Enterocolic lymphocytic phlebitis is phlebitis of unknown etiology in which lymphocytes affect veins without arteries and shows evidence of systemic vasculitis in the intestinal wall and mesentery, mainly in the small intestine and colon. Although patients present with a variety of gastrointestinal symptoms and findings like those of inflammatory bowel disease or ischemic bowel disease, there are no specific findings for enterocolic lymphocytic phlebitis. As a result, a diagnosis tends to be made after surgery. There are few case reports of enterocolic lymphocytic phlebitis, and the impact of chronic courses and immunosuppressive drugs on enterocolic lymphocytic phlebitis is not well known. A 47-year-old man was treated with infliximab and steroids for unexplained ulceration and narrowing of the ileocecal area, which was suspected to be inflammatory bowel disease with atypical findings. Lymphocytic phlebitis was noted in the surgical specimen, and enterocolic lymphocytic phlebitis was diagnosed. No recurrence of enterocolic lymphocytic phlebitis was observed postoperatively. This disease should also be considered among patients with inflammatory bowel disease-like lesions that do not respond to infliximab or steroids. Enterocolic lymphocytic phlebitis (ELP) is a lymphocytic phlebitis of unknown etiology that occurs in the mesentery and intestinal wall with predominant infiltration of T cells . It doesCases of what appear to be the same disease have been reported as mesenteric inflammatory veno-occlusive disease (MIVOD), intramural mesenteric venulitis, lymphocytic venulitis , and chrGastrointestinal symptoms such as abdominal pain, nausea and vomiting, diarrhea, and gastrointestinal bleeding have been reported in some cases –13. The There is no established method for preoperative diagnosis of ELP. In addition, medical treatments other than surgery are unknown.In this study, we encountered a case of ELP that was not diagnosed preoperatively but had been treated medically as Crohn's disease due to its long-term course. It is rare to find a case with a long-term history of ELP that underwent medical treatment prior to surgery, and the histology of this case is meaningful when considering the effect of the treatment.A 47-year-old man had been experiencing abdominal pain for a year. The patient underwent colonoscopy (CS) and computed tomography (CT) performed by a local doctor, which revealed a huge ulcer in the ileocecal region and associated narrowing of the ileocecal region. Biopsy and culture tests were repeated. However, this did not lead to a definitive diagnosis. Gastrointestinal Behçet's disease or Crohn's disease was suspected.The patient was treated with infliximab (IFX), 5-aminosalicylic acid (5ASA), colchicine, and budesonide, but the therapeutic agents were not effective enough to achieve remission.The patient visited our hospital 7 months after the onset of symptoms. At our hospital, double-balloon endoscopy (DBE) and small bowel follow-through (SBFT) were performed, and no foci other than the main lesion were found. First, IFX, 5ASA, and budesonide were continued, but due to lack of therapeutic effect, IFX and budesonide were discontinued five months after presenting to our hospital, and the treatment was switched to ustekinumab (UST). The patient was admitted to the hospital with diarrhea and vomiting, and contrast-enhanced (CE) CT revealed thickening of the wall of the ileum. CECT showed thickening of the ileocecal wall, and culture, T-spot, and C7HRP tests were all negative.Clinical symptoms were ameliorated with intravenous fluids and fasting, but stenosis of the ileocecal lesion was worse on DBE and SBFT . EndoscoSurgical specimens showed lymphocytic phlebitis in small- to medium-sized veins without arterial infiltration Figures . MoreoveIn addition, the distribution of lymphocytic phlebitis was characteristic. Lymphocytic phlebitis was found in the ascending colon and terminal ileum regions, where the mucosa covering the central and lateral portions of the lesion was normal. In contrast, in the central part of the lesion, only ulcers with severe inflammation and fibrosis were found, and no lymphocytic phlebitis was found Figures . HistoloThe patient had a good postoperative course and was discharged from the hospital. Since then, no recurrence of ELP has been observed.Two characteristics of ELP are shown in this case. First, regarding the diagnosis, the veins in the intestinal wall in the area slightly outside the main locus of the lesion need to be observed to determine findings of ELP. Second, immunosuppressive drugs may not be effective in treating ELP.It would be ideal to diagnose ELP preoperatively. However, ELP has no specific findings from endoscopy or imaging studies . The onlELP is often associated with an underlying disease , 15, 16.In fact, many cases of ELP are diagnosed after bowel resection , 17, 18 In this case, the histological findings of ELP were concentrated in the outer normal submucosal area of the lesion, and the central area was mainly characterized with ulceration and fibrosis with no phlebitis findings. Because of the relatively long history of this case from the onset of the disease to the surgery, it is likely that any specific findings had disappeared due to chronic secondary changes in the central region.Therefore, to diagnose ELP, it may be helpful to look at the veins in the intestinal wall in the area slightly outside the main site of the lesion.Secondly, there is no established medical treatment for ELP. The cause of ELP is unknown, but it has been reported to be associated with diversion colitis in the context of IBD . DiversiTherefore, it is expected that an autoimmune mechanism may be associated with changes in the intestinal microbiota . In addiHowever, immunosuppressive drugs such as IFX, UST, and steroids were used preoperatively in this case with no clinical improvement in the condition, and the specific findings from the surgical specimens indicated that these drugs may not be effective for ELP.In conclusion, for lesions that do not respond to IFX or UST and show a tendency toward narrowing, it is important to consider surgery for definitive diagnosis.Furthermore, recurrence after surgery for ELP is very rare , 14; henThere have been few reports on ELP. Moreover, the cause of ELP is unknown, and diagnostic and therapeutic methods have not yet been fully established. Therefore, a further accumulation of cases is necessary.


The most common presenting symptoms of colovesical fistulae (CVF) are pneumaturia and fecaluria. The most important aspect remains not only to investigate the aetiology, and the degree of both severity and complexity, but also the subsequent influence of this on overall management. In a younger population, management usually consists of curative surgery. However, this may not be possible in older patients where surgical candidacy is a genuine concern and a clinical challenge arises relating to pursuing a conservative strategy. We attempted to briefly outline how two patients were managed with a similar non-surgical approach due to frailty. These cases attempt to highlight the importance of multi-disciplinary specialty input, with a view to optimising patient care. A colovesical fistula (CVF) is defined as an abnormal connection between the colon and urinary bladder . The preThe male-to-female ratio of CVF is approximately 2:1 to 3:1 -10. It hWe present a case series report of two female patients, with different aetiologies contributing to the development of colovesical fistulae, along with the sequelae.Case series reportCase 1Escherichia coli with extended-spectrum beta-lactamase (ESBL) positivity. Admission blood tests showed a mildly raised C-reactive protein (CRP) 32 [An 83-year-old female was admitted with dysuria, pneumaturia, and malaise. Past medical history included diverticulosis, hypertension, gout, Barrett’s oesophagus, recurrent urinary tract infections (UTIs), stable non-Hodgkin's lymphoma, and total abdominal hysterectomy (1983). On examination, baseline observations were stable, and mild suprapubic tenderness was noted. A bedside urine test was positive for nitrites and leukocytes; the urine culture identified (CRP) 32 , with a (CRP) 32 -11. InitGiven the history of recurrent UTIs and recent pneumaturia, a CT-abdomen and pelvis was performed to exclude a colovesical fistula Figure . SubsequCase 23 heavy mixed growth. Initial management consisted of intravenous fluids and antibiotics. A CT-head excluded an acute intracranial event. Due to the recent diagnosis of type 1 diabetes mellitus in an older patient, a CT-abdomen and pelvis was performed to exclude a pancreatic malignancy. Whilst no concerning pancreatic pathology was identified, a large colovesical fistula was incidentally identified (Figure An 88-year-old female presented to the emergency department with acute confusion. Past medical history included a recent diagnosis of type 1 diabetes mellitus, severe frailty, multiple falls, sigmoid diverticulosis. Initial baseline observations were unremarkable. Admission blood tests were essentially unremarkable. A bedside urine test was positive for leukocytes and nitrite; the subsequent culture revealed >105/cmThe case was discussed in a multi-disciplinary meeting with input from clinical specialties including gastroenterology, general surgery, urology, and radiology. A decision was made in light of her severe frailty for non-surgical management. Following advice from microbiology, alternating prophylactic antibiotic regimes were commenced, rotating every three months. Periodic follow-up in the surgical outpatient clinic was also arranged and the patient was discharged.The first description of a colovesical fistula has been reported to be attributed to Rufus of Ephesus in AD 200 ; howeverA number of causes have been reported to be the aetiology of colovesical fistulae, with the most notorious being a diverticular disease, as well as a sequel to inflammatory bowel disease, more specifically Crohn’s disease ,14. HolmWe attempted to highlight the case series report of two patients. One case had the usual sequel including a background history of abdominal hysterectomy. The second case presented with no appropriate cause or iatrogenic background, presenting as a clinical challenge. Both patients were managed in a similar way following a conservative approach due to frailty and patient autonomy. This emphasizes that patients can be managed conservatively in cases where surgical intervention may not be appropriate.Colovesical fistulae can occur with or without previous history of the bowel, bladder, or gynaecological procedures. Whilst surgical input is justified in the younger population, management should be decided on a case-by-case basis, with a non-surgical approach being suitable among a frailer populace. Utilisation of imaging together with a multi-disciplinary specialty approach within secondary care permits a patient-centered approach to CVF management.


