Sliding hernia occurs when the hernia sac is partially formed by the wall of a viscus. The most common components of a sliding hernia includes the sigmoid colon, cecum, appendix, urinary bladder, and the ascending colon. However, the presence of bilateral vesical sliding hernia is rare and few cases have been reported in the literature. Patients with vesical sliding hernia may present with groin swelling with an associated lower urinary tract symptom. Computed tomography (CT) scan is the modality of choice for suspected cases which may reveal the classic pelvic micky mouse sign. The method of repair should be individualized taking in account the diagnostic findings. Although laparoscopic repair is becoming the mainstay management for inguinal hernia, the majority of sliding hernias are repaired using an open approach, which could be attributed to the presence of large hernias, associated complications, or recurrence with associated adhesions. We present a case of a 60-year-old male patient presented with bilateral inguinal swelling associated with urinary hesitancy and intermittency. He had undergone transabdominal preperitoneal (TAPP) repair for a left inguinal hernia 8 years ago. CT scan confirmed the presence of a bilateral hernia with the bladder herniating bilaterally. He underwent an elective bilateral open Lichtenstein tension-free mesh repair. •Bilateral vesical hernia has been rarely reported in the literature.•CT scan is the modality of choice for the diagnosis, which may reveal Micky mouse sign on an axial view.•Laparoscopic and open repair have been reported for such cases.•An open approach should be considered in large, complicated hernias, and those with extensive adhesions. Sliding1.1A healthy 60-year-old male not known to have any chronic medical illnesses. presented to general surgery clinic complaining of bilateral groin painless swelling, for the past 2 years. In addition, he had a history of urinary hesitancy and intermittency developed in the past few months prior to presentation. He had no other gastrointestinal or lower urinary tract symptoms. He denied any history of heavy weight lifting. He has no history of smoking. He had undergone a left transabdominal preperitoneal (TAPP) inguinal hernia repair 8 years back in another hospital. The patient is married, and works as lawyer. He is not taking any medication, and he has an unremarkable family history. Upon physical examination, the patient was well built and well nourished. He weights 78 kg, and he is 180 cm tall (BMI 24.1 kg/m2). Local examination revealed a bilateral inguinal swelling that bulged upon Valsalva maneuver. The swelling was more prominent on the left side with no evidence of scrotal extension. The urinary bladder wasn't palpable. His blood test results showed a normal complete blood count, and kidney function. Abdominal Computed tomography (CT) with contrast was obtained which showed a bilateral inguinal hernia, both containing part of the urinary bladder more evident on the left side (Pelvic micky mouse sign) , Fig. 2,2Sliding bladder hernia is a rare type of hernia in which the wall of the bladder constitutes a part of the hernia sac. Although sliding hernia are often complicated on presentation, sliding bladder hernia are usually asymptomatic. In review of the literature, it was found that Less than 7% of bladder hernias are diagnosed preoperatively, 16% are diagnosed postoperatively owing to the associated postoperative complications, and the reminding cases are diagnosed perioperatively [A systemic review performed in 2017 showed that most of the patients with vesical sliding inguinal hernia were males, and 76% of them were symptomatic. The symptoms were 60% inguinal swelling, 40% pain, 12.7% reduction of an inguinal mass after voiding, and 48% lower urinary tract symptoms (LUTS) which could be a voiding or storage symptoms. However, it is difficult to explain the significance of LUTS in these cases and whether it is attributed to an entrapment of herniated bladder or an underling disease like bladder outlet obstruction . ComplicPreoperative diagnosis of sliding bladder hernia is essential. It allows for better surgical planning and may decrease the risk of iatrogenic injury . GomellaMany different imaging modalities have been used for the diagnosis of sliding bladder hernia. Moreover, CT scan with or without contrast is the most frequent modality used . It is hVarious approaches have been advocated in the management of unilateral inguinal vesical hernia including open, laparoscopic, and robotic surgery. In the current literature, open surgery was the most common surgical approach adopted, performed in 80.4% of cases . Though 3Vesical sliding inguinal hernia is a rare entity. Moreover, bilateral sliding vesical hernia has been scarcely reported in the literature. Patients with this pathology may present with an inguinal swelling associated with lower urinary tract symptoms. US and CT scan is the modality of choice for preoperative diagnosis in patient with an inguinal hernia, when a bladder involvement is suspected. Bilateral open Lichtenstein tension-free mesh repair is safe and effective option for such cases. However, the method of repair should be tailored for each individual case, taking in account careful analysis of clinical presentation, diagnostic findings, and the patient's surgical history.The authors declare that there is no conflict of interest regarding the publication of this paper.Not commissioned, externally peer reviewed.This case report is exempt from ethical approval by our institution.This case report had no funding or sponsors.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.NM wrote the manuscript, reviewed the literature, and assisted in the surgery. MN drafted the manuscript. KA participated in data acquisition and reviewed the literature. AM contributed to the conception and the design of the study, reviewed the manuscript, and performed the surgery. HE supervised the management of the patient and reviewed the manuscript. All authors read and approved the final manuscript.The authors declare that there is no conflict of interest regarding the publication of this paper.None.Dr. Ahmed Mohammed Al. Muhsin


Hyperplastic polyps are the most frequent benign epithelial gastric polyps. Although they are considered nonneoplastic, some cases have been reported with focal adenocarcinoma. We present the case of a 59-year-old woman with a sessile lesion of 15 mm on the distal gastric body associated with an extensive atrophic gastritis. Magnifying endoscopy with Fuji Intelligent Color Enhancement (FICE) revealed an irregular microsurface pattern at the apex, suggesting malignancy. A mucosectomy was performed. The histopathology revealed that the base corresponded to a hyperplastic polyp, where a tubular adenoma with high-grade dysplasia was established, with focal well-differentiated intramucosal tubular adenocarcinoma. Hyperplastic polyps (HP) are the most common nonneoplastic polyps in the stomach . AlthougA case of gastric HP with focal adenocarcinoma is presented.A 59-year-old woman with epigastralgia was evaluated in gastroenterology and an upper digestive endoscopy was indicated. During the endoscopic examination a gastric polyp was found and the patient was referred to our hospital for treatment.Endoscopic examination of the upper digestive tract revealed extensive atrophic gastritis and a sessile lesion of 15 mm of reddish coloration in the distal gastric body . Magnify Helicobacter pylori infection.The histopathological evaluation of the resected polyp revealed that the base corresponded to a hyperplastic polyp, in which a tubular adenoma with high-grade dysplasia was established with focal well-differentiated intramucosal tubular adenocarcinoma . The lesHP are inflammatory proliferation of gastric foveolar cells, which produce mucin and cover the gastric surface . HP are  Helicobacter pylori infection), which develops on a reparative phenomenon in the form of foveolar hyperplasia; this hyperplastic tissue may disappear (24%) or persist, progressing to the formation of a hyperplastic polyp [The etiology of these polyps is still not well defined, but it has been suggested that there may be an inflammatory process (for example, due to autoimmune gastritis oric polyp .Previously, HP are commonly considered to be insignificant in terms of potential malignant conversion; however, some cases have been reported in which the hyperplastic foveal epithelium may develop to intestinal metaplasia, dysplasia, or carcinoma; this malignant transformation can occur spontaneously through the sequence hyperplasia-dysplasia-adenocarcinoma , 10.On EGD, HP are seen as sessile or pedunculated elevated lesions. They can be unique or multiple, being located preferentially in the gastric antrum, but they may appear in any part of the stomach . Most ofMagnifying endoscopy with FICE allows us to evaluate the surface structures and vascular structures of the mucosa. The margins of the cancer, in particular in case of differentiated carcinomas, are diagnosed on the basis of an irregular microsurface . HistoloThe incidence of malignant changes is relatively low, appearing mainly in those that measure more than 1 cm, being found focal intestinal metaplasia, dysplasia, and adenocarcinoma in 5-37%, 2-20%, and 2-6% of cases, respectively –14. Carc H. pylori, autoimmune gastritis and other pathologies [It is suggested to resect HP if they are symptomatic, if they present atypical characteristics, or if they are greater than 1 cm, since they have a greater potential for malignant transformation . When HPhologies . Helicobacter pylori eradication is recommended if infection is found.In our review, there are no similar cases reported in our country and none in the literature that have endoscopic images with magnifying endoscopy with FICE. The present case demonstrates that some HPs may be associated with the presence of adenocarcinoma. Magnifying endoscopy with FICE may be helpful for this diagnosis. It is important that HP of more than 1 cm or symptomatic are resected. Endoscopic treatment of HP with adenocarcinoma is considered sufficient if the criteria for endoscopic and histological cure are confirmed , 15. The


