Nasal glial heterotopia is a rare type of neoplasm consisting of meningothelial and/or neuroglial elements.A 2-month-old male was evaluated for treatment of a congenital mass in the right nasal cavity near the pharynx.The patient was preoperatively diagnosed with a congenital intranasal neoplasm.Nasal endoscopic resection of the nasopharyngeal mass was performed under general anesthesia. Histological examination of the resected tissue provided a diagnosis of intranasal glial heterotopia.The surgical outcome was good, with no surgical site infection. After 1 year of follow-up, the boy was asymptomatic with no recurrence.Excision of a nasopharyngeal mass via nasal endoscopy resulted in no recurrence during 1 year of follow-up. Before any surgical treatment for suspected glial heterotopia, the mass should be differentiated clinically and radiologically from an encephalocele to prevent the risk of cerebrospinal fluid leakage and meningitis. The incidence of congenital nasal masses is reportedly 1 in 20,000 to 40,000 live births,–7 and NGH accounts for approximately 5% of all congenital nasal masses. Congenital nasal masses are thought to develop due to faulty closure of the anterior neuropore. Most of these lesions arise from the lateral nasal wall and cause nasal obstruction. Herein, we report a case of a 2-month-old male with NGH and review similar cases reported in the literature.Glial heterotopia is a rare congenital developmental disorder in which neuroglial tissue forms in extracranial sites, usually in the midline. As glial heterotopia commonly occurs in or around the nose, it is often referred to as nasal glial heterotopia (NGH).2A 2-month-old male presented with nasal obstruction and shortness of breath since birth; he had not had a fever, had normal mental responses, and was passing normal stools and urine. On the 22nd day after birth, the nasal obstruction worsened, and the patient had difficulty breathing, a bluish face, and a decreased heart rate. The patient was given cardiopulmonary resuscitation and started on ventilation via tracheal intubation. On the 35th day after birth, fiberoptic bronchoscopy showed a 1.5 × 1.5 cm polypoid substance in the right nasal cavity near the pharynx Fig. . The sofA nasal endoscope was used to excise the nasopharyngeal mass under general anesthesia. Intraoperatively, it was found that the lobulated mass had a broad base that originated from the lateral wall of the left nasopharynx, with an upper boundary that reached the posterior nostril and a lower boundary that reached the upper polar plane of the tonsil; the mass was the same color as the mucosa. A multipoint puncture without liquid was performed, the tumor was resected with a low-temperature plasma knife, and the wounds were sutured. Pathological examination revealed that the mass was a glial heterotopia (left nasopharynx) Fig. . The imm3 NGH is mainly located in or near the nasal cavity; 60% of cases are located outside the nose, 30% are located inside the nose, and 10% are located outside and inside the nose,,10 and only 15% to 20% show intracranial communication.,11 In our case, the mass was intranasal. Although glial heterotopias are benign lesions, they can cause clinical problems depending on their location. Intranasal lesions may be accompanied by nasal obstruction, epistaxis or nasal deformity. Intranasal glial heterotopias can cause nasal congestion and dyspnea, as seen in our case.A congenital midline nasal mass is a rare developmental abnormality. NGH can be thought of as an encephalocele that has lost its intracranial connections. Most reported cases of NGH are unilateral nasal or extranasal orbital, nasopharyngeal, middle ear, or scalp swellings. but there is no fluid to fill the space connecting the lesion to the subarachnoid space. In the current case, the lesion was identified at birth, and there was no fibrous stalk connecting the swelling with the intracranial space. Glial tissue can be confirmed by assessing the immune response to glial fibrillary acidic protein or S100 protein. Neurons are rare or absent in NGH, consistent with our observations in the present case. Although ependymal tissue is not always recognized in encephaloceles, its presence is more likely to lead to a diagnosis of encephalocele. On histological examination, it is usually impossible to distinguish NGH from encephalocele, as the two lesions can contain different proportions of neurons and glia. NGH must be distinguished from encephalocele, as the two lesions have similar embryological origins and can both manifest as intranasal masses. When the encephalocele is accompanied by meninges, it is termed a meningoencephalocele.In 15% of patients with NGH, nerve growth hormone connects the lesion to the dura mater via a fibrous stalk, In frontoethmoidal encephalocele, brain tissue herniates through a defect of the frontal or ethmoidal bone into the soft tissues of the forehead, nose, and orbit, and is designated as nasofrontal, nasoethmoidal, or nasoorbital, respectively. The embryopathogenesis of nasal encephaloceles is controversial. Basal encephaloceles occur in the nasal cavity rather than as an external mass, and their developmental herniation is posterior to the cribriform plate. The incidence of related developmental abnormalities in patients with encephalocele varies from 0% to 40%. The most common site of encephalocele is occipital (75%), followed by the frontal lobe (25%). Nasal neuroglial heterotopia and encephalocele are very rare diseases that require multidisciplinary evaluation and management.Nasal encephaloceles are a subtype that may be frontoethmoidal or basal. Although it may be difficult to distinguish NGH from CH, this has no direct effect on treatment, as both lesions require surgical treatment.The clinical manifestations of NGH are similar to those of congenital hemangioma (CH), and it is difficult to diagnose CH and NGH using prenatal ultrasonography. The blood flow velocity in Doppler examination is fast for CH and slow for NGH. On magnetic resonance imaging (MRI), the two lesions show high signal intensity on T2-weighted sequence, but the intensity of NGH is lower than that of CH. Bone defects in patients with developmental abnormalities may be associated with NGH, but may not be associated with intracranial tissue. MRI is superior to CT in providing the details of soft tissue, and it is more valuable in identifying an intracranial connection. As our patient had dyspnea, tracheal intubation and ventilator-assisted breathing were instigated and MRI was not performed; thus, CT was used to exclude intracranial extension.CT and MRI are important in the diagnosis of NGH. CT is helpful in assessing bone defects, although CT may have different effects on young children whose bone structure is not yet fully formed. Thus, the present patient was not biopsied preoperatively.Preoperative biopsy and resection are prohibited without preoperative imaging to determine the extent and location of the mass and exclude any connection with the central nervous system; this is to prevent complications such as cerebrospinal fluid (CSF) leakage, meningitis or encephalocele. However, early surgical treatment is advocated because the growth of gliosis may lead to deformity and erosion of the facial bones. Furthermore, early surgical treatment may avoid serious complications such as meningitis, brain abscess, nasal septum and nasal bone deformation. The specific surgical method should be determined in accordance with the location and size of the mass. Transnasal endoscopic surgery is recommended for intranasal glial heterotopia. Due to advances in endoscopic equipment and technology, intranasal glial heterotopia can now be properly exposed and completely removed. For most intranasal and mixed NGH, endoscopic sinus surgery is feasible, with no increase in operation time, residual disease or complications. In addition, inadequate primary resection results in a recurrence rate of 4% to 10%. Our patient had no recurrence during 1 year of follow-up. Thorough preoperative imaging is necessary before glioma resection.The preferred treatment for NGH is complete surgical excision. NGH grows slowly and benignly, with no possibility of malignant transformation.4We report a rare case of NGH, which is a congenital developmental anomaly rarely reported in the literature. Before any form of surgical treatment for suspected glial heterotopia, the lesion should be differentiated clinically and radiologically from encephalocele to prevent the risk of CSF leakage and meningitis. Clinical examination supplemented by imaging investigations (such as MRI and CT) helps to achieve early diagnosis and timely surgical consultation. To prevent the risk of CSF leakage, the possibility of intracranial connections must be considered when planning surgery for congenital midline masses. Conservative surgical excision remains the accepted modality of treatment due to the rare recurrence and nonmalignant potential of NGH.We thank Kelly Zammit, BVSc, from Liwen Bianji, Edanz Editing China, for editing the English text of a draft of this manuscript.Investigation: Wei Zhang, Lixing Tang.Resources: Wentong Ge, Lixing Tang.Supervision: Xin Ni.Writing – original draft: Wei Zhang.Writing – review and editing: Pengpeng Wang.


Darier–Ferrand dermatofibrosarcoma (DFS) is a rare malignant cutaneous neoplasm characterized by local aggressiveness, high risk of recurrence, and low metastatic potential.A 60-year-old Tunisian man with recurrent abdominopelvic DFS for which he had undergone multiple excisions presented with an extensive DFS that invaded the external genitals. He underwent a large excision with emasculation and thin cutaneous graft of the abdominal wall and local skin flap in the pelvis.DFS is a rare yet recurrent skin tumor. Wide excision with free margins remains the cornerstone of treatment. We report a case of a giant DFS treated with wide excision and reconstructive surgery to cover the defect. Darier–Ferrand dermatofibrosarcoma (DFS) is a rare skin tumor, representing only 0.1% of malignant cutaneous neoplasms. DFS is usually characterized by high recurrence, slow growth, and low metastatic potential . CompletWe report herein a case of a giant and highly advanced DFS and shed additional light on a rare but dreadful disease.We present the case of a 60-year-old Tunisian man with a medical history of recurrent abdominopelvic DFS for 35 years, for which he had undergone ten surgical excisions in another clinic. Not all the histologic reports were available, but for those that were found, the margins were free of tumor. He presented with a giant DFS of the abdominal wall and pelvis. The biopsy confirmed the diagnosis and the patient was referred to our outpatient clinic.The patient had a poor general state; he was afebrile and hemodynamically stable. On physical examination, multiple tumors were observed extending from the abdominal wall to the pelvis and external genitals a distance of 1 cm from the anal margin Figs. , 3.Fig. A computed tomography scan of the abdomen and pelvis revealed multiple subcutaneous and infiltrative tumor masses of the anterior abdominal wall and pelvis that invaded the right scrotum. No metastases were found. After discussion in a multidisciplinary meeting, we opted for wide local excision combined with emasculation and reconstruction with a local skin flap for the pelvis and thin cutaneous graft for the abdominal wall Figs. , 5, 6.FiThe surgery lasted 240 minutes. No preoperative complications or blood loss occurred. The excised specimen measured approximately 33 cm × 17 cm × 7 cm in the greatest dimensions. The postoperative course was uneventful. The patient was discharged on day 7. The final histology report showed clear margins.The case was discussed again in a multidisciplinary meeting and the decision was made for close follow-up every 3 months for the first 2 years. The patient progressed well during 7 months of follow-up. No urinary problems were reported. The only concern was an episode of depression, which was followed up by a psychiatrist.DFS is a rare mesenchymal tumor, representing 1.8% of all soft tissue sarcomas and only 0.1% of all cancers. It was first described by Taylor in 1890  and thenDFS can appear at any age, but most commonly in patients between the ages of 20 and 50 years . AlthougDFS usually appears as a pink, reddish-brown, or violaceous plaque limited to the skin. With time, the tumor can evolve into multiple "protuberant" nodules that may infiltrate the subcutaneous tissue, fascia, muscles, and even bone . In our DFS is characterized by diffuse infiltration of the dermis with a low rate of mitosis and minimal atypia. Aggressive clinical behavior is correlated with the mitotic count, necrosis, and areas of fibrosarcomatous change. Tumor cells demonstrate strong positive staining for vimentin, CD34, apolipoprotein D, and nestin when subjected to immunohistochemical studies. Negative staining is found for desmin, S100 protein, factor XIIIa (FXIIIa), stromelysin-3, HMGA1 and HMGA2, tenascin, podoplanin (D2-40), CD163, and keratins [The treatment of choice is wide local excision, with negative margins of 3–5 cm from the tumor edge including the skin, the subcutaneous tissue, and the underlying fascia .Mohs micrographic surgery is an alternative to wide excision that is considered by some as the preferred treatment for DFS. It consists of a removal method that offers precise microscopic control of the entire tumor margin while maximizing the conservation of healthy tissue , 13. It Our patient underwent wide excision of the tumor that measured 33 cm, and we even performed an emasculation procedure due to invasion of the external genitals. This is the largest such tumor described in the literature –16. SincIn cases in which primary flap reconstruction is performed simultaneously with tumor excision, there should be no doubt as to the adequacy of the excision, since the presence of a flap may prevent the detection of local recurrence. In general, the reconstructive challenge in DFS such as in the trunk, extremities, head, and neck is related to large tissue defects which need covering when vital structures are exposed , 18. In As for adjuvant treatment, imatinib mesylate can be used to treat unresectable, recurrent, and/or metastatic disease in patients with a t translocation . In caseDFS is a malignant fibroblastic tumor with a high potential for recurrence. The pelvic location is not common in the literature, and wide excision in this area requires reconstructive surgery to cover the defects.Our case was extraordinary due to several aspects: we report the largest size of DFS in the pelvic area (33 cm × 17 cm × 7 cm), which itself represents a rare location for this tumor. Our patient underwent wide surgical excision for this tumor. Two types of reconstruction were used to cover the enormous defect.


