Desmoplastic fibroma (DF) is a benign, locally aggressive neoplasm that rarely occurs in the facial skeleton. It usually presents during the first three decades of life. Due to its aggressiveness and high recurrence rate, early diagnosis is imperative, and complete surgical removal of the lesion is the treatment of choice. Herein, we present three cases of DF namely a 2 year-old girl with a mandibular DF, a 9 year-old boy with a maxillary lesion and a 1.5-year old boy with a mandibular DF. Complete clinicopathological information, treatment plan and long-term follow-up of patients are discussed. Histopathologic features of 3 cases revealed non-capsulated spindle cell tumor with fascicular or swirling patterns in incisional biopsy. Immunohistochemical staining was performed to make a definitive diagnosis. Strongly positive nuclear immunoreactivity for β-catenin confirmed the diagnosis of desmoplastic fibroma in 3 cases. Segmental mandibulectomy, partial maxillectomy and hemimandibulectomy were done for the cases. There was no recurrence in our reported cases after 8 and 11 months and 3 years follow up, respectively. It is noteworthy that despite the aggressive nature of DF, young patients often respond well to wide resection treatment.  Desmoplastic fibroma (DF) is a rare, benign, and locally aggressive, but non metastasizing, tumor with connective tissue origin ,2,3. SimHerein, we report three cases of DF of the jaw and describe their surgical management and long-term follow-up results. A 2-year-old girl with an expansive mandibular lesion, a 9-year-old boy with a maxillary lesion and a 1.5-year old boy with a mandibular swelling with incisional biopsy reports of DF are presented. All parents of the patients signed informed consent forms.Case 1  scan of the lesion revealed a lytic lesion extending from the right angle of the mandible to the left lateral incisor, causing the expansion of both buccal and lingual cortical plates ., a segmental mandibulectomy through an extra-oral access with 1 cm safety margin was ultimately performed. Reconstruction of the mandible was performed using a 2.4-mm mandibular reconstruction plate  to restore the mandibular contour.At the 8-month follow-up, there was no evidence of recurrence and it seemed that spontaneous regeneration of the anterior border of ramus had begun .Case 2 A 9 -year-old boy complaining of an acute swelling on the right side of his maxilla was referred to the Maxillofacial Surgery Department of Tehran University of Medical Sciences. Clinical examination revealed a firm expansion since 5 months earlier. The symptoms of pain, tenderness and numbness were not significant. The patient’s past medical history was unremarkable and he had no history of allergy. Magnetic resonance imaging (MRI) in coronal view revealed a large mass measuring 75 × 5 × 45 mm, involving the right maxilla and extending to the infraorbital area. The medial extension of the lesion had caused airway displacement and asymmetry as the result of changes in facial plane . The initial incisional biopsy revealed a tumoral mass composed of bland spindle cells proliferated in a fibrotic to myxoid matrix with a pattern-less arrangement . IHC staining revealed positive immunoreactivity for CD 31 and SMA only in the vascular structure and CD 34 in some tumoral cells. B-catenin was strongly and diffusely positive in tumoral cells. Desmin and S-100 were negative in tumoral cells. IHC findings and histomorphometric assessments were compatible with juvenile DF. Two weeks later, the patient underwent a surgical procedure via the Weber Ferguson approach. This approach is indicated for accessing the tumors involving the maxilla and extending superiorly to the infraorbital nerve and/or involving the orbit. It provides a wide access to all areas of the maxilla and orbital floor .At the 11-month follow-up, the clinical and radiographic examinations showed no evidence of recurrence with acceptable esthetic and functional results .Case 3A 1.5-year-old boy with a swelling in the right side of his mandible was referred to an oral and maxillofacial surgeon. His parents reported a visit to a dentist and history of taking antibiotics due to a diagnosis of an odontogenic infection. The incisional biopsy of the unhealed lesion was compatible with benign spindle cell lesion. Extraoral examination revealed facial asymmetry due to the right mandibular swelling. There was no lymphadenopathy, paresthesia or pus discharge. His past medical history was unremarkable and the patient had no history of allergy. The surgeon requested to recheck the paraffin blocks to confirm the diagnosis prior to treatment planning. Histological examination during rechecking of the slides revealed tumoral tissue composed of proliferated spindle cells with ill-defined borders arranged in fascicular and swirling patterns in a non-collagenized stroma compatible with DF.Axial CT scan revealed a radiolucent, well-defined, expansile lesion in the right posterior mandible extending to the ramus area and perforating the buccal and lingual cortices. The lesion had extended to the hyoid bone in the medial aspect and had displaced the facial plane. The internal structure of the lesion appears to remnant bone. The lesion had not displaced the teeth . Two weeks after the biopsy and definite diagnosis of intraosseous fibromatosis (DF), the patient underwent right side hemimandibulectomy from the distal of the lateral incisor tooth to the ascending ramus of the mandible and coronoid process via an extraoral submandibular incision. The size of soft tissue mass was 6.5 x 5 x 3.5 cm .At the 3-year follow-up, there was no evidence of recurrence with acceptable esthetic and functional results. DF of bone is comparable with soft tissue fibromatosis or intraosseous counterpart of soft tissue fibromatosis . The diaet al. reported a slight predilection for females in the jaw tumors   with equal sex distribution. However, Said According to the World Health Organization (WHO), DF is a benign tumor with low to variable cellularity; these cells can have ovoid to elongated nuclei with no polymorphism, atypia or mitotic activity .The pathognomonic histological features of DF include the presence of mature fibrous connective tissue and spindle-shaped fibroblasts separated by abundant collagen fibers . BecauseThe histological features of fibrous dysplasia include hypocellular fibrous connective tissue with irregular shaped woven bone, giving it a ginger root appearance. Fibrous dysplasia can mimic DF especially in areas of dominant fibrous tissue with no osseous material. However, in fibrous dysplasia, the fibrous tissue is more hyper-cellular and vascular than DF.et al., S-100 is negative in 93% of DF lesions, and 63% of lesions are negative for MSA or the HHF-35 (MSA antibody). Also, 92% positive immunoreactivity is seen for vimentin, 50% for B-catenin and 77% for SMA. Moreover, 100% of the lesions display less than 5% Ki-67 labeling, indicating a very low proliferation index (7). SMA and B-catenin were positive and S-100 was negative in one of our patients (case 2). Histopathological features of fibrosarcoma consist of low to moderate cellular proliferation of spindle cells, sometime without the classic feature of Herring bone. In contrast, there is no evidence of atypia, pleomorphism or mitotic figures in DF, which are the characteristic features of malignancy ,7. AlthoThe radiographic features of DF are nonspecific. It can be unilocular or multilocular with well-defined or ill-defined borders or irregular radiolucency. It usually has a lobulated appearance that resembles soap bubbles, and thus, it may mimic some other lesions of the jaws such as ameloblastoma, myxoma, aneurismal bone cyst, central hemangioma and eosinophilic granuloma. However, presence of coarse and irregular septa can help in its correct diagnosis ,5. When the lesion damages the cortex, CT scan is recommended and if the tumor has extra-osseous growth, MRI is required to determine the exact extent of soft tissue invasion by the lesion ,9. On T1-weighted MRI, the internal structure has a low signal which helps in determining the intraosseous extent because of the contrast with the high signal from the bone marrow . Several treatment modalities have been suggested for management of DF due to the aggressive nature of this benign lesion. However, because of its aggressive behavior and high recurrence rate, complete surgical resection of the lesion with safety margins is recommended. Curettage is usually sufficient for small mandibular lesions, but long-term follow-ups are necessary. The recurrence rate is about 40% to 47% in lesions treated by curettage or intralesional resection. Radiotherapy may serve as an alternative treatment when the lesion is inoperable but it is avoided in children because of postoperative complications ,3,10,11.There was no evidence of recurrence of the lesion in our first and second cases after 6 and 9 months of follow-up, respectively. Review of the Literature:To do a comprehensive literature review, a systematic search was conducted in the PubMed database, which revealed that only 96 cases of DF of the jaw have been reported from 1968 to 2017.et al. reviewed the reported DF cases from 1965 to 2002. Thus, we decided to discuss the cases reported from 2000 until now . Thus, all patients were followed and there was no evidence of recurrence. DF of the jaw is a rare, slow-growing and well-differentiated fibrous tumor with an aggressive potential for growth and recurrence. We reported three cases of DF of the jaws with no evidence of recurrence after several months. A wide local resection can be the best treatment option to minimize the recurrence rate of this benign aggressive tumor.


Acute idiopathic maculopathy (AIM) is a condition reported to occur in young adults, which is classically characterized by the development of a unilateral serous macular detachment that commonly follows a flu-like illness. A 27-year-old female patient complained of diminution of vision in her left eye, of acute onset and progressive course for 2 weeks. She had a history of a flu-like condition 2 weeks prior. Following clinical examination, multimodal imaging analysis, and laboratory investigations, she was diagnosed with unilateral AIM, associated with atypical findings that were not previously reported to occur with AIM, including cystoid macular edema, flame shaped retinal hemorrhages, extensive hard exudates, and juxta-foveal yellowish intraretinal lesions. This may have been due to the more severe inflammation and concomitant phlebitis that was associated with this case compared to previously reported AIM cases. It is suspected to follow infection by Coxsackievirus in some reported cases [2].Acute idiopathic maculopathy is a condition reported to occur in young adults and is classically characterized by the development of a unilateral serous macular detachment that commonly follows a flu-like illness [1].The condition was initially described as a unilateral neurosensory detachment associated with thickening of the underlying layer of retinal pigment epithelium, with vitreous cells and intraretinal hemorrhages occurring in some patients. However, these manifestations usually spontaneously disappear with no recurrence. Fluorescein angiography typically shows early hypofluorescent and hyperfluorescent areas with late increase in hyperfluorescence and associated dye pooling . Later, new features associated with acute idiopathic maculopathy were reported, including papillitis with prominent veins and mild phlebitis, eccentricity of lesions, the association with pregnancy, peculiar subretinal exudation, and bilateral affection , characterized by cystoid edema of the macula, subretinal fluid, flame-shaped hemorrhages, and subretinal exudation. This may have been due to a more severe inflammation caused by a different organism or an exaggerated host immune response.This case represented a relatively more severe form of acute idiopathic maculopathy than previously reported cases . The associated phlebitis may have resulted in a picture similar to an occlusion of a retinal vein branch characterized by flame shaped hemorrhages in the retina with more severe leakage leading to the cystoid edema of the macula and subretinal exudation. The peculiar intraretinal material that was present nasally and temporally to the fovea was thought to represent the accumulation of inflammatory cells or debris as previously reported [3]. This material has also been previously reported to present as a pseudohypopyon or vitelliform material, which supports an inflammatory etiology for the condition .The presence of more severe manifestations in the reported case may have been due to the occurrence of more severe phlebitis than has previously been reported in other cases of acute idiopathic maculopathy [7].In conclusion, a unilateral acute idiopathic maculopathy case associated with more severe features than previously reported, including cystoid edema of the macula, flame shaped hemorrhages in the retina, and subretinal exudation, was reported. Depicting the unusual and rare features associated with a condition is necessary to help avoid misdiagnosis and unnecessary treatment as has been previously reported in cases of acute idiopathic maculopathy [Consent for publicationThe patient signed a consent form for publication of her images.Conflict of interestsThe author declared that she has no competing interests.FundingNo sources of funding were available.CARE guidelinesThis study adheres to CARE guidelines for reporting case reports.AcknowledgementsNone.Ethical approvalThis report was approved by Cairo University research ethics committee and followed the tenets of the Declaration of Helsinki.


