A septate uterus with a non-communicating hemicavity was first described by Robert in In this report, we review the disease, its diagnosis and treatment, and describe five cases of Robert's uterus. Three dimensional (3D) ultrasound (US) imaging was performed by the transvaginal route in four cases. In the fifth case of a 13-year-old girl, we avoided the vaginal route and magnetic resonance imaging (MRI) and 3D transrectal US yielded the correct diagnosis.The following treatment procedures were undertaken: laparoscopic endometrectomy, hysteroscopic septum resection, laparoscopic uterine hemicavity resection and total laparoscopic hysterectomy (TLH). The diagnosis and optimum treatment of Robert's uterus remains difficult for clinicians because of its rarity. A detailed and careful assessment by 3D US should be performed, followed by hysteroscopy in combination with laparoscopy, to confirm the diagnosis. Female genital malformations are defined as deviations from normal anatomy that could impair the reproductive potential of a woman or, in complex cases such as obstructive anomalies, a woman’s health. The malformations arise embryologically from the failure of Müllerian duct formation, canalisation, fusion or absorption. The anomalies are either isolated defects or occur in combination with conditions in various parts of the female genital tract. The latter results in so-called complex anomalies.The overall prevalence of uterine anomalies is 0.5%. Awareness of these conditions is extremely important in order to avoid misdiagnosis and inappropriate management .Pelvic pain, prolonged or abnormal bleeding at the time of menarche, recurrent pregnancy loss, or preterm delivery are symptoms of congenital uterine anomalies (CUAs). Some CUAs may be suspected because of associated findings on physical examination, such as a longitudinal vaginal septum. Others may be detected when imaging studies are performed to evaluate patients with severe menstrual pain, cyclical pain without bleeding in young girls, infertility, symptoms related to non-reproductive organ systems, or trauma.Classification systems are useful because they permit grouping of similar anomalies. Conclusions can be derived on the basis of several cases rather than individual case reports. The classification, diagnosis, and clinical manifestations of major congenital anomalies of the corpus , along with their associated cervical and vaginal anomalies have been discussed in the past and were summarised in the American Fertility Society Classification of 1988, by Grimbizis et al. in 2013 and 2016, and by Di Spiezio Sardo et al. in 2015 . A few cThe incidence of Robert's uterus is rare with only a few cases reported so far . It is cThe origin of the defect may be a segmental agenesis of the isthmus with a persistent septum between the upper portions of the Müllerian ducts. The first symptoms of this condition are usually experienced after menarche. The obstructed unicornuate cavity has a functional endometrium which results in the retention of menstrual blood and can cause a haematometra or even a haematosalpinx. This is followed by cyclic dysmenorrhea. Reflux of the retained menstrual blood into the peritoneal cavity may be an aetiological factor for endometriosis. The second uterine cavity communicates with the single cervix and is responsible for menstrual flow.The ESHRE-ESGE classification classified the Robert`s uterus as a Class U2bC3V0 anomaly or a complete uterus with partial cervical aplasia. Robert's uterus is also known as an asymmetric septate uterus with characteristics of a complete uterine septum, asymmetrically separating the endometrial cavity from the non-communicating hemi-uterus due to obstruction caused by the septum. With some exceptions, laparoscopy combined with hysteroscopy is the gold standard for confirming the diagnosis of Robert's uterus. The shape of the uterus may be normal on laparoscopy. There may be a normal external uterine fundal contour or a slight hollow or protrusion, with or without haematometra and haematosalpinx. On hysteroscopy the uterus has only one ostium of the fallopian tube.In the following cases, we present five different patients with Robert`s uterus in order to show the wide variety of symptoms, the heterogeneity of the patient population, and the different diagnostic and treatment options available.Three dimensional (3D) ultrasound (US) imaging: In the present study, we used an advanced 3D US imaging technique for the diagnosis and postoperative screening of Robert's uterus. In our opinion, US imaging is superior to magnetic resonance imaging (MRI) screening; the latter is expensive and less widely available. To our knowledge, this is the first report of five cases of Robert's uterus from a single centre over a 12 month period.We used the Voluson E10BT19 device transabdominally, combined with transvaginal US.We performed an endometrectomy under laparoscopic control with restitution of uterine integrity in a 13-year-old girl, one hysteroscopic septum resection, one laparoscopic uterine resection of a hemicavity, and one hysterectomy. The fifth patient had recurrent miscarriage and was scheduled to undergo a hysteroscopic septum resection, but became pregnant again before the treatment could be performed.In the following, we report in detail five cases of Robert's uterus, their 3D US diagnosis, and their subsequent hysteroscopic and laparoscopic treatments .The key factors that play a role in deciding the treatment include age of the patient, concurrent adenomyosis/ endometriosis and the desire to retain childbearing ability. Using US we diagnose the Robert`s uterus itself and also assess the thickness and vascularity of the septum and the thickness of the uterine wall.A 13-year-old girl presented with incapacitating dysmenorrhea starting three days after commencement of her menstrual cycle, associated with pain radiating to her back, vomiting and constipation.As the transabdominal two dimensional (2D) US provided no clear information  and the Transrectal US with a C1-5D probe also revealed a haematometra measuring 5x5 cm on the left side, and a right horn with an endometrium of 10 mm, type III .Transrectal US investigation with a RIC-9D probe, 3D rendering, and HD live confirmed the presence of haematometra in the left hemicavity, a unicornuate-like right hemicavity, and a septum of 1.49 cm thickness dividing the two cavities. The outer contour of the uterus was normal, thus confirming the diagnosis of Robert's uterus .The images were useful in counselling the patient regarding the surgical approach. The exact anomaly was illustrated in a line drawing and enabled clear communication between the ultrasonographer, the surgeon, and the patient .As the thickness of the septum between the two cavities was between 1.6 cm and 1.35 cm in the longitudinal aspect and was well vascularised (colour score 2), we decided to perform a laparoscopic endometrectomy. An initial diagnostic hysteroscopy revealed a single right hemicavity with a right ostium. The laparoscopic view is shown in The treatment was performed as a laparoscopic procedure. An incision was made on the fundus , and theThe excision was performed with a margin of 2-3 mm of myometrium on all sides. The myometrium was subsequently reconstructed on the left side of the uterus . The excThe procedure was selected because the patient was young and we wished to avoid an extensive operative hysteroscopy due to the risks of perforation and additional damage. Alternatively, we could have performed stepwise hysteroscopy-guided opening of the septum to release the haematometra and eventually transform both cavities into a single cavity. Post-operatively no pain was reported suggesting a good clinical outcome.A 25-year-old patient with primary infertility and regular menstrual cycles presented with severe dysmenorrhea, which persisted for seven days after the end of her period. A transverse 2D image by transvaginal US with a RIC5-9D volume probe revealed a haematometra in the left hemicavity of the uterus. The right hemicavity showed a unicornuate- like cavity and adenomyosis with myometrial cysts.The outer contour of the transverse section of the uterus appeared normal. The left ovary revealed an endometrioma of 3 cm adherent to the fundus of the uterus .The 3D rendered view demonstrated a 4.1-mm- thick septum with no vascularity (colour score 1). The outer contour of the uterus appeared normal on the coronal section . An MRI The explanatory drawing served as a basis for explaining the surgical procedure . On hystA 36-year-old patient with severe dysmenorrhea had conceived spontaneously on two occasions. She had delivered both children at full term by elective caesarean section because of breech presentation.The patient was given analgesics for dysmenorrhea and was referred for identification of the cause of her pain. 2D US revealed a normal right hemicavity and haematometra in the left hemicavity.As the patient wished to retain her childbearing ability, we performed a laparoscopic excision of the left horn with reconstruction of the uterus . No furtA 39-year-old patient presented with severe dysmenorrhea since menarche. Details of her obstetric history included 2 live children and a miscarriage in 2010. She also had a history of grade 4 endometriosis for which she had been operated on previously. An endometrioma on the left side had been excised in 2011. The patient was referred for routine ultrasonography with severe dysmenorrhea and recurrent endometriosis on the left side. As shown in As the patient had completed her family, and due to adenomyosis with recurrent endometriosis, we performed an elective hysterectomy with left-sided salpingo-oophorectomy.After the surgery, she reported no pain and felt satisfied.A 28-year-old patient with three mid-trimester miscarriages in the 16th week of gestation was sent for ultrasonography.A small communicating uterine cavity was seen on the right side, and a blind uterine cavity on the left side. The external shape of the uterus was normal. While we were planning a hysteroscopic septum resection with unification of the two uterine cavities, the patient became pregnant again. Unfortunately, due to relocation the patient was lost to follow up and there is no further information regarding pregnancy outcome.The female reproductive tract develops from a pair of Müllerian ducts that form the following structures: fallopian tube, uterus, cervix and the upper two-thirds of the vagina. The ovaries and lower third of the vagina have different embryological origins derived from germ cells that migrate from the primitive yolk sac and the sinovaginal bulb, respectively .The Müllerian duct is a mesoepithelial tube derived from intermediate mesoderm and composed of both epithelial and mesenchymal cells. Transcription factors and signaling molecules are necessary for the interaction between the epithelium and the mesenchyme in order to develop the Müllerian ducts. Some of these transcription factors include EMX2, HOXA13, PAX2, LIM1, and Wnt. Lim1(Lhx1) and Pax2 .The normal development of the Müllerian ducts depends on the completion of three phases: organogenesis, fusion and septal resorption. Organogenesis is characterised by the formation of both Müllerian ducts. Failure of this leads to a uterine agenesis/ hypoplasia or a unicornuate uterus. Fusion is characterised by fusion of the ducts to form the uterus. Failure of this results in a bicornuate or didelphys uterus. Septal resorption involves subsequent resorption of the central septum once the ducts have fused. Defects at this stage result in a septate or arcuate uterus .In our case series, we presented five different cases of patients with Robert`s uterus. Depending on the symptoms, the respective life situation, age and treatment options, individualised surgery was performed.Robert's uterus is extremely difficult to diagnose preoperatively, and is therefore missed frequently. Diagnostic modalities may include US, hysterosalpingography (HSG), and MRI. 2D US is not very sensitive for the detection of Robert's uterus; the condition is frequently misdiagnosed as a unicornuate uterus with a non-communicating rudimentary horn, as in our above-mentioned case number one. While HSG may raise suspicion of a unicornuate uterus with a typical "banana" shape and only one fallopian tube, a septate uterus cannot be clearly visualised by HSG without imaging the external fundus. MRI is the best modality to demonstrate the uterine septum, the normal external fundal contour, haematometra and haematosalpinx, but is an expensive procedure. In cases of Robert's uterus, coronal T2-weighted MRI images are ideal for demonstrating the uterine septum dividing the endometrial cavity asymmetrically along with the blind-ending cavity and haematometra. T1-weighted images show the haematometra and haematosalpinx as bright fluid in the endometrial cavity and in a dilated fallopian tube.3D US is equivalent to MRI and, in our opinion, even better in providing a correct diagnosis. Both diagnostic techniques require an experienced investigator.Gynaecological examination and 2D US are recommended for the evaluation of asymptomatic women. 