Gastric volvulus (GV) is a seldomly encountered life-threatening condition that necessitates rapid diagnosis in the emergency department (ED). The diagnosis of GV is traditionally established with cross-sectional imaging and/or endoscopy with surgical confirmation. The potential role of point-of-care ultrasound (POCUS) as a bedside tool to support the early identification of GV by emergency physicians (EPs) has not been thoroughly investigated. This case report describes the expeditious diagnosis and ED management of acute GV by implementing EP-performed POCUS into critical decision making. Gastric volvulus (GV) is a rare surgical emergency with mortality rates approaching 30%-50% when complications occur -4. The pTimely recognition and diagnosis of GV is imperative to avoid detrimental delays in the emergency department (ED) intervention and definitive management. Plain radiography and CT are the initial diagnostic modalities typically employed when GV is suspected; however, the utilization of ultrasonography to support the diagnosis has not been systematically studied -11. The A 69-year-old male with a past medical history of hypertension, atrial fibrillation, antineutrophil cytoplasmic antibody (ANCA) vasculitis, chronic kidney disease, gastroesophageal reflux disease, and hiatal hernia presented to the ED with chief complaint of abdominal pain. He described a one-day history of gradual-onset, constant, dull, moderate severity, non-radiating epigastric discomfort with associated abdominal distention, nausea, and decreased appetite. He reported two episodes of vomiting prior to arrival. He denied fevers, chills, diarrhea, constipation, dark or bloody stools, flank pain, back pain, urinary complaints, chest pain, shortness of breath, palpitations, or syncope. Initial vital signs were temperature 36.8°C, blood pressure 157/106 mmHg, heart rate 83 beats per minute, respiratory rate 14 breaths per minute, and oxygen saturation of 95% on room air. Physical examination revealed an elderly male in moderate distress secondary to pain. Significant tenderness to palpation in the epigastrium and left upper quadrant was elicited on abdominal examination without rebound, guarding, or peritoneal signs. The remainder of the physical examination was unremarkable. Electrocardiogram (ECG) revealed normal sinus rhythm without acute ischemic changes.The clinical presentation was concerning for an acute intra-abdominal emergency. CT of the abdomen and pelvis, laboratory studies, pain medications, antiemetics, and intravenous (IV) fluids were ordered. Emergency medicine resident physicians and an ultrasound fellowship-trained attending EP then performed an abdominal POCUS examination. POCUS was notable for a large, dilated, fluid-filled stomach highly concerning for an obstructive process Figures , 2.CT imaging was subsequently expedited based upon the POCUS findings. CT of the abdomen/pelvis revealed a moderate to large hiatal hernia and a distended edematous stomach with partial obstruction due to GV Figures -5. GenerThe patient was admitted for further management of GV. Gastroenterology was consulted for emergent esophagogastroduodenoscopy (EGD) that confirmed the presence of a partial organoaxial volvulus without evidence of gastric ischemia. EGD did not reveal any underlying pathology in the upper gastrointestinal tract with the exception of large hiatal hernia and mild gastritis. General surgery then performed laparoscopic reduction of GV, hiatal hernia repair with mesh, and Nissen fundoplication. The patient was soon tolerating a full liquid diet and closely managed post-operatively by general surgery and gastroenterology. The patient was discharged home in stable condition on hospital day 6 with instructions for outpatient surgery follow-up.The ED evaluation of acute abdominal pain requires a diligent and meticulous approach to identify critical underlying pathology. Symptoms may be vague or non-specific, and the classic constellation of clinical findings may not be present. Acute GV with complete obstruction will oftentimes be recognized with the classical Borchardt’s triad of intractable retching, severe epigastric abdominal pain and distention, and difficulty or inability to pass an NGT ,4. ImporThe choice of initial imaging study is significant when there is suspicion for GV, as diagnostic delay can lead to fatal complications. CT imaging is highly reliable and accurate, with two findings  with 100% sensitivity and specificity for the diagnosis of acute GV ,19. One POCUS is an effective non-invasive imaging modality for the bedside assessment of a multitude of abdominal emergencies. Moreover, POCUS offers the vital advantages of avoiding radiation exposure and is readily available for repeat serial examinations as dictated by the clinical scenario. Previous literature has reported the utilization of POCUS for identifying gastric dilation in cases of GOO ,15. We dGV is a life-threatening surgical emergency that warrants prompt EP recognition and management. Implementation of POCUS findings into clinical decision making has the potential to facilitate an expedited diagnosis. Further larger-scale studies should investigate the diagnostic accuracy and ideal role of EP-performed POCUS as a first-line imaging strategy for GV.


Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of Ewing sarcoma. It demonstrates heterogeneous morphologic pattern and complex immunophenotypic profile, with a peculiar combination of epithelial and neuroendocrine differentiation. ALES is rarely reported in the head and neck areas, including the parotid salivary gland. Till now, only 10 cases of ALES have been reported in the salivary glands. Herein, we report two cases of ALES involving the parotid gland, adding some valuable insight to the recently reported cases at this site. Ewing sarcoma (ES) is a small round cell tumor typically reported in bone and soft tissues at a young age group. In addition to their morphological and immunophenotype features, these tumors are defined by the demonstration of EWSR1 gene rearrangement. This occurs due to a translocation between EWSR1 and a gene of the ETS family of transcription factors, of which FLI1 constitutes about 85% of fusion partners . ApproxiFirst caseA 29-year-old male presented with a year history of right cheek mass that was gradually increasing in size. A superficial parotidectomy was initially done in another hospital, and subsequently, was diagnosed as adenocarcinoma. He was presented to our institution with residual disease. Computed tomography (CT) with contrast revealed an ill-defined mass measuring 8.8 cm in greatest dimension in the right parotid gland with central necrosis. It was invading the overlying skin and bulging into the right neck space Figure . CT scan Second caseA 46-year-old female presented with a history of swelling behind the right ear over the parotid area for two years. The case was a referral from outside hospital to our institution. She had a history of multifocal papillary thyroid microcarcinoma seven years before, for which a total thyroidectomy was done, and radioactive iodine was given. Radiologically, a solid mass measuring 2.0 cm in greatest dimension was identified, occupying the superficial lobe of the right parotid gland Figure . A rightALES is a rare tumor with predilection to the head and neck areas . This diThe differential diagnosis of adamantinoma-like EFT is not limited to epithelial neoplasms. Small round blue cell tumors should also be considered in the differential diagnosis. Desmoplastic small round cell tumors are rarely found in the head and neck regions and usually express CD99, cytokeratin, desmin and WT-1. However, they are negative for p63 and p40 expressions ,8. TheseThe unique nuclear monotony of ALES is reported to be an important feature in the correct reorganization and differentiation from the more commonly encountered higher grade tumors in the head and neck area . Once thIn conclusion, although ALES is a rare and recently described entity, it carries a peculiar combination of epithelial and neuroendocrine differentiation. The recognition of this cellular phenotype is important for appropriate management of patients.