A 4-month-old cat with epileptic seizures and nasal discharge was presented, and diagnosed with intranasal meningoencephalocele based on the clinical symptoms and findings of CT and MRI. As liquorrhea was suspected, the meningoencephalocele was surgically excised and the postoperative course was favorable. For cribriform plate reconstruction, the autologous tunica vaginalis was used. Rhinorrhea of the cerebrospinal fluid and seizures disappeared after surgery. On CT and MRI at 7 months after surgery, septum formation between the cranial and nasal cavities was confirmed. Currently, no seizures have occurred even though no oral antiepileptic agent was administered. There are few reports of surgical treatment for meningoencephalitis, and there are no reports of reconstruction of the cribriform plate using tunica vaginalis, so we reported the details. Meningoencephalocele is a congenital disease in which a part of the cranial content extracranially prolapses through a skull defect. Prolapse of the meninges alone is termed meningocele, and prolapse of the meninges and cerebral parenchyma is termed meningoencephalocele . In humaIn the small animal field, only a few reports on meningoencephalocele are available, and limiting to surgically treated cases, only a dog with intranasal meningoencephalocele  and a caA 4-month-old, non-castrated male Japanese cat weighing 3.0 kg was presented. Focal seizures causing facial muscle spasms and salivation as the main symptoms appeared from 4 months of age, and the cat was brought to our hospital. This symptom was seen about once every 2 weeks, and there were no signs of pre- or post-ictal sign. No loss of consciousness was noted during the seizure, and the seizure subsided in <1 min. The first examination day was designated as the first hospital day. Serous nasal discharge was noted on physical examination, but no change was observed in outward appearance. No abnormality was detected on the blood test, blood chemistry, thoracoabdominal radiography, or abdominal ultrasonography. Blood chemistry test was performed including those that could cause seizures . Excluding seizures as a neurological manifestation, no abnormality was noted on the neurological examination. Gabapentin at 8.3 mg/kg, BID, was administered (BID was adopted because TID administration caused mild ataxia) and the symptoms improved. Tonic-clonic seizures that last 2–3 min developed daily from the 27th hospital day and seizure symptoms progressed, for which oral administration of phenobarbital at 2 mg/kg, BID, was initiated. Based on the case signalment and progressive clinical course, differential diagnosis included a congenital disease, inflammatory disease, and degenerative disease. On the 36th hospital day, MRI and CT were performed to closely examine the intracranial disease.On CT , a defect of the left cribriform plate and rightward deviation of the nasal septum were observed, and an iso-dense mass lesion with cerebral parenchyma occupied the nasal cavity. This mass lesion was continuous with the cerebral parenchyma . On MRI The mass lesion was diagnosed as intranasal meningoencephalocele because the animal was young and developed progressive seizure symptoms, and prolapse of the cerebral parenchyma into the nasal cavity was suggested by CT and MR imaging.In the present case, concomitant development of bacterial meningoencephalitis was expected because the left cribriform plate was defective, suggesting that the cerebral parenchyma prolapsed into the nasal cavity, thereby causing liquorrhea as it was constantly exposed to the outside environment. In order to control the seizures, surgical resection of the encephalocele and reconstruction of the defective cribriform plate were recommended. Cephalexin at 20 mg/kg, BID, and enrofloxacin at 5 mg/kg, SID, were administered perioperatively and 1 week after surgery. Anesthesia was induced with propofol  and maintained by isoflurane  inhalation after tracheal intubation. During surgery, pain was managed with remifentanil , and blood pressure was managed with ephedrine  and dopamine . After the anesthesia was stabilized, the cat was placed in the prone position and disinfected. The cat was castrated before brain surgery, and the autologous tunica vaginalis was collected. After the scalp incision, craniotomy was carried out via the transfrontal sinus approach using a sagittal saw . Bone tissue below the frontal bone was removed as much as possible using an ultrasonic aspirator , and the lesion was approached. The right cribriform plate was confirmed, but the left cribriform plate was absent and a mass considered to be the cerebral parenchyma prolapsed from this region. The prolapsed mass was entirely yellow in color compared with normal brain tissue . In addiFor cribriform plate reconstruction, the autologous tunica vaginalis and a liquid tissue adhesive, fibrin glue , were used. The tunica vaginalis excised before brain surgery was arranged to cover the defective region of the cribriform plate over the stump of the mass, and the liquid tissue adhesive was applied to the surrounding tissue . Using aThe excised specimen was immediately fixed with formalin and histopathologically examined . In HE-sAfter surgery, no seizures occurred and serous nasal discharge was resolved. Abnormality in the sense of smell was of concern because the olfactory bulb was resected, but no abnormality was observed and the cat had a good appetite. No paralysis or gait disturbance was exhibited. Neurological tests performed 1 week, 1, 3, and 7 months after surgery showed no abnormalities in consciousness level, gait, behavior, cranial nerve test, and postural response.Seven months after surgery, the formation of the septum dividing the nasal cavity and brain were confirmed on MRI and CT . In addiMeningoencephalocele is a congenital disease in which the cranial content extracranially prolapses through a skull defect. No factor associated with its development has been clarified, but developmental abnormality of the neuroectoderm during pregnancy has been suggested .It is diagnosed based on the combination of clinical signs, such as liquorrhea and neurological manifestations accompanying meningitis, and imaging findings . CT is uJeffery reported that hemorrhage, malacia, degeneration of the white matter, and inflammatory cell infiltration were noted on pathological examination , similarIn general, surgery is the main treatment method for meningoencephalocele in humans . There aThe original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Written informed consent was obtained from the owners for the participation of their animals in this study.MY, KN, GY, AK, and HK helped with the diagnosis of this case and participated in clinical case management. HK participated in the review and editing the manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Epidermoid cyst arising from the cecum is extremely rare. Single-incision laparoscopic surgery is the latest innovation in minimally invasive surgery, and shortens incisions, improves cosmesis, and reduces postoperative pain. We report here the first description of a patient with epidermoid cyst of the cecum treated by ileocecal resection by single-incision laparoscopic surgery.A 20-year-old woman presented to our hospital with abdominal pain in the right lower quadrant. Abdominal contrast-enhanced computed tomography showed a 56 × 35-mm cystic mass in the ileocecal area. Magnetic resonance imaging revealed a 56 × 43-mm, T1-hypointense, T2-hyperintense mass attached to the cecum. Gastrointestinal tumor or duplication cyst was suspected, and ileocecal resection was performed using single-incision laparoscopic surgery. Intraoperative examination showed the tumor as a round, whitish mass arising from the cecum. Operation time was 162 min, and intraoperative blood loss was 10 ml. Macroscopic examination showed a 56 × 45-mm elastic-hard, whitish, round mass arising from the cecal wall. Microscopic examination revealed the cyst wall lined by keratinized stratified squamous epithelium. No malignant findings were identified. The final diagnosis was epidermoid cyst of the cecum. The postoperative course was uneventful and she was discharged on postoperative day 5.A rare case of cecal epidermoid cyst is reported. Single-incision laparoscopic colectomy using an organ retractor represents a promising option for treating cecal epidermoid cyst. Epidermoid cysts are generally considered to represent sequestered cystic malformations, and can develop in various parts of the body . EpidermLaparoscopic surgery has been the standard approach for colorectal surgery, with the benefit of better postoperative outcomes compared to an open approach . Single-This report offers the first description of a patient with epidermoid cyst of the cecum completely resected by ileocecal resection as an SILS.A 20-year-old woman with abdominal pain in the right lower quadrant presented to hospital. Vital signs were stable. Physical examination revealed tenderness of the right lower abdomen, but she showed no muscle defense or rebound tenderness. Laboratory data showed no inflammation or anemia. Abdominal contrast-enhanced CT showed a 56 × 35-mm cystic mass with no enhancement in the ileocecal area Fig. . MRI revA 4-cm zig-zag incision was placed in the umbilicus. An EZ access  was then inserted, and two 5-mm ports and one 12-mm port were placed . The tumor was seen as a round, whitish mass arising from the cecum Fig. . MobilizMacroscopic examination showed a 56 × 45-mm elastic-hard, whitish, round mass arising from the cecal wall (Fig. Microscopic examination revealed the cyst wall was lined with keratinized stratified squamous epithelium. No malignant findings were apparent Fig. .Fig. 5HiThe final diagnosis was epidermoid cyst of the cecum. The postoperative course was uneventful and the patient was discharged on postoperative day 5. As of 6 months postoperatively, no evidence of recurrence has been identified.The present case report describes a rare case of epidermoid cyst of the cecum treated by SILS ileocecal resection.Cecal epidermoid cyst is extremely rare, with only 9 other cases reported in the English literature . EpidermPreoperative diagnosis of cecal epidermoid cyst is difficult, and no previous reports have described reach accurate preoperative diagnosis. On CT, epidermoid cyst appears as a well-demarcated, low-density mass with enhancement of the capsule following contrast administration . On MRI,Epidermoid cyst needs to be treated by complete removal of the tumor. One reason is that residual tissue and cyst wall may lead to recurrence . The recLaparoscopic surgery has recently gained popularity as an approach to colon disease, due to better short-term outcomes including reduced blood loss, better recovery of bowel function, and shorter duration of hospitalization . EpidermSeveral randomized controlled studies have shown that SILS offers a feasible method with better short-term outcomes compared to conventional laparoscopic surgery , 14–16. In the present case, we successfully resected epidermoid of the cecum by SILS using an organ retractor. Taking into account the condition of the patient, tumor size, and malignant potential, this approach may hold promise as an option for epidermoid cyst arising from the colon.