Congenital adrenal hyperplasia is an autosomal recessive disease most commonly associated with 21-hydroxylase deficiency, an enzyme integral in the biosynthesis of mineralocorticoids and glucocorticoids. We present a case of a 49-year-old male with congenital adrenal hyperplasia and commonly associated findings of adrenal myelolipoma, testicular adrenal rest tumors, as well as primary pigmented nodular adrenocortical disease. Adrenal myelolipoma is a rare, benign disease process associated with exogenous steroid treatment noncompliance in the setting of congenital adrenal hyperplasia. Testicular adrenal rest tumors are benign testicular tumors associated with congenital adrenal hyperplasia. Primary pigmented nodular adrenocortical disease is an ACTH-independent cortisol producing lesion. Our case emphasizes the association of congenital adrenal hyperplasia with adrenal myelolipoma and testicular adrenal rest tumors as well as the importance of familiarity with these associations to guide patient management. Primary pigmented nodular adrenocortical disease (PPNAD) is a rare benign adrenal condition characterized by ACTH-independent autonomous hypersecretion of cortisol, leading to Cushing syndrome, though manifestation is often subclinical We report a case of PPNAD in a patient with history of classic congenital adrenal hyperplasia (CAH) and bilateral giant adrenal myelolipomas and testicular adrenal rests.We present a case of 49-year-old male with history of classic sub-type congenital adrenal hyperplasia, diagnosed at infancy, seen at the endocrinology clinic for salt craving, lightheadedness when working in hot conditions, increased pigmentation in sun exposed areas, and small testicular size. He reported normal facial hair growth and denied issues with libido. Reportedly, he was not on any medications for 20 years prior to presentation. His vitals were within normal limits. Pertinent laboratory workup was as follows: Androstenedione 2290  and 17-hydroxyprogesterone 8230 . After this visit he was started on Prednisone (Deltasone) 2.5 mg, twice daily.Further evaluation with CT of the abdomen and pelvis demonstrated multilobulated predominantly fatty masses with interspersed weblike areas of soft tissue density. Findings were consistent with giant adrenal myelolipomas measuring 7.3 × 2.7 × 5.8 cm on the right and 18.0 × 13.4 × 12.0 cm on the left .Fig. 1AxUltrasound of scrotum was performed, which revealed numerous bilateral mixed hyperechoic and hypoechoic masses in the mediastinum testis. Imaging features and clinical presentation were compatible with testicular adrenal rest tumors . The bilOver the next several months, the patient was followed with interval laboratory values at one and two months. One month laboratory values of androstenedione of 870  and 17-hydroxyprogesterone 4170  and two-month laboratory values of Androstenedione of 73  and 17-hydroxyprogesterone of 289 . With interval improvement in laboratory values, the patient was reimaged at two months with CT of the abdomen and pelvis, which demonstrated interval decrease in size of the large left adrenal myelolipoma and overall unchanged size on the right with the right mass measuring 7.1 × 4.2 × 5 cm and the left measuring 16.8 × 12.0 × 10.4 cm . RemaindDuring the interim 3 months, the patient established with surgical oncology with chief complaint of back and abdominal pain. Possibility of this pain being related to the large bilateral adrenal myelolipomas was discussed. At this time the decision was made to continue to treat patient medically with oral prednisone therapy improving lab values. Patient continued to be followed with laboratory values at 5 and 6 months. Laboratory values at 5 months Androstenedione of 1020  and 17-hydroxyprogesterone of 7080  and at six months Androstenedione of 707  and 17-hydroxyprogesterone of 9390 . With interval worsening of laboratory values despite medical management with prednisone, patient was reevaluated by surgical oncology for abdominal pain. Patient elected for surgical resection of the bilateral adrenal masses with the goal of symptom control.Patient underwent bilateral adrenalectomy and pathology from the bilateral adrenal masses reported primary pigmented nodular adrenal disease with accompanying myelolipoma , Fig. 6.Adrenal myelolipomas are benign tumors associated with congenital adrenal hyperplasia (CAH) especially in the setting of noncompliance with exogenous steroid treatment CT imaging findings display the constitutive histologic composition of adipocytes, appearing as well circumscribed hypodensity, and myeloid tissue, appearing as variegated increased density interspersed throughout the mass ,7. ComplThe main complication associated with TART is infertility as a result of testicular structural and blood flow impairment secondary to tumor burden Primary pigmented nodular adrenocortical disease is an ACTH-independent cortisol producing lesion. PPNAD is the most common form of ACTH-independent micronodular hyperplasia or dysplasia that leads to increased cortisol production ,2. The hCases of PPNAD may arise sporadically but is more commonly familial. The familial variant may arise as isolated PPNAD or be associated with Carney complex (CNC), with PPNAD being one of the major diagnostic criteria This case report highlights the unique pathological findings in a patient with PPNAD who did not present with typical symptoms of Cushing syndrome and does not show any findings on imaging other than enlarged gland size most consistent with adrenal myelolipoma.PRKAR1A) and chromosome 2p16 (PRKAR1A-negative patients) As briefly mentioned above, Carney complex, which occurs in association with PPNAD, is a multiple endocrine and nonendocrine neoplastic syndrome with associated skin findings. Cases of CNC can arise due to de novo germline mutations, but CNC also has an autosomal dominant inheritance pattern with high penetrance ,11,12. TThe endocrine neoplasms associated with CNC include primary pigmented nodular adrenocortical disease (occurring in 25%-60% of patients with CNC), asymptomatic growth hormone hypersecretion (up to 75%), large cell calcifying Sertoli cell tumor, and other pituitary, thyroid and ovarian tumors Diagnosis is made after clinical suspicion in patients presenting with the above cutaneous findings when 2 or more out of the major clinical manifestations of the disease are found through testing. If a first-degree relative is positive for CNC or there is an inactivating mutation of PRKAR1A gene, only one clinical manifestation is needed to make the diagnosis While this case demonstrates a patient that presented with endocrine tumors, including that of PPNAD, at time of submission the patient was not evaluated further for Carney complex due to the lack of other clinical manifestations. Our case report emphasizes the important association between that of PPNAD and Carney complex, showing that PPNAD can arise sporadically or as an isolated case without the other manifestations of CNC.Informed consent was obtained from the patient.


PTEN gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR.Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and  Papillary tumors of the pineal region (PTPR) are rare pineal parenchymal tumors with a 1% prevalence among intra-cranial tumors in adults and which more commonly arise in children ,3. AlthoPTEN) gene located on chromosome 10q23 is a tumor suppressor gene and is inactivated in many brain tumors, which are frequently encountered in PTPR [Molecular studies have demonstrated that over 75% of PTPR are characterized by loss of chromosome 10 [ in PTPR . The PTE in PTPR ,10. Cyto in PTPR . Loss of in PTPR .PTEN-deficient cancers [PTEN loss have exhibited increased PFS with everolimus treatment combined with other drugs; however, this has not been thoroughly investigated in PTPR [PTEN-mutated PTPR and a dysregulated PI3K/AKT/mTOR pathway described a durable response to everolimus within 19 months, however only after a course of temozolomide chemotherapy [In patients with PTEN loss, considerations arise for targeting downstream signaling via mTORC1 inhibitors such as everolimus, reducing the activity of the downstream effectors and inhibiting cancer cell proliferation and survival, as well as angiogenesis . Studies cancers . Metasta in PTPR ,8,9. Onlotherapy . In addiotherapy .PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus as a monopharmacotherapy after surgical excision and radiotherapy, resulting in an asymptomatic course and tumor regression, a rare phenomenon not described in the literature so far.We describe a case of recurrent intracranial anaplastic PTPR characterized by a In March 2017, a 25-year-old male diagnosed with pineal anaplastic ependymoma (WHO grade III) at another institution in 2013 presented to our tertiary healthcare center to follow up his disease status after complete resection via craniotomy followed by adjuvant radiotherapy, with 2 years of stable disease shown on Magnetic Resonance Imaging (MRI)s.However, in August 2016, an MRI showed local recurrence and disease progression, with five lesions with cystic and solid enhancement noted upon imaging; three lesions were located in the left and right cerebellar hemispheres, with the largest diameter measuring 2.5 cm, while two lesions were located in the tectal–pineal region, with the largest measuring 1.4 cm. His physician at another institution recommended re-irradiation at this point, after which the patient then presented to our institution in April 2017 to request a second opinion, reporting severe, throbbing headaches of intermittent frequency and associated with sonophobia and photophobia and not relieved by painkillers, as well as mild imbalance during tandem gait in a physical exam. He had been maintained on daily dexamethasone (1 mg) for vasogenic edema and relief of headaches since the surgical resection. Repeat brain MRIs showed disease progression in both the pineal and cerebellar masses, with extension into the left third ventricle.