Teaching point: Giant mucocele is a rare expansile lesion that may mimic other locally aggressive lesions of the cranial vault.Giant frontal mucoceles with massive osteolytic destruction mimicking an aggressive lesion are rare compared to smaller mucoceles. This article reports a giant mucocele of the frontal sinus and reviews the literature. Important imaging clues pointing toward the diagnosis of a mucocele on computed tomography (CT) and magnetic resonance imaging (MRI) are a well-defined expansile mass, an intimate relationship with the frontal sinus, subtle peripheral rim enhancement, and slow progression on serial imaging. The density on CT and signal on MRI may vary along with the lesion content. The potential role of diffusion-weighted imaging should be elaborated in future reports. Giant mucoceles of the frontal sinus are rare clinical entities, and only a few cases are published in the current literature 23456. Th2345A 72-year-old woman was admitted at the emergency department after a fall. A large bump was noted on her left forehead and subsequently a non contrast-enhanced CT of the head was performed Figure . The CT Subsequent MRI of the brain Figure  depictedParanasal mucoceles are benign, epithelium-lined cysts filled with mucoid material. Mucoceles develop when the sinus ostium is obstructed resulting in progressive accumulation of secretions and epithelial cells in the sinus cavity, with subsequent expansion of the involved sinus . Sinus eThe frontal and ethmoid paranasal sinuses are involved in up to 90% of cases. The maxillary sinus is affected less frequently 10%) and the sphenoid sinus only rarely % and the.Mucoceles that are sufficiently large may exert mass effect on the surrounding anatomic structures. The clinical symptoms vary according to the location 11.A literature search for cases of giant frontal mucoceles yielded 13 relevant articles. Giant mucoceles of other paranasal sinuses are beyond the scope of this article. Cases were included as long as they presented scientific rigour and relevant bibliographic sources. Eight cases were excluded due to a lack of descriptive parameters and/or images of the mucoceles. The remaining five cases are summarized in Table In the current literature there are no criteria regarding the use of the term “giant” mucocele. Based on the reported dimensions in other case reports, we propose 5 cm as a cut-off value of the lesion size.CT and MRI are complementary when imaging mucoceles. CT depicts an expansile, homogenous mass with remodeling of the adjacent bone . OccasioIn the three cases where contrast was administered, subtle peripheral enhancement was seen, similar to characteristics of smaller mucoceles described in the literature. .MRI can be helpful when differentiating mucoceles from other aggressive lesions. Signal intensity on T1-WI is variable (low in case of a low protein content and high in case of a high protein content). All reviewed giant mucoceles were bright on T2-WI, which may be explained by their high fluid content. However, desiccation of the mucocele contents has been described in chronic non-giant cases, resulting in decreased signal intensity on T1- and T2-weighted images .There was no diffusion restriction in our case and the case reported by Singh et al. . Future Mucoceles should be differentiated from mucus retention cysts. Unlike mucoceles, sinus retention cysts do not result in expansion and thinning of the bony sinus walls . Table 3Lastly, surgical excision is the treatment of choice .Although there is no standard definition regarding the size of a “giant frontal mucocele”, we propose 5 cm as a cut-off, based on the reported dimensions in other cases studies. The typical imaging findings of a giant mucocele consist of a well-defined expansile lesion located at a paranasal sinus and slow progression on serial imaging. Both the density on CT and signal intensity on MRI can be variable depending on the content of the mucocele. The potential role of diffusion-weighted imaging should be elaborated in future reports.


Budd–Chiari syndrome (BCS) is a rare intrahepatic vascular disease that is characterized by a hepatic venous outflow obstruction. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma. Here, we report a case of BCS that was caused by intracaval ILs. A woman presented to the emergency department (ED) with abdominal distension that had gradually progressed over a period of 3 years. Bedside ultrasonography and contrast-enhanced computed tomography (CECT) showed a large ascites and pelvic mass. The mass continued to the inferior vena cava and the right atrium. The intracaval mass was obstructing the left and middle hepatic veins. We established a tentative diagnosis of BCS caused by intracaval ILs and attempted surgical resection. Complete resection of the intracaval mass failed because of adhesion; however, she was discharged from the hospital without any postoperative complications. After 3 months, a pelvic ultrasonography showed a recurrence of a 4 × 3 cm pelvic mass. The mass size increased to 6 cm after 30 months. ILs can cause secondary BCS and can lead to life-threatening conditions. Owing to its extreme rarity, early detection in the ED is challenging. Bedside ultrasonography and CECT can enable the early recognition of BCS by ILs. Myoma is the most common type of gynecological tumor among pre-menopausal middle-aged women. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma, characterized by venous invasion of myoma tissue . Since iBudd–Chiari syndrome (BCS) is a rare heterogeneous group of intrahepatic vascular diseases characterized by a hepatic venous outflow obstruction. It manifests as the following three characteristic symptoms: abdominal pain, hepatomegaly, and ascites, and can be life-threatening if appropriate treatment is not administered ,5.Since the first case was reported in 1996 by Kuenen et al., only two cases of BCSs as complications of ILs have been reported in the English-language literature ,8. We reA female patient in her early 50 s with abdominal pain and distension visited the ED. She had first experienced abdominal pain and distension 3 years previously; however, the patient was not treated, owing to financial limitations. The abdominal distension had gradually worsened in the 3 months before her presentation to the ED. She had been diagnosed with uterine myoma 15 years previously; however, she did not undergo any medical treatment. She consumed about 1 bottle of soju for 2 weeks and took no medications other than sleeping pills. The patient’s history constituted two gravida and two abortions, irregular periods, and dysmenorrhea. She started menstruating at 14 years of age. There was usual hypermenorrhea; however, she had not menstruated in the previous 2 months.On presentation, her blood pressure was 140/80 mmHg, pulse rate was 74 bpm, and oxygen saturation was 96%; she was afebrile. On physical examination, we found that her abdomen was severely distended, and she had pitting edema on both legs. Moreover, right lung auscultation was decreased. The laboratory results included a hemoglobin concentration of 10.9 g/dL and platelet count of 373 K/μL. Serum profiles, including blood chemistry, electrolyte, and liver and renal function tests were normal; however, her albumin level was 2.69. Proteinuria was observed on urinalysis. Chest radiography showed right pleural effusion.Bedside ultrasonography for abdominal distension showed a large amount of ascites and right pleural effusion, and hepatomegaly with an irregular surface was visible a. A 10 ×Two hours after admission, her blood pressure suddenly dropped to 80/60 mmHg. Bedside ultrasonography was re-implemented, and saline was administered rapidly. There was no interval change in the abdominal findings. However, the inferior vena cava (IVC) was scanned for volume measurement, and a hyperechoic mass was found in the IVC b. The maAfter hydration, the patient’s blood pressure rose to 100/80 mmHg. Consistent with the bedside ultrasonography findings, the CECT showed large amounts of ascites and pleural effusion, multiple lobulated pelvic cavity mass, and mass in the IVC and the heart . The hypWe established a tentative diagnosis of BCS caused by ILs with IVC and heart extension and decided to perform surgical resection using a team approach. Thoracic, gynecology, and vascular surgeons participated in the surgery. First, a total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed by the gynecological surgeons. A pelvic mass measuring about 11.5 × 7.5 × 6.0 cm was removed and identified as a myoma on biopsy .Thereafter, intracardiac and intracaval tumor removal was performed by thoracic and vascular surgeons. A well-capsulated intracardiac mass was freely movable in the RA and RV; however, the intracaval tumor could not move freely and densely adhered to the RA-IVC joint, and blind traction was considered dangerous; therefore, debulking surgery was performed. The formal report of histopathology of thoracic and vascular specimens showed the following results. The first specimen consisted of a piece of an intracardiac mass (mass of the right atrium) , and visual examination showed a hard, homogeneous tan mass with a swirling pattern. There was no bleeding or necrosis. It was judged to be a thrombus. A second specimen consisting of a piece of pericardial tissue (1.7 × 0.8 cm) could be diagnosed as a benign smooth muscle tumor consistent with intravenous leiomyomatosis. The third specimen consists of three pieces of intravascular mass  and can be diagnosed as intravenous leiomyomatosis .After surgery, aspartate aminotransferase, alanine aminotransferase, and direct bilirubin increased to 1306 IU/L, 1053 IU/L, and 6.21 mg/dL, respectively, but on the 28th day of hospitalization, improved to 75 IU/L, 97 IU/L, and 0.21 mg/dL, respectively. The patient was discharged without complications 1 month postoperatively. On pelvic ultrasonography that was performed after 3 months, a 4 × 3-cm pelvic mass had recurred .Furthermore, 30 months postoperatively, pelvic ultrasonography showed that the tumor size had increased to 6 cm. Since then, the patient has not visited the outpatient clinic.In the diagnosis of BCS, clinical suspicion is most significant. However, because its clinical presentations are similar to those of liver and biliary tract diseases, which are common diseases in the ED, establishment of a diagnosis of BCS by symptoms in the ED is challenging.The clinical presentation of BCS depends on the extent and rapidity of hepatic vein occlusion and venous collateral circulation. Asymptomatic patients account for about 15% of the total patients, and about 10% show clinical symptoms of acute diseases and visit the hospital ,10. The Bedside ultrasonography is a useful imaging tool for the discrimination of causes of abdominal distension, shortness of breath, and shock in the ED. Because bedside ultrasonography is useful to detect pelvic masses and intracaval and/or intracardiac status, and can evaluate hepatic vein flow, it can be an appropriate initial imaging tool for the early recognition of ILs and BCS by ILs . CECT isMRI is the most accurate way to detect, localize, and characterize myoma. In ILs, MRI can be useful for distinguishing between tumors and blood clots . HoweverThus far, no standard treatment has been established for BCS; however, expert groups have suggested relief in the hepatic venous outflow duct obstruction as the main treatments . The natThe key to the treatment of ILs as a cause of BCS is the surgical resection of tumors, although no definite treatment has been established due to its rarity . SurgicaThe recurrence may increase depending on the residual ILs; complete removal of the tumor is the key to preventing recurrence . ILs thaHormonal therapy for ILs has been attempted on the rationale that the tumor has hormonal properties similar to uterine myoma . AlthougAnti-estrogen therapy has shown good effects on ILs in some early cases but has not shown consistent evidence of reducing tumor size or improving clinical outcomes for ILs ,25. GonaCurrently, the primary treatment for ILs is surgical resection, and hormonal therapy cannot replace this treatment. Hormone therapy can be used as an adjuvant therapy to reduce tumor size before surgery or to minimize tumor growth after incomplete resection. It can also be used as an optional treatment for patients who refuse or are unable to have surgery.Abdominal distension is a common cause for visits to the ED. ILs, a rare complication of leiomyoma, can cause secondary BCS by obstruction of hepatic out-flow. Although ILs is histologically benign, it can lead to life-threatening conditions. Owing to its extreme rarity, it is difficult to identify BCS by ILs in the ED. Bedside ultrasonography and CECT, initial imaging tools for abdominal distension, may be useful for the early recognition of BCS by IL.