Granulosa cell tumors (GCTs) of the ovary are rare, comprising less than 5% of all malignant ovarian neoplasms. While generally considered indolent, GCTs have a tendency for metastasis and delayed relapse, with recurrence developing in 20%-50%. Recurrent or metastatic disease is associated with aggressive behavior and a poor prognosis, as nearly 70% of patients developing recurrence will eventually succumb to their disease. The optimal management of relapsed disease is controversial. Initial salvage therapy typically involves surgical debulking followed by cisplatin-based chemotherapy. Unfortunately, tumor responses are durable for less than half of patients treated with this regimen. Radiation therapy is an attractive option for providing rapid palliation and improving local control without the morbidity of additional surgery or chemotherapy. Here we describe a case of multiply recurrent, rapidly growing intraperitoneal GCT refractory to repeated surgical debulking and several lines of systemic therapy. The patient was treated with two courses of palliative radiotherapy and achieved rapid symptomatic relief, achieving over a 90% reduction in tumor volume. Serum concentration of inhibin B, often inappropriately elevated in patients with GCT, decreased by 98% following irradiation with no interim systemic therapy. At one-year follow-up, the patient has no evidence of radiographic or biochemical recurrence. Malignant sex cord-stromal tumors (MSCST) of the ovary are rare, comprising less than 8% of all malignant ovarian neoplasms. The most common MSCST subtype is granulosa cell tumor (GCT), accounting for over 90% of MSCSTs and 2%-5% of all malignant ovarian neoplasms . There aGranulosa cells produce the hormone inhibin, an estradiol-stimulating growth factor that negatively regulates follicle-stimulating hormone (FSH). Serum concentrations of inhibin B, often inappropriately elevated in patients with GCT, reflect the extent of tumor burden and serve as a useful biomarker for treatment response and disease recurrence . GCT masSurgery is the mainstay of initial therapy and tumor staging. The current standard of care is total abdominal hysterectomy with bilateral salpingo-oophorectomy and peritoneal biopsy, although more conservative surgeries may be considered for women of child-bearing age interested in fertility preservation. Surgery alone is considered adequate for small early-stage tumors with a five-year disease-free survival exceeding 90%, although some experts advocate for the use of adjuvant chemotherapy when high-risk features are present . Risk faThe optimal management of the relapsed disease is controversial due to the rarity of this entity and lack of randomized studies. Initial salvage therapy typically involves surgical debulking followed by cisplatin-based chemotherapy . While iA 69-year-old woman with a history of ovarian GCT presented with increased serum levels of inhibin B concerning for disease recurrence. Eight months previously, she was diagnosed with International Federation of Gynecology and Obstetrics (FIGO) stage IC GCT, which was initially managed with hysterectomy and bilateral salpingo-oopherectomy. Adjuvant chemotherapy was recommended but she ultimately declined. At the time of biochemical recurrence, restaging computed tomography (CT) of the chest, abdomen, and pelvis with IV contrast demonstrated multiple intraperitoneal nodules. Given her favorable performance status, exploratory laparotomy with cytoreduction was felt to be appropriate. At the time of surgery, multiple macroscopic tumor deposits measuring less than 2cm were discovered in the right lower quadrant at the surface of her ascending colon and inferior liver, upstaging her to FIGO IIIC. This was followed by six cycles of carboplatin and paclitaxel. Unfortunately, restaging scans showed progressive intra-peritoneal disease. Surgical debulking was repeated at that time, but the patient was reluctant to pursue additional platinum-based chemotherapy after developing neuropathy. Subsequently, she was started on letrozole followed by tamoxifen alternated with megestrol acetate.Two months later, restaging PET/CT demonstrated a 3.7-cm hypodense lesion of the posterior segment of the right lobe of her liver with a standardized uptake value (SUV) of 5.9. Additionally, multiple sub-centimeter nodules were noted along the abdominal wall. She received three cycles of bevacizumab but further growth of the liver mass was noted on restaging scans three months later, although inhibin B was stable at 309pg/mL. The patient was referred to a surgical oncologist and underwent right hepatectomy and cholecystectomy. Final pathology of the liver mass demonstrated metastatic GCT of the ovary, upstaging her to FIGO stage IVB. Postoperatively, serum inhibin B levels were elevated at 529pg/mL.Restaging scans performed one month later demonstrated progression of multiple intraperitoneal nodules. The patient received three cycles of gemcitabine. Shortly after completing systemic therapy, she was admitted for urgent management of progressively worsening early satiety, postprandial epigastric pain, nausea, vomiting, pelvic fullness, and severe constipation. On exam, a large suprapubic mass was palpable at the midline. At that time, serum inhibin B levels were 1,014pg/mL, nearly doubling over the course of two months. Abdominal CT demonstrated a rapidly progressing large, loculated, cystic pelvic mass measuring 21.5 x 12.1 x 13.3cm Figures , 1b, andBased on institutional reports of effective palliation with radiotherapy for recurrent GCT ,10, the At three-month follow-up, inhibin B increased to 888pg/mL, and restaging CT was performed to assess treatment response. Based on imaging, the treated pelvic mass decreased significantly in size to 6.6 x 5.4 x 4.1cm Figures , achieviAt three-month follow-up, the patient reported significant improvement in the frequency and severity of her abdominal complaints. Restaging CT demonstrated a 92% volume reduction for the recently treated RUQ mass Figure . The pelWhile early-stage GCT exhibits indolent behavior, recurrent GCT tends to be aggressive and consensus for optimal management has yet to be defined. Initial management for recurrent or metastatic GCT typically involves surgical debulking followed by adjuvant chemotherapy; however, the majority of these patients will progress and ultimately die from their disease . Here weIn addition to tumor volume, a concomitant reduction in serum inhibin B was observed in response to radiotherapy. Serum inhibin B concentration reflects tumor burden and often serves as a useful biomarker for disease recurrence and therapeutic response in patients with GCT. This was demonstrated in an investigation by Mom et al. where serum levels of inhibin A and B significantly declined in response to initial surgical intervention and were predictably elevated in the setting of disease recurrence . Our casTo our knowledge, this report is the first to describe serum tumor marker response, and only the second to describe the radiologic response of recurrent intraperitoneal GCT treated with palliative radiation therapy. Our case demonstrates that palliative radiation has the potential to offer rapid tumor volume reduction and excellent local control for recurrent GCT as demonstrated by radiographic studies and serum biomarker levels. Additionally, our patient experienced rapid symptomatic relief and improved quality of life in response to treatment. As mentioned by Choen et al., there are larger retrospective series demonstrating good clinical responses to radiotherapy; however, radiographic and biomarker responses were not reported -11. LargRecurrent or metastatic GCT is associated with aggressive growth and a poor prognosis. The optimal management of the relapsed disease is controversial. Initial salvage therapy typically involves surgical debulking followed by cisplatin-based chemotherapy; however, less than half of patients achieve a durable response with this regimen. Here we present a case of symptomatic, multiply recurrent GCT refractory to repeated surgical debulking, and several lines of systemic therapy. The patient was treated with palliative radiotherapy, achieving significant reductions in tumor volume, decreased serum biomarker levels, and improved quality of life at one-year follow-up.