Colorectal carcinoma is a rare but potentially fatal disease complicating pregnancy. It occurs most frequently in patients aged 50, although some studies report increasing incidence in women under the age of 40. Diagnosis of colorectal cancer during pregnancy is usually made at an advanced stage due to unspecific symptoms. We will present a case of an acute abdomen during pregnancy due to colorectal carcinoma perforation in a 33-year-old patient in her 26th week of gestation. Because of her abdominal condition, left hemicolectomy with colostomy was performed. Two hours after surgery, the patient gave birth to a male child weighing 910 g with an Apgar score of 2/6. The pathohistological finding indicated adenocarcinoma of the colon in Dukes stage B. Colorectal carcinoma (CC) is a rare but potentially fatal disease complicating pregnancy. The incidence is highest in patients aged 50 (the range is between 40 and 74), although some studies report that it is increasingly occurring in women under the age of 40 . Since tnd week of gestation, immediately after she felt sudden abdominal pain. Her pregnancy had been developing normally, apart from hemorrhoids and a single occurrence of blood coated stool. Her genital findings were normal. Laboratory tests showed signs of anemia . The ultrasound examination revealed no abnormalities. The consulted surgeon ruled out acute abdomen. Stool which was tested for occult bleeding was negative. Repeated laboratory tests showed signs of anemia with a slight CRP increase . Internal medicine specialist was consulted. Because of sideropenic anemia, iron supplements were prescribed. In her 26th week of gestation, the patient was admitted again with severe abdominal pain and signs of acute abdomen. The cervix was shortened and the 2 centimeters were dilated. As the CTG showed contractions, tocolytic drug was administered intravenously. Laboratory findings showed a decrease in haemoglobin and an increase in CRP .The patient P. A., aged 33, G3P2, was admitted to the hospital in her 22Due to the fact that lab findings were suggestive of infection, antibiotic treatment was started (cefuroxime and gentamicin). The abdominal ultrasound showed a relatively small quantity of free intraperitoneal liquid. In the lienal flexure area an eccentric thickening of the intestinal wall was visible which indicated a possible expansive infiltrative process. An X-ray of the abdomen was taken which showed a narrow transparency below the right hemidiaphragm, possibly free air from an earlier encapsulated perforation. Acute abdomen caused by diffuse peritonitis as a consequence of a perforation of the descending colon tumor was diagnosed and the patient transferred to surgery where laparotomy, left-side hemicolectomy, and colostomy were performed. Two hours after the operation and in spite of the intravenously administered tocolytic medication the patient gave birth to a male child weighing 910 g with an Apgar score of 2/6. On the eighth day after surgery the patient was discharged after ablactation. Histologically, the placenta as well as the amniotic sac and the umbilical cord displayed no pathological alterations. Histopathology report is as follows: the material is a 30 cm long colon segment. A tumor of 3 cm in diameter is located 3 cm from the aboral resection edge filling the entire circumference of the intestinal wall, infiltrating its entire width and spreading into fat tissue. In the underlying fat there are eight normal-looking lymph nodes 0.3-07 mm in diameter. Adenocarcinoma colonis et Peritonitis acuta suppurativa .th week of gestation which manifested by abdominal pain and peritonitis [The first case of rectal cancer in a pregnant woman was reported by Cruveilhier in 1842 . CC is aitonitis . Howeveritonitis . Rowan Titonitis . The unditonitis .Discussions about the aetiology of colorectal carcinoma further mention diverse growth factors (IGF-1i VEGF), the pregnant women's immune system, and genetic predisposition , 10. GenA research including 119 pregnant women with colon carcinoma showed that in 47% of them the most frequent symptom was bleeding; 37,6% suffered from abdominal pain and 14% from obstipation, and in 2,4% a perforation occurred . SeveralCC during pregnancy is diagnosed by ultrasound, X-ray, or MRI of the abdomen . Increasth week of pregnancy, surgery may be performed and the pregnancy continued. Beyond that point, the 32nd week can be awaited so that fetal maturity is reached and then a Caesarean section, if indicated, and radical surgery may be performed [Surgery is the gold standard. If a patient is diagnosed with colorectal carcinoma in early pregnancy an abortion is advised . Up to terformed . Upon vaerformed .th week of pregnancy, ovarian biopsy is not recommended on account of the increased abortion and preterm birth rate [Pregnant women suffering from colorectal carcinoma are prone to metastases to the ovary with a documented incidence of 25% and they generally have a less favourable prognosis. During surgery both ovaries should be subject to biopsy and bilateral adnexectomy should also be considered . Before rth rate .Colon carcinoma during pregnancy has no negative influence on the fetus. Metastases in the placenta were described in only one case of colorectal carcinoma. The main problem is a higher incidence of preterm births . PregnanCC is mostly discovered in Dukes B stage 77%) and in C stage (33%) 7% and in. CC of tColorectal cancer during pregnancy is a rare but aggressive malignancy with a poor prognosis. Because presentation can overlap with the signs and symptoms of pregnancy, diagnoses are often a challenge. Diagnosis of colorectal cancer during pregnancy is usually made at an advanced stage due to unspecific symptoms. An interdisciplinary approach between a gynecologist and a general surgeon is required. In our department in the last 23 years at 47,000 births, one case was diagnosed.


Introduction: Nerve sheath tumors are tumors arising from nerve sheaths or which show nerve sheath differentiation. They are divided as benign and malignant. They are associated with Von Recklinghausen syndrome. Characterised by café-au-lait spots, lisch nodules, acoustic neuromas, pheochromocytomas.Presentation of case: A 62 year old gentleman came with complaints of Abdominal discomfort for 1month. He also had Left sided abdominal pain, Anorexia, Vomiting, No history of dyspepsia, fever, bowel disturbance. Patient is a known case of diabetes mellitus II. On abdominal examination a large mass of 10*8 cms was palpated in the left hypochondrium, left lumbar, and umbilical region. It had restricted mobility, not moving with respiration, firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe.Contrast enhanced computed tomography showed large lobulated heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium, in the region of distal body and tail of pancreas. There were no other foci of metastasis in abdomen or chest. Chest X-ray of patient was normal. Ultrasound of neck revealed a suspicious nodule in right lobe of thyroid measuring 1*1 cms with no nodal enlargement. Fnac of thyroid nodule was done under image guidance which was suggestive of papillary carcinoma of thyroid. As image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. The tumor was resected en bloc - distal pancreatectomy and splenectomy. Post opereative biopsy showed irregular spindle shaped cells with nuclei exhibiting “a wavy, buckled appearance” malignant peripheral nerve sheath tumor. Patient was reviewed after a period of 2 months. Positron emission tomography was done which revealed para aortic nodes and 2 mesenteric nodes. It also revealed a metabolically active nodule in thyroid with cervical nodal metastasis. Patient was taken up for total thyroidectomy with functional neck dissection. Histopathology confirmed papillary carcinoma of thyroid with positive lymph nodes. Patient was advised to undergo a radioactive iodine scan showed 0.3% uptake. Patient was started on chemotherapy for para aortic nodes with a regimen of paclitaxel, adriamycin, ifosumide and mesma.Discussion: Pancreatic schwannoma arise from sympathetic and Para sympathetic fibers that cross over pancreas. Patients Present with abdominal pain and weight loss commonly. Macroscopically they are well circumscribed, encapsulated lesions, homogenous lesions. Microscopically they show Antoni A & B areas. They demonstrate S100, vimentin, CD56 positivity. Most common differential diagnosis are cystic mucinous / serous neoplasms of pancreas. Malignant transformation is extremely rare with only 8 reported cases till now. Treatment is simple enucleation, but a accurate pre-operative diagnosis is difficult to make hence a oncollogically margin negative resection is done. Adjuvant radiotherapy can be given to reduce the tumor load. Chemotherapy can be given with ifosumide and doxorubicin.Conclusion: Malignant pancreatic nerve sheath tumours are very rare to be found, but have very poor prognosis, hence in cases of pancreatic tumour its better to keep the diagnosis of nerve sheath tumour also a differential diagnosis. In conclusion pancreatic tumours are to be identified at the earliest and surgery should be done for those cases He had decreased appetite, projectile vomiting, no dyspepsia, no history of fever and bowel habits were normal. On examination, a large mass of 10 × 8 cm was palpated in the left hypochondrium, left lumbar, and umblical region. It was mobile, not moving with respiration and firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe. Upper and lower gastrointestinal endoscopy was normal.Contrast enhanced computed tomography (cect) abdomen showed large lobulated, heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium in the region of distal body and tail of pancreas . There wrdpost operative day drain tube was removed on 5thpost operative day. Patient was discharged on the 12thpost operative day.As image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. At laparotomy, patient was found to have a bilobed tumor arising from the lesser sac adherent to the pancreas and abutting the stomach, transverse colon, and left adrenal and splenic hilum . The tumThe histopathology showed the presence of very irregularly shaped cells with nuclei that exhibited a “wavy, buckled appearance”. Multiple sections studied showed pancreatic tissue with adjacent fairly circumscribed neoplasm composed of spindle cells arranged in sheets and fascicles. Pancreatic margin appeared free from tumour. There are areas showing alveolar and glandular patterns.There are areas showing hyalinization, necrosis and fibrosis. Tissue organization shows great variability, with hypocellular myxoid areas and areas of major cellularity diagnostic of Malignant peripheral nerve sheath tumour (MPNST). The histologic changes of MPNST include spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci .The tumo2de novo or develop from the malignant transformation of a benign neural neoplasm, generally a plexiform neurofibroma. Approximately half of MPNST cases occur in association with neurofibromatosis type 1 (NF1).Solitary (unassociated with NF1) and localized  neurofibromas do not have malignant transformation potential. MPNST originating from the bone, soft tissues, heart, mouth, esophagus, bronchus, retroperitoneum, uterine cervix, orbit, parotid gland, as well as the spinal cord, acoustic nerve, cerebellum, and sympathetic chain, have been reported.Neural tumours are schwannomas and neurofibromas  They areTP53 mutations have been found in a subgroup of MPNSTs, indicating that a p53-mediated pathway is involved in their development [Schwanommas and neurofibroma have been reported to occur on the pancreas infrequently . Malignaelopment .MPNST is typically associated with a poor outcome compared with those of other soft tissue sarcomas. The recurrence rate is as high as 40%, and the most common metastatic sites are the lungs and the bone. The 5-year survival rate ranges from 30 to 50%. The negative prognostic factors for MPNST are similar to those for other soft tissue sarcomas and include the tumor site , a large tumor size, high-grade, and positive surgical margins. Some reports suggested that NF1 patients with MPNST experience poorer survival than do sporadic MPNST patients although this has not been a consistent finding . CompletWritten 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.No conflict of interests.No sources, self funding.The case report is exempted from ethical committee.Consent was obtained from patient for use of clinical information for academic and Educational purposes. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Study conception and design: Dr. Rekha Arcot ; Dr.Khalilur rahman ; Dr. Bharath Narayansamy.Acquisition of data: Dr.Pradeep Balineni ; Dr. Manoj Prabhu K.R ;Dr. Shantini Vaitheeswaran.Analysis and interpretation of data: Dr. Kannan Devydounder.Drafting of manuscript: Dr. Rekha Arcot ; Dr.Pradeep Balineni ; Dr. Bharath Narayansamy.Critical revision: Dr.Pradeep Balineni ; Dr. Rekha Arcot ; Dr. Manoj Prabhu K.R.Not applicable.Dr. Pradeep Balineni.Dr. Rekha Arcot.Not commissioned, externally peer-reviewed.