We present a case demonstrating the performance of different radiographical imaging modalities in the diagnostic work-up of a patient with neurofibromatosis type 1 (NF1) and plexiform neurofibroma (PN). The newborn boy showed an expansive-infiltrative cervical and facial mass presented with macrocrania, craniofacial disfigurement, exophthalmos and glaucoma. A computer tomography (CT) and a magnetic resonance imaging (MRI) were performed. The CT was fundamental to evaluate the bone dysmorphisms and the MRI was crucial to estimate the mass extension. The biopsy of the lesion confirmed the suspicion of PN, thus allowing the diagnosis of NF1. PN is a variant of neurofibromas, a peripheral nerves sheath tumor typically associated with NF1. Even through currently available improved detection techniques, NF1 diagnosis at birth remains a challenge due to a lack of pathognomonic signs; therefore congenital PN are recognized in 20% of cases. This case highlights the importance of using different radiological methods both for the correct diagnosis and the follow-up of the patient with PN. Thanks to MRI evaluation, it was possible to identify earlier the progressive increasing size of the PN and the possible life threatening evolution in order to perform a tracheostomy to avoid airways compression. The patient was born at the thirty-eighth week of gestational age by caesarean section following the finding of cerebral ventriculomegaly on last trimester ultrasound. At birth, the child had the following anthropometric parameters: weight 3090 g (34 °p), length 51 cm (77 °p), and head circumference 37.5 cm (100 °p). He presented numerous dysmorphisms due to the presence of a right abnormal facial and laterocervical mass, including macrocrania and craniofacial asymmetry with greater development of the right half-face, exophthalmos of the right eye with glaucoma.The axial T2-weighted (T2W) sequences on MRI showed an ill-defined giant cervical and facial mass of the right side a, infiltThe histologic examination after mass biopsy revealed diffuse nerve fascicles distension caused by a proliferation of spindle-shaped cells embedded in myxoid matrix (hematoxylin-eosin (EE), 2.5×) consistent with plexiform neurofibroma (PN) d.PNs arise from multiple nerve and are commonly associated with neurofibromatosis type I NF1). Although histologically benign, PN are associated with significant morbidity and mortality, both because surgical treatment is complex due to their usually very extensive and compressing ever infiltrative nature and because they are highly resistant to conventional chemotherapy. They are usually congenital, but they may instead present during the first year as a subtle soft-tissue enlargement or a large patch of cutaneous hyperpigmentation. They occur in at least 50% of patients with neurofibromatosis type I (NF1). Many are asymptomatic and the exact timing of growth of plexiform tumors is unpredictable. Boulanger JM et al. [. Althoug-Six or more café au lait macules >5 mm in greatest diameter in prepubertal individuals and >15 mm in greatest diameter in postpubertal individuals-Two or more neurofibromas of any type or one plexiform neurofibroma-Freckling in the axillary or inguinal regions-Optic glioma-Two or more Lisch nodules (iris hamartomas)-A distinctive osseous lesion such as sphenoid dysplasia or tibial pseudarthrosis-A first-degree relative  with NF1 as defined by the above criteriaNF1 is one of the most frequent hereditary neurocutaneous disorders with a birth incidence of about 1:2500. NF1 is caused by heterozygous germ-line mutations in the tumor suppressor gene NF1, located on chromosome 17q11.2. NF1 encodes a protein called neurofibromin, which functions, in part, as a negative regulator of the Ras proto-oncogene, a key signaling molecule of cell growth. NF1 has a high inter and intra familiar clinical heterogeneity :-Six or The diagnosis is in some cases difficult since many of the features are age-dependent.c.1318C > T). Further investigations, only after the newborn diagnosis of NF1, revealed that the child’s mother had the clinical stigmata of NF1 confirmed by the presence of the same constitutional mutation. She had also previously undergone surgery for a cutaneous neurofibroma, without receiving further genetic counseling.About 50% of individuals with neurofibromatosis type 1 have no family history of the disease and the disease is due to de novo mutations. In our case a specific mutation of the NF1 gene was detected  performed at birth, the characteristic sign of the neuronal origin of the lesion, defined as “target sign” a, was evIn order to define the lesion and the subsequent facial alterations more precisely, CT scan was performed at birth and it underlined bony defects, such as thickened and sclerotic bone and dysplastic change, involving the sphenoidal bone, the orbital cavity and the adjacent right skull base e. The riThe T2 target sign in our case was more visible at the follow-up a,b. In oPNs are typically congenital, but they show their clinical evidence between 2 and 5 years . DiffereThey are highly resistant to conventional chemotherapy; therefore, the management of PNs in the pediatric patient consists of serial follow up and observation. Surgery still remains the main stay of treatment but is reserved for the most severe of cases . In caseDifferential diagnosis based on imaging includes lymphatic or venous malformation, hemangioma, traumatic or inflammatory lesions.In conclusion, NF1 is one of the most frequent hereditary neurocutaneous disorders. In clinical practice, the first manifestation of the disease can be a congenital PN, especially in newborns, as in our case. MRI is immensely helpful to guide the correct diagnostic hypothesis and to weigh the differential diagnoses and eventual malignant transformation. Radiologists must be aware of typical MRI appearance of PN. This case highlights the importance of using different radiological methods (CT and MRI) for the correct diagnosis and the follow-up of the patient with PN. However, CT scan should be used sparingly in patients affected by NF1, and may provide valuable information in cases of extensive PN resection. Thanks to MRI evaluation, it was possible to identify earlier the progressive increasing size of the PN and the possible life-threatening evolution in order to perform a tracheostomy to avoid airways compression.In case of extensive surgical resection, preoperative imaging should be performed or the surgical planning. In this scenario, both CT and MRI provide complementary information to the surgeon. MRI is helpful in assessing the PN margins and the relationship with surrounding structures. Conversely, CT scan is more suitable to reveal bone involvement and relationships between the PN and the bony structures.Different radiological techniques are fundamental for the management of PNs. They allow detection of the correct diagnosis and the eventual malignant transformation. MRI and TC evaluations guide also the correct follow-up, intervening only in those cases selections that really require surgery.This case highlights the importance of using different radiological methods (CT and MRI) for the correct diagnosis and the follow-up of the patient with PN. Thanks to MRI evaluation, it was possible to identify earlier the progressive increasing size of the PN and the possible life-threatening evolution in order to perform a tracheostomy to avoid airways compression.


Synovial sarcoma (SS) is one of soft tissue sarcomas (STS), characterized by t chromosomal translocation. Clinical diagnosis of SS in groin is difficult owing to rarity and various manifestations. We reported a rare and interesting case of inguinal biphasic SS with initial impression of a vascular lesion.We reported a 72-year-old man who presented with a right inguinal progressively enlarged mass for 5 years. The mass became ulcerative with active bleeding after blunt trauma. Computed tomography (CT) showed a large heterogeneous tumor with focal vivid enhanced area, and an inguinal vascular lesion was impressed. Angiography revealed faint tumor stain without contrast extravasation or aneurysm lesion. Surgical resection of the tumor was performed. Final pathology and fluorescence in situ examination (FISH) demonstrated biphasic SS with SS18 gene rearrangement. No evidence of recurrence after one-year follow-up.SS constitutes 5%–10% of STS. The most primary sites are extremities (68.7%) and trunk (15.7%). Treatment options for SS include surgery, chemotherapy, radiotherapy, targeted therapy, and novel therapies. In our case, the initial impressions of large inguinal tumor were hematoma or pseudoaneurysm. Preoperative angiography assisted in differentiating the lesion, evaluating tumor vessels, and preventing bleeding by embolization. Our patient recovered satisfactorily after surgical excision without major complications.Primary inguinal SS is an extremely rare tumor which can present as a vascular lesion. Diagnosis is confirmed by pathology and FISH exam. Further studies are required to determine the manifestation and management of SS. SS can occur at any age, but it commonly occurs in teenagers and young adults aged 15–40 years . From a Numerous unusual locations of SS have been reported and confirmed by using molecular and immunohistochemistry techniques Cases of SS primarily originating in inguinal areas are rare in English language literature . Many le26 uL; hemoglobin level: 8.9 g/dL; and hematocrit level: 26.2%). Ultrasound examination revealed a heterogeneous mass, diameter up to 15 cm, with abundant blood supply, as assessed by Doppler mode. Computed tomography (CT) revealed a well-circumscribed right inguinal heterogeneous tumor, measuring up to 15 cm, with a vivid enhanced area, implying the possibility of vascular lesion  and trunk (15.7%) . The cel8.7% and Treatment options for SS include surgery, chemotherapy, radiotherapy, targeted therapy, and novel therapies. Wide-margin surgical resection remains the essential treatment for localized tumors. However, no consensus exists regarding the systemic treatment for SS. Recent studies have adequately discussed insight into neoadjuvant chemotherapy. Le Cesne et al. pooled two phase III clinical trials and revealed that adjuvant chemotherapy provided no overall survival or relapse-free survival benefits in completely resected SS . A phaseFour studies reporting inguinal SS in the literature were reviewed . Hiede eIn our case, the initial impressions of large inguinal tumor were hematoma or pseudoaneurysm. Preoperative angiography assisted in differentiating the lesion, evaluating tumor vessels, and preventing bleeding by embolization. We did not performed embolization in our patient due to high risk of nontargeted embolization to abdominal skin. Our patient recovered satisfactorily after surgical excision without major complications. We suggested adjuvant chemoradiotherapy, but the patient refused due to personal reasons. There was no evidence of recurrence for 12 months follow-up.4In conclusion, this is the first case report of biphasic SS in inguinal region. Various manifestations and rarity of SS lead us have the first impression of vascular lesion. Our study demonstrated diagnostic image, surgical views, and treatment options for SS. Complete surgical resection is main treatment. Additionally, we reviewed and summarized reported cases of inguinal SS. Additional case reports and studies are warranted to achieve a clearer understanding of diagnoses and treatments of SS in groin.The authors report no declarations of interest.This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.The research related to human use has been complied with all the relevant national regulations, institutional policies, and has been approved by the Research Ethical Committee of Taipei Medical University Hospital (Case number: N201910051).Written informed consent was obtained from the patient for publication of this case report and accompanying images.Data collection: Wei-Chieh Chen and Po-Chien Wu.Writing the paper: Po-Chien Wu and Ting-En Tai.Editing the paper: Wei-Chieh Chen and Chun-Yu Lin.1.Name of the registry: Research Ethical Committee of Taipei Medical University Hospital.2.Unique identifying number or registration ID: N201910051.Ting-En Tai.Not commissioned, externally peer-reviewed.


