Multifocal soft tissue sarcoma is a rare clinical entity occurring in 1 % of patients with extremity soft tissue sarcoma and in 4.5 % of patients with liposarcoma. Multifocal disease may arise either synchronously or metachronously and has been associated with poor prognosis.Herein, we report a rare case of a Syrian woman patient with six different foci of liposarcoma in five sites at the time of diagnosis.Liposarcomas are currently classified into four different subtypes based on histologic or genetic analysis according to the World Health Organization (WHO), including cell-differentiated, dedifferentiated, myxoid, and pleomorphic. In the present work, we report a neglected patient with a multicentric round cell liposarcoma in five different sites.Medicine cannot provide much care for these advanced cases of multicentric liposarcoma so far. Therefore, more research should be conducted to improve the ability to manage these entities and to identify potential novel therapies. It typically affects patients between the fifth and seventh decade of life and usually develops in the extremities or retroperitoneum [Multifocal disease is a rare clinical entity occurring in 1 % of the patients with extremity soft tissue sarcoma ,3 and inMultifocality in soft tissue sarcoma is defined as the development of a soft tissue sarcoma in two or more separate anatomical sites before the manifestation of the disease in common metastatic sarcoma sites, particularly in the lungs ,4.There are no significant differences in sex predilection, age, grade, and depth margins between multifocal and unifocal disease ,3.They can be either synchronous or metachronous and are generally associated with an aggressive clinical course and poor prognosis ,5.These fat tumors, of ubiquitous localization, commonly appear as a slowly enlarging mass with a misleadingly benign appearance. However, any soft-tissue tumor requires the need for a thorough preoperative X-Ray investigation  and a biHerein, we report a rare case of a Syrian female patient with six different foci of liposarcoma in five locations that developed six months after the resection of a thigh liposarcoma.This work has been reported in line with the SCARE criteria .2A 37-year-old Syrian woman came to our clinic complaining of four large masses in the leg, thigh, neck, and abdomen. The complaint started 30 months ago as a small hard mass in the upper of the right thigh. The patient underwent a complete resection of the mass. Histological examination showed that it was a liposarcoma. Six months later, other four masses appeared in abdomen, neck, thigh, and leg. The masses showed rapid growth. During the last twenty-four months, the patient was neglected because of the crisis that affected every part of Syria. She was not followed up by regular imaging. Even after the masses were visible and large, she did not receive any therapy until she got presented to our clinic. Her family history was remarkable. Her brother and sister had lipomas. However, genetic studies were not performed because they are expensive and not available in our country. Furthermore, her drug and psychosocial history was passive.Clinical examination revealed that the masses were hard, fixed and large . Full blA surgical biopsy from the thigh mass showed high-grade liposarcoma with prominent round cell component .Fig. 3HiBecause of rapid recurrence and multifocal disease, the multidisciplinary team suggested chemotherapy with Doxorubicin and Ifosfamide. The patient was given five cycles of chemotherapy. Postchemotherapy follow-up showed a decreasing by 30 % in the masses size. Two months after chemotherapy, the patient underwent two major surgical operations. The first one was for resecting abdominal and cervical masses and the other one was for resecting femoral and popliteal masses . ActuallThe first procedure was performed by a general surgery specialist with 15 years of experience in abdominal surgery. Abdominal incision showed seven tumor masses; a large retroperitoneal mass, an under left kidney mass, a peripheral retroperitoneal mass connected to the upper pole of the right kidney and compressing the inferior vena cava, two pelvic masses adherent to the right ovary and greater omentum, a small mass connected to the transverse colon, and a mass invading the sigmoid colon mesentery. They measured 30, 5, 10, 15, 5, 4 and 10 cm respectively. Therefore, the resectable masses were totally resected and the other masses were debulked. The cervical mass was only debulked to save neural and vascular elements and to avoid extremity amputation.Seventeen days later, the femoral and popliteal masses were resected after isolating the neurovascular bundles and nerves. This procedure was performed by an orthopedic surgery specialist with 10 years of experience in extremity cancer surgery.The pathologic report was liposarcomatosis (myxoid type). Unfortunately, a day after surgery the vital signs of the patient deteriorated. She was transformed to the surgical intensive care unit (ICU) where she died.3Liposarcomas are currently classified into four different subtypes based on histologic or genetic analysis according to WHO, including cell-differentiated, dedifferentiated, myxoid, and pleomorphic . The rouAs myxoid and round cell tumors share the same cytogenetic abnormalities, namely the translocation t   leading to the fusion of the genes DDIT3 and FUS with the generation of a hybrid protein FUS/DDIT3, some authors consider both lesions as a continuum of the same disease. This possibility seems to be supported by the frequent finding of areas of round cells in myxoid liposarcomas, which has been considered a marker of poor prognosis when representing 5 % or more of the mass in localized myxoid liposarcoma [With our patient, the specimen was not suited for cytogenetic analysis.This entity seems to be rather infrequent and some authors consider it different from ordinary liposarcoma due to both multicentric presentation and more aggressive behavior .The first reported case of multifocal soft tissue sarcoma dates back to 1934 when Siegmund described a patient with multiple fatty tumors and coined the term “Lipoblastische Sarcomatose.” Since then, less than 50 cases have been reported in the literature, mostly in the form of case studies. The largest series was reported in 1962 by Enzinger, who described 20 cases in whom the tumors were exclusively of the myxoid and round cell types, exhibiting a different pattern of spread [Debate still exists as to whether this entity represents a rare variant of an already rare disease or whether it is simply a more unusual metastatic pattern .Interestingly the multicentric tumors tend to spare classical metastatic sites of sarcomas, like the liver, the lungs or the bone and affect rare locations, like the pleura or the lymph nodes, but in our patient; the previous sites were not affected. This fact seems against a possible metastatic explanation for multicentricity. The identification of the same cytogenetic abnormalities in all the multicentric lesions cannot be considered either definite proof of their metastatic origin, for they might still be multicentric synchronous or metachronous lesions related to a common aetiopathogenic factor .In all three groups, complete surgical resection is central in treatment aimed at cure and is based on grade. Radiation can reduce risk of local recurrence in high-grade lesions or minimize surgical morbidity in the highly radiosensitive myxoid/round cell liposarcoma (M/RCLS) group. The biologic groups differ greatly in their chemosensitivity, so adjuvant chemotherapy is selectively utilized in chemosensitive histologies with metastatic potential (i.e. round cell and pleomorphic liposarcomas) but not in the relatively resistant subtype dedifferentiated liposarcoma (DDLS). An improved understanding of the genetic aberrations that lead to liposarcoma initiation is also allowing for the rapid development of targeted therapies for liposarcoma. Among such therapies are CDK4 inhibitors in well- and dedifferentiated liposarcoma (WD/DDLS) and trabectedin, which prevents FUS-DDIT3 binding to DNA, in (M/RCLS) .With our patient surgery was not performed initially because of rapid recurrence and infiltration of large vessels and neural plexus, so that she was put on a palliative chemotherapy. After chemotherapy, surgical treatment was done.As prognosis seems to be poor, chemotherapy and radiotherapy seem indicated both in the neoadjuvant or adjuvant settings. As these multicentric tumors are rare, it is difficult to determine which regimen could be the best in these patients. Nevertheless, the literature does not indicate a significant improvement of the outcome regardless of the chosen therapy .4In the present work, we report a neglected case of multicentric liposarcoma that developed in five different sites after the resection of the primary mass. For these advanced cases, medicine cannot provide much care so far and prognosis remains poor. So, we should conduct more research to improve our understanding of how each subtype of tumor responds to different therapies. By that, we can manage these entities and identify potential novel therapies.Family members should undergo periodic genetic tests for early detection of the potential cases, so they can receive early treatment.We should also draw attention to the importance of establishing referral centres to provide treatment to such advanced cases.These cases should be managed by a multidisciplinary team in order to increase the opportunity of total recovery and to reduce recurrence.Unfortunately, such advanced cases are a result of decreased general healthcare which is expected in time of crisis as happened in our country.The authors declare that the research have no funding sources or sponsors.The authors requested a full waiver for the article processing charges.Not required for case reports at our hospital. Single case reports are exempt from ethical approval in our institution.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.Ieman Alawad, Amna Ibrahim, Ruqaya Masri: Design the study, data collection, drafting, revision.Ahmad Al-Haj, Ahmad Ghazal: The Supervisors, patient care, revising critically.All authors discussed the content of the manuscript, read and approved the final manuscript.Not applicable. This case is not a part of any clinical study.Dr. Ieman Alawad.Not commissioned, externally peer-reviewed.The authors declare that they have no conflicts of interest.


Foreign body ingestion is an important problem in adult with psychological disorders. In literature ingestion such as fish bone, fork and several metallic elements were reported. The first attempt, after diagnosis, is endoscopic removalA 25-year-old male deaf patient was admitted in emergency department with complaint of abdominal pain and unable to communicate anything of his clinical history. Physical examination was normal except an epigastric tenderness. Laboratory results were normal. Direct radiography revealed a circular shaped metallic object in stomach and other several metallic objects in gastrointestinal tract A. An emeA delay in the diagnosis an dextraction of sharp or large sized foreign objects can lead to severe complications including mucosal laceration, obstruction, hemorrhage, and perforation. Therapeutic esophagogastroduodenoscopy with an esophageal overtube should be the first choice retrieval of large sized foreign bodies to avoid mucosal laceration, perforation and the surgical treatment.