® genomic test , which revealed PTEN loss with R130Q alteration and a single copy number deletion of chromosome 10, a characteristic of Papillary Tumor of the Pineal Region (PTPR), after which a definitive diagnosis of PTPR was established. A multidisciplinary discussion in June 2017 culminated in starting treatment with 10 mg of everolimus  and continuing with 1 mg of dexamethasone for headaches.Surgery was not an option at this point; a multidisciplinary team recommended comprehensive genomic profiling (CGP) and opted for a FoundationOneAn MRI done after 2 months of treatment with everolimus in August 2017 showed increases in the sizes of the cerebellar masses, with progressive perilesional vasogenic edema with mild obstructive hydrocephalus, despite no change in the pineal lesions, until September 2017 when the pineal mass increased in size.Follow-up MRIs after 6 and 9 months of treatment in December 2017 and March 2018 revealed reductions in the solid components of both the cerebellar and pineal masses, despite an increase in the cystic component and persistent hydrocephalus . Since tA maximal response to everolimus was achieved. In an attempt to maximize response and reduce tumor burden for the patient while having reached the maximum tolerable dose, a multidisciplinary team meeting yielded the decision to proceed with a suboccipital posterior fossa craniotomy with endoscopic third ventriculotomy in June 2018 to resect the cerebellar lesions, after which the patient experienced no complications and continued everolimus. An official pathology report of the resected cerebellar mass revealed monomorphic cells with scattered larger atypical cells arranged in perivascular pseudo-rosettes and true rosettes, in addition to necrosis and microvascular proliferation. The tumor intensely stained GFAP in the cytoplasmic processes, especially in perivascular tumor cells, in addition to cytokeratin 8/18.In July 2018, follow-up MRI showed regression in the size of the pineal tumor. To further maximize response, and in-line with the multidisciplinary decision, the patient then received radiotherapy for 2 months, after which MRI showed further regression, followed by stability in size noted in September 2019.MRI done in January 2020 then showed an increase in the size of the pineal gland tumor, which then remained stable until June 2020, after which surgical resection was performed. At the patient’s last follow-up in January 2021, he was completely asymptomatic while being maintained on everolimus. The patient provided fully informed, voluntary, and written consent to publish his data.PTEN P130Q alteration with chromosome 10 loss, and the patient was, thus, definitively diagnosed with PTPR (grade III) and started on 10 mg everolimus in June 2017. Slight disease progression was then demonstrated, followed by mild reduction in size then subsequent stability before further natural relapse, after which surgery was done to maximize response and quality of life after reaching the maximum tolerated dose of everolimus (An adult patient diagnosed with anaplastic ependymoma (WHO grade III) presented to our healthcare center in 2017, with disease progression in cerebellar and pineal masses noted on MRI after complete resection, radiotherapy, and stable disease for 2 years. Next-generation sequencing revealed a erolimus .Histologically, rosettes were observed in addition to positivity for GFAP and cytokeratin. Several histological similarities make it difficult to discern a definitive diagnosis of PTPR in light of a differential diagnosis of an ependymoma, as reported in the literature . HoweverPTEN P130Q alterations of this tumor type, the healthcare team chose to treat him with everolimus, although this is rarely used for PTPR. The patient experienced a smooth clinical course with no adverse effects from treatment and has been maintained on everolimus for 43 months now, with a completely asymptomatic course throughout, suggesting notable implications for patient quality of life. Control and an overall trend of tumor regression were attained over 27 continuous months on everolimus. In light of the nature of recurrent relapses in PTPR, the value of prolonged remission is essential; everolimus is deemed an essential therapeutic consideration for a prolonged window of asymptomatic disease control, with implications for prolonged PFS and improved quality of life.Considering that there are no approved therapies specific to This is the first case to report the treatment of adult PTPR (grade III) with response to everolimus as a monopharmacotherapy, with attainment of remission within less than one year of treatment with everolimus and overall control for over two years, the longest follow-up duration noted for such a case in the literature. Accordingly, there remains a necessity for future studies with large sample sizes to further validate the therapeutic effects of everolimus in adult patients with PTPR, and we recommend further investigation.PTEN and chromosome 10 loss, with implications for further consideration and investigation of the efficacy of everolimus as a possible novel treatment strategy. More studies on effective treatment strategies investigating the mTOR pathway for PTPR are needed. Further insight into the differentiating factors between ependymomas and PTPR may provide a better understanding of these tumors to facilitate quicker and more accurate diagnoses and pave the way for prognostic and therapeutic implications.Everolimus treatment in this patient exhibited its potential to treat recurrent PTPR among adults with


Although eosinophils are commonly present on the mucosa of the gastrointestinal tract, various pathological conditions may cause a secondary increase in eosinophil quantity.1).A 78-year-old man was referred to our hospital due to abdominal pain. Examinations revealed an ulcerative lesion with white moss in the terminal ileum and severe stenosis on the oral and anal sides. Tissue biopsies obtained from the ulcer margins showed a predominance of chronic inflammatory cells and abundant eosinophils in addition to lymphocytes/plasma cells. Secondary causes of tissue eosinophilia were suspected; however, the diagnosis could not be confirmed because of atypical endoscopic findings. Partial resection of the ileum was performed for therapeutic and diagnostic purposes. Histopathology of the resected specimen identified a lymphoepithelial lesion with an invasive tendency. While CD20 staining was positive, MUM-1 and Bcl-6 staining were negative. Based on these findings, the lesion was diagnosed as a small intestinal mucosa-associated lymphoid tissue lymphoma (Lugano staging, stage IIHypereosinophilia in this lesion was suggested to be secondary to chronic inflammation due to tumor growth or impaired transit.There is a type of gastrointestinal MALT lymphoma showing an invasive tendency. In such cases, it may demonstrate atypical findings and hypereosinophilia in gastrointestinal tissues. •Eosinophils are commonly present on the GI mucosa floor.•Abnormal/pathological conditions, include malignant lymphoma, can increase this concentration.•Some gastrointestinal MALT lymphomas have a strong invasive tendency.•Lymphocytes may infiltrate the deep ulcer and form severe stenosis.•Sometimes, it is difficult to identify by endoscopic findings and needs surgical specimen for definitive diagnosis. Malignant lymphoma arising from the small intestine may be challenging to diagnose due to limited examination conditions; however, it could be diagnosed by histopathological examination of surgical specimens. This case report discusses a case of mucosa-associated lymphoid tissue (MALT) lymphoma of the ileum diagnosed by examining a surgical specimen from an eosinophil-rich deep ulcerative lesion and severe stenoses due to strong infiltrative tendency. This work has been reported in accordance with the SCARE criteria 2Mycobacterium tuberculosis using small intestinal tissues was negative. Capsule endoscopy was scheduled but could not be performed because the patent capsule could not pass through the stenotic lesions. We suspected the condition to be among diseases causing secondary hypereosinophilia, such as ischemic enteritis, drug-induced enteritis, malignancy, or inflammatory bowel disease, as well as eosinophilic enterocolitis (primary hypereosinophilia). However, the endoscopic findings of this lesion did not correlate with the results of tissue biopsy performed under limited conditions, making it challenging to confirm the diagnosis. As the stenotic lesions were severe, we planned partial ileal resection for therapeutic and diagnostic purposes.A 78-year-old man was referred to our hospital because of abdominal pain after eating. The patient had been on a diet and medications  for hypertension, hyperlipidemia, hyperuricemia, and benign prostatic hyperplasia. There was no medical history of regular steroid or non-steroidal anti-inflammatory drug use. Laboratory results showed anemia . No other abnormalities were observed . Tumor markers were within the reference ranges . Abdominal contrast-enhanced computed tomography (CT) showed a contrast-effective circumferential wall thickening at the terminal ileum a. EnteroThe operation was performed under general anesthesia in the supine position. After laparotomy, we checked the entire small intestine and found no abnormalities other than these stenotic lesions, which were 30–35 cm away from the oral side of the terminal ileum. The ileum was anastomosed end-to-end after partial resection of a 10 cm ileal section that harbored the stenotic lesions. The anastomosis was located 25 cm away from the oral side of the terminal ileum.1).Histopathological examination of the surgical specimen revealed that the tumor had invaded the intestinal mucosa and submucosa, and the caved-in ulcer had formed on the mucosa a-1, b-1.Postoperative course was good, and the patient was discharged from our hospital on postoperative day 11. Esophagogastroduodenoscopy, capsule endoscopy, and positron emission tomography-CT were performed to examine the entire body. These examinations did not find any localized remnants or lesions in other organs. Based on this, no additional treatment was considered necessary, apart from continual close observation of the patient. One year and 7 months postoperatively, there is no sign of recurrence.3Generally, eosinophils are spread throughout the mucous membranes of the gastrointestinal tract. However, when the gastrointestinal mucosal epithelium is damaged by food-derived antigens, pathogens, or other molecules, eosinophils accumulate in the mucosa. Various specific receptors, such as cytokines, chemokines, chemical mediators, and immunoglobulins, are expressed on the cell surface of eosinophils. The binding of molecules to these receptors triggers functions such as tissue damage, promoting and alleviating inflammation, tissue remodeling, immune modulation, and innate immunity MALT lymphoma is a rare type of B-cell lymphoma that is associated with abdominal pain. It accounts for only 8% of non-Hodgkin's lymphoma cases and occurs in extranodal sites throughout the body. The stomach is the most common primary site of origin (85%), whereas origination from the small intestine or colon is rare (8%–28%) In this present case, we considered the possibility that the eosinophils had increased secondary to comorbidities, included malignancy. However, it was challenging to consider primary small intestinal cancer or malignant lymphoma based on the endoscopic findings, i.e., coexistent deep ulceration and severe stenotic lesions. In addition, the patient did not meet the diagnostic criteria for eosinophilic enterocolitis. Histopathological examination of the resected specimen showed an invasive tendency, as it had invaded the mucosa and infiltrated lymphocytes accumulated under the mucosa. These features suggested that in addition to the formation of broad, shallow, and sunken ulcerative lesions on the mucosal surface, multiple severe stenotic lesions had formed due to disease progression. Hypereosinophilia in this lesion was suggested to be secondary to chronic inflammation due to tumor growth or impaired transit. In such cases, surgical resection is meaningful in terms of diagnosis and treatment.4There is a type of gastrointestinal MALT lymphoma showing a strong tendency to invade. In such cases, lymphocytes may infiltrate the mucosal surface at the base of the deep ulcer and form severe stenosis. In some cases that are difficult to identify by endoscopic findings, surgical specimen is required for definitive diagnosis.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This is a case report and did not therefore require ethical approval from an ethics committee.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of written consent is available for review by the Editor-in-Chief of this journal upon request.KY is the corresponding author. KY, HO, SU, YW, TH, and ST contributed to manuscript preparation. KY, HO, SU, and YW contributed to patient management. All authors read and approved the final manuscript.Not applicable.Shinya Takahashi, Department of Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan.The authors declare no conflicts of interest.