Retroperitoneal pseudocyst (RPC) is an uncommon surgical entity. The pseudocyst is characterized by the absence of epithelial lining in the cyst wall. Mostly, it occurs as a sequela of pancreatitis. Pseudocyst due to a non-pancreatic cause, however, is very rare.We report a 49-year-old male, who presented to the emergency department with moderate intensity lower abdominal pain. Computed tomography scans revealed a huge retroperitoneal cyst which was overlying the right ureter and right iliac vessels. The patient underwent laparoscopic excision of the cyst and recovered without any complications. Histopathological examination showed a non-pancreatic RPC.In conclusion, non-pancreatic RPC is a rare surgical disease which can result in pressure symptoms depending on its location and size. In our patient, it was treated by laparoscopic excision despite its proximity to iliac vessels and ureter.A laparoscopic approach using safe surgical principles is a viable option for non-pancreatic RPC. The proximity of the lesion to the iliac vessels and the ureter can be carefully navigated safely by laparoscopy. The ureter can be confirmed by stimulating peristalsis of the duct when in doubt. Retroperitoneal cysts are uncommon with a reported incidence of 1 in 5750 to 1 in 250,000  [A 49-year-old Indian male with no known co-morbidities, presented with right iliac fossa pain for one day. The pain started gradually and progressively increased in severity. It was non-migratory and non-radiating without any aggravating factors.The patient has a history of constipation for 5-6 years for which he was taking laxatives intermittently. There was no history of weight loss or bleeding per rectum, similar prior episodes, night sweats nor any contact with tuberculosis patients. His family history was non-contributory. His past medical history was significant for left inguinal hernia repair.At presentation, his vitals were within normal range. His abdomen was non-tender, non-distended and soft to touch. He had mild fullness in right lower quadrant. However, no definite mass was palpable. Digital rectal exam was normal.InvestigationsHis labs revealed WBC of 11,400 per microliters with neutrophil of 82%, hemoglobin was 15 gm/dl, platelets 273,000 per microliters, rest of the labs were within normal limits. Quantiferon test for tuberculosis was negative and Echinococcus antibody titer was normal. Tumor markers carcinoembryonic antigen (CEA) and Alfa fetoprotein (AFP) were done and were normal.The patient underwent CT scan, which revealed a 7 x 6 cm cystic lesion with incomplete peripheral calcification in pelvis more to right side Figure . The cysDifferential diagnosisThe differentials of this lesion in the right lower quadrant were benign and malignant cystic lesions. We considered it a benign cyst, based on the history of no weight loss, normal tumor markers and radiological findings of a well-defined cyst on CT scan which does not appear to involve or invade any surrounding structures. One of the possibilities was an echinococcal cyst but it was ruled out by the absence of daughter cysts, non-enhancing wall or floating membrane, and normal echinococcal titers. The other differentials were neoplastic cystic lesions but it was less likely as the cystic mass had a well-defined wall without any features of invasion to surrounding structures.It was really challenging to figure out the diagnosis based on history and imaging. That’s why we opted for laparoscopic excision of the mass to establish a definitive diagnosis.TreatmentThe patient underwent laparoscopic cyst excision Video .The location of the cyst was clearly the retroperitoneum as there was a complete peritoneal covering over the lesion. The cyst was compressing the right ureter and in proximity with the right internal iliac vessels. It was dissected carefully from the right ureter and right iliac vessels using Harmonic scalpel and safe laparoscopic surgical principles. Identification of the ureter by gently stimulating peristalsis is clearly demonstrated in the video. Inadvertent opening of the cyst occurred during dissection of the posteromedial part of the cyst due to a very thin false capsule. The fluid content of the cyst was aspirated and tested for amylase and tumor markers (AFP and CEA) but was not detected, confirming the diagnosis of a non-pancreatic pseudocyst. The mass was excised completely and removed using a specimen retrieval bag after copious irrigation and ensuring good hemostasis.Outcome & follow-upPostoperatively, the patient recovered well with no complications. Oral fluids were started on postoperative day 1 which was progressed to full diet on the same day. On postoperative day 2, the patient was pain-free, had passed stool, had adequate urine output and was discharged home. After two weeks, he was followed up in the clinic with a normal abdominal examination and well-healing wounds.His histopathology showed a benign fibrous cyst wall containing fibrin with cholesterol clefts. There was no epithelial or endothelial lining and it was negative for granulomas or malignancy Figure .EtiologyRetroperitoneal cysts were described by Handfield in 1929, as cysts that arise in the fatty tissue in the retroperitoneum which have no connection to adult anatomical structure except the areolar tissue .Yang et al. classified these cysts into neoplastic and non-neoplastic lesions . NeoplasAlthough RPCs are classified generally as retroperitoneal cysts, one should consider that they lack a true epithelium and are, therefore, considered a separate entity. Pseudocysts commonly occur as a result of pancreatitis. Non-pancreatic RPC, on the other hand, is infrequent and has an unknown etiology. It can originate from mesentery, mesocolon or omentum .Clinical presentation & investigationsThere are no pathognomonic signs and characteristic symptoms for non-pancreatic RPC. Most of them attain large size before becoming symptomatic usually by compressing on adjacent structures. Patients usually present with vague abdominal pain, distension, referred pain to legs, lower limb edema, weight loss, and fever. Occasionally, patients have a sudden onset of abdominal pain which occurs due to infection or hemorrhage within the non-pancreatic RPC itself ,6.CT scan is a valuable tool in the evaluation of abdominal masses. It may not be able to completely differentiate benign from malignant lesions. However, it may give a clue about the diagnosis. That is why a pathological diagnosis is necessary. On CT, these lesions appear as a unilocular or multilocular cystic mass with thick walls. Occasionally, calcification of the pseudocyst wall results in an “eggshell” appearance. The contents of the pseudocyst can be hemorrhagic, purulent or serous which unlike pancreatitis is not associated with high amylase and lipase -7. In ouTreatmentThe treatment of RPC is surgery as there is a risk of infection, rupture and malignant change. Surgical options include marsupialization, drainage and cyst excision . Due to A laparoscopic approach is associated with less post-op pain, early recovery and shorter hospital stay. Pneumoperitoneum creates a space to work and with the addition of high definition angulated cameras, laparoscopy provides excellent exposure which is particularly helpful in obese patients and in the male pelvis. It also helps to identify correct tissue planes easily thus preventing inadvertent injury to adjacent vital structures . In our An idiopathic non-pancreatic RPC is a rare surgical entity which carries a wide range of differential diagnosis. Surgical excision is the only way to establish a definitive diagnosis and to exclude malignancy. A laparoscopic excision should be considered if expertise is available as it is quite useful in working in narrow space. However, care should be taken to prevent cyst rupture intraperitoneally and injuring the adjacent structures.


Traumatic diaphragmatic hernias (TDHs) are uncommon, and they mostly occur following blunt or penetrating traumatic injury and rarely as a complication of Iatrogenic procedure. The management of TDHs is through surgical repair. In this article, we present the case of a diaphragmatic herniation presenting 1 year after thoracic surgery in a 16-year-old male. The patient presented with gastrointestinal obstruction symptoms and later developed a hypovolemic shock due to stomach bleeding. Immediate exploratory thoracotomy was performed, and the patient reached a full recovery afterward. Traumatic diaphragmatic hernias (TDHs) are uncommon but life-threatening, and they remain a diagnostic and therapeutic challenge . TDHs haIn this case, we report a patient suffering from gastrointestinal symptoms caused by a delayed TDH.A 16-year-old male presented to the outpatient clinic complaining of epigastric pain that radiates to the left hypochondrium accompanied by severe vomiting immediately after eating or drinking. No other associated symptoms existed; his medical history was clear of any significant complaints. He denied any thoracic or abdominal trauma, but his surgical history included the resection of a hydatid cyst in the lower zone of the left lung through a thoracic approach 1 year previous to the current complaint. No further information about the surgery was available.On examination, we found a decrease in respiratory sounds in the lower zone of the left lung, with the absence of inflammatory signs. The remaining of the physical examination and lab test results were all within normal limits.Chest X-ray  showed aA catheter was placed and fluid infusion began, and emergency thoracentesis showed fresh blood. Immediate exploratory thoracotomy was performed . Three lAn X-ray image  was doneDiaphragmatic hernias are either congenital or secondary due to trauma . TDHs arApproximately, 0.8–3.6% of thoracoabdominal trauma cases include diaphragmatic rupture , with a Latrogenic diaphragmatic hernias are a rare complication of abdominal surgery , and theIn our case, the trauma was probably Iatrogenic, the diaphragm was most likely injured by the adjacent surgical procedure and the herniation occurred within a year.TDHs presentation varies significantly; it can be asymptomatic and might also have respiratory, abdominal or cardiac symptoms or even failure . ComplicIn our case, the patient suffered from upper gastrointestinal obstruction symptoms but was only admitted after the complication had occurred, and he presented with hypovolemic shock due to stomach bleeding which caused a hemothorax.TDH surgical repair involves diaphragm closure through an abdominal or thoracic approach . In our In conclusion, TDHs might be a differential diagnosis in patients complaining of upper gastrointestinal obstruction symptoms with a history of thoracic surgery. When the diagnosis of TDH is confirmed, immediate intervention might prevent dangerous complications.None declared.None.