3D US is recommended for the diagnosis of female genital anomalies in ‘symptomatic’ patients belonging to high-risk groups, and anomalies suspected during the routine investigation in asymptomatic women. MRI and endoscopic evaluation are recommended for patients with suspected complex anomalies or in cases of diagnostic dilemma. Adolescents with symptoms suggestive of a female genital anomaly should be thoroughly evaluated with 2D US, 3D US, MRI, and endoscopy .With the use of modern 3D US technology, a well-trained and dedicated ultrasonographer or gynaecologist will be able to establish Robert's uterus non-invasively and plan the correct surgical procedure accordingly. The high quality of tissue characterisation by MRI makes it an excellent diagnostic modality to confirm the diagnosis of Robert's uterus and differentiate between septate and bicornuate uteri. Many studies have shown the efficacy of MRI as well as its ability to demonstrate the endometrial cavity and uterine contours in exquisite detail. However, 3D ultrasonography is an effective alternative because it provides images of very similar quality as MRI . In addiIn 2000, Welch et al. proposed a real-time freehand 3D US system for image-guided surgery, which utilised a 5-MHz linear transducer and an optical positioner for location and orientation . At a raSurgical correction is the sole effective treatment of Robert's uterus. The condition was managed via laparotomy in the early 1970s. Total resection of a hemicavity or endometrectomy of the blinded cavity, abdominal metroplasty, or a combination of these procedures with hysteroscopy/laparoscopy were used. Irreversible surgeries (resection of hemicavity/endometrectomy of the blinded cavity) were performed to improve the shape and volume of the uterine cavity. A complete endometrectomy of the blind hemicavity is suitable only in patients with no communication between the blind cavity and the ipsilateral fallopian tube. A complete endometrectomy is believed to prevent the recurrence of haematometra. In 2011, Vural et al.  performeThe term metroplasty describes a reconstructive surgery used to repair congenital anomalies of the uterus, including septate uterus and bicornuate uterus. It is an accepted method of treatment in women with recurrent miscarriages and septate uteri and it significantly improves the subsequent reproductive outcome. The incidence of spontaneous miscarriage and preterm delivery seems to decrease, whereas the incidence of term delivery rate increases .Hysteroscopic metroplasty for septate uterus seems to be a simple and relatively safe procedure, but nevertheless complications can occur either during the procedure  or in subsequent pregnancy and childbirth  . NeverthAlthough the current data does not give any definite proof of a causal relationship between septate uterus and infertility, considering the simplicity of the procedure, with low associated morbidity and the reported outcomes, the procedure should be considered in women with longstanding infertility and a septate uterus .For hysteroscopic endometrial ablation or resection, surgery will be more effective if undertaken when endometrial thickness is less than 4 mm, in the immediate post-menstrual phase. However there are often difficulties in reliably arranging surgery for this time . The othOur five patients were treated in accordance with the most modern standards. The entire range of treatment options were taken into account. In the fifth patient with recurrent miscarriages and no viable pregnancy, we planned the initiation of protective contraception to avoid further miscarriage. However, the patient became pregnant again.We discuss five cases of Robert's uterus, a rare type of Müllerian duct anomaly from diagnosis to treatment. Our study underlined the value of 3D vaginal US technology in detecting this congenital uterine anomaly, followed by hysteroscopy in combination with laparoscopy for final confirmation of the diagnosis. The diagnosis of Robert's uterus remains a challenge for clinicians.


Peritoneal loose body (PLB) is a rare clinical entity. It is generally agreed that the most common origin of the loose bodies is appendix epiploica. We here report a case of PLB that looks like a “boiled egg,” which was misdiagnosed preoperatively as a lesion of hepatic origin and was confirmed by operation and postoperative pathology. PLBs are rare entities, a good understanding of their specific imaging features can help prevent misdiagnosis, but sometimes an accurate preoperative diagnosis is still difficult to achieve. Exploratory laparoscopy is a recommended method for management of PLBs. Its name originates from its changeable position. In other words, it tends to move obviously with the change in the patient’s position. The incidence of PLB is occult and preoperative diagnosis is difficult. Moreover, it is often found accidentally in imaging physical examination, abdominal exploration operation, or autopsy. Though the reason for the formation of peritoneal free bodies remains unclear, the role of infarction, saponification, and calcification after chronic volvulus of intestinal fat is speculated ,3,4,5,6.Herein we report an unusual case of PLB and review the literature. We hope that, through our case, a good understanding of their specific imaging features may help prevent misdiagnosis.2We performed a narrative review of the literature by searching “peritoneal loose bodies” and “peritoneal mouse” on PubMed and Scopus. Case reports and reviews were chosen and used to extract data regarding gender, age, clinical feature, number of lesions, size of lesions, preoperative diagnosis, surgical procedure, intraoperative lesion location, and free status . Two authors (WY and CY) independently carried out online research. Full texts of relevant articles were further assessed for inclusion in this study, and the characteristics of 50 cases that have been reported are shown in 3A 69-year-old man referred to outpatient hepatology because of a 3-month history of abdominal discomfort mainly in the upper quadrants. Other symptoms were denied. The patient’s medical history included hypertension for more than four years. Physical examination findings were normal, and the intra-abdominal mass could not be palpated. The laboratory test results including complete blood count and liver function test results were normal.The echocardiography examination revealed a hypoechoic mass with a clear boundary and regular shape in the second hilar region. It contained hyperintense echogenic foci with posterior acoustic shadowing, indicating dense calcifications, and no obvious blood flow signal was detected in the mass . The CT To confirm the diagnosis and determine the proper treatment strategy, we performed laparoscopic surgery, finding a smooth, free, and “boiled egg-like” mass. This mass was removed by laparoscopy. The PLB was about 3 cm × 2.5 cm × 2 cm in size, elastic, white-color in appearance, and smooth on the surface. The cut surface showed a core with yellow material . MicroscEthics approval and consent to participate: This study was approved by the Research Ethics Committee of Zhejiang Provincial People’s hospital. Written informed consent was obtained from this patient.4The PLB or “peritoneal mouse,” is an extremely rare clinical entity. Most of the related reports are based on individual cases. As shown in Though the exact pathogenesis of PLB is unknown, the tissue in the center of the PLB is the prerequisite for the formation of the free body. At present, scholars speculated that the tissue may come from the following substances : (1) thePLB is free in the peritoneal cavity and has no specific position, it will move with the change in body position or respiratory and gastrointestinal movement, but the migration may be substantially impeded by the adhesions or abdominal organs in the vicinity ,9,17,18.5Notably, small asymptomatic PLBs often require no special treatment and can be monitored by regular medical reviews. Nonetheless, surgery is the main treatment in symptomatic patients, which can not only relieve symptoms but also promote definitive diagnosis. Exploratory laparoscopy is a recommended means for the detection and management of PLBs, which reduces surgical damage, minimizes postoperative complications, and shortens recovery time .


A 42-year-old woman was admitted to the gastrointestinal department of Taleghani Hospital, a teaching referral hospital in Tehran, Iran, with epigastric pain, fever, and dysphagia. She complained of intermittent dysphagia, regurgitation, and epigastric pain (with no benefit from PPI treatment) one month in each year from five years before admission.The patient was married and resided in Tehran, Iran. She had no history of smoking, opium addiction, alcohol consumption, or consumption of any medications. She had no family history of gastrointestinal cancers or problems similar to hers. From five years ago, she had experienced dysphagia and pain in the throat and epigastric area which had even been treated with the diagnosis of thyroiditis in the first year of her symptoms. Her BP = 110/70, HR = 98, and T = 37.7 at the time of admission. Hemoglobin was the only abnormal laboratory finding (Hb = 10) that could be explained by minor thalassemia.An upper GI endoscopy was done for the patient, but the scope could not passed because of an esophageal lesion presence in 20-25 cm from incisura .A mediastinal CT scan showed a non-enhancing lesion with well-defined margins in the middle to distal esophagus with no invasion or lymph node involvement .Endosonography was another diagnostic procedure which showed one hypoechoic lesion with diameter of 46*25 mm and length of 13 mm (from 20 cm down to 33 cm of incisors) which contained lots of debris and was surrounded by a double layered wall . What is your diagnosis?What is the next step?Esophageal duplication cysts are usually asymptomatic gastrointestinal lesions that are infrequently seen and often diagnosed as a secondary finding .Duplications can be found along the entire length of the esophagus with varying presentations depending on their location and compression effects on neighboring structures. The complicated duplications can present with life-threatening symptoms .Esophageal duplication in adults is rare. Esophageal cyst is the second most common duplication of the GI tract .Surgery has become the best treatment for this disease . On endoThe authors declare that they have no conflict of interest.