Dissemination of glioma in humans can occur as leptomeningeal nodules, diffuse leptomeningeal lesions, or ependymal lesions. Cerebrospinal fluid (CSF) drop metastasis of glioma is not well-recognized in dogs. Ten dogs with at least two anatomically distinct and histologically confirmed foci of glioma were included in this study. The 10 dogs underwent 28 magnetic resonance imaging (MRI) examinations, with distant CSF drop metastasis revealed in 13 MRIs. The CSF drop metastases appeared as leptomeningeal nodules in four dogs, diffuse leptomeningeal lesions in six dogs, and ependymal lesions in seven dogs; six dogs had a combination of lesion types. Primary tumors were generally T2-heterogeneous and contrast-enhancing. Many metastases were T2-homogeneous and non-enhancing. Diffuse leptomeningeal lesions were seen as widespread extra-axial contrast-enhancement, again very dissimilar to the intra-axial primary mass. Primary masses were rostrotentorial, whereas metastases generally occurred in the direction of CSF flow, in ventricles, CSF cisterns, and the central canal or leptomeninges of the cervical or thoracolumbar spinal cord. Seven of the dogs had received therapy limited to the primary mass, such as surgery or stereotactic radiation, then developed metastasis in the following months. CSF drop metastasis of glioma may take a very different appearance on MRI to the primary mass, including periventricular lesions that are more homogeneous and less contrast-enhancing, rostral horn signal changes, or leptomeningeal enhancement ventral to the brainstem or encircling the spinal cord. Oligotastasis , 7.” Suctastasis , 7. Neurtastasis , 7. In otastasis .The MRI characteristics of canine glioma CSF drop metastasis are not well-described. A scheme exists for classifying the dissemination of human glioma . The goaSix male (four castrated) and four spayed female dogs, aged 2.3-12.9  years, met the inclusion criteria. This included three Boxer dogs, two English Bulldogs, and one each of French Bulldog, Staffordshire Terrier, American Pit Bull Terrier mix, Border Terrier, and Dachshund.n = 7). Group B cases had multifocal lesions (primary and metastatic) at first presentation (n = 3).We recognized two clinically distinct groups of cases . The firAll seven cases initially presented with a focal neurolocalization based upon a neurological examination, had a solitary mass on the first MRI, and received treatment. In six cases, unilateral forebrain neurological deficits and seizures accompanied a singular prosencephalic mass on MRI. The other case presented for bilateral blindness with an optic chiasm–parasellar singular mass on MRI. After metastasis, additional neurological deficits included central vestibular syndrome (Cases A1 and A2), status epilepticus, bilateral forebrain deficits, worsening unilateral forebrain deficits, neck pain, or paraparesis .n = 4), stereotactic biopsy and convection-enhanced delivery (n = 1), stereotactic biopsy and stereotactic radiation therapy , or fractionated radiation therapy (n = 1). All cases additionally received symptomatic therapy , and two received enteral chemotherapy. In three dogs, progressive disease was treated .The time course of diagnosis, therapies, and death in Group A is summarized in Necropsy (six cases) confirmed the antemortem diagnosis of CSF drop metastasis, with (four cases) or without (two cases) recurrence of the primary mass. Case A7 was confirmed based on antemortem biopsies of the primary parietal glioma and, 7 months later, the spinal metastases.This was three dogs with multifocal lesions on the original MRI. Case B1 had seizures  and was euthanized the day after MRI. Case B2  had multifocal MRI lesions. Symptomatic treatment was administered . On day 106, a second MRI revealed additional lesions. On day 135, the dog was euthanized. Case B3  had a single MRI. Treatment was with uPA-targeted oncolytic Newcastle virus (three intravenous injections). Central vestibular deficits developed, and on day 17, the dog was euthanized. In all three dogs, complete necropsy revealed intracranial and spinal glioma.n = 5; low grade n = 1; grade unknown n =1) and three astrocytomas . Metastatic lesions varied histologically  and necropsy (nine dogs). Immunohistochemistry included glial fibrillary acidic protein (five cases) and Olig2 (six cases). The final diagnosis was seven oligodendrogliomas . Finally, in all seven dogs, new, distant lesions were revealed by nine MRIs.All four MRIs performed on the dogs in Group B were reviewed.*-weighted gradient echo  and diffusion-weighted imaging (eight cases) were also performed. Magnetic field strength was 1.5 T, except Case A3 (0.2 T).Multiplanar sequences performed in all cases included T2-weighted (T2W), T2W-fluid attenuation fluid recovery (FLAIR), and T1-weighted . T2In all 10 cases, the primary mass contacted the CSF and was the largest and most rostral lesion. The association between the primary mass and the CSF can be seen in As in Cases A4 and B2 had repeat MRI without surgical excision. In both cases, there was little change in the appearance of the primary mass. However, although the chiasmal mass itself was similar in Case A4, it had disseminated into adjacent leptomeninges (discussed later).The metastatic lesions were classified according to the human system proposed by Bordignon et al. :Type I: Leptomeningeal disseminationType Ia: nodular Type Ib: diffuse Type II: Subependymal dissemination, e.g., ependymal enhancementType III: Satellite tumor: the presence of nodular tumor distant from the primary lesionType IV: Mixed: a combination of the aboveAll metastatic lesions were in contact with CSF in leptomeningeal or ependymal locations: there were no type III satellite tumors. Two dogs had type I lesions only, two dogs had type II lesions only, and six dogs had a type IV mixed combination of lesion types . All abnIn Case A3, the second MRI revealed a single new T2-hyperintense nodule in the contralateral temporal cortex, merging into the SAS on T2W . On bothThe third MRI performed on Case A5 revealed five leptomeningeal nodules, none of which had been observed on earlier MRIs : four ipOn the fourth MRI, Case A7 developed four discrete leptomeningeal nodules, with accompanying diffuse leptomeningeal enhancement (discussed later). These nodules were dorsal to the spinal cord at T9, T12, L1, and L1–L2 .The first MRI in Case B2 had shown diffuse leptomeningeal enhancement (discussed later) in addition to the primary mass. After symptomatic therapy, two nodular metastases were present on the second MRI . IpsilatIn Cases A1, B1, and B2, there was uninterrupted, strong, thick leptomeningeal contrast enhancement that fully encircled the cervical spinal cord, beginning at the caudal medulla oblongata. It was distant from, and entirely dissimilar to, the intra-axial primary cerebral mass of each case.This leptomeningeal enhancement extended ventrally to the level of the midbrain. In Case A1 , it exteThe leptomeningeal thickening in Case B2 , 7 was eAdditionally, there was irregular intramedullary T2, STIR, and HASTE hyperintensity, consistent with syringomyelia or spinal cord edema. It was mainly T1-isointense to GM, but there was also a dilated central canal with no signal on T1W or FLAIR (C1–C2), ependymal enhancement (C3), and T1-hypointense GM (C5–C7). A thoracic T2-sagittal sequence documented further dorsal intramedullary hyperintensity (T4–T12).In case A7 , the spiCase A4 presented a different type of leptomeningeal enhancement. On the second MRI, leptomeningeal contrast enhancement extended directly from the previously diagnosed chiasmal mass . Strong Finally, the SAS surrounding the midbrain of Case B3 contained FLAIR-hyperintense CSF . There wThe sixth MRI of Case A1 revealed five new ventricular lesions. Ipsilateral to the previously excised mass, there was a heterogeneously T2-hyperintense hippocampal mass bulging into the lateral ventricle, with a single line of mild ependymal contrast enhancement . Next weIn Case A2, the cerebellar lingula became asymmetrically T2-hyperintense on MRI #3 . This reThe first MRI of Case A4 had shown periventricular FLAIR-hyperintensity . On the next MRI after radiation therapy, these regions merged into largely symmetrical white matter T2-hyperintensity, and there was progressive lateral ventricle ventriculomegaly.In Case A5  on the third MRI, there was a new linear FLAIR-hyperintensity and contrast enhancement in the ependyma . It was On the second MRI of Case A6, the ependyma of both lateral ventricles was irregularly contrast-enhancing, thickened, and FLAIR-hyperintense , 10. TheArising within this thickened ependyma were at least six ependymal nodules  . Each prConcurrent to the development of thoracolumbar leptomeningeal lesions, Case A7 developed rostral horn lesions . The venIn Case B3, a single MRI revealed subtle signal changes accompanying the primary mass. There was ependymal FLAIR-hyperintensity where the primary mass contacted the lateral and third ventricles . DistantThe following major findings will be discussed. Glioma CSF drop metastases may differ markedly from the primary mass on MRI, with nodules showing more homogeneous signals and less contrast enhancement or entirely different leptomeningeal enhancement. After diagnosing an intra-axial mass, leptomeningeal or ependymal lesions may arise, with combinations of lesion types (type IV) being common. Although metastases can be clinically devastating, some are comparatively subtle on imaging. Metastatic glioma lesions may become increasingly recognized after aggressive treatment of the primary tumor, but they are also seen at first presentation.The metastatic lesions in this study were markedly dissimilar to the primary mass on MRI. The location was different, with intra-axial masses spreading to ependymal (intraventricular or periventricular) or leptomeningeal  sites. Diffuse meningeal enhancement reminiscent of meningitis was a common finding. The nodular metastases displayed very different signals to the primary mass. Heterogeneous contrast-enhancing gliomas caused homogeneous non-enhancing nodules. Examples of metastases deviating from the primary mass in key imaging criteria include an enhancing primary mass with a non-enhancing leptomeningeal nodule , a primaMost patients had multiple metastatic lesions; some developed yet more metastases over time. Patient follow-up (up to 2 years and up to six MRIs per patient) was valuable in chronicling the evolving disease process. Each of type Ia, type Ib, and type II lesions were observed in multiple dogs. Type IV, a mixed combination of these lesion types, was overall most common.These CSF drop metastases developed in varying periventricular and leptomeningeal locations without any strong predilections. However, with one exception, metastases did occur in accordance with the direction of CSF flow. Primary tumors were within or bulging into the lateral ventricle, and metastatic lesions were within the lateral ventricles, in more caudal components of the ventricular system, or in the subarachnoid space. Rostral horn lesions were either intraventricular  or periventricular . The exception was the optic chiasm mass with direct leptomeningeal extension in Case A4. This mass invaded into the third ventricle, with periventricular infiltrates in the third and lateral ventricles on MRI and necropsy. Obstructive hydrocephalus and reduced CSF flow may have allowed retrograde CSF drop metastasis. In 40% of our cases, cervical and even thoracolumbar spinal metastases were seen. It is imperative to note that in cases presenting for intracranial disease, spinal imaging was necessary to accurately document the extent of the neoplasm. Recent work has shown the substantial upward movement of CSF during forced inspiration . This stvia the CSF vs. migration under the ependyma (left) contrasting with dense infiltration of neoplastic cells under the ependyma and no neoplasm evident within the ventricle (right).Another key finding is that a few of the metastatic lesions were subtle. T2W-FLAIR sequences proved useful, displaying easily apparent lesions when T1 and T2 signals were close to normal, and contrast-enhancement was lacking. The glioblastoma (Case B3) with unilateral forebrain deficits had the least obvious MRI findings of metastasis. In the rostral horns  and the ependyma , 15, 16.It is worth noting that in most treated dogs (Group A), therapy was very much restricted to the primary mass, such as excisional surgery, SRT, or CED. In glioma surgery, opening of the ventricle is common, contributing to the risk of CSF drop metastasis . Only twvia CSF and might occur via white matter) are a common form of intra-cerebral dissemination   .The inclusion criteria were MRI of a primary intra-axial lesion combined with lesions suggestive of CSF drop metastasis and histological confirmation of glioma. It was required that anatomically distinct lesions were histologically assessed . Medical records at the Purdue, Auburn, and Virginia–Maryland Colleges of Veterinary Medicine were searched for dogs meeting the inclusion criteria. Information retrieved from the medical record included signalment, neurolocalization, therapy received, the results of all surgical biopsy, necropsy, and histopathological examinations and the dates of all MRIs, therapies, and deaths. Glioma type (astrocytoma or oligodendroglioma) and grade (low or high) were classified according to recently revised canine-specific criteria .updated Response Assessment in Neuro-Oncology (MRI was reviewed. The MRI equipment, sequence, and imaging protocol used in each case are available in Oncology  as recomOncology : completOncology , 10.The original contributions presented in the study are included in the article/Ethical review and approval was not required for the animal study because this was a retrospective study performed on information available in medical records. No animal procedures were performed. Written informed consent for participation was not obtained from the owners because no procedures were performed for this retrospective study. However, owners did provide signed consent for information about their animals to be used in research, at the time of veterinary care.RB and JR designed the study. RB, AY, AC-G, and JR provided case materials. RB, AY, and JR collected the data and drafted the manuscript. MM provided the histology figure and legend. HH edited the MRI figures and legends. All authors edited the manuscript. All authors approved the final manuscript.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.