Primary gestational choriocarcinoma in a uterine caesarean section scar (CSS) is an extremely rare entity, and its timely diagnosis and treatment is crucial in order to prevent related complications and metastatic disease. Herein, we report on a 33-year-old female who was referred to our department with an initial diagnosis of ectopic pregnancy. Transabdominal ultrasound and magnetic resonance imaging (MRI) demonstrated a nodular mass on CSS. The final histopathological diagnosis was CSS choriocarcinoma. Choriocarcinoma is a rare form of gestational trophoblastic disease (GTD), and its most common site of origin is the uterus. It may be associated with ectopic pregnancies, but these cases are extremely rare. There is only one case report in the literature presenting the transvaginal ultrasound findings of a caesarean section scar (CSS) choriocarcinoma . Herein,A 33-year-old female, gravida 2 para 1, was referred to our university hospital from a local medical clinic with a history of a positive serum beta-Human chorionic gonadotropin (β-hCG) level but no visible intrauterine gestational sac. Her laboratory findings were all normal except for the serum β-hCG, which was 146.762 mIU/mL. A gynecological examination revealed a closed cervical os and mild adnexal tenderness. The patient had regular 28-day menstrual cycles. Her first pregnancy had ended with a full-term delivery by caesarean section approximately nine years earlier. There was no history of cancer in her family.A grayscale transabdominal ultrasound showed an empty uterine cavity, but a round hyperechoic lesion was identified in the anterior inferior wall of the uterus, measuring 23x32 mm in diameter Figure . There wThe β-hCG level that became available to us on the twenty-third day of the treatment was still as high as 15.947 mIU/mL, and it then rose again. Therefore, the patient underwent an magnetic resonance imaging (MRI) examination, which demonstrated a peripherally hyperintense and centrally hypointense lesion on coronal and sagittal T2-weighted images of the uterine CSS. After gadolinium administration, the lesion demonstrated peripheral contrast enhancement on T1-weighted fat-suppressed axial images . She tolerated the therapy well, and weekly serum β-hCG measurements showed a significant drop, from 12.459 mIU/ml after the surgery to 0.7 mIU/ml following chemotherapy. A follow-up CT scan showed a significant reduction in the size of the lung metastases.Gestational trophoblastic disease (GTD) is the abnormal proliferation of pregnancy-associated trophoblastic tissue with malignant potential . It inclAlthough it is extremely rare, choriocarcinoma must be considered in the differential diagnosis of ectopic pregnancies within cesarean scars. An early diagnosis requires high suspicion, and it is crucial to reduce the potential complications. It is difficult to predict the prognosis of choriocarcinoma in CSS, as there are very few reported cases, but when we consider the aggressiveness of this type of lesion, life-threatening complications such as uncontrollable hemorrhage and uterine rupture can be presumed. Therefore, it is crucial to make a prompt and accurate diagnosis.Hydatidiform mole has the sonographic appearance of a solid collection of echoes with numerous anechoic spaces . When thIn conclusion, CSS choriocarcinoma is an extremely rare entity, and imaging and a timely diagnosis are the keys to its successful treatment and the prevention of complications. By presenting a choriocarcinoma case within the CSS, we aimed to draw the attention of both radiologists and clinicians, who should consider this rare entity in the differential diagnosis of cesarean scar pregnancies.The authors declare that they have no competing interests.


Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2.The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure .Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases.How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166. Portal venous development is completed in the 4-10 weeks of embryonic life.2 There are two types of shunts; type 1 is flowing of the entire portal blood to the VCI in relation to the congenital absence of portal venous, whereas type 2 is opening of the portal vein, which is intact but deflected, to the VCI by side-to-side or extrahepatic portosystemic shunt.3 Type 1 Abernethy malformation appears more frequently in females and is associated with other congenital anomalies such as cardiac defects (60%), biliary atresia (20%), polysplenia (20%), situs inversus (10%) and malrotation.3 Type 2 shunts are rarely encountered, mostly seen in males and generally unrelated to other congenital anoma-lies.3 The clinical findings of Abernethy malformation are various; while it can be seen in complicated cases such as hyperammonemia, hypoglycemia, hepatic encepha-lopathy, hepatopulmonary, and hepatorenal syndrome, it is occasionally seen in the asymptomatic course and incidentally diagnosed by imaging methods.45John Abernethy first described portal venous congenital absence and superior mesenteric-vena cava inferior (VCI) shunt in 1793 as a postmortem in a 10-month-old female, and it was called Abernethy malformation.Here, we present a case of a female with biliary atresia and VSD associated with Abernethy malformation type 2, who was diagnosed during the neonatal period and who was a candidate for liver transplant in the infantile period, because of differing from the literature and being a rare case.As the first infant living in the third pregnancy of a 23-year-old mother, born by cesarean section in the weight of 2280 gm and having asymmetric intrauterine growth retardation, the female baby was referred to our unit with the diagnosis of neonatal cholestasis on the postnatal day 10, due to having acholic stool since birth and development of hyperbilirubinemia on the postnatal day two. The body weight was 2275 gr (3-10%), the height was 48 cm (10%), the head circumference was 33 cm (10-50%), the vital findings were; heart rate was: 124/min, blood pressure was: 70/48 (58) mm Hg, fever was: 36.7° C (axillary), and respiratory rate was: 52/min, spO2: 98%. In the physical examination, sclera and skin of the case were icteric and dirty-yellow looking; the case had hypertelorism, slanted eyes, and discrete hand fingers; and cardiac 3°/6 systolic murmur was detected. During the abdominal examination of the case who had no respiratory complaints, the liver and the spleen were not palpable. Laboratory investigations showed that aspartate aminotransferase (AST): 35 IU/L, alanine aminotransferase (ALT): 10 IU/L, albumin 3,3 gr/dL, alkaline phosphates (ALP) :280 IU/L, lactate dehydrogenase: 854 IU/L, blood glucose : 80 mg/dL ammonia: 80 mcg/dL values were normal; total bilirubin(t bil): 8mg/dL, direct bilirubin(d bil): 4.05 mg/dL, gamma-glutamyltransferase (GGT): 1185 IU/L values were high. Complete blood count, bleeding profile, blood gas, urinary examination were normal; infection criteria were negative. Abdominal ultrasonography (US) revealed a cystic structure without a real wall structure with an irregular contour of about 3 cm on the gladder ball wall. The lesion was evaluated in accordance with the pseudo biliary, and there was no evidence of choledochus in the case who had a finding of triangular cord at the level of the right-left hepatic venous junction. These findings were evaluated as compatible with biliary atresia. However, besides biliary atresia, the portal venous whose diameter was getting thinner was seen to open to the VCI without forming an intrahepatic branch after a short follow-up of portal hilus. MR angiography confirmed the diagnosis of biliary atresia, and abdominal CT angiography confirmed Abernethy malformation type 2  and 2. TExploration was performed to the case on the postnatal day 35. During the intraoperative follow-up, the gallbladder was atrophic, the liver did not have a cirrhotic appearance, but the main hepatic ducts were fibrotic, and portal vein was getting thinner at the hepatic hilus level and continued as an obliteration structure and also showed a slight extension to the VCI. Tip-to-tip anastomosis was performed between the portal vein and the umbilical vein by planning recanalization of the portal vein into the rex area of the left portal vein into which the umbilical vein flowed so that the portal vein could provide adequate blood supply. Intraoperative Doppler US showed that there was a slight flow in the umbilical vein; however, it was determined that sufficient revascularization could not be achieved because the segmented portal branches extending from the rex region into the liver were flebo-obliterated. The operation was completed with hepatic portoenterostomy (Kasai procedure) -5.The two-month-old case who was re-hospitalized due to weight loss; had a body weight of 2950 gr (<3%) and a height of 50 cm, the infection criteria were negative, AST/ ALT: 325/144 IU/L, GGT: 102 IU/L, T bil/D bil: 1,44/ 8.9 mg/dL, bleeding profile was usual, nutrition was re-regulated by increasing calorie support.The five-month-old case which applied to the emergency service due to bloody stool, bloody vomiting, the tendency to sleep had tachycardia, tachypnea, and abdominal distension. A patient with massive upper-lower GIS bleeding due to portal hypertension and hemorrhagic shock had hemoglobin: 5 gr/dL, INR:> 2,5, AST/ALT: 1202/544 IU/L, ALP: 677 IU/L, GGT: 43 IU/L, TIV/DIV: 15/7 mg/dL. Treatment for hypovolemic shock was performed and hemorrhage control was provided at the end of the second day.Correspondingly, preparations were started for the case of a liver transplant candidate at postnatal month six due to the progression of chronic venous occlusion related to accompanying Abernethy malformation type 2 despite Kasai operation, and because the liver became fibrotic at the infantile stage and liver failure developed.6 In the intrauterine embryonic period, connections between the right and left vitelline venules occur at the end of the 4th week, and intrahepatic and extrahepatic portal veins develop from these connections. As a result of the inadequacy of anastomosis, portosystemic and portoportal shunts occur. There are two types of portosystemic shunt anomalies, and there is no intrahepatic portal vein in type 1 shunt, and there is a complete end-to-side shunt. Type 1 shunts are divided into two subgroups as type 1A; superior mesenteric vein and splenic vein flows into the VCI, the iliac vein, and the renal veins separately, and as type 1B; superior mesenteric vein and splenic vein are joined together to form a short extra hepatic portal vein.7 In type 2 shunts, there is a patented intrahepatic portal vein and there is a partial side-to-side shunt in the portal vein.6 The literature does not usually make this distinction, and Abernethy malformation is used synonymously with congenital absence of portal vein.6Congenital absence of portal vein; is a rare condition in which intestinal and splenic venous blood bypasses the liver and drains directly into the systemic circulation with a venous shunt.8 Type 2 malformations in which portosystemic encephalopathy or portal hypertension is observed can be closed by surgical ligation or invasive embolization.8 Asymptomatic cases diagnosed incidentally by imaging methods can be followed up with intermittent controls.8In our case, after a short follow-up, portal vein is opened to VCI through the portal hilus without forming an intrahepatic branch, and the diameter gets thinner; the shunt has been evaluated as Abernethy malformation type 2. Identification of the malformation type is important in terms of determining the treatment algorithm. Concomitant hepatopulmonary syndrome, liver dysfunction, type 1 malformations due to focal nodular hyperplasia may require tumor resection and/ or liver transplantation.3 Even though it is a type 2 shunt, our case is different from the literature because of the presence of biliary atresia, VSD and valvular pulmonary stenosis, and the female gender.Type 1 shunts are associated with other congenital anomalies such as cardiac defects, biliary atresia, poly-splenia, and liver tumors, defined only in females, while type 2 shunts are; rarely seen in males and independent of other congenital anomalies.910 With regard to the literature, live donor liver transplantation due to Abernethy malformation has been performed in 11 cases in the pediatric population so far.13 Kasai operation has been performed during the neonatal period in cases accompanied by biliary atresia, and liver transplantation has been performed to these cases, even in the case of biliary atresia, in the late childhood or adolescent age.11 In our case, although Kasai procedure was performed in the neonatal period and portal vein reconstruction was performed at the same session, due to the accompanying Abernethy malformation Type 2, portal vein hypertension and liver fibrosis developed when the case was six months old and became a transplant candidate in the infantile period.Abernethy malformation alone does not create an indication for liver transplantation. If there are no concomitant coexisting anomalies, it can be detected incidentally in many cases during surgery or during imaging methods such as US, CT or MR for liver disease.Despite the expectation of an increase in postoperative morbidity and mortality in association with biliary atresia, Abernethy malformation, and cardiac defects, this association does not cause a contraindication for liver transplantation. In earlier periods of life, transplant requirement may develop. Patients with neonatal cholestasis should be assessed using advanced imaging methods during the presence of biliary atresia, one of the first anatomic disorders that should be excluded, in terms of the additional vascular anomalies that accompany this condition, because it changes the course of the disease, and the success of Kasai operation, and it should be kept in mind that there may be portosystemic and portoportal shunts affecting the liver blood flow.As a result in neonatal cholestasis cases, the rapid and accurate diagnosis should be made with advanced imaging methods, and anatomical portal vascularity should be evaluated in detail. In the case of identification of Abernethy malformation accompanied by biliary atresia, it is essential to monitor the cases in well-equipped centers where living donor liver transplantation can be performed, considering postoperative complications.