Inflammatory pseudotumours of the lungs have rarely been reported. These have been described as a benign entity of unknown origin and are often locally invasive requiring extensive pulmonary resection. Complete resection is the key to prevent recurrence and the prognosis is excellent following surgery. We describe a patient with inflammatory pseudotumour of the lung. He was a middle aged man who presented with haemotysis and the chest X-ray and computerized tomography were indicative of a nonbenign lesion in the right upper lobe. Excision biopsy confirmed the diagnosis of inflammatory myofibroblastic pseudotumour of the lung. This is a rare inflammatory nonneoplastic condition commonly affecting children and young adults. •The inflammatory pseudotumor of the lung is a rare entity.•Surgical excision is usually indicated to reach a firm diagnosis and cure.•As preoperative investigation is not diagnostic, excision of the mass is imperative in order to exclude malignancy.•Despite being a benign lesion, its potential for recurrence and local invasion requires complete surgical resection.•Complete resection, when possible, is safe and leads to excellent survival and remains the key to prevent recurrence. First1.19/L. The other serum haematological and biochemical results were normal. In view of the patient's ongoing haemoptysis and lack of response to antibiotics he underwent bronchoscopy which revealed a growth in right upper lobe with endobronchial obstruction. At the same time endobronchial biopsy was taken which was sent for histopathological examination. Microscopically, the biopsy showed a heavy inflammatory cell infiltrate composed predominantly of lymphocytes, with plasma cells and histiocytes. Foamy histiocytes with macrophages were also seen, as well as occasional eosinophils and neutrophils. Focal areas of micro-abscess formation with necrosis were also noted. A marked degree of fibrosis was present with proliferating myofibroblasts. The histological characteristics were compatible with an inflammatory myofibroblastic pseudotumour (A 40-years-old male non-smoker presented to chest outpatient department with complaints of recurrent mild haemoptysis for 2 months, which was progressive in nature. Chest radiograph revealed a 2.5 × 2 cm lesion in the posterior segment of the right upper lobe of the lung . The meddotumour . On immudotumour . Surgery2Inflammatory pseudotumors of the lung are rare and were first described in the lung in 1939 . AlthougThe aetiology and pathogenesis remain uncertain. There are several theories, most of which postulate an exaggerated immunologic response to a viral or foreign antigen antibody reaction . They tyThe neoplastic subset of IMT usually shows evidence of clonal cytogenetic abnormalities involving 2p23 that encodes ALK gene, and typically occurs in children and young adults . Those tMany patients are asymptomatic, with inflammatory pseudotumor discovered incidentally on chest radiograph . If the Grossly, IMFT are well circumscribed, non-encapsulated, firm, usually yellow-white masses containing variable inflammation, haemorrhage, calcification, and rarely cavitation. Most are parenchymal but some are endobronchial and may cause airway obstruction. Less than 5% invade the mediastinum and/or chest wall. Local recurrence is attributed to incomplete resection of the primary lesion. Metastasis of the tumor to mediastinum or the brain even many years after complete resection has been described. Rarely, simultaneous intra- and extrathoracic locations may occur.Microscopically, the lesions consist of variable mixtures of fibroblasts and granulation tissue, fibrous tissue, and inflammatory cells including lymphocytes, histiocytes, giant cells, macrophages, neutrophils, eosinophils, and typically large numbers of plasma cells ,3,4. TheComplete surgical resection remains the best treatment, to exclude malignancy and to achieve cure ,17. Wedg3Although inflammatory pseudotumours of the lungs are rare and the most common clinical picture is one of an asymptomatic, well-circumscribed lung mass that mimics cancer, clinicians need to bear in mind the diverse clinical presentations. Surgical excision is usually indicated to reach a firm diagnosis and cure. As preoperative investigation is not diagnostic, excision of the mass is imperative in order to exclude malignancy. Despite being a benign lesion, its potential for recurrence and local invasion requires complete surgical resection. Complete resection, when possible, is safe and leads to excellent survival and remains the key to prevent recurrence.Written informed consent was obtained from the patient for publication of this case series and any accompanying images.We declare that there are no conflicts of interest amongst the authors.We also state that there was no source of funding for this study.Not commissioned, externally peer reviewed.There is no conflict of interest.There is no source of funding.Not applicable.Not applicable.NA.Nisha Marwah-Designed the study.Namita Bhutani-Collected the data and wrote the paper.Sakshi Dahiya-Helped in data collection.Rajeev Sen-Analysed the data.Dr Pradeep Kajal.


We briefly review the diagnosis and management of ONSM, and review the literature on the role and current status of nuclear imaging with somatostatin receptor ligands in the non-invasive diagnosis and management of meningiomas.We hereby report the case of a patient with optic nerve sheath meningioma (ONSM), whose diagnosis and multidisciplinary management was guided by the use of Gallium-68 ( Orbital space-occupying lesions include a wide variety of benign and malignant diseases, including orbital inflammatory pseudotumor, optic nerve sheath meningioma (ONSM), orbital metastases, orbital lymphoma, and optic pathway gliomas . ONSMs r68Ga)-labeled dodecanetetraacetic acid-tyrosine-3-octreotate (DOTATATE) positron emission tomography (PET)/CT scan for non-invasive diagnosis of an orbital space-occupying tumor.This is one of the few reported cases on the use of Gallium-68  of 10.8. Portions of the lesion showed increased attenuation on non-contrast CT, suggesting calcification. There was a nearby but separate focus of activity more superoposteriorly, which localized to the left aspect of the planum sphenoidale  to a total dose of 50.4 Gray (Gy) in 28 fractions was delivered. A VMAT radiation plan Figure  was chosMRI and CT are currently the most commonly used imaging modalities in the workup of orbital space-occupying lesions . In a stIn certain instances, as in our case report, MRI and CT scan may not provide sufficient diagnostic information to differentiate ONSM from other optic tumors, such as optic pathway gliomas. An open orbital biopsy can provide definitive pathological diagnosis, but this procedure is associated with significant risks, including postoperative diplopia, orbital hemorrhage, and symblepharon . Albeit Meningioma cells have been shown to strongly express somatostatin receptor subtype 2 (SSTR2) , unlike 111Indium (In)-octreotide scintigraphy (OctreoscanTM) to differentiate meningiomas from other dural-based tumors, increasing the diagnostic accuracy of MRI  and positive predictive values (89 vs. 84%) were, however, similar between the two imaging modalities  from other entities , as meningiomas demonstrated high 68Ga-DOTATATE uptake (mean SUVmax = 14.3 ± 15.4), whereas the other three lesions did not (SUVmax = 2.1 ± 1.0)  and specificity (100%) for 1 ± 1.0) . The sam68Ga-DOTATATE PET/CT has shown the potential to predict tumor growth rate. Higher expression of SSTR2 resulting in higher SUVmax on 68Ga-DOTATATE PET imaging has been shown to predict for faster growth rates in World Health Organization (WHO) grade I and II meningiomas (Despite the fact that ONSM are indolent benign tumors, they can eventually lead to proptosis, progressive loss of visual acuity, and if left untreated, to blindness , 21. Theingiomas . As such68Ga-DOTATOC PET to serve as a complementary modality to MRI (Treatment options include surgery in rare instances, and radiation therapy in most scenarios. Because of significant functional morbidity, surgery for ONSM has fallen out of favor, except in cases of rapid visual decline in which quick surgical decompression is needed, or in case of intracranial extension , 27. Stey to MRI , 40 in t68Ga-DOTATATE PET/CT and 68Ga-DOTATOC PET/CT in the diagnosis, management, and follow-up of ONSMs, and in other intracranial meningioma types.Imaging with somatostatin receptor ligands seems to be a valuable tool in the non-invasive diagnosis of orbital space-occupying lesions. Further studies are needed to better establish the potential applications of The patient described in this case report consented to have her case described in this manuscript. Written informed consent was obtained from the patient for publication of this case report. The patient's personal identifiers were not included in this manuscript.KA, CC, DY, and JJ contributed to the conception and design of the work. BC, MG, and JJ assembled and interpreted the patient's imaging data. KA and CC wrote the first draft of the manuscript. All authors contributed to manuscript revision, and provided approval for publication of the content.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.