Pancreatic schwannoma (PS) is an extremely rare benign tumor. Here we describe the Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) results of PS in a 59 years old woman, as well as a review of the literature.A 59-year-old woman consulted for atypical epigastralgia without fatigue, weight loss or fever. CT scan and MRI showed a 35 mm inhomogeneous lesion with well-defined margins located in the pancreas head. The diagnosis of pancreatic tumor was made. The pathologic examination of the biopsied mass yielded a diagnosis of pancreatic schwannoma.On CT scans, almost all benign PS are well-defined cystic or low-density masses. MRI is helpful in characterizing their typical encapsulation.The detection of pancreatic schwannoma is extremely rare. Although multiple imaging modalities are currently available, it is challenging to make an accurate diagnosis before operation. •Although multiple imaging modalities are currently available, it is challenging to make an accurate diagnosis before operation.•Pancreatic schwannomas of Antoni type A show solid masses with inhomogeneous enhancement. Those of Antoni type B show homogeneous cystic or multiseptated mass.•Schwannoma must be discussed as one possibility in the list of differential of pancreatic neoplasms. Althoug2A 59 years old woman, married with no previous pathological history and without surgical history or toxic habits, consults for atypical epigastralgia that is not accompanied by fever, fatigue or weight loss. She denied having ever had pancreatitis. The physical examination was completely normal. CA19-9 tumor serum markers and carcinoembryonic antigen were both negative. An abdominal ultrasound revealed a tiny rounded lesion with low uniform echo at the pancreas's head . The abdAfter an echoendoscopy biopsy yielded inconclusive results, a surgical open biopsy under general anesthesia was performed. The pancreatic mass's histopathology revealed spindle cells organized in a fascicular manner. The cells have elongated nuclei sometimes with an angular aspect. A pathologic testing showed that the patient had pancreatic schwannoma. In addition, immunohistochemical staining showed that for S-100, tumor cells were positive. Given the surgical risk (intimate contact with the superior mesenteric artery) and the patient's non-consent for the surgery, a decision of close monitoring was decided. At the 4-month follow-up visit, the patient was doing well.3Schwannoma, also known as neurilemmoma, is a benign tumor that develops from schwann cells found in the sheaths of cranial, spinal nerve roots, and peripheral nerves. Pancreatic schwannomas are rare neoplasms that arise from the vagus nerve branches that run through the pancreas . PS is mClassical PS tend to be divided into two distinct areas under the microscope: Antoni A and Antoni B. A hypercellular zone of closely packed, long bipolar cells (spindle cells) organized in interlacing and palisading styles characterizes the AntoniA area. There are also verocaybodies lacking mitotic figures. On the other hand, loose hypocellular areas with degenerative alterations such as hemorrhage, calcification, hyalinization, xanthoma infiltration, and cyst development characterize the Antoni B area .Histopathological examination is mostly used to determine the type of tumor. The presence of positive proteins S100, Vimentin, and CD56 in the immunohistochemistry study confirms the presence of Schwannoma. CD57 and GFAP are occasionally expressed in schwannomas, whereas cytokeratin AE1/3, CD34, c-kit, desmin, and myosin are not [The preoperative diagnosis of PS is difficult due to its rarity and non specific imaging appearance. Appearance of solid and cystic areas in these tumors depends on the proportion of Antoni A and B areas .Diagnosis of asymptomatic PS patients is difficult and Computed tomography scan is helpful to deliniate the anatomic relationship between the tumor and surrounding tissues. Well-defined round masses with numerous, low-attenuation cystic necrotic areas are the most common CT appearance of benign PS. Antoni type A patients have hypodense, solid masses with inhomogeneous or rarely multiseptated enhancement. Antoni type B are homogeneous cystic or multiseptated masses. Moreover, a previous research discovered that intravascular thrombosis occurs in Antoni B areas, causing necrosis and the development of cysts . PreviouWell-defined round masses with numerous, low-attenuation cystic necrotic areas are the most common CT findings of benign pancreatic schwannomas. Malignancy is indicated by rapid development, invasion of surrounding tissues, strong contrast enhancement of a solid inhomogeneous mass with irregular contours, and vascular thrombosis .Our patient's CT scan revealed a discretely and inhomogeneously increased mass that corresponded exactly to the scannographic appearance of PS Antoni type A reported in the literature.On MRI, a typical schwannoma appears encapsulated, hypointense in T1-weighted images and appears inhomogeneously hyperintense in T2-weighted images . MagnetiIn our case, MRI was performed to better analyze the pancreatic lesion and the pancreatic ducts. It revealed an encapsuled mass, hypointense in T1 and hyperintene heterogeneous in T2 with no calcification or dilation of the ducts of the pancreas.In the cystic neoplasms or pseudocysts differential diagnosis, cystic pancreatic schwannomas must be considered, but islet cell tumors, carcinoma, or other rare benign tumors like papillary and solid neoplasms are considered for differentiation from solid PS. Histopathologic examination is used to get the exact and final diagnosis.Location and locoregional involvement guide PS management. For benign schwannomas, simple enucleation is the best treatment option. To accomplish R0 resection in tumors with malignant characteristics, pancreatoduodenectomy or distal pancreatectomy with/without splenectomy may be required. Intraoperative frozen sections may be useful in determining whether a schwannoma is benign or malignant, hence avoiding extensive surgical resection and accompanying morbidity .4Dispete its rarity, schwannoma must be discussed as one possibility in the list of differential of pancreatic neoplasms. The imaging finding plays a critical role in the accurate diagnosis of this diseases. The rapid evolution, vascular encasement, or visceral invasion should elicit suspicion of malignant transformation.The ethical committee approval was not required given the article type (case report).None.Aichouni Narjisse: study concept, data analysis; writing review & editing. Widad Abbou: Study conception, data analysis. Nasri Siham: contributor. Wafaa Khannoussi: contributor. My Zahi Ismaili: contributor. Tijjani El Harroudi:. Contributor. Amal Bennani: contributor. Imane Kamaoui: contributor. Imane Skiker: Supervision and data validation.As this manuscript was a case report with no new medical device nor surgical techniques, not prior registration is required.Narjisse Aichouni.Not commissioned, externally peer-reviewed.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.The authors state that they have no conflicts of interest for this report.


Lipomatous neoplasms are a rare entity in the pediatric population, comprising less than 10% of soft tissue tumors in the first two decades of life. Some characteristics of pediatric adipocytic tumors are analogous to their adult counterparts, some pediatric lipomatous lesions however harbor unique features. In recent years, there have been significant advances in the understanding of the pathogenesis and hence in the classification and treatment of pediatric adipocytic tumors. This literature-based article will provide a review of the presently known clinicopathological, immunohistochemical and molecular features of pediatric lipomatous lesions. Although adipocytic neoplasms are the most common soft tissue tumors in adults, they are relatively rare in the pediatric population. Lipomatous tumors comprise less than 10% of soft tissue tumors in the first two decades of life [Lipoblastoma is a benign neoplasm composed of embryonal white fat, occurring predominantly in infancy and early childhood, and rarely in adulthood. The vast majority of cases 75% to 90%) are seen before the age of three years, with a slightly higher incidence in males % to 90% ,9,10,11.Lipoblastoma can compress adjacent structures and interfere with function, particularly in large abdominal tumors, mediastinal tumors, or cervical tumors ,23,24,25T1-weighted magnetic-resonance images reveal a nodular mass with intensity similar to (or lower than) that of lipoma or subcutaneous fat ,30,31. TMicroscopically, lipoblastoma consists of lobulated sheets of adipocytes with a spectrum of maturation, ranging from primitive stellate or spindled mesenchymal cells (myxoid areas), to multivacuolated or small, signet-ring lipoblasts, to mature adipocytes separated by fibrovascular septae see  3,4,7,3,33,34. MPLAG1 rearrangement ranging from 52–60%, thus illustrating the limits of immunohistochemical staining for PLAG1 [PLAG1 fusions are commonly cryptic and that commercially available targeted RNA sequencing panels may not cover newly emerged fusion partners, whereas PLAG1 staining would likely be positive regardless of the PLAG1 fusion partner [The adipocytes show immunoreactivity for S100, CD56 and CD34 ,37. Primor PLAG1 . On the  partner .PLAG1 , COL1A2 (7q21.3), RAD51B (14q24.1), COL3A1 (2q32.2), RAB2A (8q12.1–q12.2), BOC (3q13.2), DDX6, KLF10, KANSL1L, ZEB2 and EF1A1 [HMGA2 alterations are less common [In approximately 60% of the cases a simple, pseudodiploid or hyperdiploid karyotype can be found, featuring a structural alteration of 8q11–q13, leading to a rearrangement of see also  [4,38,39AG1 gene ,46. PLAGnd EF1A1 ,53,54,55s common .Lipoblastoma has an excellent prognosis after excision, with no risk of metastasis ,12,32,56Lipomatosis is a broad term for an overgrowth of mature adipose tissue in a wide spectrum of clinical contexts. It can occur in hamartomatous