We have presented a patient with ingrowing liver as an atypical diaphragmatic hernia recurrence and discussed major aspects of diagnostic methods and the selection of an appropriate operative treatment. (2) Case description: We discuss a case of a patient with right-sided recurrent CDH  who had primary thoracoscopic repair in newborn period. During infancy and early childhood, the patient presented recurrent upper and lower respiratory tract infections and bronchial hyperreactivity. The clinical picture was initially unclear. A CT scan was inconclusive to diagnose a recurrence. The patient was scheduled to have a re-thoracoscopy. A part of the liver was herniated into the pleural cavity. This fragment of ‘ingrowing’ liver was removed, and the diaphragmatic secondary defect was repaired. (3) Conclusions: This case proved that thoracoscopy can be a preferred technique in the diagnosis and treatment of CDH recurrence.  Recurrent diaphragmatic hernia is a relevant diagnostic and treatment dilemma that has been reported in the literature. The recurrence rate in the series available is described in a wide range of cases, and it seems to be higher for endoscopic approach—7.9% ,2,3. The recurrence of a diaphragmatic hernia is usually apparent in chest radiographs. Other patients require a US or CT/MRI (Ultrasonography or Computed Tomography/Magnetic Resonance Imaging) scan to confirm a diagnosis . The reaThe aim of this study is to present a case of an atypical recurrent CDH in a 4.5-year-old patient and to discuss the difficulties in the diagnostic process, the selection of an appropriate operative treatment and the atypical mechanism of recurrence manifested as an ‘ingrowing liver’.The 4.5-year-old girl was operated on within the first day of her life. A right-side thoracoscopy was performed, during which bowels loops and the right liver lobe were relocated into the peritoneal cavity. The medium-sized 3 cm × 3 cm (type B) diaphragm defect was primarily closed with non-absorbable interrupted sutures. The right lung appeared to be hypoplastic during the procedure.During infancy and early childhood, the patient presented recurrent upper and lower respiratory tract infections and bronchial hyperreactivity. In the beginning, the symptoms included coughing, wheezing, shortness of breath and abnormalities in the physical examination, which was completely withdrawn after drug treatment. The symptoms intensified seasonally, and they became more frequent and resistant to the symptomatic treatment and antibiotics. The patient’s psychomotoric development and weight were adequate. Conventional X-rays did not reveal anything specific. The patient was diagnosed with bronchial asthma, allergy and mild stenosis of the left main bronchus, revealed in bronchoscopy. At the age of four, the patient went down with lobar pneumonia . The X-ray revealed areas of lung inflammation typical for pneumonia. On ultrasound examination during the control visit, a structure 17–18 mm of right, supradiaphragmatic localization was described, with suggestions of recurrent hernia, postoperative residue, or lesion with other etiology. Due to an inconclusive result, a CT scan of the thorax was performed. The CT examination was performed using a 128-slices CT scanner and a low-dose scanning technique under general anesthesia. The CT examination revealed a small, rounded structure with density and contrast enhancement similar to liver tissue in the right dome of diaphragm. The CT examination did not reveal the presence of any accompanying pathological opacities in the surrounding pulmonary parenchyma or the presence of any fluid in the right pleural cavity . The exaThere was no complication in postoperative period. The hospitalization length was four days. According to a minor size of diaphragmatic defect and herniation part of liver, the prognoses of respiratory symptoms have been good, and the previous symptoms might rather have been caused by lung hypoplasia, diaphragmatic impairment and other reasons.Due to the divergent results of the ultrasound and CT, the patient was scheduled for diagnostic re-thoracoscopy. In the right pleural cavity, the surgeon cut many adhesions and identified a spherical, 2 cm × 2 cm sized structure with a narrow neck that penetrated into the diaphragm . MacroscDiagnostic difficulties presented in this case are the main aspect to discuss. The repeated conventional chest radiographs did not reveal any pathologies, except for inflammatory changes. Only the US showed supradiaphragmatic pathology with an inconclusive suggestion; however, this method depends on the experience of the radiologist performing the examination . In manyIn our case, the thoracoscopy made it possible to confirm the atypical recurrent CDH, to repair the defect in diaphragm at the same time and to take the tissue to histopathological examination. The thoracoscopy enabled distinguishing liver tissue from the diaphragm which was inconclusive in CT because of the similar density and contrast enhancement of both tissues. MIS techniques provided a very precise, detailed, enlarged picture of anatomical structures , so thatAnother aspect to discuss is the mechanism of formation of the recurrent diaphragmatic hernia in this case, which determined the unusual clinical picture and development over time. During the first thoracoscopy, the defect was completely primarily closed by several interrupted non-absorbable sutures. The re-thoracoscopy confirmed that secondary defect was very small, and the sutures were still on site. The size of the defect was similar to a narrow neck of the structure and lower than the main part of the herniation liver. It may indicate a different mechanism of recurrence and arising the herniation. The concept of ‘ingrowing liver’ may be a possible explanation. The space between primary sutures or thin, fibrous tissue in localization of repaired defect could be a gate of recurrence. The liver has regenerative potential, so it could probably grow up and into the pleural cavity. The radiologist did not describe any pleural effusion or inflammatory-atelectatic lesions, which are typical for recurrence. This proves the small size of secondary defect. In our case, the intrathoracic part of liver was very small. Probably, if the ingrowing liver, with its proliferative potential, had enlarged in the thoracic cavity over time, it would have cause respiratory symptoms as the result of lung compression. An interesting, different concept of liver presence in thoracic cavity is the hepatic heterotopia, classified into four types. The literature suggested some types are associated with CDHs, such as small, accessory liver lobe connected with the main liver, an isolated ectopic liver forming a macroscopic nodule and a microscopic, ectopic liver tissue ,8. It haThe unconventional clinical picture involves the age at diagnosis and non-specific, recurring respiratory symptoms. The respiratory symptoms can occur as a consequence of lung hypoplasia  and diapA relevant aspect is the choice of surgical technique. The considerable issue that could diminish the risk of ingrowth of liver is proper distribution of sutures—not too close to the border of defect, and not too far from each other. An alternative could be a tile-like overlap arrangement of sutures, which could be a valuable amendment in CDH repair. This technique may prevent ingrowth of the liver to the thoracic cavity. Other technical modifications are continuous suture, horizontal mattress sutures, pledgeted interrupted sutures with specific, described by Kamran et al., sandwich-type buttressed repair . They alThoracoscopy may be a useful and preferred technique in diagnosis and treatment. The thoracoscopy may be complemented with ultrasonography and multidetector computed tomography (MDCT) in the diagnostic process of questionable cases. The advantage of the endoscopic technique is the ability to confirm the diagnosis macroscopically or by taking tissue to histopathological examination and to treat the disease at the same time.Minimally invasive techniques provide a precise, enlarged picture of anatomical structures so some difficult to identify details such as a small part of ingrowing liver could be identified and treated with improved precision. The concept of ‘ingrowing liver’ may be a possible explanation of a mechanism of the atypical diaphragmatic hernia recurrence in our case.The aesthetic effect and small postoperative scarring also give the thoracoscopy an advantage over open surgery.


Objective: This study aimed to assess the operability of thalamic tumors since they are generally considered to be inoperable and to have poor outcomes. Advancements in neuroimaging, neuronavigational technology, and intraoperative neurophysiological monitoring allow accurate planning and safe resection.Methods: Clinical data and reports of 10 pediatric patients with thalamic tumors were retrieved retrospectively. All 10 patients underwent surgical intervention. Diffusion tensor tractography (DTI) was used preoperatively to select the safest surgical route. Intraoperative MRI and postoperative MRI were used to evaluate the extent of resection.Results: There were three gross total resections (GTRs), two subtotal resections (STRs), two partial resections (PRs), and three biopsies. All patients had unilateral thalamic tumors. Different surgical approaches were used according to the relationship with the internal capsule and corticospinal tract and according to the preoperative DTI. Five patients had pilocytic astrocytoma, two had diffuse pediatric-type high-grade glioma, one had ganglioglioma, one had pediatric-type diffuse low-grade glioma, and one had atypical teratoid rhabdoid tumor (ATRT). The outcomes of low-grade tumors were favorable, especially for those who underwent resection, and those of high-grade tumors were poor regardless of the extent of resection.Conclusion: Our review shows that surgical resection of thalamic tumors can be done safely and offers favorable outcomes for patients with low-grade tumors, even without adjuvant therapy. Our study provides further evidence for thalamic tumors operability and safe resection. Thalamic tumors represent approximately 1-5% of pediatric brain tumors ,3. AdvanWe reviewed the medical records of 10 pediatric patients diagnosed with thalamic tumors at the Department of Pediatric Neurosurgery National Neuroscience Institute/King Fahad Medical City. All related medical data, including history and physical examination, imaging findings, operative reports, histopathology, adjuvant therapy, and follow-up data, were retrieved. MRI of the brain was performed in all patients to determine the tumor location and relation to the surrounding structures. Diffusion tensor tractography (DTI) was done in all patients preoperatively. All images were reviewed and reported by neuroradiologists. Neuronavigation was performed in all patients, and the fiber tracking technique was used and illustrated in one patient (case illustration). Intraoperative monitoring IOM was used in all patients except those who underwent biopsy. Based on intraoperative and postoperative MRI, the extent of resection was categorized into gross total resection , subtotal resection , and partial resection . Neurological evaluations including mental status assessment and power examinations were performed in all patients, and accordingly, the patients were divided into two groups: stable, compared with the preoperative evaluation, and worsened.Clinical characteristicsTen patients were reviewed and included in the study. Their ages ranged from three to 13 years. The male-to-female ratio was 3/7. Hemiparesis was the most common symptom (n=8), followed by increased intracranial pressure (n=5). Headache and dizziness were the presenting symptoms of two patients, and they had no neurological deficits.Radiological featuresAll patients had a unilateral thalamic tumor - three had a right-sided thalamic tumor and seven had a left-sided thalamic tumor. Three had thalamopeduncular tumors, three had thalamic tumors with intraventricular extension, two had thalamic tumors with extension through the tentorium, one had a mesial thalamic tumor, and one had a thalamotemporal tumor. DTIs were performed in all patients to evaluate the relationship between the tumor and the basal ganglia and internal capsule and to choose the safest surgical route.Surgical intervention and extent of resectionWe performed three biopsies and seven tumor resections. Three cases had GTRs, two had STRs, and two had PRs. Surgical routes were selected based on the evaluation of DTI images preoperatively. Four tumor resections were performed via transcortical routes and three via transcortical-transventricular routes. Two stereotactic biopsies and one endoscopic biopsy were performed.Neurological status after surgery and histopathological findingsAmong tumor resection cases, only one patient worsened after surgery, but the patient returned to the baseline neurological status after one month. Two cases remained stable. Four cases improved after surgery. The histopathological diagnoses of the 10 patients were as follows: five patients had pilocytic astrocytoma, two had diffuse pediatric-type high-grade glioma, one had ganglioglioma, one had pediatic-type diffuse low-grade glioma, and one had atypical teratoid rhabdoid tumor (ATRT) .Adjuvant therapy and follow-upAll high-grade tumor cases had chemotherapy and radiotherapy following surgery. Among low-grade tumor cases, only one patient had chemotherapy after surgery. The mean follow-up time of the low-grade tumor cases was 27.5 months, and that of the high-grade tumor cases was 6.2 months.OutcomeAmong low-grade tumor cases, no death event was reported during the whole follow-up period (six to 96 months). There was no tumor recurrence or residual progression, but there was one case of tumor regression after chemotherapy. All high-grade tumor cases had tumor progression, and two death events were reported Table .Thalamic tumors are considered inoperable tumors, and radical surgical resection is not recommended because of the associated morbidities and poor outcomes . Biopsy In our study, we found that GTR and STR were associated with a high survival rate and low recurrence rate and residual progression, particularly in low-grade tumor cases, even without adjuvant therapy. In high-grade tumor cases, the outcome was considered to be poor because two patients who underwent STR passed away and one patient who underwent PR had progression during chemotherapy and radiation therapy. We were able to perform a radical resection with one transient morbidity and no death events related to the surgery. Radical surgical resection (GTR and STR) was achieved in 50% of our cases, which is comparable to the results of other series ,12,14. APreoperative planning, employment of DTI, neuronavigation, and intraoperative neurophysiologic monitoring are the cornerstones of surgical safety in addressing thalamic tumors. Utilization of DTI to select the surgical route in thalamic tumors has been discussed in multiple studies ,15-18. SCurrently, we employed high-definition fiber tracking (HDFT) in our cases, and we present an illustrative case. We found it to be a very useful tool that provides us with clear and precise visualization during surgical planning and intraoperatively. HDFT is used to identify the entry point, trajectory and corticospinal tract (CST), and interface between the thalamus and tumor. There is only one case series in the current literature that utilized HDFT in the surgical treatment of thalamic tumors in a pediatric population . CeltikcIllustrative caseAn 11-year-old boy presented with a three-week history of progressive weakness. His examination revealed right-sided hemiparesis with motor power 2/5. MRI demonstrated a heterogeneous mass with internal necrosis and hemorrhagic components centered within the left thalamus with involvement of the left cerebral peduncle and basis of the midbrain suggestive of high-grade glioma (Figure Neuronavigation integrated with DTI and fiber tracking was performed Figure . The patOur review shows that surgical resection of thalamic tumors can be done safely and offers a favorable outcome for low-grade tumors, even without adjuvant therapy. High-definition fiber tractography and utilization of the fiber tracking technique might be considered to maximize the extent of resection safely. The ability to identify low-grade tumors based on images needs further studies and will help neurosurgeons in decision making and surgical planning.