The de Garengeot hernia is an uncommon and potentially confounding pathologic process in which the appendix is contained within a femoral hernia. While typically a benign incidental finding, superimposed acute appendicitis is a rare but serious complication. Identification of this entity is crucial to patient management and ultimately outcome with imaging playing a critical role. Cross-sectional imaging, with either CT or MRI, should be performed in all cases of suspected incarcerated de Garengeot hernia to facilitate the appropriate diagnosis and surgical intervention. Herein, we present the fifth case of a prospective CT diagnosis of the de Garengeot hernia in a 61-year-old female who presented with an irreducible right femoral hernia. The patient underwent CT examination which demonstrated the appendix within the femoral hernia sac with an associated periappendiceal fluid collection. The patient was taken for emergent surgical intervention at which time the appendix was found within the hernia sac. The appendix was removed, the defect repaired, and ultimately the patient recovered well. In 2005, Akopian and Alexander first termed a femoral hernia containing the appendix the de Garengeot hernia (DGH) after French surgeon Rene de Garengeot, who is credited as the first to describe this entity in 1731 . DGHs arμL. A contrast-enhanced CT of the abdomen and pelvis was obtained for further evaluation which demonstrated a fat- and fluid-containing right femoral hernia (Figures A 61-year-old female with a past medical history of hypertension, HIV, and hepatitis C presented to our hospital with a 1-week history of right groin tenderness and a palpable lump in her right groin. The patient stated that the lump had been growing in size since its sudden appearance approximately 1 week prior. The patient denied fever, nausea, vomiting, or changes in her bowel habits. Physical examination of the right groin, demonstrated a minimally tender irreducible hernia without overlying skin changes. Laboratory values were within normal limits aside from a mildly elevated white blood cell count of 7700/ Figures . The app Figures .The patient recovered well, without complications, and was discharged home one day postoperatively.Abdominal wall hernias occur secondary to a weakness in either the abdominal wall musculature or peritoneal fascia which allows for the projection of intraperitoneal contents through the defect. The inguinal region is predisposed to two types of hernias: inguinal and femoral. Femoral hernias account for 3% of all external hernias and occur within the femoral sheath, below the inguinal ligament . DefinedThe etiology of DGHs remains disputed. Hussein et al. and Sharma et al. suggested that during embryological development abnormal bowel rotation results in an atypical appendiceal attachment, thus the cecal appendix is able to migrate into a femoral hernia , 14. ZisTypically, the DGH presents with symptoms of incarcerated or strangulated groin hernia. Patients often have a nonreducible groin lump that is usually painful and/or tender to palpation, with erythematous and sometimes edematous overlying skin. Signs of a systemic inflammatory response including fever and elevated white blood cell count are also common. While more unusual signs and symptoms of bowel obstruction or bowel ischemia have been reported, they are rare occurring in only 14% of patients , 13.The herniated appendix can result in appendicitis and eventually perforation and abscess formation. In a retrospective review of NCBI PubMed literature utilizing the keywords “de Garengeot hernia,” “appendix in femoral hernia,” and “perforation” yielded 15 cases reported to date of a perforated appendix within a femoral hernia sac . TheorieThe diagnosis of a DGH is difficult to make preoperatively, and only 4 reports of a preoperative CT diagnosis having been reported in the literature to date; herein, we present the fifth case , 4–6, 15It should be noted that the de Garengeot hernia should not be confused with the Amyand hernia which is an appendix containing inguinal hernia . CT may Ultrasound findings of DGH can show the appendix as a blind-ending tubular structure surrounded by fluid and echogenic material within the hernia in the inguinal region . As descGiven the rarity of DGH, no standard approach to treatment has been described in the literature. There are several methods of surgical repair described in the literature including a Lockwood's infrainguinal, Lotheissen's transinguinal, and McEvedy's high incisions and laparoscopic repair . In a reThe most common complication reported following DGH repair is wound infection, occurring in up to 29% of patients , 14. ConDGHs, although rare, should remain a consideration in elderly female patients presenting with inguinal pain. This case demonstrates the importance and value in obtaining preoperative imaging not only to aid in preoperative planning but to ensure the correct diagnosis and limit unexpected complications both in the operating room and postoperative setting.


There are three major mechanical complications after acute myocardial infarction: left ventricular free-wall rupture, ventricular septum rupture and acute mitral valve regurgitation. The left ventricular free-wall rupture is a serious and often lethal complication following an ST elevation myocardial infarction. However, very rarely this rupture can be contained by the pericardium, forming a pseudoaneurysm.We report a case of a 66-year-old man with multiple cardiovascular risk factors and previous ST elevation myocardial infarction, complaining of atypical chest pain. His electrocardiogram was in normal sinus rhythm, with the presence of Q wave in inferior leads and T-wave inversion in lateral leads. A transthoracic echocardiogram showed a left ventricular pseudoaneurysm. In the coronary angiography, multi-vessel disease was found. On-pump CABG was performed and a posterolateral left ventricular giant pseudoaneurysm were observed. Due its “petrous” consistency it was impossible to perform an aneurysmectomy.The diagnosis of left ventricular pseudoaneurysm can be difficult, as patients often present either asymptomatic or with non-specific symptoms attributed to other causes. A multimodality imaging diagnostic approach can be necessary. Immediate surgery is considered the treatment of choice because untreated pseudoaneurysms have a high risk of rupture leading to cardiac tamponade, shock and death. There are three major mechanical complications after acute myocardial infarction: left ventricular (LV) free-wall rupture, interventricular septum rupture, and acute mitral valve regurgitation . Acute oThese are rare complications . LV freeA 66-year-old man with multiple cardiovascular risk factors; such as diabetes, hypertension, smoking and STEMI 6 years ago, who did not receive a reperfusion therapy. He consulted the ER referring atypical chest pain that began 8 days prior to his visit. He showed normal vital signs with the following relevant findings in the cardiovascular physical examination: visible and palpable double systolic apical impulse with a wide apical impulse area , located in the fifth intercostal space of the left mid-clavicular line. On auscultation, an audible fourth heart sound (S4) was present. The cardiac biomarkers were negative.Normal sinus rhythm, Q-wave in inferior leads and T-wave inversion in lateral leads. Fig. .Fig. 112Mild cardiomegaly, of note, a homogeneous opacity was observed adjacent to the LV. Fig. a.Fig. 2aRevealed a saccular image in the LV posterolateral wall, which, due to its characteristics, suggested a thrombosed PSA in the LV free -wall. Fig. b.Showed a spherical-shaped left ventricular cavity with segmental wall-motion abnormalities, a LV ejection fraction of 40% by 3D method,  plus aneurysmectomy with geometric reconstruction was considered. On-pump CABG was performed with internal thoracic artery anastomosis to the left anterior descendent coronary artery. A giant posterolateral wall LV PSA of approximately 7 cm was observed; described with a “petrous” consistency and adhered to the posterior pericardium. Due to the previously mentioned findings, it was not possible to perform the aneurysmectomy, as the cardiovascular surgical team considered the benefit was not worth compared to the risk of performing the procedure, so the surgery was concluded. The patient was discharged five days after CABG and he is currently asymptomatic, in NYHA functional class II and is followed up as an outpatient in our hospital.We discuss the case of a 66-year-old male with a history of MI, six-years prior to consulting our hospital; that due to lack of access to a cath lab, the patient didn’t receive any reperfusion strategy. Thus, the patient only received medical treatment as an inpatient in a 2nd level hospital and the initial symptoms associated to the pseudoaneurysm were probably considered as part of the symptoms related to an acute MI.Myocardial rupture usually occurs within the first 3–5 days after the infarction in half of the cases (early rupture) and in the first two weeks in 90% (late rupture) . The cliThe LV free-wall rupture can be complete or incomplete . CompletLV PSA is a rare disorder but of great clinical relevance due to its high associated mortality . It usuaThe diagnosis of LV PSA can be difficult because patients are often either asymptomatic, or present with non-specific symptoms attributable to other causes. It can even be diagnosed incidentally. A multimodality imaging diagnostic approach can be necessary, including transthoracic/transoesophageal echocardiogram, cardiovascular magnetic resonance (CMR), chest CT, myocardial perfusion imaging (MPI) and LV angiography , 9.European Society of Cardiology guidelines for the management of STEMI establish that the diagnosis is confirmed with a TTE. These guidelines consider CMR can complement the diagnosis by identifying the contained cardiac rupture and its anatomical features to guide surgical interventions . In thisImmediate surgery is the treatment of choice, because an untreated PSA has a 30 to 45% rupture risk . The patThe present case report is interesting because it is unusual to find this mechanical complication of MI. Its low incidence is probably secondary to a greater accessibility to primary PCI (which has been previously described as a protective factor). This patient didn’t receive any reperfusion therapy (fibrinolysis/PCI), before attending our Centre; therefore, he was more likely to present this potentially fatal complication. In addition, the multimodality cardiac imaging used in this case, is very illustrative of this MI “serious” mechanical complication.When encountered, PSA requires immediate surgery because an untreated LV PSA has a high risk of rupture, leading to cardiac tamponade, shock and death. CABG plus aneurysmectomy with geometric reconstruction was considered for this patient following guideline recommendations; however in this case, only successful CABG was performed; the “petrous” consistency of the PSA made aneurysmectomy impossible to perform.Finally, although a high mortality without surgery is reported, our patient maintains a favorable evolution two-years post CABG; he is currently in NYHA II functional class, receiving optimal medical treatment and regular follow-up visits as an outpatient in our Centre.In conclusion, the LV free-wall rupture is a serious and often lethal complication of STEMI resulting a common finding in patients who die from acute MI. LV PSA is a rare disorder but of great clinical relevance due to the high associated mortality. The diagnosis of LV PSA can be difficult and often requires a multimodality imaging diagnostic approach. This MI “serious” mechanical complication can be prevented by primary PCI, however, when it is found, warrants surgical treatment. This particular case is atypical. The long evolution of this complication made surgical resection impossible, despite this setback, the patients’ evolution was favorable.