Endobronchial lipoma is a rare benign tumor. The standard treatment is bronchoscopic intervention or surgical resection. Here, we present a case of endobronchial lipoma treated by right basal segmentectomy via a uniportal thoracoscopic approach.A 66-year-old female presented with a persistent cough and recurring high-grade fever 4 months in duration. Chest X-ray revealed an abnormal infiltration shadow in the right lower lung field. Chest computed tomography revealed a tumor that occluded the lateral segmental bronchus of the right lower lobe. Surgical resection was planned because we failed to diagnose the tumor via bronchoscopic biopsy. Finally, uniportal, thoracoscopic right basal segmentectomy, which is less invasive than lobectomy and allows preservation of the apical segment of the lower lobe, was successfully performed. The pathological diagnosis was of endobronchial lipoma. The cough and recurrent pneumonia improved after surgery.Segmentectomy via a uniportal thoracoscopic approach could be a novel treatment option for endobronchial lipoma. Endobronchial lipoma, first reported by Carl von Rokitansky in 1854, is extremely rare, constituting only 0.1–0.5% of all lung tumors and 4.6% of all benign lung tumors . Occasio9) . The operation was performed using one-lung ventilation with the patient under general anesthesia in the lateral decubitus position. A 4-cm access incision was made in the fifth intercostal space of the anterior axillary line. Intraoperative findings revealed a dense hilum due to inflammatory changes caused by the repeated pneumonia. The duration of surgery was 170 min, and the blood loss was 50 ml (supplementary video). Histology confirmed endobronchial lipoma, and that the resected specimen had negative margins  Fig. b. Magnet9) Fig. c, d. Bro9) Fig. a. Howeveins Fig. b–d. The Additional file 1. Supplementary video. The uniportal thoracoscopic right basal segmentectomy. Intraoperative findings revealed a dense hilum due to inflammatory changes caused by the repeated pneumonia.Endobronchial lipoma is more frequent in males than females; the mean patient age is 60 years. The tumor shows a preference for an airway lobar or subsegmental location and is more frequent on the right side, as in our case . Common We failed to diagnose the tumor bronchoscopically because insufficient material was obtained; the tumor bled readily. Elsayed et al. failed to diagnose endobronchial lipoma via flexible bronchoscopy for the same reason and reported that large excisional biopsy was optimal for diagnosing benign tumors such as endobronchial lipomas . NussbauMuraoka et al. recommended surgical resection of endobronchial lipomas under the following conditions: 1) difficulty in obtaining a definitive diagnosis along with a possibly malignant tumor, (2) peripheral destructive lung disease caused by long-term atelectasis or pneumonia, (3) presence of an extrabronchial growth or subpleural lipomatous disease, and (4) expected technical difficulties during bronchoscopy due to the presence of multiple tumors  difficul. Here, wIn conclusion, segmentectomy via a uniportal thoracoscopic approach is a useful novel surgical option for endobronchial lipoma, associated with rapid recovery and preservation of respiratory function.


Rosette-forming glioneuronal tumors (RGNTs) are rare, low-grade, primary CNS tumors first described in 2002 by Komori et al. RGNTs were initially characterized as a World Health Organization (WHO) grade I tumors typically localized to the fourth ventricle. Although commonly associated with an indolent course, RGNTs have the potential for aggressive behavior.A comprehensive search of PubMed and Web of Science was performed through November 2019 using the search term “rosette-forming glioneuronal tumor.” Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. English, full-text case reports and series with histopathological confirmation were included. Patient demographics, presentations, MRI features, tumor location, treatment, and follow-up of all 130 cases were extracted.A 19-year-old man with a history of epilepsy and autism presented with acute hydrocephalus. MRI scans from 2013 to 2016 demonstrated unchanged abnormal areas of cortex in the left temporal lobe with extension into the deep gray-white matter. On presentation to our clinic in 2019, the lesion demonstrated significant progression. The patient’s tumor was identified as RGNT, WHO grade I. One hundred thirty patients were identified across 80 studies.RGNT has potential to transform from an indolent tumor to a tumor with more aggressive behavior. The results of our systematic review provide insight into the natural history and treatment outcomes of these rare tumors. Rosette-forming glioneuronal tumors have aggressive potential.While usually stable after resection, RGNT has been shown to occasionally recur.Because the current literature on RGNT is still so limited, little is definitively understood about the epidemiology, anatomical distribution, and even treatment of these tumors, especially since there is a subset of cases with tumors that recurred. Not only does our case and review of the literature unveil the more aggressive clinical course that is possible with these tumors, but it also highlights other unique characteristics. For instance, our case involves fairly novel locations; there are only 13 other documented cases of the tumor in the third ventricle and only 2 others involving the temporal lobe. We also found that the gender preference, previously documented as at least a 2:1 females:males, was actually closer to 1:1 . Other important findings are detailed in the manuscript.1 In 2007, the RGNT was designated as a World Health Organization (WHO) Grade I tumor and described as a slow-growing tumor typically localized to the fourth ventricle and composed of neurocytes that form neurocytic rosettes and glial components similar to those of the pilocytic astrocytoma.2 In the 2016 WHO classification, the RGNT was again classified as a Grade I tumor and listed under the category of “neuronal and mixed neuronal-glial tumors.” 3 Various other anatomical sites of origin have been reported, including the supratentorial ventricles,4 pineal gland,5 hypothalamus,6 optic chiasm,8 and spinal cord.9–11 Due to the rarity of the RGNT, little is known about its epidemiology and natural history. In this study, we present the case of a man with autism spectrum disorder and epilepsy who was diagnosed at 19 years of age with a RGNT. His prior MRI demonstrated stable appearance of nonspecific cortical abnormalities of the left temporal lobe that were thought to be related to his epilepsy. His case highlights the natural history of RGNT and presents a tumor in an uncommon anatomical location spanning from the temporal lobe into the basal ganglia extending to involve the cerebellum. In addition, we report the results of a systematic review of the literature pertaining to this rare neoplasm, analyzing key features of RGNT.The rosette-forming glioneuronal tumor (RGNT) is a rare, low-grade, primary CNS tumor that was first described in 2002 in a case series by Komori et al., in which the authors differentiated RGNT as a distinct entity from the dysembryoplastic neuroepithelial tumor.Patient consent was obtained for inclusion in this case report. No identifying details have been disclosed.A 19-year-old man with a history of autism spectrum disorder and simple partial epilepsy manifested by occasional generalized tonic-clonic seizures since childhood presented to our emergency department. He was first diagnosed with infantile spasms around the age of 3 months with up to 8 episodes per day and began antiepileptic therapy at that time. His childhood seizure semiology consisted of episodes of gaze deviation toward his right hand and right arm jerks, lasting 2–3 min. He also had repeated episodes of looking at his right hand, collapsing toward his right side, and heavy breathing. These episodes occurred 2–3 times weekly but were interspersed with asymptomatic periods lasting for several weeks. At the age of 6 years in 2005, the patient underwent an MRI during epilepsy workup, which revealed no pathological findings. Another MRI in 2013 demonstrated left temporal lobe cortical abnormalities with extension into the deep gray-white matter. The initial impression was focal hemimegaloencephaly of the mesial left temporal lobe with some polymicrogyria/pachygyria complex or possibly gliomatosis cerebri as read by neuroradiologists. There was no concern for neoplasm at the time. An MRI in 2016 showed the stable cortical lesions with a slight increase in the T2 signal in the superior cerebellar vermis.In 2019, at age 19, the patient presented acutely with hydrocephalus manifested by a 2-week history of vomiting and progressive lethargy. Prior to this, he spent his days playing video games and was able to ambulate without difficulties. Upon presentation to the emergency room, he was very lethargic and had difficulties walking, repeatedly leaning, and falling toward his left side. MRI findings at this time showed dramatic expansion of the previously stable left temporal lobe lesion with involvement of the left gangliocapsular region, bilateral thalami, tectum, and cerebellum. In addition, a new nonenhancing cystic component within the left foramen of Monro and third ventricle was identified, resulting in acute obstructive hydrocephalus. The MRI scans from 2013, 2016, and 2019 are shown in On the day of presentation, an external ventricular drain (EVD) was placed to alleviate his hydrocephalus, resulting in mental status improvement. Shortly thereafter, an endoscopic biopsy of the third ventricle lesion along with septum pellucidotomy was performed. The initial evaluation of the pathology demonstrated concern for possible RGNT. However, only the rosette-forming neuronal component of the tumor was present within the nonenhancing third ventricular component. Given these results and the extensive nature of the tumor, a second procedure was planned.The patient underwent both left temporal craniotomy for mesial temporal lobe tumor resection and frontal interhemispheric craniotomy for resection of the intraventricular tumor components. There were no complications intraoperatively, and the patient was transferred to the neurological intensive care unit in stable condition.His final pathology was consistent with RGNT. The postoperative exam was significant for central fever and autonomic dysregulation likely secondary to the tumor and its resection near the hypothalamus. Attempts were made to wean his EVD. However, a follow-up CT scan showed an interval increase in the size of the left lateral ventricle. Symptoms of hydrocephalus continued, and the patient subsequently underwent ventriculoperitoneal shunt placement.The patient developed a proximal shunt malfunction later that month requiring revision. Gross examination demonstrated tissue buildup within the ventricular catheter fenestrations. Approximately 3 weeks later, the patient again presented with worsening hydrocephalus. Repeat revision of the proximal catheter and shunt valve was performed. At 8 months follow-up, the patient was clinically stable and back to baseline function.4–82 Patient demographics, presentations, MRI features, tumor location, treatment, and follow-up of all 130 cases (when available) were extracted and summarized. Patient presentations, genetic characteristics of tumors, recurrence analysis, and surgical follow-up are shown in A comprehensive literature review using PubMed and Web of Science was performed through November 2019 following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Articles related to RGNTs were identified using the search term “rosette-forming glioneuronal tumor.” Upon removing duplicates, titles and abstracts were screened for relevant articles. English, full-text case reports and series were included. Non-English, abstract-only text, and review articles were excluded. Studies without mention of histopathological confirmation of the RGNT were further excluded. Ultimately, 80 references meeting the selection criteria were included.3 although many authors report sudden onset of aggressive behavior after prolonged periods of stability. While the most cases of RGNT have an excellent postresection prognosis without any deterioration, the outcome of our illustrative case and systematic review call this indolent nature into question. Our patient’s 6 years of MRIs demonstrate that RGNT has the potential to become rapidly progressive and infiltrative after years of dormancy. Our patient’s tumor showed significant progression on his scan an additional 3 years later. This MRI also revealed a second tumor in the foramen of Monroe. This type of progression is not unique to our case. Of the 91 cases in our review that reported follow-up, 13 demonstrated either recurrence  or rapid progression/dissemination, totaling 14% of the cases with reported follow-up.The RGNT is a rare neoplasm, with 130 patients across 80 studies having been identified in the literature. RGNT is currently classified as a benign tumor,25 A case described by Cabezas et al. presented with a secondary intraspinal lesion and leptomeningeal spread of the tumor.21 Silveira et al. also described the dissemination of tumor with drop metastasis in the lumbar spine only 1 