Pleural lesions form a diagnostic challenge for the radiologist. Whereas lesions can be initially detected on chest radiographs, CT and MRI imaging are the imaging modalities of choice for further characterization. In a number of cases, imaging findings can be relatively specific. In general unfortunately, imaging findings are rather aspecific. Evolution and extrathoracic imaging findings are important clues toward the diagnosis. CT and MRI are the modalities of choice for imaging (juxta)pleural tumors.Imaging findings may be aspecific and diagnostically challenging.Patient characteristics and evolution in time are important clues toward the diagnosis.The thoracic space is divided into right and left pleural cavity with a serous membrane, the pleura, contouring both cavities. The pleura is composed of two thin layers of mesothelium: the visceral and parietal pleura. The visceral pleura covers the surface of the lungs and fissures, whereas the parietal pleura covers the thoracic wall and diaphragm. In most cases, the lung does not completely fill the pleural cavity, allowing the parietal pleurae to converge, forming the pleural recess. The visceral pleura is vascularized by arterial branches of the bronchial and pulmonary arterial system, whereas the parietal pleura by intercostal vessels (systemic supply). Due to intercostal nerve innervation, the parietal pleura is sensitive to pain. Both pleurae form a two-layered pleural space containing approximately 5 ml of physiological pleural fluid. Histologically, the pleura is defined as a serous membrane composed of mesothelial cells and loose connective tissue , 2.There is a broad variation in pleural pathologies, with both benign and malignant etiologies. The real prevalence of all pleural pathologies is unclear in the literature. The most common primary malignancy of the pleura is a malignant mesothelioma, with an estimate of 10,000 new cases annually across Western Europe, Scandinavia, North America, Japan, and Australia . PleuralThis article focuses on primary pleural malignancies, other than mesothelioma, and juxtapleural malignancies. The aim of this pictorial review is to provide a structured overview of the imaging features, with clues for diagnosis and differential diagnosis Table .Table 1RConventional radiography (CR) is often the first-line imaging modality for patients with thoracic complaints. One of the signs of an extrapulmonary lesion on chest CR is an incomplete border sign, in which part of the border is invisible. Other signs suggestive of a pleural lesion are displacement of the pulmonary vasculature and respiratory-associated changes in location of the lesion . HoweverPleural lymphomas are mainly seen as secondary when in association with other thoracic abnormalities or in cases of aggressive subtypes. They arise from the direct extension from a pulmonary or nodal disease, or through hematogenous or lymphatic dissemination. Patients present with aspecific complaints, including chest pain, dyspnea, or cough. Pleural effusion is one of the main manifestations on imaging. Pleural thickening or solid pleural lesions are less commonly seen with frequently missed pleural lesions due to small size or fluid superposition. Both unilateral and bilateral pleural involvements are possible , 12.Primary pleural lymphomas are rarely diagnosed and consist of two distinct subtypes . The firThe second subtype is the pyothorax-associated lymphoma (PAL). This Epstein-Barr virus (EBV)-positive DLBCL is exclusively seen in patients with chronic pyothorax, usually due to iatrogenic-induced pneumothorax from tuberculosis treatment . The higThe term mesenchymal tumors includes a broad spectrum of various pathologies originating from transformed cells of connective tissue origin. One of the characteristics of these primary malignancies is that most of them are focal.Solitary fibrous tumors (SFTs) are the second most common primary pleural tumors following mesothelioma, although less than 800 cases have been reported in the literature. Patients from all ages have been described, but predominantly in the age range of 50 to 60 years . Since uStandard chest radiography revealed a well-circumscribed pulmonary mass Fig.  with varCT is the imaging modality of choice and allows a detailed view of a well-circumscribed mass. SFTs are generally heterogeneous on CT Fig. , with foMRI can be used to evaluate the relationship between the chest wall and diaphragm. Typically, a low to intermediate signal intensity on T1-weighted images (WI), and low signal intensity (SI) on T2-WI are seen Fig. . After iThe most diagnostic challenge in SFTs is the differentiation between benign and malignant tumors . The mosData on the use of 18-F-FDG PET imaging in SFTs are scarce, and FDG-PET seems to be of little value in the diagnosis of benign and malignant tumors and to differentiate between them. Malignant tumors tend to be more hypermetabolic than benign tumors, but there is an overlap in standardized uptake value (SUV) max values between these tumors .Fig. 5DiDesmoid-type fibromatosis, also known as aggressive fibromatosis, is a benign disease characterized by fibroblast proliferation with an infiltrative character. Although these tumors are not intrinsically malignant, they are often classified as such because of their infiltrative character and high risk of recurrence after surgical resection. Desmoid tumors are predominantly seen in young patients with a female-to-male ratio of 2:1 . An undePleural sarcomas are most frequently encountered in the context of metastases and rarely as primary pleural sarcomas.Synovial sarcomas are observed in patients with a wide age range and are mainly found as soft tissue sarcomas of the extremities. Primary pleural presentations are rare. On imaging studies, they manifest as a well-defined heterogeneous mass with intralesional necrosis or hemorrhage Fig. , and lesAngiosarcomas are predominantly observed in adult males. They range from low-grade epithelioid hemangioendotheliomas (EHEs) to high-grade angiosarcomas and are typically very aggressive, especially in the high-grade form. EHE is a rare vascular tumor that may present at different sites in the body. Only 200 cases of pulmonary EHE have been reported worldwide, and only 27 have been classified as pleural in origin . PatientThe etiology of desmoplastic small round cell tumor (DSRCT), a rare tumor, is believed to be a genetic alteration of two different tumors (Wilms tumor and Ewing’s tumor). This may explain the young age of the affected population. The clinical presentation is aspecific. DSRCT can present as a single lesion or as diffuse pleural involvement with pleural effusion. This diversity of presentation patterns can be attributed to the dual genetic alterations. Locoregional bone invasion has been described as an associated finding. Imaging findings are aspecific and impose a diagnostic challenge. In general, surgical biopsy is needed for the definite diagnosis of DSRCT , 31.Juxtapleural tumors are closely related to the pleura and chest wall and may originate from osseous structures, cartilage, soft tissue, muscles, nerves, or fat. Although imaging findings may overlap or be aspecific, some tumors may present with a particular imaging feature or specific clinical setting.Chondrosarcoma is the most common primary tumor of the chest wall and is most commonly seen in elderly patients. Chondrosarcomas are mainly located anteriorly in the chest wall and in the upper five ribs Fig. . ChondroOsteosarcomas are primarily found in long bones; chest wall location is rare and is mainly observed in an elderly population. Osteosarcomas contain new neoplastic bone and/or disorganized ossification. Imaging characteristically reveals a heterogeneous mass with associated bone destruction Fig. . HeterogThe second most common primary malignant bone tumor in children and adolescents is the Ewing sarcoma, with a slight male predilection . The primary complaint of patients is pain, which depends on the location, the feeling of a mass, or swelling. Ewing sarcoma can occur in extraskeletal sites in the chest, such as pleural and paravertebral sites. Most lesions have an aggressive appearance with locoregional bone destruction and a large soft-tissue component Figs. , 13 33]33].Fig. A leiomyosarcoma arises from the smooth muscles and can be located adjacent to the bronchus, pulmonary artery, or peripheral pulmonary parenchyma. Depending on the site, it may evoke dyspnea, chest pain, or hemoptysis. Imaging shows a well-defined smooth or lobular mass with a homogeneous or necrotic aspect Fig.  32].Fig.Fig32].FNeurogenic tumors with juxtapleural locations may arise from spinal nerve roots or intercostal nerves Fig. . Benign Liposarcomas are the malignant counterparts of the frequently found lipomas. Depending on the size, patients may present with thoracic complaints and discomfort. As these lesions grow slowly, patients mostly remain asymptomatic for a long period.  The diagA number of infectious diseases may present as tumors or tumorlike pleural or juxtapleural diseases, mimicking a malignancy, for example, a chest wall cold abscess caused by tuberculosis. This is a rare entity, often simulating a pleural tumor when seen in the chest wall region. Patients may present with thoracic pain, but the pathognomonic clinical symptoms of tuberculosis, such as fever, weight loss, or night sweats, are not always present. Imaging shows a well-defined lobular mass against the pleura Fig. , usuallyPleural lesions are a diagnostic challenge for radiologists. Patient characteristics, such as age, immune status, hypoglycemia, and previous medical history, can guide the radiologist to the correct diagnosis. Although lesions can be initially detected on chest radiographs, CT and MRI are the imaging modalities of choice for further characterization. Imaging findings can be relatively specific in a number of cases, such as in SFT , osteosarcoma (an aggressive lesion closely related to bone), or chondrosarcoma (location and chondroid matrix). In general, imaging findings are rather aspecific. Evolution is an important clue for the diagnosis. Additional extrathoracic imaging findings such as lymphadenopathies, organomegaly, and polyposis of the colon are important for accurate diagnosis. Discussion on a multidisciplinary thoracic oncology tumor board is vital for different steps in the diagnostic process, from detection on imaging studies to guiding the diagnostic approach , histopathologic diagnosis, and defining the treatment strategy.


