impossibility of complete surgical resection, radiotherapy was proposed.She underwent external radiotherapy with a total dose of 54Gy, at 2Gy/day, in 27 fractions, with photon energy of 6MV, according to the three-dimensional conformal radiation therapy (3D CRT) technique.Considering the context of glioma with the characteristics of aggressiveness and chemoresistance and the molecular profile of homozygous deletion of the CDKN2A/B genes and BRAF gene mutation , she was proposed for treatment with dabrafenib plus trametinib. Before starting treatment, the patient had dysphasia, hypesthesia and right hemiparesis grade 4. She required Prednisolone at a dose of 30 mg, daily, orally, for better symptomatic control.Dabrafenib 150 mg, twice daily, plus trametinib 2 mg, once daily, orally was started. She presented optimal tolerance and adherence, without adverse effects. She gradually reduced corticosteroid therapy until discontinuation.The MRI reassessment performed five months after treatment was started, showed a decrease in the dimensions of the neoplastic lesion (26 x 20 mm larger in diameters). The last MRI showed stable disease. The imaging evolution from the beginning of the treatment to the last MRI can be seen in Figures Currently, the patient is autonomous in daily life activities, with no deficit in strength or language disorders.Brain tumours are more responsible for shortening years of life as compared to any other cancer . Such caAlthough the current available molecular studies do not allow a final classification, the histological features and the co-occurrence of a BRAF p.V600E mutation and CDKN2A/2B homozygous deletion raises the possibility of a high-grade astrocytoma with piloid features (HGAPF) . MethylaThe diagnosis of the newly recognized HGAPF is challenging due to the non-specific histological and molecular features which they share with PA MAPK pathway gene alterations. Moreover, they may present anaplastic histological features, ATRX mutation or loss of expression (up to 40% of cases), homozygous deletion of CDKN2A/B (up to 80% of cases), and aneuploidy, factors that were previously recognized as aggressive behaviour markers in PA in previous studies .PA therapy for adults lies on resection, although chemotherapy, radiation, or bevacizumab may also be considered . Full reOn the other hand, the prognosis in HGAPF is only available in one retrospective study, with a five-year survival rate of approximately 50% [The effectiveness of the BRAF and MEK inhibitors combination in BRAF V600E mutated gliomas treatment is shown in the phase 2 studies and reported in a systematic review , 8-9. PrThe presence of CDKN2A/B deletion is associated with worse prognosis and inferior response to chemotherapy, regardless of the co-existence of mutations in the BRAF or IDH1/2 genes . HoweverOne question that may be asked is what exact role radiotherapy played in response achievement. It certainly influenced tumour reduction, but as no imaging reassessment was performed before starting treatment with BRAF+MEKi, it does not allow us to confirm its exact contribution.This case exemplifies that dabrafenib plus trametinib allow achieving an objective response and significant clinical improvement, with an acceptable toxicity profile which is quite surprising given the considerable size of the lesion and the initial symptom burden. For now, efficacy data from studies using BRAF+MEKi seem to be promising, but phase three trials are lacking to give more robustness to its use. Furthermore, there are still a few case reports in the literature sharing experience with this therapy, so the remarkable results described in our study make it unique.Further studies are needed to clarify subsequent treatment in the case of progression and to recognize the mechanisms of resistance that may arise.This case reflects the importance of performing molecular screening as a means to identify new therapeutic targets. Only in this way was it possible for us to detect the presence of the BRAF p.V600E mutation and use targeted treatment. Our study portrays the effectiveness of BRAF+MEKi in an adult patient with a BRAF V600E mutation-positive glial neoplasm.