Spigelian Hernia is an uncommon pathology of abdominal wall (0.12–2.4%), usually small sized and with vague symptoms. It rarely presents as Small Bowel Obstruction or reaches dimensions that becomes clinically remarkable.84-year-old woman entered our Surgical Department for Small Bowel Obstruction due to a giant (8 × 7 cm) abdominal wall hernia, which was intraoperatively identified as Spigelian Hernia. We performed a minilaparotomy with reduction of viable small bowel and preperitoneal positioning of polypropilene mesh. Postoperative course was uneventfull.Due to its small dimensions and infrequence, the diagnosis could be challenging even if the patient undergoes a CT scan. The presentation with clear signs of small bowel obstruction associated with a large abdominal hernia is rare and suggests a urgent surgical approach with mesh repair to avoid recurrences.Even if rarely symptomatic, the Spigelian Hernia is an entity to consider in the differential diagnosis of small bowel obstruction in a virgin abdomen. Preoperative diagnosis, when available, is mandatory to guide a correct surgical approach. Her patency was absent from 3 days. She referred the appearance of a bulging mass in the left lower quadrant of the abdomen,Her medical history was dominated by COPD, morbid obesity and cardiologic arrhythmia (treated with DOAC).Blood exams revealed mild leukocytosis  located in the left lower quadrant with tenderness and impossibility to obtain manual reduction. Auscultation revealed tympanic movement of bowel .Fig. 1CTAfter placement of a nasogastric tube, the patient underwent an abdominal CT scan which revealed several loops of small bowel introducing into a large defect (3 × 3 cm) on the left lateral abdominal wall, at the level of the iliac crest, and causing a SBO of the upper part .Fig. 2InInitial fluid resuscitation was performed and the patient was scheduled for urgent surgery.Laparoscopic approach was not proposed because of the comorbidities and the high abdominal distension.Minilaparotomy (5 cm) was performed under the umbilicus and identified the hernia sac, which was easily isolated from subcutaneous tissues and the muscolofascial plane. Opening the hernia sac, a small amount of clear fluid went out along with 60 cm of ileal loops which was viable and needed no resection . The smaPostoperative was uneventfull; patency was obtained on 2nd postop day and the patient was discharged on 3rd with a semiliquid diet.3Even if it is a rare hernia , incarceration is possible in a relevant percentage of case (17–24%) [The most difficult part in the emergency setting is the diagnostic part: clinical history and examination are usually little helpful because of SH may cause vague abdominal pain which is often referred to other causes and, moreover, its intraparietal location makes SH not palpable from the clinician in almost 36% of cases [Clinical suspect can be reinforced from a persistent abdominal pain and tenderness in the Spigelian point, but a correct diagnosis is impossible without imaging: US is a first-line useful method, especially in emergency settings, but if available the gold standard for diagnosis is still represented by CT scan, even if it has up to 32% of false negative ,7,8.Certainly, clinical presentation of SBO associated with bulging mass in the abdominal wall is very suggestive of obstructed hernia, but the definitive diagnosis of SH is made intraoperatively in almost all cases.We think our case is very interesting because it shows a giant Spigelian hernia  with an obstructive presentation, which is not so common among these rare abdominal wall herniations (described in few case reports).Surgical approach is urgent in the majority of cases and requires laparotomy  due to the distended bowel loops for the SBO; in our cases, the poor clinical status of this elderly patient, the high risk of having a poor tissue regenerative power, associated with the non-resolutive CT scan forced us to perform a minilaparotomy to enlarge the surgical field ,10.Laparoscopy could be a good approach especially in elective surgery with both intra and extraperitoneal mesh placement that seem equivalent  but it rTherefore, we suggest not to perform incision directly above the mass unless the preoperative diagnosis is certain or at least very suggestive of SH.Mesh repair is the preferred option also in urgent surgical procedures due to the little incidence of infection and the rare need for intestinal resection; recurrence rate is low (0–8%) ,13.4Among all the possibilities in differential diagnosis for SBO, Spigelian Hernia still has an important place. Preoperative diagnosis by CT scan could be essential to guide the surgeon to a mininvasive approach, even if in most cases urgent laparotomic surgery is needed due to abdominal distension.No source of funding.This study is exempt form ethical approval.Consent was obtained from the patient.Brandolin Denise and Giuliani Antonio performed the surgical procedure.Lazzarin Gianni and Romano Lucia collected data and pictures from surgery.Di Furia Marino and Salvatorelli Andrea proposed the study and wrote the paper.Schietroma Mario and Carlei Francesco supervised the paper and controlled all the analysis of results, including language.Not needed.Dr. Di Furia Marino.Not commissioned, externally peer-reviewed.No conflict of interest.


Inguinal hernia is the most common condition in both male and female subjects. Amyand's hernia is characterized by the presence of the cecal appendix, swollen or not, inside the inguinal hernia sac. It is a rare condition and represents 1% of all the inguinal hernia pathology. Littre's hernia (LH) is a condition defined by the presence of a Meckel diverticulum (MD) inside a hernial sac. It is an extremely rare condition, with less than 50 cases reported in the last three hundred years, present in only 1% of all diagnosed MD.  A six-year-old male patient presented with a history of two bilateral protruding masses in the inguinal-scrotal region that have continued to grow since birth. No gastrointestinal symptoms were reported. Physical examination showed a bilateral inguinoscrotal mass which increased in size during the Valsalva maneuver. Surgical intervention was carried out with a bilateral hernia repair being performed under an anterior method, the surgical invagination of the MD within the small intestine and the appendix within the caecum.  Both entities should be considered as a differential diagnosis when it comes to a pediatric patient with unilateral or bilateral inguinal hernias with an uncertain etiology, allowing an early diagnosis and prompt treatment. We present here the first recorded case of both Amyand's hernia and Littre's hernia presenting simultaneously in a pediatric patient. A hernitestine) , 3. Thistestine) . It reprtestine) –4. The ftestine) .Littre's hernia (LH) is a similar condition but is defined by the presence of a Meckel diverticulum (MD) inside a hernial sac as opposed to the cecal appendix , 6, 7. IThe MD was defined by Fabricius Hildanus in 1598, and in 1700, the French surgeon Alexis de Littré published the first case of an MD, referring to it as “an ileal diverticulum consequence of the traction made on the ileum.” It was not until 1809 that Johan Friedriech Meckel described the existing relationship between the MD and the omphalomesenteric duct [Both conditions mentioned in Introduction, AH and LH, occur very rarely and are reported in even fewer instances. We present the incidental finding and prompt treatment of a patient presenting with a right Littre's hernia and a left Amyand's hernia simultaneously found during routine bilateral inguinoscrotal hernia repair. The combination of two separate conditions makes this a unique study that should be recorded in detail.A 6-year-old male patient weighing 16.6 kg and with a height of 41 inches, from a rural location in Honduras, was evaluated by a pediatric surgeon. The subject presented with a history of two bilateral protruding masses in the inguinal-scrotal region that have grown bigger since birth. The mother denied past history of asthma or gastrointestinal symptoms such as diarrhea, melena, bloody stools, cramps, or pain, and the patient's bowel movements were normal. According to what was stated by the subject's mother, there was no family history of inguinal hernias. The patient comes from a setting with extreme poverty conditions. He had no history of previous surgical interventions.During a physical examination, a bilateral inguinoscrotal mass was observed that grew bigger during the Valsalva maneuver. To palpation, masses were reducible with no tenderness. The right mass dimension was 3.1 inches × 1.5 inches and the left mass was 5.5 inches × 1.9 inches  is limited to division and ligation of the hernial sac at the internal inguinal ring without narrowing the ring . The staCurrent trends favour laparoscopic and minimally invasive approaches, as they offer numerous advantages over open surgery for pediatric inguinal hernia repair, such as the opportunity to visually inspect the contralateral canal for the presence of an occult hernia without incision, superior visualization to potentially avoid trauma to the vas deferens and spermatic vessels, and opportunity to accomplish a safe high ligation of the sac at the internal ring . DespiteAH and LH should be considered as a differential diagnosis when it comes to a pediatric patient with unilateral or bilateral inguinal hernias with an uncertain etiology, allowing an early diagnosis and prompt treatment. AH and LH are infrequent conditions typically occurring in isolation. To date, there have been no cases reported in the medical literature of both AH and LH presenting concurrently. We therefore conclude to the best of our knowledge that this is the first pediatric case reported with the association of both conditions. It is also important to acknowledge that therapeutic approaches within areas with limited resources may not allow adherence to the standardized surgical practices of developed countries.