Epidermoid cyst of the buccal mucosa is rare. Nevertheless, it must be included in the differential diagnosis of swelling in this area. The diagnosis is based on anatomopathological examination. The surgical enucleation is the gold standard of treatment. Epidermoid cyst of the buccal mucosa is rare. Nevertheless, it must be included in the differential diagnosis of swelling in this area. The diagnosis is based on anatomopathological examination. The surgical enucleation is the gold standard of treatment. In the oral cavity, these are uncommon and account for <0.01% of all the oral cysts. These cysts often remain asymptomatic for years however may become symptomatic as a result of enlargement or superinfection.Epidermoid, dermoid, and teratoid cysts are nonodontogenic lesions derived from the germinative epithelium.The definitive diagnosis of epidermoid cyst is based on the anatomopathological examination. These cystic lesions were classified since 1955 by Meyer into epidermoid, dermoid, and teratoid variant. Dermoid cysts are lined by epidermis and contain skin adnexa such as sebaceous glands, sweat glands, and hair follicles. When there are no adnexa, these cysts are termed as epidermoid or epidermal with the lining containing only epithelium. Teratoid cysts consist of dermoid material plus tissue of other embryonal sources like respiratory, gastrointestinal, and connective tissues such as bundles of striated muscle and distinct areas of fat.The midline or sublingual region of the mouth floor is the most commonly affected area contrary to the buccal mucosa which seems to be an unusual site of occurrence.2A 56‐year‐old man with no past medical history was referred to the Oral Medicine and Oral Surgery department at the university clinic of dental medicine, Monastir, Tunisia, with the chief complaint of a painless swelling in the left buccal mucosa for about 5 years. The patient had neither known drug allergies nor history of surgery and/or trauma related to the lesion area.Extraoral examination revealed the presence of a solitary swelling in the left cheek covered by normal looking skin in color and texture, as well. The swelling was nontender and freely mobile to the skin, on palpation. No submental nor submandibular lymphadenopathy was detected Figure .Intraoral examination showed a poor oral hygiene and a nodule in the left buccal mucosa covered by a healthy mucosa. The mass was soft and non‐mobile on palpation Figure .Due to these clinical findings, the initial diagnosis was benign tumor of either the oral mucosa (including vascular lesions) or the salivary gland.An ultrasonography was required. It revealed a well‐defined walled hyperechoic heterogenous lesion measuring 34 × 31 × 21 mm with posterior ultrasound reinforcement in the left buccal mucosa. Vascular lesions were excluded on Color Doppler analysis. At this stage, the diagnosis of epidermoid cyst of the left buccal mucosa was evoked  OR ) AND ,” until December 2020. Moreover, the reference lists of relevant articles were manually searched for additional studies. This bibliographic research concluded to 9 case reports about epidermoid cyst of the buccal mucosa from 8 articles. The parameters extracted from these cases were summarized in Table According to this literature review, the two first cases of epidermoid cyst in the buccal mucosa were published by Schneider, Mesa in 1978, and involved women in the fourth decade of their lives.In the same year (1978), Gutman et al.Epidemiologically, dermoid and epidermoid cysts may be present at birth and also in old patients, with the majority occurring in the second and third decades of life.Even though that the frequency of occurrence of (epi)dermoid cysts is equal in both genders,rd and 4th weeks of intrauterine life.Many etiopathogenetic theories have been proposed for the development of (epi)dermoid cysts. These cysts are classified to congenital or acquired lesions.The size of the cyst is variable from millimeters till some centimeters, depending on its first clinical manifestation.There may be a left side predilection of the occurrence; as reported in the present case.Swellings in the buccal mucosa may lead to a series of clinical diagnoses, as some conditions may present in a similar aspect making the diagnosis difficult.Odontogenic infections affecting the facial spaces of buccinator and masseter muscles, pleomorphic adenoma, mucocele, cervical lymphadenopathy, hemangioma, lipoma, fibroma, neoplasms, and dermoid cyst are considered as differential diagnosis of lesions occurring in the buccal mucosa with a clinical aspect similar to the present case.In the present case, odontogenic infection was ruled out as the lesion was not associated clinical symptoms such fever and malaise. Such the lack of nodal involvement, the slow progression of the lesion and its benign appearance, neoplastic conditions were excluded, as well.To achieve the correct diagnosis and differentiate between vascular, salivary and mucosal lesions, specialized imaging techniques such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) should be carried out.The surgical excision or enucleation is the gold standard treatment via an intraoral or extraoral approach, depending on the size and the location of the cysts.Although recurrences have been described as <3%,Despite the benign nature and the good prognosis of dermoid or epidermoid cysts,4Epidermoid cyst of the buccal mucosa is extremely rare. Nevertheless, it must be included in the differential diagnosis of swelling in this area. To exclude other conditions, like benign tumors and odontogenic infection, appropriate imaging techniques are necessary in preoperative.The diagnosis is based on anatomopathological examination. The surgical enucleation is the gold standard of treatment, and usually without recurrence.None.ND wrote the manuscript. AC involved in surgery performance and manuscript revision. AS ensured patient follow‐up and involved in manuscript drafting. AB analyzed and interpreted the specimen. AB, SS, and HH involved in manuscript revision. JS revised the manuscript for important intellectual content.This manuscript is the authors' own original work, which has not been previously published or considered for publication elsewhere. All authors have been personally and actively involved in substantial work leading to the paper and will take public responsibility for its content.The written consent for publication was released by the patient.


Ectopic decidua is the presence of decidual tissue outside the uterus. Ectopic decidua of the appendix is a rare entity that can present with abdominal symptoms mimicking appendicitis. We report a case of a 39-year-old female patient at 27 weeks gestational age with a 2-day history of right lower quadrant abdominal pain.The patient was referred to our hospital with suspicion of either acute appendicitis or threatened rupture of the uterus, the latter of which was considered unlikely following close examination. Therefore, she underwent emergency appendectomy via laparotomy. Microscopic examination revealed decidual tissue with myxoid degeneration in the subserosal layer of the tip side of the appendix, without endometriosis, which was compatible with ectopic decidua (deciduosis).Because it is extremely difficult to distinguish ectopic decidua of the appendix from acute appendicitis, even with various imaging modalities, we should be aware that ectopic decidua of the appendix is a differential diagnosis for acute appendicitis in pregnant women. Normal decidua consists of endometrial stromal cells transformed during pregnancy in response to ovarian and placental hormones, specifically progesterone. Ectopic decidua is the presence of decidual tissue outside the uterus; i.e., cervix, ovary, and fallopian tube, peritoneal surface, appendix, bladder, small intestine, large intestine, mesentery, and lymph nodes, that is usually related to pregnancy . EctopicThe prevalence of acute appendicitis during pregnancy is 0.05–0.13% . Acute aIt is extremely difficult to distinguish between ectopic decidua of the appendix and acute appendicitis, clinically. We herein report a case of ectopic decidua of the appendix in a 39-year-old female patient at 27 weeks gestational age with symptoms of suspected acute appendicitis who underwent appendectomy, which permitted a pathological diagnosis.A 39-year-old female patient at 27 weeks gestational age was referred to our hospital with a 2-day history of right lower quadrant abdominal pain. She had no remarkable medical history. Physical examination revealed that the patient’s vital signs, including body temperature, were within normal limits, and slight tenderness was detected in the right lower abdomen, with a palpable uterus measuring > 20 cm in diameter. Laboratory tests showed low hemoglobin and albumin concentrations , elevated C-reactive protein (CRP) concentration 41.3 mg/L), and slightly elevated white blood cell count (8600/µL). Ultrasonography (US) revealed a fetus that was normally developed for the gestational age and no signs of threatening uterine rupture. Abdominal computed tomography (CT) axial scans showed increased attenuation in fat in the area close to the cecum  and white blood cell count . Thus, she underwent urgent cesarean section, and the baby was delivered safely. The cause of the elevated CRP level before appendectomy in our patient could not be clearly determined, although latent intrauterine infection was one of the possible causes.Ectopic decidua is commonly localized on serosal surfaces of the pelvic organs and is incidentally detected in surgical specimens or a discrete nodule or mass found during cesarean section. In a study of 307 consecutive cesarean sections, macroscopic decidua was found in 31 (10.1%) cases . In addiEctopic decidua is benign, typically asymptomatic, and basically not a target of treatment because the condition usually regresses 4–6 weeks postpartum. However, ectopic decidua sometimes causes pain and intraperitoneal hemorrhage , which iConservative treatment of appendicitis with antibiotics in pregnant woman has recently gained attention as an alternative treatment option ; howeverEctopic decidua of the appendix is rare and difficult to distinguish from acute appendicitis. We should know that ectopic decidua of the appendix is a differential diagnosis for acute appendicitis in pregnant women, although surgical resection remains the first choice.