Acute or chronic non-neoplastic diffuse mediastinal diseases have multiple causes, degrees of severity, and a wide range of management. Some situations require emergency care while others do not need specific treatment. Although the diagnosis may be suspected on chest X-ray, it is mainly based on CT. A delayed recognition is not uncommonly observed. Some findings may prompt the radiologist to look for specific associated injuries or lesions.This pictorial review will successively describe the various non-neoplastic causes of diffuse mediastinal diseases with their typical findings and major differentials.First, pneumomediastinum that can be provoked by extra- or intra-thoracic triggers requires the knowledge of patient’s history or recent occurrences. Absence of any usual etiological factor should raise suspicion of cocaine inhalation in young individuals.Next, acute mediastinitis may be related to post-operative complications, esophageal perforation, or contiguous spread of odontogenic or retropharyngeal infections. The former diagnosis is not an easy task in the early stage, owing to the similarities of imaging findings with those of normal post-operative appearance during the first 2–3 weeks.Finally, fibrosing mediastinitis that is linked to an excessive fibrotic reaction in the mediastinum with variable compromise of mediastinal structures, in particular vascular and airway ones. Differential diagnosis includes tumoral and inflammatory infiltrations of the mediastinum. Pneumomediastinum requires exclusion of tracheobronchial injury.Mediastinal post-operative changes are indistinguishable from acute mediastinitis during the first weeks following surgery.CT, 18F-FDG PET/CT, and diffusion-weighted or dynamic contrast-enhanced MRI may help to narrow the differential diagnosis and may guide tissue sampling.Diffuse mediastinal diseases comprise a heterogeneous group of entities characterized by various degrees of severity . AlthougAlthough mediastinal involvement may be suspected on chest radiographs, the signs may be easily missed; hence CT, and, in selected patients, MRI, should be conducted to evaluate the mediastinal compartments. High-resolution imaging is required to provide a detailed assessment of the mediastinal compartment and its complex anatomy, as well as associated anomalies. CT, with a water-soluble contrast agent following a non-contrast acquisition, may demonstrate the esophageal rupture in case of pneumomediastinum, or stenosis in case of chronic mediastinitis. Next, an acquisition with IV contrast may be beneficial in acute and chronic mediastinitis—simultaneous injection of contrast material via brachial veins on both sides may be considered—because this can also depict the regional environment, as well as any additional findings. 18F-FDG PET/CT and diffusion/dynamic contrast-enhanced MRI  may be hSpecific associated injuries or lesions need to be systematically determined, based on the imaging findings. Various non-neoplastic conditions will be discussed in the following sections.Pneumomediastinum, defined as free gas located within the mediastinum, may dissect along fascial sheaths Fig.  to creatThe Macklin effect, or pulmonary interstitial emphysema, which is responsible for more than 95% of cases, corresponds to an alveolar rupture with air dissection along the interstitial sheaths toward the mediastinum Fig. , eventuaAt least half of all esophageal perforations are estimated to be iatrogenic in nature. This can result from endoscopic or surgical procedures, as well as thermal injury during left atrial ablation. Typical imaging findings include pneumomediastinum, pleural effusion on the left side more commonly than on the right side, and mediastinal hematoma  with extSpontaneous rupture, known as Boerhaave’s syndrome, occurs when incomplete crico-pharyngeal relaxation during vomiting results in a sudden increase in intraluminal esophageal pressure Fig. . RuptureTracheobronchial lesions are rare, occurring in 1 to 3% of patients with blunt trauma and 2 to 9% of those who suffer penetrating cervical and/or thoracic injuries. The former preferentially causes lesions in the intra-thoracic trachea and main-stem bronchus, 80% of which are up to 2.5 cm from the carina, most commonly on the right side, and are considered less protected by the mediastinal structures. Uncommon causes include iatrogenic injuries, such as intubation-related trauma  Other causes of pneumomediastinum include acute exacerbation of severe asthma, excessive coughing, diabetic ketoacidosis, physical exertion, the Valsalva maneuver, or inhalational drug abuse , as wellMost cases of acute mediastinitis are encountered following cardiovascular or other thoracic surgical procedures. Risk factors for post-sternotomy mediastinitis may be categorized into patient-related, including age as well as co-morbidities, such as obesity, diabetes, history of smoking, COPD, and renal failure, and operative factors or environmental elements  .Other cau ses typically comprise either esophageal perforation or the contiguous spread of odontogenic or retropharyngeal infections Fig. . Rarely,In a retrospective study that included 40 various causes of acute mediastinitis, the most common abnormal findings were represented by increased attenuation of fat and pleural effusion, followed by free gas bubbles in the mediastinum, localized mediastinal fluid collections, sternal dehiscence, mediastinal lymph nodes, pericardial effusion, lung infiltrates, and pleuro-mediastinal fistula . There wIn any case, careful analysis of cartilage and bone is necessary to detect loco-regional involvement, such as a cartilaginous abscess or focal osteitis Fig. .Fatal aortic arch ruptures Fig.  have beeFibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is a condition related to an abnormal immunologic reaction that results in the proliferation of fibro-inflammatory tissue in the mediastinum and/or hila . It leadThe most common form is the focal or granulomatous subtype of fibrosing mediastinitis, mainly due to histoplasmosis (less frequently tuberculosis), and other fungal or inflammatory conditions, such as sarcoidosis. This form usually manifests as a localized, calcified mass in the paratracheal or subcarinal regions of the mediastinum or in the pulmonary hila, commonly responsible for a superior vena cava syndrome. Diagnosis can be made based on imaging findings, preventing the need for tissue sampling.The diffuse or non-granulomatous subtype accounts for about 10 to 20% of cases of fibrosing mediastinitis. It may be idiopathic or can originate in association with autoimmune disorders, methysergide exposure, or prior radiation exposure . FibrosiFibrosing mediastinitis has been reported as an unusual mediastinal manifestation of IgG4-related disease Fig.  14]. Th. Th14]. In addition to IgG4 disease, some disorders, such as Erdheim-Chester disease, must be suspected in case of multisystemic involvement. This rare, non-inherited, non-Langerhans form of histiocytosis, commonly with a BRAF mutation, is characterized by xanthomatous infiltration of the involved tissues with foamy histiocytes surrounded by fibrosis, and appears with heterogeneous systemic manifestations. A common peri-aortic infiltration that extends to the pericardium, the right coronary sulci, and/or the myocardium of the right atrium with pleural involvement may point toward this rare entity. Furthermore, bone, renal, and retroperitoneal involvement Fig.  15] hav hav15] hThe radiological differential diagnosis of diffuse fibrosing mediastinitis is extensive Fig.  16] and and16] aIn addition to CT and 18F-FDG PET/CT, diffusion and dynamic contrast-enhanced MRI sequences may be useful to ensure an exhaustive assessment of the disease, particularly its cardiovascular extent. Moreover, these sequences may point out the optimal areas to biopsy Fig.  [18].FiDue to the histological complexity of these various entities, cytological sampling with fine-needle aspirations should be avoided and surgical biopsy sampling is often required to ensure sufficient material for detailed histologic analysis, immunohistochemical staining, molecular analyses, and culturing , if needIn all cases, a systematic analysis of the pulmonary veins and arteries, as well as the tracheobronchial tree and esophagus, is advised when encountering any infiltrative soft tissue density within the mediastinum. This may help to suggest the diagnosis of chronic mediastinitis and, thus, avoid delayed recognition of this condition. Such an approach has to be combined with an overall analysis of the retroperitoneum, liver, thyroid, and pancreas.In conclusion, non-malignant diffuse mediastinal disease presents with various characteristics, depending on the mechanism involved. The differential diagnosis calls for a systematic approach that includes the patient’s history and habits, excludes a tracheobronchial or esophageal injury in acute conditions, and requires a careful and precise histological examination in diffuse mediastinal tissue infiltration.


Hutch diverticulum is a congenital diverticulum of the urinary bladder, reported infrequently in children and rare amongst adults. We present a 60-year-old male patient with bilateral Hutch diverticula, detected incidentally during an abdominal ultrasound examination performed for blunt abdominal trauma. This rare case highlights an unusual incidental presentation and opportunity to learn how to differentiate it from acquired bladder diverticula. The available treatment options are also discussed varying from simple follow-up to aggressive surgery. They are classified as congenital or acquired. Congenital diverticula are reported infrequently in children and are ever rarer in adults2 Their incidence is reported to be 1.7%3 and peaks in children below 10 years of age.4 Ninety percent of congenital bladder diverticula are located superolateral to the ureteral orifice, in proximity to the uretero-vesical junction.5 Unlike the acquired adult form, in which outlet obstruction or neurogenic dysfunction is almost always present, congenital bladder diverticula result from hypoplasia of the muscular layer of the bladder wall.1 It may be unilateral and infrequently bilateral. Patients may remain asymptomatic or may present with symptoms of urinary retention, obstruction, voiding dysfunction, recurrent urinary tract infection and calculi formation in the diverticulum.6 The symptoms are more pronounced in patients with a large diverticulum, whereas cases with a small diverticulum may remain undiagnosed. We present the rare case of bilateral asymptomatic congenital bladder diverticula discovered incidentally in an elderly male patient.Urinary bladder (UB) diverticula are herniations of the bladder mucosa through muscular fibers of the bladder wallA 60-year-old male patient presented with a history of a fall, followed by acute pain in the abdomen for which he was attended to in the emergency department. The patient was sent for Focused Assessment with Sonography for Trauma (USG FAST), which was negative. Incidental note was made of left hydronephrosis  with a lFocused detailed history taking revealed a complaint of difficulty in micturition with a subsequent sensation of incomplete voiding over the previous 1–2 years, resulting in a repetitive need to pass urine. Intermittent dull pain was also present in left flank. There was no history of passage of blood or pus in the urine, urgency for micturition, hesitancy, poor stream or terminal dribbling of urine. There was no past history of any long standing illness in the patient.As the patient’s complains were directed towards a lower urinary tract infection, retrograde urethrography (RGU) and micturating cysto-urethrogram (MCU) were performed. The RGU was unremarkable. The MCU  demonstrIn view of the setting of blunt trauma and continuing pain in abdomen, contrast enhanced computed tomography (CECT)  and 5 waThe patient was discharged with the advice of regular follow-up in the surgical outpatient department (OPD) and to report earlier if any symptoms develop.1 resulting in a thin-walled outpouching arising from the bladder lumen. Histological examination of surgical specimens after resection show similar findings and further support the theory of ‘hypoplasia of the muscularis layer’ as a cause for the herniation. The wall of the diverticulum is thinned out and empties poorly during micturition due to lack of the muscular layer.1 This leads to increased post-void residue with paradoxical enlargement of the bladder diverticulum during micturition. Congenital or primary bladder diverticula usually present during childhood4 and occur in the absence of bladder outlet obstruction as opposed to the acquired form. They are associated with congenital syndromes, namely Ehlers–Danlos (type 9) syndrome, Menkeskinky hair syndrome, Cutis Laxa syndrome (Sotos) and Williams–Beuren syndrome.6 In our patient a focused physical examination was performed to rule out these congenital syndromes.Bladder diverticula are rare clinical entities in both the paediatric and adult populations. They are classified as congenital or acquired. The basic differences between the two are highlighted in 7 whereas the acquired diverticula are associated with multiple bladder trabeculations. The congenital diverticula are usually unilateral but can be bilateral and are usually seen at the base of the trigone, in the vicinity of VUJ (6). On the contrary, the acquired diverticula can arise from any portion of the bladder wall and are usually multiple in number. The congenital variety does not have any risk for malignancy as opposed to the secondary acquired type.8On cystoscopy, congenital diverticula reveal a smooth wall,6 where Waldeyers’ sheath anatomically covers the space between the intravesical ureter and muscular layer of bladder.9 The defect eventually enlarges with voiding and finally the ureteral orifices are incorporated into the diverticulum.10Diverticula located superolateral to the ureteral orifice, without involving the trigone, are referred to as ‘Hutch diverticula’ and are usually associated with vesicoureteral reflux. It is a rare developmental anomaly that develops because of failure of muscle development at or near the ureteral orifice. Initially, a small diverticulum is formed because of herniation of bladder mucosa through the weakest point of the bladder musculature,11 Patients with Hutch diverticulum can be asymptomatic or may have diverse symptoms, secondary to urinary stasis. The most common presentation is with urinary tract infections (UTI). Other presentations may include acute urinary retention, bladder stones, enuresis and possible bladder obstruction if the diverticulum enlarges and obstructs the bladder neck distally.7There are very few reported cases, mostly in children. The presence of Hutch diverticulum in adults is very rare.8 Laparoscopic approach may also be performed as it has the advantage of minimally invasive surgery. Endoscopic transurethral incision of diverticular neck is the treatment option offered to non-surgical candidates.12 In patients with VUR, congenital defects are treated with ureteral reimplantation surgery. However, as the acquired form is mostly secondary to an inappropriate increase in detrusor muscle pressure, reimplantation is never performed in these patients prior to managing the detrusor muscle abnormality. Medical management with drugs to avoid recurrent UTI and anticholinergics to maintain the bladder wall overactivity are available.Since there is no risk of malignancy associated with the congenital form, if the patient is asymptomatic, no active intervention is required and advice is given regarding frequent micturation and complete evacuation. If the patient presents with recurrent symptoms, surgical management is warranted. The surgical options include open diverticulectomy  which may be beneficial in cases of concomitant prostatic enlargement, allowing simultaneous treatment of both entities. The extravesical approach is reserved for patients with large diverticula associated with peridiverticular adhesions or inflammation.Congenital bladder diverticula are very rare in adults and the radiologist should be able to differentiate them from the commoner acquired conditions. If asymptomatic and uncomplicated, they are usually managed conservatively, but in cases with recurrent symptoms or complications, appropriate surgical intervention should be considered.