The cellularity was increased by approximately 50–95 %.•FHH has no association with hematological disorders or other malignancies.•Based on radiologic findings, fibrous dysplasia, aneurysmal bone cyst and plasmacytoma are among differential diagnosis of FHH.•chondrosarcoma and some other bony lesions with the manifestation of hematopoietic hyperplasia must also be considered among differential diagnosis. Focal hematopoietic hyperplasia (FHH) is an unusual benign lesion that can rarely involve the ribs. In general, FHHs is a tumor-like expansion of ribs due to the abnormal proliferation of bone marrow. FHHs are mostly found incidentally at radiologic studies performed for other reasons. They are characterized by gradually enlarging osteolytic masses that involve the rib.Here, we present an unusual case of focal hematopoietic hyperplasia of the rib who arrived to our institute with a complaint of severing back pain at the level of T5-T6 vertebrae. The diagnosis of FHH has been made based on clinical and radiologic findings and confirmed by histopathologic evaluations. We also reviewed all previously reported cases of Focal Hematopoietic hyperplasia of the rib in the literature.None of the previously reported cases, same as our patient, gave a history of trauma to the site involved or family history of malignancy. There was no association with hematological disorders or other malignancies that might have initiated FHH. Histologic evaluations of previously reported cases represented a hyper cellular bone marrow merging with the fatty marrow. The morphology and maturation of all hematopoietic cell lines were normal and there was no report of morphologically abnormal hematopoietic cell or malignant tumor cells.Physicians must consider the FHH among differential diagnosis of secondary rib lesions. Surgical resection is the treatment of choice; the risk of recurrence or metastasis is neglectable. Secondary chest wall tumors do not originate from ribs and their surrounding soft tissue. Metastatic disease is the most common process that involves chest wall. Chondrosarcoma, Ewing sarcoma, myeloma, and osteosarcoma are the most common primary malignant tumors. Yet, about 63 % of primary rib tumors are benign including osteochondroma, enchondroma, and fibrous dysplasia . Benign Focal hematopoietic hyperplasia (FHH) is an unusual benign lesion. Based on our review of articles they can very rarely involve the ribs. In general, FHHs is a tumor-like expansion of ribs due to the abnormal proliferation of bone marrow. So, they must be included in the differential diagnosis of primary rib lesions, especially, when assessing the adequacy of a specimen at the time of aspiration .Previous cases of FHH were sporadic solitary lesions without any association with hematological disorders or other malignancies that might have initiated it .FHHs are mostly found incidentally at radiologic studies performed for other reasons. They are characterized by gradually enlarging osteolytic masses that involve the rib .Radiologically, this lesion appeared as an expansive and radiolucent formation and contained areas of increased density or calcification; the cortex was intact and no soft tissue extension was noted. Marrow changes were diffuse with a marked decrease in signal intensity of the spinal and pelvic bone marrow on T1-weighted MR imaging. Sparing of the epiphyses and absence of moderately increased signal intensity on T2-weighted sequences aid in differentiating reconverted red marrow from neoplasm .Histologic evaluations of previously reported cases represented a hypercellular bone marrow merging with the fatty marrow. the morphology and maturation of all hematopoietic cell lines were normal and there was no report of morphologically abnormal hematopoietic cell or malignant tumor cells. For these reasons, they are considered a pseudo-tumor while radiologic and clinical findings are suggestive of a benign tumor ,2,4.Here, we present an unusual case of focal hematopoietic hyperplasia of the rib who arrived to our academic institution, Firouzgar General Hospital, Tehran, Iran with complaint of worsening back pain. The diagnosis has been made based on clinical and radiologic findings and confirmed by histopathologic evaluations. We also reviewed all previously reported cases of Focal Hematopoietic hyperplasia of the rib in the literature. This project has been reported in line with the SCARE criteria .2A twenty-two-year-old Iranian woman arrived at our institute with a complaint of severing back pain at the level of T5-T6 vertebrae since two months ago. There was no definite initiating cause and the pain was not associated with the activity. The pain did not radiate into any other part. She did not consume alcohol or illicit drug and had no history of underlying diseases.Laboratory data were all within normal ranges except slightly elevated ESR.The radiological study was performed. The Thorax spiral CT scanning without contrast injection was done and demonstrated an expansible bony lesion involving posterior arc of the right 6th rib, costovertebral joint and a portion of the T6 vertebrae. It was measured about 68*44 mm. In MRI, Right D5, D6, and D7 paravertebral mass were seen. The intervertebral foramen was also involved by the enhanced lesion .Fig. 1ChThen CT-guided biopsy and fine needle aspiration of the lesion was taken. A lobulated and irregular margin without cortical destruction was present. Macroscopic appearance of lesion was suggestive of benign osseous mass but extension into the body and right side of posterior elements of the adjacent vertebra was also seen. The specimen was sent for further evaluations. It contained multiple tiny pieces of brown soft tissue. The histopathologic finding was suggestive of norm cellular bone marrow. There were no malignant or atypical cell .Fig. 2HiBased on previous cases we decided to perform thoracostomy under general anesthesia by an expert spine surgeon. At the operating room, we performed resection of the proximal parts of right 6th rib and partial T5 and T6 corpectomy. During the surgery, we completely excised the lesion and a safe margin to avoid local recurrence. Intra-operatively frozen section was sent for rapid analysis to determine the safe margin from the lesion in surrounding parenchyma and minimize tissue damage. Although the mass had only involved the rib, and not the surrounding area, we gently removed reactive parts of the cortex for safety. Thus, intraoperative observation also helped in determining the margin of excision to decrease excessive tissue damage.Then she was admitted at the Neurosurgery Intensive Care Unit for three days under our close observation. The post-operative period was uneventful. Wound healing occurred without infection or inflammatory response.During one-year follow-up, she did not experience any abnormality. General physical exams were normal. Laboratory evaluations were all within standard ranges. However, we performed spiral chest and abdominopelvic CT scan; no lesion or any sign of remission was found.Based on our review on literature, there were seven similar cases of Focal Hematopoietic hyperplasia reported in articles. Here we also compared our findings with their reports and discussed similar features.3Focal hematopoietic hyperplasia is a rare condition characterized by a solitary osteolytic and expansive bony lesion of the rib with a maximum size of 9 cm. Diagnosis is confirmed by a combination of clinical, radiologic and histologic features .Lesions were radiolucent and had a thin and intact cortex. ill-defined linear areas of higher density or calcification were prominent and no soft tissue extension was seen. All previously reported cases had similar radiologic characteristics .From the aspect of histopathology, FHH is a mix of hypercellular and fatty marrow. The cellularity was increased by approximately 50–95 %. In all of the previously reported cases, the marrow contained all hematopoietic cell lines  and no morphologically abnormal cells or metastatic carcinoma cells were seen. Bony tissue became osteoporotic at some parts (due to disruption and thinning of some of the bony trabeculae) and denser at other parts (due to thickening and distortion of trabeculae) .There were only seven cases of FHH reported earlier in English literature.In 1984 Gerald Eldelstein and Kyriakos presented first two cases of focal hyperplasia of the hematopoietic marrow causing rib lesions without any associated hematologic abnormality. Their first case was a 66-year-old black woman presented to ER with sudden onset vomiting, diarrhea, and abdominal discomfort. During medical evaluations, they accidentally found a 4.0 × 3.0 cm well delineated ovoid, expansible, and Lucent lesion arising near the ribs posterior articulation at a detailed roentgenogram. The second case was found during medical evaluations performed before surgical repair of inguinal hernia in a 71-year-old white man. None of their cases had any history of other benign or malignant tumors. Both patients underwent surgical excision of bony masses after symptomatic therapy. They concluded that these lesions are benign and are a form of pseudotumor, designated as "focal hematopoietic hyperplasia of rib" or "hematopoietic pseudotumor" [The third case was a 46-year-old with A large, lytic mass on the posterior aspect of the sixth rib, reported in 1997 by Lorenzo M. Galindo. Despite the superficial location and significant size, the lesion was asymptomatic and was found during radiologic evaluations performed for assessment of diverticulitis. The radiologic studies suggested the presence of a neoplastic lesion; This lesion was fairly radiolucent and had trabeculated or linear areas; similar to radiologic characteristics of the previous two cases. The diagnosis was confirmed by fine needle aspiration biopsy (FNAB) .The fourth case was a 24-year-old woman who presented with a rib mass incidentally found at chest radiograph while assessing a degenerative thyroid cyst. They performed computed tomography which revealed an expanding bony lesion with internal calcification arising from the head of the posterior rib and involved the transverse process of the third thoracic vertebra. They did not mention any signs of cortical destruction, soft tissue involvement or periosteal reaction. unexpectedly, the histopathologic study revealed the focal fibrosis and osteosclerosis which are suggestive of myelofibrosis. But then, fibrosis was limited to the endosteal surface. Before that case, there was no report of myelofibrosis associated with an expanding lesion of bone.A 34-year-old female who was presented with complaints of chronic pain at the region of the 6th to 8th left side ribs was the fifth case of FHH. It was the only symptomatic case of FHH. She had no history of trauma or family history of malignancies. They performed positron emission tomography (PET) bone scan that revealed a hot area in the posterior aspect of the left 7th rib. A 250 mm in length lesion was radically excised through a left postero-lateral thoracotomy of the left 7th rib. They evaluated a cross section of the thickened part of the rib; uniform thickening of the marrow cavity without any focal lesions was seen. Further histopathologic evaluations, revealed hyperplasia of all hematopoietic cell types in their mature form and their precursor cells; same as previous other cases of FHH.The sixth case was a 24-year old, professional athlete with a complaint of back pain following trauma. There was no abnormal finding in clinical evaluations. For evaluating the source of pain, the bone scan was performed, which did not show increased radiotracer accumulation; differently, the whole-body PET performed and demonstrated high uptake of the radiotracer in the vertebral body of L3. Also, lumbar spine MRI showed the vertebral lesion. They took a biopsy of the L3 vertebral body lesion and sent species for further histological examination which was suggestive of a chronic myeloproliferative