Ewing’s sarcoma is a common malignant bone tumor in children and young adults. Rarely, extra-skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy.We report an unusual case of primary pulmonary Ewing’s sarcoma in a 15-year-old girl who initially presented with massive hemothorax. By histopathology evaluation of surgical biopsy specimens, the diagnosis of extraosseous Ewing’s sarcoma was confirmed by both light microscopy and immunohistochemistry. Emergency, open surgery was performed by thoracic surgery at an early stage. After 3 cycles of chemotherapy, the patient was found to be stable at follow-up examination. No more hydrothorax or other symptoms.We have described an extremely rare case of primary pulmonary Ewing’s sarcoma with massive hemothorax. The patient underwent surgical resection and postoperative chemotherapy, no sign of recurrence to date as an outcome. Ewing’s sarcomas are relatively rare neuroectodermal tumors that primarily arise from the bone. Extraosseous Ewing’s sarcomas are a subset of primitive neuroectodermal tumors, which involve the soft tissues and organs such as the ovary, paravertebral soft tissue, testis, uterus, pancreas, renicapsule, and myocardium . We haveA 15-year-old girl presented with gradually increasing chest pain, cough, and dyspnea for 3 days, which then worsened in 2 hours, and she was eventually treated in the emergency department. There was no significant medical history, trauma included. On physical examination, she had low-grade fever (37.6 °C), hypotension (90/58 mmHg), tachycardia (122 beats/min), pain, weakness, and pale lips. Auscultation over the chest showed reduced air entry into the right hemithorax. Hematological investigations revealed mild anemia with a hemoglobin level of 9.2 g/dl. The other biochemical investigations were within the normal range. Computed tomography (CT) was performed using a Philips Brilliance multidetector scanner for evaluation of the lung. The images demonstrated a massive right pleural effusion with the collapse of the right lung, and punctate calcification was evident in the images . However, cytokeratin (AE1/AE3) staining was negative Fig. . The hemThe present study reports a rare case of hemothorax caused by a primary pulmonary Ewing’s sarcoma. Clinical symptoms of primary pulmonary Ewing’s sarcoma depend on the site of presentation but invariably include pain and swelling of the surrounding structures due to the mass effect. Other reported symptoms and signs such as hemoptysis are site-specific. The unusual presence of spontaneous hemothorax in association with a primary pulmonary Ewing’s sarcoma, which has not been reported in the literature, prompted us to publish this case. Non-traumatic hemothorax is a rare clinical feature, which is a potentially life-threatening emergency, which necessitates urgent surgery. It generally occurs due to the erosion of a vessel caused by a pathological lesion, such as in vascular disorders, connective tissue disease, neoplasms, extramedullary hematopoiesis, endometriosis, or pulmonary sequestration .Imaging characteristics of pulmonary Ewing’s sarcoma have been described in the literature, but the characterization has been limited. Typical pulmonary Ewing’s sarcoma images have a circumscribed solitary mass showing heterogeneous enhancement with non-enhancing necrotic areas within. Intralesional amorphous calcification, associated pleural effusion, and superior vein cava syndrome have also been reported . The chaThe diagnosis of extraosseous Ewing’s sarcoma is essentially histological and relies on the pathologic features of the specimen. The classical histopathological findings are that of round cells, with irregularly shaped chromatic nuclei surrounded by scanty cytoplasm; mitotic figures may be seen. The cells often show immunohistochemical positivity for various neurofilaments such as CD99, which is a sensitive diagnostic marker. Other markers include vimentin, CD56, Thyroid transcription factor-1(TTF1), cytokeratin 5/6, and caveolin 1, which facilitate differentiation from other small round cell tumors .EWSR1 gene translocation or amplifcation is the most reliable marker of PNETs, including those of pulmonary origin. 85% of patients had the identification t  chromosome rearrangement by fluorescent in situ hybridization and/or reverse transcription-polymerase chain reaction is used to support the diagnosis, and the remaining 15% of the patients had variants of this translocation, including 22q12, 21q12 (10% of cases) and 7p22, 17q12, 2q36 (< 1% of cases) [In addition, electron microscopic features include a specific high nucleus-to-cytoplasm ratio with aggregated glycogen granules in the cytoplasm. Detection of The treatment of Ewing’s sarcoma is aggressive, with the most effective treatment being surgical resection with combination chemotherapy and/or high-dose radiation therapy. Prognosis is mainly related to the ability to achieve disease-free surgical margins and the extent of anatomical spread to surrounding structures such as bone, pleura, and the epidural space . HistoriIn summary, we have described an extremely rare case of primary pulmonary Ewing’s sarcoma that presented with clinical features of massive hemothorax. Although rare, it should be considered in the differential diagnosis of large tumors of the lungs. The patient underwent surgical resection and postoperative chemotherapy. She has exhibited no sign of recurrence to date.


Duplication of the portal vein is a rare type of anatomic variant of the portal vein (PV) system that can be incidentally found and can lead to various challenges and consequences. Herein, we report an unusual case to increase our understanding of such anatomic variants.A 67-year-old asymptomatic woman was diagnosed with a liver space-occupying lesion by ultrasonography on a routine physical examination. The laboratory examinations from a local hospital suggested that her liver function tests were normal. The liver appeared normal on pre-contrast enhanced CT images. However, there were multiple complex abnormalities of PV found on contrast-enhanced CT scans, including two independent sources of PV (duplication), preduodenal PV, circum-portal pancreas, mimic cavernous transformation, abnormal branches of PV, and transient abnormal perfusion in the left lobe of the liver. MRI showed fatty infiltration in the left lobe of the liver.This case extends our current understanding of the anatomical variations of the PV system. Knowledge of these complex and rare anatomical variations will help clinical doctors make a confident diagnosis or assist with proper planning of a surgical procedure. The development and formation of the portal venous system is a complex process. Anatomic variants and congenital hypoplasia of the portal system can be found in 20–35% of individuals. Duplication of the portal vein is a rare type of anatomic variant of the portal vein (PV) system , 9. WithA 67-year-old asymptomatic woman was hospitalized, because she was suspected of having a liver space-occupying lesion diagnosed by ultrasonography on a routine physical examination. She was referred to our hospital for further assessment of the liver mass by computed tomography (CT) and magnetic resonance imaging (MRI). Laboratory examinations from a local hospital suggested that her liver function tests were normal, and all of her viral hepatitis surface antigens were negative. The alpha foetal protein (AFP) level was 1.5 ng/ml . She denied any chronic diseases, drug administration, alcohol consumption or venereal exposure.Pre-contrast enhanced CT scans of the abdomen showed that the shape, size and density of the liver were normal. On the arterial phase, contrast-enhanced CT images revealed transient perfusion abnormalities in the left lobe of the liver and homogeneous enhancement in both the portal vein phase and delayed phase Fig. a. HoweveDuplication of the portal vein is a rare type of variant of the PV that is only described by case reports. It is defined as two separated portal veins that course upward to the porta hepatis and divide into segmental branches , 3. DuplIn the present case, the PV system was formed by two separated main veins. PV-1 was formed by the normal development and junction of the SMV and the SV, while PV-2 was independently derived from the SV. Such duplication of PV was consistent with previous reports . HoweverPV-2 was independently derived from the SV extending a slender/tortuous vessel mimicking cavernous transformation directly into the left portal vein. However, this patient had no symptoms of portal hypertension. We considered that these events occurred due to further regression of the right umbilical vein, whereas the left umbilical vein continued to connect with both the ductus venosus and the left portal vein at a junction called the umbilicoportal confluence during the embryonic period. It has been proposed that aberrations in this process of involution can result in anatomical variations within the PV , 10. FurAs stated before, such PV malformations may directly cause some diseases of the liver, gastrointestinal or bile pancreatic system. They may also give rise to secondary portal hypertension with the development of oesophagogastric varices and may lead to a source of fatal haemorrhage. Although this patient presented with no symptoms, further follow-up examinations should be performed due to the multiple and complex malformations of the PV system, especially due to the unusual topography  and abnormal haemodynamics (mimic cavernous transformation of PV-2). For doctors, this case presented a rare and complex anatomical variation of the PV system, which could further the understanding of the variations of the PV system. Knowledge of these variations helps in the proper planning of patient management, especially for interventional surgery and laparoscopic surgery.