The first goal in orthopedic oncology is resection with a free margin while preserving as much function as possible. Techniques popularized in recent decades with the potential to advance reconstruction and thus function following resection include allograft transplantation and prosthetic replacement. Every operative technique has its limitations.,,,,New solutions can be pioneered thanks to the development of prosthetic printing technology  of the left olecranon. The patient was treated with curettage, autologous bone grafting, and fixation with plate and screws. Pathologic examination confirmed the diagnosis of chondrosarcoma grade I. Rehabilitation went uneventfully, as did removal of hardware in 2009.Seven years later, in 2016, at the age of 49, the patient was referred to our orthopedic oncology center with nocturnal pain in his elbow joint. Physical examination showed a limited extension of 40° and a palpable mass at the left olecranon. ,Dissemination studies turned out to be negative. In conclusion, radiographs, magnetic resonance imaging, and histologic evaluation pointed to a diagnosis of grade II chondrosarcoma. In case of a recurrent grade I chondrosarcoma , a recurettage would be adequate treatment. However, with a recurrent and apparent histologic grade II chondrosarcoma, rigorous treatment using wide excision is necessary to achieve clean margins.Based on CT scan of the bony structures of the ipsilateral elbow, the resection was planned and a computer-aided design model for the implant was made, including especially designed additional fixation holes for the triceps tendon and collateral ligaments . Next, tThe patient was placed in a lateral decubitus position with the left arm resting over a roll. The relevant anatomy of the elbow and the scar of the previous incision were marked over the olecranon. Prophylactic antibiotics were administered intravenously. After standard disinfection and preparation, a longitudinal incision over the proximal ulna and distal humerus was made.The ulnar nerve was released and kept intact. The distal triceps tendon was detached from the ulna, including the periosteal sleeve and anconeus fascia, to preserve the triceps tendon for reinsertion into the prosthesis. The proximal radial head could be preserved with its annular ligament. Lastly, the humeral articulation was released.The next step was en bloc resection of the grade II chondrosarcoma with the cut of the ulna based on the magnetic resonance imaging scan in order to have enough distance from the tumor and achieve adequate margins. A cut was made with a saw 8 cm distally from the olecranon tip, and the specimen was sent for histologic examination.The ulnar shaft was reamed to create a 5-mm-diameter hole of sufficient depth. A 3D-printed trial implant was seated and fitted correctly, with a focus on restoring length, rotation, and articulation with the radius and ulna.Next, the definitive implant was impacted press-fit with the right rotation. The accessory holes in the prosthesis were used to attach the triceps with its periosteal layer and the medial ulnar collateral ligaments using nonresorbing sutures. After reattachment, the elbow felt stable. The ulnar nerve was placed in a submuscular tissue envelope but not purposefully transposed. The subcutaneous and skin layers were closed over the prosthesis without tension. A bandage and shoulder-elbow immobilizer were placed on the arm. The immediate postoperative radiograph showed an adequate position of the prosthesis. The motor and sensory function of the medial, radial, and specifically the ulnar nerve remained intact directly postoperatively, with a slight sensory loss in the fifth digit.The sling was maintained for 6 weeks of complete immobilization, after which physiotherapy was started for passive motion extension/flexion and pronation/supination.Pathology report confirmed the diagnosis of grade II chondrosarcoma with wide margins of 25 mm to resection plane and 5 mm distance to the joint. In the following months, the patient recovered painlessly, with 140/10° flexion/extension and 80/50° pronation supination at the 18-month follow-up, and 140/30° flexion/extension and 90/70° pronation/supination at 2 years. Elbow function remained the same at 2.5 years' follow-up. Conventional radiographs  showed aReconstruction with a personalized 3D-printed custom-made proximal ulnar hemiarthroplasty is reported after resection of a chondrosarcoma grade II of the proximal ulna in a 49-year-old man. Follow-up at 2.5 years shows fair elbow function with prosthetic ingrowth. To our knowledge, this is the first case report describing a solitary 3D-printed proximal ulnar prosthesis.,,,When performing oncologic surgery, the challenge lies in complete resection of the tumor while preserving function by sparing as much bone, ligament, capsule, tendon, and muscle as is oncologically safe. Various reconstructive methods have been developed over the years: allograft, autograft, and tumor prosthesis surgery, all with advantages and limitations. Allograft may provide excellent support, yet there is the possibility of resorption and nonunion. Another daunting limitation of allograft is the lack of long-term incorporation, often restricted to the interface.,,Recent literature evidences the successful use of 3D-printed models, making patient-specific instruments,In our case there were several reasons to choose a hemiarthroplasty over other options: because of the large size and high grade of the tumor, en bloc excision had to be performed near the elbow joint. As a consequence, replacement of an articular surface would be insufficient to reconstruct with a vascularized fibular graft because of insufficient fit, and fixation could be problematic for vascularization. Second, because the tumor had invaded only into the proximal ulna, a total elbow prosthesis would result in unnecessary sacrifice of the humeroulnar joint. Total elbow arthroplasties have shown varying results in terms of survival,,,,,Both custom-made 3D templates and implants have shown promising result in lower- and upper-extremity osteoarthritis,Potential flaws of a custom-made 3D-printed prosthesis are unrestored rotation, chances of humeral erosion, and overstuffing. The scarce literature shows only short-term results, making long-term follow-up essential.This case demonstrates use of a custom-made 3D-printed prosthesis of the proximal ulna for the treatment of a grade II chondrosarcoma. Satisfactory function is seen at the 2.5-year follow-up. Use of 3D-printed prostheses can be a valuable adjunct for oncologic orthopedic surgeons when treating bony tumors near a joint.The authors, their immediate families, and any research foundations with which they are affiliated have not received any financial payments or other benefits from any commercial entity related to the subject of this article.


Only kings, presidents, editors, and people with tapeworms have the right to use the editorial “we.” Mark Twain .Taenia saginata (T. saginata). It was motile and the scolex had 4 suckers but lacked the rostellum and rostellar hooks (Supplementary Video). No drugs were administered to the patient for the parasite.A 53-year-old woman with a history of in-vehicle injury was admitted to the emergency department of Trakya University Hospital. Initial examination revealed that the arterial blood pressure, pulse, and respiratory rate were 90/60 mmHg, 104/min, and 22/min, respectively. Diffuse tenderness was observed during the abdominal examination; however, no defensive and rebound findings were found. The laboratory parameters did not show any specific values. Abdominal ultrasonography exhibited intraperitoneal free fluid, and computed tomography showed active intraperitoneal hemorrhage along with hematoma in the mesenteric area. The symptoms of peritoneal irritation in the upper abdominal quadrant along with imaging studies suggested a surgically acute abdomen. Afterward, urgent exploration by laparotomy was performed. Multiple injuries and bleeding foci were detected in the small intestine meso during the surgical exploration. The circulation was disrupted with no peristalsis in the small intestine segment of about 80 cm at a distance of 180 cm from the ligament of Treitz. Furthermore, segmental small bowel resection was performed. During anastomosis, a tapeworm was observed to be moving from the intestine into the abdominal cavity. The tapeworm, almost 4 m long and encountered by chance, was carefully removed from the abdominal area and sent to the laboratory . After tar hooks . The numT. saginata is distributed among humans globally, including Turkey, through various sources. Infection is due to nutritional habits, such as eating raw or undercooked beef as observed in our case. The cases with T. saginata are usually asymptomatic. The scolex of T. saginata has 4 suckers without hooklets, whereas the scolex of T. solium is armed with hooklets (hooklets ,2. In lihooklets ,4.T. saginata with scolex at the operating table. The scolex and premature proglottids of live T. saginata were observed in detail using microscopy.In this case, we completely removed the living


An umbilical hernia, although considered a benign condition of childhood, may rarely manifest with serious complications such as incarceration and viscous organ strangulation. One such case is presented in this report in an attempt to enrich the current literature, due to the relative lack of data in regard to complicated umbilical hernias in children and definite guidelines regarding the monitoring and management of uncomplicated cases. In addition, we discuss some of the latest advancements concerning the matter at issue. An umbilical hernia is a common condition of early childhood, defined as a protrusion of abdominal viscera through a defect in the umbilical ring. An incarcerated hernia is one in which the hernia cannot easily be reduced into the peritoneal cavity, while strangulation occurs when the blood supply to the entrapped tissue is compromised. We present a rare case of umbilical hernia incarceration in a 14-month-old female, which progressed to strangulation of a small bowel loop.A 14-month-old female presented to the emergency department due to abdominal pain and an episode of vomiting, while clinical examination revealed an irreducible umbilical hernia, with mild discoloration. Parents reported drowsiness and refusal to eat since that morning, accompanied by inability to pass stool for the last three days, which they misinterpreted as occasional constipation.The child underwent ultrasonographic examination that demonstrated the presence of a hernia at the level of the umbilicus, containing a small bowel loop with adequate motility and perfusion. The width of the abdominal opening was estimated at about 1 cm.The child was transferred to the operating room shortly after the admission to the pediatric surgery department . The herThe postoperative course of the child was excellent with early mobilization and satisfactory response to oral feeding, since the child was breastfed in the immediate postoperative period. Following discharge, the patient had an uneventful follow-up.The incidence of umbilical hernias is about 18–20% in term neonates and around 84% in premature neonates, as it was estimated in an epidemiologic study in Denmark ,4. WhileThe clinical presentation includes abdominal pain, vomiting, refusal to feeding, inability to pass stool or gas and an irreducible, tender to palpation umbilical bulge, potentially accompanied by discoloration ,7. PreveSo far, the consensus suggests surgical intervention after the age of 4–5 in asymptomatic children, due to spontaneous healing in 85% of patients ,8,9. A rIncarcerated nonreducible umbilical hernias in children require prompt surgical intervention owing to the increased risk of viscous strangulation. Therefore, a high level of alertness should be maintained in regard to children with asymptomatic umbilical hernias. It is imperative for caregivers to have a clear understanding of the condition. Thus, it is crucial that health professionals responsible for the care and follow-up of these children provide parents with all the necessary information, in an effort to avoid morbidity. Further studies are required to determine which children with asymptomatic umbilical hernias are prone to complications and need surgical treatment.