arrangements with other mesenchymal tissue types.PTEN, PIK3CA and TSC are part of the PI3K/PTEN/AKT/TSC/mTORC1 pathway, and germline, mosaic or somatic mutations of these genes are responsible for the different lipomatosis entities described below , diffuse lipomatosis (DL), encephalocraniocutaneous lipomatosis (ECCL), PTEN hamartoma of soft tissue (PHOST) and PIK3CA-related overgrowth spectrum (PROS), continued growth and/or recurrence after incomplete resection is common and can lead to disrupted growth, malformation and malfunction, sometimes requiring multiple surgeries in ,58,60. SLipomatosis entities can be divided into pediatric and acquired/adult onset types, each with different entities. Pediatric lipomatosis entities are highlighted below.PIK3CA mutations, suggesting CILF should be included in PROS (see below) [Congenital infiltrating lipomatosis of the face (CILF) presents at birth or in early childhood as a unilateral facial swelling with hyperplasia of the underlying bone. Hyperplasia of teeth, tongue, brain and parotid gland have also been described ,61,62. Te below) .Diffuse lipomatosis (DL) presents as a large, rapidly growing mass mostly involving the trunk or extremities in a segmental fashion. It has been described in association with the tuberous sclerosis complex ,65,66.FGFR1 activating mutations have been implicated in cases [Encephalocraniocutaneous lipomatosis (ECCL) is a sporadic neurocutaneous disorder involving tissue derived from the neural crest with ocular, cutaneous, and central nervous system abnormalities. These aberrations include ocular choristomas, colobomas, aniridia, nevus psiloliparus, lipomatosis, alopecia, intracranial and spinal lipomas, midline pilocytic astrocytoma, dysembryoplastic neuroepithelial tumor, developmental delay, and seizures. These lesions are usually found in the soft tissues of the scalp, paravertebral soft tissues, or extremities ,68,69,70in cases .Michelin tire baby syndrome (MTBS) encompasses a rare familial genodermatosis, characterized by symmetrical excess skin folds that are present at birth and resolve with age. It diffusely involves the skin in a symmetric fashion. Other features, such as facial dysmorphism , cleft palate, genital anomalies, developmental delay, ureterocele, smooth muscle hamartoma and nevus lipomatosis may be associated .PTEN inactivating mutations are central to the development of PTEN hamartoma of soft tissue (PHOST). Many PHOST patients have Cowden syndrome or Bannayan–Riley–Ruvalcaba syndrome [syndrome . Patientsyndrome . Histolosyndrome .PIK3CA mutations are identified in PROS, with a correlation between specific mutations (genotype) and their physical manifestations (phenotype) [PIK3CA-related overgrowth spectrum (PROS) presents as asymmetric and disproportionate overgrowth lesions, with enlargement of the affected region . These lenotype) ,75.Nevus lipomatosis superficialis (NLS) typically presents as one or more soft, non-tender papules, nodules, or pedunculated, exophytic lesions in later childhood or early adulthood . The matPIK3CA mutations, it has been suggested they be included in the PROS group (see above) [Lipomatosis of Nerve (LN) usually presents in later childhood or early adulthood, although lesions can present at birth . The lese above) .Liposarcoma consists of a heterogeneous group of mesenchymal tumors with adipocytic differentiation, showing a variable biological behavior, ranging from locally aggressive to metastasizing .The World Health Organization (WHO) recognizes five histologic subtypes: well-differentiated liposarcoma/atypical lipomatous tumor (WDLPS/ALT), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma (MLPS), pleomorphic liposarcoma (PLPS), and myxoid pleomorphic liposarcoma (MPLPS). MPLPS is the latest entity, recognized by the WHO since 2020. All liposarcomas clinically present as large (over 5 cm in diameter) deep-seated, painless soft tissue masses.Liposarcomas represent less than 2% of all pediatric soft tissue malignancies and nearly 90% of pediatric liposarcomas occur in the second decade of life. There is a female predominance and over 70% are myxoid types ,78,79,80Peng et al. found that the most significant difference between young and adult patients with liposarcoma is the distribution spectrum of the subtype. The most common subtype among adult patients is ALT/WDLPS/DDLPS, comprising 50–60% of all liposarcomas, followed by MLPS (20–30%), PLPS (<5%), and MPLPS (rarely seen). In their study, the incidence of the MLPS subtype was high among young individuals (69.6%). The incidence of the ALT/WDLPS/DDLPS subtypes in their study cohort was only 13.0% and PLPS comprised 4.3% of the cases (the lowest incidence in pediatric patients). Historical series showed similar findings ,79,81,82In their literature review, Baday et al. found that approximately 70% of patients with MLPS and WDLPS had surgical excision as the only treatment. Over 50% of patients with PLPS were treated with surgery, as well as radiotherapy and chemotherapy (most commonly a doxorubicin-based treatment). They concluded that more data on the efficacy of different pharmaceuticals in treating liposarcomas is needed; however, the rarity of these tumors in the pediatric population poses a challenge to obtaining this data .Most patients have an excellent overall prognosis following surgical excision with negative margins as a single-modality treatment. Stanelle et al. found that central location of the primary tumor, high tumor grade, and positive surgical margins are strongly correlated with poor survival in pediatric patients with liposarcoma. They found that the five-year survival for patients with negative surgical margins was 95%, in comparison to a five-year survival rate of 50% in patients with positive surgical margins .Baday et al. observed that prognosis was particularly determined by the histologic subtype. Patients diagnosed with MLPS and WDLPS, receiving minimal systemic therapy, had favorable outcomes: none experienced disease relapse or progression. The two patients with PLPS however, both died after receiving neoadjuvant chemotherapy with subsequent excision of the tumor .The terms “atypical lipomatous tumor” (ALT) and “well-differentiated liposarcoma” (WDLPS) are synonyms for morphologically and genetically identical lesions. They represent a locally aggressive mesenchymal neoplasm, composed either entirely or partly of an adipocytic proliferation demonstrating at least focal nuclear atypia in both adipocytes and stromal cells. The choice of terminology between ALT and WDLPS should be based on the principle of avoiding either inadequate or excessive treatment. There is no potential for metastasis unless dedifferentiation occurs, therefore justifying the term “atypical lipomatous tumor” for lesions arising at anatomical sites where complete surgical resection is curative, so as to avoid over-treatment. For lesions arising in anatomical sites which have shown greater potential for disease progression  the use of the term “well-differentiated liposarcoma” is more appropriate ,83.The extremities, followed by the head and neck, trunk, mediastinum and retroperitoneum are most commonly involved ,84,85. AMDM2 (12q15) being the main driver gene [TSPAN31, CDK4 and FRS2 (12q15)) are frequently co-amplified with MDM2. Amplification of MDM2 and/or CDK4 is almost always present, with the exception of Li-Fraumeni-associated cases, which show a TP53 mutation  is the most frequent subtype. It is composed of mature adipocytes with substantial variation in cell size, as well as cytonuclear atypia in adipocytes and/or stromal spindle cells. MDM2 and CDK4 immunohistochemical expression are typical, though in some cases difficult to evaluate, making fluorescence in situ hybridization (FISH) a valid alternative .The sclerosing subtype is most often seen in cases located in the retroperitoneum or spermatic cord. Scattered, bizarre stromal cells with marked nuclear hyperchromasia are seen, set in an extensive fibrillary collagenous stroma. The fibrous component may overshadow lipogenic areas, making it easy to miss in a small (biopsy) sample .The inflammatory type is the rarest subtype, occurring most often in the retroperitoneum and paratestis . There iMorphologic features of pediatric WDLPS/ALT are identical to those seen in older patients, dividing WDLPS into three subtypes: adipocytic (lipoma-like), sclerosing and inflammatory type ,88:AdipoWDLPS/ALT demonstrates an overall indolent course in young patients ,84. The The most important prognostic factor is anatomical location. ALTs do not recur after complete excision. WDLPSs tend to recur repeatedly. In WDLPS uncontrolled local effects or, less often, systemic spread as a result of dedifferentiation may subsequently lead to death . MDM2 inIn some cases, WDLPS shows progression to a  sarcoma of variable histological grade, hence termed dedifferentiated liposarcoma . PediatrMDM2 and CDK4  areas. The transition between the lipomatous and dedifferentiated areas may sometimes be gradual ,83. The The exact prognosis is not known due to the rarity of DDLPS in children and adolescents. However, the patient diagnosed with DDLPS in the study of Baday et al. died, despite aggressive, multimodal therapy .Myxoid liposarcoma (MLPS) is the most common liposarcoma subtype in pediatric patients. The lesions usually involve the extremities, trunk, head and neck, and abdominal regions ,79,80,81MLPS has an identical macroscopic appearance to that of the respective tumors in adults: lesions are typically larger than 10 cm, circumscribed, multinodular and intramuscularly located see also . Higher Histological features of MLPS in children are also identical to those in adult cases. It presents as a moderately cellular, lobulated tumor with increased peripheral cellularity. There is a mixture of patternless, round to spindle-shaped non-lipogenic cells with variable numbers of small lipoblasts. These are set in a myxoid stroma with arborizing “chicken-wire” vessels. The