The most common presenting symptoms of prostate cancer, a common cancer in males worldwide, are lower urinary tract symptoms. In rare cases, however, urinary symptoms may not be apparent, and patients can present with gastrointestinal symptoms instead. Even rarer is the involvement of non-regional lymph nodes such as the cervical nodes. Here, we report a case of a 50-year-old male who initially presented with constipation and an enlarging left lateral neck mass. Further work-up revealed metastatic prostatic adenocarcinoma and the patient dramatically responded to chemotherapy, androgen deprivation therapy and bone support therapy. This case highlights the importance of considering a prostate malignancy in a male patient presenting with gastrointestinal symptoms and a neck mass even in the absence of lower urinary tract symptoms. Serum prostate specific antigen, pathologic findings and immunohistochemistry staining are important to guide the clinician in making the correct diagnosis and treatment. Prostate cancer was the second most common cancer in men worldwide in 2020 . An estiBecause of the anatomic location of the prostate gland, the most common presenting symptoms of prostate cancer are symptoms related to the urinary tract such as difficulty in urination, haematuria and pelvic pain . Rarely,More unusual is the presentation of prostate cancer as a left lateral neck mass as illustrated in this case. The most frequent sites of metastasis of prostate cancer are the axial skeleton due to the rich Batson venous plexus and the pelvic and retroperitoneal lymph nodes –10. InvoA 50-year-old male farmer, past heavy smoker (10 pack-years), with no known co-morbidities, presented with a 1-year history of gradually enlarging nontender left neck mass, initially approximately 2 × 2 centimetres in size. He also complained of constipation but no abdominal pain, decreased stool calibre, melena or haematochezia. There were no reported lower urinary tract symptoms such as dysuria, urinary frequency, nocturia, haematuria, straining, hesitancy, urgency, intermittent stream, dribbling or feeling of incomplete bladder emptying. There were also no symptoms pertaining to a thyroid disease. There was no family history of any malignancy.The left lateral neck mass was progressively enlarging in size which prompted consult at a local hospital where a neck ultrasound was done showing multiple, coalescing complex foci in the left lateral neck area, the largest of which measures 2.8 cm with perilesional uptake on Doppler study. The thyroid gland was normal in size with homogenous parenchymal echogenicity. Thyroid function tests were also normal. A fine needle aspiration biopsy of the neck mass showed atypical epithelial cells.No masses, nodules, haemorrhoids or tenderness were palpated on initial DRE. There was good sphincter tone. No blood was seen per examining finger.Weight loss, occasional hypogastric pain, persistent constipation and continuous enlargement of the neck mass to approximately 7 × 7.5 cm  promptedBiopsy of the rectal mass showed medium to large sized atypical cells with high nuclear to cytoplasmic ratio, arranged in poorly formed glands and as individual cells with enlarged, pleomorphic, hyperchromatic nuclei with prominent nucleoli . A histo2 every 3 weeks with prednisone 5 mg twice daily, androgen deprivation therapy with goserelin 10.8 mg subcutaneously every 3 months and bicalutamide 50 mg once daily, and bone support therapy with denosumab 120 mg subcutaneously every month.The patient was then treated as a case of metastatic prostate adenocarcinoma. He received six cycles of docetaxel at 75 mg/mAfter 1 month of treatment, there was drastic regression in the size of the neck mass to approximately 4 × 4 cm as shown in The incidence of advanced prostate cancer invading the rectum was estimated to be 1%–11% . DespiteThe use of endoscopic ultrasonography such as in transrectal ultrasound or endoscopic ultrasound may be recommended to aid in the overall diagnosis of rectal masses. The advantages have been seen in its use in early primary rectal cancer where endoscopic ultrasound has been used in staging and fine-needle aspiration biopsy technique . In prosPreoperative pelvic magnetic resonance imaging is recommended for select patients with rectal masses to define the extent of possible surgery, as it is the most accurate test to define locoregional clinical staging . HoweverThe morphologic features of prostate and colorectal adenocarcinoma are also similar. Both malignancies may present as well differentiated tumours showing prominent gland formation with cells characteristic of their primary site of origin to poorly differentiated tumours with poorly formed glands and interspersed individual malignant cells. Both tumours may present with atypical cells showing cellular and nuclear pleomorphism and prominent nucleoli with only ‘soft signs’ pointing to either a colorectal  or a prostatic (microacinar architecture) primary –20. ThesThe clinical scenario presented in this case can lead to diagnostic pitfalls. Even though prostate cancer is a common malignancy in males, it is often overlooked as the primary site in patients presenting with cervical lymphadenopathy . A prostEven though prostate cancer is a common malignancy in males, it is often overlooked as the primary site in patients presenting with cervical lymphadenopathy. It is important to consider a prostate malignancy in a male patient presenting with gastrointestinal symptoms and a neck mass even in the absence of lower urinary tract symptoms. Serum PSA, pathologic findings and immunohistochemistry staining are important to guide the clinician in making the correct diagnosis and treatment.‘We are greatly thankful to all my doctors. For more than a year, my neck mass was continuously enlarging in size. For a long time, I was being worked-up to search for the real site of my cancer. When my doctors were able to pinpoint where it really was and I was started on treatment, I noticed a drastic improvement in my condition. Aside from the mass decreasing in size to almost nothing, my appetite also improved, and I gained weight. Even though my medications are expensive for a farmer from the province like me, we are doing our best to follow my doctors’ instructions. Even if I have stage 4 cancer, there is still hope’ (Translated from Filipino).The authors declare that they have no conflicts of interest.No funding was received by the authors with the preparation of this manuscript.


Sclerotic lesions of the jaw are uncommon but may be clinically relevant. In this pictorial review, the most common sclerotic lesions are discussed. Three categories of lesions are distinguished; odontogenic sclerotic lesions, non-odontogenic sclerotic lesions, and mixed lytic-sclerotic lesions. In each group, non-neoplastic conditions are discussed first, followed by benign and malignant neoplasms. For each disease a brief overview is given, including histological features, epidemiology, symptoms, typical location, imaging features, and treatment. This review emphasizes which basic observations are essential to the evaluation of sclerotic jaw lesions and what elements have to be taken into account to create a proper differential diagnosis. Sclerotic jaw lesions are not rare and are frequently encountered on radiographs and computed tomography (CT). However, these lesions are often underreported, mainly because the subject is not well known to general radiologists who struggle with the imaging approach and disease entities.In this paper, an overview is given of the most important types of lesions. Emphasis is on the distinction between odontogenic and non-odontogenic lesions. When a sclerotic lesion is observed in the jaw, attention should first be focused on whether and how the lesion is related to a tooth 2345. If 234The following conditions are discussed in this review: (1) odontogenic sclerotic lesions , (2) non-odontogenic sclerotic lesions , and (3) mixed lytic-sclerotic lesions .Cemento-osseous dysplasia (COD) is the most common fibro-osseous lesion found in the jaw. It represents a group of benign lesions of unknown etiology characterized by the substitution of normal bone by fibrous tissue with newly formed mineralized structures 7. Three The lesions typically do not cause any symptoms. In the early stages of the disease, a vascular fibrous stroma with osteoid and some basophilic cementoid structures can be observed. In the later stages, the stroma becomes more fibrotic. A more distinct osteoid trabeculae formation is observed with the presence of thicker curvilinear bony trabeculae, and a possible occurrence of prominent cementoid masses.Figure 1). Two additional characteristics that should be noted are: (1) the lesions are typically associated with a vital-nonrestored tooth, and (2) unlike cementoblastomas, cemento-osseous dysplasia lesions do not fuse directly to the tooth root . Focal COD is typically located in the posterior quadrant of the jaw. Periapical COD is seen adjacent to a tooth-bearing area associated with one or more vital mandibular anterior teeth . In florid COD, multiple lesions are observed that involve the mandible and/or maxilla bilaterally . The differential diagnosis includes fibrous dysplasia, Gardner’s syndrome, and chronic osteomyelitis (see below). Asymptomatic COD needs no surgical treatment.Radiographically, all three subtypes appear initially lytic and show progression to a heterogenous sclerotic lesion, often with a radiolucent rim. The radiolucent rim may in turn be surrounded by a thin rim of sclerosis, giving the lesion a quite specific appearance , often associated with a carious tooth or with antecedents of root canal therapy, periodontal disease, or tooth extraction [Condensing osteitis  represents an asymptomatic change in osseous structure presumed to be the response to a long-standing and low-grade inflammatory stimulus from an inflamed or necrotic pulp. Histologically, dense layers of compact bone are replacing fibrofatty bone marrow and cancellous bone. On imaging, condensing osteitis is seen as a periapical, poorly marginated, nonexpansile, sclerotic lesion in the posterior mandible at the apices of the premolar or molar teeth . The pericoronal rather than periapical position allows differentiation of odontomas from cementoblastomas. Treatment of complex odontomas consists of surgical excision.Odontomas are the most common odontogenic “tumors” and actually represent a developmental-anomaly (hamartoma) 8. They a8Figure 4), which is an important differential diagnostic feature not seen in other periapical lesions such as cemento-osseous dysplasia and condensing osteitis. Additional radiographic features include root resorption, loss of the root outline, invasion of the root canal, bony expansion, displacement and involvement of adjacent teeth, cortical erosion, and obliteration of the periodontal-ligament space. Surgical excision and related tooth extraction are warranted.Cementoblastoma is a rare benign odontogenic tumor of mesenchymal-ectomesenchymal origin with a typical periapical location . This tu8Figure 5). The mandibular canal may be displaced inferiorly, whereas in fibrous dysplasia the canal may be displaced in any direction [Cemento-ossifying fibroma is a benign slow-growing fibro-osseous tumor. These tumors are most often seen in women between the third and fourth decades of life and are thought to arise from the periodontal ligament. The growth of the tumor over time may lead to facial asymmetry and the mass causing discomfort or mandibular expansion 12. Accor8irection 14. TheraFigure 6). A low-attenuation rim is not seen. No treatment is required.Idiopathic osteosclerosis is an asymptomatic, nonexpansile, localized radiopaque lesion observed in the alveolar process in posterior regions without any obvious etiological agent . Being aDense bone islands, or enostomas, are common incidental findings, consisting of failure of resorption of secondary spongiosa within the trabecular bone 16. UnlikTori are protuberances of dense cortical bone that most commonly arise in adults. Occasionally, they contain a small amount of marrow. They are characterized by slow growth that usually arrests spontaneously.Figure 7) [Torus mandibularis has typical imaging features and arises above the mylohyoid line, along the lingual surface of the mandible (igure 7) .A torus palatinus is a similar nodular bony overgrowth at the midline of the hard palate .A torus maxillaris arises at the lingual surface of the posterior maxilla .Figure 8) [Fibrous dysplasia is a benign developmental disorder characterized by a dysplastic process of altered osteogenesis with subsequent substitution of normal bone by fibrous tissue that undergoes abnormal mineralization. It typically occurs in the first to third decades of life and is clinically characterized as an asymptomatic bone swelling. The maxilla is involved more frequently than the mandible. Fibrous dysplasia is typically unilateral and may occur within a single bone (monostotic form) or, more often in multiple bones (polyostotic form). Typically, the lesion is radiopaque with ground glass appearance (igure 8) 18. Its cOsteoma is a benign neoplastic lesion, characterized by persistent slow growth. It is composed of mature bone structures with characteristics of cancellous or compact bone.Osteomas most commonly arise in the craniofacial bones. The most common location in the jaw is the posterior mandibular body or condyle. Multiple osteomas may be associated with Gardner syndrome. Radiologically, osteomas present as a well-defined round radiopaque mass with no radiolucent halo . OsteomaOsteoid osteoma is very rare in the jaw. It is often painful, most pronounced at night, and mitigated by treatment with salicylates. It presents as a lytic lesion with a radiopaque central nidus surrounded by a sclerotic bony margin .Osteochondromas are rarely seen at the craniofacial bones. These tumors have a predilection for the coronoid and condylar processes of the mandible that may exhibit a focal mushroom-shaped enlargement. Larger lesions may cause a decreased mouth opening. Continuity of the cortex and the medullary bone of the lesion and the underlying host bone is a characteristic imaging finding .Figure 9).The jaws and mouth are uncommon sites for metastatic dissemination. Nevertheless, the incidence of metastatic tumors to the jaws is probably higher than suggested; micrometastatic foci in the jaws were found in 16% of autopsied carcinoma cases despite the absence of radiologic findings . All typOsteosarcoma of the jawbones account for up to 10% of all osteosarcomas. They tend to occur in an older age group usually between 30 and 39 years and have an overall better prognosis than conventional osteosarcoma. Early-stage osteosarcoma may be osteolytic. More advanced lesions are typically osteoblastic or more commonly mixed on radiographs and CBCT due to the presence of mineralized osteoid matrix. Typical features are poorly defined margins, cortex destruction, and aggressive periosteal reaction .Mandibular osteoradionecrosis (ORN) is a serious complication of radiation therapy for neoplasms of the head and neck area. It has a widely varying reported incidence of 5% to 22%. Bone necrosis may occur secondary to hypoxia, hypovascularity, hypocellularity, and fibrosis.Figure 10a, b) [The diagnosis of mandibular ORN is primarily based on clinical symptoms in the appropriate therapeutic setting. The mandible is involved more frequently than the maxilla, probably because of its less robust blood supply. The buccal cortex is more vulnerable than the lingual cortex, although the mandibular body is most commonly affected. At imaging, osteoradionecrosis appears as an area of marked sclerosis with a loss of trabeculation in spongiosa, and cortical interruptions  . Other oFigure 1c, d) [Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is associated with the use of oral or intravenous bisphosphonates to treat various bone conditions such as osteoporosis, multiple myeloma, metastasis, and Paget disease. Bisphosphonates inhibit endothelial proliferation and interrupt intraosseous circulation. Osteonecrosis may be spontaneous or may be precipitated by a traumatic procedure such as tooth extraction or dental surgery. BRONJ should be considered in patients undergoing bisphosphonate therapy with findings of bone necrosis and no history of radiation therapy. BRONJ is typically painful, but some patients may be asymptomatic. Imaging features are comparable to those seen in mandibular osteoradionecrosis  . Accordie 1c, d) , BRONJ tFigure 10e, f) [Osteomyelitis is much more common in the mandible than the maxilla, which is involved in only 1%–6% of cases because of its rich blood supply. Most patients with mandibular osteomyelitis have a history of antecedent dental caries or dental extractions. Other causes of osteomyelitis include dental or mandibular fractures, osteoradionecrosis and rarely, hematogenous spread of infection. Chronic osteomyelitis, which is characterized by a duration longer than one month, may be complicated by sinuses, fistulae, osseous sequestra, or pathologic fractures. Imaging findings of mandibular osteomyelitis include cortical interruption, sclerotic sequestra in low-attenuation zones, periosteal new bone formation, and areas of gas attenuation  may suggest a specific diagnosis. Cementoblastoma for instance, invariably has a periapical location, while complex odontoma has similar morphological features, and it is typically seen in a pericoronal position.Besides density and relationship with dentition, other patient and imaging related features may assist in formulating a differential diagnosis or specific diagnosis. These features include the patient’s age, sex, and race, location of the lesion in the jaw , and imaging features such as demarcation, cortical involvement, periosteal reaction, and soft tissue changes.In some cases, lesion characterization may be obtained by imaging alone, particularly in benign lesions.Even in cases where such characterization is not possible based on imaging features, the radiologist plays an important role and has to determine whether the lesion can be regarded as a “do not touch lesion” or be referred for biopsy.