month after initial imaging and biopsy of the tumor.69 Other reports have described similarly quick tumor recurrences postoperatively after 4 months6 and 9 months.75 However, recurrences have also been reported as late as 9 years after initial resection.28 Morris et al. described a particularly aggressive RGNT case in a 6-year-old boy.57 In this case, the tumor progressed and extended into the proximal cervical spinal cord 2 years after initial treatment involving both resection and chemotherapy.57 Another round of chemotherapy did not stop the tumor from further progression, so the tumor was totally resected.57 Unfortunately, the tumor recurred yet again 3 years later.57 There are even 2 cases that describe an RGNT with malignant transformation to glioblastoma after several years.49 As a greater number of cases are reported, it appears that some RGNTs may not be as indolent as previously thought.However, there is much inconsistency with regard to the natural history of these tumors. Chiba et al. described a tumor that was only biopsied at first but demonstrated rapid progression of the tumor as well as a new lesion in only 6 months.26 The mean age of diagnosis is 29.8 years, which is very similar to earlier findings.25 However, we noted a much less dramatic gender preference with a female:male ratio of 1.1:1  in contrast to previous reports describing a female predominance closer to 2:1.76RGNT most commonly arises in young adults, as in the case of our 19-year-old patient. However, the youngest reported patient was 4 years old, and the oldest was 81 years old.Common presenting symptoms of RGNT from our review include headache (71%), ataxia/gait disturbance (25%), vomiting/nausea (24%), visual disturbances (21%), and vertigo/dizziness (16%). Several cases were also found incidentally (13%). Presenting symptoms are displayed in 80 and only 6 others had involvement of the cerebellum.76 The appearance of the second mass is also notable, especially with its growth into the third ventricle and foramen of Monro. While there have been 13 other reports of RGNT invasion of the third ventricle, to our knowledge, this is the first case to demonstrate invasion and blockage of the foramen of Monro.The location of our patient’s tumor makes this case unique from other reports. The original lesion remained stable in the patient’s temporal lobe before progressing to involve the gangliocapsular region, bilateral thalami, tectum, and cerebellum. To our knowledge, only 2 other cases report any involvement of the temporal lobe with RGNT,60 and Chakraborti et al. showed an evidence of differentiation of stem cells in their case series on RGNT.83 Many genetic mutations have also been found in association with RGNT. The most common genetic marker associated with RGNT is the PIK3CA mutation.85  FGFR1 mutations have also been implicated in tumor pathogenesis84–86 as well as IDH1,40  KIAA1549/BRAF gene fusion,20  PPP1R1A,51 and RNF21.51The origin of RGNT has yet to be elucidated due to the rarity of the tumor and its indolent course. It has been hypothesized that the neoplasm originates from pluripotent cells of the subependymal plate,PIK3CA and FGFR1. However, a recent retrospective cohort study was able to obtain tissue samples of 18 different patients with RGNT for comprehensive genomic testing.84 Sequencing of those samples revealed that all 18 contained an FGFR1 mutation with 13 carrying a comorbid PIK3CA mutation. The findings of this study confirm that constitutive activation of the FGFR signaling likely plays a role in a large portion of these tumors. Because constitutive PI3K pathway activation (caused by PIK3CA mutation) has also been implicated in several of the cases in our review as well as in the previously mentioned cohort study, RGNT may commonly be a multiple-pathway disease, which is typically more characteristic of high-grade tumors.84 The findings of this study were not included in our genetics summary table , 62 underwent GTR (53%), 32 underwent STR (28%), 7 underwent a partial resection (6%), and 4 underwent a biopsy only (3%). Other adjunctive treatments included radiotherapy  and chemotherapy . Though there is not a large enough sample size of recurrences to draw significant conclusions regarding the most effective treatment plan, Morris et al. postulate a pattern of delayed recurrence of tumor for patients that receive GTR as opposed to earlier recurrence for those that receive STR.57 Although the results of our analysis are not quite as striking, the average time to tumor recurrence for patients that underwent GTR was longer at 6.17 years compared to 2.92 years to recurrence for patients that underwent STR. However, even with this small sample size, the probability of recurrence approximates 10% with either GTR or STR. Thus, while RGNT might have a more aggressive nature than previously thought, aggressive resection in all cases may not be warranted. Based on the potential for a rapidly progressive clinical course, it is our opinion that gross total resection should be pursued when feasible. Surgical follow-up data for each case, including location and treatment, are displayed in Currently, surgery is the mainstay of treatment. Because RGNT has historically been thought of as indolent, gross total resection (GTR) is typically only attempted if there is very low risk of damaging surrounding structures. Subtotal resection (STR), partial resection (PR), and simple biopsy with observation have all been employed for these tumors due to the risk of doing more harm with unnecessary aggressive removal.57 In this case, 4 cycles of vincristine, etoposide, and carboplatin were given after the initial resection, which resulted in stable tumor remnant for 2 years. When the tumor recurred and extended into the spinal cord, another chemotherapy regimen was attempted using weekly vinblastine for 6 months, which did not halt the progression of tumor growth.57 However, all other reported uses of chemotherapy (3) and all reported uses of radiotherapy (9) in cases of RGNT have yielded stable tumor remnant and no recurrence for varying lengths of follow-up.The effectiveness of chemotherapy and radiotherapy as primary or adjunctive therapy remains unclear. These therapies have very rarely been applied to RGNT treatment and typically only in cases of recurrent or disseminated tumors. We identified only one case, described by Morris et al., in which chemotherapy was not effective at limiting the rapid spread of RGNT.RGNT is a rare low-grade central nervous tumor usually localized within the fourth ventricle that is typically managed with surgical resection and often remains stable at follow-up. We present a case with stable tumor size and character on imaging for 3 years and sudden progression with a development of a secondary lesion 3 years later. Upon review of the literature, there are many examples of rapid progression, recurrences, and even malignant transformation, calling RGNT’s indolent reputation into question. There is insufficient evidence to support adjunctive treatment such as chemotherapy and radiotherapy in prevention of recurrence, but GTR and STR seem to have similar effectiveness as measured by patient survival and tumor recurrence. Further research is necessary to uncover the pathogenesis and genetic basis of RGNT so that better targeted therapies for aggressive cases that may be developed.vdaa116_suppl_Supplementary_Table_1Click here for additional data file.


Esophageal leiomyosarcoma (LMS) is a rare tumor that constitutes less than 1% of all malignant esophageal tumors. Concurrent occurrence of esophageal leiomyosarcoma with squamous cell carcinoma (SCC) is even rarer than isolated leiomyosarcoma.In this report, we present a case of concurrent leiomyosarcoma and SCC in a 64-year-old woman presenting with vomiting and solid dysphagia, which has not been properly diagnosed following several referrals and diagnostic modalities. At last Exploratory laparotomy with gastric pull-up was performed in addition to radical laryngectomy with partial resection of the esophagus and subtotal thyroidectomy. Pathologic evaluation of the surgical specimen showed concurrent LMS (5.2 × 4.5 × 3 cm) and SCC (1.5 × 0.6 × 0.6 cm) at the same anatomical level in the proximal esophagus.This study proposes the importance of using ancillary diagnostic tests such as immunohistochemistry (IHC) to diagnose less common cases such as concurrent LMS and SCC. Esophageal carcinoma is the eighth most common cancer worldwide with a poor prognosis due to its overly aggressive nature . The twoLeiomyosarcoma (LMS) is a high-grade slow-growing smooth muscle connective tissue tumor with late metastasis observed in different organs, such as the gastrointestinal tract and uterus.. There aThe patient was a 64-year-old woman with seven children brought to the hospital with severe vomiting and a history of solid dysphagia; she had the symptoms for three months and was referred to different physicians, left without definite diagnosis before admission to our center.. Patient’s past medical history was unremarkable. Post-contrast computed tomography (CT) of the neck showed two lesions (kissing tumors) in the upper esophagus, causing the tracheal shift to the right side with no lymph node enlargement Fig. . PhysicaThe patient was scheduled for exploratory laparotomy with the exploration of cervical organs, resection of the pharynx, thyroid , and esophagus and gastric pull up along with the insertion of a tracheostomy and bilateral chest tube, and cervical penrose.The patient was laid in a supine position. After preparation and sterilization, the abdomen was opened by a high midline incision. The surgeon inserted his finger into the mediastinum to assess the presence of any tumors. Stomach was mobilized by harmonic ligasure, lesser sac was opened, short gastric and left gastric arteries were ligated, and gastric pedicle was made on the right gastroepiploic arteries and epigastric area. Then, the esophagus was dissected bluntly by the finger in the mediastinum through the hiatus's opening and continued upward. Adhesion to the carina was found and released meticulously. Next, the collar incision was made on the neck. Then, subtotal thyroidectomy was done, and the specimen was sent for pathologic evaluation. Trocar was introduced in the sheath over the spine, and the esophagus was gently dissected and pulled up. The esophagus' blunt dissection was also carried out from the above until two hands could reach each other via the upper and lower incisions. After the esophagus' full release, resection of pharynx, larynx, and esophagus was done, and specimens of the proximal esophagus were sent for further pathological evaluation. Then, anastomosis of the mouth floor to the stomach was performed with polydioxanone suture 3/0, and the neck wound was irrigated and closed after insertion of a Penrose drain. The posterior part of the trachea was then fixed to the skin with Vicryl 3/0, and tracheostomy tube size 7.5 was inserted. The abdomen was irrigated, and the fascia was closed with a nylon loop one and irrigated. The skin was closed by nylon 3/0. Bilateral chest tubes No. 24 were inserted into the 5th intercostal space with a depth of 10 cm and fixed with silk 0 dressing. The surgery lasted for five hours and thirty minutes, and the patient received two packed cells and 6500 cc normal saline and had 4000 cc urination and 450 cc bleeding.The gross pathological evaluation showed two separate grey cream masses with a smooth glistening surface at the cricopharyngeal level that made a stricture, as shown in Fig. The patient was discharged after ten days of hospital admission and was referred to the radio-oncologist. The patient underwent radiotherapy and was stable at six months' follow-up with no recurrence.We presented a case with concurrent LMS and SCC in the proximal esophagus without invasion to other organs. Fortunately, the patient was appropriately treated with a radical laryngectomy, partial resection of the esophagus, and subtotal thyroidectomy. The case presented in this study had undergone several diagnostic investigations, including ultrasound, endoscopy, FNA, and pathologic examinations, leaving the patient without a definite diagnosis. As the presence of a tumor was suspected, we performed exploratory laparotomy for the patient with the exploration of cervical organs, resection of the pharynx, thyroid , and esophagus in addition to gastric pull up along with the insertion of a tracheostomy and bilateral chest tubes, and cervical Penrose. This intervention was chosen considering that simultaneous resection of other solid organs and esophagectomy is considered to increase the chance of survival . The speLMS is a rare tumor of the esophagus, and there are few similar cases reported in the literature , 5. It iAs esophagus LMS symptoms are not different from other esophageal carcinomas and patients present mainly with dysphagia and/or chest pain , more acBy presenting this rare case of concurrent esophageal LMS and SCC, the authors aimed to highlight the concurrency of such a diagnosis that has a high probability of being misdiagnosed in a patient. We must also propose that such cases urge ancillary diagnostic tests such as IHC in the better evaluation of a patient's tumor and lead to a more definite and proper diagnosis.