spindle cells typically lack atypia and significant mitotic activity . High-grFUS-DDIT3 fusion transcript, translated into a chimeric oncoprotein that alters transcription and differentiation  gains and losses, with the most frequent mutations being in see also  [77,105,Although slightly better than in adults, the outcome is poor ,78. PLPSMPLPS is a new entity within pediatric liposarcomas, first described in 2009 by Allagio et al. and genomically seen as a distinct subtype of liposarcoma in the current WHO  ,93,107. MPLPS occurs as a large, non-encapsulated, deep-seated soft tissue lesion with ill-defined margins . It is tHistologically, MPLPS is a high-grade lesion with areas of pleomorphic sarcoma  combined with low-grade myxoid components   77,79,1,179,107.DDIT3-rearrangement, nor does it have an MDM2 amplification, therefore, differing from MLPS and WDLPS/ALT, respectively [Immunohistochemically, MPLPS lacks expression of MDM2 and shows a loss of expression for RB1 . MPLPS dectively ,91.RB1 and a complex chromosomal profile (hyperdiploid/hypotriploid karyotype) with gains and losses of chromosomes with deletions/mutations of TP53, and deletions of KMT2D or NF1  is also known [The molecular features of MPLPS tend to show overlap with PLPS, with inactivation of see also  [33,77,9so known ,110.MPLPS is associated with a poor prognosis, with local recurrences and distant metastases to bone, lung and soft tissue. MPLPS has the highest mortality rate of all pediatric liposarcomas ,91,107.Wide local excision is preferred, with 1.5 cm margins. Adjuvant radiotherapy after surgical excision is advised in cases of microscopically incomplete resection, or if surgical margins are marginal . The rol


Leukemic involvement of the eyes is rare, therefore, treatment relies on previous case reports. The treatment of ocular complications poses additional difficulties, because the eye is considered as a pharmacological “sanctuary” for patients with acute lymphoblastic leukemia (ALL). Therefore, radiotherapy is the main therapeutic choice; however, it might lead to many important side effects. To the best of our knowledge, this is the first case report of a bilateral leukemic optic nerve infiltration that remitted with chemotherapy without adjuvant radiotherapy.A 30-year-old female patient with previous history of remitted ALL presented with a one-week history of floaters in her right eye. Her ophthalmological exam showed remarkable optic disc swelling, in both eyes. She was diagnosed with ALL relapse presenting as a bilateral optic nerve leukemic infiltration. Local radiotherapy was planned for both eyes, however, due to efficient recovery with chemotherapy, it was cancelled. Allogenic bone marrow transplantation was subsequently performed. The patient is being followed up and ALL remitted.Leukemia relapse on central nervous system, despite rare, is a sign of poor prognosis and requires prompt treatment. Its occurrence on ocular tissues is even rarer. It is hypothesized that the blood-brain barrier limits the delivery of chemotherapeutic drugs to the eye and infiltration of the optic nerve by leukemic cells might prejudice the flow of cerebrospinal fluid between the cranial space and the optic disc.The online version contains supplementary material available at 10.1186/s12886-021-02097-w. Leukemic involvement of the eyes and central nervous system (CNS) is becoming more frequent as the patients’ survival rate increases. Despite efficient combined therapy, recurrence of the disease is both a diagnostic and therapeutic challenge, especially when it occurs in unusual sites, as the eye . The treIn August 2020, a 30-year-old female patient presented with a one-week history of floaters in the right eye (OD). She had past medical history of B-cell acute lymphoblastic leukemia with chromosomal translocation t TCF3/PBX1 diagnosed in July 2018. Her treatment began in August 2018, with 6-mercaptopurine, vincristine, methotrexate, prednisone, cytarabine, idarubicin, prophylactic intrathecal methotrexate and dexamethasone. Systemic remission was achieved in May 2020. Before hospital discharge, neurological and ophthalmological exams were normal.Due to her visual complaints, she was referred for an ophthalmological examination. Visual acuity was 20/20 OU, biomicroscopic examination and pupillary reflexes were unremarkable. On ophthalmoscopy, there was peripapillary nerve fiber layer edema with protrusion of a white mass with a “creamy” aspect from the optic disc on both eyes (OU). There were small traces of white “creamy” material on inferior vitreous on OD. Macula was normal OU. Visual field showed enlargement of the blind spot in OD and was normal in the left eye (OS) Fig. . Orbit aBone marrow transplant was programmed after full hyper-CAVD drug combination chemotherapy  and intrathecal methotrexate cycle, which began in August 2020. Local radiotherapy OU was planned but on the third day after starting chemotherapy D3), ophthalmoscopy showed remarkable improvement OU (Fig. , ophthalIn December 2020, the patient was submitted to an allogenic bone marrow transplant. In February 2021 she had no ophthalmic complaints, and her exams were all normal. On ophthalmoscopy, OD showed minor optic disc pallor and OS was normal Fig. D. Acute Leukemia relapse on CNS, despite rare, is a sign of poor prognosis and requires prompt treatment. Its occurrence on ocular tissues is even rarer. Hence, management is mainly based on case reports and pharmacological theories over the chemotherapeutic agents’ mechanisms on the eye. Previous studies reported partial or unsuccessful attempts of systemic and intrathecal chemotherapy for optic nerve involvement . AdjunctIt is hypothesized that the blood-brain limits the delivery of chemotherapeutic drugs to the eye  and infiLeukemia can lead to ophthalmologic complications not only through direct infiltration . Its patPrevious studies mainly reported radiotherapy use for optic nerve infiltration by leukemic cells. As this disease mainly affects children, and also because they have better survival and remission rates when compared to non-infants, most case reports refer to the pediatric population . Within Although the remission and survival rates for the adult population have significantly improved, case reports on ophthalmologic impairments are still rare. And, to our knowledge, in all reported cases, which included chronic and acute leukemia, patients were managed with adjunct radiotherapy , 15, 16.To the best of our knowledge, this is the first case report of a bilateral optic nerve infiltration in ALL that responded to chemotherapy without adjunct radiotherapy. In our patient, intrathecal methotrexate was initiated by the oncology team and due to the fast response local radiotherapy planning was cancelled. The main limitation of this article is that it is a single case report and, therefore, our findings cannot be generalized for further treatment of similar cases. Hence, this case addresses the action of chemotherapeutic drugs on ocular tissues and reinforces the importance of ophthalmologic examination during and after treatment of leukemia. We do not intend to suggest the sole use of systemic chemotherapy for leukemic infiltration of the optic nerve, but rather encourage further studies on chemotherapeutic drugs and possibly avoid unnecessary radiation of the eye.Additional file 1. Bone marrow analysis upon diagnosis. Bone marrow analysis upon diagnosis. CD: cluster of differentiation; TdT: terminal deoxynucleotidyl transferase; PBX1: PBX Homebox 1.


A 53-year-old female presented with defective vision in the left eye of one week duration with a best-corrected visual acuity (BCVA) of 6/9. Fundus examination showed a primary retinal vasoproliferative tumor (RVPT) at the inferotemporal quadrant and a small full-thickness macular hole (MH). The patient underwent cryotherapy for RVPT, followed later by vitrectomy with internal limiting membrane peeling and gas tamponade. The tumor regressed and the MH was closed with BCVA of 6/9. RVPT can present with remote macular complications like epiretinal membrane, cystoid macular edema, macular exudates, and rarely full-thickness MH. Management should be directed towards the tumor as well as the complication. Retinal vasoproliferative tumors (RVPT) are uncommon retinal lesions that may occur in isolation (primary) or in association with another ocular condition (secondary) , 2], , , 5]. Th. Th5]. TA 53-year-old, apparently healthy female presented with complaint of distortion in vision of the left eye of a week’s time. She was diagnosed elsewhere as MH in the left eye and referred for MH surgery. She underwent hysterectomy for menorrhagia two years ago elsewhere; systemic history was otherwise unremarkable. BCVA was 6/6 in the right eye and 6/9 in the left eye, N8. Right eye examination was essentially normal. The left eye had early lens changes with the intraocular pressure of 12 mm Hg. Fundus examination of the left eye  and secondary when they occur secondary to pre-existing ocular conditions like retinitis pigmentosa, familial exudative vitreoretinopathy, Coats disease, intermediate uveitis, retinal vasculitis, previous retinal detachment surgery, etc. . IdiopatClinically, RVPT appears as a yellow-red, often ill-defined retinal mass with a mildly dilated feeding retinal artery and draining vein , 2], , , 5]. Ul. Ul5]. UThe most important differential diagnosis is retinal capillary hemangioblastoma, which manifests with more prominent retinal vessel dilatation and tortuosity , 4], , [There are no clear-cut guidelines in the management of RVPT . The preRVPT can cause vision loss due to remote macular complications despite their peripheral location. This case report underscores the importance of thorough peripheral retinal examination in cases of macular pathology to rule out conditions like RVPT, as well as the need to address both the tumor and the complication in the management plan.The authors declare that they have no competing interests.