disease. Moreover, they found hyperplastic bone marrow and fatty marrow (cellularity was increased by approximately 50–95 %) consisting of normal hematopoietic cells of all three lineages. There was also severe osteoporotic tissue while hyperactivity of osteoclastic cells was not obvious. He achieved satisfactory outcome after surgical resection of the lesion.The seventh case was a 26-year-old female with palpable rib mass. Chest Radiograph demonstrated an expanding bony lesion. Computed tomography scan showed a 5.0 × 4.5-cm mass in the body of second rib associated with internal linear calcification and thinning of the inner surface without cortical destruction. With the initial diagnosis of osteochondroma . immediately she underwent surgical resection and species were sent for further microscopic evaluations. Hypercellular bone marrow containing three lineages of hematopoietic cells and their precursor with fatty marrow in the inter-trabecular spaces was observed. There was no evidence suggesting malignant conditions.Based on our review of the literature, our case is the eighth reported case of FHH. None of the patients gave a history of trauma to the site involved or family history of malignancy. Only one of the previous cases had a history of trauma which was not at the definite site of the lesion. There was no association with hematological disorders or other malignancies that might have initiated FHH. Except for one case, others were found incidentally during radiologic studies for other non-relevant reasons. All cases share similar clinical, radiological and histological findings. Specific radiologic features made the chest routine radiographs and CT-scan, the best modalities for the diagnosis of FHH. Radiologically, these lesions are expansive and radiolucent masses with the thin cortex and containing areas of increased density or calcified trabeculae. Surrounding soft tissue involvement was not found in any of cases. Microscopic evidence in all reported cases including ours showed the characteristic pattern of mixed areas of hypercellular marrow and fatty marrow. There was no report of morphologically abnormal hematopoietic cell or malignant tumor cells. All patient underwent surgical resection, satisfactory results were achieved and no post-operative complication was reported. None of the cases experienced any episode of recurrence and there was no need for chemotherapy or re-surgery.Based on our primary evaluation, the lesion was not thought to be malignant. NO malignant cell was reported at pathologic evaluations of fine-needle biopsy. However, it was probable that the specimen was obtained from the peripheral tissue and could not accurately rule out malignancy. In addition, our patient was suffering from severe localized pain. Thus, similar to previous cases, we decided to surgically remove the lesion to first relieve the pain and then confirm the diagnosis by histopathologic studies. Considering the possible harms following a total corpectomy especially in a young patient, we performed partial T5 and T6 corpectomy. During the operation, we completely resected the lesion and a safe margin which was determined the aid of rapid analysis of the frozen section to avoid local recurrence. Also the reactive tissue of cortex was excised for safety.In discussing the differential diagnosis of FHH we should consider all benign or malignant lesions. Radiologic findings suggest some differential diagnosis for FHH including fibrous dysplasia, aneurysmal bone cyst, and The most complicated of all, plasmacytoma. From the aspect of histopathologic evaluations, chondrosarcoma and some other bony lesions with the manifestation of hematopoietic hyperplasia must also be considered among differential diagnosis. however, Myelolipoma and some hematological disorders including Thalassemia, chronic anemia, leukemia and myelofibrosis also present with increased hematopoiesis, hyperplastic and fatty marrow and thin bony trabeculae. Tumor-like hyperplasia of fat tissue and bone marrow, known as myelolipoma; are most frequently found in the adrenal gland ,4.Myelofibrosis is differentiated with concurrent hyperplasia of three hematopoietic cell lines which does not match with our findings.In cases of Thalassemia evidence of bone involvement include widened marrow cavity, cortical thinning and marrow hyperplasia due to severe hemolysis. Though the bony involvement of Thalassemia is distinct from others with the medullary expansion of multiple bones  and frequently involves skull. The posterior aspect of ribs shows cortical expansion. The patient suffers from anemia and is expected to have clinical features of anemia, skeletal deformity, and hepatosplenomegaly.FHH is not associated with anemia and hepatosplenomegaly and none of the above mentioned clinical features were present in our patient ,2,4.Three main possible mechanisms explain the pathogenesis of FHH. First of all, these lesions may grow either due to a reactive process (secondary to trauma or inflammation) or developmental anomalies. None of the previously reported cases, including ours, reported any recognized trauma or history of inflammation prior to the diagnosis of FHH. Also, no primary lesions that may have led to these lesions were found in any of cases. On the other hand, most cases were diagnosed at an advanced age and it is against the theory of originating from developmental anomalies. Thus, we believe in young healthy patients, similar to our discussed patient, FHH can develop due to late and reactive change of either inflammation or some unrecognized benign tumor. However, it is not possible to determine the exact etiology, especially in a young healthy patient like our case ,4.Furthermore, it is possible that stress itself should be considered as a risk factor and can lead to the reconversion of yellow marrow to hematopoietic marrow .Besides above-mentioned mechanisms, Stabler et al. demonstrated that hematopoietic hyperplasia can also be associated with malignancies and chronic anemia. Also, the granulocyte-colony-stimulating (GCS) factors that are used as an adjunct to radiation or chemotherapy treatments to decrease can lead to hyperplasia of the hematopoietic marrow .The ethical committee of our institute gave us permission for current publication.The patient gave informed consent and is aware of publication of this case report.4It is important to consider FHH of the rib among the differential diagnosis of solitary expanding bony lesions of ribs and vertebrae, especially when FNAB or small tissue biopsy specimens yield apparently normal marrow tissue and microscopic evaluation reveal mixed hyperplasia of marrow and fatty tissue.We had no source of fundingThe ethical committee of neurosurgery research centre at Iran University of Medical Sciences is aware of current publication and gave permission.Our patient gave us informed consent after making sure her information are published anonymously-Reza Mollahoseini – Diagnosis and surgery – supervising this report.-Paniz Motaghi – Review of articles and writing manuscript.-Alireza Dastmalchi _ surgery and help in writing manuscript.-Hanieh Zahm – Histopathologic evaluation.This study is not a clinical trial, thus has not been registered.We had no Guarantor except Dr. Reza Mollahosseini the first author.Not commissioned, externally peer-reviewed.This article has no conflict of interest.


Gastrointestinal upset is a common presentation to surgical departments, often requiring investigation with endoscopy. Pathologies such as gastritis or ulcers are common culprits. Occasionally, rare or unusual pathologies, such as gastric diverticula as was seen in the case presented, are found.A 26 year old female, with no known co-morbid conditions presented with a two week history of abdominal pain associated with nausea and vomiting. On further inquiry, she had one episode of blood stained vomiting, prompting investigation with an oesophagogastroduodenoscopy (OGD). Findings included diffuse haemorrhagic gastritis with a single outpouching measuring 1–2 cm in the gastric fundus. A gastric diverticulum was confirmed on barium swallow. Investigation with sonar and a Computed Tomography (CT) scan reported the stomach as normal.The patient was successfully treated non-operatively with proton pump inhibitor therapy for her concomitant gastritis. Gastric Diverticula are often associated with other gastric findings and their individual contribution varies from case to case.Gastric Diverticula are the manifestation of a common condition in an unusual location. Their clinical implications vary from being insignificant to life threatening when complicated by haemorrhage, perforation or malignant transformation. The associated symptoms are non-specific and diagnosis may be challenging. The case highlights the importance of selecting appropriate imaging modalities for luminal structures, being only diagnosed in 2  of the four modalities (incl. ultrasound and CT scan) used. Treatment may be conservative or surgical and is patient dependent. Written consent and ethical approval was obtained. The work is reported in line with the SCARE criteria. The dia2A 26-year-old female presented, walking, to the emergency department with a 2 week history of abdominal pain radiating to the back. This was associated with a 2 day history of nausea and vomiting following an alcohol binge. On one episode the vomitus was noted to be blood stained. She had no prior medical, surgical or family history. She consumed alcohol regularly and had a binge on the two days prior. She consumed Non-Steroidal Anti-Inflammatory Drugs (NSAID) chronically and had an eight-pack-year history of smoking.On arrival she had a blood pressure of 124/64 mmHg and was tachycardic with a heart rate of 118bpm. She had a temperature of 37 °C. Her urine dipstick and pregnancy tests were negative. On examination she had epigastric tenderness but her abdomen was soft and she had no signs of peritonitis. Her cardiovascular, respiratory and central nervous system examinations were unremarkable. She had no malaena or blood on rectal examination.3She had normal electrolytes, septic markers, liver and renal function on formal bloods. Her amylase was 35 U/L. Her haemoglobin was 14.2 g/dl. Her abdominal and chest x-rays were normal.An abdominal ultrasound (US) reported a normal pancreas, liver, spleen and kidneys. The gallbladder was normal with no thickening, pericholecystic fluid or calculi. There was no free intraperitoneal fluid noted or abnormal masses.She was subsequently sent for OGD, performed by the surgical registrar with supervision, which found diffuse haemorrhagic gastritis. A single outpouching estimated to measure 1–2 cm was noted in the gastric fundus, the mucosa was otherwise regular and there were no signs of perforation . A biopsShe was sent for a CT scan of her abdomen which was reported as normal. Based on her OGD findings, she was sent for a barium swallow. She was given 1 g of Calcium Carbonate effervescent tablets in order to assist with gastric distention. Informed by the literature, she was positioned supine with a slight left lateral tilt with the feet at a 30° incline (Trendelenburg) position . The conHer histology from the biopsy taken at OGD revealed moderate chronic superficial gastritis with no helicobacter pylori organisms found and no intestinal metaplasia, dysplasia or malignancy.41.Peptic ulcer disease: this is based on her history of symptoms, physical examination and risk factors i.e. smoking, NSAID use, ethanol history. This was excluded on OGD.2.Acute pancreatitis: this is based on the characteristic history of her pain together with a recent alcohol binge. This was excluded on formal blood results revealing normal amylase with no features of pancreatitis on sonar or CT scan.3.Symptomatic cholelithiasis: this is based on her risk factors  as well as her presentation with upper abdominal pain. This too was excluded on sonar and CT scan.Several differential diagnoses were