This mechanism may delay vascular compromise.•High level of clinical suspicion needs to diagnose intussusception with unusual presentation. Intramuscular intestinal lipoma is one of the rare benign tumours of the small intestine in children and acts as a lead point of intussusception.We describe a case in which a 4-year-old boy presented with recurrent attacks of colicky abdominal pain and non-bilious vomiting. Abdominal examination was irrelevant. Per rectum (PR) examination indicated an empty rectum without red currant jelly stool and non-palpable mass. Abdominal ultrasonography revealed a target sign of intussusception. After a period of conservative treatment, the child passed normal coloured and well-formed stool. Exploratory laparotomy referred to a long segment ileo-ileal intussusception with a polypoid lipoma as a lead point. Microscopic examination showed a benign non-capsulated intramuscular ileal lipoma.Small bowel lipoma produces symptoms of intermittent bowel obstruction. Herein, the child presented with non- bilious vomiting, we attribute this to reflex sympathetic stimulation of the pylorus leading to pylorospasm.Small intestinal lipoma is a rare finding in children that may cause intussusception which does not resolve spontaneously. Sometimes, persist due to an associated lead point. Our case had unusual symptoms and unusual lead point. This work has been reported in line with the SCARE criteria .2A 4-years-old boy was referred to our emergency department complained of recurrent attacks of colicky abdominal pain and non-bilious vomiting 5 times in the previous 24 h. The child had allergic rhinitis and recurrent attacks of gastroenteritis in the last four months.Upon presentation to our hospital, the child was conscious, alert, comfortable in bed, and his vital signs were stable. Abdominal examination by inspection showed normal contour without abdominal distention and no bulging mass, with normal respiratory movement. By palpation, the abdomen was soft and lax, with no tenderness or rigidity and no palpable mass. PR examination revealed no palpable mass and an empty rectum without red currant jelly stool; the child had last defecated 12 h before.Abdominal ultrasonography revealed a long segment small-intestinal intussusception extending from the subhepatic region through the right lumber region until the right iliac fossa region, which showed a pseudokidney appearance and no abdominal collection. Plain erect abdominal X-ray showed neither air-fluid levels nor gas under the diaphragm. Only fundic gas and a small amount of right colonic gas were observed. Abdominal computed tomography (CT) was not performed due to the poor resources of our hospital.Our decision was to perform conservative treatment with multiple ultrasonographic examinations. The conservative approach was continued for 6 h duration with nothing per mouth (NPO). He had received medical treatment in the form of IV fluid, 3rd generation cephalosporin, ampicillin/sulbactam, metronidazole and antioedematous drugs such as dexamethasone, and lasix. During this period, the child had one attack of colicky abdominal pain and non-bilious gastric vomiting. However, during this period, the child passed well-formed normal brownish coloured stool. An abdominal examination had the same results as the previous clinical examination. PR examination revealed well formed, normal brown coloured stool without any redcurrant jelly secretion. Due to the recurrent symptoms of intestinal obstruction, the senior staff made a new decision to explore the abdomen.After the child was administrated general anaesthesia, we palpated the abdomen and felt a hemispherical mass of 4 cm × 2 cm, movable in all directions without any restriction to its movement. A supraumbilical transverse incision was performed for exploration and revealed a long segment ileo-ileal intussusception approximately 50 cm long. Milking reduction was performed easily, without any intestinal ischaemia or intestinal wall oedema. The lead point of the intussusception was a hemispherical mass, canary yellow in colour and approximately 4 cm × 2 cm. It was firm, and rubbery inconsistency, did not obstruct the lumen of the ileum and originated from the antimesenteric border of the ileum. It was located 130 cm away from the ileocaecal junction . ResectiA pathological macroscopic report described a polypoid submucosal fatty tumour 4 × 2 × 2 cm in size covered with intact mucosa. Microscopic examination showed a benign non-capsulated intramuscular soft tissue tumour formed of lobules of mature fat cells separated by delicate fibrovascular trabeculae . The oppThe postoperative period was uneventful, and the patient was discharged on the 7th postoperative day. At 6 months following surgery, he is free of symptoms, and no evidence of recurrence has been reported.3The relative frequency of small-intestinal intussusception reported in the literature varies between 1.6% and 25% of all cases of intussusception .Primary lipomas of the small intestine are rare mesenchymal neoplasms, representing 2.6% of nonmalignant tumours of the intestinal tract . It arisSmall bowel lipoma produces symptoms of intermittent bowel obstruction. Very small ones may be asymptomatic . Herein,4Considering the rarity of small-intestinal intramuscular lipoma in children it should be included in the differential diagnosis of abdominal pain of doubtful origin.No conflict of interest exists for any of the authors in this case report.None.The institutional Ethics committee  had reviewed and approved my case report. EC Ref NO: asw327219.Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Sarah Magdy Abdelmohsen, the main operator of the case study, was responsible for data collection, analysis, and the writing of the article and is the "Corresponding author".Mohammed Abdelkader Osman was the main supervisor, and reviewer of the paper.Marrow Tammam Hussein was the pathologist performed the microscopic photography.I do not make any register, it is a case report.Sarah Magdy Abdelmohsen.All data and materials are available in case of request.Not commissioned externally peer reviewed.


Objective: Mesenchymal in origin, solitary fibrous tumors are primarily seen within the pleura of the lung or in serosal-lined body cavities. Constituting 1% to 2% of all soft-tissue tumors, solitary fibrous tumors are rare entities, especially when found in extrapleural and in superficial locations. A review of PubMed MEDLINE literature for superficial solitary fibrous tumors revealed 71 reports in case reports and small case series. Methods: In this report, we describe a 74-year-old man with an extrapleural superficial solitary fibrous tumor, as well as present a review of the current published literature to date. Results: We present the clinical course, surgical procedure, histopathological features, and treatment options, as well as present a compilation of the published data on superficial solitary fibrous tumors. Conclusions: Based on the current literature, solitary fibrous tumors are more common in middle-aged women and in the head and neck region. Diagnosis of solitary fibrous tumors requires tissue sampling and staining for immunohistochemical markers. Management of these tumors is based on wide local excision with histologically negative margins. If negative margins cannot be surgically achieved, adjuvant therapies including radiation have been described. With extrapleural manifestations of solitary fibrous tumors seldom reported in the literature, it is our hope that reporting these unusual instances will raise awareness of such disease manifestations and allow for earlier diagnosis and treatment. CD34 and STAT6, have been increasingly utilized in narrowing down the diagnosis. The biological course of SFTs tends be benign in nature, but malignant potential does exist. Clinically, patients may experience associated hypoglycemia, arthralgias, osteoarthritis, and clubbing. SFTs found in the cutis and subcutis are classified as superficial. Extrapleural superficial SFTs are quite rare but have been previously described around the body including both soft tissue and viscera with a tendency to present in a body cavity.A spindle-cell neoplasm of mesenchymal origin, solitary fibrous tumors (SFTs) were first reported in 1931.PubMed MEDLINE involving repots of superficial SFTs (cutaneous/subcutaneous) utilizing search terms (solitary fibrous tumor [Title/abstract]) AND  revealed 71 cases having been identified and described in the cutis and subcutis as case reports and/or small case series  staining can identify expression of Management of SFTs requires appropriate staging and evaluation for metastases with computed tomography. Benign tumors can be managed with wide local excision, with an expected 5-year survival ranging from 89% to 100%.,Overall, occurrence of SFTs is low, representing about 1% to 2% of all soft-tissue tumors.20CD34 and CD99. The histopathology was reviewed and the diagnosis of SFT was confirmed by the Department of Pathology by performing IHC staining for STAT6.A 74-year-old man was evaluated by dermatology for a suspicious painless mass located over the posterior aspect of his right trapezius muscle. The mass had been present for at least 3 years but was noted to have had a rapid increase in size within the last 2 months prior to presentation. He denied any associated symptoms or recent changes in his health. Medical and surgical histories were significant for actinic keratoses, atrial fibrillation with cardiac ablation and placement of a permanent pacemaker, coronary artery disease, hypertension, hyperlipidemia, and benign prostatic hypertrophy. He denied any family history of malignancy, other than actinic keratoses. On examination, he was noted to have a palpable, nontender, mobile mass over the posterior aspect of his right upper trapezius muscle measuring approximately 3 × 3 cm. An incisional biopsy was performed by dermatology. Pathology revealed cellular spindle cell tumor without necrosis but with up to 6 mitoses/10 HPF. In addition, IHC staining was positive for The patient was then seen in the surgical oncology office for further discussion and management. He was found to have a 3.5 × 1.2-cm mass with an overlying healing scar from his incisional biopsy. There was no evidence of satellitosis. Computed tomography of the chest, abdomen, and pelvis was performed to determine the extent of the tumor and revealed no evidence of metastatic disease. Prior to wide local excision, the patient was evaluated by the Multidisciplinary Cutaneous Oncology Clinic for any additional treatment recommendations. Neoadjuvant therapies were not recommended.A full-thickness, wide local excision with 1-cm margins was performed. Additional trapezius muscle was taken for an oncological boundary of safety. All specimens were submitted to pathology. The primary resection defect measured 6.1 × 5.4 × 3.6 cm and was reconstructed with a local rotational-advancement flap.Postoperatively, the flap reconstruction healed well without complication. Permanent pathology revealed positive deep margins with residual SFT. After extensive discussion with the patient, the decision was made to pursue adjuvant radiation therapy and forgo a secondary surgery. Radiation oncology plans for 30 treatments.SFTs are of mesenchymal origin, most often located within the pleura. Extrapleural manifestations represent about 0.6% of all SFTs, with the most common location being the head and neck.CD34 expression was found to be more commonly reported than any other molecular marker. Underreporting of STAT6 in the literature may be secondary to more recent understanding and evidence of expression of NAB2-STAT6 translocation and gene fusion that is now utilized for confirmation of diagnosis.30Summarized in CD34 positive (CD99 and bcl-2 can also show variable staining, but these stains are not lineage specific. Recently, a recurrent paracentric inversion involving chromosome 12q13 has been identified in SFTs of pleura and soft tissue that result in NAB2-STAT6 translocation and gene fusion.STAT6 that drives tumor proliferation. STAT6 immunostain is reliable surrogate marker for this molecular change and was employed for confirmation of the diagnosis in this case (31Pathological diagnosis of SFTs requires recognition of histological features coupled with supportive IHC stains. Similar to their pleural counterparts, this is a spindle cell neoplasm with alternating areas of hyper- and hypocellularity and characteristic gaping and bifurcating vessels, also known as staghorn vessels . Hyalinipositive  is indichis case . Strong his case . Age of ,Extrapleural location of SFTs does not necessarily portend a higher rate of metastasis/recurrence; however, its behavior is generally unpredictable. Reported recurrence and metastasis rates range from 10% to 37% in the literature.32With extrapleural superficial SFTs seldom reported in the literature, it is our hope that reporting our experience will add to the database of published literature, raise awareness of extrapleural manifestations, and allow for earlier diagnosis and treatment.