A 68 year-old female presents with an ulcerated mass of the 5th digit, with rapid growth during the previous month to surgery. The mass was excised and covered with a 4th dorsal metacarpal artery perforator flap. The histologic analysis was compatible with the diagnosis of fibro-osseous pseudotumor of the digit. The hand surgeon acts both as an oncological surgeon, ablating all the tumor mass, potentially compromising aesthetics and function, and as a reconstructive surgeon, trying to optimize hand function. Balancing these goals may be a demanding task .Even though the majority of hand tumors are benign, especially if they do not involve the skin, some malignant tumors arise in the hand. In the latter, a more aggressive approach would be justified .Fibro-osseous Pseudotumor of the Digits (FOPD) is a rare benign tumor with clinical characteristics that can mimic a malignant tumor. We present a case of FOPD and a review of the literature.A 68 year-old female, smoker, presented to the ER with an ulcerated mass of the dorsum of the 5th finger involving the proximal phalanx to the DIP joint . It had Excision with the overlying skin was performed down to a tumor less surgical plane, preserving the extensor apparatus . The resFOPD is a rare entity. There are 173 cases documented in the literature , totalinRadiologically, FOPD usually presents as a soft tissue mass with ill-defined margins and might reveal extraosseous ossification and bone formation. The periosteal reaction has been described . CorticaHistologically, FOPD presents as a multi-nodular lesion with irregular margins and it is localized in the dermis and subcutaneous tissues. Muscular and cartilage involvement is absent.Typically, FOPD shows fibroblastic/myofibroblastic proliferation without atypia, in a myxoid stroma, and immature trabeculae with osteoid rimmed by osteoblasts, without zoning phenomenon ,23,51. OImmunohistochemically, the majority of studies has demonstrated positive staining for vimentin 14 ,25,26,34The majority of cases demonstrated negative staining to S – 100, CD 34, cytokeratin (MAK-6 and CAM 5.2), desmin, and epithelial membrane antigen (EMA) ,18,25–27FOPD might be included in a group of USP-6-rearranged myofibroblastic neoplasms that share a genetic rearrangement of the USP-6 gene and are known for their rapid yet self-limited growth and low recurrence rate ,55. ReceThe treatment for FOPD is surgical excision. Prognosis is excellent as recurrence has been related to incomplete excision ,11,42. WNine cases of recurrence were described in the literature ,23,31,43After complete removal of the tumor, no recurrences were found with a median follow up of 15 4–168) months –168 mont,48,53. EFOPD should be considered in the differential diagnosis of fast-growing lesions of the extremities. An excisional biopsy allows for complete tumor removal and avoids overzealous treatment. FOPD should be treated without radical excisions provided if totally removed.


Desmoid tumour of the colon is a very rare and aggressive type of intra-abdominal desmoid fibromatosis. Patients can present with a range of symptoms from a mild chronic abdominal pain to those of an acute abdomen. We present a rare case of abdominal fibromatosis that presented as a rapidly growing mass with free intraperitoneal gas. Intraoperatively however, we found a large tumour arising from the wall of the transverse colon and local necrosis. No bowel perforation was noted. The tumour was removed with a wide resection of transverse colon’s wall instead of colectomy. The histopathology reported benign fibromatosis and excluded malignancy. Desmoid fibromatosis (DF) is a rare condition that can involve many organs of the human body including intrabdominal ones. Histologically, fibromatosis is an abnormal proliferation of myofibroblasts . Intra-aA 51-year-old lady presented to the emergency department (ED) with generalized abdominal pain. The patient did not report any associated symptoms. She had had similar episodes in the recent past, but the symptoms were minor and resolved on their own. Her past medical history included a recent admission to ED with renal colic and a computed tomography scan (CT) of the abdomen which was clear . She didBlood tests showed a C-reactive protein of 24 mg/l and white blood cells of 12 000/ml. The rest of the values were unremarkable, including lactates. She was tachycardic, but she was haemodynamically stable. Chest X-ray showed no free air under diaphragm. A CT abdomen/pelvis showed free intraperitoneal gas indicating a hollow viscus perforation. A 9.2 × 6.1 cm lesion in the mesentery, which at that time was of uncertain aetiology, was also found. The report mentioned that although the mass was inseparable from the transverse colon superiorly, it did not appear to arise from this and maintained a clear plane to the small bowel loops. The mass was of heterogeneous attenuation measuring up to 50 Hounsfield units. Further review of the scans with the consultant radiologist suggested the presence of a possible gastrointestinal stromal tumour or a desmoid .Subsequent to the imaging and clinical findings, a decision for surgical exploration was made. A midline laparotomy was performed and a large tumour was discovered. Its wall was well defined, and it was arising from the wall of the transverse colon. The omentum was covering a part of the tumour that looked necrotic . As the The histopathology confirmed benign fibromatosis. Macroscopically, the specimen was described as a 90 × 85 × 60 mm round mass of white tissue with attached omental fat 310 × 170 × 25 mm with no evidence of perforation. Microscopically, a spindle cell lesion composed of fascicles in places with a perpendicular pattern was revealed. The tumour cells showed diffuse nuclear staining for beta catenin and they were negative for S100, CD34, CD117 and desmin. Mitoses were seen scattered and typical in morphology excluding colonic malignancy. It was a margin-negative (R0) resection.The patient had an uneventful post-operative period. The case was discussed in our multidisciplinary team meeting (MDT), and it was decided to continue follow-up with interval scans without chemoradiotherapy. The CT scans 6 and 12 months post-operatively showed no recurrence or signs of metastatic disease or lymphadenopathy.The word desmoid comes from the Greek word desmos, which means tendon-like . These aWe present the first case report in literature describing a DT arising from the transverse colon of a patient who presented with acute abdomen and underwent an emergency laparotomy and wide resection without extended colectomy. There are three case reports in literature describing a colonic DT, one was a finding in an elective and two in an emergency setting, but in all of those cases, patients finally underwent a hemicolectomy , 5, 6.DF remains an enigmatic disease with a variable course that can range from an incidental small tumour that can remain small and stable or become large and grow rapidly, causing death in a matter of months or years . In our In our case, a colonic DT mimicking a hollow viscus perforation can be anticipated as diagnosis. Even though free intraperitoneal gas had been seen in the CT, no bowel perforation was noted intraoperatively. As the tumour had a necrotic area, covered with omentum, it seems that the gas was originating from the tumour necrosis. Finally, no perforation was described macroscopically in the histopathology report.The surgical management of gastrointestinal fibromatosis is governed by the location, size and local invasion of the tumour. R0 resection has historically been the gold standard for the treatment of abdominal wall and intra-abdominal DTs; however, microscopically positive margin resection is acceptable, as recurrence rates may not be significantly affected . In our DT of the colon is a very rare and aggressive type of intra-abdominal DF. Patients with colonic DT can present with a range of symptoms from a mild chronic abdominal pain to those of an acute abdomen. CT is an essential tool for the final diagnosis. The surgeon should review the scans together with the consultant radiologist in order to form the best plan preoperatively and intraoperatively. Complete resection of the tumour with negative margins macroscopically prevents local recurrence. The decision of an extended resection of the involved bowel or just local excision should be made intraoperatively depending on the tumour’s location, local invasion and surgeon’s experience. An MDT discussion, as well as follow-up, is mandatory.None declared.None to declare.All procedures performed in studies involving human participants were done in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.Informed consent was obtained from all individual participants included in the study. Full verbal and written informed consent has been obtained from the patient for submission of this manuscript for publication.Mr Ioannis Gerogiannis is acting as a guarantor for this manuscript.