considered:5Prior to her gastroscopy, an Intravenous (IV) line was inserted and she was given fluid rehydration with crystalloid fluids (i.e. BALSOL) and given an immediate Intravenous dose of Pantoprazole 80 mg. Based on local treatment guidelines for gastritis, following the OGD she was started on a course of Proton Pump Inhibitor (PPI), omeprazole 20 mg orally 12 hourly for 4 weeks, and she was given Paracetamol 1 g orally 6hourly as analgesia. She had no further episodes of hematemesis while in hospital and was discharged 2 days later.6On follow up after a month, she remained asymptomatic. On clinical examination she had no pallor and had an unremarkable abdominal and per rectal examination. It was deduced that her abdominal symptoms were more likely attributable to her gastritis. She was counselled on the potential dangers of NSAID use and their contribution to her condition, smoking cessation and responsible drinking. She was advised to return for a repeat scope should symptoms recur, at which stage further intervention could be considered.7A Gastric diverticulum (GD) is an outpouching of the gastric wall which most often occurs in the posterior wall of the fundus, as was demonstrated in the case above . They arThese may be classified as: congenital or acquired, true or false. The difference between true and false diverticula is based on the former containing all the layers of the gastric wall as opposed to false diverticula which do not. Congenital diverticula are typically true and constitute ¾ of all GD’s, while those that are acquired are typically false . CongeniGD may be asymptomatic but when they do occur, patients typically present with a long history of vague abdominal complaints such as: dyspepsia, upper abdominal pain, nausea, vomiting and early satiety. Due to the non-specific nature of the symptoms, these may be attributed to an array of other abdominal pathologies and a diagnosis is often made incidentally in the workup for such. In 1951, following the review of 49 symptomatic cases of GD, Palmer suggested that symptomatic diverticula are usually seen in the presence of another gastric abnormality such as ulceration or gastritis (as was seen in our case) . This brThe gold standard investigation modality is OGD. This not only confirms the diagnosis but co-existing pathologies may be identified and unusual features  may be biopsied. Another modality is an contrast meal, but this carries a 5% false negative rate . This maManagement of GD is patient dependant based on the severity of the presenting complaints, size, presence of complications and patient profile. Medical treatment with the use of protein pump inhibitors, antacids or antihistamines, have been reported to provide a degree of symptomatic relief without resolving the underlying pathology ,5. SurgiIn conclusion, gastric diverticula represent a common condition in an unusual location. The symptoms of it vary and the clinical significance more so. Our patient was found to have concomitant gastritis which is in keeping with the findings by Palmer et al. that these diverticula are often associated with other pathologies . She was“Coming to the hospital I was very worried, especially when I started vomiting blood. I was worried that this pain and vomiting was all related to the alcohol I had taken. I had heard from people that taking certain painkillers could give you ulcers but I had also being told that it rarely happens. I agreed to have all the investigations done because I wanted to know what was wrong with me and to get better. I find it interesting that I have such a rare condition but happy that it is not life threatening”There are no conflicts of interest to declare for any of the authors.No funding was received to conduct this researchEthics was granted from the Human Research Ethics Committee at the University of Witwatersrand. The approval was unconditional with the following reference number: M190583.Written informed consent was obtained from the patient for publication of this case report and accompanying images. Confidentiality was maintained in the writing of this report.Nolitha Morare- Conceptualization, Validation, Investigation, Writing – Original Draft, Writing – Review & Editing, SupervisionEugene Baloyi- Writing, Original Draft, ResourcesDavid Rose- Software, Writing – Review & Editing, VisualizationThis is a case reportNolitha Morare (corresponding author) is the primary guarantor. All of the authors however, accept full responsibility for the work.Not commissioned, externally peer-reviewed


Hepatoblastoma is the most common childhood liver malignancy. The management of hepatoblastoma requires multidisciplinary efforts. The five-year overall survival is approximately 80% in developed countries. Surgery remains the mainstay of treatment for hepatoblastoma, and meticulous techniques must be employed to ensure safe and effective local control surgeries. Additionally, there have been several advances from both pediatric and adult literature in the way liver tumor surgery is performed. In this review, we highlight important aspects of liver surgery for hepatoblastoma, the management of metastatic disease, and the most current technical advances in performing these procedures in a safe and effective manner.  Hepatoblastoma is the most common liver malignancy in children. The incidence is approximately 1.2/1,000,000 and about 100 new cases are diagnosed annually in the United States ,4,5. HepA variety of radiographic tools can be used to image hepatoblastoma, and surgical resectability should be evaluated based on the combination of these imaging findings. Ultrasound is the preferred modality for the initial screening and diagnosis of an abdominal mass. Hepatoblastoma usually presents with a heterogeneous echo signal and significant mass effect on adjacent organs. Ultrasound can confirm the hepatic origin of the tumor by evaluating the movement of the mass with respiration or its vascular supply emanating from the portal vein and hepatic artery . UltrasoMagnetic resonance imaging (MRI) is the preferred cross-sectional imaging modality for the primary tumor because it provides superior soft tissue contrast resolution and does not employ ionizing radiation . On MRI,Since approximately 20% of the newly diagnosed hepatoblastomas present with lung metastasis , multi-dPositron emission tomography (PET)/CT has no definitive role in the diagnosis of hepatoblastoma. However, PET/CT can provide valuable information in the assessment of relapsed cases and is especially useful for detecting small early recurrences ,20,21.The pre-treatment extent of tumor (PRETEXT) system was developed by SIOPEL to standardize imaging evaluation and risk stratification for hepatoblastoma prior to neoadjuvant chemotherapy ,22,23,24In addition to these classifications, PRETEXT annotation factors, such as hepatic venous involvement, portal venous involvement, extrahepatic disease, multifocality, rupture, caudate involvement, lymph node metastases, and distant metastases, should also be carefully addressed based on MRI/MDCT findings, according the 2017 PRETEXT guideline . The PREThe Children’s Hepatic Tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) has been proposed for the newly launched Pediatric Hepatic International Tumor Trial (PHITT) which will prospectively validate this proposed model . Based oThe International Childhood Liver Tumors Strategy Group and Children’s Oncology Group approach a newly diagnosed hepatoblastoma with different strategies. The International Childhood Liver Tumors Strategy Group tends to give neoadjuvant chemotherapy to all hepatoblastoma patients and then performs a delayed surgery ,25,26. TIt is equally important to determine the optimal timing of delayed surgery for those who undergo neoadjuvant chemotherapy. The International Childhood Liver Tumors Strategy Group tends to give four cycles of neoadjuvant chemotherapy for standard risk hepatoblastoma ,26. MurpHepatoblastomas that are unresectable  with standard liver resection should be referred for liver transplantation ,35. EarlIn the scenario of possible insufficient future liver remnant (FLR), the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure will be of great usefulness for increasing the volume of FLR ,42,43. TIn recent years, new technological advances have been employed as adjuncts to pediatric hepatic resections with exciting results. These include the use of intraoperative ultrasound, the use of image guided three-dimensional reconstruction, and the use of indocyanine green (ICG) during liver resection and metastatic lesion resection. Newer approaches to local control surgery have been to use laparoscopic liver resection and more extreme resection techniques.Intraoperative ultrasound (IOUS) during hepatic resection in adults was first described with good results as early as the 1970s. The use of IOUS as an adjunct to adult liver resections in cases of both hepatic metastasis from gastrointestinal malignancies as well as for defining the proper hepatic transection planes is well reported with good results . The useTechnological advances have now created 3D image-processing software dedicated to virtual simulation in liver surgery . Such teVirtual resections can substantially contribute to surgical strategies for children with complex hepatic tumors . This toAnother adjunct for pediatric hepatic resection is the use of ICG imaging. The use of preoperatively injected intravenous ICG and near infrared imaging equipment has emerged as a novel therapy in cases of solid organ malignancy and metastatic resections . The useLaparoscopic liver resection is becoming increasingly popular for adult liver resection. In experienced hands, laparoscopic liver resections are safe with acceptable morbidity and mortality for major hepatic resections . LaparosExtreme liver resection for advanced-stage (POST-TEXT III and IV) hepatoblastoma seems to have comparable overall survival when combined with chemotherapy as compared to liver transplantation ,73. Two The lung is the most common metastatic site for hepatoblastoma and approximately 20% of the initially diagnosed hepatoblastoma cases have also presented with lung metastasis . The iniLung metastasis can be safely resected with traditional thoracotomy or thoracoscopic surgery. The traditional thoracotomy provides the ability to manually palpate the lungs, which was once considered essential in identifying lung metastasis. For video-assisted thoracoscopic surgery, CT-guided lung nodule localization using the combined techniques of methylene blue blood patch and hook wire is safe, technically feasible, and successful in children. Using this combination of techniques will consistently yield a pathological diagnosis .Intraoperative ICG fluorescence imaging is feasible and useful for identifying small viable metastatic lung lesions . KitagawRelapsed hepatoblastoma occurs in less than 12% of patients with complete remission after the first line of treatment . Most ofOrthotopic liver transplantation is an option for those with unresectable local relapse . HoweverLung relapse can be solitary or multiple, unilateral or bilateral . The valThe PRETEXT system and platinum-based chemotherapy have laid out a foundation for the current management of hepatoblastoma. Substantial progress has been made in the surgical arena of hepatoblastoma. Other than the promising outcomes of transplantation, the most exciting part of the surgical management of hepatoblastoma may be the introduction of ICG-guided and 3-D model guided surgery. These new technologies have led to precise resection of hepatic tumors that may not otherwise be resectable and can be effective for the treatment of primary or recurrent lung metastases. Furthermore, ALLPS further pushes the envelope for the success of surgical resection by increasing the volume of potentially insufficient future liver remnants. All of these advancements will enable surgeons to provide better surgical outcomes for hepatoblastoma.