A 62-year-old female patient diagnosed with oesophageal adenocarcinoma underwent radical treatment consisting of neoadjuvant chemotherapy and oesophagectomy with no major complications. Eleven months later, she re-presented with a mass at one of the chest drain sites. A PET-CT scan and biopsy demonstrated this to be a single recurrence of the oesophageal adenocarcinoma. Excision of the metastatic lesion was considered as per metachronous single site metastasis. However, the operation was postponed due to acute kidney injury. Restaging after 6 weeks revealed progressive metastatic disease. The patient underwent palliative therapy and passed away soon after. Oesophageal cancer recurrence has a very poor prognosis, and factors such as the disease-free interval, site of recurrence and tumour pathological factors must be considered when stratifying for suitability for metastasectomy. A period of watchful waiting followed by restaging is essential to rule out patients with indolent metastatic disease. First proposed by Hellman and Weichselbaum in 1995 . ManagemThere are no documented cases of oesophageal adenocarcinoma seeding to a chest drain site. However, metastasectomy ± adjuvant therapy of single PEG site recurrences from direct seeding of head and neck cancers has been shown to achieve long-term survival in patients –6. AdditAlthough seldom attempted, reports have shown that long-term survival following oesophageal cancer recurrence can be achieved after resection of solitary oligometastases in the lung , and adrThe outcomes following resection of liver metastases from gastric cancer have also been investigated . This stTo the best of our knowledge, this is the first report of a metachronous recurrence of an oesophageal adenocarcinoma at a chest drain site. Due to the initial presentation as a single chest wall oligometastasis shown by CT and PET/CT, it was thought that seeding of the cancer to the chest drain site was solitary and had occurred directly, in a fashion similar to port site metastasis, where direct seeding is thought to be a more likely mode of transmission than haematogenous spread , 14. AddA 62-year-old obese lady presented with a 2-month history of dysphagia for solid foods which had worsened over the past 2 weeks, progressing to odynophagia. She was otherwise well and not on any medications. She did not smoke, and only drank moderately.Oesophago-gastroduodenoscopy (OGD), biopsy and CT revealed a 5-cm-long circumferential, invasive and poorly differentiated adenocarcinoma at the GOJ Fig. . SubsequAt the time of her initial presentation, our trust protocol for patients with a tumour staged as being T2 N0 or above was neoadjuvant chemotherapy without radiotherapy. She underwent three cycles of epirubicin, cisplatin and capecitabine (ECX) neoadjuvant chemotherapy. Restaging with PET-CT 3 months later showed that the cancer stage remained the same at IIA and that there was a marginal reduction of the SUV to 4.4.A hybrid Ivor Lewis oesophagectomy with laparoscopic gastric mobilisation was performed with no intra-operative complications. Lymphadenectomy was performed en bloc of stations 4R, 7, 8L, 8M, 9, 10L, 10R, 15–20 . As the specimen was removed through the thoracotomy site, a wound protector was not used. No spillage or perforation of the tumour was noted at time of surgery. The oesophagogastric anastomosis was performed at the level of the thorax, above the azygous vein and 3 cm below the thoracic inlet. Two chest drains were placed as per standard practice. She recovered with no major post-operative complications and was discharged home day 8 post-operative.http://www.rcpath.org)]. The tumour was also seen to be spreading along the submucosa and subserosa of the stomach. Three of 25 lymph nodes examined were tumour positive. There were no regressive changes from neoadjuvant chemotherapy corresponding to a Mandard tumour regression grade of 5. Histologically, the tumour was a poorly differentiated adenocarcinoma (diffuse type) with scattered signet ring cells and overlying reactive squamous mucosa  L1 V1 M0 R1 tumour, which was not present in the proximal oesophageal margin block and was therefore at least 5 mm from the proximal surgical resection margin. However, the pathological circumferential resection margin (CRM) was tumour positive (R1), defined as tumour involvement within 1 mm of the surgical resection margin [British Royal College of Pathology , which was standard local practice for a R1 specimen.Eleven months after the oesophagectomy, our patient re-presented with a non-tender and irreducible mass in the right chest wall at the site of the previous chest drain. There were no further lesions or ascites evident on clinical examination.CT scans confirmed the presence of a chest wall mass Fig. . A PET sFollowing MDT discussion, it was decided to proceed with surgical resection after considering the disease-free interval (DFI) of 11 months, and that the recurrence appeared to be a solitary oligometastasis which has seeded directly. This was favoured over radical chemoradiotherapy as given the age and life expectancy of our patient, we believed that surgical resection would give her the best chance of long term survival. The resection of the metastasis did not take place as our patient was found to have acute kidney injury (AKI) during the pre-operative assessment. Ultrasound and CT scans demonstrated moderate functional bilateral hydronephrosis Fig.  with no Six weeks after presenting with a chest wall mass, and after the resolution of the AKI, our patient was restaged with a PET-CT scan. This revealed a focus of increased FDG uptake in the liver and also in an incidental Spigelian hernia Fig. . It alsoManagement of oligometastatic recurrence after oesophageal cancer is controversial, with long-term survival after metastasectomy being variable. When selecting suitable candidates to undergo resection, one may wish to consider prognostic factors such as the disease-free interval (DFI), site of recurrence, and the pathological characteristics of the primary tumour.In a case series by Chen et al. , five paIt has been reported that resection of metachronous oligometastases in the lung increases the average survival of selected and suitable patients . HoweverAt re-presentation, our patient had a solitary mass in the chest wall with no other lesions or ascites detectable, and so it initially appeared to be an oligometastasis. We considered our patient’s metachronous single site metastasis to be similar to a PEG or port site metastasis from direct transmission, hence she was considered for surgery. However, the pathological features of her tumour were unfavourable for long-term survival , 16, parThe delay needed to optimise her renal function led to an important change in the treatment plan, ultimately avoiding unnecessary surgical intervention. As illustrated by this case, a period of active monitoring and restaging is essential when dealing with oligometastatic recurrence of oesophageal cancer. There are no reports or studies recommending a specific waiting time after initial re-presentation before aggressive treatment. For our patient, 6 weeks from initial re-presentation were enough for the widespread metastases to be detected. The outcomes for our patient would have been dismal if we proceeded immediately with a wide surgical excision, as she not only had an oligometastasis from direct seeding, but also initially unrecognised blood borne and transcoelomic metastases in the peritoneum. Further staging with laparoscopy and thoracoscopy may have a role in these situations too, as dissemination may initially be difficult to identify only with PET-CT. However, our patient’s AKI prevented us from doing this early on.Evidence from previous reports have suggested good long-term survival for oesophageal cancer recurrence if the metastases are single and metachronous with a significant DFI, and also occurring in sites such as the lung. As shown by our case, an 11-month DFI was not long enough to predict a truly solitary recurrence and potential long-term survival after resection. A longer DFI may be required and more studies are required to delineate an estimate for a positive prognosis. By chance, our case also highlighted that an assessment period following recurrence should be adhered to, allowing metastases which are undetectable through imaging to be excluded. The precise length of this period is also yet to be determined.


Cholesterol granuloma is a benign mass that commonly involves the petrous apex but rarely affects other structures, such as the mastoid cavity. It is diagnosed histologically by the presence of giant cells, and Its management is individualized based on some factors such as the size and location of the lesion.The first case was a 33-year-old man who presented to the outpatient clinic with a two-year history of right-sided pulsatile tinnitus, hearing loss, and vertigo. Upon investigations, a large, destructive mass in the tympanomastoid region was found and managed surgically. The other case was for a 41-year-old man who presented to the emergency department with loss of consciousness. Urgent CT was done and revealed an aggressive hypodense posterior fossa mass destroying the right temporal bone that was managed surgically.In this report, we describe two patients with familial hypercholesterolemia who developed bilateral cholesterol granuloma that were managed surgically.These cases are reported because of their rare location and presentation since few cases of bilateral cholesterol granuloma have been reported in the literature. Cholest2A 33-year-old man with a diagnosis of familial hypercholesterolemia treated by simvastatin presented to the outpatient clinic in the otolaryngology department of a tertiary hospital with a two-year history of right-sided pulsatile tinnitus, hearing loss, and vertigo. The patient had no history of ear pain or discharge in the right ear and reported no complaints in the left ear. The patient denied any previous surgery or trauma to either ear. His family history was positive for familial hypercholesterolemia, with three first-degree relatives affected. Physical examination revealed multiple xanthomas around the joints in both hands and feet, and there are bilateral eyelid xnthelasma . CranialHistopathological evaluation with hematoxylin and eosin staining confirmed the presence of a destructive infiltrate containing foamy macrophages, foreign body giant cells, and cholesterol clefts . On revi3A 41-year-old man with known type 2 diabetes, hypertension, secondary adrenal insufficiency, and homozygous familial dyslipidemia was brought to the emergency department with sudden loss of consciousness. He had had no complaints prior to this episode. His chronic conditions were managed by hydrocortisone, aspirin, lisinopril, hydrochlorothiazide, warfarin, allopurinol, and ezetimibe. His past surgical history was positive for coronary artery bypass surgery and aortic valve replacement at the age of 36 years. His family history included three first-degree relatives with familial dyslipidemia and one first-degree relative with premature cardiac disease. On neurological examination, the patient was disorientated but obeyed commands and opened his eyes spontaneously. No cranial nerve deficit was detected except for hearing loss in the right ear. Pure tone audiometry performed after resolution of the acute condition showed normal ranges in both ears. However, the patient’s laboratory results included a fasting plasma cholesterol level of 18.40 mmol/L and a low-density lipoprotein cholesterol level of 15.4 mmol/L. An urgent CT scan of the head performed in the emergency room showed an aggressive hypodense posterior fossa that was soft tissue mass destroying the right temporal bone and the adjacent occipital bone. The mass measured 45 mm × 65 mm, extended upwards to the posterior temporal bone, and was causing compression and deviation of the fourth ventricle and brain stem with obstructive hydrocephalus. A similarly obstructive lesion involving the mastoid portion of the petrous bone with a small tissue component was seen in the posterior fossa on the left side . MRI sho4Cholesterol granuloma was first described by Manassé in 1917, but it was not until 40 decades later that Birrell (1956), Sheehy and McKibbem (1956), and Friedmann (1959) refocused attention on this benign mass . The patA search of the literature revealed only three cases of familial hypercholesterolemia with bilateral cholesterol granuloma. The first case was a 35-year-old man with known familial hypercholesterolemia who had a destructive lesion in the left mastoid region, the second case was a 19-year-old patient in whom CT showed large cystic lesions bilaterally, and the third case was a 45-year-old woman who had cholesterol granuloma in the cerebrum. All three of these patients had familial hypercholesterolemia and were managed surgically. The second and third cases underwent coronary artery bypass graft surgery at an early age before resection of cholesterol granuloma . There s5Cholesterol granuloma is a benign lesion characterized histologically by the presence of giant cells. Diagnostic radiological studies are reliable. Management is individualized according to the site, size, and extent of the lesion. Surgical intervention is reserved for advanced cases.No source of funding.IRB approval has been guaranteed by King Fahad Medical City. IRB Log number: 18-678.Written informed consent was obtained from the patients 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.Nouf Albakheet has contributed in writing the introduction, case, and discussion.Yazeed Al-shawi has contributed in writing the case.Yasser Orz has contributed in writing the case.Mohammed Bafaqeeh has contributed in writing the case.Hanadi Fatani has contributed in writing the case.Ibrahim Shami has contributed as the supervisor.researchregistry4999.Dr. Ibrahim Shami.Patient perspective about the treatment has not been asked.Not commissioned, externally peer-reviewed.No conflict of interest.