Epithelioid sarcoma is known as one of the rarest types of sarcomas and was identified as its own diagnosis by Dr. Franz Enzinger in 1970 after his realization of its massive overlap with many other diseases. This tumor has an aggressive clinical course with high recurrence and metastasis rates.This report will detail the case of a 39-year-old male who was diagnosed with Epithelioid Sarcoma and later succumbed to this disease.This report will emphasize epithelioid sarcoma morphology and immunohistochemistry with discussions on predisposition, prognostic factors, and current options for treatment modalities.Future studies are needed to determine clear predisposition and screening practices; however modern pharmaceuticals have shown hopes of optimizing the course of this cancer. They moEpithelioid sarcomas were most commonly confused with synovial sarcomas until Dr. Enzinger realized these tumors didn’t fit that exact picture . And eveThe intended purpose of this case report is to discuss the genetic predisposition, prognosis, and treatment, and ultimately aid as an educational tool for optimal patient-centered care. This work has been reported in line with the SCARE criteria .2In 2016, a 39-year-old male presented to the general surgery outpatient office with a three-month history of a right-hand mass at the base of thumb and a right axillary mass. The hand lesion had caused him pain and numbness of the hand and lateral aspect of the second digit. The axillary lesion had caused him a pulsatile, sharp pain when he laid down. Previously, the hand lesion had been attributed to scar tissue, until the axillary mass appeared, to which more workup was pursued. MRI of the brachial plexus was performed and showed a 5.3 × 9.4 × 8.8 cm lobulated right axillary mass and involvement of the right lateral chest wall inferior to the right brachial plexus. It demonstrated increased heterogenous, focal nodular intensity, and scattered internal non-enhancement, suggestive of necrosis. An MRI of the right hand showed a heterogenous soft tissue mass, near the median nerve. An excisional biopsy confirmed metastatic epithelioid sarcoma from a distal-type tumor at the right hand. Immunohistochemistry showed positivity for tumor biomarkers AE1, AE3, EMA, vimentin, and CD34 and negativity for INI1 and S100. When treatment was discussed, he declined all therapy. CT showed metastasis to lung and suspicious metastasis to thoracic, lumbar, and sacral spine. The patient succumbed to his disease three months later.3Epithelioid sarcomas are a mixture of both mesenchymal cells and epithelial cells ,2,7. AndRefer to TableTable 11 [It is also common to see negative biomarkers: S-100, CD31, and SMARCB1/INI1. Up to 90% of epithelioid sarcomas have a homozygous loss of SMARCB1/INI1 expression ,7,8, conTable 11 ,7.Table Overall, there is incredible overlap between malignant rhabdoid tumors and epithelioid sarcomas , puttingMalignant rhabdoid tumors are considered hereditary SWI/SNF complex deficiency syndromes , which dAlthough these tumors are similar, there may be a strong possibility that the age at which SMARCB1 inactivation occurs is what dictates the earlier onset and predisposition to malignant rhabdoid tumors versus epithelioid sarcomas .Thus, in patients with epithelioid sarcoma, genetic screening is not indicated, as family history has not been shown to be a risk factor. Instead, epithelioid sarcomas etiology remains unknown, posing a potential cause as scar tissue or trauma ,3,5.The ability to determine prognosis at the time of diagnosis can dictate the quality of life and intervention. According to a clinicopathological analysis of 106 epithelioid sarcomas: having a proximal-type tumor, a tumor size greater than 5 cm, tumor multifocality, and high mitotic index had a shorter five-year survival rate; additionally, tumors greater than 5 cm and a high mitotic index had a shorter five-year metastasis-free survival rate . SimilarIt is also worth noting that in the past, it was advised against using a grading system for epithelioid sarcomas. But recently, FNCLCC grading system established prognostic value . There iGiven the aggressive nature of this tumor, early surgical management is the treatment of choice , where rChemotherapy has recently shown progress, especially adriamycin, vincristine, cytoxan, actinomycin D, and methotrexate . While sRecently, a wave of biomarker-targeted therapy has provided new opportunities to tackle treatment, especially targeting AKT/mTOR pathway. Losing SMARCB1 leads to hyperactivation of the AKT/mTOR pathway, which is involved in cell proliferation and cancer survival . EveroliLastly, in a current clinical trial, an oral EZH2 inhibitor, tazemetostat, has shown a 26% disease control rate, defined as any duration of stable disease lasting ≥ 32 weeks . EZH2 is4Epithelioid sarcoma is a rare soft tissue tumor that poses a challenge due to its aggressive nature and broad morphology. Certain tumor biomarkers have even been identified as either present or absent on immunohistochemistry. It is important to determine predisposition, prognosis, and treatment once diagnosed, with wide surgical excision as the mainstay and new therapeutic techniques on the horizon.The authors declare that there are no conflicts of interest regarding the publication of this article.The authors declare that there was no involvement of funding for this research.This study has been exempt from ethical approval by Lake Health West Medical Center.Written and signed consent has been obtained; statement of consent is present at the end of manuscript.•Avalon Regalbuto : study concept and design, obtainment of patient consent, data collection, literature search, writing and editing of the paper, manuscript preparation for publication•Andrew Tudosie : obtainment of patient consent, data collection, literature search, writing and editing of the paper, manuscript preparation for publication•Eveline Klenotic : obtainment of patient consent, editing of the paperAuthor roles have been further defined by the CRediT criteria on Page 1 of the manuscript.1.Name of the registry: Research Registry2.Unique identifying number or registration ID: researchregistry53953.https://www.researchregistry.com/browse-the-registry#home/registrationdetails/5e5c299f55b3d20019743b8c/Hyperlink to your specific registration (must be publicly accessible and will be checked): Avalon Regalbuto.Guarantor for this manuscript: Not commissioned, externally peer-reviewed.Avalon Regalbuto: Conceptualization, Investigation, Resources, Writing - original draft, Writing - review & editing, Visualization, Supervision, Project administration. Andrew Tudosie: Investigation, Resources, Writing - original draft, Writing - review & editing, Visualization. Eveline Klenotic: Investigation, Writing - review & editing.


A 29-year-old woman with a 5-year history of primary infertility underwent in vitro fertilization-embryo transfer (IVF-ET) treatment. Hemorrhagic shock caused by retroperitoneal hematoma after oocyte retrieval was treated promptly by the evaluation of diagnostic laparoscopy and angiography. The patient was recovered and discharged from the hospital 7 days later without any complications. She was later diagnosed with Von Willebrand disease by a hematologist. The first transvaginal ultrasound-guided oocyte retrieval has been performed since 1983 . SiristaA 29-year-old nulligravida woman underwent assisted reproductive technique in our hospital because of 5-year history of infertility. The patient medical history was bilateral obstruction, she had no obstetrics history and had a regular menstruation, normal endocrine profile, no personal and family history. Her blood coagulation profile, blood routine and biochemical tests were normal.She started to ovulate with gonadotropin (Recombinant Human Follitropin Alfa for Injection) and antagonist (Cetrorelix Aacetate powder for Injection). a scheduled oocyte retrieval via vaginal ultrasound under the intravenous anesthesia after 36 hours of human chorionic gonadotophin administration. Nine oocytes were obtained, everything went well in the procedure. About fifteen minutes later, the cardiogram monitor showed a rapid increase in heart rate to 120–150 per minute and the blood pressure was dropping from 120/70 mmHg to 85/40 mmHg. The patient was still in sedation with anesthesia, she was appeared to be pale and could be waken up complaining abdomen pain and vomiting. Her abdomen was distended and diffusely tender, there was no significant rebound in the lower abdomen, no obvious mobile dullness. Vaginal examination revealed that there is no bleeding in the puncture point, the posterior fornix of the vagina is full and tender. Transvaginal sonography revealed a 3-4 cm free fluid in pelvic, blood gas analysis revealed her hemoglobin concentration was 7.6 g/L. She was immediately given 1 gram of intravenous tranexamic acid, 1 unit Hemocoagulase Atrox for injection and 2 unit of packed red blood cell. Preoperative preparation has been done and the patient agree to laparoscopy procedure.Laparoscopy revealed about 200 ml pelvic free fluid, there was no evidence of active bleeder from pelvic vessels, a retroperitoneal hematoma 6-7 cm in diameter up to the left iliac fossae down to 5 cm above the uterine rectal fossae . The sigThe retroperitoneal hematoma appeared stable and no expanding, also no increase in tension. The hemoglobin concentration reexamination showed no significant change. Considering the bleeding has stopped, the trauma surgeon recommended no further incision for the retroperitoneal hematoma after consultation. Digital subtraction angiography (DSA) was done after operation to further confirm no more artery bleeding. DSA revealed the inferior mesenteric artery and the iliac artery were intact  and 3. TShe was discharged from hospital after seven days without any complication. The patient recovered well and the retroperitoneal hematoma disappeared within one month. She was referred to see hematologist and was diagnosed with Von Willebrand disease (VWD).To our knowledge this is the first emergent case of hemorrhagic shock with retroperitoneum hemorrhage after oocyte retrieval, a case had been managed conservatively with retroperitoneal hematoma during laparoscopy. Two previously reported cases of retroperitoneal hemorrhage occurred after oocyte retrieval. Foad Azem reported a 42-year-old woman with retroperitoneal hematoma 7 cm in diameter 6 hours after oocyte retrieval. Laparoscopy revealed the injury of the mid sacral vein. The hematoma was removed and a mental clip was used to control the active bleeding . The retAragona reported that clinical factors related the bleeding complication maybe the factor IX deficiency, ovarian necrotizing vasculitis and anticoagulant treatment, surgical experience is also an important factor . VWD is The management of retroperitoneal hematoma is quite challenging. It is not recommended except the organic injury, the increase tension or with a pulse of the hematoma . OtherwiIn conclusion, although transvaginal ultrasound oocyte retrieval is a general technology in assisted reproductive technology, It is necessary to monitor patients closely after oocyte retrieval for physician. Any changes in vital signs and complaints of abdominal discomfort should be taken seriously, Even there is no significant pelvic free fluid, retroperitoneum bleeding should also be considered especially to those people with abnormal coagulation.None declared.Supported by Qingdao Key Health Discipline Development Fund.