Desmoid tumors are rare, monoclonal myofibroblastic neoplasms that occur in the extremities, the trunk, and the abdominal cavity. We present a case that is significant for its rarity and for consideration of its treatment plan. A 17-year-old male reported swelling of his abdomen and abdominal pain. He was referred to our hospital with no history of surgery, trauma, or familial adenomatous polyposis. A large tumor in the abdominal cavity was detected by computed tomography, and surgical resection was performed. The tumor was thought to have developed from the anterior lobe of the transverse colon mesentery. It weighed 5.9 kg. Tumor cells with collagen fibers were observed in histopathological examination, but heteromorphism and the nuclear fission image were not apparent. Immunostaining revealed beta-catenin expression in the tumor cell nucleus. Diagnosis was an intra-abdominal desmoid tumor. Currently, there are no signs of recurrence. In this case, preoperative diagnosis was difficult, but surgery was the optimal treatment according to the symptoms. Desmoid tumors have invasive development and common local recurrence, so sufficient range of resection including normal tissue and strict follow-up are necessary. Desmoid tumors (DTs) are benign, deep-seated monoclonal myofibroblastic neoplasms that slowly grow, infiltrate, and arise from musculoaponeurotic stromal elements . They arA giant intra-abdominal desmoid tumor (DT) developed in a young male without history of surgery, trauma, or FAP. The tumor contacted both his stomach and pancreatic tail. We performed surgical resection of the tumor with partial resection of the stomach and pancreatic tail. DTs have invasive development, and many recur locally, so it is thought that complete resection with a negative margin is important , 2.A 17-year-old male noticed swelling of his abdomen from six months previously. He reported pain at the left side of the umbilicus. Body weight increased by 5 kg in one year. Computed tomography (CT) was performed at another hospital. A larger abdominal tumor was detected, so he was referred to our hospital for examination. Vital signs and laboratory data were normal, but the abdomen was bulging slightly. CT showed a giant tumor occupying the majority of the abdominal cavity Figures . The tumOn the gadolinium-enhanced image, the contrast effect was poor, and the high signal area was only slight.Preoperative diagnosis was a giant abdominal cystic tumor. Differential diagnosis was gastrointestinal stromal tumor (GIST), DT, or lymphangioma.Surgery was performed for definitive diagnosis and improvement of the symptoms.A lower midline incision was performed to observe the large tumor contained within a membrane . The tumThe tumor is shown in Figures Pathological findings included spindle-shaped or star-shaped tumor cells proliferating diffusely with abundant collagen fiber. Heteromorphism was not noticeable, and the nuclear fission image was not apparent. Beta-catenin was positive in the tumor cell nucleus on immunohistochemistry Figures .Final diagnosis was an intra-abdominal desmoid tumor.Postoperative pancreatic fistula occurred but was improved with nonoperative therapy. The patient was discharged on the 16th postoperative day. After discharge, colonoscopy (CS) was unremarkable. The patient is now being followed-up as an outpatient.DTs are benign, deep-seated monoclonal myofibroblastic neoplasms that slowly grow, infiltrate, and arise from musculoaponeurotic stromal elements . DTs areFor diagnosis of DTs, especially intraabdominal DTs, representative symptoms are intestinal obstruction, bowel ischemia, and abdominal distention . Many paConcerning imaging study, both CT and MRI play important roles. On CT, DTs usually appear as a well-circumscribed homogenous lesion isodense or hyperdense relative to muscle, although GIST may have a similar appearance . DiffereThe tumor in our patient had two parts; one part was a single cystic lesion without contrast enhancement, and the other part seemed to have a solid component inside, the contrast effect of which was mild. About the MRI, signal intensity is reflective of the proportion of collagen fibers, spindle cells, and extracellular matrix present . Most inThe contrast enhancement pattern is variable, moderate-to-marked enhancement , 7. AlthRegarding treatment, the optimal therapy for DTs is also difficult to ascertain. DTs are rare, and anatomical presentation is varied, so large randomized trial studies are difficult , 2, 8. CIn spite of the complete resection of the tumor, the recurrence rate of the DTs ranges between 30 and 40% . The absIn summary, we encountered a rare case of giant intra-abdominal DT in a young male who had no history of trauma, surgery, or AFP. Although he had symptoms, definitive diagnosis was not possible, so optimal treatment was surgery. DTs have a high rate of recurrence, so complete resection and close follow-up are necessary.


Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that arises from the dermis and invades deeper tissue. The cellular origin of DFSP is not clear. Evidence supports the cellular origin being fibroblastic, histiocytic, or neuroectodermal.A 38 years old, women presented with slow-growing large right parotid mass. A total parotidectomy performed with auriculectomy and reconstruction using ALT flap. Diagnosis confirmed by pathology and immunohistochemistry. Tumor recurred in 10 months, the second surgery with subsequent chemoradiotherapy performed. Patient initially treated with wide resection, 4 cycles of chemotherapy and postoperative radiotherapy 40 Gy, with the recurrence in 10 months. We performed a second surgery followed by radiotherapy. She is disease-free for more than two years under follow-up.The main treatment of DFSP is surgical resection with wide negative margins or Moh’s surgery. Advanced cases treated with addition of radiotherapy or chemoradiotherapy, but with unclear benefits. In our case, huge tumor located in the parotid region recurred after initial surgery and adjuvant treatment.Clinically, DFSP usually manifest as well circumscribed, slow-growing, smooth, and painless masses. In cases with advanced tumor in parotid region, recurrence may occur despite aggressive initial treatment with wide resection and chemoradiotherapy. Hoffman officially coined the term dermatofibrosarcoma protuberans in 1925 . MetastaThe work has been reported in line with the SCARE criteria .2A 38-year old Russian woman presented with the complaint of a slow growing, painless pretragal swelling of eight years duration. The patient had a resection of parotid gland under local anesthesia eight years ago in the local regional hospital. Microscopic examination showed signs of angiofibrosis. In 1 year there was a recurrence of the tumor, and histological examination did not reveal any malignant cells. After seven years the patient noticed the fast growth of the tumor. General practitioner referred the patient to our institute, tertiary research cancer center. The clinical examination found a dense, slightly painful on palpation mass in the right parotid region with exophytic growth on a wide base with spread to the temporal, mastoid region and extension to the auricle cartilage measuring 18.0 × 8.0 × 9.0 cm. There was no facial paralysis or cervical lymph node enlargement .Fig. 1PaMRI scan revealed the homogeneous mass in the right parotid region with good margins, unmineralized, nodular soft-tissue mass involving the skin and subcutaneous adipose tissue. The features were suggestive of a sarcoma .Fig. 2MRAccording to, the decision of the tumor board, wide resection of the tumor including right total parotidectomy, auriculectomy, and the reconstruction of the postoperative defect with the anterolateral thigh flap on the microvascular anastomosis performed.Histopathological examination revealed short spindle cells, hypercellularity, moderate to marked atypia, nuclear pleomorphism and high mitotic activity with the tight storiform pattern . On immuIn postoperative period she decided to get treatment in a regional cancer hospital. Four cycles of dacarbazine 400 mg on 1–5 days, doxorubicin 100 mg and 40 Gy radiotherapy delivered to the primary tumor bed by her regional cancer hospital. Tumor recurred after ten-month follow-up. The PET-CT examination found a 4 cm mass in the right parotid area.Resection of recurrent tumor performed without complications . The mic3DFSP is a fibrohistiocytic tumor of low to intermediate malignancy, having infiltrative margins with high local recurrence (about 50%), and rare distant metastasis . These tRoutine workup consists of MRI (preferable) or CT and biopsy with histological examination. On computed tomography, these tumors appear as well-defined masses that are hypointense to muscle and demonstrate homogenous contrast enhancement. On magnetic resonance imaging, DFSP is homogeneous and iso- or hypo-intense to muscle. They are strongly enhanced post-contrast on T2-weighted images .The macroscopic characteristics of the tumor include a well-defined and encapsulated mass which may be accompanied by bone destruction, though it is normally free of infiltration.Histological features suggesting malignancy include high mitotic rate (four or more mitoses in 10 high power fields), hypercellularity, moderate to marked atypia and nuclear pleomorphism, tumor necrosis and infiltrative borders. Histologically, a diagnosis of DFSP is also difficult because many tumors display similar findings. DFSP, unlike a solitary fibrous tumor, shows remarkable uniformity, a lack of the hemangiopericytic pattern and a distinct storiform pattern around an inconspicuous vasculature. Because of overlapping immunohistochemical results for DFSP and solitary fibrous tumor, caution required in the differential diagnosis. Benign fibrous histiocytoma may resemble DFSP, but it is usually negative for CD34 and Bcl-2. Schwannoma contains Antoni A and B areas, and it is S-100 protein positive which not seen in DFSP [The main treatment of DFSP is surgical resection with wide negative margins. Due to the giant size of the tumor in our case, we decided to carry out the one-stage reconstruction of the postoperative defect with the anterolateral thigh flap on the microvascular anastomosis. Mohs technic recommended by several publications gives the possibility to have narrow margins .The 5-year survival rate for head and neck sarcomas is approximately 50%. Most authors agree that the same prognostic factors– grade, size, and depth-apply to sarcomas no matter where they arise. In the head and neck, however, local recurrence has more significant consequences because of the difficulty of subsequent management. In general, salivary gland sarcomas are aggressive neoplasms with recurrences in about 40–64%, hematogenous metastasis and mortality rates ranging from 36 to 64%. The prognosis for parotid DFSP is not clear due to the scarcity of cases reported in the literature .In our case recurrence was observed within 11 months after treatment despite aggressive postoperative treatment including 4 cycles of chemotherapy and 40 Gy radiotherapy. After second surgery only radiotherapy performed as adjuvant therapy . Some stSince recurrence and metastasis can take place after several years, a lifelong clinical and imaging regular follow-up is compulsory.4Dermatofibrosarcoma protuberans is a rare tumor, with infiltrative margins, high local recurrence rate, and rare distant metastasis. Parotid gland or parotid region is a very rare site with few published case reports, shares the common features of trunk and extremities DFSP. The complete resection is the most important prognostic factor, and no evidence supports the efficacy of any therapy different to surgery. Radiotherapy or chemoradiotherapy can be applied to large and recurrent cases, but with unclear benefit. Due to the frequent local recurrence even after many years of remission, a long-term follow-up is guaranteed.No conflicts of interest to declare.It is a case report study. No funding was obtained to perform a study.Not applicable, observational case reports are exempt from ethical approval in our Institution.We have obtained a written consent from a patient; despite there is no possibility to identify a patient on provided pictures.DA – study concept, and final approval. DA, FK, ST – acquired and interpreted the data and drafted the manuscript. DA, DAh. - performed the operation and perioperative management of the patient, revision of the manuscript. All authors read and approved the final manuscript.Not applicable.Dauren Adilbay.Not commissioned, externally peer-reviewed.