Cases of inguinal bladder hernia are rare, with bladder involvement seen in 1–4% of inguinal hernias. The majority of cases are diagnosed intraoperatively, with only 7% of bladder hernias identified prior to surgery. Diagnosis may be challenging as patients are often asymptomatic or have nonspecific symptoms. Surgical repair is currently the standard treatment, and careful surgical planning is necessary to avoid complications including bladder injury.A 58-year-old man presented to our Emergency Department with 2-day history of progressively worsening left lower quadrant pain, groin bulge, and dysuria. Physical exam revealed an irreducible left inguinal hernia associated with urinary urgency on attempted reduction. Plain CT was ordered and demonstrated inguinal hernia with bladder protrusion into the left scrotum. The patient underwent open surgical reduction and hernia repair and made a quick postoperative recovery without complications.Inguinal bladder hernia most often presents in older, obese males and clinicians should have a high index of suspicion when assessing patients with inguinal hernia. Preoperative diagnosis based on history, physical, and radiologic imaging allow for careful surgical planning and prevention of severe complications including bladder injury and leakage.We present a case report of inguinal bladder hernia in a middle-aged man that presented as left lower quadrant pain, groin pain, and dysuria. Diagnosis was confirmed preoperatively with radiographic imaging. The hernia was surgically reduced and the defect repaired without complications. Risk faDiagnosis of inguinal bladder hernia may be challenging because the majority of patients are asymptomatic, in which case preoperative diagnosis depends on incidental discovery on radiography . PatientSurgical repair of the hernia after bladder reduction is currently the standard treatment, and consists of intraoperative reduction or less commonly resection of bladder followed by herniorrhaphy . If the Here in, we describe the case of a 58-year-old obese man with left sided inguinal bladder herniation presenting as worsening abdominal pain, groin pain, and dysuria. This case has been reported in line with the SCARE criteria .2A 58-year-old obese male presented to our Emergency Department with 2-day history of progressively worsening left lower quadrant abdominal pain, urinary urgency, and 3/10 groin pain. The patient also reported dysuria. Comorbidities included hypertension and Class 1 obesity with body mass index of 32. He denied experiencing similar symptoms in the past and reported no other past medical history, no smoking history, or relevant family history.Physical exam was significant for obvious 7 cm left inguinal hernia bulge with tenderness to palpation. On attempt to reduce the hernia, the patient reported urinary urgency. Laboratory studies and urinalysis were within normal limits. A computed tomography (CT) scan revealed left inguinal hernia containing a portion of the urinary bladder A–C.Fig. The patient was taken to the Operating Room the same day for planned open left inguinal hernia repair with mesh. Intra-operatively, a portion of the urinary bladder was found to be inside the inguinal hernia. The inguinal hernia sac was initially difficult to differentiate from the bladder due to its thickness A, B. TheThe patient had no intra-operative complications. On post-operative day (POD) 1, the patient appeared well with minimal groin pain. Incision was clean, dry, and with no drainage or signs of infection. Patient was able to ambulate and tolerate diet. His Foley catheter was removed and the patient was able to urinate without pain or difficulty. Patient was discharged to home on POD 2 with controlled pain, regular diet, and normalized white blood cell count. He was seen in clinic 2 weeks post-operatively free of pain and with no urinary voiding symptoms. Incision was clean, dry, and healed.3Our patient was a 58-year-old man who presented with 2-day history of left lower quadrant abdominal pain, groin pain, and urinary urgency. Physical exam revealed an irreducible inguinal hernia with tenderness to palpation and feeling of urinary urgency on attempted reduction. CT revealed bladder involvement in the hernia. Preoperative diagnosis of inguinal bladder herniation allowed careful planning and a successful reduction of the bladder into the abdominal cavity and repair of the hernia defect with mesh.The pathophysiology of an inguinal bladder herniation involves pulling of the bladder and a sheath of peritoneum that forms its sac through a weak point in the abdominal fascia . SeveralCareful history and physical examination is key in establishing a preliminary diagnosis. Ultrasonography, cystography, and CT have all been utilized to confirm the diagnosis . Given tStandard treatment of inguinal bladder hernia involves bladder reduction or on rare occasion partial resection followed by hernia repair, which historically was done with a midline laparotomy but more recently has been done with an open inguinal incision or with a laparoscopic approach . Current4Inguinal bladder hernia is an unusual condition that requires a high index of clinical suspicion for diagnosis. In this report, we demonstrate a case of inguinal bladder hernia presenting as worsening left lower quadrant pain, groin pain, and dysuria. Reduction of the hernia and bladder, followed by hernia repair with mesh was performed through an open approach. Bladder distension with normal saline was via a bladder catheter was utilized to better visualize the bladder and aid in reduction. The patient had no complications. This case demonstrates the importance of preoperative diagnosis in avoiding surgical complications including bladder injury.None.None.This is a case report study. Informed written consent has been obtained and all identifying information is omitted. This work has been conducted in compliance with institutional ethical standards.Informed written consent has been obtained and all identifying information is omitted.Adel Elkbuli, Dessy Boneva, Raed Ismail Narvel, Mark McKenney – Conception of study, acquisition of data, analysis and interpretation of data.Adel Elkbuli, Dessy Boneva, Raed Ismail Narvel – Drafting the article.Dessy Boneva, Mark McKenney – Management of case.Adel Elkbuli, Raed Ismail Narvel, Dessy Boneva, Mark McKenney – Critical revision of article and final approval of the version to be submitted.This is a case report study.Dessy Boneva.Mark McKenney.Not commissioned, externally peer-reviewed.


Zinner syndrome (ZS) is the association of congenital seminal vesicle cysts and ipsilateral upper urinary tract anomalies, such as multicystic displastic kidney (MCDK). This condition is rare in pediatric age and both diagnosis and treatment are challenging. The aim of this study was to analyze the issues in diagnosis, management, and treatment of ZS in pediatric age. The medical records of two patients with ZS were examined. Furthermore, a review of the literature on this topic in pediatric age was performed. In our experience the diagnosis of ZS was incidentally achieved in the first months of life, as a consequence of studies performed for a prenatal diagnosis of MCDK. The first patient presented unspecific and transient symptoms, the second infant was completely asymptomatic. They were conservatively treated, with a long-term follow-up planned at least until adolescence. Fifty cases of ZS in pediatric age have been reported in the literature up to now. Only 12/50 were diagnosed in the first year of life. The diagnosis was demanding, as the clinical presentation was unspecific and the results at imaging studies needed a differential diagnosis with other retrovesical masses. More than 80% of these cases were asymptomatic at long-term follow-up. Therefore, a conservative management of ZS has been accepted for asymptomatic or poorly symptomatic patients, with occasional, transient, and unspecific symptoms, such as urinary tract infections or orchyepididimytis. As the surgical management is challenging, it is proposed only in those symptomatic patients. In conclusion, ZS is rare in pediatric age. However, it should be considered in the differential diagnosis of cystic masses within the pelvis in males with ipsilateral renal anomalies. A conservative treatment with a long-term follow-up is a safe option in the management of asymptomatic or poorly symptomatic patients, thus reserving the surgical approach only in those cases with symptoms. Congenital seminal vesicle cysts (CSVCs) associated with anomalies of the ipsilateral upper urinary tract are uncommon . This coWe report two cases of ZS, both incidentally discovered after birth and successfully treated conservatively. Moreover, a review of the literature on this topic in pediatric age was performed, aiming to analyze the issues with its diagnosis, management, and treatment.Following institutional review board approval, the medical records of two patients with ZS were reviewed. Both patients had a prenatal diagnosis of multicystic displastic kidney (MCDK). ZS was incidentally identified in the first months of life, as a consequence of studies performed for the prenatally detected renal anomaly—i.e., ultrasonography (US), voiding cysto-uretrography (VCUG), and dimercaptosuccinic acid (DMSA) renography.The first patient was a boy with a prenatal diagnosis of right MCDK. The baby was delivered by Cesarean Section due to fetal distress ; the post-natal period was uneventful. A renal US was performed in the fifth day of life and it confirmed the right MCDK and identified two ipsilateral retrovesical fluid-filled cysts . A renal US was performed in the fourth day of life and it confirmed the prenatal diagnosis and identified two ipsilateral retrovesical cysts (15 × 11 × 8 and 11 × 9 × 7 mm) with a dilatation of the distal left ureter (7 mm). A VCUG was performed at 4 months of life. The study revealed reflux into the left seminal vesicle, which appeared dilated by the cystic formations, and a further reflux into the vas deferens . The DMSSeminal vesicles cysts may be congenital or acquired . AcquirePrimary pathologies of seminal vesicles are rare , 7, 10. The incidence of ZS in the pediatric age is difficult to define . Sheih eTo the best of our knowledge, ZS has been reported in 19 papers and 50 patients in pediatric age, with a median age at diagnosis of 12.5 years range 0–18 years)  years 8–22. The . The 22.Symptoms in ZS are commonly present between puberty and the fourth decade of life, the period of more intensive sexual activity, when seminal fluid accumulates in the seminal vesicles due to the stenosis of the ED , 9. ClinThe diagnosis of CSVCs is difficult to achieve in pediatric age. The paucity of this anomaly limits the clinical experience and the development of a correct diagnostic work up. Commonly, the most frequent feature achieved during the imaging studies is a retrovesical cyst. Symptoms, when present, may mimic other more common diseases of the lower urinary tract . The difWhen ZS has been detected, a follow-up with annual clinical and US analysis has been suggested, even in those asymptomatic cases , 10, 22.The gold standard treatment for CSVCs is controversial. Surgical management is proposed only in those symptomatic cases , 21–23. Minimally invasive surgery seems to solve this problem, allowing a better visualization of the working space. To the best of our knowledge, 4 cases of laparoscopic excision have been described in children, with a median age of 4 years (range 10 months −17 years) , 19, 20.In younger children, the indications for surgery were recurrent UTIs and orchiepidydimitis , 11, 12,Conservative management of CSVC is commonly accepted for asymptomatic or poorly symptomatic patients, with a prolonged monitoring . In the CSVC are rare in pediatric age. However, they should be considered in the differential diagnosis of cystic masses within the pelvis in males with ipsilateral renal agenesis or dysplasia. US, VCUG, and cystoscopy are useful tools to perform an accurate diagnosis. MRI or CT scan should be reserved for patients whose diagnose is not obvious. Conservative treatment is an efficient and safe option in the management of asymptomatic or poorly symptomatic patients, reserving the surgical approach only in those cases with recurrent symptoms. Follow-up is recommended at least until the post-pubertal age or in the young adulthood, as symptoms may appear after the beginning of the sexual activity.Written informed consent for publication of the case series and figures was obtained from the parents of patients.VC designed the study and performed the literature review. VC, DD, VG, and GL wrote and reviewed the manuscript. PL critically reviewed the manuscript. Approval of the manuscript all authors.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.


Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal tissue tumor. Distinguishing PDS from similar conditions, such as atypical fibroxanthoma (AFX), its less aggressive tumor counterpart, is difficult, as they are clinically and histologically similar. We present a case of a 77-year-old man presenting with a large nodular scalp lesion of three weeks duration. Pathology revealed a 3.3 cm invasive pleomorphic dermal sarcoma. Surgical excision with 2 cm margins was performed with successful healing of the graft. This case highlights a rare case of a large pleomorphic dermal sarcoma and discusses the histological features and management of PDS. Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS), formerly known as malignant fibrous histiocytoma (MFH), are rare mesenchymal tissue tumors. These tumors are clinically and morphologically similar, and it is debated whether PDS represents a separate clinical entity from AFX or it is merely a more aggressive variant. AFX occurs more frequently than PDS; however, both of these entities are quite uncommon. AFX cases have been moderately well-documented, thus allowing for a study of the risk factors and the genetics of these mesenchymal malignancies.AFX tumors affect elderly patients  -2, favorDistinguishing AFX from PDS is vital, given that PDS is a much more clinically aggressive tumor, requiring prompt diagnosis and decisive treatment. However, clinically distinguishing the two cutaneous diseases is often difficult because of the similarities in their gross and microscopic morphologies. Several studies support the treatment of AFX with Mohs surgery -15 but lWe present a case of a mesenchymal tissue tumor affecting the scalp of an elderly male patient. We discuss the pathological diagnosis of the patient’s tumor and the successful surgical management of the lesion. Our patient’s case illustrates the rare pleomorphic dermal sarcoma malignancy. This case may help clinicians be more aware of the related atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) mesenchymal tumors and of the surgical management that can be used for the management of these rare conditions.A 77-year-old man with no history of skin cancer presented to the outpatient dermatology clinic for a scalp lesion of three weeks duration. He reported rapid lesion growth, but no change in the overall appearance nor any associated symptoms. Examination revealed a mobile, round, exophytic nodule with overlying ulceration and hemorrhagic crust, approximately 1.5 cm in diameter Figure . Given aA histopathologic examination of an excisional biopsy of the cutaneous lesion revealed a proliferation of spindle and pleomorphic tumor cells, which flattened the overlying epidermis Figure  and exteA surgical, wide, local excision was planned. Preparatory computed tomography (CT) imaging revealed an approximately 3.9 cm focus of enhancement with central ulceration in the soft tissue of the posterior scalp and confirmed a lack of bony erosion in the underlying calvarium. No metastatic adenopathy was appreciated. Figure Given the high risk of malignant behavior in pleomorphic dermal sarcoma (PDS) tumors, it is important to distinguish PDS from its more benign atypical fibroxanthoma (AFX) counterpart. This decision is made difficult by the morphological similarities between AFX and PDS, such as spindle or round cell histology, the presence of pleomorphism and atypical mitotic figures, and CD10 marker positivity. PDS sometimes reveals more aggressive histological features than AFX, such as evidence of deeper subdermal involvement, the presence of lymphovascular or perineural invasion, and/or necrosis .In the case presented here, the histological examination did not reveal any evidence of lymphovascular or perineural invasion. Likewise, as diffuse CD10 positivity is observed in both AFX and PDS tumors, CD10 positivity alone could not be used to make the diagnosis of PDS. However, the involvement of the deep subcutis favors a diagnosis of PDS  and giveLess is known overall regarding PDS management given the tumor’s rarity. Complete surgical excision is considered as the first line option for PDS management , and an Pleomorphic dermal sarcoma is a rare tumor affecting mesenchymal tissue. We present the case of pleomorphic dermal sarcoma occurring on the scalp of a 77-year-old male patient. Successful treatment with a wide, local excision was performed. This case highlights the morphologic and histologic characteristics of PDS and discusses what is known about the common treatments of rare mesenchymal malignancies such as atypical fibroxanthoma (AFX) and PDS.


Oncocytic adrenocortical neoplasms (OANs) are very rare. Although most cases have benign behavior, the risk of recurrence/metastasis is variable. Based on Lin-Weiss-Bisceglia (LWB) system criteria, OANs can be classified as benign, borderline, or malignant. A concomitant development of OANs with second primary neoplasm is extremely uncommon, and is limited to very few case reports. None of these reported cases was found to be associated with hepatocellular carcinoma (HCC). In this case report, we present a 64-year-old female patient who had a progressively increasing left supra-renal mass over a three-year interval. During her regular imaging-based follow up after successful left adrenalectomy, a new suspicious solitary, hypodense liver mass was detected and removed. All necessary work-up was done and strongly support the diagnosis of two distinct primary tumors including borderline malignant potential OAN and subsequent HCC. A significant clinical and morphological characteristic of OANs make its identification valuable. Oncocytic neoplasms are defined as tumors rich with specific type of epithelial cells known as oncocytes. Oncocytes have abundant granular eosinophilic cytoplasm. Ultrastructurally, oncocytes possess numerous cytoplasmic mitochondria. Oncocytes can be arranged in various growth patterns such as diffuse sheet-like, alveolar, trabecular, and glandular . OncocytThe case represents a 64-year-old female patient known with hepatitis C virus infection, liver cirrhosis, hypertension, type 2 diabetes mellitus, and hypothyroidism on medications. In 2015, she presented to surgery clinic complaining of left flank mass associated with recurrent abdominal pain in the previous three years. By physical examination, the left abdominal mass was palpable with tenderness. The mass was progressively increasing in size from 5 cm to ~15 cm in greatest dimension in a three-year interval. Liver enzymes (AST and ALT) were high. Vital signs were within normal limits. Since the patient is hypertensive, pheochromocytoma was clinically suspected. Accordingly, urine analysis of metanephrine was done and it was within normal range. Further radiological investigations through abdominal computed tomography scan revealed a markedly enlarged heterogeneous left supra-renal mass (8.9 × 8.5 × 7.5 cm), for which, she underwent exploratory laparotomy and complete excision of the mass and sent for histopathology evaluation. The patient gave consent prior surgery. Grossly, the mass measured 10 × 7.5 × 5 cm, well-circumscribed, and encapsulated. Cut section showed a lobulated, yellow/tan to orange surface with foci of hemorrhage. No necrosis was seen. A portion of adrenal gland was found attached to the outer surface of the mass measuring 1.3 × 0.7 × 0.5 cm. Microscopic examination revealed a neoplasm composed predominantly of diffuse polygonal cells with abundant granular and eosinophilic cytoplasm. They have large nuclei and prominent nucleoli. Occasional mononuclear and binucleated giant cells are seen. Neither nether vascular invasion nor necrosis was identified. Rare mitotic figures are noted . The morα. Variable positivity is seen for pancytokeratin anti-bodies CK8, CK18, and CD10. Immunostaining for CK20, chromogranin, S-100 protein, HMB-45, and EMA is usually negative. The current case shows a similar immunoprofile pattern. The OANs' immunohistochemical profile is identical to that of adrenocortical neoplasms of conventional type [According to the available literatures, many studies emphasizing the importance of recognition of OANs. The LWB system is applied for assessment of malignant potential of OANs. A malignant OANs present with any of major criteria , borderline malignant potential present with at least 1 of the 4 minor criteria , and neither of major nor minor criteria present in a benign oncocytoma. OANs have been described in adults in their fourth-sixth decades of life with Female: male ratio of 1.8 : 1. Left adrenal gland is more affected than right adrenal gland. Most OANs are nonfunctioning and usually incidentally discovered during abdominal imaging. A small percentage of hormonal secreting adrenocortical neoplasms present with various clinical symptoms such as Cushing syndrome, pheochromocytoma-like syndrome, and virilisation . Most OAnal type . Recentlnal type . In conc