Cranial hemophilic pseudotumor (cHPT) is a very rare disease, which is easy to misdiagnose. It is also difficult to manage such patients. We reported the first case of occipital cHPT.Here, we presented a rare case of an occipital bone mass in a 3-year-old boy who was diagnosed with hemophilia A. The mass was misdiagnosed as an aneurysmal bone cyst by pathological examination. After resection, the patient underwent one-stage cranioplasty. However, the patient was admitted again for hematoma caused by an invasive procedure. A second surgery and one-stage cranioplasty were performed at the same time. A follow-up 3 months after discharging showed the patient was uneventful, and the titanium mesh was well fixed.The diagnosis of cHPT requires the combining of history, radiological examination, and pathological examination. Resection is the best choice for symptomatic cHPT. Replacement treatment and less invasive treatment can make perioperative management safer. One-stage cranioplasty for resection of an occipital cHPT can improve the quality of life. Hemophilia could cause severe hemorrhage complication but is rare in the skull. The cranial hemophilic pseudotumor (cHPT) is a severe complication, which occurs in 1–2% of hematoma in hemophilic patients –5. PerioA 3-year-old boy was admitted for progressively enlarging occipital mass and persistent headache. The mass was found 1 year ago and grew obviously within 2 months. The child had a traumatic history in the occipital (about 1.5 years ago).Magnetic resonance angiography (MRA) did not find obvious arterial supply within the lesion Fig. a. CT sugLaboratory examination on admission suggested that the activated partial thromboplastin time (APTT) was prolonged (64 s), and the clotting factor VIII was decreased (48.4%). The hematologist considered the patient as a hemophiliac. A replacement treatment was subsequently performed using massive fresh frozen plasma (FFP). After controlling the APTT within 11.6 to 15.9 s, the coagulation was subsequently monitored for 2 days. Confirming that the coagulation was suitable for surgery, surgical resection was immediately arranged.What we saw intraoperatively was an organizing hematoma with scattered calcification, massive fibrous tissue hyperplasia around the lesion, and several small bony cysts within the lesion. After total resection, one-stage cranioplasty was performed too. The volume of intraoperative bleeding was 300 ml. Postoperative CT was negative Fig. d. After The final pathological diagnosis was cranial hemophilic pseudotumor Fig. e, f.The patient was admitted for subcutaneous hydrops 12 days after discharge. A puncture was performed. One hour later, the patient felt nausea and headache. A compression and sign of cerebral hernia caused by epidural hematoma was found on CT (Fig. Three months after discharging, a radiological follow-up showed the titanium mesh healed well with surrounding tissue without subcutaneous hydrops Fig. e, f. TheThe cHPT is so rare that only seven cases of this disease have been reported Table . Here, wIt is difficult to diagnose a cHPT for similar features between cHPT and some diseases, such as ABC , 8. TheyThe surgical resection is the preferred treatment –16. HoweDue to the risk of postoperative bleeding and little impact on the quality of life, no one-stage repair has been reported in previous cases , 12, 13.The diagnosis of cHPT requires the combining of history, radiological examination, and pathological examination. Resection is the best choice for symptomatic cHPT. Replacement treatment and less invasive treatment can make perioperative management safer. One-stage cranioplasty for resection of occipital lesion can improve the quality of life.


Parosteal osteosarcoma is a low-grade, bone-forming neoplasm that arises on the surface of bone. It accounts for about 4% of all osteosarcomas. An 18-year-old male patient with no particular past medical history, consulted for a painless mass in the right thigh that had appeared at the age of 17 years and progressively increased in volume. The physical examination revealed a 6 cm mass at its largest above the right popliteal fossa with knee flexion slightly limited. The X-ray revealed a well-limited mass in the lower third of the femur that was dense and attached to the metaphyseal cortex by a wide base. Histological examination of the biopsy specimen established the diagnosis of parosteal osteosarcoma. The patient underwent wide resection of the femoral tumor (A) preceded by a course of first-line chemotherapy. Histological examination showed a malignant mesenchymal proliferation, moderately cellular, made up of long, linear and eosinophilic material, sometimes calcified with no osteoblastic cells in the periphery (B). The tumor cells were spindle-shaped, with little eosinophilic cytoplasm and a long or ovoid, hyperchromatic, and moderately atypical nucleus. Mitoses were rare. There were no areas of dedifferentiation. Postoperative course was uneventful. During the one-year follow-up period, there was no recurrence or metastasis of the tumor. Parosteal osteosarcoma is characterized by its insidious growth and favorable prognosis. It rarely leads to metastasis. Its treatment is mainly surgical.


Case Report. We are presenting a case of a 25-year-old man who underwent laparoscopic sleeve gastrectomy for morbid obesity, which was complicated with the development of gastrobronchial fistula, despite an unremarkable postoperative course.  Obesity is one of the leading causes of morbidity and mortality in countries all over the world, and its prevalence has been increasing dramatically in recent years. Bariatric surgery is considered the gold standard of care for patients who failed conservative management. Laparoscopic sleeve gastrectomy (LSG) is of increasing popularity. One of its vicious consequences is the development of acquired fistula between the stomach and the tracheobronchial tract due to intractable gastric leak.  Acquired gastrobronchial fistula due to bariatric surgery is not reported widely in radiologic literature; hence, there is lack of consensus of the diagnostic modality of choice. However, there is a myriad of tests available for diagnosing gastrobronchial fistula, with contrast study of the upper gastrointestinal tract which is the widely accepted diagnostic test. Obesity is one of the leading causes of morbidity and mortality all over the world , and itsThe most common chronic complications of LSG are gastroesophageal reflux disease and gastric stricture , while hA 25-year-old male presented to the emergency department 2 months post-LSG with an unremarkable postoperative course. At presentation, he had a left upper quadrant abdominal pain associated with repeated vomiting, subjective fever, generalized body aches, and cough for two weeks. The cough was productive of greenish sputum with a tinge of blood and provoked by any oral intake. A review of other systems was unremarkable.2 99%, and temperature: 36.4°C. Abdomen examination shows soft and lax, mild generalized tenderness especially in epigastrium, normal bowel sounds. Chest examination shows no increased work of breathing, normal chest expansion, percussion note slight dullness in the left lower zone, chest sounds decreased breathe sounds in the left lower zone with bronchial breathing, and fine crackles.Physical exam shows the following: RP 103 BPM, BP 129/70, RR 18, SpO3/μL , CRP: 6.7 mg/L ; blood and sputum culture were negative.Diagnostic testing shows the following: WBC: 8.5 × 10Initial chest radiograph  showed aFor more anatomic delineation of gastrobronchial fistula, an upper GI study with water-soluble contrast  was perfAfter confirmation and proper surgical planning of gastrobronchial fistula, bariatric surgery was intervened by the laparoscopic approach. The operative technique was resection of the fistula (found connected to GE junction) and gastrojejunal anastomosis. The patient had a successful postoperative recovery.Moeller and Carpenter first classified the causes of gastrobronchial fistula into five categories: (1) trauma, (2) esophageal or gastric surgery, (3) neoplasm, (4) gastric ulcer, and (5) subphrenic abscess . GastricDistal stenosis following LSG may be the explanation of development of GBF, which decreases gastric emptying and thereby increases pressure in the stomach and directing the gastric contents into the fistula tract. This facilitates persistent communication between the stomach and the respiratory tract , 15.The leak of gastric contents can lead to the development of a subphrenic abscess, which may spread above the diaphragm facilitated by lymphatic flow or by direct erosion through the diaphragm, leading to the development of a lung abscess that may end up opening into a bronchial tree . The devIn our case, the postoperative period was unremarkable with no history of intervention, so we propose that the gastrobronchial fistula occurred secondary to a chronic silent microleak, which might be not significant to cause symptoms. That said, this possibly resulted in the spread of gastric contents into the left lower lung lobe creating a gastrobronchial fistula.Clinically, patients present with symptoms related to the development of a subphrenic abscess , pulmonary symptoms , or even vomiting and expectoration of food particles and gastric contents , 18–20. Due to the very rare incidence of gastrobronchial fistula with limited published data in radiology literature, the diagnosis of gastrobronchial fistula is challenging to radiologists. CT is more likely to identify secondary signs, though less likely to identify the fistulous tract itself. Endoscopy does not diagnose GBF but can identify its internal opening, as seen in our case.Despite the scarcity of evidence-based data about the gold standard diagnostic test for detection of GBF after LGS, a contrast study of the upper gastrointestinal tract is the widely accepted means of diagnosing a GBF , 22, 23.When the upper gastrointestinal study was considered in our case, there was a dilemma regarding which type of contrast material to choose. There are several types of contrast materials used for UGI series. Barium sulphate is most commonly used. However, due to the concerns of gastric leak, this agent was not used as it can lead to peritonitis. Water-soluble contrast materials , on the other hand, are safer in case of suspected leak because they are absorbed rapidly from the interstitial spaces and peritoneal cavity .Another potential risk taken in consideration was pulmonary aspiration as the patient's clinical presentation of postprandial cough and vomiting raised suspicion of severe gastroesophageal reflux or fistula, which can result in aspiration to an uncertain extent. High osmolality contrast materials  may, if introduced into the bronchial system due to aspiration or by fistula as in our case, induce severe bronchial irritation and life-threatening pulmonary edema. In our case, low osmolar iodinated contrast (Omnipaque 350) was used as it safe in case of suspected leak or aspiration .The treatment of gastrobronchial fistulas should be tailored to the clinical state of the patient, and in the absence of sepsis, conservative management shall be tried : managemAlthough the development of gastrobronchial fistula postlaparoscopic sleeve gastrectomy is rare, it is a serious and a difficult complication to manage. Post-LSG gastrobronchial fistula is not reported widely in radiologic literature; hence, confirming the diagnosis might be challenging; however, a contrast study of the upper GI tract is considered the ideal diagnostic test. Most of the post of the reported cases have evidence of postoperative gastric leak/subphrenic collection, in contrast to our case which has unremarkable postoperative recovery. Management of GBF should be individualized to each case, and it ranges from conservative and noninvasive to aggressive surgical approaches.