Midline cervical cleft is a very rare congenital anomaly. According to a literature search, until 2014 only 205 cases were reported.We present a classic case of congenital midline cervical cleft. This was a case of a 3-year-old Middle Eastern boy. The lesion was excised and the defect was closed via multiple Z-plasties.Midline cervical cleft, although a rarity, when presented needs surgical treatment, which comprises surgical excision and closure that lessens the possibility of scar visibility and contracture. Midline cervical cleft (MCC) is a rare congenital anomaly. According to a literature search, by 2014 only 205 cases had been reported . It is aIt is not considered a true cleft because no skin gap exists. It represents a variant of the cleft category number 30 of the Tessier classification system of craniofacial defects , 6, 7.The clefts have a remarkably consistent appearance and are typically noticed at birth as a defect at the ventral aspect of the neck. Superiorly, there is a skin tag with the inferior margin being formed by a short sinus, normally approximately 1 cm in length. A mucosal surface divides the two. No antenatal problems are generally identified and Caucasian women seem to be the most frequently affected .MCC is diagnosed by clinical examination, and the main characteristic is a protruding lesion at the midline of the anterior region of the neck, between the chin and the suprasternal notch . The carTreatment is surgical excision, which is required largely to improve the cosmetic appearance of the neck and to avoid contracture formation of the ventral neck , 9. We pA 3-year-old Middle Eastern boy presented with a defect in the midline of his neck. He was born at full term by normal vaginal delivery and had no significance in his past medical history. There was no family history of congenital defects or consanguinity. The anomaly was located in the ventral midline of his neck Fig. . The supHe had an MRI done elsewhere, indicating a soft tissue mass without any fistula tract. Despite contrast material being injected through the opening at the caudal end of the lesion, the diagnosis of MCC was established. No evidence of any other neck anomaly was found Fig. . The sinA surgical removal and immediate closure with multiple Z-plasties were performed. Surgical removal was done with an incision 1–2 mm from the periphery of the lesion, deepened down to the supraplatysmal plane Fig. . During A pathological examination of the specimen confirmed our clinical diagnosis. The findings were consistent with stratified squamous epithelial cells covering the cleft with few adnexial structures at the dermal level Fig. .Fig. 5HiOne month follow-up examination revealed an uneventful healing period, with redness along the incision scar and some nodularities, which were most probably due to the subcutaneous suture material. He was able to move his head in all directions without any restriction or pain Fig. .Fig. 6OnA 14-month follow-up examination showed an acceptable level of scarring causing no restriction of neck movements Figs.  and 8.FiThe MCC consists of epidermis, a mucosal surface skeletal muscle , and glandular tissue. There have been many theories that attempt to explain these findings. Some propose that pressure from the pericardial roof on the developing branchial arches results in pressure necrosis and scarring. Others postulate that rupture of a pathological adhesion between the epithelium of the cardiohepatic fold with that of the ventral part of the first branchial arch causes tissue ischemia, again with localized necrosis and scarring. These theories, however, do not explain the consistent anatomy of the MCC or the presence of skeletal muscle and glandular tissue .Another hypothesis that does help to explain the histological findings is the abnormal development of the first branchial arch. On day 22 in the human embryo, the branchial arches are forming. The first arch is divided into maxillary and mandibular processes with a horizontal cleft dividing each side at the midline. The mandibular process merges on day 26. A delay in this could result in ectodermal cells with underlying mesodermal cells being deposited in the ventral aspect of the neck. These would continue to differentiate and form skeletal muscle (tongue derivative), salivary glandular tissue, and a mucosal surface, thus resulting in the MCC defect , 8. ThisMCC is diagnosed by clinical examination, and the main characteristic is a protruding lesion at the midline of the anterior region of the neck, between the chin and the suprasternal notch. This lesion extends caudally as a longitudinal cleft lined by a reddened epithelium that is most commonly desquamative. Usually, a mucus-secreting opening of the fistulous tract can be found in the most caudal portion of the cleft. Palpation reveals a subcutaneous fibrous cord that extends to the entire cleft or part of it, from the submentonian region to the suprasternal notch . The sinOn histological examination, the lesion consists of keratinized stratified squamous epithelium and fibrous tissue and is characterized by the absence of epithelial adnexa in the dermis. Salivary gland tissue and pseudostratified ciliated columnar epithelium are observed in the fistulous tract and in the cyst. In the area of the nipple-like protuberance, there is normal skin, but cartilage and muscle can also be found , 10.Treatment of MCC involves surgical resection and proper reconstruction. Delay in treatment of MCC changes the growth of the lower third of the face and particularly affects mandibular development and extension of the neck . ReconstGiven the rarity of MCC, we decided to report this case. Our findings are consistent with the literature. Our patient has recovered uneventfully from the surgical treatment.MCC is an unusual congenital defect that may be encountered by the otolaryngologist, pediatrician, pediatric surgeon, or other health care professional. Surgical excision and reconstruction of the entire defect with multiple Z-plasty reconstruction yields good cosmetic results while improving neck mobility and reducing the risk for long-term cervical tethering and mandibular defects.


HLXB9 mutation in chromosome 7q36 in 50% of cases. The disorder is mostly hereditary as it can also be sporadic with a variable expression spectrum.Currarino syndrome is a rare congenital disorder characterized by a triad of anorectal malformation, a sacral bone defect, and a presacral mass. It results of an abnormal separation of the ectoderm from the endoderm caused by The case of a previously healthy 3-month-old girl with abdominal distension, post-prandial vomiting, obstipation, and anuria of 5 days’ history is presented in this article. Abdomino-pelvic magnetic resonance imaging (MRI) showed a large cystic multilobulated mass in the sacrococcygeal region with a dural communication evident of an anterior sacral meningocele. 1 year later, the child came back with constipation and was found to a have a malignant mixed germ cell tumor in the presacral area, a very rare presentation in Currarino syndrome.In a child presenting with at least one of the features of Currarino syndrome’s triad, a diagnosis should be suspected. After reviewing the literature, the syndrome is usually missed and hence is under diagnosed. MRI is the best imaging modality for diagnostics and follow-up for any mass, benign or malignant, can bring life saving measures. Most masses are benign but can undergo malignant transformation even after resection. De novo malignancy is very rare and is described in our case.Physicians treating patients with spinal dysraphism should suspect a diagnosis of Currarino syndrome by follow up imaging for any new benign or malignant growth. It is the result of an abnormal separation of the ectoderm from the endoderm caused by of cases . The disof cases .2A previously healthy 3-month-old girl was transferred to our hospital for severe abdominal distention, post-prandial vomiting, obstipation, and anuria for the last 5 days. CT scan done prior to presentation at another hospital showed a cystic abdominal mass displacing the girl’s bowels, bladder globus, and bilateral hydroureteronephrosis (not shown). Her creatinine level was elevated reaching 4.99 mg/dl upon admission. An abdomino-pelvic MRI showed an 8.2*3.7*3.2 cm homogenous cystic multilobulated pelvic mass in the sacrococcygeal area with a 1.5*0.4*0.6 cm dural canal communicating with the mass at the S4-S5 level consistent with an anterior sacral meningocele . To restSurgical resection was performed on the fifth day with a posterior approach starting with an incision from S3 to the coccyx and a laminectomy to expose the sacral canal. The dural communication was ligated from the rest of the thecal sac followed by cyst cerebrospinal fluid drainage.The next day, post-operation echography showed residual cysts in the intra-abdominal cavity. Abdominal laparoscopy was done on the eleventh day to drain the remaining cysts which enabled urinary foley removal and patient’s discharge symptoms free.A year later, the child presented back with constipation. A lumbosacral MRI showed a solid lesion of 7.5 cm of height and 6.5 cm of diameter associated with adenopathies . An ingu3HLXB9 homeobox gene on chromosome 7 (7p36) was found to be involved in 90% of familial Currarino [Currarino syndrome is an autosomal dominant congenital malformation defined by a triad described by Currarino et al. in 1981 consisting of a sacral bony defect, a presacral mass, and an anorectal malformation or constipation  with onlome 7 7p3 was founThe presacral mass can be anterior sacral meningocele (ASM), teratoma, and/or neuro-enteric, dermoid, and epidermoid cysts. ASM occurs as a result of a sacrococcygeal defect or herniation of the meningeal sac from the sacral foramen to the anterior and occasionally with neural elements into the pelvis presenting as a presacral pelvic mass . Our patAlthough rarely symptomatic, local pressure by the ASM may cause constipation, obstipation, and urinary retention. Second, local neurological deficits may present as poor sphincter control, sacral anesthesia, or lower extremity deficit. Finally, central neurologic symptoms may present as nausea and headache with straining due to increased abdominal pressure on the meningocele. Our patient presented with severe abdominal distention, acute urinary retention, obstipation, and post-prandial vomiting, due to the increased pressure in the abdomen from the ASM that was detected by MRI evaluation . NeurosuPart of the Currarino triad is anorectal malformation causing constipation or obstipation which pathogenesis is attributed to either mechanical compression by a presacral mass, a tethered cord, a sacral nerve roots compression, or an anteriorly located anus ,8,10. ThMRI is best imaging modality in detecting the communication between the ASM and the spinal subarachnoid space due to high soft tissue contrast and multiplanar imaging. It also helps exclude any other pathologies associated with ASM such as a presacral mass . Bony deMalignancy is very rare in Currarino syndrome and may involve malignant transformation of a benign mass with only 10 cases being reported, 6 of them during childhood . The ratNothing to declare.Balamand University will only pay for publishing fees for the journal if manuscript is accepted; otherwise no funding was involved in the writing and conduction of the article.The study is exempt from ethical approval by our institution.Written informed parental consent was obtained on behalf of the patient since patient is a minor 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.-Dr. Paul Hage and Dr. Cedric Kseib: study concept, data collection, data analysis, writing of the manuscript.-Dr. Reva Matta: data collection and interpretation.-Dr. Carmen Adem: imaging studies provision and interpretation.-Dr. Camil J. Chouairy: pathology images and interpretation.Nothing to declare.Dr. Paul Hage, Neurosurgeon.Not commissioned, externally peer reviewed.