Incomplete obliteration of urachus during the fetal period can lead to urachal abnormalities. One of these abnormalities is the urachal sinus that can be asymptomatic, or it can be symptomatic by becoming infected or being malignant, and it can mimic other diseases' symptoms. Although it is rare in adults, it should be considered a significant differential diagnosis in patients with abdominal pain or umbilical discharge. This article presents a young patient with urachal sinus mimicking umbilical pilonidal sinus symptoms. Although umbilical urachal sinus is a rare condition in adults, it should be considered as a significant differential diagnosis in patients with abdominal pain or umbilical discharge due to the development of infection or malignancy. He claimed that he attended a doctor at first and was prescribed clindamycin and ciprofloxacin tablet for ten days with suspicion of infected umbilical pilonidal sinus, but the medications did not have any positive effect, the discharge continued. The past medical history was unrevealing. The patient had a deep umbilicus, dense hairy abdomen, a tuft of hair was seen in his umbilical area, the umbilical area was erythematosus in appearance, and it was tender, but other physical examinations did not include abnormal findings. His vital signs were in the normal range. He was asked to do an upright chest X‐ray and an upright abdominal X‐ray, as shown in Figures 3The urachus is a vestigial of embryonic structures, which remains a fibrous cord between the anterior transversalis fascia and the peritoneum posterior. This fibrous tissue attaches the umbilicus to the bladder.In this case report, a young male with a deep umbilicus, dense hairy abdomen, a tuft of hair which was seen in his umbilical area, and erythematosus umbilical area presents with the complaint of discharge of pus and blood presented to the surgical clinic. All the symptoms could be considered as an umbilical pilonidal sinus.Umbilical pilonidal sinus is one of the types of pilonidal sinus disease, which is defined as a miscellaneous clinical presentation from cysts and sinuses containing hair without any symptoms to large abscess with different symptoms that can happen in various parts of the body.There are two main treatment methods for umbilical pilonidal sinus, including conservative and surgical methods, and choosing the proper method depends on medical team preferences and the patient's condition.So, due to the rare possibility of the urachal sinus in adults, side effects of this disease such as infection or malignancy, and nonspecific clinical findings, it should be considered a differential diagnosis in patients presenting with lower abdominal pain or umbilical discharge.4Urachal sinus is a type of urachal abnormalities caused by incomplete urachal obliteration. This disease is rare in adults, and it is more in males. However, as our study showed this disease can be asymptomatic until it becomes infected, malignant, and subsequently mimic other diseases' symptoms such as appendicitis, urinary tract disease, or umbilical pilonidal sinus. So, because of the misdiagnosis and probability of urachal sinus malignancy or infection, it is crucial to accurately identify and diagnose this disease by further imaging investigations and consider it an important differential diagnosis in patients with abdominal pain or umbilical discharge, even in adults.The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript. The patient has consented to the submission of the case report for submission to the journal.All authors contributed equally to the manuscript and read and approved the final version of the manuscript.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Small bowel volvulus (SBV) represents a rare and life-threatening cause of gastrointestinal obstruction among adults. SBV can be classified as primary and secondary subtypes. Preoperative diagnosis of SBV is a challenge because of the absence of pathognomonic clinical, radiographic and laboratory findings. Surgery represents the correct treatment of SBV.A 69-year-old Caucasian male presented to the Emergency Department with a two-day history of abdominal pain, inability to pass gas or stool, nausea, vomiting. Physical examination revealed abdominal distension, generalized abdominal pain without guarding or rebound tenderness, a partially reducible and painless right inguinal hernia. Laboratory tests reported neutrophilic leukocytosis. Abdominal computed tomography revealed massive gastroduodenal dilatation with pneumoperitoneum and small bowel loops in the right inguinal sac. The patient underwent exploratory laparotomy: a jejunal volvulus (JV) located within the right inguinal hernia sac, causing gastrointestinal obstruction, was devolvulated and a right prosthetic inguinal hernia repair was also performed. The patient was discharged on the 10th postoperative day.Secondary SBV is due to any congenital or acquired lesions and rarely occurs among adults in Western countries. This is the first literature report of a JV located within an inguinal hernia sac causing gastrointestinal obstruction.Secondary JV represents an extremely rare abdominal emergency necessitating early diagnosis to prevent the development of intestinal ischemia, bowel necrosis and peritonitis. Diagnosis of JV needs a high index of suspicion and may be facilitated by imaging, often it is made intraoperatively. Surgery represents the appropriate treatment of JV. •Jejunal volvulus (JV) represents a rare cause of gastrointestinal obstruction among adults.•Diagnosis is a challenge because of the absence of specific clinical presentation, pathognomonic radiographic and laboratory findings.•Abdominal contrast-enhanced computed tomography (CECT) remains the most relevant imaging modality for diagnosis.•Early diagnosis and early surgical intervention are the keys for the successful management of JV. The patient was initially managed with fluids, intravenous broad-spectrum antibiotics and bowel rest. After abdominal radiography showing gastric dilatation with suspected pneumoperitoneum and intestinal loops in the right inguinal hernia sac (A 69-year-old Caucasian male presented to the Emergency Department with a two-day history of abdominal pain, inability to pass gas or stool, nausea, vomiting; vital signs were normal. He referred habit on smoking and alcohol consumption; there was no history of previous abdominal surgery or changes in dietary habits. His past and familial medical histories were normal. The patient was retired from the work, married and of medium socio-economic status. Physical examination revealed abdominal distension, severe and generalized abdominal pain without guarding or rebound tenderness, hypoactive bowel sounds and a partially reducible and painless right inguinal hernia. Laboratory tests reported neutrophilic leukocytosis (WBC 13.700 10rnia sac , the patrnia sac , 3. The rnia sac , duodenurnia sac . There w3This clinical case describes an extremely rare JV causing gastrointestinal obstruction. Volvulus occurs most commonly in the large bowel than in the small bowel and stomach 4JV represents an extremely rare surgical emergency that should be considered in the differential diagnosis of patients with acute gastrointestinal obstruction. Its diagnosis is a challenge because of the absence of pathognomonic clinical, radiographic and laboratory findings and needs a high index of suspicion. Early diagnosis and early surgical intervention are the keys for the successful management of JV.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval has been exempted by our institution because this is a case report and no new studies or new techniques were carried out.Written informed consent was obtained from the patient, for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Giuseppe Evola: Operated on the patient, drafting the manuscript, literature research.Mario Scravaglieri: Operated on the patient, drafting the manuscript.Giovanni Francesco Di Fede: Drafting the manuscript, literature research.Carla Di Stefano: Drafting the manuscript, literature research.Salvatore Sarvà: Drafting the manuscript and literature research.Luigi Piazza: Revising the manuscript.Not applicable.Giuseppe Evola.Not commissioned, externally peer-reviewed.The authors have no conflict of interest to declare.


Chondrosarcomas (CS) are malignant mesenchymal tumours with cartilaginous differentiation that rarely affects the maxillofacial region. It accounts for approximately 10-20% of malignant bone tumours. The CS is rare in occurrence with aggressive course with high malignant potential, and poor prognosis.Here we report a rare case of a 37year old female presenting tumoral mass in the lower jaw, for the past 3 years, which was gradually progressive in nature with an area of skin ulceration. The CT revealed a well- defined lesion with soft tissue component measuring 15x12x10 cm in size infiltrating both right and left masseter. Biopsy confirmed the diagnosis of grade IIICS. Considering the size and aggressive nature of the lesion, surgical resection was done. The reconstruction of the mandible was done with vascularised fibula pedicle flap to achieve acceptable cosmesis. The patient was discharged uneventfully. Though few cases of high grade CS have been reported in literature but CS of this enormous size has not been reported yet. In this case report we documented the management of a relatively rare but challenging reconstructive maxillofacial surgery. Since in this case the CS was enormous in size and aggressive surgery was required, the cosmetic and functional outcomes were challenging. In our case report we have found that a vascularised fibula pedicle flap gives a good functional and cosmetic outcome and can be used for reconstruction of complete mandible. Majority of CS of head and neck arise from maxilla, with very few cases arising from mandible with a high rate of occurence. The World Health Organization (WHO) classified CS “as a malignant tumour with pure hyaline cartilage differentiation characterized by the formation of cartilage scans revealed a large relatively well defined soft tissue density lesion with amorphous, ring and arch like calcifications seen in relation to mandibular bone with erosion and destruction of its body, alveolar margin and bilateral rami. The soft tissue component measured 15x12x10 cm in size. The lesion was involving the right masticator space with the infiltration of the right masseter. Infiltration into the left masticator space was also noted with the involvement of the left masseter muscle. Encroachment and compression of the sublingual space was also seen .Microscopic examination of the incisional biopsy revealed chondrocytes and lacunae in lobular patterns with pleomorphic and hyperchromatic nuclei. Mitosis was seen. A prominent cellular spindle cell proliferation was seen with minimal areas of chondroid. A high grade III CS was confirmed. The general workup of the patient revealed no distant metastasis. The surgical resection was executed based on the size and aggressiveness of lesion. The prognosis and management was explained to the patient and after taking consent, a mandibulectomy with wide margins of the lesion leaving bilateral condyloid processes of the mandible was done . The reconstruction of the mandible was done with free vascularised fibula flap based on peroneal artery and it`s perforators. The arterio-venous anastomosis was done with the facial vessels. Titanium plates with screws were used for giving shape to the fibula as a jaw bone. The patient was tracheostomised at the end of the procedure, successfully extubated and shifted to ICU uneventfully . The pathological specimen sent post operatively, showed chondroid tissue along with myxoid tissue. The chondroid tissue at periphery show presence of sheets of tumour cells separated by thick fibrous collagenous stroma. The cells are ovoid to spindly, hyperchromatic, pleomorphic nuclei with prominent nucleoli in some. Mitotic activity also increased at places along with small areas of necrosis .6.thpost operative day, and was further advised for radiotherapy and chemotherapy. The patient was receiving further treatment when she met with a road accident and died.The bony and soft tissue surgical margins were free of tumour. Immuno-histochemistry revealed strong expression of KI-67, thus confirming the diagnosis of high grade CS. The patient was discharged uneventfully on a tracheostomy tube on 18CS is rare in the head and neck region which accounts for 4.2-6.7% of cases. Most CS of the head and neck region occur in the maxilla; others are found in descending order of frequency in the body of the mandible, ramus, nasal septum and the paranasal sinuses(CS are malignant tumors arising from cartilage cells that tend to maintain their essentially cartilaginous nature throughout their evolution. These neoplasms mostly occur in the long bones, pelvis and ribs . Though  The most common clinical finding is a painless swelling, expansion of the buccal and the lingual plates, premature eruption or exfoliation of teeth. The mass is usually rapidly growing and covered with mucosa which can ulcerate followed by pain at later stages . These fThere are no radiographic findings that are pathognomonic for CS.The radiological pattern of CS is variable. These lesions may appear radiographically as osteolytic, with radiolucent shadows with a wide zone of transition and irregular or indistinct borders. Alternatively, they may demonstrate an ill-defined cloud-like matrix with the calcified “whorls and arcs” typical of a chondroid matrix plus or minus aggressive features such as endosteal scalloping, cortical disruption, periostitis, and or soft tissue mass effect . These lHistopathology is mainstay of diagnosis. Grade 1 CS closely mimic the appearance of a chondroma, composed of a chondroid matrix and chondroblasts that show only a subtle variation from the appearance of normal cartilage. Calcification or ossification of the cartilaginous matrix is often prominent and mitosis being a rare feature. Grade II CSs show a greater proportion of moderately sized nuclei and increased cellularity. The cartilaginous matrix tends to be more myxoid. The mitotic rate is low. Grade III CSs are highly cellular and show spindle cell proliferation with mitosis. Easily recognizable cartilage may be rare with a higher proliferative index -13, thisBecause of the rarity of CS of the jaw there are no established evidence-based treatment protocols. For this reason, many of the current treatment strategies for this disease have been extrapolated from protocols developed and tested for CS of other more typical locations outside the head and neck region or other types of sarcomas. The most effective therapeutic modality is wide surgical excision . Wide loThe main prognostic factors are surgical resection, stage, grade and primary site. The mesenchymal and dedifferentiated forms are known to have poor prognosis. The prognosis is poor because tumours have a tendency for early recurrence either locally or as metastasis. Metastasis is hematogenous and the most common site is the lung . The proFive-year survival is about 90% for grade 1 CS and around 50% for combined grade II and III CS. Occasionally, CS show the coexistence of various histological grades in the same tumour . In a stThough cases of grade III CS have been reported in literature, but CS of this enormous size and aggressiveness has not been reported yet. Because of the rarity of CS of the jaw there are no established evidence-based treatment protocols. The most effective therapeutic modality is wide surgical excision. We recommend radical approach for resection of such giant and aggressive lesions and ideal reconstructive methods which provide both aesthetic and functional results as we did in this case by a free vascularised fibula flap, due to the ease to tailor the flap precisely to the defect which provided not only reconstruction of the shape but a functional mandible.