This is a case that emphasized the need for detailed observation of the entire lesion in dermoscopic examination. Novel dermoscopic findings within a collarette. The whitish‐pink, ovoid nest and translucent‐brown globules with poorly visualized vessels in the collarette are shown (arrows) The clue was found in a collarette, which is thought to be a nonspecific site.Eccrine poromas (EPs) are rare benign adnexal tumors of various histological types that are dermoscopically challenging to diagnose.22.1A 65‐year‐old man presented with a 6.5 × 4.5‐mm, symmetrical, well‐circumscribed, non‐pigmented, deep‐red papule surrounded by an indented moat with collarette scales, on the right thigh Figure . The patDermoscopic examination revealed milky‐red globules and chalice‐like vessels surrounded by a well‐circumscribed hyperkeratotic rim showing a white double–ring configuration due to thick scales. In the collarette, a whitish‐pink ovoid nest and translucent‐brown globules representing poorly visualized chalice‐like vessels were observed Figure . ChaliceHistological examination revealed sharply circumscribed, small cuboidal epithelial cells with monomorphous ovoid nuclei and compact eosinophilic cytoplasm. Cytoplasmic vacuoles were observed. The lesion was a poroid neoplasm mainly on the dermis, although it slightly extended to the epidermis as well. The surrounding stroma was edematous with dilated vessels of various sizes. The area of the tumor showed parakeratotic hyperkeratosis with crusting, and the granular layers had thinned or disappeared Figure . At the 2.2A clinical diagnosis of EP was made, and surgical resection of the affected tissue with a 2‐mm margin of normal tissue was performed. Written informed consent was obtained from the patient for the use of the tissue samples in this study.2.3The final diagnosis based on histopathological findings was EP, which was completely curatively resected. The postoperative course was encouraging, showing no evidence of a local recurrence. At present, 1 y after the operation, no findings suggesting a recurrence have been recognized and careful postoperative follow‐up is planned.3Poroma was first reported in 1956A collarette in dermatology refers to a narrow rim of loosened keratin overhanging the periphery of a circumscribed skin lesion and attached to the normal surrounding skin. The outer margin of the collarette is adherent, while the inner margin is free. In practice, however, the skin forming the collarette may be scaly or thick and ridge‐like, forming a rim around the lesion. While many disorders primarily exhibit such a morphology, it may be a secondary or a nonspecific manifestation in certain others. Dermatoses exhibiting a scaly collarette have been reported in skin tumors such as clear‐cell acanthoma, pyogenic granuloma, acral fibrokeratoma, supernumerary digit, cutaneous horn, subungual exostosis, eccrine poroma, acral fibromyxoma, stucco keratosis, and juvenile xanthogranuloma.The dermoscopic findings and the corresponding pathological findings in this case can be stratified according to (a) collarette parts and (b) non‐collarette parts. In collarette parts, the dermoscopic findings were: (1) whitish‐pink ovoid nest, (2) translucent‐brown dotted with poorly visualized vessels, and (3) white double‐ring translucent collarette. The pathological findings were as follows: (1) invasion and anastomotic images from the tumor nest recognized outside the epidermal invagination, (2) mild edema surrounding the stroma and absence of branched vessels with rounded endings, and (3) well‐circumscribed hyperkeratic rim showing a double‐ring configuration due to thick scales. The inner ring‐like segment was consistent with the epidermal invagination. In non‐collarette parts, the dermoscopic findings were (1) milky‐red globules, (2) chalice‐like vessels, and (3) fragments of scaly tissues. The pathological findings were as follows: (1) poroid neoplastic lesions mainly on the dermis and continuing into the epidermis; small ducts and a few cystic spaces in tumor cell clusters with edema surrounding the stroma and dilated vessels of various sizes; (2) polymorphous vascular pattern, including at least two types of vascular structures showing irregular linear vessels with semi‐elliptical endings giving the appearance of a chalice; and (3) parakeratotic hyperkeratosis with crusting at the tumor site and thinning or absence of granular layers. The characteristic dermoscopic findingsThe dermoscopic image mainly comprised tumor with vascular structures located in the interstitial tissue, and the histopathology image further identified alterations in the spread of the tumor in the dermis, degree of inflammation, vascular hyperplasia in the interstitial tissue, and morphology of the lumens. Multiple elements were considered to combine to form a dermoscopic image. In this study, we report the diagnostic clue of dermoscopy of non‐pigmented EP in a collarette, which is thought to be a nonspecific site. Because this is a single case report, an accumulation of further studies is required. In conclusion, this was a case that recognized the need for detailed observation of the entire lesion in dermoscopic examination.None declared.TT: Collected the data and wrote the manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images.


Pregnancy has been demonstrated as a significant risk factor of splenic artery aneurysm (SAA) formation and rupture. However, prompt diagnosis of SAA rupture in a pregnant patient showing acute abdomen has been practically challenging in light of its rarity and vague initial presentation.A 40-year-old woman  at 35 weeks’ gestation presented to the emergency department with upper abdominal pain and nausea. Because of fetal dysfunction, emergency caesarian section was performed by a Pfannenstiel incision. Following delivery, 400 g of hemorrhage was removed from the upper abdominal cavity. Computed tomography showed a 37-mm SAA associated with copious adjacent fluid. Although selective angiography did not demonstrate active extravasation, interventional isolation of the SAA was not performed because of multiple surrounding arteries. Relaparotomy with an upper midline incision was then performed. Sudden cardiac arrest occurred upon opening the lesser sac to irrigate clots, and cardiac massage and proximal and distal clamping of the SAA were required. Eventually, splenectomy with excision of the SAA and pancreatic tail was successfully performed, but gauze packing of the open surgical wound was required because of severe coagulopathy. Following removal of the packs and closure of the abdomen 2 days after splenectomy, the patient and infant satisfactorily recovered without sequelae.Given continual awareness of abdominal vascular collapse during pregnancy, undelayed diagnosis and safer intervention might be achieved.Awareness at initial presentation and multidisciplinary efforts might be essential to achieve maternal and fetal survival in SAA rupture during pregnancy. The preHowever, lethal complications of SAA rarely develop during pregnancy. A nationwide study in the United States showed that the estimated maternal mortality rate was 23.8 per 100,000 live births . AlthougAlthough SAA rupture is rare, its prompt consideration as a differential diagnosis when encountering acute situations in pregnancy may increase the chance of a successful outcome. We herein present a case of SAA rupture in a pregnant patient with achievement of maternal and fetal survival through the efforts of a multidisciplinary team. This case is reported to promote awareness regarding SAA rupture as a noteworthy complication during pregnancy. This case has been reported in line with the SCARE criteria .2A 40-year-old woman  at 35 weeks’ gestation with a medical history of mild hypertension and no family history was admitted to the hospital with the principal complaint of upper abdominal pain and nausea. Previous pregnancy course had been uneventful, and recent conducted treatment and/or prescription, which could lead to reduced fetal heart rate, were absent. Since pallor of the face and coldness of limbs were observed despite of lack of apparent laboratory anemia (Hemoglobin 12.0 g/dL) and hypotension (blood pressure 99/74 mmHg), bleeding problem in her abdomen was suspected. Bedside ultrasound demonstrated a low fetal heart rate (<60 beats/min). Therefore, emergency cesarean section with a Pfannenstiel incision was performed 20 min after arrival based on a provisional diagnosis of uterine rupture or placental abruption despite the absence of vaginal bleeding. Following rapid delivery of a female infant weighing 2,002 g and with an Apgar score of 3 at 1 min and 5 at 5 min, 400 g of hemorrhage was found in the upper abdominal cavity; however, a gynecological origin of the hemorrhage was not found. After closure of the uterus and performance of intraperitoneal irrigation, the abdomen was closed when the bleeding had ceased. No fresh bleeding was seen through the lower abdominal incision.For further evaluation of the intra-abdominal hemorrhage, enhanced abdominal computed tomography was promptly performed. This revealed an SAA of 37 mm in diameter located near the splenic hilum, associated with massive adjacent fluid collection a. While About 6 h after arrival, the patient underwent relaparotomy with an upper midline incision. During irrigation of the space in the lesser sac, which was full of blood, sudden cardiac arrest occurred. After approximately 30 min of cardiac massage and proximal and distal clamping of the SAA, sinus tachycardia was achieved. Splenectomy with excision of the aneurysm was then performed. Because pancreatic injury occurred at the time of clamping, pancreatectomy at the small part of the tail was performed with staple closure a. AdditiThe patient and her daughter were eventually discharged in good condition with no neurological sequelae on postoperative day 38 and 41, respectively. Maternal recovery had been relatively complicated by a Clavien–Dindo grade IIIa pancreatic fistula. Their ambulant follows have been uneventful.This study was carried out in accordance with the principles of the Declaration of Helsinki, and informed consent for publication was obtained from the patient.3We have herein described a case of SAA rupture during pregnancy in which maternal and fetal survival without chronic sequelae was eventually achieved by multidisciplinary interventions. Because of lack of awareness of the SAA rupture at the initial visit, definitive treatment (splenectomy and excision of the SAA) was performed approximately 6 h after arrival, which is less than ideal. If we had accurately born in mind abdominal vascular collapse during pregnancy, an earlier diagnosis of SAA rupture might have been achieved based on a more meticulous exploration at the time of caesarian section. Some obstetricians have suggested that a midline incision rather than a Pfannenstiel incision should be primarily selected for caesarian section when intraperitoneal hemorrhage is suspected .In the current case, although the patient maintained relatively stable vital signs for several hours (from caesarian section to the second laparotomy), sudden collapse occurred during irrigation of the lesser sac. This phenomenon has been described as “two-stage rupture,” meaning that the initial rupture is contained within the lesser sac for a variable number of hours before it spreads into the greater sac, leading to massive intraperitoneal hemorrhage . BecauseAlthough the incidence of SAA rupture during pregnancy is reportedly low, as mentioned above , cases oThe necessity of screening for SAA in selective gravidas has been discussed because of the dismal mortality rate associated with rupture . However4Because of its rarity compared with several other obstetric complications, SAA rupture during pregnancy can be easily overlooked at the initial visit, which might result in a subsequently life-threatening condition. Earlier awareness of SAA rupture during pregnancy, even with a vague presentation, may further contribute to maternal and fetal survival based on successful multidisciplinary team efforts.The authors report no declarations of interest.No grant support or other funding was received.This study was carried out in accordance with the principles of the Declaration of Helsinki, and informed consent for publication was obtained from the patient.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.MF, AF, SY (Yanai), and JT performed the actual treatments. MF drafted the manuscript, and SY (Yamashita) revised the manuscript. All authors read and approved the final manuscript.Not applicable.Manato Fujii.Not commissioned, externally peer-reviewed.


Dunbar syndrome or median arcuate ligament syndrome is a rare disorder. In this disorder, a malposition of the arcuate ligament compresses the celiac trunk and causes nonspecific symptoms including postprandial pain, abdominal bruit and weight loss. Surgical management is the primary treatment.Pancreatic neuroendocrine tumors (PNETS) are also rare. It comprises about 1–3% of pancreatic neoplasm. The patient could be symptomatic or asymptomatic depends on the tumor being functional or nonfunctional. In addition, surgical therapy is the choice.In this paper, we report a case of 28 old female patient complaining from a long term of recurrent abdominal pain that doesn't releive on any kind of treatment, the multislices computerised tomography scan showed compress of the median arcuate ligament with an incidental mass in the tail of pancreas turned out to be a pancreatic neuroendocrine tumor.The Dunbar syndrome and the Pancreatic neuroendocrine tumors must be kept in mind of phyciciens while the differential diagnosis of any recurrent abdominal pain. •Dunbar syndrome or median arcuate ligament syndrome is a rare disorder which presented with abdominal pain.•Celiac artery compression can be diagnosed with Multi slices computer tomography and selective catheter angiography, surgery is the treatment of choice.•The Dunbar syndrome and the Pancreatic neuroendocrine tumors must be kept in mind during differential diagnosis of recurrent abdominal pain. It comprises only about 1–3% of pancreatic neoplasms . PNETs cSome studies report the coexistence of Dunbar syndrome with pancreatic carcinoma  with pre1.1A 28-year old female presented in our center suffering from a long term of recurrent abdominal pain. The chronic recurrent postprandial pain started six years ago.The daily pain localized in the epigastric region, the pain wakes her up in the night and she had no complete relieve on analgesics. No nausea, vomiting or weight loss were recorded. Moreover, no diarrhea or constipation were detected. No previous history for medical or family conditions. Only She had an appendectomy ten years ago and two cesareans, and cholecystectomy five years ago.Three upper endoscopies showed normal findings with almost six months between one and another. She has a previous diagnosis of irritable bowel syndrome to explain her recurrent abdominal pain.At admission, abdominal examination, laboratory tests and ultrasound were within normal limits. Multi slice computed tomography (MsCT) revealed a 3.5 cm heterogeneous mass in the tail of pancreas with stenosis up to 40% in the celiac trunk with 45° of angulation .Fig. 1A-The diagnosed of DS was probable, and a surgical intervention was proposed to resolve the celiac trunk compression and to resect the pancreatic tumor. Abdominal exploration was performed via midline incision; a 3.5 cm mass in the tail of the pancreas was detected with no metastasis or ascites. Distal pancreatectomy with splenectomy was performed. Liberation of the celiac trunk was accomplished by cutting the median arcuate ligament .Fig. 2A-The patients discharged from the hospital three days after surgery in good situation. Follow up after showed a complete relieve for her pain.The pathology result showed neuroendocrine tumor in the tail of pancreas.A well-demarcated mass in the pancreatic tail, measures 3cm in the greatest dimension, composed of proliferation of sheets and nests of medium and large cells with eosinophilic and finely granular cytoplasm, nuclei are round with salt and pepper chromatin. Atypical and bizarre cell present with prominent mitotic figures and tumor necrosis. Rich vascular network in the stroma, with fibrotic areas.Immunohistochemistry (IHC) revealed that neuron specific enolase (NSE), synaptophysin and CD56 were positive (diffuse), negative chromogranin, focal cytokeratins (CK) and the KI67 was high index (20%). Finally, Diagnosis is a well-differentiated neuroendocrine tumor, Grade 3 .Fig. 3Im2MALS or called Dunbar syndrome is a rare reason for abdominal pain with nonspecific symptoms. It was first described in 1963 by Harjola et al. .Symptoms can be explained by two theories, the first is the malposition of the median arcuate ligament, which compresses the celiac trunk and causes mesenteric ischemia. The second is the direct irritation of the celiac ganglion or compression of the ganglion plexus lead to painful neuro stimulation ,3.Nonspecific symptoms and vague pain makes delay the diagnose .MALS or celiac artery compression can be diagnosed with Doppler ultrasound, Multi slices computer tomography, selective catheter angiography, and magnetic resonance angiography (MRA) . Pre- anIn our case the our diagnosis was made by computed tomography angiography (CTA), and no collateral circulation was observed, the patients with symptomatic celiac axis stenosis often have multiple vessel obstruction or severe stenosis. When the collateral circulation is poor, that is Gastroduodenal artery hypoplasia, the patients with single celiac axis stenosis cloud have postprandial abdominal pain.Surgical decompression of the celiac trunk and celiac plexuses is the treatment of choice, it can be done by either open or laparoscopic approach .Neuroendocrine tumors are also rare tumors, which can be located in different organs as gastrointestinal tract, pancreas and ovaries .Pancreatic neuroendocrine tumors (PNETs) comprises about 1–3% of all pancreatic neoplasms .PNETs can be silent or nonfunctional, contrarily to insulinomas, glucagonomas, VIPomas, and somatostatinomas, and other rare types, which caused symptoms according to the hormone secretion .PNETs can be detected by computer tomography (CT), magnetic resonance imaging (MRI), endoscopic ultrasonography (EUS), and somatostatin receptor scintigraphy (SRS) which is recommended in most guidelines .Surgical therapy for Dunbar syndrome or MALS can be varied from transaction of median arcuate ligament, destruction of the celiac ganglion or revascularization of the decompressed celiac truck .In our patient the pain was chronic and recurrent daily non-relieving despite of all treatments. Surgical decompression of the median arcuate ligament relieved her symptoms after surgery.Moreover, our patient had a neuroendocrine tumor in the tail of pancreas, which makes the question could her pain caused by the mass and MALS syndrome discovered incidentally or the pain caused by MALS and the mass detected incidentally.There is an increasing number of incidentally discovered PNETs on abdominal imaging performed for other reasons .PENTs can be associated with other conditions like Multiple Neoplasia Type 1 (MEN1), Von Hippel Lindau syndrome and Neurofibromatosis Type 1 .Yasuo Shima et al. and Jen-Wei Chou et al. described tumors in the head of pancreas with MALS. However, no other articles documented MALS with neoplasm in the tail of pancreas ,18.To our knowledge, this is the first documented MALS syndrome with neoplasm of tail of pancreas.For locoregional PNETs, surgical resection is the treatment. Functional PENTs give symptoms and help to discover the tumor in early stages.On the other hand, nonfunctional tumors are being locally advanced when it has been diagnosed.Most PENTs are malignant except for insulinomas, and oncologic principles should be followed during the resection .Our patient had incidentally discovered the mass during the MSCT performed to detect any compression on the celiac artery, And with one surgery relieved her pain by removing the compression on celiac artery and distal Pancreatectomy with splenectomy accomplished to remove the mass.3Although MALS are rare, we should keep in mind Dunbar syndrome or MALS as a differential diagnosis in any patient with abdominal recurrent pain.As to PNETs still incidentally detected and mysterious as long they are nonfunctional tumors.4Ethical Approval upon data access and consent to participate was given from the directory of surgical department in Al-assad university hospital.5Written informed consent was obtained from the patient for publication of this case report and any accompanying images.6All data are available.The authors declare that they have no competing interests.No funding was received in support of this work.The operation was done by RE and BA. The literature search was done by FA and MA. FA, MA, and BA wrote the manuscript. RE revised the manuscript.Not commissioned, externally peer reviewed.The authors declare that there are no conflicts of interest regarding the publication of this paper.